International Center for Colorectal and Urogenital Care, Children's Hospital Colorado, Aurora, USA.
Research in Outcomes for Children's Surgery, Center for Children's Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, USA.
Pediatr Surg Int. 2024 Jul 6;40(1):179. doi: 10.1007/s00383-024-05766-1.
Women born with anorectal malformation (ARM) or Hirschsprung disease (HD) may have impaired urologic function resulting in sequelae in adulthood. This study assessed and compared self-reported urinary outcomes in adult females born with ARM or HD to a reference population.
This was an IRB approved, cross-sectional study of female-born patients with ARM or HD, who completed surveys between November 2021 and August 2022. Female patients between the ages of 18 and 80 years were included. Lower Urinary Tract Symptom Questionnaires were administered through REDCap and the responses were compared to a reference population using Chi-squared or Fisher's exact tests.
Sixty-six born female patients answered the questionnaires, two of them identified as non-binary. The response rate was 76%. Median age was 31.6 years. The majority were born with cloaca (56.3%), followed by other type of ARMs (28.1%), complex malformation (9.4%), and HD (6.3%). A history of bladder reconstruction was present for 26.6%. Catheterization through a channel or native urethra was present in 18.8%. Two had ureterostomies and were excluded from the analysis. Seven had chronic kidney disease or end-stage renal disease, three with a history of kidney transplantation. Patients with cloaca had significantly higher rates of urinary incontinence, urinary tract infection, and social problems due to impaired urological functioning, when compared to an age-matched reference population (Table 3).
This study emphasizes the need for a multi-disciplinary team that includes urology and nephrology following patients with ARM long term, especially within the subgroup of cloaca.
III.
患有肛门直肠畸形（ARM）或先天性巨结肠（HD）的女性可能存在泌尿系统功能受损，导致成年后出现后遗症。本研究评估并比较了患有 ARM 或 HD 的成年女性患者的自我报告的泌尿系统结局，并与参考人群进行了比较。
这是一项经机构审查委员会批准的、针对 ARM 或 HD 女性出生患者的横断面研究，患者于 2021 年 11 月至 2022 年 8 月期间完成了调查。纳入年龄在 18 至 80 岁之间的女性患者。通过 REDCap 发放下尿路症状问卷，并使用卡方检验或 Fisher 精确检验将结果与参考人群进行比较。
共有 66 名女性出生患者回答了问卷，其中 2 人自认为是非二元性别。应答率为 76%。中位年龄为 31.6 岁。大多数患者为 Cloaca 型（56.3%），其次为其他类型的 ARM（28.1%）、复杂畸形（9.4%）和 HD（6.3%）。26.6%的患者有膀胱重建史。18.8%的患者通过通道或自然尿道进行导尿。2 人有输尿管造口术，被排除在分析之外。7 人患有慢性肾脏病或终末期肾病，其中 3 人有肾移植史。与年龄匹配的参考人群相比， Cloaca 型患者的尿失禁、尿路感染和因泌尿系统功能受损导致的社交问题发生率显著更高（表 3）。
本研究强调需要一个多学科团队，包括泌尿科和肾病科，对 ARM 患者进行长期随访，特别是 Cloaca 亚组患者。
III 级。
