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女性新生儿持续性泄殖腔的管理原则

Principles of management of the persistent cloaca in the female newborn.

作者信息

Kay R, Tank E S

出版信息

J Urol. 1977 Jan;117(1):102-4. doi: 10.1016/s0022-5347(17)58357-3.

DOI:10.1016/s0022-5347(17)58357-3
PMID:556633
Abstract

The persistent cloaca in the female newborn is one of the most complex and challenging developmental malformations that the pediatric urologist will encounter. A review of 5 patients seen at this center and 34 completely documented cases was undertaken to characterize the defects and devise a logical diagnostic and therapeutic approach. Diagnostic steps include the search for associated anomalies, which were present in the upper urinary tract (33 per cent), gastrointestinal tract (13 per cent), cardiovascular system (13 per cent), central nervous system (10 per cent) and respiratory tract (5 per cent). Delineation of the cloaca and its anatomic relationships is determined by abdominal x-rays, injection of contrast material into the cloacal channel, excretory urography and endoscopy with selective catheterization. Surgical treatment begins with a diverting right transverse colostomy in the newborn period followed by rectal pull-through when the patient weighs 25 pounds. Vaginal pull-through should be performed at the same time as the rectal pull-through if the vagina enters the cloaca high. If the cloacovaginal communication is low a vaginoplasty by a posterior flap procedure can be done when the child is 10 to 12 years old. Even is anatomy is reconstructed in an ideal manner urinary and fecal continence cannot be assured since congenital neurologic dysfunction is not uncommon.

摘要

女性新生儿的持续性泄殖腔是儿科泌尿科医生会遇到的最复杂且最具挑战性的发育畸形之一。对该中心诊治的5例患者以及34例有完整记录的病例进行了回顾,以明确缺陷并制定合理的诊断和治疗方法。诊断步骤包括查找相关异常,这些异常出现在上尿路(33%)、胃肠道(13%)、心血管系统(13%)、中枢神经系统(10%)和呼吸道(5%)。通过腹部X线、向泄殖腔通道注入造影剂、排泄性尿路造影以及选择性插管的内镜检查来确定泄殖腔及其解剖关系。手术治疗始于新生儿期行右侧横结肠造口术转流,待患者体重达25磅时再行直肠拖出术。如果阴道高位进入泄殖腔,则应在直肠拖出术的同时进行阴道拖出术。如果泄殖腔与阴道的通道位置低,可在患儿10至12岁时采用后皮瓣法进行阴道成形术。即使解剖结构以理想方式重建,由于先天性神经功能障碍并不罕见,仍无法确保尿便自控。

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