ACROMEGALY WITH SPONTANEOUS VENTRICULOSTOMY - A RARE PHENOMENON

Abstract

INTRODUCTION
Spontaneous ventriculostomy is a unique condition that occurs in patients with chronic obstructive hydrocephalus wherein spontaneous ventricular rupture results in communication between the ventricular system and subarachnoid space. We present a case of acromegaly who presented with spontaneous ventriculostomy on magnetic resonance imaging (MRI) of the pituitary with no prior history of neurosurgical intervention.

CASE
A 66-year-old male with chronic hypertension presented with long standing bilateral peripheral vision loss, worsening over the right eye for 3 months, obstructive sleep apnea symptoms and no symptoms of pituitary apoplexy. Clinically, he had classical features of acromegaly and ophthalmological assessment confirmed bitemporal hemianopia with no optic atrophy.

Pre-operative investigations confirmed elevated IGF-1: 240 ng/ml (NV: 40.2 - 225), random growth hormone (GH): 3.7 ng/ml (NV: <3) and prolactin (PRL): 805 mIU/L (NV: 45 - 375). Thyroid and cortisol levels were normal. Pre-operative MRI of the pituitary revealed a heterogenous sellar mass with cerebrospinal fluid (CSF) fistulous connection between the floor of third ventricle and sella turcica. He underwent endoscopic chiasmopexy which revealed dense arachnoid adhesions in the sellar region with CSF gush on manipulation. No tumour was removed as a discrete adenoma could not be identified. Post-operatively, IGF-1 normalised to 184 ng/ml (40.2-225), but GH and PRL remained elevated at 4.9 ng/ml and 474 mIU/L, respectively. Failure of GH suppression following 75 g OGTT at 2.8 ng/ml denotes persistent active acromegaly. As he had mild acromegaly with prolactin co-secretion and was unable to afford somatostatin receptor ligand therapy, he was commenced on cabergoline 0.25 mg twice a week.

CONCLUSION
Preceding case reports of spontaneous ventriculostomy secondary to obstructive hydrocephalus were due to neoplastic disease or benign aqueduct stenosis. We report a case of acromegaly with prolactin co-secretion who presented with a rare finding on pituitary MRI of spontaneous ventriculostomy.