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Brain malformations common cause of pediatric central diabetes insipidus
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Pediatric central diabetes insipidus is more commonly caused by brain malformations than previously thought, according to recent study findings published in The Journal of Endocrinology & Metabolism.
David Werny, MD, MPH, of the University of Washington and Seattle Children’s Hospital, and colleagues evaluated 147 children (mean age at diagnosis, 7 years; mean follow-up, 6.2 years) with central diabetes insipidus from 2000 to 2013 to determine the different causes of the condition.
Overall, the most common diagnosis was craniopharyngioma (25.2%), followed by septo-optic dysplasia (14.3%) and Langerhans cell histiocytosis (12.2%). A brain malformation was the underlying cause of central diabetes insipidus in 24% of patients. After age 2 years, 33% of patients with a congenital anatomic cause were diagnosed with central diabetes insipidus.
The most common causes of central diabetes insipidus were acquired infiltrative and tumor etiologies (56.7%). Familial central diabetes insipidus or a presumed genetic cause was identified in 7% of patients.
Central diabetes insipidus was diagnosed in 29 patients without any medical conditions known to be associated with it. Abnormal initial MRI or clinical evaluation suggesting an underlying cause was present in 24% of these patients.
Twenty-two patients had no known cause for central diabetes insipidus and were considered initially idiopathic.
No initial cause of central diabetes insipidus could be identified in 12 patients, and they had normal pituitary stalk thickness on the initial MRI.
Similar rates of anterior pituitary hormone deficiencies in patients with anatomic malformations as patients with tumor/infiltrative forms of central diabetes insipidus were found (P = .09). Patients with genetic/familial central diabetes insipidus had no anterior pituitary hormone deficiencies compared with 39% of those with idiopathic central diabetes insipidus.
Throughout the whole patient group, 60% had GH deficiency, 59% has thyroid-stimulating hormone deficiency and 57% had adrenocorticotropic hormone deficiency. Normal posterior pituitary bright spot was identified in 13% of patients despite having central diabetes insipidus.
“We have confirmed our suspicion that idiopathic [central diabetes insipidus] is relatively rare and anatomic brain malformations are a more common cause of pediatric [central diabetes insipidus],” the researchers wrote. “Patients with anatomic brain malformations have a high rate of [anterior pituitary deficiencies], and many have persistence of the [posterior pituitary bright spot]. Our follow-up of patients with idiopathic [central diabetes insipidus] shows that providers can consider less frequent MR imaging after 3 years from diagnosis, and that having a standardized assessment of the infundibulum on the initial MRI may help predict later development of [Langerhans cell histiocytosis] or germinoma.” – by Amber Cox
Disclosure: The researchers report no relevant financial disclosures. The study was funded in part by the Nordstrom family.
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