Congenital Aganglionic Megacolon": A Rare Case Report of Hirschprung Disease with Recurrent Surgical Complications

An uncommon congenital disorder known as Hirschsprung disease (HSCR) affects roughly 1 in 5,000 live infants.It occurs when ganglion cells are absent in the colon, leading to an obstruction in the gut. This case study focuses on an 8-years old girl diagnosed with a history of HSCR, previously underwent stage-1 re-section during her neonatal period and a Leveling colostomy on November 2023, due to severe bowel obstruction. On May 2024, she presented with multiple complications, including abdominal distension, intermittent fever, respiratory distress, pedal edema, and underwent stage-II Soave pull-through surgery. Despite intensive management, unfortunately, she experienced persistent fever, electrolyte imbalance and pleural effusion. After seven months, she returned with symptoms of a colonic-leak, Disseminated Intravascular coagulation (DIC), abdominal rupture, sepsis and non-healing abdominal wound while on ileostomy. A post-colon closure scan indicated mild bowel wall edema with persistent leukocytes, suggesting ongoing inflammation. This case highlights the challenges and complexities of post-operative care in pediatric patients with HSCR. Treatment included laparotomy, ileostomy, and secondary suturing to address these complications.