PANHYPOPITUITARISM FROM EMPTY SELLA SYNDROME ASSOCIATED WITH IDIOPATHIC PORTAL GASTROPATHY IN AN ADULT MALE

Abstract

INTRODUCTION
Hormone deficiencies from hypopituitarism have been linked to liver dysfunction in the neonates up to adulthood with a spectrum ranging from cholestasis, elevated transaminases, NAFLD, hepatitis and cirrhosis. The exact mechanisms are still unclear but points to growth- and adrenocorticotrophic hormone deficiencies affecting liver metabolic pathways. This paper presents a rare case of panhypopituitarism associated with portal gastropathy.

CASE
A 19-year-old male presented with hematemesis from bleeding esophageal varices, requiring rubber band ligation. He has no prior or family history of hepatic and metabolic disorders. Diagnostics revealed elevated transaminases, recent normal bilirubins but high alkaline phosphatase, and bicytopenia consistent with hypersplenism. Hepatitis profile and iron overload markers were normal. Work-up for common causes of cirrhosis were negative and a diagnosis of idiopathic portal hypertension was made. He was referred to endocrinology due to eunuchoid body habitus, signs of hypogonadism and symptoms of hypothyroidism and hypocortisolism. Hormonal evaluation revealed anterior pituitary and target-organ hormone deficiencies. Delayed skeletal maturity was seen on skeletal survey. Cranial MRI revealed bilateral hippocampal atrophy with unremarkable sellar structures. Hormone replacement with steroids and Levothyroxine was initiated with improvement of symptoms. He plans to start testosterone therapy and is scheduled for surveillance endoscopies.

CONCLUSION
Case reports have suggested that early hormone replacement therapy in hypopituitarism may still reverse liver dysfunction such as cholestasis but if initiated later, the pathology will most likely be persistent. Though rare, in patients presenting with idiopathic liver dysfunction and signs of hormone deficiencies, a neuroendocrinologic cause such as hypopituitarism should be entertained and addressed.