Clin Pediatr Hematol Oncol.  2006 Apr;13(1):72-79.

Central Diabetes Insipidus as a Manifestation of Langerhans Cell Histiocytosis: Report of 3 Cases with Literature Review

  • 1Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea.


Central diabetes insipidus (CDI) is the most common manifestation of central nervous system involvement in Langerhans cell histiocytosis (LCH). Therefore, in patients with endocrinologically proven CDI, a comprehensive diagnostic evaluation is crucial to identify possible extracranial sites of LCH. However, once established, DI is mostly associated with life-long dependence on antidiuretic hormone (ADH) replacement therapy. We here report 3 cases of CDI in association with LCH, diagnosed by a water deprivation test. Two patients who presented with CDI as the initial manifestation showed thickened pituitary stalk on the sellar MRI. The 3rd patient developed CDI as a late sequela of LCH. Regression of pituitary thickness on MRI did not correlate with the clinical recovery of CDI, as shown in 2 cases.


Langerhans cell histiocytosis; Diabetes insipidus; Pituitary stalk thickening; MRI

MeSH Terms

Central Nervous System
Diabetes Insipidus
Diabetes Insipidus, Neurogenic*
Histiocytosis, Langerhans-Cell*
Magnetic Resonance Imaging
Pituitary Gland
Water Deprivation
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