Anorectal malformations – surgical aspects and transition

Sammanfattning: Background: Anorectal malformations (ARM) occur in 1/5000 live births (1.2–1.6:1 male:female). Associated malformations are common, influencing, together with ARM-subtype complexity, long-term outcome, follow-up and need of transitional care. Reconstructive surgery, the posterior sagittal anorectoplasty (PSARP), is performed in infancy, either as a single-stage or multi-stage procedure with a colostomy. Aims: 1. Assess frequency and risk factors of post-PSARP wound dehiscence. 2. Explore patient-expressed needs and expectations of transitional care. 3. Assess accuracy of pre-PSARP fistula diagnostics, and 4. significance of postoperative abdominal scarring and propose a scar treatment approach. Methods: 1. Retrospective study of all ARM infants treated in Lund between 2001–2016 evaluating PSARP wound complications and management including multivariate logistic regression analysis of potential risk factors. 2. Focus group study of ARM adults with qualitative content analysis. 3. Retrospective study of male ARM infants treated in Lund 2001–2020 defining pre-PSARP fistula diagnostic accuracy of radiological- and endoscopical modalities compared to intra-PSARP subtyping. Diagnostic superiority receiver-operating characteristic curve (ROC) analysis. 4. Patient- and observer-reported cross-sectional study of previously multi-stage PSARP treated ARM children (>5y) and adults, evaluating abdominal scar symptoms through the validated Patient and Observer Scar Assessment Scale (POSAS), including Pearson’s r for correlation with age, and pictorial evaluation of possibility of scar treatment by a plastic surgeon. Ethical approval was obtained.Results: 1. 90 infants (41% female) were included. Colostomy protected against wound dehiscence; 11(22%) versus 17(43%) p=0.04. No risk factor was identified. 2. 16 adults (63% female) were included. Identified key elements of adequate transition: improved knowledge of ARM among patients and adult care givers, support with coping strategies, structured communication between patient, paediatric- and adult care, and easy access to specialised adult care through patient navigators. 3. 38 male infants were included. Cystoscopy and high-pressure colostogram had the highest diagnostic accuracy (70% and 66%, respectively). No diagnostic superiority was identified. 4. 27 children and adults (48% female) were included. 6(22%) reported scar pain, 5(19%) pruritus and 9(33%) altered behaviour. Worse POSAS score and increasing age correlated, r=0.4 p=0.04. 21(78%) were technically suitable for plastic surgery, among whom 8(30%) requested treatment. No gender differences were identified.Conclusions: Colostomy seems to protect against post-PSARP wound dehiscence. Patients identify knowledge, structured communication and easy access to specialised adult care as key factors for adequate transition. Current pre-PSARP fistula diagnostics only reach a maximum accuracy of 70%. Postoperative scar morbidity should be addressed in follow-up, and plastic surgery considered.