Heap David, Haffar Ahmad, Crigger Chad B, Martheswaran Tanisha, Hirsch Alexander, Maxon Victoria, Sponseller Paul D, Di Carlo Heather N, Gearhart John P
Robert D. Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institutions, Johns Hopkins Hospital, Johns Hopkins Medical Institutions, Charlotte Bloomberg Children's Hospital, Baltimore, MD, USA.
Johns Hopkins School of Medicine, Charlotte Bloomberg Children's Hospital, Baltimore, MD, USA.
J Pediatr Surg. 2025 Jan;60(1):161995. doi: 10.1016/j.jpedsurg.2024.161995. Epub 2024 Oct 9.
Cloacal exstrophy (CE) remains one of the most severe birth defects compatible with life with a constellation of anomalies involving the bladder, genitalia, hindgut, and spinal cord. Pelvic osteotomy and immobilization have been utilized to facilitate bladder closure, yet their role as adjuncts remains a topic of debate. The authors sought to evaluate the outcomes of CE closure without the use of osteotomy or lower extremity (LE)/pelvic immobilization.
An institutional database of 173 CE patients was reviewed for patients closed without osteotomy and/or limb immobilization. Patient records were reviewed for continence procedures, reclosure operations, and continence outcomes.
A total of 59 closure surgeries that met inclusion criteria were identified in 56 unique patients. Thirty-seven closure procedures developed eventual failure (63%) with secondary closure events also resulting in failure. Most closures did not use an osteotomy, 93.2%. LE immobilization-only was used in most closures (43/59), of which only 37% were successful. Failures were attributed to dehiscence (14/37), bladder prolapse (19/37), or both dehiscence and prolapse (4/37). The median age at closure was 3 days old (1-18.5 IQR) with the majority of closure events (47) closure events taking place in the newborn period. Median diastasis prior to primary closure was 6 cm (4.8-8 cm IQR). The median number of closure attempts needed to close the bladder was 2 (1-2 IQR). Of the 56 patients, 31 have >3 h of daytime continence, with the entirety of these patients catheterizing a stoma or below.
These results highlight the critical role of osteotomy and lower limb immobilization in successful closure of the bladder and abdominal wall in CE.
Treatment Study.
Level III.
泄殖腔外翻（CE）仍然是最严重的与生命相容的出生缺陷之一，伴有一系列涉及膀胱、生殖器、后肠和脊髓的异常。骨盆截骨术和固定术已被用于促进膀胱闭合，但其作为辅助手段的作用仍存在争议。作者试图评估在不使用截骨术或下肢（LE）/骨盆固定术的情况下CE闭合的结果。
回顾了一个包含173例CE患者的机构数据库，以查找未进行截骨术和/或肢体固定术而闭合的患者。查阅患者记录，了解控尿程序、再次闭合手术和控尿结果。
在56例独特患者中，共确定了59例符合纳入标准的闭合手术。37例闭合手术最终失败（63%），二次闭合手术也导致失败。大多数闭合手术未使用截骨术，占93.2%。大多数闭合手术（43/59）仅使用了LE固定术，其中只有37%成功。失败归因于裂开（14/37）、膀胱脱垂（19/37）或裂开和脱垂两者（4/37）。闭合时的中位年龄为3天（1-18.5四分位间距），大多数闭合事件（47例）发生在新生儿期。初次闭合前的中位分离距离为6厘米（4.8-8厘米四分位间距）。闭合膀胱所需的中位闭合尝试次数为2次（1-2四分位间距）。在56例患者中，31例白天控尿时间超过3小时，所有这些患者均通过导尿口或其下方进行导尿。
这些结果突出了截骨术和下肢固定术在CE患者膀胱和腹壁成功闭合中的关键作用。
治疗研究。
三级。
