P3C245: Abdominal Compartment Syndrome as a Delayed Presentation of Hypoganglionosis

Introduction: Abdominal compartment syndrome (ACS) is a feared complication of many intra-abdominal pathologies including trauma, ascites, sepsis, but is rarely associated with constipation in the pediatric population. ACS is defined as Intra-abdominal Pressure (IAP) of >20 mmHg and multiorgan involvement particularly renal, respiratory and circulatory systems with a high fatality rate. While there are some reports of adults with chronic constipation and ACS, reports of such presentation in the pediatric population is further rare.

Case Description: A 9-year-old male with history of chronic constipation managed with daily polyethylene glycol presented with worsening abdominal pain, distension and watery stools of 1 week duration. Mother reported history of gastroenteritis 2 months ago with no formed bowel movements since. Physical examination showed a distended and tender abdomen with normoactive bowel sounds. Abdominal radiograph showed extensive stool burden without signs of obstruction. He was started on continuous polyethylene glycol (PEG) via nasogastric tube, scheduled enemas and IV fluid hydration. While initial labs were stable, subsequent studies showed severe, worsening, electrolyte abnormalities with hypokalemia, hypomagnesemia and hypocalcemia, associated with a prolonged QT interval on the electrocardiogram (EKG). He required aggressive electrolyte replacement with follow up labs and EKG for close monitoring. Gastroenterology and pediatric intensive care (PICU) were consulted. Worsening abdominal pain and distention overnight on day 3 of admission with decreasing urine output and severe dependent edema over lower extremities and scrotum were alarming. Due to a scarcity of PICU beds, the patient was unable to be transferred to the unit and have direct monitoring of his intra-abdominal pressure. A computed tomography scan at this juncture showed colonic stool burden with mass effect on multiple organs including the inferior vena cava, concerning for abdominal compartment syndrome (Fig. 1, 2). He was taken for surgical disimpaction, removing a large, softball-sized stool ball and several liters of liquid fecal matter. Rectal biopsy showed hypoganglionosis of the rectum. He had significant symptomatic improvement and stabilization of electrolyte derangements following the procedure and was discharged home a day after disimpaction.

Discussion: Compared to frank ‘aganglionosis’ in Hirschsprung's, ‘hypoganglionosis’ presents with less severe constipation that often delays recognition of the pathology. International groups have reported delayed diagnosis well into adulthood, but this rarely occurs in the United States. We report a rare, delayed and severe case of hypoganglionosis that presented with findings concerning for ACS, requiring urgent surgical intervention.

Conclusion: Hypoganglionosis can be unrecognized in rare cases and lead to severe constipation. Although ACS rarely stems from constipation, it can be seen in extreme cases as noted in our patient. Thus, one must be cautious in dealing with cases of severe chronic constipation to avoid the morbidity and mortality associated with ACS.