Current management of Hirschsprung’s disease in Egypt: A survey of members of the Egyptian Pediatric Surgical Association

Abstract

Background/purpose Significant progress has been made in the management of  Hirschsprung’s disease (HD). The choice of the management plan, surgical  approach, and operative details is still variable among pediatric surgeons. This survey aims to determine the current preferences of Egyptian pediatric surgeons in the management of HD.
Materials and methods A survey was circulated individually to the members of the Egyptian Pediatric Surgical Association  (EPSA) during the General Assembly Meeting. An electronic form of the survey was sent by e-mail to all Egyptian consultant  pediatric surgeons registered to EPSA through the ‘EPSA online’ e-mail group. A second round of e-mails was sent 2 weeks later.
Results Responses were received from 112 surgeons; seven responses were  excluded (incomplete and duplicate responses), yielding 105 survey charts for  analysis. The 105 responses represent 80.7% of the 130 fully trained pediatric surgeons  currently working in Egypt. A total of 76.2% of responders use both  contrast enema and rectal biopsy for the preoperative diagnosis of HD. Contrast enema alone is used by 13 (12.4%). A further 11.4% combine this with anorectal manometry. Intraoperative frozen section tissue diagnosis is used routinely by only 4.8% of the responders. Surgery is preferred to be  performed during the neonatal period by 21.9% of the responders, from 1 to 3 months (28.6%), or after the third month of life  (49.5%). A definitive one-stage colonic pullthrough is always used by 59%, whereas 7.6% are still using staged operations. The rest of the responders had no specific predilection. The preferred surgical technique for rectosigmoid HD is transanal endorectal pull-through (68.5%), Soave transabdominal endorectal  pull-through (19.0%), laparoscopic-assisted pull-through (9.5%), and Duhamel with GIA staplers (2.9%). The Soave operation is preferred for HD extending to the right side and total colonic HD (45.7%) and redo surgery (37.1%).
Conclusion The EPSA survey indicated that most surgeons are moving toward a one-stage pull-through using transanal endorectal pull-through for patients with rectosigmoid HD. There are large variations in preference for the age at  pull-through. There is a need for a routine practice of intraoperative frozen section tissue diagnosis, and popularizing laparoscopic surgery for patients with long segment disease.

Keywords: diagnosis, Hirschsprung’s disease, management, survey