Development of an enterocutaneous fistula from an intestinal perforation in a patient with autosomal dominant polycystic kidney disease

We herein report a case of enterocutaneous fistula in a patient with autosomal dominant polycystic kidney disease (ADPKD). A 37-year-old Japanese man was admitted to our hospital. Three months prior to transfer to our hospital, he developed intense flank pain with gross hematuria. His serum creatinine had decreased to 7.8 mg/dL and hemodialysis was started, but gross hematuria persisted and he developed hypotension. Upon admission, plain chest radiography did not reveal any free air, but computed tomography (CT) showed generalized ventral subcutaneous air from the head to the lower extremities and enlarged kidneys. Enterography showed leakage of contrast medium from the descending colon into the subcutaneous area. C-reactive protein was 23.1 mg/dL. A colostomy was placed in the transverse colon proximal to the perforation, and systemic subcutaneous drainage was performed. The fever subsequently resolved, and the C-reactive protein test became negative. Three months later, renal artery embolization was performed, and 12 months thereafter, CT showed a marked decrease in kidney size. We assume that a markedly enlarged kidney leaded to intestinal perforation, which developed into an enterocutaneous fistula. Consequently, intestinal fluid leaked into the subcutaneous cavity of the abdominal wall and spread systemically, resulting in extensive subcutaneous abscesses.