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《伴有孤立异位肠段的外胚层-泄殖腔畸形综合征:病例系列及文献复习》

Exstrophy-Epispadias Complex With Isolated Ectopic Bowel Segment: A Case Series and Literature Review.

机构信息

Department of Urology, Shanghai Children's Hospital, School of medicine, Shanghai Jiao Tong University, Shanghai, China.

Department of Urology, Shanghai Children's Hospital, School of medicine, Shanghai Jiao Tong University, Shanghai, China.

出版信息

Urology. 2022 Dec;170:179-183. doi: 10.1016/j.urology.2022.08.003. Epub 2022 Aug 13.

Abstract

OBJECTIVE

To present a case series of the exstrophy-epispadias complex (EEC) with isolated ectopic bowel segment (IEBS) with the literature review, highlighting the clinical findings and treatments.

MATERIALS AND METHODS

We present 3 cases of bladder exstrophy (BE) with IEBS in our institute and reviewed the literature in PubMed with the terms "("bladder exstrophy" OR "epispadias") AND ("visceral sequestration" OR "sequestered" OR "ectopic bowel")."

RESULTS

There were 2 males and 1 female. The IEBS was detected by physical examination in 2 cases and by ultrasonography in another one. All cases were BE accompanying with lower abdominal mass which adhered to the bladder wall but was separated from the digestive system. All cases underwent the IEBS excision and BE repair simultaneously. Pathological result of IEBS suggested the histological structures of colon. There were totally 13 cases of EEC with IEBS reported in the literature, including 2 (15%) epispadias, 9 (69%) covered BE, 1 (8%) duplicate BE and 1 (8%) classic bladder exstrophy. Although their clinical manifestations were various, IEBS excision were safely conducted in all cases.

CONCLUSION

EEC with IEBS is an extremely rare congenital malformation. Physical and imaging examinations are important for diagnoses. Surgical excision is safe and effective for managing IEBS.

摘要

目的

通过文献复习,介绍一组伴有孤立异位肠段(IEBS)的膀胱外翻-尿道上裂复合畸形(EEC)病例系列,重点介绍其临床发现和治疗方法。

材料和方法

我们在本院介绍了 3 例伴有 IEBS 的膀胱外翻病例,并在 PubMed 上使用“(膀胱外翻或尿道上裂)和(内脏隔离或隔离或异位肠)”的术语检索文献。

结果

有 2 例为男性,1 例为女性。2 例通过体格检查,另 1 例通过超声检查发现 IEBS。所有病例均为 BE,伴下腹肿块,与膀胱壁粘连,但与消化系统分离。所有病例均同时行 IEBS 切除和 BE 修复。IEBS 的病理结果提示为结肠的组织学结构。文献共报道 13 例 EEC 伴 IEBS,包括 2 例(15%)尿道上裂,9 例(69%)覆盖性 BE,1 例(8%)双重 BE 和 1 例(8%)经典膀胱外翻。尽管临床表现各异,但所有病例均安全地进行了 IEBS 切除。

结论

EEC 伴 IEBS 是一种极为罕见的先天性畸形。体格检查和影像学检查对于诊断很重要。手术切除是治疗 IEBS 的安全有效的方法。

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