胎儿肢体-体壁综合征一例并文献复习

doi:10.12280/gjfckx.20230370

摘要/Abstract

摘要：

肢体-体壁综合征（limb body wall complex，LBWC）是一种复杂的胎儿多发畸形，LBWC预后很差，超声医师的早期发现对临床及时处理、终止妊娠至关重要。报告1例32岁女性患者，孕23⁺⁴周在我院行胎儿系统超声筛查发现胎儿脊柱骶尾部弯曲成角，腹壁肌层及皮肤连续性中断，腹腔内脏器向外膨出，腹腔内可见深约9 mm液性暗区，双足均呈内翻姿势，左足显示六趾，脐带过短，长约49 mm，胚外体腔似可见。超声诊断考虑LBWC可能。引产后临床诊断为LBWC。通过报道该病例并结合文献复习对LBWC进行分析介绍，提高基层医生对本病的认识。

关键词: 超声检查，产前, 畸形，多发性, 胚胎发育, 病例报告, 肢体-体壁综合征

Abstract:

Limb body wall complex (LBWC) is a complex multiple fetal malformation. The prognosis of LBWC is very poor. Early detection by ultrasound doctor is crucial for timely clinical management and termination of pregnancy. A 32-year-old female patient, 23 weeks plus 4 days gestation, underwent fetal ultrasound screening in our hospital. It was found that the fetal spine was curved at the sacrococcygeal region, the abdominal wall muscle layer and the skin continuity were interrupted, the abdominal organs were bulked out, a dark liquid area about 9 mm depth was visible in the abdominal cavity, both feet were in the position of pronation, the left foot showed 6 toes, the umbilical cord was too short, about 49 mm in length, and the extrembryonic body cavity was visible. LBWC may be considered for ultrasound diagnosis. The clinical diagnosis was LBWC after induction of labor. By reporting the case and reviewing the literature, LBWC was analyzed and introduced, so as to improve the basic doctors′ understanding of the disease.

Key words: Ultrasonography, prenatal, Abnormalities, multiple, Embryonic development, Case reports, Limb body wall complex

胡梦双, 梅靖. 胎儿肢体-体壁综合征一例并文献复习[J]. 国际妇产科学杂志, 2023, 50(5): 519-522.

HU Meng-shuang, MEI Jing. Fetal Limb Body Wall Complex：A Case Report and Literature Review[J]. Journal of International Obstetrics and Gynecology, 2023, 50(5): 519-522.

图/表 5

图1 胎儿脊柱二维超声图注：上图见胎儿脊柱排列异常、弯曲、成角。下图箭头示胎儿骶尾部一稍高回声团向椎管外膨出。

图2 胎儿脊柱三维超声图注：胎儿脊柱侧弯，箭头所示为侧弯返折处。

图3 胎儿腹部二维超声图注：上图见胎儿腹部脏器膨出，腹壁缺损。下图胎儿腹部横断面见腹腔积液，腹部膨出部分与胎盘、羊膜分界不清。

图4 胎儿左足二维超声图注：胎儿左足六趾畸形，足底与小腿在同一切面。

图5 引产后标本图注：左图见胎儿脊柱侧弯，骶尾部皮肤完整。中间图见胎儿腹裂并腹部膨出，膨出物可见肝脏、胃泡、肠管等。右图见胎儿双足内翻，左足六趾畸形。

参考文献 8

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