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如睾丸沿睾丸引带末端的其他分支下降至耻骨部、会阴部或股部,未降至阴囊底部,则成为异位睾丸。
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2.腹内压作用腹内压增高造成睾丸离开腹部进入腹股沟管,其证据是一种先天性腹壁肌肉发育不良的综合征,李状腹综合征(Prune-bellysyndrome),常伴有双侧腹腔内隐睾。
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3.内分泌调节内分泌因素在促进睾丸降入阴囊过程中肯定起了重要作用,但其具体的机制尚不明了。
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【病因】睾丸下降的机制尚未阐明,对隐睾的病因也不太清楚。
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1.内分泌失调临床研究表明,下丘脑-垂体-睾丸轴失衡导致隐睾患者睾酮水平低于正常。
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睾丸下降过程与睾酮水平密切相关,睾酮-双氢睾酮与精索和阴囊表面的受体蛋白单侧隐睾?
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2.副中肾管抑制物质(MIS)不足胚胎初期,胎儿同时具有副中肾管和中肾管。
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随着男性胎儿原始性腺发育为睾丸,睾丸内间质细胞分泌睾酮,支持细胞分泌副中肾管抑制物(MIS),抑制副中肾管发育。
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如果MIS不足,副中肾管可残留或完全没有退化,对睾丸的下降造成障碍。
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3.解剖障碍隐睾者鞘状突多终止于耻骨结节或阴囊上方,而异常的引带残余及筋膜覆盖阴囊入口,这些都可阻止睾丸下降隐睾睾丸常有不同程度的发育不全,体积明显小于健侧,质地松软,有时还有附睾和输精管发育畸形,发生率约为36%~79%。
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隐睾的病理组织学主要表现为生殖细胞发育的障碍,其次是间质细胞数量亦有减少。
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隐睾的曲细精管平均直径较正常者小,曲细精管隐睾的病理组织学改变随年龄增大而愈加明显,很多研究认为,2岁以后睾丸的组织病理学改变将难以恢复,故手术应在2岁以前完成。
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成人的隐睾,其曲细精管退行性变,几乎看不到正常精子。
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病理学改变的程度也和隐睾位置有关,位置越高,病理损害越严重,越接近阴囊部位,病理损害就越轻微。
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【隐睾并发症】隐睾可发生于单侧或双侧,以单侧较为多见。
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单侧隐睾者,右侧的发生率略高于左侧。
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但即使是双侧隐睾,仍有适量的雄激素产生,可维持男性第二性征的发育,也很少影响成年后的性行为。
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1.生育能力下降或不育隐睾的病理主要是生殖细胞发育障碍,因此影响生育能力。
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一般而言,双侧隐睾者生育能力明显下降,但如隐睾位置较低,可望留有部分生育能力。
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单侧隐睾的生育能力,如对侧降入阴囊的睾丸与附睾发育正常,则影响不大。
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2.鞘状突未闭隐睾的鞘状突基本都未闭,有肠管疝入则形成斜疝,可发生嵌顿,手术要同时结扎鞘状突。
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3.睾丸损伤由于睾丸处在腹股沟管内或耻骨结节附近,比较表浅,容易受到外力的直接损伤。
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4.隐睾扭转未降睾丸发生扭转的几率较阴囊内睾丸高20~50倍隐睾扭转一般表现为腹股沟部疼痛性肿块颇似腹股沟疝嵌顿但无明显胃肠道症状右侧腹内隐睾扭转,其症状与体征颇似急性阑尾炎。
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5.隐睾恶变隐睾恶变的几率比正常睾丸高18~40倍。
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高位隐睾,特别是腹内隐睾,其恶变发生率比低位隐睾高6倍。
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6岁以前行睾丸固定术而后发生恶变的,比7岁以后手术者低得多。
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隐睾恶变的发病年龄多在30岁之后。
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【诊断】诊断并不困难,体检可见患侧阴囊扁平,双侧者阴囊发育较差。
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触诊时,患儿阴囊空虚,无睾丸。
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大约80%的隐睾可在腹股沟管区扪及,压之有胀痛感,活动度大,不能被推入阴囊。
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小儿因提睾肌反射比较活跃,受到某些刺激,如寒冷或惊吓后,提睾肌收缩,可将本来位于阴囊内的睾丸提至阴囊近端,甚至进入腹股沟管内,临床表现颇似隐睾。
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另有些睾丸可从腹股沟部被逐渐地推入阴囊,但松手后,睾丸即上缩回原来位置,应属于隐睾。
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体检应注意仔细探查股部、耻骨部和会阴部,以除外异位睾丸。
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约20%的隐睾在触诊时难以触及,但这并不意味着这些隐睾都位于腹内。
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触不到的隐睾在手术中,约80%可在腹股沟管内或内环附近被发现,而其余的20%经手术探查,仍未能发现。
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如为一侧找不到睾丸,称为单睾或单侧睾丸缺如,发生率约占隐睾手术探查的3%~5%,约5000个男性中有1例单侧睾丸缺如;如双侧隐睾经探查,均未能发现睾丸,称为无睾畸形,约20000个男性中仅有1例。
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对于不能触及的隐睾,至今尚无满意的方法可于术前来判断睾丸的存在与否及隐睾所处的位置。
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超声、CT及磁共振检查,由于肠道气体的影响往往难以获得满意的结果。
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双侧触不到睾丸者,视外生殖器情况要做染色体检查,并可通过HCG激发试验来初步判断睾丸的存在与否,但手术探查是唯一可靠的办法。
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【治疗】隐睾常在新生儿体检时发现,一经诊断,即应随访。
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生后6个月,如睾丸仍未下降,则自行下降的机会已经极少。
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1.激素治疗隐睾的发生可能与内分泌失调有关,激素用于治疗隐睾受到普通重视。
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绒毛膜促性腺激素主要成分是黄体生成激素(LH),LH刺激间质细胞,产生睾酮。
[ { "id": 0, "entity": "绒毛膜促性腺激素", "start_offset": 0, "end_offset": 8, "label": "dru" }, { "id": 1, "entity": "黄体生成激素", "start_offset": 13, "end_offset": 19, "label": "dru" }, { "id": 2, "entity": "LH", "start_offset": 20, "end_offset": 22, "label": "dru" }, { "id": 3, "entity": "LH刺激间质细胞", "start_offset": 24, "end_offset": 32, "label": "bod" }, { "id": 4, "entity": "睾酮", "start_offset": 35, "end_offset": 37, "label": "bod" } ]
应用时可出现阴茎增大及睾丸胀痛,如果剂量掌握不当,或较长期使用,可导致骨骺早期愈合。
[ { "id": 0, "entity": "出现阴茎增大及睾丸胀痛", "start_offset": 4, "end_offset": 15, "label": "sym" }, { "id": 1, "entity": "如果剂量掌握不当,或较长期使用,可导致骨骺早期愈合", "start_offset": 16, "end_offset": 41, "label": "sym" } ]
目前已有报道提出,在1~3岁的小儿中使用HCG反而引起睾丸发育不良。
[ { "id": 0, "entity": "在1~3岁的小儿中使用HCG反而引起睾丸发育不良", "start_offset": 9, "end_offset": 33, "label": "sym" } ]
我们一般使用一个疗程,总计量5000~10000单位,分10次,间隔1~3天,注射完成。
[ { "id": 0, "entity": "一般使用一个疗程,总计量5000~10000单位,分10次,间隔1~3天,注射完成", "start_offset": 2, "end_offset": 43, "label": "pro" } ]
从效果来看,并不如文献报道的那样好,尤其是睾丸完全降入阴囊的病例很少。
[ { "id": 0, "entity": "睾丸", "start_offset": 21, "end_offset": 23, "label": "bod" } ]
LHRH作用于垂体前叶,促使垂体释放LH和FSH,被释放的LH发挥与HCG相同的作用。
[ { "id": 0, "entity": "LHRH", "start_offset": 0, "end_offset": 4, "label": "dru" }, { "id": 1, "entity": "垂体前叶", "start_offset": 7, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "垂体", "start_offset": 14, "end_offset": 16, "label": "bod" }, { "id": 3, "entity": "LH", "start_offset": 18, "end_offset": 20, "label": "bod" }, { "id": 4, "entity": "FSH", "start_offset": 21, "end_offset": 24, "label": "bod" }, { "id": 5, "entity": "LH", "start_offset": 29, "end_offset": 31, "label": "bod" }, { "id": 6, "entity": "HCG", "start_offset": 34, "end_offset": 37, "label": "bod" } ]
LHRH的优点是已可采用鼻黏膜喷雾给药,每侧鼻孔200μg,每天3次,每天总量1.2mg,连续28天,鼻LHRH治疗药无任何痛苦,即使感冒流涕仍可继续治疗。
[ { "id": 0, "entity": "LHRH", "start_offset": 0, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "鼻黏膜", "start_offset": 12, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "每侧鼻孔200μg,每天3次,每天总量1.2mg,连续28天", "start_offset": 20, "end_offset": 50, "label": "pro" }, { "id": 3, "entity": "LHRH治疗", "start_offset": 52, "end_offset": 58, "label": "pro" } ]
对经术前应用LHRH治疗,睾丸未能下降的隐睾进行活检,结果显示其组织学表现较之未接受激素治疗者有明显改善。
[ { "id": 0, "entity": "睾丸", "start_offset": 13, "end_offset": 15, "label": "bod" }, { "id": 1, "entity": "隐睾", "start_offset": 20, "end_offset": 22, "label": "dis" } ]
总之,激素治疗隐睾,由于目前对隐睾尚无统一的分类,疗效也缺乏统一客观的评价标准,各报告者之间有很大差异。
[ { "id": 0, "entity": "隐睾", "start_offset": 7, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "隐睾", "start_offset": 15, "end_offset": 17, "label": "dis" } ]
腹内隐睾的激素治疗几乎无效。
[ { "id": 0, "entity": "隐睾", "start_offset": 2, "end_offset": 4, "label": "dis" } ]
我们也碰到HCG注射时睾丸下降,注射一结束,睾丸又回缩的情况。
[ { "id": 0, "entity": "HCG注射", "start_offset": 5, "end_offset": 10, "label": "pro" } ]
2.手术治疗隐睾经确诊后均可接受手术治疗,手术应在2岁之前进行,为睾丸下降固定术。
[ { "id": 0, "entity": "隐睾", "start_offset": 6, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "手术治疗", "start_offset": 16, "end_offset": 20, "label": "pro" }, { "id": 2, "entity": "睾丸下降固定术", "start_offset": 33, "end_offset": 40, "label": "pro" } ]
一般可在腹股沟管及其附近找到睾丸,在结扎鞘状突后充分游离精索,使其能无张力地降入阴囊,固定于阴囊肉膜外。
[ { "id": 0, "entity": "腹股沟管", "start_offset": 4, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "睾丸", "start_offset": 14, "end_offset": 16, "label": "bod" }, { "id": 2, "entity": "结扎鞘状突后充分游离精索", "start_offset": 18, "end_offset": 30, "label": "pro" }, { "id": 3, "entity": "阴囊", "start_offset": 40, "end_offset": 42, "label": "bod" }, { "id": 4, "entity": "阴囊肉膜", "start_offset": 46, "end_offset": 50, "label": "bod" } ]
如在腹股沟管附近未能发现睾丸,可切开鞘状突,于腹膜腔内探查。
[ { "id": 0, "entity": "腹股沟管", "start_offset": 2, "end_offset": 6, "label": "bod" } ]
或于内环部切开腹内斜肌和腹横肌2~3cm,于腹膜后去探查输精管和睾丸。
[ { "id": 0, "entity": "腹横肌", "start_offset": 12, "end_offset": 15, "label": "bod" }, { "id": 1, "entity": "腹膜", "start_offset": 22, "end_offset": 24, "label": "bod" }, { "id": 2, "entity": "输精管", "start_offset": 28, "end_offset": 31, "label": "bod" }, { "id": 3, "entity": "睾丸", "start_offset": 32, "end_offset": 34, "label": "bod" } ]
如经上述步骤仍未发现睾丸,在做出无睾的诊断前一定要明确看到精索,并其末端为盲端。
[ { "id": 0, "entity": "睾丸", "start_offset": 10, "end_offset": 12, "label": "bod" }, { "id": 1, "entity": "精索", "start_offset": 29, "end_offset": 31, "label": "bod" }, { "id": 2, "entity": "盲端", "start_offset": 37, "end_offset": 39, "label": "bod" } ]
广泛游离精索时,要保护好睾丸动、静脉,以避免睾丸缺血萎缩。
[ { "id": 0, "entity": "精索", "start_offset": 4, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "睾丸动、静脉", "start_offset": 12, "end_offset": 18, "label": "bod" }, { "id": 2, "entity": "睾丸", "start_offset": 22, "end_offset": 24, "label": "bod" } ]
如果对输精管周围组织做过多剥离,则可能引起输精管蠕动障碍而失去输送精子的能力。
[ { "id": 0, "entity": "输精管", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "输精管蠕动障碍而失去输送精子的能力", "start_offset": 21, "end_offset": 38, "label": "sym" } ]
精索经广泛游离后,几乎都能将睾丸无张力地置入阴囊。
[ { "id": 0, "entity": "精索", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "睾丸", "start_offset": 14, "end_offset": 16, "label": "bod" }, { "id": 2, "entity": "阴囊", "start_offset": 22, "end_offset": 24, "label": "bod" } ]
少数病例虽经广泛游离,精索长度仍不足以将睾丸无张力放入阴囊,可先将睾丸固定于当前所能达到的最低位置,半年至一年后再次手术,期间可应用HCG,绝大多数病例可将睾丸放入阴囊。
[ { "id": 0, "entity": "精索", "start_offset": 11, "end_offset": 13, "label": "bod" }, { "id": 1, "entity": "睾丸", "start_offset": 20, "end_offset": 22, "label": "bod" }, { "id": 2, "entity": "阴囊", "start_offset": 27, "end_offset": 29, "label": "bod" }, { "id": 3, "entity": "睾丸", "start_offset": 33, "end_offset": 35, "label": "bod" }, { "id": 4, "entity": "HCG", "start_offset": 66, "end_offset": 69, "label": "dru" }, { "id": 5, "entity": "睾丸", "start_offset": 78, "end_offset": 80, "label": "bod" }, { "id": 6, "entity": "阴囊", "start_offset": 82, "end_offset": 84, "label": "bod" } ]
另一可考虑的手术是Fowler-Stephens术,在尽可能高位切断精索血管,使高位隐睾能一次性降入阴囊,但保留输精管与精索血管间系膜样结构,通过侧支血供来供应睾丸隐睾,在腹股沟管内未能找到睾丸,但如发现有精索盲端,则提示已无睾丸,不必再作广泛探查。
[ { "id": 0, "entity": "Fowler-Stephens术", "start_offset": 9, "end_offset": 25, "label": "pro" }, { "id": 1, "entity": "尽可能高位切断精索血管,使高位隐睾能一次性降入阴囊", "start_offset": 27, "end_offset": 52, "label": "pro" }, { "id": 2, "entity": "输精管", "start_offset": 56, "end_offset": 59, "label": "bod" }, { "id": 3, "entity": "精索血管", "start_offset": 60, "end_offset": 64, "label": "bod" }, { "id": 4, "entity": "隐睾", "start_offset": 82, "end_offset": 84, "label": "dis" }, { "id": 5, "entity": "腹股沟管", "start_offset": 86, "end_offset": 90, "label": "bod" }, { "id": 6, "entity": "睾丸", "start_offset": 95, "end_offset": 97, "label": "bod" }, { "id": 7, "entity": "精索盲端", "start_offset": 103, "end_offset": 107, "label": "bod" }, { "id": 8, "entity": "睾丸", "start_offset": 113, "end_offset": 115, "label": "bod" } ]
如果只发现盲端输精管或附睾,应考虑输精管、附睾可能与睾丸完全分离,必须继续在腹膜后探查,直至睾丸原始发育的部位。
[ { "id": 0, "entity": "盲端输精管", "start_offset": 5, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "附睾", "start_offset": 11, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "输精管", "start_offset": 17, "end_offset": 20, "label": "bod" }, { "id": 3, "entity": "附睾", "start_offset": 21, "end_offset": 23, "label": "bod" }, { "id": 4, "entity": "睾丸", "start_offset": 26, "end_offset": 28, "label": "bod" }, { "id": 5, "entity": "腹膜", "start_offset": 38, "end_offset": 40, "label": "bod" }, { "id": 6, "entity": "睾丸", "start_offset": 46, "end_offset": 48, "label": "bod" } ]
睾丸原始发育虽为腹膜后器官,但不少高位隐睾在腹膜腔内,精索周围常有腹膜包裹,形成系膜,在探查时应加以注意。
[ { "id": 0, "entity": "睾丸", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "腹膜", "start_offset": 8, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "腹膜腔", "start_offset": 22, "end_offset": 25, "label": "bod" }, { "id": 3, "entity": "精索", "start_offset": 27, "end_offset": 29, "label": "bod" } ]
三、心脏内部结构的演变一个简单的心管发育成为一个复杂的心脏。
[ { "id": 0, "entity": "心脏", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "心管", "start_offset": 17, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "心脏", "start_offset": 28, "end_offset": 30, "label": "bod" } ]
这一过程体现了由低等动物的心脏到高等动物心脏的种系发展过程。
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心脏胚胎成分与解剖结构关系见表9-9。
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表9-1心脏胚胎成分与解剖结构关系(一)房间隔的形成当胚胎第四周时,心房身体正中线背侧,右肺静脉的右侧脊向房室交界处延伸成为第一房间隔(原发隔,Ⅰ),将心房分为左右两半。
[ { "id": 0, "entity": "心脏胚胎", "start_offset": 4, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "房间隔", "start_offset": 20, "end_offset": 23, "label": "bod" }, { "id": 2, "entity": "心房身体正中线背侧", "start_offset": 34, "end_offset": 43, "label": "bod" }, { "id": 3, "entity": "右肺静脉", "start_offset": 44, "end_offset": 48, "label": "bod" }, { "id": 4, "entity": "右侧脊向房室交界处", "start_offset": 49, "end_offset": 58, "label": "bod" }, { "id": 5, "entity": "第一房间隔", "start_offset": 62, "end_offset": 67, "label": "bod" }, { "id": 6, "entity": "原发隔", "start_offset": 68, "end_offset": 71, "label": "bod" }, { "id": 7, "entity": "心房", "start_offset": 76, "end_offset": 78, "label": "bod" } ]
第一房间隔近内膜处留一镰状孔道即为第一房室孔(原发孔),胚胎在心房水平的血流可由此自右向左通过。
[ { "id": 0, "entity": "第一房间隔", "start_offset": 0, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "内膜", "start_offset": 6, "end_offset": 8, "label": "bod" }, { "id": 2, "entity": "第一房室孔", "start_offset": 17, "end_offset": 22, "label": "bod" }, { "id": 3, "entity": "原发孔", "start_offset": 23, "end_offset": 26, "label": "bod" }, { "id": 4, "entity": "心房", "start_offset": 31, "end_offset": 33, "label": "bod" } ]
第一孔因第一隔下缘与心内膜垫融合而逐渐闭合,与此同时第一隔的后上方(头端)局部组织逐渐被吸收成为筛孔状孔道,此为第二孔,或继发孔。
[ { "id": 0, "entity": "第一孔", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "第一隔", "start_offset": 4, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "心内膜垫", "start_offset": 10, "end_offset": 14, "label": "bod" }, { "id": 3, "entity": "第一隔", "start_offset": 26, "end_offset": 29, "label": "bod" }, { "id": 4, "entity": "第二孔", "start_offset": 56, "end_offset": 59, "label": "bod" }, { "id": 5, "entity": "继发孔", "start_offset": 61, "end_offset": 64, "label": "bod" } ]
约在胚胎第7周时,于第一隔的右侧又发生一片状隔膜,此为第二隔(继发隔,Ⅱ)。
[ { "id": 0, "entity": "第二隔", "start_offset": 27, "end_offset": 30, "label": "bod" }, { "id": 1, "entity": "继发隔", "start_offset": 31, "end_offset": 34, "label": "bod" } ]
第二隔朝向房室管方向生长,但中途停止,因此与房室管间留有卵圆形通道,此为卵圆孔。
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此时第一隔上的第二孔为第二隔所遮盖,第二隔上的卵圆孔则为第一隔所遮盖,此时卵圆孔成为卵圆窝。
[ { "id": 0, "entity": "第一隔", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "第二孔", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "第二隔", "start_offset": 11, "end_offset": 14, "label": "bod" }, { "id": 3, "entity": "第二隔", "start_offset": 18, "end_offset": 21, "label": "bod" }, { "id": 4, "entity": "第一隔", "start_offset": 28, "end_offset": 31, "label": "bod" }, { "id": 5, "entity": "卵圆窝", "start_offset": 42, "end_offset": 45, "label": "bod" } ]
胚胎时肺组织未膨胀,肺循环阻力大,右心房压力高于左心房压力。
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右心房血流可冲开卵圆窝底部的第一隔,斜经第二孔入左心房。
[ { "id": 0, "entity": "右心房", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "卵圆窝", "start_offset": 8, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "第一隔", "start_offset": 14, "end_offset": 17, "label": "bod" }, { "id": 3, "entity": "第二孔", "start_offset": 20, "end_offset": 23, "label": "bod" }, { "id": 4, "entity": "左心房", "start_offset": 24, "end_offset": 27, "label": "bod" } ]
而出生后两肺开始呼吸,肺循环开放,肺动脉压力下降,右心房压力低于左心房,第一隔受左心房压力作用紧贴于卵圆孔上,左右心房间的分流现象即告终止,此即卵圆孔的功能性闭合。
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卵圆孔的功能性闭合早于解剖闭合,胎儿出生后4~12月内解剖闭合才逐渐形成,而成年人中尚有25%~30%未实现解剖闭合,仍可探测到存在自左向右的血流通过,而此种情况不作房间隔缺损论。
[ { "id": 0, "entity": "卵圆孔", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "房间隔", "start_offset": 83, "end_offset": 86, "label": "bod" } ]
房间隔的发育异常:第一孔未及时闭合即为房间隔缺损的原发孔型,如第二孔未闭则为房间隔缺损的继发孔型。
[ { "id": 0, "entity": "房间隔", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "第一孔", "start_offset": 9, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "房间隔", "start_offset": 19, "end_offset": 22, "label": "bod" }, { "id": 3, "entity": "第二孔", "start_offset": 31, "end_offset": 34, "label": "bod" }, { "id": 4, "entity": "房间隔", "start_offset": 38, "end_offset": 41, "label": "bod" } ]
房间孔形成时,孔中有残余组织未被吸收,在房间孔上形成网状结构,此即Charis网。
[ { "id": 0, "entity": "房间孔", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "组织", "start_offset": 12, "end_offset": 14, "label": "bod" }, { "id": 2, "entity": "房间孔", "start_offset": 20, "end_offset": 23, "label": "bod" }, { "id": 3, "entity": "Charis网", "start_offset": 33, "end_offset": 40, "label": "bod" } ]
部分房间隔缺损的病例中可有Charis网的存在。
[ { "id": 0, "entity": "房间隔", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "Charis网", "start_offset": 13, "end_offset": 20, "label": "bod" } ]
覆盖卵圆窝的第一房间隔组织松弛,呈袋囊状向右房膨出,此一现象称为房隔瘤。
[ { "id": 0, "entity": "卵圆窝", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "第一房间隔组织", "start_offset": 6, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "右房", "start_offset": 21, "end_offset": 23, "label": "bod" }, { "id": 3, "entity": "呈袋囊状向右房膨出", "start_offset": 16, "end_offset": 25, "label": "sym" }, { "id": 4, "entity": "房隔瘤", "start_offset": 32, "end_offset": 35, "label": "sym" } ]
(二)心内膜垫的胚胎发育原始心管的心室和心房两个膨大之间为一凹陷的共同房室环,房室环中有三层组织所构成,其外层为心外膜,中间为心肌层,内层为心内膜。
[ { "id": 0, "entity": "心内膜垫", "start_offset": 3, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "心管", "start_offset": 14, "end_offset": 16, "label": "bod" }, { "id": 2, "entity": "心室", "start_offset": 17, "end_offset": 19, "label": "bod" }, { "id": 3, "entity": "心房", "start_offset": 20, "end_offset": 22, "label": "bod" }, { "id": 4, "entity": "房室环", "start_offset": 35, "end_offset": 38, "label": "bod" }, { "id": 5, "entity": "房室环", "start_offset": 39, "end_offset": 42, "label": "bod" }, { "id": 6, "entity": "心外膜", "start_offset": 56, "end_offset": 59, "label": "bod" }, { "id": 7, "entity": "心肌层", "start_offset": 63, "end_offset": 66, "label": "bod" }, { "id": 8, "entity": "心内膜", "start_offset": 70, "end_offset": 73, "label": "bod" } ]
共同房室环构成房室共同管。
[ { "id": 0, "entity": "房室环", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "房室共同", "start_offset": 7, "end_offset": 11, "label": "bod" } ]
胚胎第4周末(28天左右)房室共同管的心内膜增生,形成突起形成心内膜垫。
[ { "id": 0, "entity": "共同管", "start_offset": 15, "end_offset": 18, "label": "bod" }, { "id": 1, "entity": "心内膜", "start_offset": 19, "end_offset": 22, "label": "bod" }, { "id": 2, "entity": "心内膜垫", "start_offset": 31, "end_offset": 35, "label": "bod" } ]
此时心内膜与心外膜直接融合。
[ { "id": 0, "entity": "心内膜", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "心外膜", "start_offset": 6, "end_offset": 9, "label": "bod" } ]
共同房室管内共有6个内膜垫:背侧左、右心内膜垫,腹侧左、右心内膜垫,左侧内膜垫与右侧内膜垫。
[ { "id": 0, "entity": "共同房室管", "start_offset": 0, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "内膜垫", "start_offset": 10, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "右心内膜垫", "start_offset": 18, "end_offset": 23, "label": "bod" }, { "id": 3, "entity": "右心内膜垫", "start_offset": 28, "end_offset": 33, "label": "bod" }, { "id": 4, "entity": "右侧内膜垫", "start_offset": 40, "end_offset": 45, "label": "bod" } ]
1.心内膜垫与房室孔、房室分隔的形成胚胎6周时,房室环的背侧内膜垫与腹侧内膜垫相互融合成为房室环的中间间隔而出现房室分隔,同时将房室管分为左右两半形成左、右房室孔。
[ { "id": 0, "entity": "心内膜垫", "start_offset": 2, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "房室孔", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "房室", "start_offset": 11, "end_offset": 13, "label": "bod" }, { "id": 3, "entity": "房室环", "start_offset": 24, "end_offset": 27, "label": "bod" }, { "id": 4, "entity": "内膜垫", "start_offset": 30, "end_offset": 33, "label": "bod" }, { "id": 5, "entity": "内膜垫", "start_offset": 36, "end_offset": 39, "label": "bod" }, { "id": 6, "entity": "房室环", "start_offset": 45, "end_offset": 48, "label": "bod" }, { "id": 7, "entity": "房室管", "start_offset": 64, "end_offset": 67, "label": "bod" }, { "id": 8, "entity": "左、右房室孔", "start_offset": 75, "end_offset": 81, "label": "bod" } ]
两者的内界为中间间隔,外界为房室环的左右侧壁。
[ { "id": 0, "entity": "房室环的左右侧壁", "start_offset": 14, "end_offset": 22, "label": "bod" } ]
2.心内膜垫与房室瓣的形成三尖瓣主要由右侧的腹、背侧及右侧三个心内膜垫及圆锥隔所组成。
[ { "id": 0, "entity": "心内膜垫", "start_offset": 2, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "房室瓣", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "三尖瓣", "start_offset": 13, "end_offset": 16, "label": "bod" }, { "id": 3, "entity": "心内膜垫", "start_offset": 31, "end_offset": 35, "label": "bod" }, { "id": 4, "entity": "圆锥隔", "start_offset": 36, "end_offset": 39, "label": "bod" } ]
其中隔瓣由右腹、背侧心内膜垫及右侧心内膜垫底一部分组成;后瓣由右侧心内膜垫组成;前瓣则大部分由圆锥隔组成。
[ { "id": 0, "entity": "隔瓣", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "心内膜垫", "start_offset": 10, "end_offset": 14, "label": "bod" }, { "id": 2, "entity": "心内膜垫底", "start_offset": 17, "end_offset": 22, "label": "bod" }, { "id": 3, "entity": "后瓣", "start_offset": 28, "end_offset": 30, "label": "bod" }, { "id": 4, "entity": "心内膜垫", "start_offset": 33, "end_offset": 37, "label": "bod" }, { "id": 5, "entity": "前瓣", "start_offset": 40, "end_offset": 42, "label": "bod" }, { "id": 6, "entity": "圆锥隔", "start_offset": 47, "end_offset": 50, "label": "bod" } ]
二尖瓣均由左侧的三个内膜垫所组成。
[ { "id": 0, "entity": "二尖瓣", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "内膜垫", "start_offset": 10, "end_offset": 13, "label": "bod" } ]
前瓣的前外部与后内部分别由腹侧垫和背侧垫所组成;后瓣由背侧垫组成;而后内侧联合及前外联合分别由左侧垫与背侧垫、腹侧垫共同组成。
[ { "id": 0, "entity": "前瓣", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "腹侧垫", "start_offset": 13, "end_offset": 16, "label": "bod" }, { "id": 2, "entity": "背侧垫", "start_offset": 17, "end_offset": 20, "label": "bod" }, { "id": 3, "entity": "后瓣", "start_offset": 24, "end_offset": 26, "label": "bod" }, { "id": 4, "entity": "背侧垫", "start_offset": 27, "end_offset": 30, "label": "bod" }, { "id": 5, "entity": "左侧垫", "start_offset": 47, "end_offset": 50, "label": "bod" }, { "id": 6, "entity": "背侧垫", "start_offset": 51, "end_offset": 54, "label": "bod" }, { "id": 7, "entity": "腹侧垫", "start_offset": 55, "end_offset": 58, "label": "bod" } ]
当左侧的腹背内膜垫融合不全可出现二尖瓣前叶裂,而当腹侧和背侧的左右心内膜垫相互融合时即形成共同房室瓣。
[ { "id": 0, "entity": "内膜垫", "start_offset": 6, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "二尖瓣", "start_offset": 16, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "二尖瓣前叶裂", "start_offset": 16, "end_offset": 22, "label": "sym" }, { "id": 3, "entity": "心内膜垫", "start_offset": 33, "end_offset": 37, "label": "bod" }, { "id": 4, "entity": "房室瓣", "start_offset": 47, "end_offset": 50, "label": "bod" } ]
3.室间隔的发育胚胎第4周心房内原发隔形成时,心室底部突出肌性的室间隔基胚并向房室管方向生长,形成室间隔的小梁部,使心室分成左右两半,但在其上缘与房室管下缘间形成半月形的室间孔,室间孔周围组织向心性生长形成室间隔光滑部。
[ { "id": 0, "entity": "室间隔", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "心房", "start_offset": 13, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "原发隔", "start_offset": 16, "end_offset": 19, "label": "bod" }, { "id": 3, "entity": "心室", "start_offset": 23, "end_offset": 25, "label": "bod" }, { "id": 4, "entity": "室间隔", "start_offset": 32, "end_offset": 35, "label": "bod" }, { "id": 5, "entity": "房室管", "start_offset": 39, "end_offset": 42, "label": "bod" }, { "id": 6, "entity": "室间隔", "start_offset": 49, "end_offset": 52, "label": "bod" }, { "id": 7, "entity": "心室", "start_offset": 58, "end_offset": 60, "label": "bod" }, { "id": 8, "entity": "房室管", "start_offset": 73, "end_offset": 76, "label": "bod" }, { "id": 9, "entity": "室间孔", "start_offset": 85, "end_offset": 88, "label": "bod" }, { "id": 10, "entity": "室间孔", "start_offset": 89, "end_offset": 92, "label": "bod" }, { "id": 11, "entity": "室间隔光滑部", "start_offset": 103, "end_offset": 109, "label": "bod" } ]
至胚胎第7周时室间隔上缘的结缔组织、漏斗部及心内膜垫融合成膜部室间隔使室间孔完全闭合,室间隔分隔完成。
[ { "id": 0, "entity": "结缔组织", "start_offset": 13, "end_offset": 17, "label": "bod" }, { "id": 1, "entity": "漏斗部", "start_offset": 18, "end_offset": 21, "label": "bod" }, { "id": 2, "entity": "心内膜垫", "start_offset": 22, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "膜部室间隔", "start_offset": 29, "end_offset": 34, "label": "bod" }, { "id": 4, "entity": "室间孔", "start_offset": 35, "end_offset": 38, "label": "bod" }, { "id": 5, "entity": "室间隔", "start_offset": 43, "end_offset": 46, "label": "bod" } ]
室间隔发育过程中任何部分出现异常即可出现室间隔缺损,其中以室间隔膜周部缺损最常见。
[ { "id": 0, "entity": "室间隔", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "室间隔", "start_offset": 20, "end_offset": 23, "label": "bod" }, { "id": 2, "entity": "室间隔膜", "start_offset": 29, "end_offset": 33, "label": "bod" } ]
4.漏斗部的形成胚胎第3周时,心球膨大向右扭转并向尾端移行,至胚胎第4周时心球与心室均移至心房的尾端,心球此时分成头端与尾端两个部分,尾端发育成右心室的流入道,头端发育成流出道或称漏斗部。
[ { "id": 0, "entity": "漏斗部", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "心球", "start_offset": 15, "end_offset": 17, "label": "bod" }, { "id": 2, "entity": "心球", "start_offset": 37, "end_offset": 39, "label": "bod" }, { "id": 3, "entity": "心室", "start_offset": 40, "end_offset": 42, "label": "bod" }, { "id": 4, "entity": "心房", "start_offset": 45, "end_offset": 47, "label": "bod" }, { "id": 5, "entity": "心球", "start_offset": 51, "end_offset": 53, "label": "bod" }, { "id": 6, "entity": "右心室的流入道", "start_offset": 72, "end_offset": 79, "label": "bod" }, { "id": 7, "entity": "流出道", "start_offset": 85, "end_offset": 88, "label": "bod" }, { "id": 8, "entity": "称漏斗部", "start_offset": 89, "end_offset": 93, "label": "bod" } ]
此时的漏斗部为左右心室的共同流出道。
[ { "id": 0, "entity": "漏斗部", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "左右心室", "start_offset": 7, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "共同流出道", "start_offset": 12, "end_offset": 17, "label": "bod" } ]
此后,在心球头尾端之间的腹背侧分别向心形成突起成为腹侧及背侧心球嵴并逐渐相互融合,最后与心内膜垫及心室间隔融合而将漏斗部分成左右两半,其中右侧发育成肺动脉漏斗部,与右心室相通;左侧发育成主动脉漏斗部,与左心室相通,但此后主动脉漏斗部被逐渐吸收,正常情况下不形成主动脉下圆锥。
[ { "id": 0, "entity": "心球", "start_offset": 4, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "心球嵴", "start_offset": 30, "end_offset": 33, "label": "bod" }, { "id": 2, "entity": "心内膜垫", "start_offset": 44, "end_offset": 48, "label": "bod" }, { "id": 3, "entity": "心室间隔", "start_offset": 49, "end_offset": 53, "label": "bod" }, { "id": 4, "entity": "漏斗", "start_offset": 57, "end_offset": 59, "label": "bod" }, { "id": 5, "entity": "肺动脉漏斗部", "start_offset": 74, "end_offset": 80, "label": "bod" }, { "id": 6, "entity": "右心室", "start_offset": 82, "end_offset": 85, "label": "bod" }, { "id": 7, "entity": "主动脉漏斗部", "start_offset": 93, "end_offset": 99, "label": "bod" }, { "id": 8, "entity": "左心室", "start_offset": 101, "end_offset": 104, "label": "bod" }, { "id": 9, "entity": "主动脉漏斗部", "start_offset": 110, "end_offset": 116, "label": "bod" }, { "id": 10, "entity": "主动脉", "start_offset": 130, "end_offset": 133, "label": "bod" } ]