LYMPHOCYTIC HYPOPHYSITIS PRESENTING WITH DIABETES-INSIPIDUS IN A 14-YEAR-OLD GIRL - CASE-REPORT AND REVIEW OF THE LITERATURE

Lymphocytic hypophysitis is a rare disorder predominantly affecting fe males during the antepartum or postpartum period. It is characterized by destruction and lymphocytic infiltration of the pituitary gland, pr obably by an autoimmune process, leading to a pituitary mass lesion an d/or various degrees of hypopituitarism. The lesion is usually confine d to the adenohypophysis. Posterior pituitary gland or stalk involveme nt is rare, although patients presenting with diabetes insipidus have been reported. We describe a girl aged 13 years 9 months with lymphocy tic hypophysitis who presented with diabetes insipidus and secondary a menorrhea. MRI of the brain revealed a 1 cm enhancing mass in the pitu itary stalk. A biopsy of the mass by right pterional craniotomy showed lymphocytic infiltration without neoplastic cells or granuloma format ion. To our knowledge, this is the youngest reported patient with a di agnosis of lymphocytic hypophysitis. In this case report, her clinical presentation is discussed along with a review of the literature. Conc lusion We present the first childhood case of lymphocytic hypophysitis which is an autoimmune inflammatory disorder of the pituitary gland. Although this is a rare condition in adults, it also needs to be consi dered in the pediatric population. Conservative management is preferre d unless there are signs of increased intracranial pressure. Most impo rtantly. close monitoring for multiple hormone deficiencies is indicat ed in this condition.