Colonic Perforation As the Initial Presentation in Cushing's Disease

Presentation Number: SUN-0774
Date of Presentation: June 22nd, 2014

Pooja Raghavan*1, Talia Diker-Cohen2, Dhyan P Rajan3, Susmeeta T. Sharma4 and Lynnette K. Nieman2
1National Institutes of Health, North Bethesda, MD, 2National Institutes of Health, Bethesda, MD, 3Nassau University Medical Center, East Meadow, NY, 4Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD


Introduction:Exogenous glucocorticoid administration has been associated with many gastrointestinal complications, including colonic abscesses, perforation and peritonitis. Very few cases of colonic perforation have been reported in endogenous Cushing’s syndrome (CS); all in patients over 50 years of age in whom diverticulitis is more common. We report two CS patients, <50 years of age, with colonic perforation as the initial presentation.

Case 1:31-year old male with hypertension (HTN) and diabetes mellitus type 2 (DM2) presented with abdominal pain and hematochezia. CT abdomen revealed a sigmoid diverticular perforation fistulizing to the retroperitoneum with intraperitoneal free air. The patient underwent emergent sigmoid resection with end colostomy but due to poor wound healing, required seven re-operations over four weeks before final abdominal closure was achieved with full thickness skin grafting.  Laboratory tests confirmed ACTH-dependent CS: elevated 24-hour urinary free cortisol (UFC) (470 mcg/day, normal (N) 3.5-45), abnormal late night salivary cortisol (LNSC, 1270 ng/dl, N<100), elevated plasma ACTH (79 pg/ml, N<46), and an abnormal 1mg overnight dexamethasone suppression test (DST). An 8 mg DST suggested an ectopic source. However, CRH stimulation test, MRI pituitary (3mm hypointense lesion), and inferior petrosal sinus sampling (peak post-CRH petrosal-to-peripheral ACTH ratio = 23) were consistent with a pituitary source. Transsphenoidal resection (TSR) of the microadenoma led to remission of hypercortisolemia. Pathology confirmed ACTH-staining pituitary adenoma.

Case 2:43-year old male with HTN, DM2, and obstructive sleep apnea presented with severe abdominal pain, was found to have a diverticular perforation, and underwent emergent intestinal resection with end colostomy. Laboratory tests revealed ACTH-dependent CS: UFC=79-146 mcg/day, LNSC=0.22 mcg/dl (N<0.09), plasma ACTH=95 pg/ml, and an abnormal 1mg DST. CRH stimulation test, 8mg overnight DST and a pituitary MRI (7mm hypointense lesion) indicated a pituitary source. TSR of the microadenoma led to remission of CS. Pathology confirmed ACTH-staining pituitary adenoma. Colostomy reversal was successfully completed 6 months post-remission.

Conclusion: By masking signs of acute inflammation and causing an impaired immune response, prolonged endogenous hypercortisolemia can result in colonic perforation.  Early diagnosis and treatment may help prevent disabling complications in CS.


Nothing to Disclose: PR, TD, DPR, STS, LKN