Johns Hopkins Hospital, Baltimore, MD, USA.
J Pediatr Surg. 2010 Jan;45(1):171-5; discussion 175-6. doi: 10.1016/j.jpedsurg.2009.10.030.
Cloacal exstrophy is a rare and complex congenital anomaly requiring coordination among multiple pediatric subspecialties. There is currently no consensus regarding the fate and function of the hindgut, which plays an integral role in patients' long-term gastrointestinal health and genitourinary reconstruction.
A retrospective chart review was performed evaluating 77 patients with cloacal exstrophy treated during the previous 44 years at our institution.
Seventy-seven patients with cloacal exstrophy were treated between 1965 and 2008. Sixty-five were white, 6 were African American, 3 were Asian, and 3 were Hispanic. Genotypes included 44 XY, 32 XX, and 1 XYY. Fifty-one were reared as females and 26 as males. The hindgut length was 2 to 5 cm in 11 patients, 6 to 10 cm in 18 patients, 11 to 15 cm in 6 patients, 16 to 20 cm in 7 patients, and greater than 20 cm in 2 patients. The hindgut length was unknown in 33 patients. Forty-seven patients had tubularization of the cecal plate with an end colostomy, and 30 patients had an ileostomy placed for bowel diversion purposes. Four patients had short gut syndrome. Thirty-one patients had genitourinary reconstruction, 12 using small bowel and 19 using colon. Eight patients had hindgut pull-through procedures.
Gastrointestinal ramifications of the cloacal exstrophy complex include the occurrence of short gut syndrome and significant fluid and electrolyte derangements in patients receiving an ileostomy for initial intestinal management. This has caused a paradigm shift of initial intestinal management to tubularization of the cecal plate with end colostomy placement. This shift has eliminated the occurrence of short gut syndrome and enabled patients to be candidates for intestinal pull-through procedure if these patients are able to form solid stool, have a reasonable degree of pelvic neuromuscular development, and are able to comply with a bowel management program.
泄殖腔外翻是一种罕见且复杂的先天性畸形，需要多个儿科亚专业的协调。目前对于后肠的命运和功能尚无共识，后肠在患者的长期胃肠道健康和泌尿生殖系统重建中起着至关重要的作用。
对本机构过去 44 年期间治疗的 77 例泄殖腔外翻患者进行回顾性图表分析。
77 例泄殖腔外翻患者于 1965 年至 2008 年接受治疗。77 例患者中 65 例为白人，6 例为非裔美国人，3 例为亚洲人，3 例为西班牙裔。基因型包括 44 XY、32 XX 和 1 XYY。51 例患者被养育为女性，26 例为男性。11 例患者的后肠长度为 2 至 5 厘米，18 例患者的后肠长度为 6 至 10 厘米，6 例患者的后肠长度为 11 至 15 厘米，7 例患者的后肠长度为 16 至 20 厘米，2 例患者的后肠长度大于 20 厘米。33 例患者的后肠长度未知。47 例患者行盲肠板成形术并进行末端结肠造口术，30 例患者行回肠造口术进行肠道转流。4 例患者患有短肠综合征。31 例患者进行了泌尿生殖系统重建，其中 12 例使用小肠，19 例使用结肠。8 例患者行后肠拖出术。
泄殖腔外翻患者的胃肠道并发症包括接受回肠造口术进行初始肠道管理的患者发生短肠综合征和显著的液体和电解质紊乱。这导致初始肠道管理发生转变，即采用盲肠板成形术并进行末端结肠造口术。这种转变消除了短肠综合征的发生，并使患者能够成为肠拖出术的候选者，如果这些患者能够形成固体粪便，具有合理程度的骨盆神经肌肉发育，并且能够遵守肠道管理方案。
