title: Clinical, hormonal and imaging findings in 27 children with central diabetes insipidus.
abstract: Clinical, auxological, biological and neuroradiological characteristics of 27 children with central diabetes insipidus (CDI) were retrospectively analysed. Median age at diagnosis was 8.6 years (range: 0.3-16.1 years). Final aetiologies were postsurgical infundibulo-hypophyseal impairment (n=7), cerebral tumour (n=8), Langerhans cell histiocytosis (n=3), septo-optic dysplasia (n=1), ectrodactyly ectodermal dysplasia clefting syndrome (n=1), and idiopathic (n=7). In the non-postsurgical CDI patients, major cumulative and often subtle presenting manifestations were: polyuria (n=20), polydipsia (n=19), fatigue (n=11), nycturia (n=10), growth retardation (n=9), and headache (n=9). An associated antehypophyseal insufficiency, mainly somatotropic, was documented in 11 children. All patients except one who initially had a cerebral tomography, underwent magnetic resonance imaging revealing the lack of the physiological posterior pituitary hyperintense signal. One third of the idiopathic patients initially had a thickened pituitary stalk. All patients with idiopathic CDI were intensively followed up with 3-monthly physical examination, antehypophyseal evaluation, search for tumour markers, and cerebral MRI every 6 months. In one of them the pituitary stalk had normalized after 4.3 years. In one patient Langerhans cell histiocytosis was diagnosed after 7 months of follow-up, and in another patient a malignant teratoma was found after 2.4 years of follow-up.
  
  
  CONCLUSIONS
  CDI may be the early sign of an evolving cerebral process. The association of polyuria-polydipsia should incite a complete endocrine evaluation and a meticulous MRI evaluation of the hypothalamo-hypophyseal region. A rigorous clinical and neuroradiologic follow-up is mandatory to rule out an evolving cerebral process and to detect associated antehypophyseal insufficiencies.
authors.affiliation: Department of Paediatric Endocrinology, Hôpital Universitaire des Enfants Reine Fabiola, Avenue Jean Joseph Crocq 15, 1020 Brussels, Belgium.
authors.forename: Julie
authors.initials: J
authors.lastname: De Buyst
authors.forename: Guy
authors.initials: G
authors.lastname: Massa
authors.forename: Catherine
authors.initials: C
authors.lastname: Christophe
authors.forename: Sylvie
authors.initials: S
authors.lastname: Tenoutasse
authors.forename: Claudine
authors.initials: C
authors.lastname: Heinrichs
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000150:complications / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D020790:Diabetes Insipidus, Neurogenic / Q000175:diagnosis* / Q000209:etiology; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology; D012189:Retrospective Studies