Smith E A, Woodard J R, Broecker B H, Gosalbez R, Ricketts R R
Department of Surgery, Emory University School of Medicine, Egleston Children's Hospital, Atlanta, GA, USA.
J Pediatr Surg. 1997 Feb;32(2):256-61; discussion 261-2. doi: 10.1016/s0022-3468(97)90190-1.
Since 1980 the authors have treated 12 infants with cloacal exstrophy (10 classical and 2 variants). Eleven patients had repair, and are all surviving. The initial phases of management that led to improved survival have previously been reported. Quality of life is now a major focus for the cloacal exstrophy patient. During the past 10 years, nine of the 11 patients had lower urinary tract reconstructive procedures. This review evaluates experience with reconstructive efforts to achieve bowel and bladder control and to improve the quality of life in this complex group of patients.
Through review of patient charts and by patient interviews, data were collected to evaluate the ability to provide urinary and bowel control. A continence score was applied to provide a measure of success: voluntary control, 3; control with an enema program or intermittent catheterization, 2; incontinence with a well-functioning stoma, 1; and incontinence without a stoma, 0. The best continence score is 6 (genitourinary and gastrointestinal). Surgical complications, urodynamic and metabolic sequelae of continent urinary diversion were reviewed.
At the time of the authors' previous report, eight of 11 patients had a continence score of 2 or less. Currently, eight of 11 patients have a score of 3 or better (five with enteric stoma and continent urinary diversion, two with enema program and continent urinary diversion, and one with enema program and continent bladder). Urinary-diversion procedures have included two gastric augmentations and five gastric reservoirs, two of which have required subsequent bowel augmentation. Gastric augmentations carry a definite risk of metabolic problems with three of our patients demonstrating significant episodes of metabolic alkalosis. In addition, results of urodynamic monitoring suggests that gastric reservoirs may be less compliant than reservoirs formed using other bowel segments.
Modern principles of continent urinary diversion have been successfully applied to the cloacal exstrophy patient further improving their quality of life. Use of gastric flaps with preservation of intestinal length has been central to urologic reconstructive efforts. Use of stomach alone for formation of urinary reservoirs may produce suboptimal compliance, and composite ileogastric construction should be considered if the gastric flap is of marginal size.
自1980年以来，作者共治疗了12例泄殖腔外翻患儿（10例典型病例和2例变异型）。11例患者接受了修复手术，均存活。先前已报道了使存活率提高的初始治疗阶段。生活质量现在是泄殖腔外翻患者的主要关注点。在过去10年中，11例患者中有9例接受了下尿路重建手术。本综述评估了为实现肠道和膀胱控制以及改善这一复杂患者群体生活质量而进行重建努力的经验。
通过查阅患者病历并与患者访谈，收集数据以评估实现尿液和肠道控制的能力。应用控尿评分来衡量成功程度：自主控制为3分；通过灌肠方案或间歇性导尿实现控制为2分；造口功能良好但仍失禁为1分；无造口且失禁为0分。最佳控尿评分为6分（泌尿生殖系统和胃肠道）。回顾了可控性尿流改道术的手术并发症、尿动力学和代谢后遗症。
在作者先前的报告时，11例患者中有8例控尿评分为2分或更低。目前，11例患者中有8例评分达到3分或更高（5例采用肠道造口和可控性尿流改道术，2例采用灌肠方案和可控性尿流改道术，1例采用灌肠方案和可控性膀胱术）。尿流改道手术包括2例胃扩大术和5例胃储尿囊，其中2例随后需要肠道扩大术。胃扩大术有发生代谢问题的明确风险，我们的3例患者出现了明显的代谢性碱中毒发作。此外，尿动力学监测结果表明，胃储尿囊的顺应性可能低于使用其他肠段形成的储尿囊。
可控性尿流改道的现代原则已成功应用于泄殖腔外翻患者，进一步改善了他们的生活质量。保留肠管长度使用胃瓣一直是泌尿外科重建努力的核心。单独使用胃来形成尿储尿囊可能产生不理想的顺应性，如果胃瓣尺寸有限，应考虑采用复合回肠胃构建。
