Endocrine Abstracts

A previously well 26-year old male developed polydipsia and polyuria in July 2002. Cranial diabetes insipidus was confirmed on water deprivation test and hormonal profile showed testosterone deficiency. MRI showed abnormalities of the pituitary stalk with lesions in the skull. On questioning, the patient had noticed painful swellings on his scalp. This raised suspicion of Langerhans’ cell histiocytosis (LCH) and skull biopsy confirmed diagnosis. He was commenced on vasopressin and testosterone replacement, and referred for fertility treatment.

In September 2004 endocrine profiles showed further pituitary failure. Thyroxine, hydrocortisone and growth hormone (GH) were added to his therapy. This indicated progression of multi-system LCH and the oncology team initiated radiotherapy. MRI showed mass reduction in the pituitary stalk and the patient was again placed on surveillance.

Concerns were raised in September 2005 when the patient complained of hip pain. X-ray showed probable deposits in the right and left ilium. This was confirmed at bone scan and chemotherapy was started. Treatment was completed in April 2006 having achieved good symptomatic response.

Conductive hearing loss and blurring of vision with papilloedema initiated further MRI in January 2007. This showed disease progression around the pituitary fossa and left orbital floor, but did not explain papilloedema. Lumbar puncture was carried out revealing a high opening pressure of 56 cm H₂O. Formal perimetry confirmed visual field loss. Acetazolamide was commenced and a ventricular-peritoneal shunt was placed for intracranial hypertension (ICH). This was presumed secondary to GH therapy which was subsequently discontinued. In November 2007 the patient reported improvement in his vision and good quality of life without GH replacement.

GH associated ICH has been reported in paediatric LCH, but there are few reports of similar complications in adults. Adults on GH replacement with symptoms suggestive of ICH should be investigated promptly.