Context: Patients taking exogenous glucocorticoids are at risk for gastrointestinal (GI) complications, including peptic ulcer disease with perforation and gastric bleeding. However, little is known about the GI comorbidity in patients with endogenous hypercortisolemia.
Case descriptions: We describe six patients with endogenous Cushing syndrome (CS) who developed sudden perforation of colonic diverticula necessitating urgent exploratory laparotomy. Most of these patients shared the following features of CS: skin thinning, severe hypercortisolemia (24-hour urinary free cortisol ≥10 times the upper limit of normal), ectopic secretion of ACTH, and severe hypokalemia. At the time of diagnosis of diverticular perforation (DP), these patients had minimal signs of peritonitis and lacked fever or marked leukocytosis. The diagnosis of DP was established by having a low threshold for obtaining an imaging study for evaluation of nonspecific abdominal pain.
Conclusions: Patients with CS can develop spontaneous surgical abdomen with rapid decompensation within hours. Prompt recognition is critical in the successful treatment of these patients.