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. Conclusion: 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.
Similar content being viewed by others
Abbreviations
- CDI:
-
central diabetes insipidus
- MRI:
-
magnetic resonance imaging
- HP:
-
hypothalamo-pituitary
- EEC:
-
ectrodactyly ectodermal dysplasia and cleft lip/palate syndrome
- SOD:
-
septooptic dysplasia
- TRH:
-
thyreotropin releasing hormone
- GnRH:
-
gonadotropin releasing hormone
- AFP:
-
alpha foetoprotein
- β HCG:
-
beta human chorionic gonadotrophin
- CAT scan:
-
computed tomography scan
- CSF:
-
cerebral spinal fluid
- GHD:
-
growth hormone deficiency
- LH:
-
luteinizing hormone
- FSH:
-
follicle stimulating hormone
References
Argyropoulou M, Perignon F, Brunelle F, Brauner R, Rappaport R (1991) Height of normal pituitary gland as a function of age evaluated by magnetic resonance imaging in children. Pediatr Radiol 21:247–249
Cheetham T, Baylis PH (2002) Diabetes insipidus in children: Pathophysiology, diagnosis and management. Pediatr Drugs 4:785–796
Czernichow P, Pomarede R, Brauner R, Rappaport R (1985) Neurogenic diabetes insipidus in children. Front Horm Res 13:190–209
Czernichow P, Garel C, Leger J (2000) Thickened pituitary stalk on magnetic resonance imaging in children with central diabetes insipidus. Horm Res 53:61–64
Fujisawa I, Nishimura K, Asato R, Togashi K, Itoh K, Noma S, Kawamura Y, Sago T, Minami S, Nakano Y, Itoh H, Torizuka K (1987) Posterior lobe of the pituitary in diabetes insipidus: MR findings. J Comput Assist Tomogr 11:221–225
Greger NG, Kirkland RT, Clayton GW, Kirkland JL (1986) Central diabetes insipidus. 22 years’ experience. AJDC 140:551–554
Imura H, Nakao K, Shimatsu A, Ogawa Y, Sando T, Fujisawa I, Yamaba H (1993) Lymphocytic infundibuloneurohypophysitis as a cause of central diabetes insipidus. N Engl J Med 329:683–689
Leger J, Velasquez A, Garel C, Hassan M, Czernichow P (1999) Thickened pituitary stalk on magnetic resonance imaging in children with central diabetes insipidus. J Clin Endocrinol Metab 84:1954–1960
Maghnie M (2003) Diabetes insipidus. Horm Res 59(Suppl 1):42–54
Maghnie M, Cosi G, Genovese E, Manca-Bitti ML, Cohen A, Zecca S, Tinelli C, Galluci M, Bernasconi S, Boscherini B, Severi F, Arico M (2000) Central diabetes insipidus in children and young adults. N Engl J Med 343:998–1007
Maghnie M, Sommaruga MG, Beluffi G, Severi F (1993) Role of MR imaging in the evaluation of the functional status of the posterior pituitary gland: the view of a pediatric endocrinologist. AJNR 14:1443–1445
Maghnie M, Triulzi F, Larizza D, Preti P, Priora C, Scotti G, Severi F (1991) Hypothalamic-pituitary dysfunction in growth hormone-deficient patients with pituitary abnormalities. J Clin Endocrinol Metab 73:79–83
Maghnie M, Villa A, Arico M, Larizza D, Pezzotta S, Beluffi G, Genovese E, Severi F (1992) Correlation between magnetic resonance imaging of posterior pituitary and neurohypophyseal function in children with diabetes insipidus. J Clin Endocrinol Metab 74:795–800
Majzoub JA, Muglia LJ (2003) Disorders of water homeostasis. In: Lifshitz F (ed) Pediatric endocrinology, 4th edn. Marcel Dekker, New York, pp 755–785
Mootha SL, Barkovich AJ, Grumbach MM, Edwards MS, Gitelman SE, Kaplan SL, Conte FA (1997) Idiopathic hypothalamic diabetes insipidus, pituitary stalk thickening, and the occult intracranial germinoma in children and adolescents. J Clin Endocrinol Metab 82:1362–1367
Pomarede R, Czernichow P, Rappaport R, Royer P (1980) Le diabète insipide pitrosensible de l’enfant. Arch Fr Pediatr 37:37–44
Prosch H, Grois N, Prayer D, Waldhauser F, Steiner M, Minkov M, Gadner H (2004) Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis. Pediatr Blood Cancer 43:594–599
Pivonello R, De Bellis A, Faggiano A, Di Salle F, Petretta M, Di Somma C, Perrino S, Altucci P, Bizzaro A, Bellastella A, Lombardi G, Colao A (2003) Central diabetes insipidus and autoimmunity: relationship between the occurrence of autoantibodies to arginin vacopressin-secreting cells and clinical, immunological, and radiological features in a large cohort of patients with central diabetes insipidus of known and unknown etiology. J Clin Endocrinol Metab 88:1629–1636
Robertson GL (1995) Diabetes insipidus. Endocrinol Metab Clin NA 24:549–572
Robertson GL (2001) Disorders of water balance. In: Brook CGD, Hindmarsh PC (eds) Clinical pediatric endocrinology, 4th edn. Blackwell Science, Oxford, pp 193–221
Seregni E, Massimino M, Nerini MS, Pallotti F, van der Hiel E, Cefalo G, Spreafico F, Fossati F, Bombardieri E (2002) Serum and cerebrospinal fluid human chorionic gonadotrophin (hCG) and alfa-fetoprotein (AFP) in intracranial germ cell tumors. Int J Biol Markers 17:112–118
Sklar CA, Grumbach MM, Kaplan SL, Conte FA (1981) Hormonal and metabolic abnormalities associated with central nervous system germinoma in children and adolescents and the effect of therapy: report of 10 patients. J Clin Endocrinol Metab 52:9–16
Van Maldergem L, Gillerot Y, Vamos E, Toppet M, Watillon P, Van Vliet G (1992) Vasopressin and gonadotrophin deficiency in a boy with the ectrodactyly-ectodermal dysplasia-clefting syndrome. Acta Pediatr 81:365–367
Wang LC, Cohen ME, Duffner PK (1994) Etiologies of central diabetes insipidus in children. Pediatr Neurol 11:273–277
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
De Buyst, J., Massa, G., Christophe, C. et al. Clinical, hormonal and imaging findings in 27 children with central diabetes insipidus. Eur J Pediatr 166, 43–49 (2007). https://doi.org/10.1007/s00431-006-0206-0
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00431-006-0206-0