A case of split notochord syndrome with congenital ileal atresia, the total absence of a colon, and a dorsal enteric cyst communicating to the retroperitoneal isolated ceca with a vesical fistula
A case of split notochord syndrome with congenital ileal atresia, the total absence of a colon, and a dorsal enteric cyst communicating to the retroperitoneal isolated ceca with a vesical fistula
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DOI：
10.1007/s00383-008-2206-9
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发表时间：
2008-09-01
影响因子：
1.8
通讯作者：
Tanaka, Hiroaki
中科院分区：
文献类型：
--
作者：
Asagiri, Kimio;Yagi, Minoru;Tanaka, Hiroaki
脊索分裂综合征（SNS）是一种极其罕见的异常现象。本报告介绍了一名患有 SNS 的男性婴儿的病例，该婴儿患有先天性回肠闭锁和背侧肠囊肿，该囊肿与腹膜后孤立的盲肠相通，并伴有膀胱瘘。背侧瘘管造影和膀胱造影对于详细研究该患者的拓扑结构非常有用且必不可少。结合文献报道的19例病例，讨论其胚胎学机制和病因学理论。
Split notochord syndrome (SNS) is an extremely rare anomaly. This report presents the case of a male infant with SNS associated with congenital ileal atresia and a dorsal enteric cyst communicating to the retroperitoneal isolated ceca with a vesical fistula. Dorsal fistulography and vesicography were useful and essential for the detailed study of the topology in this patient. The embryological mechanism and etiologic theories are discussed with a review of 19 cases reported in the literature.