Anterior chamber cleavage syndrome (reese-ellsworth syndrome; peters-plus syndrome)

General: Abnormalities in the embryologic development of the anterior chamber due to failure of normal migration of mesode 151b14b rmal cells across the anterior segment of the eye or failure of later differentiation of the mesodermal elements; various conditions described as congenital: central anterior synechiae, persistent mesenchymal tissue in the chamber angle, posterior embryotoxon, congenital corneal hyaline membrane, posterior marginal dysplasia, prominent Schwalbe line, mesodermal dysgenesis, and internal corneal ulcer seem all to fall in this same category of the anterior chamber cleavage syndrome; condition is present at birth; about 80% are bilateral; autosomal dominant inheritance; may be associated with congenital sensory neuropathy and ichthyosis.

Ocular: Increased intraocular pressure; adhesions between the iris and cornea; persistence of mesenchymal tissue in the chamber angle; usually shallow anterior chamber; iris coloboma and hypoplasia; prominent Schwalbe ring; contiguous hyaloid membrane; corneal opacities of various density with or without edema, usually at the site of iris adhesion; anterior pole cataract; remains of hyaloid artery.

Clinical: Dental anomalies; mental retardation; cleft palate; syndactyly; craniofacial dysostosis; myotonic dystrophy.

Kolbert GS, Seelenfreund M. Sclerocornea, anterior cleavage syndrome and trisomy 18. Ann Ophthalmol 1970; 2:26.

Merin S. Anterior chamber cleavage syndromes. In: Merin S, ed. Inherited Eye Diseases, Diagnosis and Clinical management. New York: Marcel Dekker, 1991:74-78.

Quinlivan R, et al. Congenital sensory neuropathy in association with ichthyosis and anterior chamber cleavage syndrome. Neuromuscul Disord 1993; 3:217-221.

Reese AB, Ellsworth RM. The anterior chamber cleavage syndrome. Arch Ophthalmol 1966; 75:307.