Long-term follow-up of anorectal malformation - how long is long term?

Introduction The ultimate goal of treatment in patients with anorectal malformation (ARM) has moved from mere survival to alleviating symptoms and improving quality of life (QoL), which has become established as an important endpoint in medical care. Adolescents and adults with ARM face several major functional and psychological problems requiring continuity of care. Materials and methods All patients more than 15 years of age presenting with ARM between June 2010 and 2015 were evaluated. Clinical features, investigations, type of surgery done at birth, present treatment given and outcome were analysed. Anal continence was assessed by Kelly's scoring system. Psychological assessment was done using QoL score. Results Six male and seven female patients, aged 16-32 years, presented to our institute with problems related to ARM. Two presented with anal stenosis, two with mucosal ectropion, four had constipation and soiling despite adequate opening, two had megarectosigmoid and three had undergone ileostomy elsewhere for distension/ obstruction owing to impacted faecoliths during adolescence. Surgical treatment was offered to 10 patients, out of whom two patients on ileostomy refused to undergo any surgery. Patients underwent Malone's antegrade enema procedure (two), ectropion excision (two), revision anoplasty (two), excision of pouch and anoplasty (two). Psychological assessment showed that patients felt embarrassed and depressed and used lifestyle-coping behaviour. After bowel management and surgery, mean continence and QoL scores improved from 2.7 to 5.1 and 1.26 to 3.04, respectively, which was statistically highly significant (P=0.001). Conclusion Long-term follow-up of patients with ARM is essential to maintain overall QoL. Improvement in continence by bowel management programme and appropriate surgical intervention leads to improvement in QoL. (C) 2018 Annals of Pediatric Surgery.