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腹裂畸形产前腹部缺损闭合:病例报告及文献综述

Prenatal closure of abdominal defect in gastroschisis: case report and review of the literature.

作者信息

Tawil A, Comstock C H, Chang Ch C H

机构信息

Department of Anatomic Pathology, William Beaumont Hospital, 3601 West Thirteen Mile Road, Royal Oak, MI 48073-6769, USA.

出版信息

Pediatr Dev Pathol. 2001 Nov-Dec;4(6):580-4. doi: 10.1007/s10024001-0081-7.

Abstract

With the routine use of fetal imaging studies during prenatal care, increased numbers of unusual intrauterine events are now detected. Prenatal closure of the abdominal defect in gastroschisis is an example. We report a 34 5/7-week stillborn who had prenatal closure of a ventral abdominal wall defect, which had been seen earlier on fetal ultrasound examination. Two ultrasound examinations performed at 15 1/7 weeks and 19 1/7 weeks showed a mass of exteriorized bowel that herniated through the abdominal defect, just to the right of the umbilical cord. At 30 1/7 weeks, no exteriorized bowel was seen, but thickened and dilated intraabdominal bowel was identified. No abdominal defect or exteriorized bowel was found at autopsy. There was a severely dilated proximal jejunum with the absence of the rest of the small intestine and the right side of the colon. The remaining left side of the colon was small and blind proximally. Six similar isolated examples have been reported since 1991. Prenatal closure of an abdominal defect was associated with long-segment atresia of the midintestine in each case. We believe that the spontaneous closure of this abdominal defect was associated with atresia and resorption of exteriorized bowel. It is likely some of the cases of long-segment atresia may in fact be associated with closed gastroschisis.

摘要

随着产前检查中胎儿影像研究的常规应用,现在检测到的异常宫内事件数量有所增加。腹裂腹壁缺损的产前闭合就是一个例子。我们报告一例孕34⁵/₇周的死产儿,其腹前壁缺损在产前已闭合,该缺损在早期胎儿超声检查中可见。在孕15¹/₇周和19¹/₇周进行的两次超声检查显示,一团外置肠管通过腹壁缺损疝出,位于脐带右侧。孕30¹/₇周时,未见外置肠管,但发现腹腔内肠管增厚、扩张。尸检未发现腹壁缺损或外置肠管。近端空肠严重扩张,其余小肠和结肠右侧缺失。结肠其余左侧短小,近端呈盲端。自1991年以来,已报告了6个类似的孤立病例。在每个病例中,腹壁缺损的产前闭合均与中段肠管的长段闭锁有关。我们认为,这种腹壁缺损的自发闭合与外置肠管的闭锁和吸收有关。很可能一些长段闭锁病例实际上可能与闭合性腹裂有关。

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