Abstract
BACKGROUND: Hypoganglionosis is a rare gastrointestinal acquired motility disorder that resembles Hirschsprung's disease and can manifest in the adult life. Abdominal compartment syndrome, a condition characterized by an increase in intra-abdominal pressure with physiological disturbance can be caused by severe massive fecal impaction. CASE SUMMARY: A 33-year-old female presented to the emergency room with massive abdominal distension that rapidly progressed to abdominal compartment syndrome. The patient was diagnosed with hypoganglionosis. Life-saving emergent proctocolectomy was performed to save the patient. CONCLUSION: Abdominal compartment syndrome can develop secondary to excessive colonic distension. This extreme but rare situation must be addressed immediately. Hypoganglionosis is a potential cause of severe constipation that may present in adulthood.