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pmc-6452523-1 | A 39-year-old man presented to our outpatient clinic to undergo ICL implantation surgery. He had no history of systemic or ocular diseases other than refractive error. His preoperative uncorrected visual acuity (UCVA) was 20/500 in each eye, while his distance-corrected visual acuity (DCVA) was 20/13 (S, −7.00; Cyl, −1.75 × 20°) in the right eye and 20/13 (S, −6.25; Cyl, −2.5 × 10°) in the left eye. The intraocular pressure (IOP) was 16 and 15 mm Hg, and the corneal endothelial cell density was 3118 and 3318 cells/mm2 in the patient's right and left eyes, respectively. In the right and left eye, respectively, the pupil sizes were 8 and 8 mm in a dark examination room, and 5.7 and 5.8 mm in a bright examination room; the anterior chamber depths (from the corneal endothelium to the anterior lens capsule) were 3.58 and 3.55 mm, the white-to-white (WTW) diameters were 11.4 and 11.4 mm, the sulcus-to-sulcus (STS) distances were 11.7 and 11.68 mm, and the corneal thicknesses was 539 and 528 μm. Two months before the toric ICL (TICL) implantation surgery, laser iridotomy was performed in both eyes. Based on the calculations of the TICL software (STAAR Surgical Company, Monrovia, CA, USA), we chose the TICMV4 model, with a power of −13.5 + 3.5 × 89° in the right eye and −13.5 + 2.5 × 101° in the left eye, and a diameter of 12.6 mm in both eyes. The TICLs were implanted without any complications at 16° and 0° anticlockwise from the horizontal meridian in the right and left eyes, respectively. In both eyes, the 3-month postoperative UCVA was 20/10; the DCVA was also 20/10 (S, +1.00; Cyl, −0.50 × 135° in the right eye and S, +0.50; Cyl. −0.50 × 20° in the left eye). The IOP was 12 and 13 mm Hg, respectively, and the corneal endothelial cell density 3 months after surgery was 3125 and 3086 cells/mm2. The central vault, which is the distance between the TICL and crystalline lens, was 0.9 and 1.05 mm 3 months after surgery. Although the central vault was high, we followed it without surgical interventions because the angle was open and there were no complications such as IOP rise, iris contact with the corneal endothelium, or pigment dispersion syndrome. Eleven months after the initial ICL implantation surgery, the patient developed blurred vision in his right eye after being struck by another player's hand during a futsal match. The patient presented to the outpatient clinic the day after the injury. The UCVA in his right eye was 20/13, while the DCVA was 20/13 (S, +1.25; Cyl, −0.50 × 130°). Slit-lamp examination revealed mild inflammation in the anterior chamber, and both the superior and inferior temporal haptics of the ICL were dislodged into the anterior chamber and entrapped within the pupil, without touching the corneal endothelium (Figure A). We performed repositioning surgery on the same day. After filling the anterior chamber with viscoelastic material, we used the ICL manipulator from the preexisting wound to reposition the ICL behind the iris. The patient used 1.5% levofloxacin, 0.1% bromfenac sodium hydrate, and 0.1% betamethasone ophthalmic solutions for 1 week after the operation. One month after the repositioning surgery, his UCVA was 20/13 and his DCVA was 20/13 (S, +0.75; Cyl, −0.75 × 135°). The blurred vision in his right eye was resolved. The IOP in the affected eye was 13 mm Hg; the corneal endothelial cell density was 3001 cells/mm2, and the vault distance was 0.82 mm. No complications such as iris damage, cataract formation, or ICL rotation were noted. Two years and 2 months after the initial TICL implantation surgery, the patient again developed blurred vision in his right eye after being struck by another player's foot during a futsal match. The UCVA in his right eye was 20/17, and the DCVA was 20/17 (S, +0.75; Cyl, −0.75 × 135°). We observed mild inflammation in the anterior chamber, and the inferonasal haptic of the ICL was dislodged into the anterior chamber and entrapped within the pupil, without touching the corneal endothelium (Figure B). We performed repositioning surgery the next day. The patient used 1.5% levofloxacin, 0.1% bromfenac sodium hydrate, and 0.1% fluorometholone ophthalmic solutions for 1 month after the operation. One month after surgery, his UCVA was 20/10, while his DCVA was 20/10 (S, +0.50; Cyl, −0.50 × 105°). The IOP was 17 mm Hg, the corneal endothelial cell density was 3050 cells/mm2, and the vault distance was 0.79 mm. No complications associated with the repositioning surgery or trauma were observed. We observed pigment deposition on the posterior surface of the ICL after the surgery, but the amount of the pigment had not changed 1 year after injury. We explained to the patient the risk of ICL prolapse during futsal at night, advising him to use protective glasses. |
pmc-6452525-1 | A 53-year-old female was referred by her physician to the Department of Otorhinolaryngology with a swelling in her tongue that she noted after she choked on a piece of food. Biochemical signs of hyperthyroidism (TSH: 0.005 mU/mL, normal ranges: 0.27-4.20; plasma-free T4: 2.75 ng/mL, normal ranges: 0.93-1.71; plasma-free T3: 5.36 pg/mL, normal ranges: 2.04-4.40) were detected in a routine analysis three months before she choked. Positive antinuclear antibodies (ANA: positive in 1/40), antithyroid peroxidase (anti-TPO: 127, 8 U/ml, normal ranges: 0.00-34.00), anti-TSH receptor (2.22 U/L, normal ranges: 0.00-1.75), and antithyroglobulin (anti-TGB: 11.98 U/ml, normal ranges: 0.00-115.00) were also detected. Serum calcium was normal (9.95 mg/dl, normal ranges: 8.6-10.4).These features are consistent with Graves’ disease, although the patient had none of the classical symptoms of this disease. Her familial history was unremarkable, without antecedents of endocrinal or genetic diseases. On physical examination, a nodule bulging under the posterior aspect of the tongue was observed. Neither dysgeusia nor dysarthria were detected. Head and neck computed tomography (CT) scan evidenced a nodule at the base of the tongue with intense enhancement after contrast administration, suspicious for a hemangioma (Figure ). No other masses were detected on the head and neck area. Thyroid gland ultrasound identified two nonspecific millimetric nodules in a normal sized gland. Radioiodine scan showed an orthotopic thyroid gland with a diffusely increased uptake. After the diagnosis, a thoracoabdominal CT scan ruled out adrenal or extra-adrenal masses. The patient underwent an endoscopic transoral resection of the lingual nodule (Figure A). Healing was uneventful.
The surgical specimen was fixed in 10% buffered formalin for routine histology and immunohistochemistry. The latter was performed using the avidin-biotin technique. Tested antibodies (all from Dako, Glostrup, DK-Denmark) included chromogranin A, neuron-specific enolase (NSE), S-100 protein, CK AE1/AE3, smooth muscle antigen (SMA), CD-31, carcinoembryonic antigen (CEA), thyroglobulin, calcitonin, TTF-1, HMB-45, and Ki67. Immunohistochemistry and sequencing analysis for SDHx mutations were performed in another center using anti SDH B rabbit polyclonal antibody (Sigma-Aldrich Corp). SDHA was also tested on tumor sample as previously described. For molecular analysis, Sanger and next-generation sequencing (NGS) were applied. It was used a TruSeq Custom Amplicon 1.5 Kit (Illumina, San Diego, CA) to study DNA from tumor and blood samples as previously described.
The surgical specimen consisted of a rubbery brown nodule, measuring 17 × 15 × 15 mm, with well circumscribed borders, partially covered by an intact mucosa. Microscopic examination revealed a highly vascularized tumor composed of nests of uniform cells with finely granular cytoplasm and round nuclei without atypia, mitosis, or necrosis. These cells expressed chromogranin A and NSE. A second population of S-100 positive sustentacular cells were detected at the periphery of the nests with the classical “zellballen” appearance described in paraganglioma (Figure B,C). The tumor was focally transected by surgical margins. Other markers tested for differential diagnosis are summarized in Table . Ki 67 showed a very low proliferation rate (<1%). Other potentially associated tumor components derived from the neural crest, such as ganglioneuroma and melanocytes, were not identified. Immunohistochemistry for SDHB was negative (Figure D) while SDHA was positive. This result suggested the presence of a mutation in any of the SDHx genes but SDHA. Mutational analysis confirmed a c.689G>A/p.Arg230His mutation in SDHB gene. |
pmc-6452528-1 | This is the case of an 85-year-old female who presented with a strangulated recurrent rectal prolapse less than a month after a Delorme surgical repair. During the first encounter with her surgeon for her rectal prolapse, she was offered the Delorme intervention to avoid any surgical stress due to her associated Takotsubo syndrome that was diagnosed early in 2016. No preoperative radiological examinations were performed, and the patient was operated on in December 2016.
Shortly thereafter, she was referred to our department by her family physician for a recurrent prolapse of 10 cm. At our clinic, the prolapse was reducible and the anal tone was weak with barely any tone while squeezing. The patient was hardly passing stool since the recurrence of the prolapse.
A corrective mesh ventropexy (D'Hoore) surgery was proposed, and the patient gave consent.
On admission day, the prolapse was even more exteriorized at approximately 20 cm, with circumferential necrosis of its extremity of approximately 10 cm. The suture line of the previous operation was visible and intact (Figure ). The patient complained of abdominal discomfort and constipation. The prolapse was irreducible, and the patient was in a subocclusive state.
Because of our clinical findings, and after combining the weak anal tone and the necrosis, we decided to proceed with a Hartmann intervention after obtaining consent from the patient, primarily to avoid fecal incontinence for a better quality of life.
An infraumbilical laparotomy was performed. The patient was in obstruction status caused by a colorectal intussusception, due to a very redundant sigmoid (Figure ).
A manual reduction of the prolapse was possible by pushing from the outside and pulling on the sigmoid from the inside of the abdominal cavity. Upon examining the rectum, we found patches of necrosis and a sealed perforation.
We performed a Hartmann procedure leaving a very short rectal stump, with a left terminal colostomy.
The postoperative period was short and uneventful.
The histopathology report showed severe ulcerations and acute inflammatory response. No other pathology was detected.
The patient was seen 1- and 6-month postoperatively. She was doing fine and able to handle her colostomy with the help of her son. No residual prolapse was seen, and the digital rectal exam showed the very weak sphincter tone and a 3-cm rectal stump. |
pmc-6452529-1 | A 39-year-old male with a past medical history of polysubstance abuse on methadone presented to the emergency department (ED) for worsening shortness of breath. He was recently diagnosed with Hodgkin's Lymphoma within the past month, after initially presenting with a left-sided chylous pleural effusion. He had required multiple thoracenteses over the past three weeks prior to admission to the hospital and was instructed to start a <20g/day fat diet due to the pleural fluid triglyceride level of 775mg/dL with 74% lymphocytes. The patient had started chemotherapy with Adriamycin, Adcetris, Vincristine, and Dacarbazine two days prior to presenting to the ED at his outpatient oncologist's office. In the ED, he was found to have an oxygen saturation of 88% on room air, which improved to 92% on 4L nasal cannula. The patient was frail appearing with temporal and diffuse muscle wasting with moderate respiratory distress. A chest radiograph was obtained, showing moderate bilateral pleural effusions that had reaccumulated over the past week since his last thoracentesis (). The patient was evaluated by Cardiology while still in the ED and a STAT transthoracic echo was performed () showing compression of the right atrium and right ventricle during diastole. Cardiothoracic surgery was immediately consulted and the patient was taken for an emergent pericardial window.
A pericardial window was created with drainage to the right pleural space. Approximately 500mL of chylous fluid was drained from the pericardial space, 2L of similar fluid drained from the right pleural space, and 2.5L of chylous fluid was drained from the left pleural space through placement of a tunneled pleural catheter. Intraoperatively, pleural studding was noted throughout the right parietal pleura and pericardium. Biopsies were taken followed by talc pleurodesis. Pleural and pericardial fluid was sent for analysis, which showed a triglyceride level of 135mg/dL. The patient also had a serum triglyceride level of 105mg/dL. The patient was brought to the ICU and started on a fat-free, clear liquid diet and Octreotide 100mcg every 8 hours subcutaneously. Overnight to the morning of postoperative day (POD) 1, the patient drained another 1.9L of chylous fluid from the left chest tube () and 1L from the right chest tube. Both tubes were clamped to prevent further volume and protein loss. His diet was advanced to a regular <10g/day fat diet and a PICC line was placed to start total parenteral nutrition (TPN) in order to supplement nutrition with the total elimination of long chain fatty acids from the patient's diet. The patient had his chest tube clamped overnight with subsequent drainage every morning up to 1L from each side in order to limit further fluid and nutrition loss given his frail state. On POD 2, the patient was started on TPN and had 1.3L of chylous fluid drained from his left and 1L from his right chest tube. On POD 3, 1L of chylous fluid from the left and 900cc of chylous fluid was drained from the right. The patient was clinically stable and was downgraded from the ICU to telemetry where the chest tubes continued to be drained for 1L bilaterally and fluid color improved from milky white to serous. Repeat pleural fluid testing on POD 5 showed that the triglycerides had decreased to 46mg/dL and the patient's chest radiograph continued to improve. TPN was stopped and he tolerated 100% of his nutrition on an oral <10g/day fat diet. The patient's right chest tube was removed after output decreased to 250cc in 24 hours, and the output from the left tunneled pleural catheter continued to drain 1L daily of serous fluid. The patient's imaging remained stable and he was subsequently discharged home on POD 10 with thorough education on his dietary restrictions.
Unfortunately, due to lack of insurance, the patient could not continue the subcutaneous octreotide as he was unable to afford the out-of-pocket costs of the medication. Two days after discharge, he returned to the ED complaining of dyspnea. A chest radiograph at that time showed an unchanged small loculated pleural effusion on the right and a new large left pleural effusion. Only 1L of the estimated 2.5Ls in the pts left pleural space was drained from the patient's left tunneled pleural catheter which was milky white and was sent for analysis which showed a triglyceride level of 745mg/dL. He was reeducated on proper dietary habits and the importance of starting chemotherapy, which was initiated the next day. Two months later, the patient's repeat PET scan showed a significant decrease in the extent of the FDG avid foci consistent with the known lymphoma and small-to-moderate left pleural effusion which was draining <1L every 3 days at that time. |
pmc-6452533-1 | A 59-year-old gentleman with end-stage kidney disease due to hepatitis C with focal segmental glomerulosclerosis, on hemodialysis for seven years, underwent deceased donor renal transplantation. The donor kidney had a KDPI of 60%. There was a 4 antigen HLA mismatch with Class II panel reactive assay (PRA) of 62.31%. Class I PRA was 0%. His donor specific antibody testing was positive for an antibody to DQ7. The donor had died as a result of anoxic brain injury following cardiopulmonary arrest resulting from anaphylactic shock. The cold ischemic time was 12 hours, 7 minutes and the warm ischemic time was 51 minutes. Basilixumab was used for immunosuppression induction and the patient underwent early steroid withdrawal. His maintenance immunosuppression was tacrolimus (targeting trough levels 5-8) and mycophenolate sodium 720 mg BID.
The patient's postoperative course was complicated by delayed graft function, necessitating continuation of renal replacement therapy. He was hemodynamically stable throughout his hospital course. A renal allograft ultrasound showed good blood flow to the entire kidney with a resistive index of 0.64. A repeat flow crossmatch was negative, but he remained positive for a persistent low-level donor specific antibody to DQ7 (1000 MFI). He underwent a renal transplant biopsy on his 4th day postoperatively. This showed extensive acute tubular necrosis with associated peritubular capillaritis and interstitial nephritis (). Oxalate crystals were seen in several tubules. One large caliber artery showed active endothelialitis, but no tubulitis or glomerulitis seen. C4d staining was negative in the peritubular capillaries. Electron microscopy revealed minimal effacement of podocyte foot processes. The patient received methylprednisolone 500 mg x 3 doses to treat moderate acute cellular rejection. His tacrolimus dose was also optimized as his trough levels had been running low at between 3.5 and 6. He continued to take mycophenolate sodium at a dose of 720 mg BID. A decision was made not to treat for antibody-mediated rejection given that no glomerulitis was seen and that there was minimal capillaritis with a negative C4d stain.
He remained dialysis dependent with minimal urine output for three weeks after transplantation. A urine protein : creatinine ratio was elevated to 1070 mg/g when assessed after hospital discharge when his urine output started to gradually improve. His urine microalbumin : creatinine ratio was 450 mg/g. A decision was ultimately made to readmit the patient on day 12 after transplantation for IV thymoglobulin 1.5 mg/kg to treat his Banff Type IIa T cell mediated rejection, given his lack of response to pulsed IV steroid treatment. Given lack of improvement in renal function and urine output by day 16 after transplantation, a second renal allograft biopsy was performed. This revealed residual acute tubular necrosis with associated mild peritubular capillaritis and interstitial nephritis (). Extensive oxalate crystals were now visible in many tubules. His plasma oxalate level was concurrently elevated to 19.3 μmol/L (reference range ≤ 1.9 μmol/L). No signs of persistent acute antibody- or cell-mediated rejection were seen. Again, there was minimal segmental effacement of the podocyte foot processes seen on electron microscopy and no evidence of recurrent focal segmental glomerulosclerosis present.
He commenced calcium citrate along with dietary oxalate restriction to manage his hyperoxaluria and his serum creatinine improved to a nadir of 1 mg/dl (88 μmol/L), with a concomitant gradual reduction in his proteinuria and microalbuminuria to undetectable levels. Of note, the patient had no history of malabsorptive intestinal disease and denied any GI symptoms throughout this time period. He had never suffered from renal calculi. At three weeks after transplantation, his urine output and creatinine clearance had recovered sufficiently to enable him to become dialysis independent. A decision was made to continue low-dose oral steroids in the long-term given the presence of early acute cellular rejection on his first allograft biopsy. An interval renal biopsy performed 2 months later showed no ongoing evidence of oxalate deposition, tubular necrosis or cellular rejection. A concurrent repeat plasma oxalate level showed interval reduction to the normal range. |
pmc-6452535-1 | An otherwise healthy 33-year-old woman in her eighteenth week of pregnancy presented with a five-day history of high spiking fever, generalized body aches with myalgia, and frontal headache. She had noticed a generalized maculopapular rash on the third day of the illness. On the fifth day, she noticed that her hearing got progressively impaired, worst towards the end of the day. She had no seizures, alteration of consciousness, or behavioral changes. On admission to us, she was febrile and had a widespread erythematous maculopapular rash involving the palms and soles but sparing the face (). She had no eschar. There were no signs of meningeal irritation. Except for the involvement of the eighth cranial nerve, there was no other cranial nerve involvement. Other focal signs were absent, and the optic fundus was normal. She had no lymphadenopathy or hepatosplenomegaly. She could not recall any history of tick bite. Her immunization was up-to-date, and she was immunized against measles, rubella, and chicken pox in the past.
Her total white cell count was 3490/μL with 80% neutrophils and 15% lymphocytes. She had a mild thrombocytopenia of 128,000/μL. Her inflammatory markers were elevated including CRP of 120 U/L and ESR of 85 in the 1st hour. Except for a mild transaminitis (AST 58 U/L and ALT 60 U/L), rest of the liver functions were normal. Cerebrospinal fluid (CSF) analysis performed on the 6th day of the illness was normal. The audiometry studies confirmed bilateral sensorineural deafness (). Neuroimaging was not performed since the patient was reluctant to undergo computerized tomography or magnetic resonance imaging due to the pregnancy. The virology screens (HIV, HSV, CMV, mumps, measles, and rubella) and serology for syphilis were negative. The Weil–Felix test was positive with a high reactivity of OX19 and OX2 antigens. She received oral azithromycin for a total of 10 days with a presumed diagnosis of rickettsial spotted fever. With this treatment, her fever settled, and her general condition dramatically improved with the maculopapular rash gradually disappearing. The inflammatory markers came down with a CRP of <5 U/L and ESR of 35 mm in the 1st hour at the end of ten days of treatment. Hearing impairment persisted and showed mild gradual improvement after one month (). Her diagnosis of spotted fever was serologically confirmed with very high titers of Rickettsia conorii IgG (>1/450) after two weeks of the illness. After about five to six months, hearing was restored back to her normal, and by this time, she had an uncomplicated delivery. The baby did not have any physical abnormalities. |
pmc-6452536-1 | A 66-year-old female presented to the gynecology oncology clinic with a 6-month history of increasing abdominal girth, decreased energy, and a reduction in appetite. She reported a twenty pound weight gain over the preceding two months.
The patient reported a previous hysterectomy at age 28 for pelvic pain and then a bilateral salpingo-oophorectomy at age 45 for a pelvic mass. Past medical history was significant for atrial fibrillation, type 2 diabetes mellitus, hypertension, and gastro-esophageal reflux disease. Her family history was significant for a maternal aunt with a diagnosis of breast cancer at age 58 and subsequent ovarian cancer at age 90.
A diagnostic paracentesis was performed preoperatively and returned positive for an adenocarcinoma. An abdominal CT scan revealed a query 4.8 x 2.1 cm left ovarian cyst and omental cake. CA-125 was elevated at 278 U/mL (Normal High <=35). On examination, BMI was 38. Auscultation of the heart and lungs was normal. Abdominal examination showed shifting dullness. Rectovaginal examination revealed ascites, but no pelvic mass or nodularity in the cul-de-sac.
With a presumed diagnosis of ovarian cancer, the patient was presented with two options, neoadjuvant chemotherapy, or primary cytoreductive surgery. After a discussion of risks and benefits of each approach, the patient consented for primary cytoreduction.
A laparotomy was performed, and upon entering the peritoneal cavity, diffuse inflammation of the peritoneum and 12L of ascites were noted. Extensive carcinomatosis involving most peritoneal surfaces was identified with involvement of the ascending colon. Preoperatively we had queried the possibility of some residual ovary; however no ovary could be identified within the peritoneal cavity, nor with dissection into the retroperitoneum. A supracolic omentectomy, peritoneal stripping of both upper abdomen and pelvis, and right sided hemicolectomy with primary reanastomosis were performed.
In the postoperative period, the patient developed rapid atrial fibrillation and became persistently hypotensive despite aggressive fluid resuscitation. She was brought to the intensive care unit for resuscitation, but continued to decline. At the patient's request, treatment was withdrawn; she died shortly thereafter.
Pathology specimens revealed a high grade metastatic papillary serous carcinoma consistent with a primary gynecologic origin. The patient's previous surgical and pathology reports were obtained. The operative note from 1993 described that the patient had undergone a laparotomy with removal of both fallopian tubes and ovaries. Final pathology from that surgery confirmed removal of a normal left ovary and tube and the removal of a right ovary (containing a serous cyst) and a normal right fallopian tube. No borderline or malignant changes were identified. |
pmc-6452545-1 | A 60-year-old lady with satisfactory premorbid state, presented with dry cough for recent few months. She was admitted to the hospital in mid-Dec 2018 because of abdominal pain, joint pain, shortness of breath, and fever. She was noted to have renal impairment (serum Creatinine 538μmol/L) on presentation. She had normocytic normochromic anemia, hemoglobin level 7.1g/dL, and elevated erythrocyte sediment ratio (ESR) of 130 mm/h. Radiological studies revealed bilateral lung infiltrates and normal-looking kidneys. There was mild proteinuria. Autoantibody testing showed positive antineutrophil cytoplasmic antibody (ANCA) and markedly elevated anti-PR3 antibody titer. Anti-GBM antibody was negative. Microbiological studies did not yield any positive bacterial culture, although her urine Streptococcal antigen was positive. She developed hemoptysis and respiratory failure 2 days after her hospitalization was and transferred to ICU for further care.
She was assessed by the Rheumatologist and suspected to have microscopic polyangiitis. She was advised to receive plasmapheresis, pulse steroid, iv IG, and cyclophosphamide. She was also covered with broad spectrum antimicrobial regimen.
Echocardiogram showed normal ventricular function and no valvular lesions. Bronchoscopy was performed in ICU showing diffuse blood-stained fluid from both sides of the airway. There was no endobronchial lesion. Due to the pulmonary hemorrhage, she had persistent desaturation (<80%) after ICU admission, despite escalation in mechanical ventilator support. The Murray's score was 3.7. Venovenous extracorporeal membrane oxygenation support was decided, to bridge for the effect of the immunosuppressive therapy. Her oxygenation improved right after the ECMO support was initiated. Blood flow rate was 3.5L/min.
As the Thromboelastogram (TEG) upon ICU admission showed hypercoagulable state, tight heparin was started upon initiation of ECMO with a target of 45-50s. TEG and aPTT were repeated for monitoring of the clotting status. The TEG and corresponding aPTT were depicted in . There was increase in hemoptysis on day 3 of ECMO support, and aPTT was <60s which was within the therapeutic range for anticoagulation. However, the TEG suggested worsening of coagulopathy, as compared with TEG of previous day. Heparin dose was reduced and hemoptysis improved.
Plasmapheresis and continuous venovenous hemofiltration were continued during ECMO support. Hemoptysis subsided on day 4 of ECMO support. Lung infiltrate improved from day 4 and urine output improved on day 5. ECMO support was weaned off on day 7. Patient was extubated 3 days after ECMO decannulation. |
pmc-6452548-1 | We describe the case of a 45-year-old Hispanic man who presented to the psychiatric emergency room on account of depressed mood and forgetfulness. He was found by his niece sitting in the bathroom batting away imaginary flies and crying, stating that he could not remember anything which prompted his niece to call emergency medical services (EMS).
The patient was emotionally labile and could not remember his name or address at the time of presentation. He was hyperverbal and difficult to interrupt, and his speech was disorganized. The patient stated that prior to admission, he left his home and suddenly could not remember how he got to the location he had traveled to. He then returned home and entered the bathroom to look for a belt to hang himself with, because he could not remember any of the evening's events. He stated that he felt lonely and helpless and that he had suicidal thoughts. The patient stated that his sleep had been poor. He endorsed a perceptual disturbance of seeing fleas that were trying to infest his body. He also endorsed an auditory hallucination of a male voice calling his name. Collateral information from his niece, who called the EMS, revealed that the patient had been acting bizarre with two previous episodes of new-onset wandering behavior in the past six months, both associated with heavy alcohol use. She also reported that the patient had a 15-year history of schizophrenia and that he had had similar episodes in the past, which were usually brief and resolved without the need for hospitalization. During a similar episode three years ago, the patient began attacking his family members and was hospitalized after the police were called. The patient also received a diagnosis of major depressive disorder five years ago. The patient was admitted to the inpatient psychiatry unit with a diagnosis of major depressive disorder. Urine toxicology at the time of admission was negative for controlled substances, illicit drugs, and alcohol. The patient's admission Complete Blood Count (CBC) and kidney liver function tests were within normal limits. Rapid regain test was negative. Serum sodium and potassium were 138mmol/L (136–144.0mmol/L) and 4.4mmol/L (3.6–5.1), respectively. Other routine urine analyses and coagulation profiles were also within normal limits as were routine chest radiograph and ECG. Serum thyroid stimulating hormone was below the lower limit of normal 0.409 uIU/ml (0.450–4,500 uIU/ml) and free T4 was 1.09 ng/ml (0.82–1.77 ng/dL). The patient had no symptoms of hyperthyroidism.
Other chemical laboratory investigations were within normal limits except for dyslipidemia. Computerized tomography (CT) and magnetic resonance imaging (MRI) performed during admission revealed partial agenesis of the corpus callosum with the absence of the posterior body and the splenium as shown in .
On day 1 of hospitalization, the patient was hyperactive and restless on the unit. He was treated with escitalopram 10 mg PO daily and risperidone 2 mg PO BID. By day 2 of hospitalization, the patient was able to recall his name and his perceptual disturbances resolved, but he was still hyperverbal, with increased activity. By day 9 of hospitalization, the patient's condition had stabilized, and he was discharged.
According to the patient's mother, the pregnancy was reported to be complicated at five months, and the patient was born at seven months. He had normal gross motor development, but language was delayed until the age of 7 years. The mother reported a history of cognitive developmental delay and intermittent behavioral disturbances that led to his dropping out of school in fifth grade. |
pmc-6452550-1 | A 16-year-old left handed girl suffered a spontaneous brain hemorrhage into her left occipital lobe. She lost consciousness and was resuscitated from respiratory arrest. Imaging showed left occipital and left frontoparietal subdural hematomata. Drug induced coma was implemented and maintained for eight days prior to left parietooccipital craniotomy and excision of the causative left occipital arteriovenous malformation (Figures and ). At surgery small arterial feeders were evident 5cm from the midline. The hematoma cavity was evacuated. The arteriovenous malformation extended into the lateral horn of the left lateral ventricle and across the surface of the tentorium, draining into the transverse sinus. The medial aspect of the left occipital lobe was disconnected from the midline and the affected 4 x 4cm area was hinged up onto the draining vein. The vascular pedicles were coagulated and divided, and the vascular malformation was removed. Shortly following the surgery, the patient displayed intermittent jerking of the right upper limb, as well as marked weakness of the left side. EEG testing revealed irregular brain waves in the posterior temporal and parietal regions with a right-sided bias. Both the jerking and the weakness resolved over 4 days. A right-sided homonymous hemianopia became evident. On discharge, the patient and her parents were informed of a possibility of spontaneous recovery of the hemianopia, but this was deemed unlikely. Her neuropsychological tests showed focal visual dysfunction, suggestive of difficulties in visual search (), but their origin was not identified at the time. For the following 17 years, the patient experienced challenges with many aspects of her life, including her mobility, her ability to access learning material at school, her social interactions, specific daily living activities including finding items on a supermarket shelf or clothes in her wardrobe, and the control of emotion. In 2013, at a conference presentation on identifying and supporting children with CVI, the patient recognized the difficulties described as being identical to her own. The patient introduced herself to the presenter and explained this.
Visual assessment confirmed persisting right hemianopia. No evidence of visual impairment in the sighted visual field [] was elicited. A body turn to the right (without a head turn) slightly extended the field of vision to the right, suggestive of an additional element of right inattention. Nevertheless, repeated small discreet finger movements made by the examiner in multiple locations within the hemianopic field were correctly intuited on more than 70% of occasions, consistent with blindsight. Uncorrected visual acuities and stereopsis were in the normal range. Structured history taking for evidence of perceptual visual impairment [] revealed great difficulty finding an item from within clutter or within a pattern and great difficulty copying, consistent with simultanagnostic vision. Standard eye movement examination was normal, with normal convergence, pursuits, and saccades to command (e.g., ‘look right'), but instruction to look at specific items nominated by the examiner resulted in anarchic visual search movements until the specified item was eventually found. This feature is consistent with apraxia of gaze, a phenomenon known to accompany simultanagnostic vision []. When asked to look at a large group of people, the patient could only identify the presence of one person. Careful observation of eye movements during such search consistently showed them to be sparse and apparently random. In an outdoor café she was asked to count the number of support poles for the sun-shading roof. She randomly found and identified 3 out of 11, with the same lack of visual search strategy.
The accuracy of the patient's visual guidance of reach was evaluated. Careful observation of the in-flight gap between the fingers, and their orientation while reaching for discreet targets in the intact visual field showed consistent features of a gap much wider than necessary, with inaccuracy of orientation []. These features are consistent with a degree of accompanying optic ataxia.
The patient was given a detailed tutorial concerning the nature of her vision and strategies that she could employ to make best use of her vision. |
pmc-6452551-1 | A 38-year-old woman, gravida 4 para 1, was referred due to monochorionic-triamniotic triplet pregnancy at 10 weeks of gestation. We confirmed monochorionic-triamniotic triplet gestation, with absence of cardiac activity in one triplet. Reversed flow in the fetal umbilical cord was demonstrated on Doppler ultrasound examination. These findings were consistent with monochorionic-triamniotic gestation complicated with twin reversed arterial perfusion (TRAP) sequence. The acardiac fetus did not have an upper body.
Using Doppler ultrasound, the presence of anastomotic vessels was confirmed between triplets A (acardiac fetus) and B (direct pump fetus) but not between triplet A and triplet C (indirect pump fetus). The Doppler measurements of feeding vessel (umbilical artery) in the acardiac twin revealed pulse rate that was similar to FHR of twin B. The direct pump fetus could be distinguished from the indirect pump fetus by measuring FHR. The estimated fetal body weight (EFBW) of the acardiac fetus increased as pregnancy progressed. EFBW of an acardiac fetus can be obtained by the following equation: (grams) = (-1.66 × longest length [cm]) + (1.21 × longest length [cm]2) []. At 14 weeks, body weight was 136 g for triplet A, 56 g for triplet B, and 59 g for triplet C. At 16 weeks, body weight was 227 g for triplet A, 109 g for triplet B, and 120 g for triplet C. In triplets B and C, we could not confirm polyhydramnios, absent umbilical artery end-diastolic velocity, umbilical venous pulsation, and absent or reverse blood flow in the ductus venosus.
We usually perform RFA when EFBW of an acardiac fetus is 0.7 times or more as much as that of a pump fetus, and heart failure and amniotic fluid excess are recognized. In the current case, weight of the acardiac fetus exceeded twice that of the pump fetus and weight of the acardiac fetus had increased further. We speculated that the pump fetus would develop heart failure with high probability in the future; thus, radiofrequency ablation (RFA) was performed at 18 weeks. The acardiac fetus was stuck to the anterior uterine wall and the other sac did not interfere with needle insertion (). We used a 480 kHz radiofrequency generator and 20 cm-long, 17-gauge, internally cooled electrode with a 2 cm-long exposed metallic tip (Cool-tip RF system; Valleylab, Boulder, Co., USA). The patient had strong anxiety in a conscious state, so we performed surgery with general anesthesia. An electrode was transabdominally introduced and inserted into the fetal abdomen adjacent to the umbilical cord insertion area under continuous ultrasound guidance. Coagulation was initiated for 90 seconds, and complete blood flow cessation was successful in the acardiac fetus. In triplets B and C, preoperative and postoperative middle cerebral artery peak systolic velocities were the same and fetal anemia was not observed ().
During follow-up by weekly ultrasound, twin-to-twin transfusion syndrome, selective intrauterine growth restriction, and twin anemia–polycythemia sequence did not develop. At 37 weeks, a cesarean section was performed; two male newborn infants were delivered. Triplet B weighed 2,662 g, with Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. Triplet C weighed 2,031 g, with Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. Placental examination showed four vascular anastomoses between triplets B and C () and vascular anastomoses (i.e., umbilical artery and umbilical vein) between triplets A and B. The umbilical artery between triplets A and B was anastomosed with a thin umbilical artery of triplet C. The umbilical vein between triplets A and B was anastomosed with two umbilical veins of triplet C. At 12 months, both children showed normal neurodevelopment. |
pmc-6452553-1 | A 64-year-old male presented with a two month history of difficulty urinating and was found to have a fungating penile mass involving 50% of his penis. The mass was hard and fixed and extended from the glans proximally up the shaft. He also had bilateral palpable inguinal lymphadenopathy. There were no associated constitutional symptoms. Given there was a high suspicion for malignancy, the patient underwent partial penectomy within a month of presentation. Biopsy results confirmed a pT2 tumor with invasive keratinizing squamous cell carcinoma, poorly differentiated, and tumor size of 5 × 4 × 2.5 cm, with corpus spongiosum and lymphovascular involvements.
Following the procedure, the patient had PET-CT for staging, and imaging revealed enlarged hypermetabolic bilateral axillary lymph nodes concerning for metastatic disease. In addition, there was a large centrally necrotic lymph node conglomerate in his left groin that had increased FDG avidity. The patient had left inguinal and bilateral pelvic lymph node dissections revealing metastatic squamous cell carcinoma in multiple lymph nodes. The left inguinal mass was also found to be metastatic well-differentiated SCC. His diagnosis was staged at T2N3M0.
After his surgical procedures, patient was started on adjuvant chemotherapy. He began first line chemotherapy with paclitaxel, ifosfamide, and cisplatin (TIP). He underwent 4 cycles of TIP but eventually developed disease progression on repeat imaging. At this point, the patient was started on cetuximab given EGFR amplification on tumor analysis with the FoundationOne testing platform. However, the patient had an allergic reaction to cetuximab, so his treatment was changed to panitumumab. The patient had stable disease and a progression-free survival of 6 months with anti-EGFR treatment, which is clinically significant given that this treatment was given in the second-line setting for an aggressive tumor type that other than chemotherapy there is no other approved drug to date.
The patient was ultimately started on the PD-1 inhibitor nivolumab. He had initial response to immunotherapy followed by stable disease, so he had a disease control rate of an additional 6 months with this investigational agent at that time. Ultimately, he was placed on hospice and passed away two years from the the time of diagnosis. |
pmc-6452553-2 | A 79-year-old male with longstanding history of advanced prostate cancer on androgen deprivation therapy presented to his urologist after noticing a mass on the tip of his urethral meatus. A subsequent biopsy of the mass was positive for SCC, and the patient underwent partial penectomy and lymph node dissection that revealed positive right inguinal lymph nodes (three out of seven) revealing pathologic T2N2M0 disease. He received adjuvant chemotherapy by extrapolating data of its benefit when given in the neoadjuvant setting. The standard TIP regimen was not pursued given patient's concern for side effects. The patient proceeded with alternative plan of chemoradiation with 5 weeks of weekly low dose carboplatin and paclitaxel. In addition, he received radiation with a total dose of 5000 cGy over 25 fractions to the right inguinal region. However, the patient developed disease recurrence with nodal involvement nine months later. On restaging CT imaging, the patient was found to have new involvement of the left pelvis. A nodal conglomerate measuring 31×58 mm with central necrotic change was identified in the left inguinal region.
Given the patient's age, performance status, and local recurrence of disease, he was started on therapy with chemoradiation with curative intent one month later. Treatment with an additional round of chemoradiation with low dose carboplatin and paclitaxel was given for 5 weeks. He had radiation with a total dose of 5000 cGy over 25 fractions to the left pelvic region. He had stable disease with chemoradiation, but he eventually developed disease progression within a year from the end of chemotherapy. At that point, he was considered for second-line therapy with the PD-L1 inhibitor atezolizumab. After being on atezolizumab for approximately 2 years, he developed biopsy-proven bullous pemphigoid, an immune-mediated toxicity of the skin that has been described with those agents. A restaging scan at approximately 2 years showed near complete response, so patient has been placed on treatment holiday at the time of this report. He was started on prednisone 1 mg/kg per immune-mediated management guidelines and had quick resolution of his blistering symptoms []. |
pmc-6452554-1 | A 31-year-old woman (gravida 5, para 2) was referred to our hospital due to labor pain. She was suspected to have SLE because of facial erythema at age 29 years, but clinical and serological findings failed to satisfy the diagnosis criteria for SLE. She gave birth to two boys without any problems at age 23 years (birth weight, 2822 g) and 26 years (birth weight, 2946 g). The fetal heart monitor showed non-reassuring fetal status. A female neonate was born by emergency cesarean delivery at 35 weeks and 3 days. The neonate weight was 1,909 g. She had fetal growth restriction (FGR) and Apgar scores of 5 and 7 (1 minute and 5 minutes). Her skin was pale. She had congenital erythematous and scar lesions on the face, back, and upper and lower extremities (). An examination revealed a slight elevation in hepatic transaminases, thrombocytopenia, and mild cardiac failure. No heart blockage was detected. The serological examination of the neonate showed elevated anti-SSA/Ro (281 U/mL) and anti-SSB/La antibodies (≧1000 U/mL). Other antibodies were normal range (anti-DNA antibody ≦2.0 IU/mL, anti-RNP antibody <2.0 U/mL), and complements were not reduced (C3; 92 mg/dl, C4; 22 mg/dl). The serological examination of the mother also showed elevation of these antibodies, and a histological examination demonstrated lymphocytic infiltration of the minor salivary gland; therefore, a diagnosis of Sjögren syndrome was made. The symptoms of the neonate had almost resolved by 7 months of age.
The placenta was 17 x 13.5 x 2 cm and weighed 285 g. The amnion color was green, and the cut surface showed anemia. Histological examination revealed collapsed capillaries in the terminal villi (). No apparent inflammatory cells or thrombus formation was found in the fetal vessels. Partial focal maternal vessel thrombosis was noted, but no apparent infarction was seen. A meconium stain with amnion degeneration was observed in the amnion. Focal maternal thrombosis and increased syncytial knots were also present.
Immunohistostaining of a normal placenta (), placenta with maternal SLE (), and the placenta involved in this case () was performed using C4d (ABGENT, San Diego, CA, USA). In the normal placenta, focal or weak C4d deposits were present at the syncytiotrophoblasts, but not in the lumen of the vessels (). C4d deposits were strongly observed at the syncytiotrophoblasts in SLE cases (). The placenta with NLS showed C4d deposition in the lumen of the capillaries of the terminal villi (). C4d deposition was also seen in the stem vessels, chorionic vessels, and umbilical vessels in this case. C4d deposition was not detected in the maternal vessels. |
pmc-6452555-1 | A 7-month-old boy was referred to the Maternity and Children Hospital in Makkah for further management of left grade 2 hydronephrosis, right renal agenesis, and right impalpable, undescended testis. The patient presented to the outpatient department (OPD) for assessment. Genital examination revealed a normal-size, circumcised penis, sizable left testis in the scrotum, and an impalpable, undescended right testis. The patient was booked for examination under anesthesia and laparoscopic exploration.
Laparoscopic exploration was performed on February 13, 2017, and revealed a sizable right testis proximal to the internal inguinal ring by 4 cm with short spermatic vessels, and an absent right vas deferens and epididymis (). This left the choice of performing either Orchiectomy or Orchiopexy. For the sake of hormone generation, we decided to perform Orchiopexy depending on the gubernacular vessels. We therefore performed first-stage Stephen-Fowler Orchiopexy by clipping the spermatic vessels.
After 6 months, the patient was admitted for second-stage Stephen-Fowler orchiopexy on September 11, 2017. Intraoperative findings showed that the testis was the same size. We performed peritoneal dissection lateral to the testis; the medial peritoneal aspect and the gubernaculum remained untouched (). The testis was brought through the inguinal canal into the right hemiscrotum, where a dartos pouch was created () and the testis was fixed in this position. The dimension of the testis was 12 × 5 mm.
At three-month follow-up, the right testis was assessed in the OPD; both testes were present in the scrotum with normal sensation, and the right testis was comparable in size to the left. Six months after the 2nd stage procedure, ultrasound of the scrotum was performed to assess the size, echogenicity, and vascularity of the testis; this revealed a normal anatomical location of both testicles, with a homogenous echo pattern and normal vascularity. The right testis measured 13 × 6 mm and the left testis measured 16 × 8 mm ().
At the 12-month follow-up, both testicles appeared to have a normal anatomical location and parenchymal echogenicity. The right testicle measured 10 × 7 × 8 mm, and the left testicle measured 15 × 8 × 10 mm (). Vascularity was good according to doppler ultrasound. |
pmc-6452556-1 | A 40-year-old multiparous African American woman at 21 weeks' 4-day gestation, with known chronic hypertension, was transferred to our medical center for management of hypertensive emergency. Prior to transfer, the patient had marked blood pressure elevation (peak 192/129 mmHg) and laboratory evaluation notable for hemoglobin 11.0 g/dl, platelet count 66 k/μl, alanine transaminase (ALT) 20 U/L, aspartate transaminase (AST) 40 U/L, and creatinine 0.7 mg/dl ( for laboratory trends and reference range). Urine dipstick detected 4+ protein, 4+ blood, and +nitrites, and urine drug screen was positive for methamphetamines and marijuana. Intravenous ceftriaxone was given empirically for urinary tract infection.
On arrival to our medical center, blood pressure peaked at 205/114 mmHg and laboratory findings were like those noted above. In addition, lactate dehydrogenase (LDH) level was 985 U/L, with haptoglobin <8 mg/dl and 3 schistocytes per high power field on peripheral smear. Urine protein/creatinine ratio was 2.61 mg/mg (normal <0.3 mg/mg). Estimated fetal weight by ultrasound was 451g, which was appropriate for her gestational age. We suspect that methamphetamine use precipitated her hypertensive crisis, but the clinical picture and laboratory findings were concerning for severe preeclampsia and HELLP syndrome. Due to her previable gestational age and the life-threatening nature of her condition, termination of pregnancy was recommended, and she agreed. Magnesium sulfate was initiated for seizure prophylaxis, and she was managed in the intensive care unit on an intravenous nicardipine drip. Within 12 hours of her first misoprostol dose for labor induction, she had a precipitous vaginal delivery of a nonviable female neonate. Pathology examination of the placenta revealed decidual arteriopathy and accelerated villous maturation, which are findings reflective of severe hypertension and placental hypoxia.
Following delivery, severe hypertension (≥160/110 mmHg) persisted throughout the postpartum period despite triple agent antihypertensive therapy. On postpartum day 5, she continued to have severe range blood pressures, but declined inpatient stay and was discharged home on an oral regimen of chlorthalidone (50mg daily), amlodipine (10mg daily), and telmisartan (80mg daily). During her postpartum course in the hospital, patient had a work-up for secondary causes of hypertension. She had maternal echo showing mild left ventricular hypertrophy, but normal left ventricular ejection fraction (69%) and no evidence of aortic coarctation. She had normal renal artery Dopplers, ruling out renal artery stenosis. Serum aldosterone/renin ratio was normal, ruling out primary aldosteronism. She did not complete a 24-hour urine collection to measure total metanephrines and therefore pheochromocytoma was not formally ruled out. We suspect that methamphetamine use precipitated her hypertensive emergency, but blood pressures remained severe despite 7 days of inpatient observation and drug cessation.
TMA also persisted despite termination of pregnancy and drug cessation. On postpartum day 1 laboratory values were as follows: hemoglobin 8.4 g/dl, platelet count 90 k/μl, LDH 706 U/L, haptoglobin <8 mg/dl, 2-3 schistocytes per high power field on smear, and reticulocyte count 4.6% (normal 0.5-2.0%). Her reticulocyte production index was noted to be suboptimal at 2.1% (normal >3.0%), after adjustment for low hematocrit (30.5%) []. Considering that the patient's liver enzymes remained normal after delivery despite ongoing hemolysis and thrombocytopenia, the diagnosis of HELLP syndrome was questioned. Alternative TMA etiologies were considered, including aHUS, TTP, and vitamin B12 deficiency. Serum creatinine peaked at 0.9 mg/dl and aHUS genetic panel was negative for disease-associated complement gene variants (Machaon Diagnostics), making a diagnosis of aHUS less likely. ADAMTS13 activity was 75% (normal >67%), making a diagnosis of TTP less likely. Meanwhile, vitamin B12 level was undetectable <146 ρg/ml (normal 213-816 ρg/ml) and intrinsic factor blocking antibodies were positive, consistent with pernicious anemia. Mean corpuscular volume was paradoxically low at 75.3 FL (normal 80-100 FL), and other causes of anemia were ruled out with normal iron panel, hemoglobin electrophoresis, and folate level.
Following diagnosis of pernicious anemia, the patient was started on subcutaneous B12 injections at a dose of 1000 mcg daily. She received 5 injections of vitamin B12 during her postpartum hospital stay. While blood pressures remained severely elevated on the day of discharge (postpartum day 5), laboratory parameters showed signs of improvement: Hemoglobin 9.5 g/dl, platelet count 175 k/μl, and LDH 404 U/L ( for laboratory trends). The patient was discharged home with a plan for continued B12 supplementation and close outpatient follow up. At 2 weeks postpartum the patient had normal blood pressure (127/82) and was clinically well, but she declined additional laboratory testing. |
pmc-6452561-1 | A 55-year-old Ghanaian woman presented with a one-month history of mouth ulcers, painful swallowing, and fever. She had also developed cutaneous ulcers on the left lower leg, back, and scalp. She reported being nonspecifically unwell for 1 year, with fatigue, poor appetite, and 8 kg weight loss over three months. She was not taking any regular or new medications prior to this illness and had a background of asthma and asymptomatic mild-moderate mitral regurgitation. She did not consume alcohol or recreational drugs and smoked 10 cigarettes per day. She was born in Ghana and came to the UK aged 11. There was no travel history for 3 years prior to admission and no recent new sexual partners.
On admission, she was febrile at 38.2°C. Several painful mouth ulcers were seen, affecting the hard palate and the oropharynx (). No other mucous membrane involvement was present and specifically no genital ulcers. There was a 5 cm diameter circular pigmented nodule on the left calf, with central necrotic ulceration. She had a 10 cm diameter inflammatory well-demarcated plaque over the left central back () and smaller ulcers over the scalp, all less than 1 cm diameter.
Laboratory tests revealed mild anaemia with a haemoglobin of 119 g/L, platelet count 227 × 109/L, white cell count 9.0 × 109/L, albumin 33 g/L (normal 35–55), an elevated erythrocyte sedimentation rate (ESR) of 69 mm/hr, and C-reactive protein (CRP) 73 mg/L (normal < 5). The ANCA test was negative, complement component C4 was elevated, and the rest of the autoimmune screen was negative. Blood and urine cultures and a scalp ulcer and throat swab revealed no bacterial growth. Viral throat swab was negative. Coeliac serology was also negative. Upper gastrointestinal endoscopy revealed marked oropharyngeal ulceration without contact bleeding (). A CT scan of the chest, abdomen, and pelvis revealed no evidence of malignancy, while an MR angiogram of the aorta revealed no evidence to suggest systemic PAN.
Two 4 mm punch biopsies were taken from the scalp lesions along with an 8 mm punch biopsy from the left lower leg nodule. These underwent haemotoxylin and eosin (H and E) and direct immunofluorescence staining. Immunohistochemistry from both locations revealed loss of epidermis with necrosis and a brisk inflammatory infiltrate in the dermis composed of neutrophils and histiocytes, the latter coalescing into poorly formed granulomas (Figures and ). Abundant cytoclastic debris was present. In areas, the inflammatory cells were clearly centred around and obscuring blood vessels, which showed foci of fibrinoid necrosis (). The process involved small dermal blood vessels and larger muscular vessels deep in the biopsy. PAS (Periodic acid-Schiff) and ZN (Ziehl–Neelsen) stains were negative. Direct immunofluorescence was negative. The overall impression was of a necrotising vasculitis with granulomatous features, findings consistent with a diagnosis of cutaneous PAN.
Data are limited on the treatment used in cutaneous PAN. However, due to the severity of her presentation and oropharyngeal involvement precluding her from a safe swallow, systemic treatment was opted for in preference to topical therapy. The patient was initially prescribed intravenous hydrocortisone and subsequently oral prednisolone 40 mg and methotrexate 10 mg weekly. The latter was uptitrated to 15 mg weekly prior to discharge, and the prednisolone was slowly weaned. Once treatment was established, no further lesions developed and the existing oropharyngeal and cutaneous ulcers began to heal. After 23 days, when able to maintain good oral intake, the patient was discharged.
At clinic follow-up six weeks later, all the oral ulcers and the lesions on the leg, back, and scalp had healed, and no new lesions were seen. The patient reported persistent fatigue and muscle weakness, and there was evidence of mild proximal weakness in the hip flexors. Acute phase markers and serum albumin were within normal range. The methotrexate dose was increased from 15 mg to 17.5 mg weekly and the prednisolone dose was progressively weaned. At subsequent follow-up after 6 months, the patient was well with no active skin lesions. She continued methotrexate 20 mg weekly and prednisolone 5 mg daily. At 12-month follow-up, she remained in remission with CRP 2.0 g/L and ESR 24 mm/hr. |
pmc-6452579-1 | A 37-year-old man presented to the emergency room with symptoms of worsening headaches, dysarthria, and visual and tactile hallucinations in the setting of recent 3-week hospitalization for alcohol detoxification. During the patient’s prior hospitalization at a community hospital in south central Pennsylvania, his course was complicated by seizures, delirium tremens, and acute hypoxic respiratory failure secondary to aspiration pneumonia. The patient was stabilized and was discharged on levetiracetam for seizure prophylaxis. At home, his family reported that his mental status never returned to baseline, and he continued to exhibit visual and tactile hallucinations, as well as worsening episodic dysarthria and drooling, leading to representation.
In terms of the patient’s social history, his wife disclosed that the couple had been married for 4 years and had been in a monogamous relationship to her knowledge. The patient was employed as a tow truck operator and also worked on a farm training horses. He was born in Georgia, resided in Maryland, and traveled to Virginia, Massachusetts, and eastern Canada over the past 3 years. He had no history of intravenous drug use, though the patient had a 30-pack-year cigarette smoking history. He had a history of heavy alcohol use for 20 years. He had not had an alcoholic beverage to her knowledge since his return home from initial hospitalization.
In the emergency department, the patient was febrile to 39.3°C; otherwise vitals were within normal limits. His examination was remarkable for a Glasgow Coma Scale score of 14/15 (verbal response mildly slowed), disorientation to person, place, and date with extreme agitation, and meningismus exacerbated by neck flexion. A complete neurologic examination was unable to be performed, due to mental status, but cranial nerves II to VIII, X, and XI were intact, and the patient had spontaneous movement in all 4 extremities. Laboratory studies revealed a normal basic metabolic panel (), with the exception of an elevated serum creatinine, a leukocytosis of 18 750 cells/µL, and a negative urine drug screen and blood alcohol level. A non-contrast computed tomography image of the head was negative for acute bleeding, edema, or mass effect. A lumbar puncture (LP) was performed in the emergency department, which showed 130 nucleated cells (81% lymphocytes), protein 76 mg/dL, glucose 15 mg/dL, and an opening pressure of 47 mm Hg (). Viral serologies—including herpes simplex virus- polymerase chain reaction, Epstein-Barr virus, cytomegalovirus, and varicella-zoster virus—were sent with initial cerebrospinal fluid (CSF) analysis, along with a Venereal Disease Research Laboratory test, Toxoplasmosis gondii, and Lyme studies. The patient was started empirically on acyclovir, ceftriaxone, vancomycin, and ampicillin, and was admitted to the Internal Medicine service.
CSF results revealed no bacteria, but few encapsulated yeast identified as C neoformans. CSF cryptococcal antigen returned positive at 1:60, prompting discontinuation of empiric antimicrobial therapy and initiation of antifungal therapy (liposomal amphotericin and flucytosine). Serial LPs were performed daily, in accordance with the Infectious Diseases Society of America guidelines for the treatment of cryptococcal meningitis, until opening pressure remained under 20 mm Hg for 2 consecutive days. Computed tomography of the chest, abdomen, and pelvis was suggested to evaluate for other manifestations of cryptococcosis, ultimately disclosing a 1.0-cm right upper lobe ground glass nodularity, along with clustered right middle lobe nodules and linear scarring. HIV and hepatitis C virus antibodies and viral load were checked and were negative. Blood culture was only positive at time of admission, which revealed C neoformans.
The patient was maintained on intravenous amphotericin and flucytosine for 26 days. CSF culture for fungal isolates was negative at the 14-day mark while cryptococcal antigen remained elevated at 1:40. His mental and functional status slowly improved. The only complication during his hospitalization was an episode of sinus tachycardia—felt due to paroxysmal sympathetic hypersensitivity as a result of cryptococcal meningitis—which was treated with propranolol. He was transitioned to oral fluconazole on day 27 of treatment, with a goal to complete 56 days (8 weeks) of total therapy. He was ultimately stable to discharge to a rehabilitation facility on hospital day 30. |
pmc-6452583-1 | A 16-year-old male with past medical history of recurrent sinusitis and otitis media requiring bilateral tympanostomy tube placement and tonsillectomy at age 4 and environmental allergies requiring allergen immunotherapy presents with 1-day history of irritation, redness, and mobile swimming foreign body sensation in left eye.
At onset of symptoms, the patient was participating in after-school sports practice on a natural grass football field at a local high school in a southern San Joaquin Valley municipality in California. Review of field maintenance records reveal recent fertilization with manure. The patient’s eye became increasingly red and irritated, which was not relieved with artificial tears or irrigation with tap water. Mechanical removal was successfully completed by the patient and in the emergency department. Eleven larvae removed were consistent with the first instar stage of O ovis (-) as per pathological examination on saline wet mount with light microscopy and comparison with standard entomologic texts. The patient had left conjunctiva injection, intact extra ocular movements, and bilateral normal visual acuity. On slit lamp examination, multiple larvae were appreciated in the bulbar conjunctiva and palpebral fornix of left eye. They did not appear to be burrowing. The patient was given polysporin-bacitracin-neomycin ophthalmic solution and albendazole 400 mg daily for 3 days for prophylaxis. Follow-up ophthalmology examination confirmed clearance of larvae at 2 days.
Family history was significant for diabetes, hypertension, and dyslipidemia. Travel history was limited to southern San Joaquin Valley, California. Animal exposure includes 4 dogs and 1 young chicken at home. Denies other livestock exposure in his neighborhood, specifically no exposure to sheep, goats, bovines, or other livestock. |
pmc-6452584-1 | A 42-year-old woman presented to the emergency department with acute abdominal pain and was diagnosed with a hemoperitoneum from a ruptured ovarian cyst. The laparoscopic evaluation was complicated by continuous bleeding. Additional workup confirmed a partial thromboplastin time (PTT) of 42 seconds (normal 20-36 seconds), which was corrected by mixing study, suggesting factor deficiency. Factor X (FX) activity was 11%, while others were normal. She required multiple units of fresh frozen plasma (FFP) and aminocaproic acid for bleeding control. FX activity did not significantly improve after FFP infusion but improved transiently with prothrombin complex concentrates (PCC). However, to maintain a FX activity over 20%, a higher dose and more frequent infusions were required. Serum protein electrophoresis showed IgG kappa monoclonal protein of 0.4 g/dL, and serum-free kappa and lambda light chain levels were 14.5 and 72.2 g/dL, respectively. Bone marrow aspiration and biopsy showed 3% kappa-restricted plasma cells. Congo red staining of bone marrow was negative, but rectal biopsy tissue was positive. Immunofluorescent staining confirmed kappa-restricted amyloid protein. Amyloidosis of the kidney was also suspected based on 3 g/24 h proteinuria, though kidney biopsy was not performed. She had no hepatosplenomegaly. Troponin, pro-BNP levels, and echocardiogram were normal. The patient had ongoing bleeding complications including heavy periods requiring uterine ablation and intermittent lower gastrointestinal bleeding. She started induction therapy using bortezomib, cyclophosphamide, and dexamethasone regimen (CyborD). She developed grade 2 painful sensory neuropathy and only achieved marginal response after 3 cycles of therapy. Her FX activity remained between 10% and 15%. She proceeded to stem cell collection, followed by high-dose melphalan and autologous stem cell transplantation. During the myelosuppressive period, she received PCC and platelet transfusion to keep the platelet level over 20 × 109/mL. She achieved complete remission as evidenced by the normalized serum lambda light chain and correspondingly had a gradual improvement of FX level. |
pmc-6452584-2 | A 67-year-old man presented with an ankle swelling and cutaneous purpura. Additional workup showed 7 g/dL of albuminuria, and kidney biopsy confirmed lambda-restricted amyloid protein deposition. The patient had a bleeding complication from kidney biopsy, which led to additional investigations confirming a low serum FX activity (20%). Splenomegaly was noted on the computed tomography of the abdomen. He had no bleeding history but reported easy bruising for the past 3 months. Serum protein electrophoresis showed 1.0 g/dL of IgG lambda monoclonal protein. Serum-free kappa and lambda light chain levels were 18 and 34 g/dL, respectively. Bone marrow aspiration and biopsy confirmed 5% of lambda-restricted plasma cells. The patient opted against stem cell transplantation and underwent induction therapy with the bortezomib-based regimen. He achieved complete hematologic remission. Serum FX level improved but remained low at 35%. The patient was in an auto accident resulting in a splenic rupture. He underwent emergent splenectomy and required red blood cell transfusion during the procedure. The serum FX level improved to 52% within 1 month after the surgery. |
pmc-6453298-1 | A 35-year-old woman, gravida 2 para 1, was admitted to our inpatient clinic for a vaginal birth during her second pregnancy.
In her obstetric history, she had undergone a secondary caesarean section due to a prolonged second stage of labour due to high birth weight (GA: 41 weeks and 3 days, 4130 g, p80–84) and a cephalic presentation in the occiput posterior position. In her current pregnancy, there was elaborate counselling regarding the delivery mode, she opted for a vaginal birth after caesarean section. Her pregnancy course and clinical examination were completely normal, except a fetal abdominal circumference above p99. We therefore decided to induce labour at 39+2 weeks of gestation.
At the day of delivery, she had a cervical dilatation of 4 cm and we artificially ruptured the membranes at 08:00. At 08:45, oxytocin was started to stimulate uterine contractions. It was difficult to stimulate the uterus, but on high dose oxytocin the patient eventually reached active phase of labour. At 17:10, she had a pain relief request due to severe labour pains. At that time, her cervix was 8 cm dilated and we decided to start with patient-controlled remifentanil. At 21:10, she reached full cervical dilation and she started second stage of labour while the caput of the fetus was just above the Hodge plane 3. Twenty minutes into the secondary stage of labour, the cardiotocography showed a fetal tachycardia and complicated, but variable, decelerations. At that moment, the fetal caput was at Hodge 4. Because there was rapid progression in the delivery an episiotomy in combination with fundal expression was applied. Directly after these manoeuvres, at 21:48, the fetal head was easily delivered with external rotation. This was immediately followed by a turtle sign. We unsuccessfully applied the McRoberts’ manoeuvre with suprapubic pressure followed by an also unsuccessful Woods corkscrew manoeuvre. Then, the Rubin II on all four was applied, which also failed. As a last step, we tried to manually deliver the posterior shoulder by traction on that posterior axilla; however, this technique also failed. In the moment before we moved on performing a symphysiotomy, we decided to try the posterior axilla sling traction technique. At this time the patient was still in the all fours position.
At 21:50, a neonatal suction tube was fed around the posterior axilla without any problems, creating a sling around the posterior shoulder. Thereafter, we applied gentle vertical traction on the suction tube and as a result the posterior shoulder was immediately born. |
pmc-6453613-1 | A 33-year-old male involved in a road traffic accident (MVA) in July 2016 was admitted to our hospital. He sustained a T12 burst fracture, thus requiring open reduction and internal fixation from T10-L1. As a result of SCI, he developed paraplegia (T11 AIS-A), a pressure injury, and double incontinence. Upon admission to the rehabilitation hospital, he was found to have a left ischial pressure ulcer (stage II). The pelvis X-ray showed bilaterally symmetrical NHO in both hips (Figure ) and knees (Figure ). Subsequently, TC-99m methylene diphosphonate (MDP) bone scintigraphy (Figure ) showed findings compatible with NHO around both knees and hip joints bilaterally, and it appeared to be immature. He was managed conservatively and commenced on indomethacin. The surgical referral was not considered, as the NHO was not affecting the patient’s transfers, lower body dressing, seating, skin, and other aspects of daily living. |
pmc-6453617-1 | A 58-year-old Caucasian woman was admitted to our hospital with complaints of generalized weakness, lethargy, anorexia, and weight loss. She was diagnosed with metastatic breast cancer 17 days prior to this admission. She also had a past medical history of treated hypertension and chronic back pain. Before this admission, she had complained of a breast lump in the previous year but never got it examined. The primary breast tumor was found on ultrasound to be approximately 4 cm by 5 cm and was found to be an invasive, poorly differentiated, ductal carcinoma with extensive necrosis. It had no expression of the hormone receptors, estrogen, and progesterone and was human epidermal growth factor 2 (HER2) positive. At the time of presentation, the cancer was advanced with innumerable hepatic metastases and multiple bilateral pulmonary metastases. There was also a small-moderate right pleural effusion. It had spread to the spine, causing a bony lytic lesion at the T9 vertebrae. On physical examination, she also had jaundice of the skin and mild splenomegaly, likely secondary to extensive liver disease.
The patient had undergone the planned chemotherapy four days prior, which was a treatment of gemcitabine 1600 mg. Gemcitabine has long been shown to be an effective agent in the treatment of metastatic breast cancer []. A Port-A-Cath had been placed successfully without any complications two days before the first chemotherapy treatment. On this present admission, her blood tests showed high uric acid levels (18.2 mg/dL), hyperphosphatemia (6.7 mg/dL), hyperkalemia (5.4 mmol/L), calcium (9.6 mg/dL), increased creatinine (3.38 mg/dL) and decreased glomerular filtration rate (14 mL/min). Nephrologists were consulted and they recognized this as TLS. It was recommended to give the patient vigorous intravenous (IV) fluid hydration with normal saline at 125 cc/hr as well as transfuse packed red blood cells to maintain the hemoglobin levels above 8 g/dL. Allopurinol 100 mg three times a day was also given. Hematologists/oncologists were consulted and they recommended chemotherapy treatment to be on hold for now until the patient’s labs become more stable.
By day two of admission, the patient appeared jaundiced and lethargic but was still alert and oriented. Her blood tests showed high uric acid levels (15.1 mg/dL), hyperphosphatemia (6.1 mg/dL), potassium (4.7 mmol/L), calcium (9.0 mg/dL), increased creatinine (2.69 mg/dL), and decreased glomerular filtration rate (18 mL/min). Rasburicase was not started at this time because it was not readily available at the current medical facility. Over the course of the next few days, the patient’s platelet count continued to drop, likely due to the initial chemotherapy treatment. The creatinine levels remained elevated, and the patient’s bilirubin and other liver function enzymes continued to rise, making the option of chemotherapy less feasible.
By day six of admission, the patient’s blood tests showed high uric acid levels (11.1 mg/dL), potassium (4.0 mmol/L), calcium (8.7 mg/dL), increased creatinine (2.71 mg/dL), and decreased glomerular filtration rate (18 mL/min). On examination, clinical deterioration was evident and the patient appeared even more lethargic and sleepy. She was difficult to wake with verbal stimuli.
Despite optimal management, by day seven of admission, she was drowsy and minimally responsive and had a slow response to any stimuli. At times, she could not open her eyes. At this time, it was decided by the patient, husband, and daughter that the patient would have a ‘do not resuscitate’ order and would be transferred to inpatient hospice when stable. |
pmc-6453618-1 | An 18-year-old female who was previously diagnosed with Glanzmann’s thrombasthenia had undergone laparoscopic aspiration of the ovarian cyst three weeks back at another center. She was on synthetic progesterone and tranexamic acid which is an antifibrinolytic agent for menorrhagia. She presented to us with lower abdominal pain, fever, loose stools, and intermittent rectal bleeding. Her blood picture revealed low hemoglobin of 8 gm/dl, increased white cell count of 14000 cells per cubic millimeter, normal platelet count, and normal prothrombin time and partial thromboplastin time. Contrast-enhanced computed tomography (CECT) of the abdomen showed pelvic collection with an air pocket and thickened sigmoid colon (Figure ). Colonoscopy was performed as she had persistent bleeding per rectum with low hemoglobin and it showed multiple colonic mucosal hemorrhages and a fistulous opening in the sigmoid colon, probably an iatrogenic perforation which happened during prior laparoscopic cyst aspiration. She was planned for computed tomography (CT) guided drainage of the pelvic collection under platelet cover as she was hemodynamically stable and the CT showed only localised collection. Since source control with radiology-guided drainage was inadequate and the patient had persistent fever with loose stools, she was taken up for laparotomy and pelvic abscess drainage. A sigmoid colectomy with proximal end colostomy was also performed as there was a sigmoid perforation which resulted in pelvic abscess and dense adhesions. She received single donor platelets and irradiated packed red blood cells during surgery. In the postoperative period, she had a surgical site hematoma and her hemoglobin dropped to 5.6 gm/dl. Irradiated packed red cells and human leukocyte antigen (HLA) matched single donor platelets were transfused. Thromboelastography (TEG) was performed pre- and post-platelet transfusion to assess the response as she had previously received platelet transfusions and the risk of alloimmunisation was high. Following transfusion, her hemoglobin was stable and there was no further active bleeding. She had a prolonged hospital stay and delayed postoperative recovery due to protracted ileus, hematoma, and superficial surgical site infection. Her central line and skin staples were removed under platelet cover as per the instructions of the hematology team. |
pmc-6453618-2 | A 26-year-old male who was previously diagnosed to have PNH and had been in treatment with steroids and anti-thymocyte globulin, presented with acute abdominal pain and fever. He presented with signs of peritonitis. His blood picture revealed low hemoglobin of 8 gm/dl and low white cell count of 2900 cells per cubic millimeter and normal platelet count. He was taken up for emergency laparotomy as he was septic with peritonitis after adequate blood transfusion. He was found to have jejunal intussusception and proximal ileal segmental infarction with thrombosed mesenteric veins. This segment of small bowel was resected and viable ileal ends were anastomosed. In the postoperative period, he was started on parenteral anticoagulant for mesenteric vein thrombosis probably due to PNH. He was closely monitored in the intensive care unit and was started on total parenteral nutrition, as there was a delay in starting the enteral feed due to prolonged ileus. Histopathological examination of resected bowel showed an extensive transmural hemorrhagic infarction. |
pmc-6453619-1 | A 47-year-old female with triple negative right-sided breast cancer on carboplatin and paclitaxel chemotherapy, underwent a molar tooth extraction for toothache with concern for abscess. The procedure was uneventful until about two weeks after when she started to develop toothache, with sudden left-sided frontotemporal headaches radiating down her face, ear and neck, left ear fullness associated with hearing loss, and left orbital pain with blurry vision. She returned to her dentist and was given 10 days of amoxicillin for suspected sinus infection. Her symptoms worsened and she presented to emergency department (ED). In the ED she was afebrile and hemodynamically stable. Her physical exam was remarkable for left conjunctival injection with tenderness along the left side of the orbit and pain with lateral/upward/downward gaze ocular movements. Her right eye was normal in appearance. She had reduced hearing in her left ear when compared to her right, mild mastoid tenderness on the left with no edema or fluctuance with serous amber colored effusion in the left middle ear with no evidence of otitis media. The tympanic membranes bilaterally appeared intact with no effusion in the right middle ear. Her neurological exam was unremarkable with no focal neurological deficits. The rest of her physical exam was unremarkable. Initial labwork was significant for an elevated white count of 11.4 cells/mm3 (4-10.5), and computed tomography (CT) of the head did not show any abnormalities. Given her aural fullness and hearing loss, magnetic resonance imaging (MRI) of the brain and internal auditory canal was performed, which showed thrombosis of the left transverse and sigmoid sinus (Figure ). A magnetic resonance venogram (MRV) was performed as per neurology recommendations, which re-demonstrated occlusion of the left transverse sinus, sigmoid sinus, and internal jugular vein (Figure ). A CT scan of the internal auditory canal performed at the time showed fluid in the middle ear and multiple left mastoid sinus air cells, consistent with acute otomastoiditis (Figure ). As the imaging helped to clarify the diagnosis, unfractionated heparin drip and broad spectrum antibiotics with vancomycin and cefepime were immediately initiated and later changed to meropenem and cefepime for the acute mastoiditis to cover Gram positive and Gram negative organisms. She was evaluated by an ear, nose and throat (ENT) specialist and no surgical intervention was indicated. Her symptoms dramatically improved and she was transitioned to subcutaneous enoxaparin, continued on intravenous (IV) antibiotics mentioned above and seen in follow-up with complete resolution of symptoms. |
pmc-6453620-1 | A 28-year-old female with no known co-morbidity was admitted to a tertiary care hospital in Karachi in March 2018 with a history of mid-dorsal back pain and moderate intermittent fever for five months. She also complained of a dry cough and significant weight loss of about 10 kg for the past two months. The patient described the back pain as gradual in onset, moderate in intensity, nonradiating, and dull in character. It aggravated with physical activity and improved with rest and the use of non-steroidal anti-inflammatory drugs (NSAIDs). It was associated with arthralgia of small joints of hands, wrists, and ankles. The patient denied having any morning stiffness but complained of worsening fatigue throughout the day. On physical examination, she had a blood pressure of 110/80 mmHg, a temperature of 101°F, a pulse rate of 106/min, and a respiratory rate of 18/min. Pallor of skin and conjunctiva was present. All peripheral pulses were palpable with regular rhythm but low volume. No radio-radial, radio-femoral delay or difference in blood pressure of the upper extremities was noted. No bruit was audible on neck, chest, or abdomen. Mild tenderness over mid-dorsal spine at D7-8 vertebral and paravertebral region was found. There was a full but slightly painful range of motion of the spine. Findings of examination of all other systems, including gynecological and obstetrics examination, were unremarkable.
Laboratory investigations revealed normocytic normochromic anemia with hemoglobin of 9.2 g/dL and hematocrit of 28.9%. Total leukocyte count (TLC) was 9,600 cells/μL with 83% neutrophils and 13% lymphocytes. Erythrocyte sedimentation rate (ESR) was elevated on repeated tests with the latest reports showing levels of 135 mm/h, while C-reactive protein (CRP) levels were normal. Total serum proteins and serum albumin levels were within normal range. Serum globulin level was raised (4.9 g/dL), while albumin to globulin ratio (A/G) was slightly below normal values (0.6). The results for renal function tests, liver function tests, coagulation profile, uric acid, and angiotensin-converting enzyme (ACE) levels were all within normal range. Tests for autoimmune markers including anti-nuclear antibody (ANA), anti-smooth muscle antibody (ASMA), anti-mitochondrial antibody (AMA), anti-cyclic citrullinated peptide (anti-CCP), and rheumatoid factor (RF) were all negative. Blood cultures were also negative. Upon imaging, chest X-ray, echocardiography, and ultrasound abdomen and pelvis showed no abnormalities. X-ray and magnetic resonance imaging (MRI) with contrast of dorso-lumbar spine were also unremarkable.
After a week of extensive inpatient workup with no identifiable cause of fever, the case was labeled as pyrexia of unknown origin. Considering a long-standing history of back pain, fever, weight loss, and an elevated ESR in a tuberculosis (TB) endemic region, there was high suspicion of extra-pulmonary TB. To evaluate for extra-pulmonary TB, and also for vasculitis, possible abscess, and hidden malignancy, a computed tomography (CT) scan with contrast of chest and abdomen was performed. As shown in Figure , it revealed diffuse intimal thickening and dilation in ascending aorta, aortic arch, descending thoracic, and abdominal aorta. Superiorly, the lesion involved both common carotid arteries. Inferiorly, it involved bilateral renal arteries with extension into both common iliac, external and internal iliac arteries. Dissecting intimal flap was seen at the junction of thoracoabdominal aorta. Furthermore, multiple infarcts were noted in the upper lobe of the right lung along with nodularity and septal thickening in the lingular segment. These findings were suggestive of vasculitis.
Assuming a diagnosis of vasculitis, a confirmatory CT abdominal aortography was performed which similarly revealed diffuse intimal thickening of thoracoabdominal aorta along with its focal fusiform dilation and an intimal dip as shown in Figure . Multiple focal intimal thickening and narrowing were noted in superior mesenteric and bilateral renal arteries. Origin of celiac trunk was narrowed. Based on these findings, a final diagnosis of TA type five with aortic dissection and vasculitic pulmonary infarcts was made.
The patient was treated with long-term oral corticosteroid therapy with a dosage of 1 mg/kg daily, which led to a resolution of back pain and fever and decline in ESR levels. Azathioprine was added to sustain remission. The patient is being followed up and remains in remission till date. |
pmc-6453622-1 | A 32-year-old male with no prior medical or surgical history presented emergently with lower abdominal pain, nausea, and vomiting worsening over a two-day period. A physical exam revealed peri-umbilical tenderness with no abdominal distension, guarding, or tender rebound. Of note, an umbilical piercing was present, placed three weeks prior to presentation, surrounded by minor skin erythema. A full blood count revealed an elevated white cell count of 16.5 x 103/uL with a relative neutrophilia of 78%. Normal liver function tests, electrolytes, and amylase were noted. Contrast-enhanced computed tomography (CT) of the abdomen and pelvis was performed, demonstrating a small (2.5 cm) air and fluid-filled collection deep to the umbilicus and extending between a small bowel loop (Figure ).
The appendix appeared normal on CT and a preliminary diagnosis of an infected collection (related to the recently placed umbilicus ornamentation) with intra-abdominal extension was made. The patient was admitted to a general surgical ward and commenced on intravenous (IV) piperacillin and tazobactam, IV fluids, and he was kept nil by mouth. Following 24 hours of conservative management, the patient deteriorated clinically with fever, hypotension, tachycardia, and worsening abdominal pain, now radiating to the right iliac fossa. Blood cultures were drawn and the patient underwent an open surgical intervention. The caecum was identified and appeared normal. The appendix was noted in a retrocecal position and also appeared normal. Following identification of the terminal ileum, the distal ileum was then carefully examined in a retrograde fashion until a blind-ending, inflamed Meckel’s diverticulum was encountered, arising approximately 50 cm from the ileocecal valve proximally (Figure ).
The purulent mesenteric collection identified on CT was also noted, lying deep to the umbilicus but separate from the navel piercing and extending between loops of distal ileum. These findings suggested a perforated Meckel’s diverticulum, rather than an infectious complication related to the recently placed body ornamentation.
A small bowel resection with functional end-to-end anastomosis was successfully undertaken and the surgical site thoroughly irrigated with normal saline solution. The remainder of the small bowel was unremarkable in appearance with no evidence of bowel ischemia.
The post-surgical period was uncomplicated and the patient was discharged on the third postoperative day. He remained well at clinical follow-up eight weeks after the date of his surgery.
Histopathological assessment of the resected specimen of the bowel confirmed MD, the surgical specimen demonstrating features of chronic, transmural ulceration with a small pocket of heterotopic gastric mucosa. |
pmc-6453639-1 | A 69-year-old man with a history of pancytopenia and monoclonal gammopathy of undetermined significance presented with a chief complaint of "the skin on my face and scalp feels uncomfortable". He complained of diffuse facial fullness and burning of approximately three-week duration. Physical examination demonstrated facial edema with firm, violaceous papules subtly coalescing into infiltrative plaques (Figures -).
Punch biopsy obtained from the left cheek revealed a pan-dermal lymphoid infiltrate comprising monomorphous, medium to large lymphocytes with high-grade nuclear atypia (Figure – hematoxylin-eosin, 40x magnification). Immunoperoxidase staining was performed to query the differential diagnosis, which ultimately included precursor B-cell leukemia/lymphoma, myelogenous leukemia cutis, blastic mantle cell lymphoma, and natural killer (NK)/T-cell lymphoma. The atypical lymphocytes lacked cluster of differentiation (CD) 3 or CD56 expression, excluding cutaneous T-cell lymphoma and NK/T-cell lymphoma, respectively. Myeloperoxidase staining was negative, thus excluding myelogenous leukemia. By contrast, the tumor cells strongly expressed Paired Box 5 (Pax-5), as shown in Figure (Pax-5 staining, 40x magnification), and displayed partial positivity for CD79a. Based on these findings, a high-grade B-cell neoplasm was suspected, and subsequent staining for terminal deoxynucleotidyl transferase (TdT) immunostaining revealed avid immunopositivity. The histomorphology and immunophenotype (Pax-5+, CD79a+/-, and TdT+) substantiated a diagnosis of precursor B-cell leukemia/lymphoma in the skin, pending a systemic workup.
Hematologic-oncologic evaluation was initiated. The patient was found to have a profound leukocytosis (43.4 x 103/microliter). Approximately 40% of these cells were atypical myelocytes of uncertain lineage. A bone marrow biopsy showed hypercellular marrow with 94% blast cells. Marrow blast cells expressed Pax-5, TdT, CD19, and CD79a, and were variably positive for CD20, ultimately confirming a diagnosis of acute B-cell lymphoblastic leukemia. The patient was admitted for induction therapy with cyclophosphamide, vincristine, adriamycin, dexamethasone, methotrexate, and cytarabine (Hyper CVAD A). At the last available follow-up, human leukocyte antigen typing was pending in anticipation of a matched unrelated donor transplant. |
pmc-6453640-1 | An 89-year-old female presented with severe, worsening lower back pain for one week. She had a history of severe osteoporosis with previous kyphoplasties at T6, T7, T12, L2, and L4. Four years earlier, the patient underwent an L4-L5 instrumented fusion. An L4 kyphoplasty was performed at the time of the initial L4-L5 fusion. Upon evaluation, there was no neurological deficit. The patient could ambulate with a walker but was severely limited due to pain. Radiographic evaluation revealed a fracture involving the inferior endplate of L5 with associated edema on magnetic resonance imaging (MRI) and computed tomography (CT) (Figures -). Her preoperative visual analogue scale (VAS) in regard to pain intensity was ranked between five to seven on a scale of ten with analgesic medication. After failing conservative treatment with analgesics, and demonstrating almost no tolerance for ambulation, the patient was offered a kyphoplasty and informed consent was obtained. Post-anesthesia care unit (PACU) evaluation after the procedure revealed almost complete resolution of lower back and leg pain. Postoperative VAS evaluation occurred on operative day and revealed a score of two out of ten. The patient was discharged to a subacute rehabilitation facility on the day after the kyphoplasty. On discharge, the patient ambulated with only minimal walker assistance. At a two-month postoperative evaluation, the patient demonstrated continual improvement in ambulation with near complete resolution of her lower back pain.
Surgical technique
The patient was positioned prone on a biplane table. Biplanar fluoroscopy was brought into the field and the targeted vertebral body was localized in both anterior-posterior (AP) and lateral projections. After infiltrating the skin and soft tissue with local anesthetic, a stab incision was made over the right pedicle. The kyphoplasty was performed using the Inflatable Vertebral Augmentation System (IVAS) developed by Stryker® (Kalamazoo, MI, USA). A Jamshidi needle was introduced from a transpedicular approach into the body of L5 via the right pedicle, inferior and lateral to the pedicle screw under biplanar fluoroscopic guidance. The osteo-introducer was then introduced into the vertebral body (Figures -). This created the tract for the inflatable balloon. The balloon was then inserted and inflated to create a cavity within the vertebral body. After the balloon was removed, 5 milliliters of polymethylmethacrylate (PMMA) cement (high viscosity radiopaque bone cement, Stryker®) was infused into the vertebral body. There was no noted extravasation of cement beyond the border of the vertebral body. Once the cement had cured, the Jamshidi needle was removed. Final intraoperative X-rays were performed with fluoroscopy in both AP and lateral projections, confirming acceptable cement fixation without any significant breaching of any borders (Figures -). |
pmc-6453799-1 | A 64-year-old gentleman previously fit and well, apart from mild hypertension, generally active and enjoyed running, presented to the emergency department (ED) with an episode of acute shortness of breath and dizziness after running. He described episodes of progressive breathlessness over the previous few weeks. He was found to have atrial fibrillation (AF) which resolved spontaneously. The diagnosis of paroxysmal AF was made and his symptoms were attributed to his dysrhythmia. CT brain was normal, but a plain PA chest X-ray showed mild to moderate mediastinal calcification. The patient was discharged from ED without further treatment. However, he continued to deteriorate with progressive breathlessness and limitation of exercise tolerance. Eventually he was referred to a cardiologist and diagnosed with CP. The diagnosis was confirmed mainly on MRI scan which showed pericardial thickening. The interventricular septal morphology was abnormal with bowing into left ventricle consistent with ventricular coupling. This is exaggerated during dynamic inspiration and normalises during expiration consistent with constrictive physiology. Subsequently the patient was referred for surgery (, , ).
He underwent successful percardiectomy. The pericardium was stripped from the surface of the heart anteriorly, laterally from phrenic to phrenic and inferiorly. The right atrium was also released up to and around both cavae. He had an uneventful post-operative recovery. He was extubated 6 h post op. He intensive care stay was 40 h, during which he was kept on small dose dopamine (1.3 mcg/kg/min) as a precaution. His hospital length of stay was 5 days. He has returned to full activity and remains asymptomatic. |
pmc-6453824-1 | A 29-year-old male with a history of right tibial shaft and lateral malleolus fractures following a fall injury underwent open reduction and internal fixation with a locking plate for the fibula fracture and minimally invasive percutaneous osteosynthesis for the tibial shaft fracture at another hospital. Postoperatively, the patient was followed up at our hospital for 1 year due to residential issues, and the removal of the plate was scheduled after confirmation of bone union. According to the previous hospital’s medical records, a 9-hole locking plate (APIS®, Gwangju, Republic of Korea) was used for the distal tibia, with three 5.0-mm locking screws for proximal fixation and six 3.5-mm locking screws for distal fixation. Under spinal anesthesia, a plate removal surgery was performed, following which the fibular plate was easily removed. However, five of the six distal locking screws were damaged, and they could not be removed with a screwdriver. This situation was explained to the patient during the operation, and we could confirm that the patient wanted to remove the plate irrespective of the amount of time required. At the time, a tourniquet was used for approximately 1 h, and screw removal was attempted using a conical extraction screw, but only one of the six screws could be removed. Previously proposed techniques requiring additional skin incisions were not considered because there were multiple damaged screws that could not be removed, these techniques would require extended amount of time for removal, and the surrounding soft tissues could be damaged. Hence, we decided to use a screw extractor (IRWIN®, Huntersville, NC, USA), a non-medical instrument, following sterilization with ethylene oxide. In brief, 6-mm drill bit of the extractor was used, four screw heads were drilled, the locking screws and locking plate were separated, and the plate was removed. Of the remaining four screws, one was removed using a vice grip; the remaining three screws could not be removed with the vice grip owing to a short extruded portion. A hollow reamer could not be used because the three screws were adjacent to each other and the reamer could cause a large bone defect; hence, we created sufficient space around the screws by forming small holes around them with a 1.8-mm K-wire, following which all screws could be removed using the vice grip. After saline irrigation, debridement of the soft tissues contaminated with metal debris was performed. The sizes of the metal debris were found to be relatively large, which facilitated easier debris removal. With 2-h use of a tourniquet, all implants could be removed and the skin could be closed (). Postoperatively, no complications were noted during physical and radiological examinations. Remarkably, no problems were noted at the final outpatient visit at 3 months after discharge; therefore, follow-up was terminated. The patient had not visited an outpatient clinic for more than a year since the last follow-up, thus we thought that he had no specific complications including infection and re-fracture. |
pmc-6453832-1 | A 71-year-old gentleman with a history of degenerative left hip osteoarthritis status post left hip arthroplasty 2 months prior presented for evaluation due to several weeks of a worsening back and left leg pain, and new urinary retention with overflow incontinence. Physical exam was notable for pain-limited weakness of the left leg in all muscle groups (4+/5 strength on manual muscle testing) and decreased sensation to light touch from the anterolateral thigh down to the dorsum of the left foot and toe in an L4 dermatomal distribution. As shown in , lumbar MRI demonstrated severe thecal sac stenosis at L3-L4 secondary to bilateral, left greater than right juxtafacet cysts causing compression of the cauda equina nerve roots. A non-contrast computed tomographic (CT) scan showed severe L3-L4 stenosis and lumbar flexion/extension films showed no instability. After discussion of various treatment options, the patient agreed to undergo direct removal of the JFCs with placement of the Coflex® device for dynamic stabilization to avoid a single-level instrumented fusion while attempting to minimize the risk of cyst recurrence. Surgery was performed three days after initial clinic presentation.
Decompression of the thecal sac at L3-4 was accomplished via partial inferior L3 and superior L4 laminectomies and mesial facetectomies via a standard midline approach in the usual fashion (). The juxtafacet cysts were seen bilaterally and completely excised using microsurgical techniques by the senior author. After decompression, a Coflex® (Paradigm Spine, LCC, New York, NY) implant was placed between the remaining L3 and L4 lamina and spinous processes and the tissues were closed in the usual fashion. Total estimated blood loss was approximately 35cc. Juxtafacet cysts were confirmed on pathology. Postoperatively, the patient had immediate improvement in his left leg pain and no complications. The patient was transferred to inpatient rehabilitation on post-operative day 1. At 1-year follow-up the patient continues with complete resolution of his left leg pain and urinary symptoms. demonstrates post-operative standing films and 6-month post-operative CT. |
pmc-6453834-1 | A 45-year-old woman presented for dermatologic examination after a clinical diagnosis of TSC based on a history of facial papules since she was in her twenties, 1 of which was a biopsy-proven AF, as well as seizures during her teens, multiple pneumothoraces, and renal cysts. The physical examination revealed numerous gray-white to skin-colored papules on the nose and medial aspects of her cheeks (, A) and a single gingival papule, but no other significant mucocutaneous findings. A shave biopsy specimen of a nasal alar papule was obtained, and it revealed stellate-shaped fibroblasts with fibrotic dermal collagen displacing solar elastosis, which is characteristic of AF (, B). Computed tomography scans of her chest, abdomen, and pelvis revealed multiple pulmonary and renal cysts. A magnetic resonance imaging scan of her brain revealed no TSC-associated changes.
The lack of additional TSC-related mucocutaneous and internal findings introduced uncertainty in the diagnosis of TSC and prompted evaluation of the patient's 56-year-old sister. Her sister had no significant pulmonary, renal, or neurologic history. Computed tomography scans of her chest, abdomen, and pelvis revealed pulmonary and renal cysts, and a magnetic resonance imaging scan of her brain showed no significant abnormalities. Her skin examination revealed gray-white papules on the face and neck, gingival papules, and axillary and inframammary acrochordons, all characteristics of BHDS. Punch biopsy specimens obtained from papules on her neck and posterior ear revealed cystically dilated infundibular portion of hair follicles containing keratin debris with epithelial strands emanating from the follicular infundibulum () with characteristic dermal collagen, all of which are representative features of FF.,
Given her sister's findings, the patient was reevaluated for additional skin lesions consistent with BHDS. Punch biopsy specimens were obtained from 2 papules on her jawline, and the histopathologic examination showed findings of FF. Germline FLCN testing revealed a nonsense variant (c.1844C>G, p.Tyr463X) in exon 12. |
pmc-6453943-1 | A 20-year-old man presented to the emergency department after four days of progressively worsening periumbilical pain. He was moving heavy boxes for his job when he began experiencing pain and was unable to finish his work. He reported pain with defecation but denied fever, chills, nausea, emesis, weight loss, and recent travel or illness. Past surgical history included branchial cleft cyst excision as a child. On abdominal exam, a one square-centimeter erythematous infra-umbilical mass was exquisitely tender to palpation. Laboratory data on admission demonstrated a WBC count of 10.7 × 103 cells/μL and urinalysis was unremarkable. Based on history and physical exam, the patient underwent diagnostic evaluation for suspected incarcerated umbilical hernia.
CT abdomen/pelvis revealed a four-centimeter segment of organized periumbilical inflammation with central lucency passing the ventral abdominal wall into the anterior abdominal compartment (). The process was extraperitoneal and there was no evidence of communication with the urinary bladder. These findings were consistent with an inflamed urachal remnant complicated by abscess. Our patient received intravenous antibiotics in preparation for an operation. The following day he underwent abscess incision and drainage followed immediately by urachal cyst excision through a four-centimeter infra-umbilical midline mini-laparotomy. The urachal cyst and remnants were dissected inferiorly to confirm no communication with the urinary bladder before total excision (A). Investigation of the cyst contents revealed white sebaceous material (B). Pathology examined the 4 × 3 x 0.7-centimeter segment of fibromembranous tissue and confirmed intraoperative impressions of the specimen ().
The patient was admitted to the surgical floor where he noted his pain was markedly improved. The next day he was discharged to home on post-operative day two with adequate pain control. Two-week follow up in the outpatient surgery clinic confirmed an uncomplicated recovery. |
pmc-6453983-1 | A 70-year-old Korean female was referred to our oral and maxillofacial department with recurrent keratosis in the lower lip over the course of 3 years (Fig. a). She was diagnosed with oral lichen planus (OLP) in another hospital 2 years prior and received a corticosteroid application without complete symptom relief.
The patient looked healthy without any other skin or oral mucosal diseases and had her natural dentition without any removal dentures or prosthetics replacing her anterior teeth on both the maxilla and mandible. The patient also had no history of smoking, alcohol consumption, or hospitalization. Her occupation over the past 30 years involved selling crabs in a large fish market; thus, she often smelled fresh crabs and tasted marinated and seasoned crabs.
The hyperkeratotic white plaque lesion was round and superficial in the lower middle lip site (Fig. a). The patient desired surgical examination after its location altered to the lateral side (Fig. b). A superficial excisional biopsy (Fig. c) was performed, and an initial stage of SCC was revealed. We hypothesized that OLP transformed into malignancy due to chronic irritation of her lower lip. Therefore, additional cancer work-ups such as supplemental images like computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography-computed tomography (PET-CT) were obtained. No metastasis, significant hypermetabolic lesion in the neck, or remaining suspicious lesions were observed in these examinations.
After the first excisional biopsy, a keratotic whitish plaque lesion suspicious of recurrence appeared on the patient 6 months later (Fig. d). After having a consent form for operation, we performed a wide V-shaped wedge resection (Fig. e). Superficial lip mucosa with underlying orbicularis oris muscles were excised with a 5.0-mm safety margin on the lip surface, and direct closure with layered sutures was performed after negative margin confirmation in frozen biopsy.
The specimen was sent to the Department of Oral Pathology at GangneungWonju National University Dental Hospital and fixed, embedded with paraffin, and microsectioned at 4-μm thickness for pathologic diagnosis. The microsections were routinely stained with hematoxylin and eosin and observed under ordinary light microscopy (U-POT®, Olympus Co., Japan). The microscopic images were captured by a digital camera (DP-70®, Olympus Co., Japan) and analyzed for the article submission under the approval of the Institutional Review Board of Seoul National University (S-D2017006).
The microsection exhibited normal architecture of lip mucosal epithelium and fibromuscular adipose tissue containing minor salivary glands (MSGs). The MSGs showed marked ductal hyperplasia with inflammatory cell infiltration. The epithelial tumor became severely keratinized and exhibited comedo-type necrosis and luminal sequestration of the keratinized epithelium mimicking the glandular duct structure, and the tumor cells were relatively well-localized and typically surrounded by abundant lymphoid tissue (Fig. ). Some areas of keratinized tumor epithelium showed the typical features of epimyoepithelial islets seen in Mikulicz disease, and some tumor epithelium formed pseudo-ductal structures with active lymphocytic reactions under high magnification.
The pathologic lesion was confined to the vermilion border without the involvement of the oral mucosa or orbicularis oris muscle with a 2-mm lesion depth. In the periphery region, the tumor cells did not grow invasively, but proliferated in a budding and branching fashion similar to glandular ductal growth. In cytokeratin immunostaining, the keratinized tumor epithelium seemed to float in the lymphoid stroma with no feature of infiltrative growth into adjacent fibromuscular adipose tissue (Fig. ). Therefore, this lesion was finally diagnosed as lower lip EC originating from AC. The patient was instructed to avoid any trauma to her lips and exhibited a favorable outcome during the 5-year follow-up period. |
pmc-6453986-1 | A 34-year-old Filipino man who worked as a sailor presented to our hospital with a 2-day history of acute right lower quadrant abdominal pain and a body temperature of 39.1 °C. He had no past history of medication use or surgical treatment. It was the first time that he had complained of right lower quadrant abdominal pain. On physical examination, he had right lower quadrant abdominal pain with signs of peritoneal irritation, including rebound tenderness. His laboratory data revealed a white blood cell count of 31,500/μl and a C-reactive protein level of 40.14 mg/dl. The results of liver and kidney function tests were within the normal ranges. Computed tomography demonstrated thickening of the appendix associated with mural calcification (Fig. , white arrow) and fluid collection around the cecum, and mural calcification of the descending colon to the rectum (Fig. , white arrowhead). Based on these findings, the preoperative diagnosis was acute appendicitis. We performed an emergency operation on the same day.
Laparoscopic appendectomy was performed. The patient was placed in the supine position and an entry hole into the abdomen was created using an umbilical incision (25 mm). A small wound retractor (EZ Access round type, Hakko, Nagano, Japan) was placed at the umbilicus using a 25-mm incision as an access port with two 5-mm trocars (EZ Trocar, Hakko, Nagano, Japan). An additional 5-mm port was placed at the right lower abdominal region. Swelling of the appendix and contaminated ascites was detected intraoperatively (Fig. ), but there was no evidence of appendiceal perforation. After dissecting the mesoappendix using an ultrasonically activated device (SonoSurg, Olympus, Tokyo, Japan), the base of the appendix was ligated by loop suture (ENDOLOOP Ligature, Ethicon, Somerville, NJ, USA) and resected.
The patient’s postoperative course was uneventful. However, a histopathological examination revealed inflammation of the appendix wall and numerous ovoid bodies within the submucosa, many of which were calcified (Fig. ). Severe infiltration of lymphocytes and fibrosis were recognized around the oval bodies. The numerous oval bodies were morphologically consistent with schistosomiasis (Fig. ). The final diagnosis was acute phlegmonous appendicitis associated with the presence of schistosome eggs. We recognized the type of schistosome eggs as belonging to Schistosoma japonicum (S. japonicum) morphologically. We examined the patient for signs of adult worm activity, but the results of stool ova and parasite examinations performed twice were negative. We decided that additional medicine was not necessary. In addition, the patient hoped to undergo further observation and treatment in his own country, so we did not prescribe praziquantel. He was discharged and returned to his country on postoperative day 9. |
pmc-6454618-1 | A 76-year-old woman was referred to our hospital because of a nodule in the right upper lobe. The patient had no respiratory symptoms. The computed tomography (CT) scan of the chest revealed a 2.3 × 1.6 cm nodule, and the patient was diagnosed with pulmonary adenocarcinoma (cT1bN0M0, stage IA, according to the TNM classification of the Union for International Cancer Control (UICC), 7th edition; cT1cN0M0, stage 1A3, according to the UICC, 8th edition). The patient had no smoking history. Her comorbidities consisted of hypertension and hyperlipidemia. The right upper lobectomy was performed in November 2015, and the pathological stage was pT1bN2M0, stage IIIA. Genomic analysis revealed the epidermal growth factor receptor (EGFR) gene mutation L858R in exon 21. Immunohistochemical analysis revealed a programmed death-ligand 1 (PD-L1) tumor proportion score (TPS) of < 1%. The patient was under watchful observation without adjuvant chemotherapy. Multiple mediastinal and right hilar lymph node metastases were found in February 2018. Radiation therapy alone was selected because the recurrence of the disease was limited to the local region and the patient was 79 years old at the time of recurrence. Radiation therapy amounting to a total dose of 60.0 Gy, distributed in 30 fractions, was performed over a period of 6 weeks. The 3D radiotherapy-planning technique was used. In the first 4 weeks, 40.0 Gy was distributed in 20 fractions using opposing anterior-posterior fields (Fig. a, b). In the subsequent 2 weeks, an additional 20.0 Gy was distributed in 10 fractions using 10 beams (Fig. c, d) for covering multiple mediastinal lymph node metastases but sparing the spinal cord and hilum of the left lung. The planning target volume included multiple mediastinal and right hilar lymph nodes, with a 10 mm margin to account for microscopic disease, internal moving, and setup errors. The percentage volume of lung receiving a dose of more than 20 Gy (V20) was 29.3%. Static radiotherapy was delivered using a 10 MV X-ray and five fractions per week. A chest CT scan performed 6 weeks after the irradiation therapy showed reduction of the lymph node metastases. However, left hilar and right supraclavicular lymph node metastases and multiple pulmonary metastases were newly observed outside of the irradiation field (Fig. ). EGFR tyrosine kinase inhibitor (TKI) treatment was planned as a start. Interestingly, a follow-up chest CT scan performed 12 weeks after the completion of irradiation therapy showed complete disappearance of the multiple pulmonary metastases associated with radiation pneumonitis (Fig. ). However, there was no change in the size of the left hilar and the right supraclavicular lymph node metastases, which was confirmed by 18-fluorodeoxyglucose positron-emission tomography (PET)/CT (Fig. a, b). The patient was therefore under watchful observation, without receiving EGFR-TKI treatment. A follow up CT scan performed 6 months after the completion of irradiation therapy showed a slight increase in the size of the lymph node metastases, but no reappearance of multiple pulmonary metastases nodules (Fig. ). The levels of serum carcinoembryonic antigen (CEA) dropped from 154.5 ng/mL to 30.9 ng/mL after the irradiation therapy and further decreased to 6.8 ng/mL by 3 months post irradiation therapy. The levels, however, increased again to 19.7 ng/mL at 6 months after completion of the irradiation therapy (Fig. ). No cytotoxic chemotherapy or EGFR-TKI was given during the period. The number of CD8+ lymphocytes was 190/μL at 3 months and 356/μL at 4 months after irradiation. |
pmc-6454619-1 | A 49-year-old European man who frequently travelled to West Africa for business was admitted to the intensive care unit of the hospital after travelling to Liberia. He had not taken anti-malarial prophylaxis. The patient was diagnosed with severe P. falciparum malaria based on hyperparasitaemia (25%), acute kidney injury, respiratory distress and hyperbilirubinaemia. Following the hospital protocols, after the first intravenous AS dose, red blood cell exchange was performed. Subsequently, four more doses of intravenous AS were administered. The patient had an excellent clinical, analytical and parasitological evolution with a parasite clearance time of 70 h. Anti-malarial treatment was then completed with a 3-day course of dihydroartemisinin/piperaquine. Seven days after being discharged the patient had to be readmitted due to haemolytic anaemia, with a nadir haemoglobin level of 7.7 g/dL at day 16. DAT was positive for the C3d fraction of the complement and daily treatment with 100 mg methylprednisolone was started. Finally, a blood transfusion was performed after the DAT was negative. The patient was finally discharged with haemoglobin levels of 8.4 g/dL. Two weeks after, the patient remained in good clinical condition and the haemoglobin levels raised up to 9.6 g/dL. |
pmc-6454623-1 | A 6-year-old boy was born of the second pregnancy and first childbirth to healthy nonconsanguineous parents. The first pregnancy ended in an early miscarriage. Family history is not burdened.
Pregnancy was with threatened spontaneous interruption. He was delivered by scheduled Cesarean section due to high myopia. His birth weight was 3650 g and height was 52 cm. Apgar scores were 8 and 9 at 1 and 5 min respectively. He was noted to have hypotonia at birth.
His motor milestones were delayed: held head by 7 months, rolled from back to side at 8 months, put into sitting position by 24 months, walked with support at 2,5 years and walked independently at 4 years. His speech development was delayed: at the age of 6 years he had no words.
At 8 month he was hospitalized to Scientific and Practical Centre of Pediatric psychoneurology. Brain computed tomography revealed signs of leukodystrophy and cortical atrophy of frontal, parietal and temporal areas. Radiological studies (neuroimaging) have never been performed until the age of 8 months.
At 13 months he was admitted to the National Medical Research Centre for Children’s Health and was monitored for the next 5 years.
At admission in 13 months brain MRI revealed signs of leukodystrophy, lissencephaly of left occipital lobe, polymicrogyria of both frontal lobes, secondary ventriculomegaly, brain atrophic changes with enlargement of sub-arachnoid spaces. Biochemical analysis showed creatine kinase (CK) level to be elevated to 2024 U/L (normal range 25–140 U/L), alanine aminotransferase (ALT) to 59 U/L (upper limit of normal 40 U/L), aspartate aminotransferase (AST) to 82 U/L (upper limit of normal 42 U/L) and lactate dehydrogenase (LDH) level to 318 U/L (upper limit of normal 225 U/L). On electromyographic examination, signs of primary muscle lesion have been identified.
At admission in 6 years weight was 21 kg, height was 113 cm, head circumference was 53 cm. Convergent strabismus, insufficiency of convergence and accommodation were noted. He had hypotonia, distal more than proximal. Trunk ataxia, walking on his toes with an atactic component, flat valgus foot were noted. Patient reacted favorably to examination. He can maintain short-term eye contact; speech is misunderstood. The face is hypomimic. In active speech there are long vocalized sounds, rarely - simple babbling syllables, no words. Behavioral features are manifested by periodic fading with subsequent rapidity of breathing and stereotyped swings of hands (Fig. ).
Orthopedic examination revealed thoracic hypokyphosis, lumbar hyperlordosis, double-sided coxa valga, pathological antetorsion on the right, equinox flat valgus feet. Limb movements were not restricted except for restriction of back flexion in the ankles.
Ophthalmological examination revealed congenital high myopia, partial atrophy of an optic nerve and retinal atrophy in both eyes.
MRI revealed hypoplasia of temporal lobes and opercular areas from two sides, hypoplasia of caudal parts of the cerebellum worm with the expansion of external subarachnoid spaces of the cerebral hemispheres (more in the temporal areas), expansion of retrocerebellar cistern, symmetrical expansion of the ventricular system, pachygyria-polymicrogyria of cerebral hemispheres with preservation of the occipital lobe. Increased intensity of MR signal was observed in T2 and T2 FLAIR images. Decreased intensity of MR signal was observed in T1 image. In the cerebellum multiple small cysts, hypoplasia of the corpus callosum and pons with the extension of the anterior and covering cisterns are revealed (Fig. ).
Biochemical analysis showed CK level to be elevated to 2253 U/L (normal range 25–194 U/L), ALT to 70,4 U/L (upper limit of normal 40 U/L), AST to 62 U/L (upper limit of normal 42 U/L) and LDH level to 503 U/L (upper limit of normal 314 U/L). Other blood and urine indicators were within normal limits. Patient level of CK was increased 10–15 times and for two years decreased from 3126 (3 years 4 months) to 1986 (at 5 years 2 months). The level of CK-MB was also increased - 61.1 (upper limit of normal 25 U/L).
On electroneuromyographic examination (ENMG), signs of primary muscle lesion have been identified. Motor unit potentials (MUP) were short in duration and low in amplitude. The number of polyphasic MUP has increased. Spontaneous activity in each test muscle was determined.
Electroencephalography (EEG) did not reveal epileptiform activity.
The electrocardiogram (ECG) showed a vertical position of the electrical heart axis, mild bradycardia and arrhythmia, nonspecific intraventricular block, early repolarization syndrome of the ventricles.
A standard karyotype was normal 46, XY. Tandem mass spectrometry did not reveal any changes in the plasma level of amino acids and acylcarnitines.
Genotek Ltd. did clinical exome sequencing. Genomic DNA from peripheral blood sample was extracted using QIAamp DNA Mini Kit (Qiagen) according to manufacturerʼs protocol. DNA libraries were prepared using NEBNext Ultra DNA Library Prep Kit for Illumina (New England Biolabs) with adapters for sequencing on Illumina platform according to manufacturer’s protocol. We used SureSelect XT2 (Agilent Technologies) for target enrichment. Enriched samples were sequenced on Illumina HiSeq 2500 using pair-end 100 base pairs reads. After sequencing, we trimmed 3′-nucleotides with read quality below 10 using Cutadapt []. Raw reads were aligned to reference genome hg19 (GRCh37.p13) using BWA MEM []. Deduplication of reads was done using SAMtools rmdup []. FastQS was used for data quality control []. We called short variants using GATK HaplotypeCaller [] according to GATK Best Practices DNA-seq [, ]. The effect of each mutation was assessed using snpEff []. To assess pathogenicity and conservatism, the data was extracted from the dbNSFP [], Clinvar [, ], OMIM database [] and HGMD [], as well as using the SIFT [] and PolyPhen-2 [, ] utilities to predict pathogenicity of the mutation. Information on frequency of mutations was taken from 1000Genomes project [, ], ExAC [, ] and Genotek frequency data. Pathogenicity was predicted according to the Standards and Guidelines developed by ACMG (American College of Medical Genetics and Genomics), AMP (Association for Molecular Pathology) and CAP (College of American Pathologists) []. Copy number alterations were determined using CNVkit [].
Sanger sequencing confirmed POMGNT1 pathogenic variants identified by exome sequencing. For labeling amplicons with fluorescent labels, BigDye Terminator Cycle Sequencing Kit v3.1 (Thermo Fisher Scientific) was used. Sanger sequencing was performed on ABI PRISM 3500 Genetic Analyzer (Applied Biosystems) according to manufacturerʼs protocol.
After exome sequencing, patient was commenced on muscle relaxants and anticonvulsants. His condition is stable. |
pmc-6454674-1 | A previously healthy 14-year-old girl presented with cough, sputum and shortness of breath after activity. She had a history of trauma 10 days prior to presentation. She was previously admitted to another hospital and diagnosed with tuberculosis. Antituberculosis treatment was ineffective; therefore, she was transferred to our hospital. She had no family history of genetic or osteolytic disease. She was admitted to the respiratory department with dyspnea and persistent cough. Examination revealed tachypnea, diminished breathing sounds, a deformity on her back, and tenderness. She exhibited percussion pain in the T6–T9 vertebrae and an absence of motor power in the thoracic spine. Neurological examination was normal.
Plain radiographs revealed an osteolytic lesion in the thoracic spine (Fig. ). Thoracic computed tomography (CT) showed a moderate right-sided pleural effusion and atelectasis (Fig. ). Her thoracic spine CT revealed the presence of ill-defined lytic lesions in the ribs and the T6–T9 vertebrae as well as a fracture in the T7 vertebra (Figs. and ). Magnetic resonance imaging (MRI) scans revealed a pathological fracture and spinal canal stenosis at the T7 vertebra and high intensity in the T6–T9 vertebral bodies (Figs. and ). Whole-body bone scintigraphy was performed, and radiolucent foci were observed in the fracture lesion on the radiographic images. Blood analyses indicated nearly normal biochemical levels, except for a high concentration of cross-linked N-terminal telopeptides of type I collagen (111.60 ng/ml) and decreased vitamin D (8.99 ng/ml).
Recurrent chylothorax was managed via repeated thoracentesis, and percutaneous fine needle aspiration of the lesion yielded more than 1000 ml/day of a reddish turbid, nonodorous fluid. Analysis of the aspirate revealed a positive Rivalta test result, which was reported as chylothorax. The patient was transferred to the thoracic surgery department to control the pleural effusion. A thoracic duct ligation and pleurodesis along with chest drainage was planned. The biopsy could not be analyzed because insufficient tissue was taken from the lesion during the process. Chest CT showed bilateral pleural effusions 2 days after surgery (Fig. ), and the chest was drained on the left side. To investigate the lesion pathology, the patient underwent another incisional biopsy of the T6–T9 vertebral bodies at the department of spine surgery. The bones appeared honeycomb-like intraoperatively. Postoperational pathological examination of the incisional biopsy revealed many dilated sinusoids with hemorrhaging, mononuclear and lymphocytic infiltration, fibrous tissue and dead bone (Figs. and ). Based on the clinical, radiological and pathological findings, we confirmed the diagnosis of GSS because the biopsy material was negative for bacterial and fungal growth, and osteolysis was clearly demonstrated in the imaging results.
No treatment has been approved for GSS; thus, several treatment methods are used. In our case, the treatment plan was discussed and confirmed in a multidisciplinary clinic meeting. Bisphosphonates and vitamin D therapy were administered to treat the disease because the patient was vitamin D deficient, and the disease is self-limiting. Because the neurological exam showed no abnormalities, conservative treatment was considered, and a custom-made polypropylene body jacket was prescribed to prevent kyphotic deformity. Her clinical status improved steadily following the oral bisphosphonates and vitamin D supplementation. A final thoracic CT (Figs. and ) was performed 2 years after diagnosis and showed a successful reduction in the amount of pleural fluid and stabilization of the thoracic spine deformity. |
pmc-6454690-1 | An 89-year-old Japanese woman was admitted to our hospital because of general fatigue, nausea, muscle weakness of the lower limbs, and ophthalmoplegia that had appeared 2 months earlier and gradually worsened. She noticed diplopia at 1 year before this hospitalization. None of her family members had a history of renal or autoimmune disease. There was no past history of allergic diseases such as bronchial asthma. The patient had never smoked. She had been diagnosed with hypertension and treated with an angiotensin-receptor antagonist (losartan, 50 mg daily).
The physical examination identified a slight fever, anemic conjunctiva, eyelid ptosis, and muscle weakness of the lower limbs; her blood pressure was 135/67 mmHg. The laboratory examination revealed elevated serum levels of creatinine (1.91 mg/dL, 170 μmol/L) and C-reactive protein (8.12 mg/dL). A hemogram showed anemia (hemoglobin concentration, 9.2 g/dL), but her white blood cell count was in the normal range (5400 /μL). On urinalysis, occult blood and protein and cellular (granular and red blood cell) casts were detected. Her urinary protein excretion was 2.13 g/g creatinine. The serological examination revealed an elevated level of serum IgG (3494 mg/dL), but serum complement levels were within normal limits: the serum C3 level was 95.1 mg/dL, the serum C4 level was 21.7 mg/dL, and the serum CH50 level was 44.7 U/mL. Positivity for MPO-ANCA (176 U/mL) and PR3-ANCA (125 U/mL) was observed, but rheumatoid factor, antinuclear antibody, anti-glomerular basement membrane antibody, cryoglobulin, and monoclonal immunoglobulin were not detected.
We also assayed for various types of ANCA in the patient’s serum. A sample was collected in a separator tube before the patient’s initial treatment and was separated at 1000 g for 15 min. The diluted serum sample was measured by an enzyme-linked immunosorbent assay (ELISA) using a Wieslab® ANCA panel kit (EuroDiagnostica, Malmo, Sweden), in duplicate. The ELISA plate was read on a microplate reader (Sunrise Remote®: Tecan Japan, Kanagawa, Japan) set at 405 nm wavelength. The patient showed positivity for azurocidin-ANCA (optical density [OD] ratio: 4.05, normal: < 3.0), but not bactericidal/permeability increasing protein (BPI)-ANCA (OD ratio: 1.61, normal: < 3.0), cathepsin G-ANCA (OD ratio: 1.20, normal: < 3.0), elastase-ANCA (OD ratio: 0.99, normal: < 3.0), lactoferrin-ANCA (OD ratio: 2.77, normal: < 3.0) or lysozyme-ANCA (OD ratio: 1.47, normal: < 3.0) [see Additional file ].
The patient’s symptoms and inflammatory findings did not improve with antibiotic treatment (ceftriaxone, 2 g daily for 6 days), and her serum creatinine level deteriorated to 2.42 mg/dL (Fig. ). On abdominal ultrasound examination, her kidney size was relative small (right, 78 mm × 40 mm; left, 87 mm × 46 mm). We diagnosed rapidly progressive glomerulonephritis.
Light microscopic findings of a renal biopsy sample showed cellular crescents in 50% of 14 obtained glomeruli, and a fibrocellular crescent was revealed in one of those glomeruli. Mononuclear inflammatory cell infiltration to the interstitium was widely observed. Vasculitis was not observed, but intimal thickening of the interlobular arterial walls was seen (Fig. ). On immunofluorescence findings, immunoglobulins and complement components were not detected. We therefore diagnosed microscopic polyangiitis with cellular-type renal involvement.
Chest X-rays showed a wide mediastinum, and chest computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 40-mm-sized lobulated tumor in the anterior mediastinum (Fig. ). On additional serological examination, anti-acetylcholine-receptor antibody was present (0.9 nmol/L), but anti-muscle specific kinase (MuSK) antibody was not detected. We thus additionally diagnosed myasthenia gravis with thymoma.
After the renal biopsy, the patient was treated with 500 mg/day of methylprednisolone for three consecutive days, followed by 30 mg/day (0.8 mg/kg) of oral prednisolone. Rituximab 500 mg (375 mg/m2) was intravenously administered, but it was discontinued after only one administration because of an adverse effect (atrial fibrillation). Three months after the remission induction therapy, the patient’s MPO-ANCA was decreased to 25.3 U/mL, PR3-ANCA was decreased to 15.2 U/mL, and elevated serum IgG level was normalized to 1297 mg/dL. Her symptoms had improved; at 2 months after the initiation of treatment, anti-acetylcholine-receptor antibody became negative and the size of her thymoma had decreased to approx. 20 mm (Fig. ). However, hemodialysis was initiated at 1 month after this treatment because the patient’s renal function was deteriorated. Her renal function did not unfortunately recovered, and she was moved to another hospital 3 months after the therapy because she needed rehabilitation for her muscle weakness due to disuse syndrome. |
pmc-6454696-1 | A 31-year-old male presented with chest pain and hoarseness of recent onset. The patient had suffered a traffic accident 3 months before admission, resulting with femur, radius, ribs and sternum fractures; and had undergone internal fixation of the femur and radius. The physical examination was normal. Computed tomography (CT) showed a ruptured aortic arch with pseudoaneurysm. The crevasse measured 20 mm and was located in the posteroinferior aspect of the aortic arch, involving the posterior wall of the innominate artery and the origin of left common carotid artery. The pseudoaneurysm of 40 mm × 48 mm × 30 mm was located in the upper mediastinum. The distance from the junction of aortic sinus and ascending aorta to the rupture was 7.9 cm (Figs. a-c). The recent appearance of symptoms suggested that the pseudoaneurysm had increased rapidly and compressed the left recurrent laryngeal nerve. In this case, to reduce the risk of cardiopulmonary bypass or cardiac arrest under deep hypothermia [], a hybrid operation was performed: debranching + thoracic endovascular aortic repair (TEVAR). The involved branches of the aortic arch were rebuilt with artificial vessels, and a covered stent was placed in the aortic arch for endovascular repair via femoral artery. A hybrid operation is safer, more feasible, and more comprehensive than other treatments for some high-risk patients. Thoracic and abdominal CT angiography performed on the 7th postoperative day showed that the contrast agent did not leak and that the reconstruction of blood vessels was smooth (Figs. d and e). Recovery was uneventful. |
pmc-6454698-1 | A 50 year-old male, with a history of coronary arterial bypass grafting 14 years back, presented with shortness of breath and dry cough. An X-ray revealed a large mass in the left hemithorax adjacent to the heart silhouette. A chest CT demonstrated the presence of a mass with smooth edges, in middle mediastinum next to the heart and partially intrapericardial (Fig. ). The mass was of heterogeneous density and of 11 cm size. Presence of atelectasis at the left lower lobe abating the mass was clearly seen. Based on clinical and radiologic evidence, we did proceed with CT guided FNA of the mass. The cytology findings revealed inflammatory lesion. Laboratory tests were normal. Based on patient symptoms, history and the presence of a mass potentially compressing the cardiopulmonary structures in vicinity, we decided to offer exploratory surgery for diagnosis and treatment.
Standard hemodynamic monitoring and general anesthesia were followed by positioning, prepping and draping patient in left lateral decubitus position. An anterolateral left thoracotomy was carried out and entrance in the hemithorax was made without any challenge. The mass was assessed and found to be leaning medially on the surface of the lateral wall of the left ventricle, including the pericardial layer and had smooth edges which didn’t infiltrate the lung (Fig. ). We started dissecting the mass from its smooth capsule, making it through all its layers. An old and degraded piece of surgical swap was visualized (Fig. ). The surgical swap was removed along with the capsular layer of this mass. Patient tolerated the procedure very well and blood loss was minimal. A chest tube was inserted in the left hemithorax and chest wall was closed following standard procedures.
In the immediate post-operative phase, patient improved steadily and on day four was discharged home symptom-free. In the long-term follow -up, patient was found to remain without symptoms. |
pmc-6454710-1 | A 74-year-old male visited a local doctor in May 2018 due to a 20-day history of progressive dysphagia. He had no other symptoms suggestive of esophageal cancer. Electronic gastroscopy showed an elevated tumour in the lower segment of his esophagus, which revealed an esophageal adenocarcinoma on biopsy. The complete blood count revealed a total leucocyte count of 24,870/mm3 and the peripheral blood smear showed differential counts of 89, 5, and 4% for neutrophils, lymphocytes, and monocytes 4%, respectively. Since he had no fever, the treating physician did not suspect an inflammatory reaction or abscess, but considered this to be a manifestation of a secondary haematologica disorder. Chest computed tomography (CT) showed thickening of the wall of the esophagus, pulmonary inflammation, and mild bronchiectasis. Aspirates and a biopsy from the bone marrow (Fig. a) revealed granulocytosis that had proliferated actively, abundant droplet bodies in the cytoplasm, and an increased number of megakaryocytes. The positivity rate of alkaline phosphatase expression was also increased. These results confirmed a diagnosis of LR and excluded chronic myelogenous leukaemia (CML). The patient was referred to our hospital in Jun 2018 for further evaluation and treatment. The patient had an unremarkable family history. On examination, there were no palpable supraclavicular and bilateral cervical lymph nodes, and his body temperature was normal. His vitals were stable with a normal blood pressure, regular heart rate, and normal pulse rate. However, his weight had decreased by 4.5 kg since he had begun to experienc dysphagia. Laboratory investigations included a routine blood test (total WBC: 29,960/mm3, neutrophils: 89.5%, lymphocytes: 7.5%, monocytes: 2.3%, and haemoglobin: 138 g/l) and analysis of tumour markers (alpha-fetoprotein: 1.41 ng/ml, squamous cell carcinoma: 0.1 ng/ml, and carcinoembryonic antigen: 1.01 ng/ml). C-reactive protein was elevated (14.36 mg/dl). Yhe uric acid levels and liver and kidney function tests were within normal limits.Hyperleukocytosis, elevated C-reactive protein, and the neurologic features on the day of admission were indicative of an infectious pathology. Initially, the hyperleukocytosis was considered to be related to an infection. Subsequently, the patient was treated with biapenem (0.3 g twice dailyfor days 1–6) and voriconazole (200 mg twice daily for days 1–6). His WBC counts were 25,290/mm3 and 28,730/mm3 on the 4th and 6th days of treatment, respectively. All the blood cultures taken were negative. Persistent leucocytosis after 6 days of antibiotic treatment indicated that this could not be caused solely by infection. We therefore concluded that the LR was most probably caused by esophageal adenocarcinoma. A radiographic examination of the upper gastrointestinal tract (Fig. b-c) revealed a mass in the lower esophagus. A chest CT (Fig. d) showed thickening of the wall of the esophagus, corresponding regions of luminal stenosis, and several enlarged lymph nodes around the lesser curvature of the esophagus. No primary or metastatic tumours were observed. An abdominal ultrasound was performed, which identified no metastases in the liver. A radical esophagectomy was then performed. The resected specimen showed an elevated tumour measuring 6.5 × 6.0 × 1 cm in the lower segment of the esophagus and gastroesophageal junction (Fig. e). Microscopically, the tumour was a moderately differentiated adenocarcinoma invading the serous layer (Fig. f). A prominent intravascular proliferation of tumour cells was observed, which occasionally formed tumour thrombi. Clearance of lymph nodes during the radical esophagectomy revealed no grossly enlarged nodes. The tumour was diagnosed to be of stage IIB (T3N0M0), according to the American Joint Committee on Cancer guidelines.
Postoperatively, the WBC counts successively decreased to 10,450/mm3 within 1 week and 8670/mm3 at 1 month. The patient received 4 cycles of nedaplatin (200 mg on day 1), fluorouracil (1.0 g on days 1--5), and calcium folinate (0.3 g on days 1--5), and has been free of recurrence for 6 months. |
pmc-6454718-1 | A 70-year-old type II diabetic male with the history of gut gangrene presented at surgical department of a public sector tertiary care hospital in Lahore, Pakistan. He went through surgical procedure of partial small intestinal resection, sparing 1 ft proximal jejunum and 2–3 ft distal ileum. Drainage jejunostomy was performed. After the surgery, the patient was kept on inadequate peripheral parenteral nutrition (PPN) (2 l/day) for 4 days. Excessive losses through the fistula during this period and unnoticed hydration inadequacy led to the development of hypovolemia. Based upon biochemical analysis, elevated BUN (115 mg/dl), and creatinine levels (7.2 mg/dl), the patient was diagnosed with acute renal failure. The random blood glucose levels were found to be within controlled range (148 mg/dl). He was referred to nephrology department of the same health care facility where hypovolemia was reversed through the administration of intravenous rehydration fluids (saline 5% dextrose solution with KCl 2.5 cc/ml). After 15 days, acute renal failure was settled; BUN and creatinine reached within normal range (16 mg/dl and 1.1 mg/dl, respectively). However, the patient on discharge was given an inappropriate ileostomy feed plan including ORS (800 ml/day), milk (600 ml/day), polymeric supplemental formula (1800 ml/day), and low dose aspirin therapy through feeding ileostomy. Moreover, he was given broth and blended bread slices by his caregivers through ileostomy. Poorly planned ileostomy feed led to several complications, resulting in increased hospital stay, and serious implications on his physical, psychological, and social wellbeing.
After 23 days, the patient returned to the hospital with relapse of acute renal failure (BUN = 32 mg/dl, creatinine = 1.6 mg/dl). The patient showed signs of severe dehydration along with pedal edema upon physical examination. A history of 16% weight loss within the last month (BMI = 19.5 kg/m2), mild anemia (Hb = 11.7 g/100 ml), and hypoalbuminemia (2.8 g/dl) indicates poor nutrient uptake and declining nutritional status. Because of the declining health of the patient, early anastomosis was carried out to restore intestinal continuity. At this point, a trained dietitian intervened and planned a post-surgical progressive diet along with careful monitoring of hydration status. Total parenteral nutrition [TPN] was recommended initially with a gradual transition to oral feed. The target caloric requirement of the patient was calculated to be 2400 kcal with fluid intake of 6000–7000 ml.
For the first 2 days post-surgery, electrolyte and fluid balance were maintained through intravenous solutions. From the 3rd day onwards, an individualized TPN plan along with the administration of rehydration fluids through feeding ileostomy was started with the target of improving the nutritional status, allowing for postoperative stress and maintaining the hydration and electrolyte status (Table ).
The caloric requirement of the patient for initial total parenteral feed was calculated to be 1700 kcal (BEE + 20% sedentary factor + 15% for uncomplicated surgery + 15% for severe weight loss). Initial TPN plan provided 1500 kcal from elemental formula (amino acids), sorbitol, and 25% dextrose solution. An intravenous fat emulsion (20% Liposyn) was infused on alternate days providing 20 g fat/day for preventing the fatty acid deficiency. In addition, a 10 ml ampoule of multivitamin (Multibionta) was administered daily in order to fulfill the requirements of vitamins A, E, C, and B complex (Table ).
TPN was slowly tapered off by slow addition of ileostomy feed (Table ) and then to final transition towards oral feed (Table ).
A high protein, high carbohydrate (low lactose, low fiber), and low-fat diet was planned (Table ). On the 5th day after anastomosis, the patient received first oral intake of clear fluid diet along with TPN. The total calories were kept within the range of 1100–1500 kcal. Upon introduction of oral feed, severe dysphagia and withdrawal of food were observed which, upon probing, was revealed to be due to the overlooked oral hygiene and prolonged NPO period with subsequent pharyngitis and mouth ulcers. Antiseptic mouthwash was started promptly. The patient’s caregivers were counseled regarding effective feeding and caring practices. The patient also contracted urinary tract infection for which antibiotics were prescribed. Mobility was encouraged by suggesting supported sitting and walking along with frequent side changing while lying in order to recover from bed sores. After maintaining the tolerance of clear fluid diet for 2 days, the patient was shifted to full fluid diet along with TPN. Oral fluid intake (drinking water) was also started. On the next day, the patient was suspected to develop TPN-induced hepatic dysfunction based on clinical signs of jaundice which was confirmed by the abnormal pattern of hepatic enzymes (ALP = 465 U/L, ALT = 40 U/l, AST = 52 U/l) and hypoalbuminemia. TPN was discontinued.
Soft, bland diet (Table ) consisting of easily digestible food items from all food groups and nutritional supplement along with PPN (10% dextrose), intravenous albumin infusion, and oral multivitamin syrups was then introduced. Soft diet was continued for 2 weeks with gradual increase in calories. The patient was discharged with a recommendation of a detailed soft, bland diet plan able to meet 1750 Kcal (Table ).
The patient’s caretakers were guided for food substitutes for adding variety in diet and increasing the palatability and acceptance. The caregivers were guided for foods to avoid and were given balanced diet recipes for incorporating all the food groups in the diet (Table ).
Throughout the nutritional management, the blood glucose levels of the patient were carefully monitored (below 180 mg/dl) and insulin units were precisely adjusted for the grams of carbohydrates fed. Two weeks after discharge, the patient was called for a follow-up. He showed compliance with dietary recommendations and medical prescription, and his health showed a gradual improvement. The patient was suggested to decrease the amount of nutritional supplement while increasing the exchanges for other foods. Dietary recommendation of adding 2 teaspoons of fat (canola oil) and chicken/vegetable curry was given. Local flat bread (chapatti) made of extracted wheat flour was allowed. On the next follow-up visit, the patient showed slight weight loss and reduced compliance to diet. The dosage of dietary supplement was again increased, and caregivers were given strict instructions for dietary compliance until the patient regained weight and gradually started having family meals with slight modifications. |
pmc-6454729-1 | A 22-year-old woman presented with intense pain in the shoulders that began 2 weeks after injury during a CrossFit competition, especially on the right side, without edema or reduced range of motion. She started the CrossFit practice routine 2 months before the injury. The patient trained CrossFit three times a week without any other sport activity on the remaining week days. Olympic weightlifting (overhead movement) was described as the last activity before the acute pain started. The initial overhead load was 55 pounds and was part of a “novice” training routine with reported adequate technique. One week prior to the CrossFit competition the patient was oriented to increase the Olympic weightlifting load to 75 pounds as a requirement for competition. Repetitions were set to her own limitation. After the injury, she stopped her exercises and applied ice to the shoulders and upper arms. During the following days, she experienced continuous pain and sought medical attention. Physical examination did not show any reduction of passive and active arcs of movements. Also, Jobe and Bear Hug tests were negative. There was only a mild ligamentous laxity and 10 degrees of elbow hyperextension. Her medical history did not include medications, prior fractures, neoplastic disease or prior surgery on the upper extremities. Also, she did not have history of anabolic or glucocorticoid steroid use. The patient’s height was 1.53 m and weighted 54 Kg, with a BMI of 22.9 and was physically active for more than 3 years. She has never had CrossFit lessons or prior training. Before she started CrossFit practice, the patient used to swim three times a week in college, but did not participate in competitions. The patient had an episode of amenorrhea 3 years ago, considered to be due to weight loss, probably due to intense training and reduced caloric intake. Laboratory tests and densitometry were performed in the same period of the imaging studies and showed normal mineral bone density and Vitamin D insufficiency (serum Vitamin D of 24.8 ng/mL). During the two initial weeks of the patient’s bilateral upper limb pain, she attributed the symptoms to “overtraining” but she sought medical attention when the pain became progressively worse. The patient did not refer any specific diet, nutritional supplementation or reduced caloric intake during the time of the shoulder lesion.
Radiographs of the shoulders were performed 1 week after onset of symptoms.
These were interpreted as normal, showing no evidence of bone lesion, fracture line, arthritis, or periosteal bone formation. However, small and incomplete fracture lines may not be seen on early radiographs. At that time, the patient was presumed to have bilateral impingement syndrome. MRI of the shoulders were performed 2 weeks after the onset of symptoms (Figs. and ) to exclude other causes of shoulder pain such as rotator cuff injuries, bursitis, bone marrow edema and small fractures The MR images of the right shoulder showed extensive bone marrow edema throughout the right humeral medullary cavity. In addition to marrow edema, there was an incomplete transverse fracture line adjacent to the medial cortex of the surgical neck and mild soft tissue edema (Fig. ). Further, there was an intense edema of the subscapularis muscle, with scattered pattern suggesting overload injury probably related to delayed onset of muscle soreness (DOMS), without tears. (Figs. and ) On the left side the findings were less prominent, and similarly showed bone marrow edema of the proximal humerus, without a defined fracture line, considered to be the result of joint / bone overload (Fig. ). Additionally, there was a mild edema of the subscapularis muscle, without tears (Fig. ).
The patient was treated nonoperatively with activity modification, without immobilization. She received oral non-steroid anti-inflammatory drugs for 5 days and 20 physiotherapy sessions for analgesia, stretching and strengthening of the rotator cuff and deltoid. Also, the patient had vitamin D reposition with 7000 IU twice a week in the first month, with 7000 IU weekly as maintenance. One month later the patient was asymptomatic and 3 months later she resumed CrossFit training against medical advice not to return to sports without follow-up imaging.
Follow-up MRI was done 4 months after the initial studies and showed complete resolution of bone edema and consolidation of the transverse fracture line on the right proximal humeral diaphysis. Also, there was complete resolution of bone marrow edema on the left humerus, and no visible bilateral subscapularis muscle edema or tear (Figs. , , , ). After 6 months of follow-up the patient was asymptomatic. |
pmc-6454752-1 | A 64-year-old female was referred to the hospital by her gastroenterologist after a same-day colonoscopy revealed a large rectosigmoid mass resulting in near total rectal occlusion. She had a past medical history of tobacco smoking and NSCLC (T1bN3M0 stage IIIB), diagnosed 1 year prior (Fig. ). She was thought to be in remission following radiation and immunotherapy with pembrolizumab.
On admission, she complained of progressively worsening abdominal pain and constipation. Vital signs and measurements were unremarkable. Physical examination was primarily benign. Notable laboratory findings only included elevated carcinoembryonic antigen of 4.2 ng/dL. Computerized tomography (CT) imaging showed a severe colonic stool burden and a soft tissue left upper lobe lung mass consistent with patient’s NSCLC history. A single large soft tissue mass with possible mucosal invasion in the rectosigmoid colon was noted (Fig. ). She underwent urgent diagnostic laparoscopy that was quickly converted to open exploratory laparotomy due to numerous bowel-to-bowel and bowel-to-anterior abdominal wall adhesions. At that time, a rectal mass appeared to be invading into the small bowel. Ultimately, colostomy, ileocecectomy and anastomosis, and rectosigmoid mass resection with tissue sampling were performed. She tolerated the procedure well, and her immediate postoperative course was uneventful.
Rectosigmoid mass biopsies revealed positivity for high-grade NSCLC and favored metastatic poorly differentiated adenocarcinoma of lung origin. Hematoxylin and eosin (H&E) staining showed rectosigmoid mass tissue exhibiting extensive necrosis, focal mucosal involvement, and negativity for regional lymph node carcinoma. Also, normal appearing colonic glandular cells were surrounded by atypical cells infiltrating the colonic stroma. To evaluate these high-grade and poorly differentiated malignant changes further, properly controlled routine immunohistochemical (IHC) stains for cytokeratin 7 (CK7), thyroid transcription factor-1 (TTF-1), Napsin-A, epithelial specific antigen/EpCAM (Moc-31), Ber-EP4, p63, cytokeratin 5 or 6 (CK5, CK6), caudal type homeobox 2 (CDX2), and cytokeratin 20 (CK20) were performed not only based on the patient’s age, gender, and past medical history, but also her recent clinical, radiologic, and operative findings. Additional properly controlled IHC stains for leukocyte common antigen (CD45), melanoma antigen recognized by T cells (MART-1), gross cystic disease fluid protein 15 (GCDFP-15), estrogen receptor (ER), synaptophysin, neural-cell adhesion molecule (NCAM/CD56), and chromogranin were performed due to the unusual presentation and nature of the case. The malignant cells exhibited strong positive immunoreactivity for CK7, and positive TTF-1 Napsin-A, Moc-31, and Ber-EP4, while showing only minimal focal staining for p63 and cytokeratin 5 or 6 (CK5, CK6). The tumor was negative for CDX2, CK20, CD45, MART-1, GCDFP-15, ER, synaptophysin, NCAM/CD56, and chromogranin (Fig. ). Mucicarmine staining was equivocal for intra-cytoplasmic mucin. This IHC staining profile (strongly positive CK7 and positive TTF-1/Napsin-A with negative CDX2/CK20) supported metastatic adenocarcinoma of lung origin, rather than primary colorectal adenocarcinoma. This hypothesis was supported by numerous colonic and regional lymph node samples lacking malignant carcinoma cells and properly controlled IHC stains of right colon and ileum biopsy cells exhibiting negative immunoreactivity for CK7, TTF-1 Napsin-A, Moc-31, and Ber-EP4.
Her postoperative course was uneventful, and she was discharged home. The patient was started on systemic chemotherapy with carboplatin and pemetrexed followed by radiation to the pelvic region for metastatic NSCLC. She did not tolerate chemoradiation therapy well. During the treatment period, she developed considerable pelvic pain resulting in a significant performance status decline. She also experienced multiple prolonged hospitalizations due to infections. Subsequent positron emission tomography–CT (PET-CT) scans suggested refractory pelvic tumor growth. Additional radiation for palliation of pain by reducing pelvic tumor size was determined reasonable. However, the patient experienced a debilitating stroke and was transitioned to hospice care. |
pmc-6454808-1 | A 39-year-old New Zealand man of European descent initially presented with syncopal events in the context of excessive daytime sleepiness and exertional dyspnoea. He was a never-smoker and had a community clinical diagnosis of asthma with no objective evidence of bronchial hypersensitivity. Examination revealed morbid obesity (body mass index 48.8 kg/m2) and retrognathia. Epworth sleepiness score was 23/24 and arterial blood gas showed awake hypercapnia consistent with obesity hypoventilation syndrome. Overnight sleep study confirmed severe sleep disordered breathing (apnoea-hypopnoea index 143/h, lowest saturations of 61%). He was issued home continuous positive airway pressure treatment, however was not adherent to therapy. Subsequently he presented to hospital five times over six months with increasing dyspnoea. While he was unable to perform acceptable spirometric manoeuvres, carbon dioxide transfer factor was 5.1 mmol/kPa/min (62% predicted). Peak expiratory flow rates during admission showed minimal reversibility (350 mL, 70% predicted) and symptoms persisted despite treatment with long- and short-acting bronchodilators. Transthoracic echocardiogram was attempted but technically limited due to the patient's body habitus. It showed normal concentric left ventricular wall thickening, with both ventricles of normal size and systolic function, and no valvular pathology seen.
The patient underwent a high-resolution computed tomography (CT) to exclude abnormalities of lung parenchyma contributing to dyspnoea. The predominant abnormality was large volumes of mediastinal and pleural fat. The pleural fat was predominantly based around the upper lobes with obtuse margins without any significant lobulation and had an average density of −110 Hounsfield units, consistent with lipomatous tissue (Figs. , ). Following multi-disciplinary team discussion, review of images, and all available results, a diagnosis of exertional dyspnoea secondary to obesity with significant pulmonary restriction due to pleural and mediastinal lipomatosis was made. The recommendation was adherence to CPAP and weight loss. |
pmc-6454817-1 | A 51-year-old female with a history of rheumatoid arthritis and a 10.5-pack-year smoking history presented with an aspergilloma in her right lung. After failing medical management, she was treated with a right pneumonectomy at an outside institution. This was complicated by BPF and empyema of the pneumonectomy cavity. She underwent two additional thoracotomies requiring rib resection, and placement of serratus anterior and later latissimus dorsi flap to close the fistula. Seven months following her last operation, she presented to us with stridor, persistent cough, and dysphagia, concerning for postpneumonectomy syndrome. Review of last computed tomography (CT) imaging from three months after the pneumonectomy revealed a multiloculated pleural space, with air fluid levels in the pneumonectomy cavity. An updated CT scan showed interval progressive rightward mediastinal shift with nearly complete obliteration of the pneumonectomy cavity by the heart (Fig. ). A bronchoscopy was performed, which demonstrated narrowing of the left mainstem bronchus (Fig. a) and stenosis of the lower lobe bronchial orifice due to external compression of the airways. Results of a previous complex right-sided BPF with two areas of disrupted bronchial staple line were noted (Fig. b).
The patient elected to proceed with operative correction of her postpneumonectomy syndrome. A thoracotomy in the fifth intercostal space was performed and dense adhesions in the chest with rotation of mediastinal structures were faced. Upon entering the pleural space, a small loculated serous fluid collection was encountered. To rule out an infected field, the pleural rind and fluid samples were sent for intraoperative gram stains, which returned negative. Cultures were also submitted. The mediastinum was mobilized from the chest wall, taking care to avoid damage to the muscle flaps, which had previously sealed the BPF. As a result, only the mid and inferior portion of the mediastinum was mobilized. A saline immersion test was performed to ensure the integrity of the muscle flap seal over the right mainstem bronchus. The implants were sized based on measuring the amount of saline instilled in the chest, and close hemodynamic monitoring of arterial and central venous pressures. Before placing the implants, the thoracotomy was closed temporarily after placement of implant sizers, monitoring hemodynamics to ensure there was no right heart compression. Two implants (250 mL and 100 mL) were placed into the pleural cavity, and the thoracotomy was closed. The postoperative recovery was uneventful. The patient was discharged on post-operative day 5. She noted complete resolution of her stridor, cough, as well as dysphagia four weeks post-operatively. Her post-operative chest radiograph showed partial medialization of the inferior mediastinum with persistent rightward deviation of the proximal trachea (Fig. ). At time preparation of this manuscript, the patient continues to have full resolution of symptoms at fourteen months following surgery. |
pmc-6454929-1 | On May 10, 2017, a 49-year-old Chinese woman was admitted to the Third Affiliated Hospital of Harbin Medical University due to gross hematuria for four days, with a presentation of intermittent right loin pain for two years. Besides hematuria, pyuria was found in initial urinalysis. Physical examination showed notable percussion pain over the right costovertebral angle. Magnetic Resonance Imaging (MRI) showed that the right kidney was enlarged and severely damaged (). Histopathology results for the right kidney were consistent with renal cell carcinoma. The patient was advised to undergo radical nephrectomy. However, she refused surgery and was discharged from hospital. On October 10, 2017, the patient expelled two living worms, and presented gross hematuria and loin pain. The worms were blood red in color, approximately 25 cm in length and 5–7 mm in width and tapered at both the anterior and posterior ends. On the following day, one worm was sent to our department for confirmation. Based on the morphological characteristics of the worm, it was identified as D. renale (). The patient was advised to take albendazole as treatment. During the treatment, she expelled another 13 worms and some fragments. After that, her symptoms improved over the following days. However, recently, cancer cells spread to the lungs and bones and the patient could not walk. |
pmc-6455819-1 | A 67-year-old woman with cholecystitis was hospitalized for upper abdominal cramps. Laparoscopic cholecystectomy was performed, and her abdominal cavity was explored under monitoring. Her gallbladder was then removed and packed in a specimen bag to be taken out for examination. The doctor inadvertently perforated her intestinal tract with the laparoscopic machinery. The doctor attempted to conceal the accident from the patient's family and secretly suture closed the crevasse to escape responsibility. As a result, the patient developed severe peritonitis and died about 2 weeks after the operation. An autopsy and contrast-enhanced PMCT were performed. External forensic examination and conventional autopsy were carried out. The isolated intestine was then prepared for the examinations described below.
This study was approved by the Academic Committee of the Institute of Forensic Science, Ministry of Justice, People's Republic of China. Written informed consents were obtained from the victim's family to publish these case details.
Contrast-enhanced PMCT of the isolated intestinal tract was carried out after the autopsy. First, we inserted a urinary catheter into the upper end and ligated the 2 ends of the intestine. The whole process was divided into 5 steps: CT scanning, air-enhanced CT scanning, water-enhanced CT scanning, formalin-unfixed intestinal CT scanning, and formalin-fixed intestinal CT scanning. The isolated intestinal tract was supported by a handmade device (Fig. ) and scanned using a 40-slice multislice CT system (SOMATOM Definition AS; Siemens Medical Solutions, Munich, Germany). Air was then injected by an air pump, and water and contrast medium (meglumine diatrizoate and 0.9% normal saline at a 10:1 ratio) were injected through the urinary catheter. Raw data were acquired using the following settings: voltage, 120 kV; current, 240 mA; and collimation, 6.0 × 1.0 mm. Image reconstruction was achieved at slice thicknesses of 5.0 mm and 0.625 mm.
The body was 154 cm tall and of medium build. Observations included about 400 ml of yellow turbid liquid within the abdominal cavity, omental adhesion, and a 2-cm crevasse 24 cm from the ileocecal section that was sewn with 6 stitches. No leakage of intestinal contents was detected in the crevasse. Another 0.9-cm × 0.3-cm crevasse was observed next to the stitches. The edge of the crevasse was regular, dark brown, and deep within the abdominal cavity. Squeezing the intestinal tract resulted in overflow of a small amount of intestinal contents from the crevasse (Fig. ).
CT scanning showed partial gas-filled intestine, but most of the intestine was not obvious. No significant lesions were detected (Fig. ). Air-enhanced CT scanning showed air overflow through the crevasse, but injected air was still present in the intestinal tract behind the crevasse because the air pump was being continuously ventilated. As a result, inflation of the entire intestinal tract increased the difficulty of finding the crevasse (Fig. ). Water-enhanced CT scanning was then performed, and radiographs showed a gas- and water-filled intestinal tract (Fig. ). The effect of water was not as obvious as that of contrast medium, and the result was not well appreciated. The most appreciated effect was the contrast-enhanced unfixed intestine (Fig. ). The filling effect of formalin-fixed intestine was not as good as formalin-unfixed intestine because of the increased brittleness and expansion of the fixed intestine. In the three-dimensional volume rendering of the contrast-enhanced intestine, a trace of contrast agent flowing out of the crevasse could be detected (Fig. ). Three-dimensional volume-rendered CT images were also obtained in the other steps. |
pmc-6455880-1 | A 73-year-old Chinese man was found a cervical mass for 1 month. He had a history of coronary heart disease for 2 years, but no relevant personal or family history of malignancy. Physical examination showed bilateral neck asymmetry, the trachea was deflected to the left, the carotid pulse was normal, approximately 6 × 5 cm solid masses were felt in the right lobe of the thyroid gland. The tumor mass border was unclear with smooth surface, and the tumor moved up and down with swallowing. The left lobe of the thyroid gland had no palpable mass and the neck had no palpable swollen lymph nodes. Accessory examination of neck color Doppler ultrasound at our hospital showed increase in size of the right lobe of the thyroid gland, which had abnormal shape. The upper right pole of the thyroid gland had an approximately 5.6 × 6.0 × 4.2 cm sized mass, with unclear margin, and multiple cystic nodules in the thyroid. The CT of thyroid showed increase in size of the right lobe of the thyroid gland, and an approximately 5.6 × 6.0 × 4.2 cm sized mass, the trachea was compressed and deflected to the left, the thyroid cartilage was compressed, deformed and reached the right subglottic region. The right thyroid lump had multiple calcifications, indicative of a tumor (Fig. ). Electronic laryngoscope examination showed ventricular bands thickening, the right ventricular bands compartment showed a rice-like projection, arytenoid region movement was poor (Fig. ). Thyroid function test was 5.07 mIU/L. The patient underwent subtotal thyroidectomy and excisional biopsy of neck mass under general anesthesia. Intraoperative findings indicated a hard mass in the thyroid gland area, the lateral border and the lower bound were closely related to the surrounding soft tissues, but there was an extremely close relationship between the medial border, the thyroid cartilage, and cricoid cartilage, with no gap between them. An ENT doctor was invited for intraoperative consultation, who suggested that some of the tumors should be removed and frozen. The rapid pathology of the tumor (thyroid right lateral lobe) indicated chondroma. The ENT doctor suggested laryngeal chondroma resection and tracheotomy under general anesthesia. Intraoperative findings indicated that the right lower half of the thyroid cartilage and the right half of the cricoid cartilage were chondroma, and complete resection of the tumor was performed. Postoperative pathology examination showed (right lobe of thyroid gland and larynx) chondroma (Fig. ). Given the advanced age of the patient, long surgical duration and poor cardiorespiratory function, the patient suffered sudden cardiac death after the operation. |
pmc-6456117-1 | An 18-year-old female student was referred to our hospital presented with dry cough and dyspnea on exertion for 6 months. The patient denied fever, hemoptysis, weight loss, or tobacco use. Past medical history included immune thrombocytopenia for 3 years before the visit, for which she received 1.5 years of oral glucocorticoid. Laboratory examinations revealed iron deficiency anemia (hemoglobin = 90 g/L), platelet count of 6 × 109/L, and total white cell count of 4.4 × 109/L. Urinalysis, liver function, electrolytes, and creatinine were within normal ranges. Her erythrocyte sedimentation rate (>140 mm/h), C-reactive protein (150.1 mg/L), and serum IL-6 (7.3 pg/mL), IL-8 (279 pg/mL), tumor necrosis factor-α (175.0 pg/mL) were significantly elevated. Serum measurement of immunoglobulins showed elevated total IgG of 27.59 g/L, while serum IgG4, M protein, serum, and urine immunofixation electrophoresis and light chain were negative or normal. Hypocomplementemia (C4 = 0.079 g/L), positive antinuclear antibodies (titer 1:160), and positive Coombs test were found. Additional clinically relevant autoantibodies, (1–3)-β-D-glucan, galactomannan, interferon gamma release assay, and tumor markers were negative or normal. Pulmonary function tests revealed restriction and decreased diffusing capacity with forced vital capacity of 2.05 L (65.9% predicted), total lung capacity of 2.83 L (68.9% predicted) and diffusing capacity for carbon monoxide of 28.8% predicted. Chest computed tomography (CT) showed multiple well-defined nodules randomly distributed in both lung fields and mediastinum lymphadenopathy, with no significant pleural effusion (Fig. ). Bronchoscopy demonstrated no endobronchial pathological lesions, and a culture of bronchoalveolar lavage fluid showed no evidence of tuberculosis or fungal infection. Cytological evaluation was negative for malignant cells. A CT-guided percutaneous lung biopsy was performed, and histopathological examinations revealed some defining features for hyalinizing granuloma, including homogenous hyaline lamellae around small blood vessels and dense infiltrates of lymphocytes, plasma cells with frequent lymph follicle formation (Fig. ). The patient was diagnosed with PHG, and received prednisone 1 mg/kg/d and tacrolimus. Unfortunately, 6 months after initiation of therapy with prednisone gradually tapered, neither pulmonary lesions nor platelet level revealed remarkable interval change. As detailed investigations in clinic failed to uncover any more underlying condition, the patient underwent video-assisted pulmonary wedge biopsy.
Intraoperatively, multiple white and tough-elastic nodules were found in the upper and lower lobes of lung, partially fused to mass, with pulmonary parenchyma edema surrounded. One isolated lesion was dissected from the left upper lobe, which contained 2 nodules. The diameters of nodules were 1 and 2 cm, and specimens were sent for microorganism culture and pathological examination, respectively. Microorganism culture was negative. Pathologic exam revealed nodular interstitial pink deposits in lungs which stained pink with hematoxylin and eosin, and pathologic examination also showed lymph node hyperplasia and follicular architecture along broncho vascular bundle and under the pleura, with plasma cell infiltration (Fig. a). On immunohistochemical stains, the hyperplastic cells were positive for CD20 (follicle), CD138 and negative for CD3, IgG, and IgG4. Congo-red, alcoholized Congo-red (Fig. b), alkaline Congo-red stains, and kappa and lambda light chain immunostains were positive. Histological evaluation demonstrated a revised diagnosis of intrapulmonary CD with secondary amyloidosis. Human herpesvirus-8 (HHV-8) was tested to be negative. |
pmc-6456129-1 | A 73-year-old man was admitted to our department following the incidental finding of a focal liver mass in computed tomography (CT) scan. The patient reported no symptoms, other than upper abdominal discomfort. He had no significant medical history and no remarkable family history. Physical examination did not show any abnormalities. Findings for all clinical laboratory tests conducted at our institution, including those for the carcinoembryonic antigen and α-fetoprotein tumor markers, were within the normal range (Table ). However, he tested positive for serum markers of hepatitis B virus (HBV) infection (i.e., antigens and antibodies to hepatitis B surface protein, hepatitis B e protein, and hepatitis B core protein), with a viral load of 1.99 × 103 IU/mL, but was negative for hepatitis C virus and human immunodeficiency virus.
The mass in the left hepatic lobe (segment II) was detected on plain CT as a hypodense lesion (Fig. A). Contrast-enhanced CT and gadopentetate dimeglumine-enhanced magnetic resonance imaging (MRI) were performed for precise imagistic evaluation. The CT revealed the mass as a faint enhancement during the arterial phase (Fig. B). However, in the MRI, the lesion appeared to have low intensity on T1-weighted imaging (Fig. C), slightly high intensity on T2-weighted imaging (Fig. D), and intense restriction of diffusion on diffusion-weighted imaging (Fig. E).
Based on the clinical and radiologic findings, the lesion was diagnosed as malignant. The patient consented to undergo a laparoscopic left lateral segment liver resection (segments II–III). The gross finding following the resection was a white-colored, 1.8 cm, nodular tumor mass (Fig. ). Histologic analysis of the resected tissue revealed a large number of atypical lymphocytes diffusely infiltrating the hepatic lobule and the portal area and lymphoepithelial lesions with small to medium-sized lymphocytes on some of the bile capillaries. Immunohistochemical analysis showed that the lymphocytes were positive for CD20, Ki67, PAX-5, BCL-2, CD79a, CD21, and CD23, but negative for Bcl-6, CD3, CD5, CD10, CD43, CD56, CD138, MUM1, and cyclin D1 (Fig. ).
Based on the above-mentioned pathologic features, the patient was diagnosed with primary hepatic MALT lymphoma, specifically a low-grade malignant extranodal marginal zone B-cell lymphoma. The patient refused any additional treatment after the surgery and remained disease free throughout the 6 months of follow-up (at the time of writing of this report). |
pmc-6456133-1 | A 38-year-old woman (gravida 5, para 3+1) had been delivered of 2 healthy sons and 1 healthy daughter via lower segment CSs in other hospitals 15, 11, and 8 years, respectively, prior to the current presentation. At 32+1 weeks of gestation, she presented at the emergency department of our hospital because of spotting. Her current pregnancy was not registered with any hospital and she had received no antenatal care. She had noted intermittent minor spotting between 28 and 30 weeks of gestation but had not visited any hospital or received any treatment.
On presentation to our hospital, she was found to have a hemoglobin (HGB) concentration of 87 g/L. Ultrasonography revealed that the lower edge of the placenta was completely covering the internal cervical orifice and was suspicious of the presence of PPP (Fig. ). A plain magnetic resonance imaging (MRI) revealed that the pernicious placenta previa was completely covering the internal cervical orifice, she had diffuse placenta accreta and also placenta percreta on the lower anterior uterine wall, and distended and twisted vessels were visible on the surface of the lower anterior uterine wall (Fig. ). A diagnosis of PPP was made. The patient was admitted to the obstetric ward and treated with dexamethasone to promote pulmonary maturation.
At 33 weeks gestation, she had 450 mL vaginal bleeding and was immediately transferred to the operation room for emergency CS. On arrival at the operating room, her blood pressure was 125/75 mm Hg and heart rate 90 beats/min. A resuscitation team comprising senior staff of the departments of gynecology, obstetrics, anesthesiology, and neonatology, the intensive care unit (ICU), blood bank and laboratories, and a nursing team was immediately established. The anesthesiologist immediately punctured an artery to enable invasive blood pressure (BP) monitoring, and devices for autologous blood recovery were assembled. Nurses established four 16 G intravenous lines immediately for fluid infusion. The cumulative preoperative blood loss was 1290 mL (including the 450 mL episode). Because the patient's preoperative HGB was 87 g/L and she had known placenta accreta, 4.5 IU of blood were obtained from the blood bank before CS.
Remifentanil 70 μg, propofol 120 mg, and succinylcholine 100 mg were intravenously infused, and tracheal intubation performed under general anesthesia. The infant was delivered 4 minutes after commencing the operation, had an Apgar score of 8-9-9, and was transferred to the neonatal ICU for further treatment. Rapid and massive bleeding occurred immediately after delivery but was decreased markedly in the surgical field by binding the lower uterine segment with a tourniquet. However, the BP inexplicably remained at about 75/45 mm Hg despite rapid blood and fluid transfusion. The explanation for this became evident when it was found that a large amount of blood had flowed from the vagina onto and beneath the operating table as a result of the failure of the cervix to contract (Fig. ). An emergency protocol for massive bleeding was immediately initiated. After an on-the-table consultation, hysterectomy was performed immediately.
The total intraoperative blood loss was 13,400 mL. The patient was transfused with autologous blood (1781 mL), allogeneic leukocyte-free red blood cell suspension (21 IU), fresh frozen plasma (2200 mL), cryoprecipitate (6 IU), machine-collected platelets (1 IU), fibrinogen (4 g), crystalloid fluid (8700 mL), and hydroxyethyl starch (1000 mL). Upon completion of the surgery, her HGB was 76 g/L and activated partial thromboplastin time (APTT) 48 seconds.
Postoperative inspection of the uterus confirmed placenta accreta in the anterior uterine wall of the lower uterine segment. Parts of the placenta had implanted into the cervical canal, particularly its posterior wall, where it almost reached the external cervical orifice. Postoperative pathologic examination of the operative specimen again confirmed the presence of placenta accreta (Fig. ). Postoperatively, the patient was returned to the ward safely and recovered well. Her HGB was 71 g/L on the 2nd postoperative day. On the 6th postoperative day, her HGB was 81 g/L and the APTT and prothrombin time were normal. Both the parturient and neonate were well and discharged. |
pmc-6456136-1 | A 45-year-old Chinese male, suffering from intermittent diarrhea and bloody mucosanguineous feces for 3 years and aggravation for 4 months was referred to our department after 1 week of recurring low-grade fever. The patient had been experiencing diarrhea with dull pain in the left abdomen and a loose, visible mucus, and purulent sanguineous stool, 3 to 4 times a day for 3 years. He had initially responded to combined therapy with oral mesalazine (1 g/tid), local mesalazine suppository (0.5 g/tid), followed by oral triplex live bacteria capsules (630 mg/tid). The patient responded positively to this treatment for 3 months, passing yellow stool 1 to 2 times daily, with no mucus, pus, or abdominal pain. However, for 4 months before admission, the patient had diarrhea 6 to 7 times a day, with bloody mucosanguineous feces, and abdomen pain, with no response to local mesalazine suppository (1 g/tid). He had a body temperature of 37.4°C for 1 week before admission, had smoked for 20 years, and had a history of hepatitis B (HBV). A physical examination found no obvious abnormality of the abdomen.
All abdominal enhanced CT showed lesions of distal colon, descending colon, sigmoid colon, and rectum consistent with UC, as well as reactive lymph node hyperplasia. The patient was diagnosed with UC based on a colonoscopy (Fig. A) and histology of a biopsy (Fig. B). The colonoscopy showed diffuse erosive ulcers in the descending colon, sigmoid colon, and rectum. A pathologic biopsy showed acute and chronic inflammation of the mucous membrane with superficial erosion, the occasional cryptic abscess, mild atypical hyperplasia of some glands, and infiltration of lamina propria lymphocytes, plasma cells, neutrophils, and a few eosinophils.
The patient received oral mesalazine (1 g/tid) and hydrocortisone (0.3 g/d) but symptoms did not improve. After 10 days, the treatment was changed to oral methylprednisolone (0.6 g/d) and a hydrocortisone enema (0.1 g/late). The patient underwent a total colectomy and ileostomy because he did not respond to treatment after 2 weeks and had HBV replication. Furthermore, 30 mg Urbason was given for symptom treatment postoperation.
Gastroscopy was performed 12 days after the operation, revealing no lesion in the gastroduodenal area, because approximately 600 mL of tartar-like stool was found from anastomosis. Twenty days later, a 2nd gastroscopy was performed, revealing diffuse erosive ulcers and bleeding in the descending duodenum. A pathologic biopsy showed acute and chronic inflammation of the mucous membrane with superficial erosion (Fig. ). An arteriography of the gastroduodenal artery was performed and can be seen the contrast agent spillover and intestinal canal staining in the branch of the gastroduodenal artery, and be considered the arterial branch of the pancreas and duodenum. Vital signs gradually steadied after embolization. After 23 days, the patient presented with hematemesis, abdominal pain, and low fever. Abdominal drainage was yellow-green, and abdominal CT examination indicated intestinal perforation. The anterior wall of the duodenal descending segment was necrotic and defect about 2 × 3 cm during surgery. Following surgery, the patient died of septic shock. |
pmc-6456136-2 | The patient, a 55-year-old Chinese female, presented with intermittent diarrhea with little bloody mucosanguineous feces, 4 to 6 times a day, and pain in the lower left abdomen. The symptom could relieve for 10 years. An endoscopy showed rough and eroded mucosa of the rectum and sigmoid colon, the vascular network was not clear, there were a few pus secretions, and 3 polypoid protrusions were seen in the sigmoid colon. An endoscopic polypectomy was performed in 2010, and postoperative pathology revealed inflammatory polyps. The patient was diagnosed with UC according to histopathologic criteria. She received intermittent oral sulfasalazine and mesalazine for maintenance treatment, intermittent hormone enemas, and Chinese medicine. This treatment relieved symptoms, but episodes of diarrhea continued.
The patient experienced aggravated symptoms from 2013. Endoscopic examination revealed stenosis of 4 cm at the entrance to the large intestine, mucosa was rough and bleeding easily, and the endoscope could not pass through the colon. Furthermore, the pathologic biopsy revealed rectal adenocarcinoma (Fig. ). On January 5, 2013, a total colectomy and ileostomy were performed with the patient under general anesthesia. The postoperative histology biopsy showed: the ileum 4 cm in length and 4 cm in diameter; the total colorectal anal canal 84 cm in length and 3.5 to 7 cm in diameter; chronic inflammation present in the mucous tissue along the 35 cm ranging from the pectinate line; dilated and congested interstitial blood vessels; erosion ulcers and recess abscesses; focal glands showing atypical hyperplasia; and increased submucous fibrous tissue. The medium and low differentiated adenocarcinomas were found 3 cm from the pectinate line. The volume of the adenocarcinoma was 6 × 3.5 × 0.5 to 0.8 cm, and there was invasion of the fibrous membrane, the recidivist nerve, and the tumor thrombus in the vascular canal. There was no cancer invasion on both sides and metastatic carcinoma in the mesenteric lymph nodes. The clinical stage was T3N2Mx, Dukes C stage. Combined with the clinical history, these factors indicate carcinogenesis in UC. After surgery, the patient received N1-(2 tetrahydrofuryl)-5-fluorouracil (FT-207) and 8 g, 300 mg, and 100 mg in oxaliplatin chemotherapy, and biologic therapy.
Eighteen months after surgery, the patient was admitted to hospital following upper abdominal pain and acid regurgitation. A gastroscopy found inflammation in the descending part of the duodenum. Endoscopic ultrasonography revealed: diffuse thickening of the muscularis mucosa in the post and descending duodenum; rough and eroded mucous membrane, normal submucosa and inherent muscularis mucosa structure, narrowing of the descending intestinal cavity, and nonspecific inflammatory changes. The endoscope found that duodenal sphere and the descending stenosis, making it difficult to insert the mirror; mucous membrane edema; extensive ulcers, white mass scattered in the polypoid protrusion, and a lesion approximately 6 cm in length over the narrow segment and the smooth mucous membrane of the distal intestinal mucosa. Pathologic biopsy revealed mucosal superficial ulcers, a large amount of acute and chronic inflammatory cell infiltration, and moderate hyperplasia of the glandular epithelium (Fig. ). The patient recovered well without recurrence by taking proton pump inhibitor. |
pmc-6456151-1 | In October 2017, a 73-year-old male presented with abdominal distension and dark-red bloody stool of 6-month duration. He also complained of general fatigue and weight loss of 15 kg. He had no familial history of cancer, no prior pathological conditions, and no concomitant medication use. The patient's carcinoembryonic antigen (CEA) and hemoglobin levels were 6.9 ng/mL (0–5 ng/mL) and 101 g/L (131–175 g/L), respectively. The results of all other laboratory tests were normal, including cancer antigen 19 to 9 (CA 19–9), biochemical, and hematologic tests. Endoscopic examination revealed an obstructing neoplasm in the hepatic flexure, about 4.5 cm in diameter, with surface depression, erosion, and a propensity for bleeding. A biopsy of the lesion established a diagnosis of moderately differentiated adenocarcinoma. Whole-abdomen computed tomography (CT) revealed wall thickening of the hepatic flexure with proximal incomplete intestinal obstruction (Fig. A). The CT scan also revealed a single low-density lesion of about 5.7 cm diameter in the spleen (Fig. B). Genetic testing of the biopsy material indicated non-mutated KRAS, NRAS, and BRAF genes. After the first MDT discussion, due to the poor general condition of the patient, we decided to remove the primary lesion and biopsy the splenic mass during the operation. The biopsy indicated the presence of splenic metastasis from adenocarcinoma. The patient underwent a laparoscopic right hemicolectomy due to the histopathological finding of a moderately and poorly differentiated adenocarcinoma invading the serosa. Twenty-two lymph nodes were removed and 7 showed metastases (pT3N2M1, stage IV). One month later, the patient's CEA level had decreased to 3 ng/mL. The patient's postoperative recovery was uneventful, and 1 month after surgery he began treatment with Xelox (capecitabine 1000 mg/m2, oxaliplatin 130 mg/m2) every 3 weeks for 3 months. After 4 courses of Xelox therapy, the patient's clinical response was excellent, with II degree vomiting and no obvious bone marrow suppression or neurotoxicity. The diameter of the splenic lesion had decreased from 5.7 to 2.5 cm (Fig. C), and a partial response had been achieved after 4 courses of chemotherapy. In April 2018, based on a second MDT discussion, the patient underwent laparoscopic splenectomy. The histological findings showed that the splenic tumor was a moderately and poorly differentiated adenocarcinoma, similar to the tumor of the hepatic flexure (Fig. A). Negative staining for cytokeratin 7 and positive staining for cytokeratin 20 was consistent with splenic metastasis of an adenocarcinoma of the hepatic flexure (Fig. B and C). The patient's postoperative course was uneventful and he completed 4 courses of postoperative chemotherapy using the original chemotherapy regimen Xelox (capecitabine 1000 mg/m2, oxaliplatin 130 mg/m2). The patient's clinical response was good, with II degree vomiting, II degree bone marrow suppression, and II degree neurotoxicity. The patient was subsequently followed up every 3 months and no signs of recurrence were noted in a recent examination on January, 2019. |
pmc-6456155-1 | The patient, a 31-year-old woman, was admitted to the department of neurosurgery at The First Hospital of Jilin University due to a “right occipital mass for 5 months”. The patient was in good health, and her family members did not have similar lesions. A physical examination showed that the mass was in the right occipital region at the upper margin of the sternocleidomastoid muscle and exhibited slight tenderness without obvious pulsation or vascular murmurs during auscultation.
A head CTA examination at admission suggested that the lesion was approximately 3 × 5 cm in size, was mainly composed of vascular components, presented as a “wool mass”, was located in a subcutaneous region, involved the muscles, and showed obvious enhancement. The lesion was mainly supplied by the occipital and muscular branches of the vertebral arteries. The venous drainage of lesion communicated with the suboccipital venous plexus and the paravertebral venous plexus, and the venous drainage communicated with the subcutaneous cervical superficial veins (Figs. and 2).
A further DSA examination revealed that the blood supply of the lesion was mainly from the occipital artery and less from the muscle branches of the vertebral arteries (Fig. ). The lesion was considered an AVM based on its imaging characteristics and was recommended for surgical removal. The surgery was carried out under general anesthesia. The lesion could be touched when the occipital skin was incised during the operation. The lesion showed a clear boundary. The occipital artery touched the anterior margin of the lesion, was exposed and ligated, and was removed around the lesion. The lesion consisted of massive blood vessels, and the surrounding muscles were swollen, indicating IH.
After the lesion was removed, the normal muscle tissue around the lesion was also removed. The specimen was sent to pathology, and postoperative pathology confirmed that the lesion was IH accompanied by thrombolytic recanalization. HE staining showed that the lesion almost purely consisted of capillaries with only rare cavernous components among the muscles. CD31 staining was positive in the vessel endothelium, indicating that the lesion was an IH (Fig. ). The patient achieved a good recovery after surgery and was discharged from the hospital. A head CTA review performed one year later showed partial residual IH (Fig. ). The patient had no obvious discomfort, and the hemangioma could not be touched from the surface, and the patient was recommended for radiation therapy. The patient refused radiation therapy. Follow-up 2 years later showed no enlargement of the IH. |
pmc-6456281-1 | The patient was a 73-year-old man who was initially admitted for an elective right-sided total knee replacement to alleviate many years of suffering from severe osteoarthritis. His past medical history consisted of coronary artery disease, which led to a coronary artery bypass graft (CABG) procedure that was completed six years prior to this admission. The patient's baseline electrocardiogram (EKG) showed a normal sinus rhythm (NSR) with a first-degree atrioventricular (AV) node block and an incomplete right bundle branch block (RBBB) (Figure ). His most recent echocardiography revealed an ejection fraction of 41%-45% with mild aortic valve stenosis. The surgery was completed without any complications. However, the patient received spinal anesthesia with bupivacaine preoperatively, as well as a right femoral nerve block with ropivacaine postoperatively. Subsequently, he was noted to have three episodes of bradycardia, hypotension, and one incident of cardiac arrest following the procedure. Upon a review of the telemetry strip, the patient developed severe sinus bradycardia with progressive sinus slowing to a heart rate in the 20s, PR interval prolongation, followed by a brief period of asystole (Figure ). At the time of cardiac arrest, chest compressions were started, atropine was administered, and return of spontaneous circulation (ROSC) was achieved within 20 seconds. A transvenous pacer was placed and the patient was treated for suspected local anesthetic toxicity in the postanesthesia care unit (PACU) with Intralipid, with a resolution of symptoms. The patient’s heart rate and blood pressure returned to baseline following the post-infusion of Intralipid. He was then evaluated by electrophysiology (EP) and a loop recorder was placed without any evidence of a complete heart block or a high degree of atrioventricular (AV) nodal block. |
pmc-6456283-1 | A 36-year-old, previously healthy Caucasian woman, presented with complaints of sudden onset of chest pain. The patient described the pain as crushing, pressure-type, located in the sub-sternal region and radiating to the left arm. The pain started while she was showering in the morning, and after the shower, she felt light headed and called the emergency medical services (EMS). The patient has a 20 pack-year history of smoking. Upon arrival of the EMS, the patient went into cardiac arrest with ventricular fibrillation. She required defibrillation two times in the field with successful return of spontaneous circulation; she was then brought to the emergency department (ED). In the ED, the patient's electrocardiogram (ECG) showed ST-segment elevations in the anterior leads with ST-segment depression inferiorly with reciprocal changes (Figure ).
Her laboratory evaluation was also significant for hemoglobin at 16.5 g/dL, hematocrit at 50.3%, and a platelet count of 1095 x 103/ul. She was hypokalemic with a potassium of 3.0 mmol/L, HCO3 was low at 16 mmol/L with an anion gap of 18. Her troponin level was elevated at 0.16 ng/ml initially and later peaked to 42.8 ng/ml.
The patient was immediately taken to the cardiac catheterization lab and coronary angiography was performed which revealed 100% occlusion in the proximal left anterior descending (LAD) artery with thrombus. The thrombus was treated with intra-coronary eptifibatide; AngioJet (Boston Scientific Corp., Natick, MA) aspiration thrombectomy was performed with placement of a single drug-eluting stent in the proximal LAD (Figure ).
The patient did well post-procedure without any major complications. Work up for patient's thrombocytosis was initiated. Microscopic evaluation of peripheral smear was notable for thrombocytosis and erythrocytosis in the blood. Platelet function collagen/epi and adenosine diphosphate (ADP) were high. At this stage, as a myeloproliferative neoplasm was suspected, the patient was started on cytoreduction therapy with hydroxyurea. She was also tested for BCR/ABL1 gene re-arrangement, which was found to be normal. The patient's Janus kinase 2 (JAK2) V617F mutation testing was positive which increased suspicion for ET, with a small possibility of primary myelofibrosis. She also had a bone marrow biopsy which showed normocellular bone marrow with myeloid and megakaryocytic hyperplasia and no increase in reticulin fibrosis (Figure ).
The morphologic findings in conjunction with the positive JAK2 V617F mutation analysis of the peripheral blood specimen confirmed the diagnosis of a myeloproliferative neoplasm. The differential diagnosis includes ET versus early stage/prefibrotic stage of primary myelofibrosis (PMF). The overall findings favoured ET.
The patient continued to be on hydroxyurea with a gradual fall in platelet count. She did not require plateletpheresis or a second agent to lower her cell count. |
pmc-6456285-1 | A 47-year-old homosexual male presented to the emergency room with a five-day history of intermittent frontal and retro-orbital headache, progressive blurriness of vision, and photophobia associated with redness, excessive watering and pain in his left eye. Two weeks before presentation, he developed left knee swelling and pain accompanied by a non-specific skin rash, which resolved spontaneously within two to three days. His past medical history was remarkable for chronic kidney disease stage II and HIV-1 infection with a latest CD4 count of 1022 cells/mm3. The patient was allergic to sulfa drugs. He was compliant with his antiretroviral therapy, which included dolutegravir, darunavir, tenofovir, emtricitabine, and ritonavir with no renal dose adjustments required as creatinine clearance (CrCl) was > 60 mL/min. The patient had unprotected anal intercourse with a new partner four months as well as one month prior to this admission.
On physical examination, the patient was in non-acute distress, alert, and fully oriented; other vitals signs were as follows: afebrile, heart rate of 91 bpm, blood pressure 126/80 mmHg, respiratory rate 18 rpm, and oxygen saturation 100% at room air. An ophthalmologic examination revealed bilateral visual acuity of 20/70. The pupils were equally round and reactive to light; there was no relative afferent pupillary defect. A slit-lamp examination revealed in the left eye 2+ injection of the conjunctiva, 3+ cells in the anterior chamber and posterior synechiae at 7 O’ clock position (Figure ). Indirect ophthalmoscopy revealed +1 cells in the left vitreous, blurred posterior margins bilaterally with cup-to-disk ratio of 0.1, consistent with papilledema (Figure ). In short, the patient had left eye uveitis and bilateral papilledema. There were no meningeal signs or neurological signs of focalization. There were no additional physical exam findings.
Initial workup revealed creatinine at baseline level, normal platelet, red and white blood cell counts. Initial imaging studies, computed tomography (CT) and magnetic resonance imaging (MRI) of the brain showed no abnormalities. A lumbar puncture was performed and the patient was started on empiric treatment for meningitis with ceftriaxone 2 grams every 12 hours, vancomycin 1000 mg every 12 hours, ampicillin 2 grams every four hours, acyclovir 800 mg every eight hours, and dexamethasone in order to cover for the most typical pathogens such as Streptococcus pneumoniae, Neisseria meningitidis, Listeria monocytogenes, aerobic Gram negative bacilli and Herpes simplex virus. Doxycycline 100 mg every 12 hours was added to the regimen as well due to suspicion of Reiter’s syndrome given the uveitis and joint involvement. His outpatient anti-retroviral therapy was continued. Cerebrospinal fluid (CSF) analysis revealed 45 cells/μL white blood cells, 2% being neutrophils, 94% lymphocytes, 4% monocytes; RBC of 80 cells/mm3; glucose of 49 mg/dL; and protein of 126 mg/dL suggestive of lymphocytic meningitis. Further, the results revealed that his CSF was negative for Herpes simplex virus 1 and 2, Cryptococcus, Epstein-Barr Virus (EBV); the Venereal Disease Research Laboratory (VDRL) test was non-reactive and no organisms were detected on initial Gram stain. A blood workup revealed an absolute CD4 count of 1022 cells/mm3, a rapid plasma reagin (RPR) test reactive with a titer of 1:128, and a positive microhemagglutination test for antibodies to Treponema pallidum (MHA-TP). The RPR test had been negative five months prior to this admission during a routine outpatient clinic visit. Gonococcal and chlamydial cultures on nasal, throat, and anal swabs were negative.
Treatment was initiated with intravenous penicillin G, 24 million units/day given as 4 million units every four hours for 14 days and cyclopentolate, prednisone drops for the affected eye. During the hospital course, the patient’s ocular symptoms including blurriness of vision and redness improved within two to three days and he was discharged home. On follow-up at the Infectious Disease Clinic, the patient was asymptomatic with constantly decreasing RPR titers from 1:128 at the time of admission to 1:16 at three months and 1:8 at six months from admission. Follow-up at the ophthalmology clinic showed no evidence of uveitis or papilledema. The patient refused a repeat lumbar puncture on his visit after six months. |
pmc-6456357-1 | A 64-year-old male presented to the emergency department with acute onset epigastric pain. Work-up with admission ultrasound revealed a common bile duct of 7.7 mm, pericholecystic fluid, positive sonographic Murphy’s sign, and cholelithiasis. The patient had a past medical history of hepatitis C secondary to intravenous drug abuse, alcohol abuse, major depressive disorder, and insomnia. He was afebrile throughout his hospital course without tachycardia or hypotension. He did not have any abnormal elevations in his total bilirubin or liver function tests at admission. At admission, his white blood cell (WBC) count was 12.07 x 103 /uL with 87% neutrophils. He was taken to surgery on the day of admission for laparoscopic cholecystectomy with IOC.
Upon gross inspection of the intraperitoneal cavity, pus surrounded aspects of the end of the liver capsule and the gallbladder appeared gangrenous. A large stone was palpable with laparoscopic instruments within the infundibulum of the gallbladder. After dissecting the cystic duct free of surrounding inflamed tissue, a partial transection of the cystic duct was made so that a cholangiogram catheter could be threaded into the cystic duct and clipped to secure the catheter. A cholangiogram was subsequently performed with a taut radiopaque introducer (Teleflex Medical, Wayne, Pennsylvania, US) and needle (3.0mm x 2.4mm x 8.9 cm) and a 4.5 Fr (1.5mm) x 45.7 cm taut operative cholangiogram catheter (Teleflex Medical). The initial image of the cholangiogram is shown in Figure , demonstrating a lack of contrast in the common bile duct near the ampulla of Vater, suggestive of choledocholithiasis.
Opacification within the area of the ampulla as well as the reflux of contrast medium into the pancreatic duct suggested the presence of a stone in the proximal common bile duct. One gram of glucagon was administered to the patient and two minutes later, an IOC was repeated. This second cholangiogram showed persistent opacification at the ampulla and limited contrast medium reaching the duodenum, with reflux into the pancreatic duct (Figure ). Rather than performing a conventional choledochotomy on the common bile duct and exploring the common bile duct with a choledochoscope, an endovascular equipment set was employed to provide further intervention within the proximal common bile duct, as follows: the taut cholangiogram catheter was removed and a laparoscopic Maryland grasper (Medline Industries, Northfield, Illinois, US) was used to thread a 0.89 mm diameter, 180 cm length, flexible tip length 3 cm angled Radifocus® Glidewire® (Terumo, Tokyo, Japan, US) into the cystic duct. Under continuous fluoroscopic visualization, the glidewire was threaded to the ampulla of Vater. Upon meeting resistance at the ampulla, a 7Fr x 45cm x 0.97 mm Flexor® Check-Flo® Introducer Set (Cook Medical, Bloomington, Illinois, US) was threaded over the glidewire. The catheter was then removed and a sturdier TEMPO AQUA® catheter (Cordis, Milpitas, California, US) was threaded into the sheath over the glidewire and passed through the ampulla of Vater. This enabled the glidewire to pass through the TEMPO AQUA® catheter (Figure ). A cholangiogram was then repeated and demonstrated persistent constriction of the ampulla of Vater and reflux of contrast into the pancreatic duct.
At this time, the imaging indicated the possibility of persistent occlusion with stones or a possible stricture of the ampulla. The TEMPO AQUA® catheter was then retrieved and a 4 mm x 20 mm x 75 cm Mustang Percutaneous Transluminal Angioplasty (PTA) Balloon Dilation Catheter (Boston Scientific, Marlborough, Massachusetts, US) was passed over the glidewire and through the ampulla. This balloon was then inflated and the cholangiogram was repeated, demonstrating a modest improvement in the flow of contrast through the duodenum (Figure ). Due to the persistence of the opacification of the ampulla, the dilation was repeated with a 7 mm x 20 mm x 75 cm Mustang PTA Balloon Dilation Catheter (Boston Scientific).
This second dilation opened up the ampulla and repeat IOC demonstrated free-flowing contrast medium into the duodenum from the cystic duct (Figure ). The glidewire and balloon catheter and sheath were removed under direct laparoscopic guidance. The cystic duct was then transected with laparoscopic scissors and the proximal stump of the cystic duct was tied off with two PDS II ENDOLOOP® violet monofilament sutures (Ethicon, Somerville, New Jersey, US). The gallbladder was dissected off the fossa and a drain was left within the subhepatic space. The following day, the patient demonstrated a slight increase in his total bilirubin to 1.8 mg/dl but remained asymptomatic and had an unremarkable recovery following surgery. The following day, his total bilirubin decreased to 0.8 mg/dl and the patient was discharged tolerating a full diet, with minimal pain and no complications. He was discharged post-op day 1 and his subsequent follow-up appointments were uneventful for six months post-admission. |
pmc-6456359-1 | A 71-year-old man with a history of multiple non-melanoma skin cancers and an ascending aortic aneurysm completed chest computed tomography (CT) for cardiopulmonary surveillance on 5/27/2015 and was found to have multiple bilateral lung masses. Subsequent CT abdomen and pelvis demonstrated a 4 cm omental mass concerning for malignancy. Systemic staging with fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT demonstrated intense FDG-avidity of the lung masses, omental mass, and bilateral hilar nodes (Figures , ). A CT-guided biopsy of a left lung lower mass demonstrated poorly differentiated metastatic malignant melanoma. Immunohistochemical staining (IHC) was positive for: S100, and Melan A, and negative for: TTF1, P63, and CK7/20. Given severe claustrophobia, a CT head was performed and demonstrated no brain metastases.
Taken together, based on AJCC8, the patient had cTxNxM1c (stage IV) melanoma []. Genetic testing revealed no mutations in the BRAF gene. Intravenous (IV) systemic therapy was initiated with dual checkpoint blockade using ipilimumab (3 mg/kg) and nivolumab (3 mg/kg) given every three weeks.
After one cycle of dual checkpoint blockade, the patient had multiple grade 1-3 side effects which were felt to be ipilimumab-related, and ipilimumab was subsequently discontinued. After three cycles of nivolumab, four months from diagnosis, surveillance CT imaging showed interval partial response (PR) of the pulmonary parenchymal metastases and hilar lymph nodes. After nine additional cycles of nivolumab, seven months from diagnosis, surveillance CT imaging demonstrated continued PR of the right lung and omental metastases. Additional sites of metastatic disease were stable (SD) and no new metastases were identified.
After 20 cycles of nivolumab, 10 months from diagnosis, surveillance CT imaging demonstrated interval enlargement of two left lower lobe pulmonary nodules, and several left hilar lymph nodes (Figure ). The omental metastasis had radiographic near complete response (CR) and no new sites of metastatic disease were identified.
After 35 cycles of nivolumab, 12 months from diagnosis, the patient completed stereotactic body radiation therapy (SBRT) to the two enlarging left lower pulmonary nodules to 50 Gray (Gy) in five fractions. One month later, 13 months from diagnosis, surveillance CT imaging demonstrated interval improvement of metastatic disease in the chest (PR) and no evidence of metastatic disease in the abdomen/pelvis (Figure ).
After 43 cycles of nivolumab, 21 months from diagnosis, surveillance CT imaging demonstrated PR in the chest, and no evidence of disease in the abdomen/pelvis. After 60 cycles of nivolumab, 30 months from diagnosis, surveillance PET/CT imaging showed systemic metabolic CR with continued stable PR in the chest and no evidence of metastatic disease in the abdomen/pelvis (Figures , ). Plans for additional systemic therapy with nivolumab were discontinued.
PET/CT imaging completed five months later after discontinuation of nivolumab, 35 months from diagnosis, demonstrated interval increase in left hilar FDG activity; however, repeat PET/CT imaging three months later demonstrated decreased FDG activity in this area and no new metastases. The patient is now 41 months from diagnosis and recent surveillance imaging is stable, without evidence of FDG-avid disease or new metastases (Figures , ). |
pmc-6456360-1 | A 34-year-old Indian male patient presented with two days history of fever, myalgia, and arthralgia mainly in the shoulders, hips, and hands. The patient had travelled from India to Qatar six days prior to presenting to the hospital. He also complained of diarrhea (five watery stools per day) and dark colored urine. He had no history of sick contacts but reported mosquito exposure in India.
Review of systems revealed productive cough and eye redness, otherwise, it was unremarkable.
On initial examination, his oral temperature was 39.4°C, and blood pressure was 90/40 mmHg. There was conjunctival injection, and muscle tenderness in the shoulders and thigh muscles, but otherwise, the examination was normal.
The patient was resuscitated with five liters of normal saline and then blood pressure normalized.
Investigations
Laboratory investigations showed the following (Table ):
He had 3+ blood in the urine dipstick but urine microscopic analysis revealed no red blood cells.
Differential diagnosis
The initial differential diagnosis included viral infection (e.g., including influenza, Dengue fever, Chikungunya virus infection) and parasitic infection (e.g., malaria). Bacterial infection was thought to be less likely because of lack of a focus of infection and symptoms (e.g., myalgias, arthralgias) pointing more towards a viral etiology.
Serologies for hepatitis C virus, hepatitis B virus, human immunodeficiency virus, and parvovirus; and serum polymerase chain reaction (PCR) for Epstein-Barr virus, cytomegalovirus, and adenovirus were negative. Blood smears for malarial parasites were negative. Dengue virus IgG was positive. However, IgM antibody was negative, suggesting past infection. Additionally, nasal swabs were sent for influenza, parainfluenza, corona viruses PCR and were all negative. Two sets of blood cultures came negative.
Blood for Chikungunya IgM antibody test came positive, while IgG antibody test was negative suggesting acute infection (Figure ) [].
Treatment
The patient was given supportive treatment, and over his hospital stay, his symptoms improved and the laboratory abnormalities started to improve. He was discharged to home after six days. |
pmc-6456820-1 | The first patient is a 63-year-old male patient with a known coronary 3-vessel disease. Twelve years ago, he underwent a coronary-aortic bypass surgery procedure with 3 grafts in the setting of an acute elevation of the ST-segment of the electrocardiogram, indicating a total occlusion of a coronary artery (ie, ST-elevation myocardial infarction) 1 month earlier. The left ventricular systolic function appeared normal. The last coronary angiography was 3 years ago where 2 drug-eluting stents were deployed. His cardiovascular risk profile comprises arterial hypertension, previous smoking (30 pack-years), hypercholesterinemia, diabetes, and positive family history for a premature cardiovascular disease. The arterial hypertension is currently well treated with combination therapy of perindopril, indapamide, and amlodipine. Diabetes did not necessitate treatment. Hemoglobin A1c, a long-term indicator of the quality of glucose control in diabetes, is currently 7.1%. Furthermore, he is on aspirin, bisoprolol, and rosuvastatin. Currently, he is free of symptoms; however, in light of secondary preventive aims, cardiovascular rehabilitation is warranted. |
pmc-6456820-2 | The second patient is a 62-year-old male who experienced an ST-elevation myocardial infarction 2 years earlier. The left anterior descending artery was successfully treated with 3 drug-eluting stents. On echocardiography, a normal left ventricular ejection fraction was noted; however, with regional wall motion abnormalities corresponding to the territory of the infarction (anterior and anteroseptal). His cardiovascular risk profile comprises a history of smoking (20 pack-years), treated hypercholesteremia, obesity (body mass index, 30 kg/m2), and psychosocial stress. His present medication comprises aspirin, bisoprolol, lisinopril, and rosuvastatin.
Both patients are currently free of symptoms; however, in light of secondary preventive aims, cardiovascular rehabilitation in both patients is warranted. |
pmc-6456914-1 | A 56-year-old never-smoking female patient was diagnosed with stage IV lung adenocarcinoma (cT3N3M1b, according to American Joint Committee on Cancer, 8th edition) in September, 2017. Her tumour involved left lower lung (LLL), right supraclavicular, left infraclavicular to bilateral mediastinal lymph nodes, brain, T9 and L12 spine, and bilateral adrenal glands. A compound mutation in EGFR exon 20 (H773L/V774M complex) was found by Sanger sequencing of the tumour DNA extracted from paraffin-embedded bronchoscopic biopsy specimens (Fig. A). The patient received first-line afatinib (40 mg daily) but experienced rapid disease progression with enlargement of brain and lung tumours in first follow-up three months later, indicating a pattern of primary resistance. Anti-cancer therapy was shifted to pemetrexed (500 mg/m2 every three weeks, 15 cycles) and cisplatin (70 mg/m2 every three weeks, six cycles), with bevacizumab (7.5 mg/m2 every three weeks, 14 cycles) being added since the second cycle of chemotherapy. Sequential radiotherapies to whole brain (3300 cGy/10 fractions) and LLL tumour (3500 cGy/10 fractions) were also administrated. These managements led to a partial response until 10 months later while increased bilateral lung metastases developed. The therapies were then switched to a combination of ramucirumab (8 mg/kg every three weeks) and docetaxel (60 mg/m2 every three weeks), which resulted in a partial response after two cycles of treatments in the last follow-up (November 2018). The treatment course was summarized in Figure B. |
pmc-6457045-1 | A 46-year-old male who had presented with a one-month history of hoarseness and chest pain was referred to our hospital. He had no relevant medical history. On physical examination, chest auscultation revealed slightly diffuse expiratory rhonchi, but no other significant abnormality. His peripheral arterial blood oxygen saturation was 96% on room air. He was a current smoker. Chest computed tomography (CT) revealed a superior mediastinal mass (Fig. A) and pulmonary emphysema (Fig. B). Fluorodeoxyglucose-positron emission CT revealed a hypermetabolic lesion at the site of the superior mediastinal mass (Fig. C); thus, we suspected a malignancy. The mass lacked the CT bronchus sign but lay adjacent to the trachea and the oesophagus.
We initially chose EBUS-TBNA for diagnosis. A total of 5 mL 2% (w/v) lidocaine was sprayed into the pharynx, and another 5 mL of 2% (w/v) lidocaine was administered via the channel during the procedure. A conventional flexible bronchoscope (BF-F260; Olympus) was employed; intubation was achieved using a siliconized, uncuffed tracheal tube of internal diameter 7.5 mm (Portex; Smiths Medical, St. Paul, Minnesota, USA). A bronchoscope (BF-UC290F; Olympus) was orally inserted during fentanyl- and midazolam-induced conscious sedation. The patient received fentanyl (100 μg) and midazolam (8 mg) during the procedure. However, the EBUS-TBNA approach failed because of his severe cough and an inadequate EBUS view (Fig. D). During the same endoscopic session, we performed EUS-B-FNA using a transoesophageal approach. The BF-UC290F enabled smooth access through the oesophagus and a clear EBUS view of the mass, attributable (respectively) to the compact distal tip and the powerful angulation. He rarely coughed during the procedure. We identified a clear ultrasound image and punctured the mass with a 22-G ViziShot 2 needle (Olympus) (Fig. E). Rapid on-site cytology revealed that an adequate specimen had been obtained, and we terminated the procedure immediately. Histologically, the mass was a squamous cell carcinoma (Fig A, B). The stage was thus T4N0M0, and chemoradiotherapy was prescribed as first-line treatment. |
pmc-6457284-1 | A 54-year-old Japanese female visited an oral surgery clinic with a complaint of swelling in the inner region of the left cheek for the past one month. On clinical examination, a mobile swelling (size, 1 cm x 0.75 cm) with a clear boundary was observed on the left buccal mucosa. No associated pain was reported and the overlying mucosa was normal in appearance. The swelling was clinically diagnosed as benign buccal mucosa tumor. The tumor was excised under local anesthesia and was diagnosed as acinic cell carcinoma (AcCC) after histopathological examination. The margins were still positive for the tumor and further resection was advised. The patient reported to the Health Sciences University of Hokkaido Hospital for resection of the residual tumor two months after the initial surgery. Clinically, the patient was asymptomatic. The level 1B lymph nodes on both sides were palpable, bean sized, mobile, elastic, and soft. Intraorally, a surgical scar of about 7 mm was present on left buccal mucosa. There was no pain on pressure in the region of the scar (). The patient had a history of noninvasive ductal carcinoma (ductal carcinoma in situ [DCIS]; Tis N0M0) in the right breast, which was treated by excision and 57 Gy of radiotherapy five months ago. On investigation for oral lesion, no obvious abnormalities were detected on the computed tomography- (CT-) scan, contrast MRI, and ultrasonogram. Positron emission tomography- (PET-) CT did not suggest transition to and from any of the distant organs. The margin was resected under general anesthesia and sent for histopathological examination (). No relation to the parotid gland was found at the time of surgery.
Histopathologically, the excised margin appeared as a fragmented tissue with no encapsulation. The tumor tissue was composed of cells with dominant microcystic structure with eosinophilic cytoplasm and eosinophilic secretory material. Papillary and tubular pattern of cell arrangement were also found but were limited to small area. A few vacuolar cells and some areas with hemosiderin deposition were observed. Furthermore, normal muscle tissue and atrophied salivary gland tissues were also seen ().
The secretory material was positive for diastase digested Periodic acid-Schiff (d-PAS), Mucicarmine, and Alcian Blue staining. No zymogen granules were found in the tumor cells. Immunohistochemistry (IHC) revealed strong positive reactions to vimentin, cytokeratin-19, and S100 protein. Mammaglobin was strongly positive, whereas discovered on gastrointestinal stromal tumors 1 (DOG-1) showed a negative reaction (). The histological sections of breast carcinoma were examined in suspicion of metastases; however, features of ductal carcinoma in situ that appeared completely different from those of buccal mucosa tumor were noted. Based on these histomorphologic and IHC profiles, the case was diagnosed as SC of the minor salivary gland in the buccal mucosa. |
pmc-6457288-1 | An 85-year-old male with a medical history pertinent for HHT and congestive heart failure presented with epistaxis, altered mental status, and melena. Physical exam revealed a lethargic male with generalized abdominal tenderness, asterixis, and telangiectasias on the lower lip and dorsal aspect of the hands. Ammonia level was 68 umol/L (ref: 11-35 umol/L), alkaline phosphatase 244 U/L (ref: 0-153 U/L), AST 25 and ALT 29 U/L (ref: 10-50 U/L), bilirubin 0.7mg/dL (ref: 0-1.2 mg/dL), INR 4.11 (ref: 0.87-1.17), and hemoglobin 6.8 g/dL (ref: 13.7-16.5 g/dL). Doppler abdominal ultrasound showed coarse liver parenchyma with multiple scattered hyperechoic lesions suggestive of hepatic AVMs (). Computed tomography (CT) confirmed the presence of hepatic venous enhancements, consistent with AVMs (). The patient was diagnosed with HHT-induced portosystemic encephalopathy secondary to AVMs and recurrent epistaxis. He was started on lactulose with complete resolution of his symptoms. Epistaxis was managed with nasal packing. He remained stable and was discharged. |
pmc-6457295-1 | The patient is a 58-year-old man that presented with an insidious history of intermittent left shoulder pain, which worsens after vigorous physical training. He denied any suspicious traumatic injury over the left shoulder girdle. Clinical examination revealed no gross deformities or muscle atrophy on the left shoulder comparative to the contralateral upper limb. The range of motion of the left shoulder was well preserved with functional shoulder symmetry (). Palpation revealed a very specific site of pain over the left acromioclavicular (AC) joint, aggravated by overhead and across-body movements. Relevant tests for degenerative rotator cuff disease and shoulder instability were negative.
Radiographs were obtained, demonstrating abnormal morphology of the left humerus head, characterized as an anteromedial exostosis arising from the epiphysis of this bone. There was no cortical discontinuity, periosteal reaction, calcified matrix, or articular incongruity. In addition, there were no signs of AC disease, although there was slight proximal migration of the distal part of the left clavicle, compatible with a Rockwood and Neer grade 2 AC dislocation ().
The initial diagnosis was DEH and he was asked multislice computed tomography (CT) and magnetic resonance (MR) images. CT showed an ossified mass in the inferomedial aspect of the proximal humerus epiphysis with no articular or soft tissue involvement ().
T1-weighted, T2-weighted, and STIR MR images of the left shoulder revealed the inferomedial mass projecting toward the inferior capsular recess, with sharpening of the articular cartilage of the humeral head, but no signs of osteoarthritis (OA). No foci of calcification or soft tissue involvement was noted ().
The lesion was judged to be localized to the lesser tuberosity and therefore was classified as extra-articular type B1 (affecting the upper limb, localized to a single bone), according to Clarke's classification system []. Curiously, although MSCT and MR images were very characteristic of DEH, all radiologists' descriptions did not mention this developmental condition. Actually, according to the medical reports, the diagnosis was OA of the left shoulder.
Biopsy was not performed as imaging exams were very conclusive, showing typical alterations of DEH in the growth of the proximal humerus epiphysis. Patient was managed with analgesics and physical therapy protocol, including extracorporeal shock wave therapy (ESWT), and oriented to temporarily reduce overhead and across-body exercises. AC joint symptoms completely disappeared and he fully returned to his previous level of physical activity. Patient is periodically followed up without any recurrence of the symptoms on this shoulder. |
pmc-6457300-1 | A 29-year-old female presented with a mass on the anterior abdominal wall present for 1 year. The mass had gradually increased in size over this period of time. She complained of continuous discomfort and pain which became worse during menses. The mass increased in size during menses as per the patient's description. She had undergone a caesarean section six years back. There was no other significant history. Her menses were regular with no bladder or bowel disturbances.
Physical examination of the abdomen revealed a circumscribed mass measuring approximately 6 cms in diameter in the infraumbilical region to the left of the midline ().
The mobility of the swelling became restricted on contracting the underlying muscles suggestive of infiltration of the underlying musculoaponeurotic structures.
Laboratory investigations were within normal limits. A contrast enhanced CT scan was done during her menses. The CT scan revealed a contrast enhancing lesion in the subcutaneous tissues infiltrating the underlying musculoaponeurotic structures highly suggestive of an abdominal wall endometrioma ().
She underwent surgical resection. The endometrioma was resected along with the portion of the underlying aponeurosis and rectus abdominis muscle (Figures and ).
A polypropylene mesh was placed over the defect created by the resection and fixed all around the defect to the anterior rectus sheath with nonabsorbable sutures (). The postoperative course of the patient was uneventful. The histopathological evaluation of the resected specimen revealed endometrial glands and stroma with clear resection margins ().
Following the procedure there was no seroma at the operative site and the patient noted complete relief of symptoms. |
pmc-6457313-1 | The patient is a 56-year-old married, retired Caucasian male with a 6-year history of PD. He previously worked in a scientific lab at a large academic institution and seven years prior to presentation, he began to experience a right-handed tremor that increased in severity over several months. He was evaluated by neurology after which he was diagnosed with PD. He was treated with various dopaminergic medications, including carbidopa-levodopa, with poor symptom relief. Due to poor response to medications, the patient decided to elect for DBS treatment. He received DBS in the left VIM 3 years prior to presentation and experienced significant relief of his right-handed tremor. Over time, the patient began to develop a worsening left-handed tremor and bradykinesia that required restarting treatment with carbidopa-levodopa. The dosage of carbidopa-levodopa was increased over the years for continued control of his Parkinsonian symptoms. The settings of DBS were also increased for continued control of the right-handed tremor. During this time, the patient began to experience changes in behavior including apathy and poor communication skills in social settings. One month prior to presentation, family members noted that the patient began to display uncharacteristic hypersexuality, anger, and impulsivity. The patient was subsequently found by family members in the car garage after what appeared to have been a suicide attempt by carbon monoxide poisoning. According to the patient and family members, this was the patient's first suicide attempt. Emergency services were able to arrive on time to transport the patient to the ED where he was found to have oxygen saturation of 94%, and an arterial blood gas study found a carboxyhemoglobin level of 49.3 (normal <2). Neurology and psychiatry were consulted to evaluate the patient's neurological and mental status. Neurology (internal medicine) found that the patient was taking 3 tablets of carbidopa-levodopa 25-100 (25 mg of carbidopa and 100 mg of levodopa) 5 times a day and was concerned that the behavioral changes may be due to the high dose of carbidopa-levodopa and attempted to reduce the dose to 1 tablet 3 times a day. However, the patient developed acute neck dystonia and the carbidopa-levodopa dose was increased up to 1 tablet 5 times a day. The patient was subsequently admitted to inpatient psychiatry.
Upon intake to inpatient psychiatry, on the lowered dose of carbidopa-levodopa, the patient was noted to display low levels of positive affectivity and was chronically nihilistic. Additionally, he displayed a lack of affect, the lack of appreciation or interest in the nearly completed suicide attempt, and a lack of appreciation of how out of character this was for him. He appeared to have poor insight into his current situation and was often superficial and vague with his responses. No significant behavioral issues were noted.
Investigation into the cause of the patient's behavioral decline and eventual suicide attempt subsequently began with a systematic approach to all the possible offending agents. Both DBS and carbidopa-levodopa are well documented independent causes of behavioral changes. The pathology of PD itself is also associated with behavioral changes such as hypersexuality, pathological gambling, and mood swings. Hypersexuality in particular appears to be a common variable associated with all three factors. We therefore approached each risk factor independently.
It is known that a dose dependent association exists between carbidopa-levodopa and altered behavior including hallucinations and/or hypersexuality [, ], quite a contrast to the baseline depression and psychological akinesia seen in PD. Increased dopaminergic activity is indeed the hallmark of illicit substances such as cocaine and amphetamines so the development of behavioral changes is therefore not surprising with high doses of carbidopa-levodopa. In this case, Mr. C clearly demonstrated these behavioral changes as evidenced by his increasingly bizarre sexual advances on his wife which were occurring with higher frequency as his dose of medication increased. shows the gradual increase in carbidopa-levodopa dosing over the years for the patient.
clearly shows the increase in carbidopa-levodopa required to treat the patient's Parkinsonian symptoms and the high daily dose the patient was taking when he made his suicide attempt. The decision was therefore made to decrease his carbidopa-levodopa dosage from 1 tablet 5 times a day to 1 tablet 4 times a day and to continue to monitor his behavior on the inpatient unit. The patient was also started on fluoxetine as depression and apathy are known to occur when the dosage of carbidopa-levodopa is changed or stopped. It was believed that the patient's PD would also benefit from the mild dopaminergic nature of fluoxetine.
The patient did well on the reduced dose of carbidopa-levodopa and did not exhibit any strange behavior, hypersexuality, or impulsivity while on the unit. He also did not endorse any suicidal ideation, thoughts, or plans and appeared to tolerate fluoxetine well.
Subthalamic nucleus (STN) stimulation is considered an established surgical treatment for Parkinson's disease (PD). Episodes of depression and suicide have however been reported in patients treated with DBS of STN, notably when stimulation targets the inferior part of the STN which can induce acute depression symptoms []. As the stimulation improves motor symptoms, patients are more able to attempt suicide. Limited data however exists regarding VIM stimulation especially with regard to its cognitive and behavioral effects. Very little data also exists on the impact of positional changes of the device itself over the years. This was especially relevant for the patient after family members reported a mild concussion he had suffered a few months prior.
The decision to obtain a CT scan was therefore justified with the aim of ruling out potential positional changes within the past 3 years. shows CT scans from 2015 and 2018.
Upon comparison with a CT from the time of DBS placement, radiology determined that there was no significant change in DBS position and it was concluded that a positional change was not the cause of the patient's behavior. It is therefore reasonable to assume that VIM stimulation by itself might have been, despite the high doses of carbidopa-levodopa, the cause of his depression and eventual suicide attempt. This was especially important to note as the patient and his neurologist were beginning to consider DBS of the right VIM to control his worsening left-handed tremor.
The patient's family played a large role in his treatment as they visited daily to speak to both the patient and his care team. They also agreed that the patient's behavior had improved from the time of presentation. It was decided, after 8 days of inpatient stay, that he was ready for discharge with close follow-up with both neurology and psychiatry. |
pmc-6457320-1 | A 12-year-old boy, with body weight 39 kg, presented with eye deviation, trismus, and hypertonia of the limb, and was admitted to the pediatric emergency department. He had undergone upper gastrointestinal endoscopy 1 day prior as a regular examination for duodenal ulcer. He had been administered haloperidol (total 4.5 mg) intravenously for sedation because he had exhibited a drug rash suspected to be a reaction to previously administered midazolam. His symptom developed during exercise about 24 hours after the administration of haloperidol. He had not been administered antipsychotics, including haloperidol and antiemetics, previously. He had no history of drug abuse or alcohol intake. He had no known allergy.
In the pediatric emergency department, his vital signs were within the normal ranges for his age. Eye position repeatedly showed bilateral left or upward deviation. He exhibited trismus, left deviation of the lip, lip smacking, puckering and pursing, jaw swinging and chewing, torticollis, opisthotonus, hypertonia of the limb, and action tremor. The symptoms were observed while he was awake but disappeared when he was asleep.
The results of the laboratory tests were normal, including white blood cell count (4200/μL; 4000-10700/μL), hemoglobin (13 g/dL; 12.2-15.7 g/dL), C-reactive protein (0.06 mg/dL; <0.15 mg/dL), aspartate aminotransferase (26 IU/L; 15-31 IU/L), alanine aminotransferase (14 IU/L; 9-32 IU/L), blood urea nitrogen (11 mg/dL; 6.8-19.2 mg/dL), creatinine (0.51 mg/dL; 0.39-0.62 mg/dL), serum sodium (141 mEq/L; 138-144 mEq/L), serum potassium (4.6 mEq/L; 3.6-4.7 mEq/L), serum calcium (9.8 mg/dL; 8.7-10.1 mg/dL), and creatine kinase (170 IU/L; 62-282 IU/L). Analysis of cerebrospinal fluid revealed it to be clear in appearance, with a cell count of 1/μL, and normal glucose and protein levels (68 and 24 mg/dL, respectively). Cranial computed tomography, and magnetic resonance imaging showed no abnormalities. Blood concentration of haloperidol, while at the emergency department (about 24 hours after administration), measured using enzyme immunoassay method was 2.8 ng/mL (therapeutically effective concentration range is 3.0-17.0 ng/mL).
Although the diagnosis was not clear at that time, we transferred the patient to a tertiary care institution, as he additionally showed stridor and desaturation. The pediatric neurologists performed examinations, including electroencephalogram analysis. Paroxysm was not indicated and neither was epilepsy. Because the symptoms had appeared only when the patient was awake, he was diagnosed with acute dystonia due to haloperidol. Within a few minutes of administration of hydroxyzine, he fell asleep and was symptom-free, and he did not require intubation. Subsequently, he was symptom-free and was discharged after 3 days. One week later, he attended a follow-up visit and showed no symptom recurrence. |
pmc-6458213-1 | A 76-year-old man was referred to our hospital with IHCC. Five years earlier, he had been treated for ITP at another hospital, and had been administered prednisolone 5 mg/day. At the time of ITP diagnosis in the prior hospital, the value of the platelet-associated IgG (PAIgG) was elevated and all of the anti-phospholipid antibody syndrome (APS)-related antibodies were negative. In addition, only the number of the megakaryocytes increased in bone marrow aspiration, which suggests the hematopoietic efficacy in bone marrow was maintained. Helicobacter pylori was previously eradicated in the other hospital.
His past medical history was otherwise unremarkable. More recently, his sister had been diagnosed with pancreatic cancer, and he therefore requested a tumor marker evaluation. The evaluation revealed elevated carbohydrate antigen 19-9 (CA19-9), and a liver tumor was detected by computed tomography (CT). Thus, he was referred to our hospital for detailed examination and treatment for the liver tumor.
Multidetector row CT revealed a liver tumor that was 25 mm in diameter and had a low density with poor enhancement. The peripheral bile duct branch of segment 3 was dilated; therefore, we suspected the periductal infiltrating type of IHCC (Fig. ), which prompted us to perform left hemi-hepatectomy.
His laboratory findings on admission are summarized in Table and included a platelet count of 3.5 × 104/μL. To increase platelet levels prior to surgery, romiplostim was administered subcutaneously (70 μg per week for 3 weeks) and eltrombopag was administer orally (25 mg per day for 23 days), as TPO-RA. Because oral medications we are more convenient, romiplostim was switched to eltrombopag. His platelet count increased to 14.1 × 104/μL (Fig. ). After starting eltrombopag, fibrin degradation product (FDP) and d-dimer levels were slightly elevated. Therefore, we performed lower limb venous ultrasonography, which revealed venous thrombosis in the veins of both soleus muscles. We consulted with cardiovascular medicine and hematology teams about our patient’s case. Because his thrombosis was venous rather than arterial, it was thought to be largely unrelated to the platelet increase. Accordingly, the patient’s cancer was suspected to have caused his thrombosis, via increased activation of clotting factors. In addition, we speculated that the risk of the thrombus in the soleus muscles causing pulmonary thromboembolism was extremely low. Thus, eltrombopag was discontinued. After eltrombopag cessation, the platelet count slightly decreased to 10.0 × 104/μL, but remained at a sufficient level; subsequently, the operation was performed on schedule (Fig. ).
We performed left hemi-hepatectomy combined with left-sided caudate lobectomy and regional lymphadenectomy, because the lymph nodes around the common hepatic artery were larger than the preoperatively measured size. The operative time was 412 min, and blood loss was 395 mL, requiring no transfusion. Pathological findings were cholangiocellular carcinoma of the mass forming type, and bile duct infiltration type.
Although his platelet count decreased to 4.3 × 104/μL just after the operation, eltrombopag and prednisolone were administered on the following day. His platelet count decreased to 2.4 × 104/μL at postoperative day (POD) 3, but increased gradually thereafter, which made blood transfusion unnecessary. Drainage characteristics were not remarkable and bleeding and bile leakage was not observed after the operation. Although chylorrhea developed on POD 8, it was improved through the use of a fat-restricted diet. The drain was removed on POD 14, and he was discharged from the hospital on POD 18 without complications (such as bleeding, sepsis, pneumonia, or portal vein thrombosis). Eltrombopag was continued until POD 37. The platelet count decreased to 3–4 × 104/μL again, which was the same as the preoperative level. However, rebound phenomenon requiring platelet transfusion was not observed. |
pmc-6458225-1 | The patient was a 75-year-old woman who presented with severe bilateral leg edema and epigastralgia. There was no past history of chronic hepatitis or blood transfusion. Admission laboratory tests revealed an elevated serum aspartate aminotransferase level (48 U/L), mildly decreased serum albumin (3.6 g/dL), mildly decreased serum (74%), mildly elevated D-dimer (12.9 μg/mL), and mildly elevated fibrin degradation product (12.6 μg/mL). The tumor marker AFP was slightly elevated to 585 ng/mL. The Child-Pugh classification was A, and the indocyanine green retention rate at 15 min was 12%. CT scans showed a huge tumor, 21 cm in diameter, in the right liver and TT, 37 mm in diameter, in the right atrium (Figs. and a). The TT was pedunculated, swung like a pendulum with the heartbeat, and reached the tricuspid valve on a transesophageal ultrasound video (Fig. b). The symptomatic patient had a risk of sudden death due to TT in the right atrium. Therefore, we explained the high surgical mortality rate to the patient and her family, and they decided to proceed with surgery.
At first, a partial sternotomy was made, and cardiopulmonary bypass (CPB) via the aorta, superior vena cava, and right femoral vein and electrical ventricular fibrillation were performed after intravenous injection of 25,000 units of heparin sodium. Although the TT in the right atrium was very soft and fragile, thrombectomy from the right atrium and inferior vena cava (IVC) was successful (Fig. a–d). After the removal of TT, the IVC was clamped with a tourniquet above the diaphragm and the right atrium was sutured with 5–0 Prolene. After DC defibrillation was carried out, CPB was stopped, the tourniquet on the IVC was released, and 100 mg protamine was administered for the neutralization of heparin. The open heart surgery time was 23 min, and the CPB time was 42 min. Next, a reverse T incision was made, and right hepatectomy by anterior approach and IVC tumor thrombectomy were performed. After ligation of the anterior and posterior Glissonean pedicles, the liver parenchyma was transected under the Pringle maneuver and IVC clamping below the liver. After the IVC and right hepatic vein were confirmed by anterior approach, the IVC was opened and a residual TT in the IVC was removed under total hepatic vascular exclusion (THVE) (Fig. a–d). Finally, the huge tumor was removed with the diaphragm without pulmonary embolism. The total operation time was 9 h, and the total blood loss was 8200 mL. The tumor was of the massive type macroscopically (Fig. ), and cancer cells showed moderately to poorly differentiated HCC with invasion to the portal vein, hepatic vein, and diaphragm. The patient’s postoperative course was uneventful, and she was discharged 14 days after surgery. She is still alive 14 months after surgery with recurrence in the lung. |
pmc-6458608-1 | In a 55-year-old female patient (Patient 1, see Table ), a sweat test was performed due to recurrent pneumothoraces and bilateral bronchiectasis with mucoid impaction apparent in thoracic computed tomography (CT). Sweat test showed borderline values of sweat chloride two times (52 and 41 mmol/L); therefore, genetic testing for mutations in the CFTR gene was initiated. Indeed, two mutations, F508del and R117H-7T, were detected. Pulmonary function testing revealed a severe obstructive ventilation disorder with a FEV1 (forced expiratory volume in 1 sec) of 27% predicted and respiratory failure with the need for oxygen therapy. Indications of the presence of a liver or pancreas involvement in the context of the underlying disease were not found. The repeatedly determined pancreatic elastase was in normal range (> 200 μg/g). In sputum samples, P. aeruginosa was cultured several times. Serologically, antibodies (Elastase and Exotoxin A) against P. aeruginosa were significantly increased as an indication of chronic respiratory tract infection. The patient reports a long history of disease with shortness of breath, productive cough and recurrent pulmonary infections with frequent antibiotic therapies until the final diagnosis was made. She had used bronchodilators (short- and long-acting beta 2-sympathomimetic agents) and inhaled corticosteroids for many years assuming she suffers from a chronic obstructive pulmonary disease (COPD) although she was non-smoking and was not exposed to occupational hazards. Due to severe obstructive pulmonary disease the patient has been repeatedly treated with systemic corticosteroids in the past.
In addition to pulmonary involvement, the patient also had a long-standing affection of the paranasal sinuses. Due to chronic infection of the respiratory tract with P. aeruginosa, we initiated an inhalative antibiotic therapy with tobramycin 300 mg twice a day and later changed to colistin 2 MIU twice a day because of an increase of bronchial obstruction. Hypertonic saline 3% and Dornase alfa were introduced for improving airway clearance. Furthermore, with help of the respiratory therapists, the patient learned airway clearance techniques (ACT) to perform this independently. At that time, nearly monthly hospitalization was necessary as the clinical condition worsened.
After diagnosis of the index patient, her twin sister (Patient 2, see Table ) was introduced to us for further diagnostics. Here, too, a borderline sweat test was found (sweat chloride 44 mmol/L). A repeated sweat test showed a nearly normal value (33 mmol/L). Subsequent genetic testing also revealed a compound heterozygosity for F508del and R117H-7T.
This patient had markedly less symptoms such as cough and shortness of breath. Also, no recurrent pulmonary infections were reported. However, chronic nasal congestions and recurrent sinusoidal infections were reported. A CT-scan of the upper respiratory tract revealed chronic pansinusitis with accentuation of the maxillary sinus. The patient has undergone sinus surgery multiple times in the past. In addition, the patient reported to suffer from allergic bronchial asthma, especially in spring. In this regard, there exists an on-demand therapy with an inhaled corticosteroid and a long-acting beta-agonist bronchodilator.
Pulmonary function showed only a moderate obstructive ventilation disorder (FEV1 = 74%pred.). A CT-scan of the chest revealed bronchiectasis in both upper lobes and a consolidation in middle lobe. In repeated sputum samples, Staphylococcus aureus was detected. Serologically, the reciprocal antibody-titres against P. aeruginosa were all negative. Notes on exocrine or endocrine pancreatic insufficiency or liver involvement were not documented.
Both patients grew up together under the same conditions and in the same area. Both were non-smokers and were not exposed to any substances harmful to the lungs, privately and professionally. There was no family history. Because of the severity of the lung disease, Patient 1 was unable to do any work and was retired while her twin sister was doing a full-time job.
After diagnosis, both patients were placed on ivacaftor. Initially, the drug was well tolerated by both. Patient 2 showed two to three times elevated liver enzymes (ASAT, ALAT) shortly after administration of ivacaftor 150 mg twice a day, which is why we were forced to reduce the dose of ivacaftor. At half the dose, a normalization of the liver function was recognized. Patient 1 initially complained of a bronchial tightness, which could not be detected any more after approximately 14 days under symptomatic therapy.
After initiation of ivacaftor therapy, the pulmonarily more affected Patient 1 did not show improvement in lung function (see Fig. ). A clinical response of the ivacaftor therapy could be documented by reduction of exacerbation rate, weight gain (+ 6 Kg), improved airway secretion mobilization, a reduction of hospitalization rate and a lower need for antibiotic therapy. Due to the lack of pulmonary response of standard and potentiator-therapy, we decided to list the patient for lung transplantation. Ivacaftor therapy was continued because of its stabilizing effect on the disease state. A sweat test repeated after 6 months now shows a normal value of sweat chloride (20 mmol/L).
The pulmonarily less affected twin sister showed improvement in lung function with an increase in FEV1 and FVC after initiation of therapy (see Fig. ). In the repetition of the sweat test, a normal value could be determined (19 mmol/L), as with the sister, even under half the dose of ivacaftor. The existing chronic paranasal sinus complaints were also declining. In addition, the patient reported an improved daily workload. Inpatient treatment was not necessary before or after treatment initiation with ivacaftor. |
pmc-6458629-1 | A 28-year-old Togolese woman of Ewe ethnic origin working part-time in a prison setting with no previous history of disease was admitted in August 2017 for left hemibody sensory problems with ataxia. These problems were observed while the patient was hospitalized for a few days in the hepato-gastroenterology (HGE) department. She had been referred to the HGE department for vomiting, abdominal pain and persistent hiccups lasting for about a month, which were thought to be due to gastritis with multiple ulcers based on a digestive endoscopy. Prior to admission to the HGE department, she initially received anti-ulcer and antiemetic drugs, but the outcome was marked by persistent vomiting and the appearance of episodes of prolonged loss of consciousness. Before the appearance of digestive problems, the patient presented with headaches and auditory and visual hallucinations due work-related stress. The examination also confirmed the existence of evening fever, weight loss without cough and secondary amenorrhea unrelated to pregnancy.
On day 1 of neurological problems, an examination confirmed the persistence of digestive symptoms, apyrexia, the existence of a headache, left hemibody ataxia with nystagmus when looking to the left, pronounced left osteotendinous reflexes, and left hemibody hypoesthesia up to the base of the neck. Encephalic magnetic resonance imaging (MRI) showed a hypersignal lesion in the bulbar more lateralized on the left in the fluid-attenuated inversion recovery (FLAIR) sequence not enhanced after a gadolinium injection (Fig. ). The infectious assessment showed a normal pulmonary X-ray, a negative human immunodeficiency virus (HIV) serology, negative plasmodium tests but the presence of Mycobacterium tuberculosis deoxyribonucleic acid (DNA) in the cerebrospinal fluid (CSF) (using GeneXpert) with normal cytochemistry and a sedimentation rate (SR) of 120 mm in the 1st hour. The pregnancy test was negative. On day 3 of the neurological problems, digestive problems subsided, and even though the results of the examinations were not yet compiled, the patient presented three episodes of cardiopulmonary arrest with a fever of 39 °C without an infectious contact point. She successfully benefitted from orotracheal intubation with broad-spectrum antibiotic treatment and intravenous corticosteroids in the intensive care unit. In the presence of the digestive symptomatology with a bulbar lesion, we alluded to an APS within the framework of an NMOSD, and a sample for the anti-AQP4 antibody tests was sent to Paris, France. Upon leaving the intensive care unit after 72 h, anti-tuberculosis treatment was established. The outcome was favorable with apyrexia, a modification in the nystagmus and an improvement in the ataxia. Subsequently, the result of the anti-AQP4 antibody test was positive (indirect immunofluorescence on transfected cells, anti-AQP4 Euroimmun reagent, CERBA file No. 17 T0483884 of 01/09/2017). In September, 2 months from the onset of digestive problems, with Lhermitte’s sign and hand and foot contracture access without vesico-sphincter problems were established. At the neurological examination, the osteotendinous reflexes were pronounced. Cervical medullary MRI showed an additional intramedullary hypersignal lesion in the T2 sequence at the C2 level without contrast enhancement after a gadolinium injection (Fig. ). In addition to the symptomatic treatment of spasticity, a second course of intravenous corticosteroids was administered, and anti-tuberculosis treatment was continued. The outcome was favorable, and the patient resumed her usual activities as of February 2018, which was 8 months after the first onset. After 8 months of anti-tuberculosis treatment, the patient was started immunosuppressive therapy (azathioprine 50 mg twice daily) to limit the risk of recurrence of NMOSD. |
pmc-6458660-1 | Our patient is a 61-year-old Caucasian male, with a 10-year history of multiple myeloma (IgM kappa multiple myeloma). He presented to clinic with a sharp pain at left lower one-third tibial region with a small mass (15 × 15 × 10 mm3) palpated, he rated the pain score up to 10/10 based on visual analog scale and described it as sharp shooting pain that radiates to his toes and occasionally goes up to the proximal leg. On physical exam, there is a palpable nodule proximal to the medial malleolus with a positive Tinel’s sign upon percussion of the tibial nerve. Pain has been going on for approximately 7 years and has been progressively worsening over time.
The patient was treated by chemotherapy using fludarabine and rituximab in 2009 without response, then followed by a combination of bortezomib and dexamethasone, which brought in an excellent partial remission. Currently, he was treated by trametinib and lenalidomide.
Approximately 4 months before presenting to clinic, the patient was examined by a whole-body positron emission tomography computed tomography (PET/CT) scanning to monitor the metastatic progression of multiple myeloma and response to chemotherapy. PET/CT showed a hypermetabolic focus in the soft tissues of the posterior left medial ankle (). The lesion went undiagnosed by the oncologist and radiologist and the patient continued to experience pain in the region until a magnetic resonance imaging (MRI) scanning was performed 2 months after the PET/CT was obtained. The left ankle contrast MRI showed a T1 hypointense () with mixed T2 hyperintense () mass lesion noted in the posterior medial soft tissues of the distal tibia, which demonstrated intense enhancement and abuts the tibial nerve. This was measured approximately 2.2 cm in maximum craniocaudad dimension () and approximately 1.9 × 1.0 cm2 in maximum axial dimension (). Preoperative biopsy was performed indicative of the mass to be schwannoma.
After careful assessment in a multidisciplinary team, the patient was referred to a specialist foot surgeon, and the tumor was surgically removed under general anesthesia. Prior to start of the surgical procedure an ultrasound was used to localize the area of the tibial nerve lesion, which was then marked using a skin marker. A longitudinal incision was made 1 cm wide behind the medial malleolus in line with the tibial nerve to expose the fascia of the tibial nerve. An enlarged mass of posterior nerve was seen. A freer elevator was used to lift the fascia away from the nerve and neurovascular bundle. Meticulous dissection was carried on to reserve the functional nerve fibers. A hemostat and blunt tenotomy scissors were used to dissect the nerve lesion, and the epineurium was gently incised longitudinally running in line with the nerve fibers and was dissected and retracted extracapsularly. The epineural tissue layer was meticulously dissected. It was found that the tumor surface can be exposed and the whole tumor can be freed via blunt dissection from the vascular bundle posterior medial distal leg ().
Gentle dissection along the plane of the tumor capsule from the epineural layers allowed the tumor to be shelled out as a whole (). The proximal and distal poles were approached, and two fascicles were isolated and cut from the nerve. A nerve stimulator (Vari-Stim III, nerve locator; Medtronic Xomed Inc, Jacksonville, FL, USA) was used to interrogate the nerve to identify the inactive nerve fascicles. A well-encapsulated tumor and inactive nerve was dissected and separated from the active nerve (). The tumor was an approximately 22 × 19 × 10 mm3 well-encapsulated and circumscribed lesion. The rest of the posterior nerve was reserved. The specimen was sent for histopathological analysis. Next, a nerve stimulator was used again to check the motor function of the foot, which was noted to be intact.
The postoperative histopathological analysis of the specimen revealed bland spindle cells and hyalinized stroma. Majority of the spindle cells were strongly immunoreactive to S-100. A few cells were immunoreactive to epithelial membrane antigen (EMA) ( and ). Neurofilament (NF) immunostaining showed scattered entrapped axons. The findings were consistent with the diagnosis of peripheral nerve schwannoma.
At 12 months of follow-up after surgery, the patient was asymptomatic without pain or numbness and fully able to walk without any limitation. No instability or muscular weakness was present. |
pmc-6458792-1 | A 53-year-old woman was diagnosed with vitiligo in September 2017 and was treated with oral Qubaibabuqi tablets (15 tablets three times daily; Xinjiang Yinduolan Uyghur Pharmaceutical Company Limited, Urumqi, China), 10 mg of prednisone acetate tablets (Xinhua Pharmaceutical Company Limited, Zibo, China) once daily, and narrowband-ultraviolet B (NB-UVB) phototherapy (Sigma household narrowband-ultraviolet phototherapy instrument [SS-01B] pocket portable; Shanghai Sigma High Technology Co., Ltd. Shanghai, China) every other day. The prednisone acetate tablets were self-discontinued 3 months later; however, she continued to take Qubaibabuqi tablets orally and NB-UVB phototherapy was undertaken at home. The patient seldom saw her physician, and bought Qubaibabuqi tablets from a pharmacy over the next 7 months. The patient presented to our clinic on March 27, 2018. She complained of weakness, nausea, and vomiting for 3 days. She was admitted to the Gastroenterology Department.
Physical examination revealed severe, diffuse yellow staining of the skin and sclera, and bilateral lower extremity edema. Patches of vitiligo involved the left frontal region, left chest, and right lower abdomen. The skin lesion in the left frontal region was nearly normal, and the color of the surrounding skin lesions on the left chest and right lower abdomen were black due to therapy. She had no history of cigarette smoking, alcohol consumption, or autoimmune diseases. She was otherwise healthy and denied taking any medications other than Qubaibabuqi tablets. A liver biopsy after hospitalization showed acute cholestatic hepatitis. Additional laboratory analyses revealed the following: normal routine blood tests; alanine aminotransferase (804.40 U/L; normal 7–40 U/L); aspartate aminotransferase (896.30 U/L; normal 13–35 U/L); total bilirubin (335.20 μmol/L; normal 5.00–24.00 μmol/L); direct bilirubin (233.00 μmol/L; normal 0.24–7.10 μmol/L); and indirect bilirubin (102.20 μmol/L; normal 2.80–23.80 μmol/L), negative HIV and syphilis antibodies; normal hepatitis A, B, and C serology and negative Epstein-Barr virus, herpes simplex virus, and human cytomegalovirus. Causality assessment using the updated Council for International Organizations of Medical Sciences scale showed 10 points (a probable causality) for Fructus Psoraleae contained in Qubaibabuqi tablets. Based on these data, the patient was diagnosed with drug-induced hepatitis.
After admission, the patient was given a high carbohydrate, low fat, and moderate protein diet, and actively maintained a balance of water, electrolytes, and pH, and corrected hypoproteinemia. Measures to prevent infection and provide oral care were adopted. She actively used medications (Magnesium Isoglycyrrhizinate Injection, Ornithine Aspartate Injection and Ademetionine 1, 4-Butanedisulfonate for Injection, and so on.) to protect liver function and treat hepatic coma. Due to poor treatment efficacy, the patient’s condition was aggravated and she was treated with an artificial liver support system besides the routine medicinal therapy. Although the patient was given active treatment, her condition continued to deteriorate. Her spirit gradually changed from sobriety to wandering, and then coma. Some symptoms indicated that her life was in danger, and these symptoms included fetor hepaticus, flapping tremor, multiple patches of flaky ecchymoses, elevated liver enzymes, and a coagulopathy. She died 5 days later due to acute liver failure and multiple organ dysfunction. This serious adverse event has been reported to the China Food and Drug Administration through the National Adverse Drug Reaction Surveillance System (No. 3701011011107321800095). |
pmc-6458794-1 | A 26 year-old man was identified to donate marrow for his brother. His height was 178 cm and his weight was 79.2 kg (Body mass index 25.0). He had no bleeding history or other medical problem. Bone marrow harvesting was performed under spinal anesthesia []. The patient was put in the prone position, and the bony landmarks of the posterior iliac crest and sacroiliac joint were palpated for the identification of a proper puncture site (Fig. ). Aspiration trocar and needle were pushed through the skin and subcutaneous tissue to the posterior iliac crest, and the cortical bone was punctured. Bone marrow aspiration was performed after positioning the needle tip within the cortical wall of the posterior crest []. There was no repositioing of the needle. The total surgery time was 62 min. A total of 900 cc of bone marrow(450 cc per site) was collected which yielded 1.46 × 108 CD34-positive cells from the two puncture sites shown in Fig. . No special problems occurred during the procedure. The donor was hospitalized one more day after bone marrow harvesting to check complications and to control the pain. There was no evidence of hematoma on the puncture sites. While in hospital, he suffered mild pelvic pain which had responded to an oral non-steroidal anti-inflammatory drug (NSAID).
Two days after the bone marrow harvesting, a pain of tingling and stabbing nature appeared on his left posterior thigh and calf. Pain score was noted at Visual Analogue Scale (VAS) 7 points on resting and aggravated with motion. Allodynia was present. Sensory of all dermatome was intact, and no muscle weakness was present. However, there was gait disturbance due to pain.
We conducted a pelvic magnetic resonance image (MRI), nerve conduction study (NCS), and electromyography (EMG) for evaluation. T1 and T2 weighted images of the pelvis magnetic resonance image (MRI) showed patchy edematous change with enhancement in the sacrum, retrosacral muscles, and subcutaneous layer, and the left S2 neural foramen (Fig. a, b). Imaging studies indicated that the left S2 nerve root was injured by mechanical damage when the puncture needle was inserted and that the nerve irritation and inflammation were the cause of the patient’s symptoms [, ].
After 1 month since the pain developed, nerve conduction study (NCS) and electromyography (EMG) were performed. Nerve conduction study (NCS) revealed normal velocity and amplitude of the common peroneal nerve, tibial nerve, sural nerve, and superficial peroneal nerve. Hoffmann reflex, pudendal evoked potential were within normal limits. Electromyography (EMG) showed abnormal spontaneous activities, which are denervation potentials, in the S2-innervated intrinsic foot muscles and the S1-S2 nerve root innervated muscles such as the soleus, gastrocnemius, and lumbar paraspinalis muscle (Table ) [–]. The amplitude of the abnormal spontaneous activities were about 100 μV, indicating that the development of muscle membrane instability following neural injury occurred within 1 month [] (Fig. ). Electrodiagnostic results along with the patient’s clinical presentation and MRI findings led us to a diagnosis of left S2 radiculopathy.
The patient took pregabalin 75 mg two times per day to control the pain, and after 3 months of medication, the patient’s pain improved from VAS 7 to 5 []. A follow up nerve conduction study (NCS), electromyography (EMG) and pelvic magnetic resonance image (MRI) were performed 3 months after onset. Consistently, nerve conduction study (NCS) and Hoffmann reflex were within normal limits, and abnormal spontaneous activities were observed in S2 nerve root innervated muscles. In the pelvic magnetic resonance image (MRI), little residual enhancement was still present along the left S2 nerve root (Fig. c, d).
At 6 month follow up, visual analogue scale (VAS) further improved to VAS 3, and electromyography (EMG) showed motor unit action potentials (MUAPs) of re-innervation pattern instead of abnormal spontaneous activities, indicating recovery state (Table , Fig. ). The patient took pregabalin for a total of 8 months. After that, the patient stopped medication. One year later, the patient’s pain was reduced to a level that was not inconvenient, and we did not prescribe any further medication. Additional nerve conduction study (NCS), electromyography (EMG), and magnetic resonance image (MRI) were not performed. |
pmc-6458828-1 | A 39- years old multiparous woman has suffered from mild MS for 10 years, as a result of childhood bacterial tonsilopharyngitis (rheumatic MS). The first attacks of symptoms appeared in her last year of second decade, during her second pregnancy. Her chief complaint was exertional dyspnea. Two - dimensional echocardiography (2D-ECG) revealed a decreased mitral valve area (1.5 cm2). She had no other medical conditions and her past medical history included nothing else of note. Recently the symptoms worsened and she began to experience dyspnea in ordinary activities, AF and hemoptesia (hemoptysis). 2D-ECG positive findings were: critical diminished mitral valve area (0.8 cm2), left atrial enlargement without any organized clot attachment, thickened leaflets, commissural and subvalvular fussions and mean diastolic pressure gradient across valve =12 mmHg. She was scheduled for MVR with mechanical prosthesis. Open cardiac surgery with CPB using bicaval connulation was carried out. Early post-operative course was uneventful and she was discharged to home on POD = 7 with warfarin prescription without any prohibition about taking it, since she had completed her family members. She felt face tenderness and hearing problems besides stuffy and clogged nose on POD = 20. She underwent ENT consultation and based on the clinical and paraclinical finding, met ARS diagnosis (Fig. ). As soon as an antihistamine, anticongestion and macrolide antibiotic was initiated. She came back to ENT service with persistent ARS as well as relapsing preoperative couphs and dyspnea besides lower extremities edema (Fig. ) and toenails discoloration (Fig. ). After some modulations of therapeutic drugs, she was referred to pulmonary clinic on POD = 30. CXR confirmed a significant left pleural effusion (Fig. ) and she was readmitted in our service while taking lasix and SABAs (albuterol inhaler) in addition to aforementioned medicines on POD = 33. Immediately a pigtail catheter was secured with the purpose of both assessment and management. At first the fluid was bloody then became milky. Finally we attained 1850 cc frankly white fluid. Bedside ether test revealed the presence of fat which was confirmed by lab study (triglycerides = 1750 mg/dl chylomicron). We encountered with a patient who had different signs such as chylothorax, lymphedema and yellow discoloration of toenails. After dermatologist consultation we arrived at Yellow Nail Syndrome on POD = 35. Immediately conservative treatment was taken place such as bed rest, massage and compression therapy of legs using garments, restricted diet consist of high protein low fat with medium chain triglycerides and octreotide. Since the decline of chylous drainage was unsatisfied (330 cc/d), we exchanged the pigtail catheter with an appropriate size chest tube and TPN was initiated. Then talc and doxycycline pleurodesis was carried out besides Vitamine E supplement on POD = 41. Fortunately chest tube output substantially decreased, allowing transition to low fat oral intake diet on POD =45. Chest tube was removed and the patient was discharged on POD = 47. At 4 weeks follow ups, CXR was clear without effusion and nails discoloration and legs lymphedema resolved. |
pmc-6458836-1 | This 50-year-old female patient complained of a slow decline of her cognitive speed over several months and became symptomatic with generalized seizures. Her previous history revealed a myocardial infarction at the age of 42 years. Computerized tomography (CT) and magnetic resonance imaging (MRI) of the head showed a right temporal mass with focal calcifications, moderate perifocal edema and a reticulated contrast enhancement comprising neo- and allocortical regions (Fig. ). Suspecting a high-grade glioma with an oligodendroglial component, the tumor was resected subtotally. Unexpectedly, the intraoperative finding was a firm, partially calcified and pink to greyish mass that was hypovascularized and poorly demarcated, so that no clear resection border could be defined intraoperatively.
Postoperatively, the patient recovered well, displayed a gradual cognitive improvement during the 24-month follow-up. She kept seizure-free under antiepileptic medication to date during the 8-year follow-up. MRI did not show any recurrence so far. |
pmc-6458853-1 | A 43-year-old woman complained of rotatory positional vertigo and nausea in the morning, particularly when lying down. She was examined at a local emergency department, on the same morning. Neurological examination and brain computed tomography (CT) revealed no abnormal findings, and she was discharged. Owing to persistence of symptoms, she visited our clinic 7 days after the onset. She denied any history of hearing loss, tinnitus, headache, or facial neurological symptoms. She had a history of BPPV 3 years ago. Her past medical, surgical, and family history was unremarkable; there was no history of head trauma. On examination, there was no dysdiadochokinesis, dysmetria, or tremors. Her gait was not ataxic, and there was no spontaneous or gaze-evoked nystagmus. Pure tone audiogram, neurological, and eye movement examinations, including tests of eye tracking, saccades, and drum optokinetic nystagmus test, were normal. Otolithic function was tested using cervical vestibular evoked myogenic potentials (VEMPs); ocular VEMPs showed no pathological findings. The positional and positioning nystagmus test, including the supine head roll and the bilateral Dix–Hallpike tests, was recorded using an infrared charge-coupled device camera. The supine head roll test revealed upbeat nystagmus for >110 s with a latency of 2 s on changing from the upright seated to straight supine position (). Video-oculography was performed using the public domain software ImageJ and a Windows computer []. Head position was changed from the upright seated to the straight supine position by tilting the backrest of the electric chair backwards over a period of 8 s. In the supine head roll test, turning from the straight to the left ear-down supine position led to immediate disappearance of upbeat nystagmus; on turning from the left to the right ear-down position, slight torsional nystagmus towards the right was observed for >22 s with a latency of 6 s. In the Dix–Hallpike test, left head-hanging position provoked torsional nystagmus towards the right for 50 s with no latency. In the prone seated position, downbeat nystagmus with the torsional component towards the left was observed for 45 s with a latency of 3 s. The right head-hanging and upright seated position during the Dix–Hallpike test did not provoke nystagmus. Ten days after the onset, nystagmus and vertigo disappeared with no medical or physical treatment. |
pmc-6458857-1 | A 57-year-old male with a history of sickle cell disease (HbSC) without long-term indwelling vascular access complicated by recurrent cholecystitis and choledocholithiasis presented to our quaternary care hospital with a chief complaint of right upper quadrant and back pain. His vital signs on presentation included a temperature of 99.2°F, heart rate of 107 beats per minute, blood pressure of 105/58, respiratory rate of 22/min, and oxygen saturation of 96% on room air. His exam was remarkable for scleral icterus and right upper quadrant tenderness. Initial labs were notable for a leukocytosis of 38,200 cells/mm3, direct hyperbilirubinemia of 26.1 mg/dL, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) of 425 IU/L and 303 IU/L, respectively, and a lactate of 7.3 mmol/L. He was admitted to the intensive care unit for management of shock. Blood cultures drawn on the day of admission grew Klebsiella pneumoniae and Citrobacter koseri. He was started on intravenous piperacillin-tazobactam. Imaging at the time of presentation displayed evidence of prior cholecystectomy with obstructive choledocholithiasis and multiple liver abscesses less than 2 cm in diameter. He underwent emergent endoscopic retrograde cholangiopancreatography (ERCP) with balloon dilation of the common bile duct and biliary stent placement. Transthoracic echocardiogram done at that time showed decreased ejection fracture with otherwise normal valve structure and function. Despite biliary stenting, the patient continued to complain of right upper quadrant and lower back pain and his blood cultures remained persistently positive after stenting.
On hospital day 6, the patient was reimaged with magnetic resonance cholangiopancreatography (MRCP) which displayed evidence of possible ongoing biliary obstruction. A midline was placed for ease of access for medication administration. On hospital day 7, repeat ERCP showed a patent stent from his prior procedure, but a second stent was placed to relieve a left hepatic lobe stricture. Even with this attempted source control, polymicrobial bacteremia persisted with both Klebsiella pneumoniae and Citrobacter koseri through hospital day 12. A transesophageal echocardiogram (TEE) was completed to evaluate for infectious endocarditis as an ongoing source of bacteremia. A 10 mm mobile vegetation was visualized on the pulmonic valve with mild pulmonary regurgitation (). The Infectious Diseases consult team advised switching from piperacillin-tazobactam to combination therapy of intravenous ceftriaxone and oral ciprofloxacin. His midline catheter was removed, but tip was not sent for culture. His blood cultures cleared on hospital day 13. Repeat CT abdomen pelvis completed on hospital day 18 revealed an overall improvement of the previous liver abscess, except for one right lobe abscess that was slightly increased in size, and a new subpleural left lower lung lobe nodule concerning for septic embolus. Surgical evaluation was not pursued given his comorbidities and poor surgical candidacy. Ultimately, he was discharged to complete 6 weeks of intravenous ceftriaxone and oral ciprofloxacin. At the time of this case report, he has not required further intervention on the heart valve. |
pmc-6458861-1 | The presented patient is a 5-year-old male Jack Russell Terrier and it is considered as a member of the family, lives mainly indoors, and is correctly vaccinated against distemper, parvovirosis, leptospirosis, Rubarth disease, parainfluenza, and rabies. The dog is treated monthly against external parasites with afoxolaner. The dog and owner live in Belgium (province of Liège). They are used to do long recreational hikes in the forest all year long and ticks were occasionally found and removed from its skin. The dog has no history of travelling abroad.
In December 2017, the owner observed sudden weakness and a reluctance of his dog to move from his basket. The following day, because of the persistency of the signs, the dog was presented to the local veterinarian who noticed a reluctance to move, generalized muscular pain, hindquarters articular pain, and a mild fever. Results of hematology examination and biochemistry were unremarkable. Serum was sent to an external laboratory only for serological examinations for Toxoplasma gondii and Borrelia spp.: the results of serology for toxoplasmosis were negative and Lyme serology (immunofluorescent antibody testing) was positive (1: 1024). As soon as the results were available, the dog was prescribed doxycycline 5 mg/kg per os twice a day for 30 days. Three days after initiation of the treatment, the dog's condition improved, the clinical signs disappeared and, from that moment, it never presented similar symptoms again. |
pmc-6458862-1 | Core biopsy from a large intraosseous and extraosseous, cortex-based tumor of the diaphysis of the right femur in a 34-year-old woman () showed a partially necrotic, cellular proliferation of monomorphic, small, blue, and round cell population, which was embedded in a sclerotic stroma, resulting in a striking trabecular appearance. There was no evidence of typical osteoid or mineralisation. The immunophenotype was nonspecific (CK−, S100−, Des−, and CD45−); however diffuse CD99 expression was found. An unusual rearrangement pattern with an amplified and split red signal was found in the EWSR1 FISH, which at the time of the diagnosis 11 years ago had not been previously described and was considered difficult to interpret and unusual for ES. The RT-PCR of the EWSR1-FLI1 and EWSR1-ERG fusions was negative. Although largely a diagnosis of exclusion, the microscopic findings were considered consistent with sclerosing epithelioid fibrosarcoma (SEF). MUC4 immunostaining was not available at the time of the diagnosis and was negative retrospectively. No further tumor manifestations were found on staging. The clinical decision was made to treat the patient with preoperative chemotherapy according to the EURAMOS protocol []. Subsequent complete tumor resection revealed no chemotherapy-induced necrosis. 4.5 years after the initial presentation, a 1 cm skin metastasis on the ipsilateral thigh was completely excised and showed identical microscopical pattern as the primary. The patient was regularly followed, and 10.5 years after the first presentation, a solitary 7 mm lung metastasis was resected. Currently (11 years follow-up), the patient is doing well without known tumor manifestation. NGS analyses performed retrospectively on the skin metastasis revealed the EWSR1-NFATC2 gene fusion. |
pmc-6458862-2 | Due to difficult histologic interpretation and extensive necrosis, two core biopsies were performed at external institutions, followed by the curettage of an intraosseous and extraosseous process of a 42-year-old man with clinical suspicion of an osteomyelitis of the left tibia (). The tumor cells were small and inconspicuous, grouped in solid nests and trabecula, embedded in collagen-rich matrix. The cytoplasm was clear with distinct borders. There were few mitoses and a low proliferation index. The immunophenotype revealed diffuse, weak expression of cytokeratin, focal CD99 positivity, and coexpression of EMA, CD10, and CD117. Immunolabeling for S100, SMA, desmin, p63, CD34, and CD45 was negative. Nuclear expression of INI1 was retained. EWSR1 FISH showed a rearrangement pattern of an amplified split red signal. RT-PCRs of the EWSR1-FLI1 and EWSR1-ERG fusions were negative. Based on the microscopic appearance, cytokeratin expression and low proliferation rate, the diagnosis of a primary low-grade myoepithelial carcinoma was rendered. No further tumor manifestations were found upon staging. Local complete resection with reconstruction was performed. Persistent surgical complications led to amputation 7 years later. No adjuvant therapy was given. Neither local nor systemic tumor manifestations were found with a follow-up of altogether 8.5 years. Retrospective NGS of the curettage sample revealed the EWSR1-NFATC2 fusion. |
pmc-6458862-3 | Core biopsy of an intra-abdominal epigastric mass in a 60-year-old woman () showed mesenchymal tumor with abundant extracellular matrix, composed of a trabecular network of monomorphic cells. Immunohistochemistry was negative for GIST markers as well as S100, HMB45, SMA, desmin, synaptophysin, STAT6, and MUC4. The nuclear expression of INI1 was retained. Rare cells expressed cytokeratin and EMA. Diffuse weak CD99 expression was observed. The differential diagnosis included EMC and myoepithelial tumor. As both categories may show rearrangement of the EWSR1, FISH was performed revealing rearrangement with low-level amplification of the red signal. NR4A3 FISH showed a normal pattern, excluding the diagnosis of EMC. NGS analysis of the resection specimen revealed the EWSR1-NFATC2 fusion. No adjuvant therapy was given, and no further tumor manifestations were found upon staging. |
pmc-6458862-4 | Core biopsy of a metaphyseal mass of the right humerus in a 12-year-old boy () revealed an intramedullar bland spindle-cell proliferation with focal siderin depositions and few osteoclast-type giant cells. No necrosis or mitotic activity was observed. There was no osteoid or cartilage production. Given the radiologic differential diagnosis of an aneurysmal bone cyst (ABC), USP6 FISH was performed which was inconclusive. NGS, initially performed on the core biopsy and repeated independently on the subsequent curettage, revealed the same FUS-NFATC2 fusion in both specimens. FUS FISH performed for verification of the NGS showed the classical break-apart pattern without amplification. The lesional tissue showed very low proliferative activity in the MIB1 staining (<5%). Diffuse expression of EMA and CD99 was seen, while the reaction for SMA, desmin, S100, CD34, and synaptophysin remained negative. The curettage did not contain high-grade tumor. At 8 months follow-up, there was no evidence of recurrent tumor on the control MRI. |
pmc-6458863-1 | A 34-year-old female with no notable past medical history or manifestations of hyperandrogenism underwent evaluation by her gynecologist for abnormal menstrual cycles. She described her menstrual cycle as regularly occurring every 40 to 35 days and reported it as heavy. She was found to have elevated dehydroepiandrosterone (DHEA) of 336 mcg/dL (23-266 mcg/dL), as well as borderline total testosterone of 45 ng/dL (2-45 ng/dL) and free testosterone level of 6.5 pg/mL (0.1-6.4 pg/mL), with no reported intake of exogenous androgens, hormonal birth control modalities, or glucocorticoids. Also, the patient had no known heart disease, osteoporosis, or anorexia nervosa. Endocrinology consultation and additional hormonal evaluation were unremarkable, with estradiol of 24 pg/mL (19-144 pg/mL), 17-hydroxyprogesterone of 42 ng/dL (23-102 ng/dL), FSH of 7.2 mIU/mL (2.5-10.2 mIU/mL), LH of 4.5 mIU/mL (1.9-12.5 mIU/mL), and TSH of 1.74 mIU/L (0.4-4.5 mIU/L). She subsequently underwent an abdominal computed tomography (CT) scan due to concern for an underlying adrenal adenoma. No ovarian or adrenal abnormalities were noted in imaging; however an indeterminate pancreatic tail mass measuring 2.7 x 1.3 x 2.0 cm was seen. Subsequent MRI of the abdomen further characterized the lesion as an enhancing pancreatic tail mass, concerning for pancreatic neoplasm.
The patient then underwent endoscopic ultrasound (EUS) for further evaluation and biopsy. EUS confirmed a hypoechoic mass in the pancreatic tail, measuring 2.3 x 1.7 cm (). An intact interface was seen between the mass and adjacent structures suggesting an absence of invasion and cytology revealed SPN (Figures , , and ). The patient was advised to undergo distal pancreatectomy and splenectomy, which she tolerated well, without any complication. Immunohistochemical staining of the resected specimen was positive for beta-catenin, CD10, and nuclear progesterone receptors (Figures , , and ). Estrogen receptors were predominantly negative. Chromogranin and synaptophysin were negative.
Postsurgically, laboratory testing revealed normalized total testosterone of 33 ng/dL (2-45 ng/dL) and free testosterone level of 2.6 pg/mL (0.2-5.0 pg/mL); however dehydroepiandrosterone (DHEA) remained elevated at 351 mcg/dL (23-266 mcg/dL). The remaining hormonal workup was unremarkable, with estradiol of 162 pg/mL (56-214 pg/mL), 17-hydroxyprogesterone of 134 ng/dL (129-431 ng/dL), FSH of 1.7 mIU/mL (1.5-9.1 mIU/mL), LH of 3.4 mIU/mL (0.5-16.9 mIU/mL), and TSH of 1.98 mIU/L (0.4-4.5 mIU/L). |
pmc-6458875-1 | A 75-year-old African American female with a history of hypertension, hyperlipidemia, peripheral artery disease, and essential thrombocythemia, complicated by sequential bilateral central retinal vein occlusion, was seen for follow-up. She first presented to hematology in March 2015 with a history of left central retinal vein occlusion, stable claudication from peripheral arterial disease, and progressive thrombocytosis. She had quit smoking three months prior. Laboratory review revealed persistent increased platelet counts (>800 × 109/L) over the previous six months. Blood smear was notable for increased large mature megakaryocytes relative to red blood cells; no left shift, leukoerythroblastosis, or dysplasia was observed. Iron studies, lactate dehydrogenase, BCR-ABL translocation, and JAK2 V617F mutation studies were unremarkable. The patient refused bone marrow biopsy, and a presumptive diagnosis of ET was given, supported by calreticulin (CALR) mutation detected. Low-dose aspirin and hydroxyurea were initiated, and platelets were at goal (<400 × 109/L) two months later. Her history was further complicated by right central retinal vein occlusion in December 2016 during a period of poor compliance with cytoreductive therapy.
In May 2018, she reported progressive fatigue and 15 lb unintentional weight loss over the preceding three months. Laboratory evaluation was notable for a macrocytic anemia and platelets at goal (WBC 6 × 109/L; Hb 10 g/dL; MCV 101.7 fL; Plt 375 × 109/L). She agreed to bone marrow evaluation which revealed a normocellular marrow with an increased number of enlarged and hyperlobated megakaryocytes and variable mild reticulin fibrosis (less than MF-1); blasts were not increased. Fluorescent in situ hybridization analysis for BCR/ABL, PDGFRA, PDGFRB, and FGFR1 was negative. Peripheral blood molecular analysis revealed a mutation in the calreticulin (CALR) gene; JAK2 V617F, JAK2 exon 12, and myeloproliferative leukemia virus (MPL) oncogene mutations were not identified. These findings confirmed a diagnosis of essential thrombocythemia and excluded myelofibrosis as the cause of her fatigue and weight loss. Further evaluation with CT scan of the chest, abdomen, and pelvis demonstrated atherosclerosis and completely occlusive thrombosis of the infrarenal aorta, extending bilaterally into the common iliac arteries, with reconstitution of flow distally via collaterals (). There was no splenomegaly. Subsequent CT angiogram of the abdomen showed redemonstration of the aortic thrombus without occlusion of the mesenteric arteries, the celiac trunk, or the hepatic, splenic, and renal arteries.
The patient's only symptom was mild chronic claudication which had not changed in quality; she denied lower extremity swelling or skin mottling. She reported compliance with her prescribed medications for hypertension, hyperlipidemia, and ET. Lower extremity ultrasound and ankle brachial index were not significantly changed from prior studies. An evaluation for inflammatory disorders and thrombophilia, including erythrocyte sedimentation rate, C-reactive protein, anti-nuclear antibodies, anti-neutrophil cytoplasmic antibodies, lupus anticoagulant, anti-phospholipid antibodies, factor V Leiden mutation, prothrombin gene mutation, and protein C and protein S, was unremarkable.
In light of her very mild symptoms and good collateralization on imaging, she was offered medical management with surgical intervention reserved for treatment refractoriness or acute ischemia. Clopidogrel 75 mg daily was added to the aspirin 81 mg and hydroxyurea 1000 mg she was already taking. Additionally, she was started on cilostazol 100 mg twice daily by her vascular surgeon to treat her claudication. She reported initial improvement in her symptoms one month after starting therapy. She did not return for follow-up until February 2019 due to temporarily relocating out of the state. She had been noncompliant with therapy for ET. Her platelet count was 948 × 109/L, and a CT scan showed no significant interval change in the occlusive thrombus of the infrarenal abdominal aorta and proximal common iliac arteries. She refused surgical intervention and was restarted on previous medical therapy. |
pmc-6458877-1 | A 2650 g preterm female was born at 39 weeks' gestation to a 30-year-old Asian-Indian primigravida by induced vaginal delivery because of prolonged rupture of membranes (PROM). Prenatal care was unremarkable except for PROM (120 hours). The mother-to-be was admitted for induction of labor 2 days prior to delivery. She developed a temperature of 101 Fahrenheit during labor and received 2 courses of ampicillin and gentamicin. Vacuum suction was applied twice to assist the delivery. The amniotic fluid was noted to be “meconium-stained.” Apgar scores were 3, 6, and 7 at 1, 5, and 10 minutes, respectively. Intermittent positive pressure ventilation was applied for 3 minutes after which spontaneous breathing was noted. Physical examination revealed a weight of 2650 g (5th centile), length 52 cm (75th centile), and head circumference 33 cm (25th centile). Cephalhematoma was noted at the occipital area secondary to vacuum extraction. The baby had respiratory distress and was admitted to intensive care unit (NICU). During the first few hours of life, the patient required 100% supplemental oxygen via nasal continuous positive airway pressure (CPAP). Umbilical arterial and venous catheterizations were performed. Arterial blood gas at 1 hour of age showed pH 7.18, pCO2 28, pO2 44, and base deficit −17 meq/dL. Blood culture was obtained, and high-dose ampicillin and gentamicin were begun. Complete blood count at 12 hours of age showed high band count with a ratio of immature and total neutrophils (I/T ratio) of 0.5. Chest X-ray was unremarkable, and the patient rapidly recovered from respiratory distress. Umbilical arterial and venous catheters were removed within 24 hours of life. The blood culture was negative, and the patient was discharged after receiving a 7-day course of intravenous (IV) antibiotics.
Three days after hospital discharge, the patient was noted to be lethargic and had a poor oral intake. She was referred from the pediatrician office to the emergency department, where blood culture and lumbar puncture (LP) were performed. Cerebrospinal fluid (CSF) was cloudy. Intravenous ampicillin and gentamicin were given within an hour after LP. Immediately after admission to the NICU, ceftazidime was added to the treatment. The patient was febrile upon NICU admission. The Gram stain of the CSF specimen revealed numerous Gram-negative rods. Within 24 hours, the blood and CSF cultures revealed a growth of Gram-negative bacilli.
The bacteria were subsequently identified as E. coli. Seventy-two hours after admission, the initial broth microdilution antimicrobial susceptibility results (Microscan) for both isolates were as follows: resistant to ampicillin, gentamicin, tobramycin, cefazolin-ceftriaxone, levofloxacin, and sulfamethoxazole-trimethoprim; susceptible to meropenem, aztreonam, piperacillin-tazobactam, amikacin, and meropenem; and intermediately sensitive to ceftazidime and augmentin. Thereafter, IV meropenem and amikacin were begun. It took 5 days to sterilize the bacteremia, and the CSF became sterile 7 days after admission. Complete blood count showed an I/T ratio of 0.5. Serial MRIs were performed after 2 and 3 days, and 2, 3, 4, and 7 weeks of therapy showed subdural hydrocephalus, ventriculitis, abscesses, and subdural empyema (Figures and ). A ventricular tap was done 10 days after admission. Ventricular reservoir placement and subdural empyema tap were performed 14 days after admission. CSF culture from the ventricle was negative but the subdural exudate grew ESBL E. coli. She developed dysphagia after readmission to the NICU, and it took 11 weeks to achieve full oral feeding. After completion of 9 weeks course of meropenem and 6 weeks course of amikacin, she was discharged at 83 days of age; his weight was 4480 g (10th centile), length 58 cm (50th centile), and head circumference 38 cm (10th centile). Repeat MRI prior to discharge showed trace amount of the subdural empyema, mild ventriculitis, and ventriculomegaly (). |
pmc-6458881-1 | In February 2015, a 78-year-old white male was referred to us for the occurrence of a solitary rapidly growing nodule on the scalp. The nodule measured 3 cm in diameter. Clinical examination revealed a subcutaneous, bright red, ulcerated, dome-shaped lesion with irregular margins. The patient underwent an intervention of surgical excision, covering the substance loss with a skin graft from the left inguinal region. Subsequently, accurate haemostasis and suture were performed, and a compressive medication was applied. The histopathology described an ulcerated undifferentiated malignant neoplasm consisting of cells of different dimension and high mitotic index. The immunohistochemistry showed a weak positivity for S-100 and HMB45, negativity for CK, and moderate positivity for CD68. The surgical margins were clear and the diagnosis was atypical fibroxanthoma.
After a month from the surgery, in May 2015, a new nodule appeared in correspondence of the graft, suggesting a recurrence (). Considering disease relapse and the rarity of atypical fibroxanthoma, a histopathological revision of the case was requested. The second pathology report described a subcutaneous malignant neoplasm with spindle cells and pleomorphic epithelioid cells, with necrosis and atypical mitosis. De novo immunohistochemistry showed positivity for S-100 and ML actine, focal for HMB45 and MART-1, negativity for P63. Immunohistochemistry revision revealed negativity for CD68 and focal positivity for pan-CK and HMB45. This analysis suggested a diagnosis of undifferentiated pleomorphic sarcoma, but it has not excluded an acromial melanoma with aberrant ML actine expression.
A local treatment with ECT has been performed in the region of the cutaneous relapse using a Cliniporator® EPS-02 produced by IGEA®. The procedure is reported following the guidelines by Campana et al. []. Electroporation was performed after 8 minutes from the end of slow bleomycin intravenous infusion (Bleomycin TEVA, 28500 IU-15000 IU/m2 of body surface area, diluted in 100 cc of physiologic solution in 15 minutes). The patient underwent a general anesthesia (deep sedation with Propofol—Diprivan®) before an adjustable linear needle electrode by IGEA® was introduced into the tumor mass at a depth of 15 mm; also, a safety margin of 1 cm was treated around it. A series of 8 pulses of 1000 V/cm was delivered at a frequency of 5 kHz and duration of 100 microseconds, as recommended by ESOPE guidelines []. In order to guarantee that the tumor received a sufficient amount of bleomycin, the treatment was completed within 15 minutes after the end of the infusion. A sterile plate medication was finally applied on the treatment site. The patient did not report residual pain and did not have any kind of complication. Four weeks after ECT treatment, the mass diminished in diameter and appeared completely necrotized (). Eight weeks after treatment, all that remained was an eschar that detached, leaving an erythematous zone of reepithelialization (). This area disappeared within three months, with a complete restitutio ad integrum (). |
pmc-6458882-1 | A 71-year-old man with a 25 pack-year history of tobacco use presented for continuing care, and physical examination revealed a painless right parotid gland mass previously unnoted by the patient. It was palpable as a soft 0.5 cm mass. Cranial nerve examination was without deficits, and no cervical lymphadenopathy was detected. He had a history of left parotidectomy for Warthin tumor three months prior and Mohs surgery of the right cheek for nonmelanoma skin cancer five years prior. CT scan revealed a 1.4 x 1.3 cm right superficial parotid mass. For diagnostic and therapeutic purposes, a right superficial parotidectomy was performed. Intraoperative frozen section examination revealed mucoepidermoid carcinoma, intermediate grade. Thus, the decision was made to perform a right deep lobe parotidectomy with preservation of facial nerve and right neck dissection. The patient underwent definitive IMRT 60Gy radiation therapy following recovery from the surgery and has been followed up for four months.
On gross examination of the resected right superficial parotidectomy specimen, serial sections revealed a 1.3 x 1.1 cm firm, tan-white intraparenchymal tumor nodule with ill-defined borders. In addition, a cyst measuring 0.6 cm was present 1.1 cm away from this tumor, with grossly unremarkable intervening parenchyma. Histologic examination of the nodule showed two distinct lesional components that were well-demarcated from each other with no transition zone (). One portion showed well-circumscribed multinodular proliferation composed of dark blue, basaloid tumor cells arranged in nests with frequent peripheral palisading. Cytologically, the tumor cells demonstrated ovoid, basophilic nuclei and scant cytoplasm. Distinct, dense ribbons of eosinophilic hyaline material were noted surrounding the islands of tumor cells (). These histologic findings were those of basal cell adenoma, membranous type. Immunohistochemical stains for p63 and CK5/6 highlighted a prominent abluminal population, and CK7 highlighted patchy cells throughout, with a subset of luminal cells staining intensely (). The other portion of the tumor was characterized by a multinodular proliferation of multiple distinct cell populations arranged in frequent mucin-containing glandular spaces and as solid tumor cell nests. The lesional cells consisted of an admixture of mucous cells (mucocytes), polygonal epidermoid cells, and intermediate cells (). A special stain for mucicarmine highlighted intracellular mucin within the mucocytes as well as the extracellular mucin (). Cytologic atypia was moderate to focally marked. Necrosis was not seen and the mitotic activity was scant. Immunohistochemical stains for p63 and CK5/6 highlighted a predominant abluminal epidermoid/squamoid cell population, and CK7 highlighted mucocytes and cells adjacent to extracellular mucin of the glandular lumens. The Ki-67 proliferation index was approximately 3-4% (). The histologic findings were consistent with intermediate-grade mucoepidermoid carcinoma. Microscopically, the grossly noted 0.6 cm cyst consisted of a simple cyst composed of cytologically bland cuboidal lining cells with focal mucinous metaplasia, consistent with a salivary duct cyst. The parenchyma between this region and the tumor was histologically unremarkable as was the deep lobe of the parotid. Eighteen sampled lymph nodes were negative for tumor.
To further investigate the association between the two tumor components, molecular profiles of both were analyzed by next-generation sequencing (NGS) (FoundationOne®, Cambridge, MA). Among the 315 genes tested, the MEC and BCA components shared five genomic variants in common, all characterized as variants of unknown significance (VUS). Additionally, each showed one or two distinct likely driver genomic alterations (). Interphase fluorescence in situ hybridization (FISH) for MAML2 rearrangement, a genetic alteration commonly found in MEC, was attempted, but testing was uninformative as no interpretable hybridization signals were present to complete the analysis. |
pmc-6458894-1 | A 78-year-old man presented with constipation and abdominal distension. His medical history was remarkable for diabetes mellitus and dilated cardiomyopathy. Laboratory data were unremarkable except for a slightly increased level of cancer antigen 19-9 (45.8 U/ml). Colonoscopy revealed a circumferential impassable tumor located 28 cm from the anal verge. Contrast-enhanced computed tomography (CT) showed irregular colonic wall thickening with infiltration into the urinary bladder (). No lymph node enlargement or distant metastases were found. Histological examination of the biopsy revealed adenocarcinoma. The RAS/RAF mutational status was not investigated. The pretreatment diagnosis was LACC stage IIC (T4bN0M0). We surmised that immediate radical surgery would result in substantial bladder resection with impaired urinary function. Therefore, neoadjuvant chemotherapy before radical surgery was planned.
Firstly, we performed loop transverse colostomy for symptomatic colonic obstruction. Secondly, we planned 6 courses of chemotherapy with CAPOX and the treatment was initiated 1 month after the loop colostomy. We did not add molecular targeted agents because of his reduced cardiac function (ejection fraction of 21%). Follow-up CT after 3 courses of CAPOX revealed significant tumor shrinkage (). However, after 5 courses of CAPOX, grade 1 neurotoxicity and grade 2 neutropenia were observed. As he did not wish to receive oxaliplatin, the 6th course comprised capecitabine alone. Follow-up CT after the treatment (5 courses of CAPOX and 1 course of capecitabine alone) showed tumor disappearance ().
Thereafter, we performed sigmoid colectomy, partial resection of the bladder, and diverting ileostomy. The postoperative course was uneventful, and he did not suffer from neurogenic voiding dysfunction or urinary incontinence. Well-tolerated pathological examination revealed no residual tumor cells in the resected specimen, which was consistent with pathological complete response (pCR) and grade 3 effect according to the Japanese Classification of Colorectal Carcinoma () []. The final pathological diagnosis was ypT0, ypN0, M0, ypStage 0. We then planned 8 courses of adjuvant chemotherapy with CAPOX. After 4 courses, he developed grade 2 neutropenia despite a dose reduction (70%) for oxaliplatin. Therefore, the following 4 courses of chemotherapy comprised capecitabine alone. Subsequently, he underwent ileostomy closure and had no recurrence at 25 months after the initial diagnosis. |
pmc-6458894-2 | A 79-year-old man presented with abdominal pain. His medical history was significant for high blood pressure without the need for medication. Laboratory data revealed an increased level of carcinoembryonic antigen (77.5 ng/ml) and a normal level of cancer antigen 19-9 (33.7 U/ml). Colonoscopy revealed an advanced tumor with 80% of the circumference in the sigmoid colon. Histological examination of the biopsy revealed adenocarcinoma. The RAS/RAF mutational status was not investigated. Contrast-enhanced CT showed irregular colonic wall thickening with infiltration into the urinary bladder (). There were enlarged regional lymph nodes suggestive of metastases but no distant metastasis. The pretreatment diagnosis was LACC stage IIIC (T4bN2M0).
Similar to Case 1, we initially performed loop transverse colostomy. Subsequently, we planned 6 courses of neoadjuvant chemotherapy with CAPOX plus bevacizumab followed by surgery; however, the patient wished to continue chemotherapy rather than have surgery. No significant adverse events occurred. After 13 courses, CT showed significant tumor shrinkage and reduction of bladder wall thickening (). Colonoscopy revealed the disappearance of the primary tumor. Thereafter, he agreed to undergo surgery.
We performed Hartmann's operation with partial resection of the bladder. The postoperative course was uneventful, and he did not suffer from neurogenic voiding dysfunction or urinary incontinence. Pathological examination revealed no residual tumor cells in the resected specimen with foci of fibrotic tissue and inflammatory cell infiltration, which was consistent with pCR and grade 3 effect according to the Japanese Classification of Colorectal Carcinoma () []. The final pathological diagnosis was ypT0, ypN0, M0, ypStage 0. He then received 8 courses of adjuvant chemotherapy with CAPOX. Hartmann's reversal was not performed because he did not wish to do so. There was no recurrence at 65 months after the initial diagnosis. |
Subsets and Splits
Exclude ER emergencies
Retrieves 100 descriptions that do not contain the terms 'ER' or 'emergency', providing a basic filter of the dataset.