Evolution in the management of Hirschsprung’s disease in the UK and Ireland: a national survey of practice revisited

Abstract

INTRODUCTIONThe management of Hirschsprung’s disease continues to evolve. This questionnaire survey aimed to determine current surgical management strategies for Hirschsprung’s disease in Britain.SUBJECTS AND METHODSThe survey was sent electronically to all British paediatric surgeons. Initial questions explored individual experience and regional service provision. Additional questions, reserved for surgeons who perform definitive Hirschsprung’s disease surgery, addressed specific clinical scenarios.RESULTSSurveys were sent to 142 surgeons yielding 85 responses. After exclusions, 64 surveys from 21 centres were analysed. Forty-seven respondents worked in centres with designated ‘Hirschsprung’s disease surgeons’. Forty respondents perform definitive Hirschsprung’s disease surgery. In a well neonate with left-sided Hirschsprung’s disease, 34 of 40 surgeons favour primary pull-through following bowel decompression with rectal washouts; 35 of 40 surgeons aim to perform definitive surgery at less than 3 months of age, with 17 favouring laparoscopic-assisted Soave–Boley and 15 favouring an open Duhamel pull-through. Of the 40 surgeons, 36 use a staged approach to right-sided/total colonic Hirschsprung’s disease with 23 favouring a Duhamel or Long Duhamel pull-through.CONCLUSIONSThe primary pull-through, using an open Duhamel or laparoscopic-assisted Soave–Boley technique, during the first 3 months of life, has become the operative strategy of choice in rectosigmoid Hirschsprung’s disease in Britain. Marked variation in practice remains for right-sided Hirschsprung’s disease.