Clinical Study

Intestinal Perforation in ACTH-Dependent Cushing’s Syndrome

Table 1

Features of Cushing’s syndrome of our six patients with intestinal perforation.

CaseAge GenderPresentationLaboratory InvestigationsImaging InvestigationsEtiologyTreatment

172, FemaleNausea, vomiting, abdominal pain, hypokalemia from diverticular rupture;
cushingoid features,
hypertension and type 2 diabetes mellitus
At presentation/around time of perforation:
Cortisol 3243 nmol/L, ACTH 33.2 pmol/L UFC 5296 nmol
Chromogranin A – 81 U/L
Glucagon – 91.2 ng/L
CT head – negative
CT abdomen - 6 cm pancreatic mass with calcifications
Octreotide scan – uptake within pancreatic mass
Biopsy confirmed pancreatic neuroendocrine tumorHartmann procedure; ketoconazole, metyrapone, and octreotide for hypercortisolism

261, FemaleKnown metastatic medullary thyroid carcinoma, hypokalemia, dyspnea, abdominal pain from diverticular ruptureAround time of perforation:
Cortisol 1925 nmol/L, ACTH 49.9 pmol/L
UFC – not done
CT abdomen – 2 cm bilateral adrenal masses with no uptake on MIBG scanPresumed ectopic from medullary thyroid carcinomaConservative management

368, FemaleKnown Cushing’s disease with recurrence ten years after surgery;
worsening glycemic control, left lower quadrant abdominal pain, elevated liver enzymes from diverticular rupture
At presentation:
Cortisol 600 nmol/L, ACTH 16.3 pmol/L
UFC 1088 nmol (<300 nmol)
Around time of perforation:
Cortisol 797 nmol/L, ACTH 48.0 pmol/L
UFC 410 nmol
Before second rupture:
Cortisol 568 nmol/L, ACTH 18.7 pmol/L
UFC 348 nmol
At presentation:
MRI sella – left-sided adenoma
At time of recurrence:  MRI sella - 3 mm lesion not amenable to surgery
Cushing’s disease recurrence, corticotroph adenomaIV antibiotics, cabergoline after initial rupture;
Right hemicolectomy for diverticular tear

471, FemaleHypertension, type 2 diabetes mellitus, hypokalemia, lower abdominal pain;
chronic perforation, extensive sigmoid diverticular disease
At presentation/around time of perforation:
Cortisol 1228 nmol/L, ACTH 13.5 pmol/L
UFC 1533 nmol (N <220 nmol)
MRI sella - 3 mm adenoma;
CT abdomen – stable 2 cm bilobed left adrenal adenoma/ hyperplasia
Cushing’s disease, corticotroph adenomaIV antibiotics before transsphenoidal surgery

554, MalePersistent Cushing’s disease post-surgery, on cabergoline; lower abdominal pain, sigmoid colon perforationAt presentation:
Cortisol 469 nmol/L after 1 mg dexamethasone, ACTH 19.4 pmol/L, UFC 374 nmol (N<220 nmol)
Around time of perforation:
UFC 328 nmol (N<166 nmol)
MRI sella – normalCushing’s disease, corticotroph adenomaSurgical management of stercoral ulcer perforation and resumption of cabergoline

652, FemaleAbdominal pain with symptoms of vaginal fistula and symptoms of of hypercortisolism15 months post-surgical repair of diverticular rupture:
Cortisol 1125 nmol/L, ACTH 11 pmol/L
UFC 885 nmol
MRI sella – 4 mm left-sided adenomaCushing’s disease, corticotroph adenomaSurgical management

Reference range for plasma cortisol (85-620 nmol/L), ACTH (2.2-10.1 pmol/L), and UFC (<230 nmol) if not specified.