HIRSCHSPRUNG DISEASE AND CORRECTION OF CONGENITAL HEART DISEASES: COMBINED SURGICAL PROCEDURES FOR CONGENITAL DISEASES OF THE BOWEL AND HEART

Abstract

Introduction: Hirschsprung's disease (HD) and congenital heart disease (CHD) are complex congenital malformations that affect the intestine and the heart, respectively. HD is characterized by the absence of ganglion cells in the intestinal plexuses, leading to functional intestinal obstruction. CHD encompasses a broad spectrum of structural defects of the heart, with varying degrees of severity. The combined occurrence of these two conditions, although rare, presents a significant therapeutic challenge, especially in neonates and infants. Surgical correction of both conditions, when necessary, requires meticulous planning and coordination between pediatric surgical teams, cardiologists, and other specialists. The decision to perform combined or sequential procedures is influenced by the severity of each condition, age, and clinical status of the patient. Objective: This systematic review of the literature aims to analyze the clinical experience and outcomes of combined surgical procedures for correction of Hirschsprung's disease and congenital heart disease in pediatric patients. Methodology: A comprehensive search was performed in the PubMed, SciELO and Web of Science databases, using the descriptors "Hirschsprung Disease", "Congenital Heart Defects", "Pediatric Surgery", "Combined Procedures" and "Neonatal Surgery". The search included articles published in the last 10 years, in English or Portuguese, that reported cases or case series of pediatric patients with HD and CC who underwent combined surgical correction. The inclusion criteria were: studies that described patients with a confirmed diagnosis of HD and CC, who underwent surgical procedures to correct both conditions, and that presented data on the type of procedure performed and postoperative outcomes. Studies that did not fit these criteria, such as isolated case reports, studies in adults or studies that did not describe combined surgical correction, were excluded. Results: The review identified 15 studies that described combined surgical correction of HD and CC. The case reports and case series included different types of congenital heart disease, with emphasis on complex cyanotic heart disease. The surgical procedures varied according to the severity of each condition, ranging from primary correction of both conditions in a single procedure to sequential approaches, with initial correction of the CHD followed by correction of the HD. The decision of which surgical approach to adopt was influenced by the age of the patient, their clinical status and the experience of the surgical team. The postoperative results were variable, with some patients presenting good evolution and others developing complications, such as infections, bleeding and respiratory difficulties. Conclusion: The combined surgical correction of Hirschsprung's disease and congenital heart disease represents a complex therapeutic challenge, especially in pediatric patients. The available literature suggests that the surgical approach should be individualized, taking into account the severity of each condition, the age and clinical status of the patient. Collaboration between pediatric surgical teams, cardiologists and other specialists is essential for the success of the treatment. Further studies are needed to establish best practices and optimize outcomes in these complex patients.