Composite Functional and Quality of Life Outcomes in Adults Operated for Cloacal Malformation

L Örtqvist; G Holmdahl; H Borg; K Bjornland; H Lilja; P Stenström; N Qvist; T Sæther Hagen; M Pakarinen; T Wester; R Rintala

doi:10.1016/j.jpedsurg.2024.161981

Composite Functional and Quality of Life Outcomes in Adults Operated for Cloacal Malformation

J Pediatr Surg. 2024 Oct 1;60(1):161981. doi: 10.1016/j.jpedsurg.2024.161981. Online ahead of print.

Authors

L Örtqvist¹, G Holmdahl², H Borg³, K Bjornland⁴, H Lilja², P Stenström⁵, N Qvist⁶, T Sæther Hagen⁴, M Pakarinen⁷, T Wester², R Rintala⁷

Affiliations

¹ Karolinska Institutet, Department of Women's and Children's Health, Stockholm, Sweden; Karolinska University Hospital, Department of Pediatric Surgery, Stockholm, Sweden. Electronic address: elisabet.ortqvist@ki.se.
² Karolinska Institutet, Department of Women's and Children's Health, Stockholm, Sweden; Karolinska University Hospital, Department of Pediatric Surgery, Stockholm, Sweden.
³ Department of Pediatric Surgery, Queen Silvia's Children's Hospital, Göteborg, Sweden.
⁴ Department of Pediatric Surgery, Oslo University Hospital, University of Oslo, Oslo, Norway.
⁵ Department of Pediatric Surgery, Children's Hospital, Skåne University Hospital Lund, Lund University, Sweden.
⁶ Research Unit for Surgery, and Centre of Excellence in Gastrointestinal Diseases and Malformations in Infancy and Childhood (GAIN), Odense University Hospital, Odense, Denmark; University of Southern Denmark, Odense, Denmark.
⁷ Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, The New Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.

PMID: 39395844
DOI: 10.1016/j.jpedsurg.2024.161981

Abstract

Introduction: Composite long-term outcomes of cloacal malformations remain unclear. We aimed to evaluate bowel and bladder control, sexual function, reproductive health, and quality of life in adulthood.

Methods: A Nordic multi-center cross-sectional observational study evaluating the outcome of adult patients with cloacal malformations was performed. Patient characteristics were retrieved from case records. Established questionnaires were sent to the patients to evaluate bowel- bladder- and sexual function and quality of life.

Results: Thirty-four of 48 (70%) eligible patients with median age 28 years (18-45) responded. Eight (24%) patients had a common channel >3 cm. Imaging-confirmed spinal and sacral abnormalities were present in 4 and 17 patients respectively. 85% (n = 29/34, four patients with permanent urinary diversions after bladder neck closure excluded) reported no urinary leakage without physical activity or urge to urinate. Three patients had a permanent enterostomy. 42 % (n = 11/26) reported acceptable bowel function according to Bowel Function Score (with eventual ongoing bowel management). The median Profile of Female Sexual Function Score was below normative values. Quality of life was comparable to the reference population, but thirty percent scored within the distress domains. Eight patients (24%) had been pregnant (15 pregnancies), resulting in seven live births, but the need of assisted reproduction techniques (40%, n = 6/15) and miscarriage (40%, n = 6/15) was common.

Conclusions: Adequate spontaneous bowel control was rare, while most patients were dry for urine without additional procedures. Cloacal malformation also have a negative impact on sexual function health related quality of life and reproductive health. Long-term follow-up is crucial, not only regarding bowel and bladder function, but also for sexual and reproductive function, which may be important negative factors for health-related quality of life.

Level of evidence: Level IV.

Keywords: Adults; Bowel function; Cloacal malformation; Constipation; Fecal incontinence; Long-term; Pregnancy; Quality of life; Sexual function; Urinary incontinence; Urinary tract.