ESPE Abstracts

Central diabetes insipidus (CDI) is the end result of a number of disorders that affect the hypothalamic-neurohypophyseal region (HPR). The diagnosis of the underlying condition despite the use of new biochemical parameters including copetin is challenging and raises several concerns for patients and parents as it requires long-term follow-up. Thus, a proper etiological diagnosis can be achieved via a series of steps that start with clinical observations and then progress to more sophisticated techniques. Thickening of the pituitary stalk is the most common finding on MRI scans in several inflammatory conditions and autoimmune diseases affecting the HPR or germinoma, but it is not specific to any single subtype. A progressive increase in the size of the anterior pituitary gland should alert physicians to the possibility that a germinoma is present, whereas a decrease can suggest the presence of an inflammatory or autoimmune process. Tumor-associated CDI is uncommon in children younger than 5 years of age. Most children with acquired CDI and thickened pituitary stalk have anterior pituitary hormone deficiencies during follow-up. Biopsy of enlarged pituitary stalk should be reserved for patients with hypothalamic-pituitary mass and progressive thickening of the pituitary stalk since spontaneous recovery may occur. Serial MRI and integration with T2-DRIVE images and recent “mismatch imaging pattern” provides an important framework towards a better diagnosis of pituitary lesions and MRI STIR Technology is promising for the early identification of disease specific-dependent CDI. We also underscore the paramount importance of long-term clinical follow-up and reassessment of endocrine function as well as monitoring of signs or symptoms of Langerhans cell histiocytosis reactivation over time.