The Gist of a Rathke’s Cleft Cyst

A child with a Rathke’s cleft cyst (RCC) can present asymptomatically, or with headaches, visual complaints, and pituitary hormone deficiencies. The diagnosis must be differentiated from a cystic craniopharyngioma as well as adenomas, arachnoid cysts, and xanthogranulomas. A computerized tomography (CT) scan can help to distinguish a RCC from a cystic craniopharyngioma where calcifications are common. A RCC can be monitored conservatively if neuro-ophthalmological evaluation is normal and there is no additional central nervous system (CNS) concerns. We showcase a 9-year-old male who presented with polyuria, polydipsia, fatigue, and growth failure. He is found to have a RCC with associated diabetes insipidus (DI), central hypothyroidism, hyperprolactinemia, and growth hormone (GH) deficiency. Over the ensuing 3 years, his RCC continued to decrease in size as did the degree of hyperprolactinemia. However, the hypothyroidism, GH deficiency, and DI continued. This chapter reviews the presentation and diagnosis of RCC and considerations for therapy.