Congenital hypopituitarism variable presentation for the same diagnosis

Background: Congenital hypopituitarism is a rare clinical syndrome of deficiency in pituitary hormones. It is a life threatening. Design and selling: A retrospective hospital- based study was conducted at King Khalid University Hospital (KKUH), Riyadh, Saudi Arabia during the period January 1989 and December 2014 Methods: Medical records of patient who were diagnosed to have congenital hypopituitarism were retrospectively reviwed. Data included age, sex, clinical presentation and results of the relevant laboratory and radiological investigations. Results: Eight patients were diagnosed to have congenital hypopituitarism. Hypoglycemia was the commonest presentation in five (62.5%) patients. Three (37.5%) patients presented with micropenis and bilateral undescended testicle with neonatal cholestasis was the clinical presentation in one. Conclusion: Congenital hypopituitarism is a rare disorders. It had variable presentations. Hypoglycemia, micropenis in boys and neonatal cholestasis were among the commonest.