ESPE Abstracts

Background: Central diabetes insipidus (CDI) is a rare disorder in the pediatric population. CDI may be the first symptom of a brain tumor, metastatic lesions, or granulomas. The close follow-up of patients with CDI may early detect pathologic processes, especially intracranial tumors.

Methods: We present four pediatric patients diagnosed with CDI of different etiology.

Patient 1: A 5-year-old girl with 10 months history of polydipsia and polyuria was diagnosed with CDI. At the time of diagnosis, moderately increased β-hCG, thickened pituitary stalk (PS), and heterogeneous signal in the anterior lobe in MRI was observed. After 1 month, multihormonal pituitary insufficiency (PI) developed, and GCT of a suprasellar region secreting β-HCG was diagnosed. Four years after completing oncologic therapy, the girl is disease-free, but panhypopituitarism is still present.

Patient 2: A 13.5-year-old girl with a history of polydipsia and polyuria from early childhood. The multihormonal PI, including CDI, was diagnosed. Based on the patient's overall clinical picture (PI, features of optic nerves hypoplasia, and midline brain malformations like septum pellucidum deviation and thinning of the corpus callosum), septo-optic dysplasia diagnosis was brought up.

Patient 3: A 3 years-old girl with chronic otitis media history, polydipsia, and polyuria. MRI of the pituitary gland and a clinical picture correlated with CDI diagnosis. A few months later, the patient was admitted to diagnose a right parotid gland region lesion. Further diagnostics revealed Langerhans cell histiocytosis.

Patient 4: A 10 years-old boy with a history of polydipsia and polyuria for 3 months. Due to laboratory tests and MRI, CDI was diagnosed. Coexisting GH deficiency was present, although his height was normal. At the age of 11.25 years, the control MRI showed thickening of the PS. After the next 6 months, a pathological change in PS and the anterior lobe was described. Laboratory tests revealed hyperprolactinemia and hypothyroidism. Although β-hCG and AFP in multiple laboratory tests were normal, GCT was suspected.

Conclusions: CDI was the first manifestation of a tumor or granulomas in all presented patients with acquired pathology. Lack of posterior pituitary bright spot was present in all cases and is an inherent part of CDI diagnosis. The coexistence of other pituitary hormones deficiencies suggests a more complicated diagnostically problem. Because in the last case, from the CDI diagnosis to the occurrence of pathological change in PS, 1.5 years have passed, this highlights the need for a high clinical suspicion in patients with CDI.