Levitt Marc A, Bischoff Andrea, Breech Lesley, Peña Alberto
Department of Pediatric Surgery, Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
J Pediatr Surg. 2009 Jun;44(6):1261-7; discussion 1267. doi: 10.1016/j.jpedsurg.2009.02.046.
Vestibular fistulas are the most common anorectal malformations (ARMs) in females. Associated gynecologic defects are rarely mentioned in the literature but may have serious clinical implications if undetected. The definitive repair of the ARM offers an opportunity for diagnosis and treatment of these conditions.
Two hundred seventy-two patients with vestibular fistula were retrospectively reviewed, with emphasis on gynecologic defects.
Forty-eight patients (17%) had 83 gynecologic anomalies. Fourteen patients had a vaginal septum, all with 2 uterine cervices. All septa were resected at the main repair. Twenty-six patients had no vaginal opening. Twenty of them had absent vagina. Eighteen of those had an absent uterus. Patients with absent vagina underwent vaginal replacement with distal rectum (12), sigmoid (6), and ileum (2). Six patients had a patent upper vagina; 3 reached the perineum after mobilization and 3 required replacement; 2 with sigmoid and 1 with rectum.
Vaginal septa are easily diagnosed and can be resected during the repair of the vestibular fistula. The presence of 2 cervices has important obstetric implications. Absent vagina requires a technically demanding repair, with special preoperative planning. Vaginoscopy or careful inspection should precede surgical reconstruction.
前庭瘘是女性最常见的肛肠畸形(ARM)。相关的妇科缺陷在文献中很少被提及,但如果未被发现可能会有严重的临床影响。ARM的确定性修复为诊断和治疗这些疾病提供了机会。
回顾性分析272例前庭瘘患者,重点关注妇科缺陷。
48例患者(17%)有83处妇科异常。14例患者有阴道纵隔,均有2个子宫颈。所有纵隔均在主要修复时切除。26例患者无阴道口。其中20例无阴道。其中18例无子宫。无阴道患者行远端直肠(12例)、乙状结肠(6例)和回肠(2例)阴道再造术。6例患者上阴道通畅;3例经游离后到达会阴,3例需要再造;2例用乙状结肠,1例用直肠。
阴道纵隔易于诊断,可在前庭瘘修复时切除。2个子宫颈的存在有重要的产科意义。无阴道需要技术要求较高的修复,术前需特殊规划。手术重建前应先行阴道镜检查或仔细检查。