The Clinical, Neuroradiographic, and Endocrinologic Profile of Patients with Bilateral Optic Nerve Hypoplasia

Purpose: The purpose of the study was to expand on ophthalmologic and endocrinologic data and report the neuroimaging findings of 35 patients with bilateral optic nerve hypoplasia (BONH). Methods: A retrospective chart review of 35 children with BONH was conducted. Data on visual acuity, refractive...

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Veröffentlicht in:Ophthalmology (Rochester, Minn.) Minn.), 1997-03, Vol.104 (3), p.493-496
Hauptverfasser: Siatkowski, R. Michael, Sanchez, Juan C., Andrade, Roberto, Alvarez, Anita
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Sprache:eng
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Zusammenfassung:Purpose: The purpose of the study was to expand on ophthalmologic and endocrinologic data and report the neuroimaging findings of 35 patients with bilateral optic nerve hypoplasia (BONH). Methods: A retrospective chart review of 35 children with BONH was conducted. Data on visual acuity, refractive error, and presence of nystagmus and strabismus were collected. Twenty-six children underwent full-endocrinologic evaluation and magnetic resonance imaging or computed tomography scanning. Results: The male:female ratio was 2:1. Ten percent of eyes had visual acuity of 20/60 or better, whereas 34% had no light perception. Eighty-six percent of eyes had acuity less than 20/200, and 80% of patients were legally blind. Most patients (86%) had nystagmus or strabismus or both. Forty-six percent had absence of the septum pellucidum and corpus callosum on neuroimaging. Twenty-seven percent of patients had endocrinologic abnormalities, with growth hormone deficiency being the most common. Panhypopituitarism occurred in 11.5% of children. Conclusions: Although the visual prognosis of children with BONH generally is poor, 10% of such patients have excellent acuity. In contrast to previous reports, endocrine abnormalities were seen in only one quarter of patients, and the full-blown deMorsier syndrome (septo-optic dysplasia with panhypopituitarism) was seen in only 11.5% of patients with BONN.
ISSN:0161-6420
1549-4713
DOI:10.1016/S0161-6420(97)30286-3