Endocrine Abstracts

A 26 year old man of Angolan descent presented to the endocrine clinic with poorly controlled hypertension (systolic blood pressure >200 mmHg). He had been treated with Amlodipine for almost six years, and more recently the addition of Irbesatan and Indapamide had not led to adequate blood pressure control. His hypertension was diagnosed at age 19 and progressive features of Cushing’s disease had remained unnoticed, with truncal striae, easy bruising, myopathy, puffiness around the face and lower leg swelling. In the previous year he had had a skin graft to his right leg following a football injury, which prompted his referral. Investigations showed failure of cortisol suppression on a low dose dexamethasone suppression test (time=48 hours, cortisol 1107 nmol/l) and magnetic resonance (MRI) imaging demonstrated a right-sided pituitary macroadenoma of 13 mm. Urgent petrosal venous sampling was scheduled, but he acutely decompensated in the interim after being admitted with newly diagnosed diabetes mellitus, a hyperosmolar hyperglycaemic state (HHS) and multiple cranial nerve dysfunction, including facial nerve palsy. Brainstem MRI imaging was unremarkable and the working diagnosis was imminent pontine myelinolysis secondary to osmotic change. He was treated appropriately for the HHS and recovered fully. Hypercortisolaemia was treated with Ketocanozole and he went on to have urgent transphenoidal pituitary surgery. Histology confirmed tumour cells expressing ACTH with P-53 overexpressed at 2% and the Ki-67 index high at 5%. Cushing’s post-operative work-up showed he was not biochemically cured and an interval MRI showed residual tissue extending into the right cavernous sinus. Further treatment options are being considered including a second trans-sphenoidal procedure and/or stereotactic radiotherapy. This rare case demonstrates two interesting presentations; firstly, a rapid decompensation of Cushing’s disease resulting in HHS, and secondly a rising osmolality in HHS causing cranial nerve dysfunction.