Evaluation and Initial Management of Hypopituitarism.

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1. Melinda Pierce, MD* 2. Lisa Madison, MD* 1. *OHSU Department of Pediatrics, Division of Pediatric Endocrinology, Portland, OR. Signs and symptoms of hypopituitarism, in particular diabetes insipidus and decreased linear growth velocity, are frequently overlooked in patients with craniopharyngioma. (1) After completing this article, readers should be able to: 1. Describe the various pathophysiologic causes associated with hypopituitarism, including craniopharyngioma. 2. Recognize the clinical features of pituitary hormone deficiencies. 3. Describe the principles of hormone replacement therapy for hypopituitarism. Hypopituitarism affects between 1 in 4,000 and 1 in 10,000 live births, with increasing incidence with age. (2) The pituitary, sometimes referred to as the master gland, is positioned in the anterior midline of the brain in the sella turcica. The pituitary controls endocrine function through the release of multiple hormones that can have a direct growth effect on target tissue (trophic effects) or stimulate that tissue to release a hormone that has its effects on other tissues (tropic effects). The anterior pituitary forms as an outpouching of the embryonic oral cavity (Rathke pouch). It is responsible for the release of growth hormone (GH), corticotropin, thyrotropin, prolactin, and the gonadotropins luteinizing hormone (LH) and follicle-stimulating hormone (FSH). The posterior pituitary is composed of neuronal projections from the hypothalamus and is responsible for the storage and secretion of oxytocin and antidiuretic hormone (ADH). This article examines the pathologic causes of pituitary dysfunction, their diagnosis, and treatment. ### Congenital Congenital hypopituitarism most often results from genetic or embryologic pathologies. Septo-optic dysplasia (SOD) is the most common congenital cause of hypopituitarism, with an incidence as high as 1 in 10,000. SOD results in variable hormonal deficiencies, absence of the septum pellucidum (Figure), and hypoplasia of the optic nerves, often resulting in blindness. (3) Although SOD can be …