Comparison of surgical techniques in the classic form of Hirschsprung's disease in relation to long-term functional prognosis and quality of life.

• Grant number: 22/14170-4
• Support Opportunities: Scholarships in Brazil - Scientific Initiation
• Start date: February 01, 2023
• End date: January 31, 2024
• Field of knowledge: Health Sciences - Medicine - Surgery
• Principal Investigator: Pedro Luiz Toledo de Arruda Lourencao
• Grantee: José Rodrigues de Castro Neto
• Host Institution: Faculdade de Medicina (FMB). Universidade Estadual Paulista (UNESP). Campus de Botucatu. Botucatu , SP, Brazil

Abstract

Hirschsprung's disease is a typical disease of the pediatric age group, clinicallyexpressed by intestinal obstruction in the neonatal period or severe constipation in childhood. It is a congenital malformation of the enteric nervous system, characterized by the absence of ganglion cells in the submucous and myenteric plexuses of the distal intestine, which results in an aperistaltic, spastic segment and constitutes a real obstacle to intestinal transit. The treatment of Hirschsprung disease is surgical. The techniques used have as principles the removal of the aganglionic segment and the reconstruction of the intestinal transit, bringing the ganglionic segment to the anus, with preservation of the sphincter function. The lack of randomized clinical trials in pediatric surgery is mainly due to the rarity of the studied congenital pathologies and the ethical obstacles to approving intervention research in children. This scenario is reproduced in relation to pathology. This project aims to analyze and compare all surgical techniques in classic forms of Hirschsprung's disease in terms of long-term functional prognosis (eg, bowel habits, constipation, and fecal incontinence) and quality of life. This is a systematic literature review, with methodology proposed by the Cochrane Handbook and the recommendations of the PRISMA statement. Upon completion of the study, we expect to obtain a response that will allow us to properly analyze and report the best classical surgical technique indicated for the treatment of classical forms of Hirschsprung's disease. Allowing comparisons to the level of scientific evidence, comparing to clinical applicability in the long-term prognosis of thesepatients.