BACKGROUND: Abdominal compartment syndrome (ACS), characterized by an increased intra-abdominal pressure and new-onset organ dysfunction, is a critical and potentially fatal condition, with no case of ACS caused by intestinal gas without intestinal lesion being reported to date.

CASE REPORT: A 2-year-old girl with a chromosomal abnormality of 1p36 deletion presented with fever and diarrhea following upper-gastrointestinal series for the evaluation of gastroesophageal reflux. After 20 days, she experienced septic shock and multiple-organ failure, accompanied with rapidly growing, severe abdominal distension. A marked increase in the intra-abdominal pressure was indicated by the complete loss of elasticity in the extremely hard and distended abdomen. She died 14 h after the onset of shock. Her autopsy examination revealed extensive pneumonia and excessive intestinal gas, despite no occlusive intestinal lesion present.

CONCLUSIONS: It is critical to be aware that secondary ACS can occur following sepsis due to the accumulation of extensive intestinal gas, without an occlusive intestinal lesion.

Keywords: Chromosome Aberrations, Disseminated Intravascular Coagulation, intra-abdominal hypertension, Sepsis, Child, Preschool, Fatal Outcome, Multiple Organ Failure