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Ocular Manifestations of Donnai-Barrow Syndrome

Department of Ophthalmology and Visual Sciences, University of Nebraska Medical Center, Omaha, Nebraska

Thomas Hejkal, MD

Department of Ophthalmology and Visual Sciences, University of Nebraska Medical Center, Omaha, Nebraska

Allen Katz, CRA, COT

Department of Ophthalmology and Visual Sciences, University of Nebraska Medical Center, Omaha, Nebraska

Eyal Margalit, MD, PhD

Department of Ophthalmology and Visual Sciences, University of Nebraska Medical Center, Omaha, Nebraska, emargalit{at}unmc.edu

Donnai-Barrow syndrome is a rare autosomal recessive disorder first described in 1993. This report presents ocular manifestations of this rare autosomal recessive disorder through 2 additional cases. Ocular features include hypertelorism, down-slanting palpebral fissures, iris coloboma, high myopia, and retinal detachment. The extreme congenital myopia in these patients is a significant risk factor for retinal detachment, and prophylactic barrier photocoagulation may be considered to prevent retinal detachment and its associated functional disability.

Key Words: Donnai-Barrow syndrome • high myopia • retinal detachment

Journal of Child Neurology, Vol. 22, No. 4, 462-464 (2007)
DOI: 10.1177/0883073807301933


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