Introduction: Central Diabetes Insipitus (CDI) results from the inability to secrete ADH secreted by the neurohypophysis system to control water-electrolyte metabolism. In the etiology of CDI many congenital and acquired CNS tumors, infiltrative diseases,infections, autoimmune events, head trauma and idiopathic can be responsible. In this article, a child case with CDI due to intracranial occurence which is very rare in etiology is presented and the approach and follow up are discussed.
Case: Twenty-month-old girl presented with excessive thirst and water intake and frequent urination for about three or four months. The general condition of the patient was good, there were normal physical examination findings. and the somatic development was compatible with age. It was learnt that there were no traits in the medical history.
Results: The urine excretion of case was found to be polyuric (11.68 ml/kg per hour) and the ratio of urine osmolarity to serum osmolarity was found to be 0.27. Serum ADH level of <0.5 pmol/l, and observed >50% increase in urine osmolarity was observed after desmopressin administration in case. In the contrast-enhanced pituitary MRI, protein and lipid-containing semisolid structures in the size of 2.5×2 mm were noted in the nasopharyngeal localization, suggesting that this formation led to narrowing of the neurohypophysis volume. This appearance was evaluated as likely to be choristoma-hibernoma.CDI was diagnosed in the light of these data.Desmopressin treatment was begun.
Discussion: Hibernoma is a benign soft-tissue well-limited tumor that develops from brownish fatty tissue that is extremely rare in childhood and is observed mostly in adults. Up to now, approximately 100 cases have been reported in the literature and 10% are located in the head and neck region. Although they are benign total excision is suggested when the tumor reaches a certain size due to the possibility of reaching very large dimensions and pressurizing the surrounding tissues. This lesion, which is detected in a very small size in the nasopharynx region of our case, by narrowing the neurohypophysis and causing central diabetes insiput is very important in terms of it being the first in literature. On the other hand, our case is valuable in that it shows the value of imaging in the approach of diagnosis and treatment of SDI We also think that the follow-up of this patient in terms of mass size is a necessity because it may have cautionary value in terms of other hormone defects that can develop.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology