OPTIC-NERVE HYPOPLASIA - ABSENCE OF POSTERIOR PITUITARY BRIGHT SIGNALON MAGNETIC-RESONANCE-IMAGING CORRELATES WITH DIABETES-INSIPIDUS

PURPOSE: Magnetic resonance imaging has been used to examine children with optic nerve hypoplasia for pituitary abnormalities who may be at risk for anterior pituitary hormonal deficiencies. We correlated the s ellar and optic pathway anatomic findings on magnetic resonance imagin g in children with optic nerve hypoplasia with findings from their end ocrinologic and ophthalmologic examinations to determine whether magne tic resonance imaging findings predict anterior and posterior pituitar y dysfunction. METHODS: A retrospective review identified five childre n with optic nerve hypoplasia and endocrinopathy who also underwent hi gh resolution volumetric magnetic resonance imaging. RESULTS: All chil dren had severe bilateral optic nerve hypoplasia and anterior pituitar y hormone deficiencies. Three children had no recognizable intrasellar or ectopic posterior pituitary bright spot on magnetic resonance imag ing; all had clinical evidence of diabetes insipidus. Two patients wit h a recognizable but ectopic posterior pituitary did not have diabetes insipidus. CONCLUSION: Children with optic nerve hypoplasia and no re cognizable posterior lobe of the pituitary gland on magnetic resonance imaging are at risk for both anterior and posterior pituitary dysfunc tion, whereas those with a posterior lobe on magnetic resonance imagin g appear to have intact posterior pituitary function.