HIRSCHSPRUNG'S DISEASE: CLINICAL EVALUATION AND SURGICAL MANAGEMENT

Abstract

Hirschsprung's disease is a congenital condition characterized by the absence of ganglion cells in the myenteric and submucosal plexus of the intestine, resulting in functional obstruction and proximal dilation. This disease affects around 1 in every 5000 live births, being more common in boys and patients with Down syndrome. The main symptoms are chronic constipation, abdominal distension, enterocolitis and failure to thrive. The definitive treatment is surgical, consisting of the removal of the aganglionic portion of the intestine and the anastomosis of the normal segment with the anus. Objective: to evaluate the scientific evidence on the clinical evaluation and surgical management of Hirschsprung's disease, as well as the outcomes and associated complications. Methodology: followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) checklist, which consists of 27 items that guide the preparation, conduct and presentation of systematic reviews. The databases used were PubMed, Scielo, Web of Science, which were searched in January 2023. The descriptors used were: “Hirschsprung disease”, “clinical assessment”, “surgical management”, “outcomes” and “complications”. The inclusion criteria were: articles published in the last 10 years, in Portuguese or English, that addressed clinical or surgical aspects of Hirschsprung's disease in children. The exclusion criteria were: duplicate, irrelevant, incomplete or low methodological quality articles. Results: 13 articles were selected. The main topics inherent to the theme were: genetic and environmental risk factors for the development of the disease, available diagnostic methods and their advantages and limitations, the most used surgical techniques and their variations, short and long-term clinical outcomes, such as survival, quality of life, intestinal function and continence, and the most frequent and serious complications, such as infection, bleeding, stenosis, fistula, enterocolitis and intestinal pseudo-obstruction. Conclusion: The conclusion of this review was that Hirschsprung's disease is a complex pathology that requires a multidisciplinary approach for its diagnosis and treatment. Clinical assessment must be careful and based on appropriate complementary exams. Surgical management must be individualized and adapted to the characteristics of each patient. Outcomes and complications must be monitored and prevented with effective preventive and therapeutic measures. Hirschsprung's disease has a significant impact on the quality of life of patients and their families, requiring comprehensive and humanized care.