Spontaneous Jejunal Perforation Caused by Cholesterol Crystal Embolization in a Patient with a Shaggy Aorta: A Case...

Cholesterol crystal embolization (CCE) is a systemic disease that is caused by cholesterol crystals and plaque debris that break away from atherosclerotic plaques and most commonly affects the kidneys and skin. Gastrointestinal involvement is uncommon, and perforation of the small intestine is an exceptionally rare and life-threatening complication. The preoperative diagnosis of gastrointestinal involvement is often challenging because symptoms and imaging findings are frequently nonspecific, particularly when contrast-enhanced imaging is contraindicated.

An 80-year-old man with end-stage renal disease (ESRD) on maintenance hemodialysis, a shaggy aorta associated with Stanford type B aortic dissection, and a history of CCE presented with mild lower abdominal pain. Initial noncontrast CT revealed localized jejunal wall thickening suggestive of enteritis or ischemia-reperfusion injury. A careful re-evaluation revealed subtle free air adjacent to the affected bowel, and a repeat CT the following day showed progression to a moderate amount of free air with intraperitoneal fluid leakage. Emergency surgical exploration revealed a focal jejunal perforation with localized ischemic change. Indocyanine green fluorescence imaging confirmed preserved macroscopic perfusion. Limited jejunal resection with primary anastomosis was performed, and a histopathological examination revealed CCE within small submucosal vessels. Therefore, the diagnosis of CCE-related intestinal ischemia was confirmed.

Perforation of the small intestine caused by CCE is an extremely rare but critical complication that may progress rapidly despite mild initial symptoms. For patients who are at high risk, particularly those with a shaggy aorta or end-stage renal disease, careful interpretation of serial noncontrast CT findings and timely surgical intervention are essential to achieving favorable outcomes.