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先天性短肠综合征:一种致命异常的产前超声检查结果

Congenital short-bowel syndrome: prenatal sonographic findings of a fatal anomaly.

作者信息

Aviram R, Erez I, Dolfin T Z, Katz S, Beyth Y, Tepper R

机构信息

Department of Obstetrics and Gynecology, Sapir Medical Center, Kfar Saba, Israel.

出版信息

J Clin Ultrasound. 1998 Feb;26(2):106-8. doi: 10.1002/(sici)1097-0096(199802)26:2<106::aid-jcu11>3.0.co;2-k.

DOI:10.1002/(sici)1097-0096(199802)26:2<106::aid-jcu11>3.0.co;2-k
PMID:9460641
Abstract

The sonographic findings in a fatal case of congenital short-bowel syndrome are reported. Sonography at 11 weeks of gestation showed a 11 x 6 mm hyperechoic mass interpreted to be a midgut umbilical hernia. A repeat scan 2 weeks later showed an intact anterior abdominal wall, no umbilical herniation, and appropriate fetal growth. Forty-eight hours after full-term, vaginal delivery, the infant began vomiting bile and passing blood rectally. Imaging studies showed distended bowel loops without air-fluid levels and incomplete bowel obstruction. Laparotomy showed malrotation and short small bowel without volvulus. The infant died at 9 weeks of age. When delayed return of the midgut to the abdominal cavity is noted on prenatal sonograms, follow-up sonograms should be done throughout the second trimester, especially in patients with a family history of short-bowel syndrome, to search for dilated short bowel loops. If such loops are found, patients should be given options for pregnancy termination.

摘要

报道了一例先天性短肠综合征致死病例的超声检查结果。妊娠11周时的超声检查显示一个11×6毫米的高回声团块,被解释为中肠脐疝。两周后复查显示前腹壁完整,无脐疝,胎儿生长正常。足月阴道分娩48小时后,婴儿开始呕吐胆汁并经直肠排血。影像学检查显示肠袢扩张但无气液平面,存在不完全性肠梗阻。剖腹探查显示肠旋转不良和小肠短小但无肠扭转。婴儿在9周龄时死亡。当产前超声检查发现中肠延迟回纳入腹腔时,应在整个孕中期进行超声随访,尤其是有短肠综合征家族史的患者,以寻找扩张的短肠袢。如果发现此类肠袢,应向患者提供终止妊娠的选择。

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引用本文的文献

1
Congenital short bowel syndrome: a case report and review of the literature.先天性短肠综合征:一例病例报告及文献综述
Can J Gastroenterol. 2008 Jan;22(1):71-4. doi: 10.1155/2008/590143.
2
Congenital short-gut syndrome.先天性短肠综合征
Pediatr Radiol. 2004 May;34(5):424-7. doi: 10.1007/s00247-003-1087-2. Epub 2003 Dec 16.