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PEDIATRICS Vol. 100 No. 4 October 1997, pp. 667-670

Endocrinologic Disorders and Optic Pathway Gliomas in Children With Neurofibromatosis Type 1

Received Dec 3, 1996; accepted Feb 26, 1997.

Marjon H. Cnossen*, Dagger , Esther N. Stam*, Linda C. M. G. Cooiman*, Huibert J. Simonsz§, Hans Stroinkparallel , Arnold P. Oranje, Dicky J. J. HalleyDagger , Arja de Goede-Bolder*, Martinus F. NiermeijerDagger , and Sabine M. P. F. de Muinck Keizer-Schrama#

From the Departments of * Pediatrics, Dagger  Clinical Genetics, § Ophthalmology, parallel  Neurology,  Dermatology, and # Pediatric Endocrinology, University Hospital Sophia/Dijkzigt, Erasmus University, Rotterdam, The Netherlands.

Objective.  To establish the prevalence of endocrinologic disorders in children with neurofibromatosis type 1 (NF1) and the relationship between these disorders and cerebral abnormalities on magnetic resonance imaging.

Design.  A prospective follow-up study.

Setting.  A multidisciplinary neurofibromatosis clinic.

Patients.  A total of 122 children diagnosed with NF1 according to diagnostic criteria set by the National Institutes of Health.

Results.  Central precocious puberty (CPP) was diagnosed in 3 children and growth hormone deficiency (GHD) in 3 children. Optic pathway gliomas were observed in 15 children; in 9 of the 15 cases, the optic chiasm was involved. Of the 3 children with CPP, only 1 showed a chiasma glioma on magnetic resonance imaging. In 1 case with GHD, an optic chiasm glioma was detected on neuroimaging. Two of the 9 children with an optic chiasm glioma presented with CPP or GHD.

Conclusions.  It has been suggested that CPP in children with NF1 is found exclusively in the presence of a chiasma glioma. We conclude that chiasma glioma may not be obligatory in children with NF1 and CPP or GHD. Moreover, we report a prevalence of GHD in children with NF1 of 2.5%, which has not been established earlier.

Key words: neurofibromatosis type 1, endocrinologic disorder, central precocious puberty, growth hormone deficiency, optic pathway glioma.


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