Volvo De Sigmóide em criança associado á Doença de Hirschsprung: um estudo de caso

Abstract

Introduction: Hirschsprung's disease (HD) is a congenital pathology characterized by the absence of ganglion cells in the myenteric and submucosal plexuses, leading to functional obstruction and abdominal distension.Case Presentation: This report describes the case of a 12-year-old male patient with a history of prolonged constipation and a delayed diagnosis of HD. The patient sought care due to abdominal pain and distension, with an absence of stool or flatus for five days. The initial radiograph revealed significant distension and fecal impaction, while the CT scan demonstrated sigmoid volvulus, confirmed by exploratory laparotomy, which showed distension without necrosis or perforation. Rectal biopsy identified aganglionosis 2 cm from the pectinate line and normal ganglion cells in the 4-cm and 6-cm samples, confirming ultrashort-segment HD. Management involved volvulus reduction, double-mouth colostomy, subsequent sphincterotomy, followed by reconstruction of intestinal transit, resulting in a physiological evacuation pattern.Conclusion: This case highlights the late and rare presentation of HD with sigmoid colon volvulus, an atypical complication. It demonstrates that patients with a history of prolonged constipation should be investigated for HD. It emphasizes the importance of early diagnosis and detailed histopathological analysis for appropriate management. This report highlights the need for multidisciplinary management and stepwise surgical interventions in complex presentations of HD.