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膀胱外翻、尿道上裂以及泄殖腔和泌尿生殖窦异常。

Exstrophy, epispadias, and cloacal and urogenital sinus abnormalities.

作者信息

Jeffs R D

机构信息

Division of Pediatric Urology, James Buchanan Brady Urological Institute, Johns Hopkins Hospital, Baltimore, Maryland.

出版信息

Pediatr Clin North Am. 1987 Oct;34(5):1233-57. doi: 10.1016/s0031-3955(16)36328-3.

Abstract

The congenital defects in the pelvis and perineal area, which are derived from defective development of the cloacal membrane, the urorectal septum, the mesonephric and paramesonephric (müllerian) ducts and the urogenital sinus, have been described. These may be life threatening, are often complex, and are certainly devastating to the parents. With knowledge of their embryologic origin, of their anatomy in detail, and the effect on function and drainage, treatment can be planned so that a good quality of life usually can be expected. Improved reconstructive programs and techniques can usually achieve near-normal appearance and function, with the expectation of good social adjustment.

摘要

骨盆与会阴区域的先天性缺陷源自泄殖腔膜、尿直肠隔、中肾管和副中肾(苗勒)管以及泌尿生殖窦的发育缺陷,相关情况已有描述。这些缺陷可能危及生命,通常较为复杂,对患儿父母而言无疑是沉重打击。了解其胚胎学起源、详细解剖结构以及对功能和引流的影响后,便可制定治疗方案,有望实现较高的生活质量。改进后的重建方案和技术通常能够实现近乎正常的外观和功能,预期患儿能较好地融入社会。

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