A six-week-old male infant was admitted for investigation of cholestasis and pale stools. He became lethargic and apnoeic with prolonged seizures after a percutaneous liver biopsy. Subsequent investigations showed conjugated hyperbilirubinaemia, elevated liver enzymes, and hypoglycaemia. The radinuclide hepatobiliary scintigraphy was non-excretory. After an operative cholangiogram, the infant developed Addisonian-like crisis with bradycardia, hypotension, respiratory distress, metabolic acidosis, hypoglycaemia, hyponatraemia, and hyperkalaemia. Blood investigations confirmed congenital hypopituitarism. Hormone replacement therapy with L-thyroxine and cortisone acetate resulted in dissolution of jaundice and the reduction of the liver size.
A patient with beta hCG-secreting germ cell carcinoma of the pineal and suprasellar regions presented with hydrocephalus, Parinaud's syndrome, hypopituitarism and polyuria. Central diabetes insipidus was strongly suspected although the water deprivation test was not diagnostic. The polyuria however, responded to ADH analogue when the hypothyroidism and hypocortisolism were treated. Pubertal development was evident and serum testosterone was normal despite the low FSH/LH, suggesting hCG stimulation of Leydig cells. This case illustrates that a beta hCG-germ cell tumour of the suprasellar region causing hypopituitarism can mask the presence of central diabetes insipidus and hypogonadotrophic hypogonadism.
Idiopathic granulomatous hypophysitis (GH) is an uncommon inflammatory disease of the pituitary with impairment of pituitary gland function due to infiltration by lymphocytes, plasma cells and macrophages. We report the case of a 39-year-old woman who presented with worsening of headaches for 1 month and blurring of vision over 5 days. An MRI revealed a homogeneous supra-sellar mass evoking a pituitary tumour with bulky pituitary stalk extending into the left and right cavernous sinuses. Hormonal investigations showed anterior pituitary hormone deficiencies; meanwhile histopathological examination revealed an aspect of hypophysitis. Clinical and radiological remission occurred immediately postglucocorticoid therapy with the addition of a steroid-sparing agent later in view of recurrence of symptoms on glucocorticoid dose reduction. GH has important diagnostic and therapeutic implications, as clinical and radiological features ameliorate via medical treatment. With further understanding and recognition of the disease, we hope to highlight a case of GH, in which signs and symptoms improved after initiation of corticosteroids.