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June 1993

Ectodermal Dysplasia, Ectrodactyly, and Clefting Syndrome and Bilateral Retinal Detachment

Author Affiliations

Boston, Mass; Bronx, NY

Arch Ophthalmol. 1993;111(6):734. doi:10.1001/archopht.1993.01090060020009

The complex of congenital malformations, including ectodermal dysplasia, ectrodactyly, and clefting of the lip and palate is known as the EEC syndrome. Urinary tract involvement is frequent and may be added to the acronym (EECUT). The disease has variable presentations, with incomplete forms (two of three malformations) being described herein. The ocular malformations are generally confined to the ocular adnexa and external eye.1 One case has been reported of a patient with EEC syndrome with a total retinal detachment due to a giant tear.2 We describe herein a patient with the incomplete form of the EECUT syndrome with bilateral total traction detachment that resembled stage 5 retinopathy of prematurity (ROP).

Report of a Case.  —The patient was a 2.5-kg product of a full-term uncomplicated pregnancy that had been induced by artificial insemination. No supplemental oxygen was required. There was sparse development of the hair,

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