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

Abstract

INTRODUCTION The 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 METHODS The 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. RESULTS Surveys 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. CONCLUSIONS The 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.

Survey on the management of anorectal malformations (ARM) in European pediatric surgical centers of excellence

“…This indicates that with a low case load per surgeon the training opportunities for new surgeons may be limited. A similar conclusion was drawn for the treatment of Hirschsprung disease in the survey conducted in the UK by Bradnock et al [3].…”

“…Several surveys on the management of other colorectal diseases, such as Hirschsprung disease (HD), have been published [3,4]. A survey among pediatric surgeons from the UK showed that the primary pull-through during the first 3 months of life, using an open Duhamel or laparoscopic-assisted Soave-Boley technique, had become the operative strategy of choice in rectosigmoid HD in the UK.…”

“…A survey among pediatric surgeons from the UK showed that the primary pull-through during the first 3 months of life, using an open Duhamel or laparoscopic-assisted Soave-Boley technique, had become the operative strategy of choice in rectosigmoid HD in the UK. Marked variation in practice remained for right-sided HD [3]. A survey conducted among US American surgeons revealed that a 1-stage transanal operation with or without laparoscopy had become the most common strategy for the surgical management of the typical baby with HD.…”

A survey of current practices in management of Hirschsprung′s disease in Nigeria

“…[5] Similar survey of members of British Association of paediatric surgeons (BAPS) in 2008 showed equal preference for both Soave and Duhamel procedures. [14] Since the introduction of complete transanal pull through for HD by De la Torre-Mondragón and Ortega-Salgado in 1998, [4] many groups have reported on this procedure and have shown it to be a safer option. [15][16][17][18][19] It is a simple approach eliminating the need for abdominal access by either laparoscopy or laparotomy.…”

“…[30] A recent survey of BAPS members by Bradnock and Walker revealed that 86% of 36 respondents now performed primary pull through in a well-child. [14] Singh et al in a survey of 126 children managed for HD on a prospective basis over 4 years by members of the AAPS reported that 51% of the children had primary pull through. [5] The findings of the of the present survey with only 40% of the respondents performing primary pull through for more than half of their patients is rather low compared with other reports.…”