Long-term clinical assessment after surgical treatment of patients with Hirschsprung's disease: does the association with Intestinal Neuronal Dysplasia type b worsen the results?

• Grant number: 19/03441-4
• Support Opportunities: Scholarships in Brazil - Scientific Initiation
• Start date: June 01, 2019
• End date: September 30, 2022
• Field of knowledge: Health Sciences - Medicine - Surgery
• Principal Investigator: Pedro Luiz Toledo de Arruda Lourencao
• Grantee: Mariana Patti Sanches Coelho
• Host Institution: Faculdade de Medicina (FMB). Universidade Estadual Paulista (UNESP). Campus de Botucatu. Botucatu , SP, Brazil

Abstract

Hirschsprung's disease (HD) is a congenital malformation of the enteric nervous system, typical of pediatric patients, clinically expressed by an intestinal obstruction in the neonatal period or severe intestinal constipation in childhood. It is characterized by the absence of ganglion cells in the submucous and myenteric plexuses and should be treated surgically. Intestinal Neuronal Dysplasia type B (IND-B) is another pathological entity from gastrointestinal neuromuscular diseases, with a clinical condition similar to that of HD. The hyperplasia of the submucous nerve plexuses is the morphological finding that defines IND-B. Histopathological findings compatible within-B, present in the segments located proximal to the areas of aganglionosis, are not uncommon and have been considered as a possible cause of the persistence of obstructive symptoms in patients submitted to HD treatment. Objective: to compare long-term clinical results, after surgical treatment, of 26 patients with HD associated with IND-B to 43 patients with isolated HD. Methodology: This is a single-center study, am bispective, observational, longitudinal and comparative. At first, the previous data will be retrieved from patients' medical records, including signs and symptoms present at the time of diagnosis, prior to surgical treatment, particularly those related to intestinal habits. In a second moment, these patients will be invited to participate in an asemi-structured interview, in which aspects related to intestinal functioning and to quality of life will be evaluated after minimum interval of 5 years from post-surgical treatment. The results obtained will be statistically analyzed, allowing a comparative analysis between the two groups.