探讨对复杂型一穴肛患儿的规范诊断、重建方式及治疗疗效。

选择2012年至2013年广州市妇女儿童医疗中心收治的2例复杂型一穴肛患儿(患儿A、患儿B)的临床病历资料为研究对象。采用回顾性研究方法分析2例患儿不同的术前处理、手术重建方式及术后恢复情况。

本组2例一穴肛患儿均为女性，年龄分别为2.0及1.5岁。术前膀胱镜检查结果显示，共同通道长度分别为6.5和3.3 cm。患儿A合并双侧输尿管反流、马蹄肾，出生后半年出现阴道积液；患儿B合并左侧肾缺如和双侧输尿管反流。患儿A完全游离泌尿系及生殖系，子宫阴道完全游离后下移至外阴，共同通道成形大部分尿道，其余尿道为外阴皮肤修补延长，双侧输尿管再植。患儿B共同通道成形新尿道，末端直肠游离后替代阴道和残余子宫吻合，结肠成形肛门。患儿A术后出现会阴体轻度裂开，术后2周出现直肠黏膜外翻。对其术后留置膀胱造瘘管半年，经训练后可自行排尿，膀胱残余尿测定小于膀胱容积的10%，但反复出现尿路感染，大便无法控制，行Malone术给予顺行灌肠。患儿B术后1个月复查，无膀胱输尿管反流、尿道梗阻或狭窄症状，阴道周围无瘢痕样变化，自行排尿，无残余尿；结肠封瘘后可自行排便。

对复杂型一穴肛患儿应于术前进行膀胱镜检查结合影像学检查，明确结构畸形，准确测量共同通道长度，制定手术方案。复杂型一穴肛重建手术最关键的是尿道重建，共同通道是尿道重建的首选材料。对复杂型一穴肛的诊治需采取系统化、程序化及个体化措施，手术最终目标是达到控尿、控便功能及恢复性功能，甚至生育功能。

Many reports have concerned about simple cloaca (common channel < 3 cm) with less complications and total urogenital molization are performed in most cases. But as to complex cloaca (common channel >3 cm), due to its multiple, diverse domestic reconstruction surgery, it has been rarely reported. This article is to explore systemic clinical treatments, reconstruction procedures of complex cloaca.

The clinical data of two cases of complex cloaca (patient A and patient B) treated in Guangzhou Women and Children's Medical Center in 2012-2013 were collected. The different preoperative evaluation, different surgical reconstruction and postoperative recovery were analyzed.

The 2 patients, A and B, both were performed endoscopy exam to make sure the length of common channel, they were 6.5 cm and 3.3 cm respectively. For the definitive reconstruction, both patients in the series were managed with a posterior sagittal approach, and also required a laparotomy. Hydrocolpos was present in the patient A with common channel 6.5 cm, vaginal scopy was performed. Vaginal reconstruction involved a complete detach of vaginal and the vaginal pull-through in patients common channel 6.5 cm, and vaginal replacement (rectum) in patient common channel 3.3 cm. Patients B with common channel 3.3 cm had voluntary bowel movements, but not for the patients with common channel 6.5 cm. Malone procedure was performed on patients with common channel 6.5 cm and kept clean for 24 hours.

Cystoscopy is necessary for diagnosis of the common channel of patients with complex persist cloaca. Reconstruction of urethral tract is the most important of cloaca reconstruction and the common channel is first choice for the urethral tract reconstruction. The ultimate goal of complex persist cloaca treatment is to achieve continence, sexual function and even reproductive function.