Abstract
In up to 25% of cases of children with central diabetes insipidus no organic cause can be documented. We present three boys (age 2.2, 2.3 and 6 years at diagnosis) with acute onset central diabetes insipidus, in whom the only pathological finding using MRI was a thickened central part of the pituitary stalk (>2.5 mm). Recent reports demonstrate similar MRI findings in adults with Langerhans cell histiocytosis (LCH), sarcoidosis, or tuberculosis, and in children with proven LCH and diabetes insipidus. In those adults with LCH, the pituitary stalk lesion has been histologically verified as a sequela of LCH. In contrast, in two of our three patients pituitary stalk thickening preceded the typical peripheral lesions of LCH by several months, whereas in the third patient there is as yet no evidence of systemic disease. We conclude that thickening of the central part of the pituitary stalk might represent the first manifestations of LCH clinically presenting with diabetes insipidus. MRI investigation of the pituitary stalk in children in children with unexplained central diabetes insipidus and accurate follow up in patients with thickening of the pituitary stalk in necessary to avoid missing other manifestations of a systemic disease.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
Abbreviations
- DI:
-
Diabetes inspidius
- DDAVP:
-
1-desamino-8-D-arginine vasopression
- LCH:
-
Langerhans cell histiocytosis
References
Buchfelder M, Fahlbusch R, Walther M, et al (1989) Endocrine disturbances in suprasellar germinomas. Acta Endocrinol (Copenh) 120:337–342
Cacciari E, Zucchini S, Carla G, et al (1990) Endocrine function and morphological findings in patients with disorders of the hypothalamo-pituitary area: a study with magnetic resonance. Arch Dis Child 65:1199–1202
Chiumello G, Di Natale B, Pellini C, et al (1989) Magnetic resonance imaging in diabetes insipidus (letter). Lancet I (8643): 901
Chu T, D'Angio GJ, Favara B, et al (1987) Histiocytosis syndromes in children. Lancet I:208–209
Dunger DB, Broadbent V, Yeoman E, et al (1989) The frequency and natural history of Diabetes insipidus in children with Langerhans cell Histiocytosis. N Engl J Med 321:1157–1162
Gnehm HE, Bernasconi S, Zachmann M (1979) Posttraumatic anterior pituitary insufficiency in childhood. Helv Paediatr Acta 34:529–532
Graif M, Pennock JM (1986) MR Imaging of Histiocytosis X in the central nervous system. Am J Neuro Radiol 7:21–23
Greger N, Kirkland RT, Clayton GW, et al (1986) Central diabetes insipidus. Am J Dis Child 140:551–554
Maghnie M, Villa A, Arico M, et al (1992) Correlation between magnetic resonance imaging of posterior pituitary and neurophypophyseal function in children with diabetes insipidus. J Clin Endocrinol Metab 74:795–800
Miyamoto S, Sasaki N, Tanabe Y (1991) Magnetic resonance imaging in familial central diabetes insipidus. Neuroradiology 33:272–273
Pomarède R, Czernichow P, Rappaport R, et al (1980) Le diabète insipide pitressonsensible de l'enfant. Arch Fr Pediatr 37: 37–44
Prader A, Largo RH, Molinari L, et al (1989) Physical growth of Swiss children from birth to 20 years of age. First Zurich longitudinal study of growth and develoment. Helv Paediatr Acta [Suppl] 52:1–125
Rosenfield NS, Abrahams J, Komp D (1990) Brain MR in patients with Langerhans cell histiocytosis: findings and enhancement with Gd-DTPA Pediatr Radiol 20:433–436
Tien R, Kucharczyk J, Kucharczyk W (1991) MR imaging of the brain in patients with diabetes insipidus. Am J Radiol 157: 123–132
Tien RD, Newton HT, McDermott MW, et al (1990) Thickening pituitary stalk on MR images in patients with diabetes insipidus and Langerhans cell histiocytosis. Am J Neuro Radiol 11:703–708
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Schmitt, S., Wichmann, W., Martin, E. et al. Pituitary stalk thickening with diabetes insipidus preceding typical manifestations of Langerhans cell histiocytosis in children. Eur J Pediatr 152, 399–401 (1993). https://doi.org/10.1007/BF01955896
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01955896