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一名31周死产儿的胸廓裂合并膈疝。

Thoracoschisis associated with diaphragmatic hernia in a 31-week-old stillbirth.

作者信息

Derbent M, Balci S

机构信息

Department of Pediatrics, Başkent University Faculty of Medicine, Ankara, Turkey.

出版信息

Turk J Pediatr. 2001 Jul-Sep;43(3):269-71.

Abstract

Thoracoschisis is a very rare congenital anomaly and is frequently found with other congenital defects the of limbs and the abdominal wall as a part of limb-body wall complex (LBWC). Early vascular disruption, amnion rupture and intrinsic embryonic maldevelopment are related to the pathogenesis of this complex. We present a case of thoracoschisis with ipsilateral diaphragmatic hernia. This case had none of the associated anomalies which are seen in LBWC. By ultrasonography performed at 31 weeks' gestation the fetus was misdiagnosed as gastroschisis. In this report we discuss the pathogenetic mechanism of LBWC and our case.

摘要

胸裂是一种非常罕见的先天性异常,常与肢体和腹壁的其他先天性缺陷同时出现,作为肢体-体壁复合体(LBWC)的一部分。早期血管破坏、羊膜破裂和内在胚胎发育异常与该复合体的发病机制有关。我们报告一例伴有同侧膈疝的胸裂病例。该病例没有LBWC中常见的相关异常。在妊娠31周时进行超声检查,该胎儿被误诊为腹裂。在本报告中,我们讨论了LBWC的发病机制及我们的病例。

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