EEC syndrome: A rare entity.

Aftab Ahmed RANA ¹, Suresh CHANDRAN ¹, Joshua GEORGE ²

¹ Department of Paediatric and ² Department of Ophthalmology, RIPAS Hospital, Brunei Darussalam

ABSTRACT

Ectrodactyly, Ectodermal dysplasia and Cleft lip/palate (EEC) syndrome is a rare combination of multiple congenital anomalies.  Although the anomalies are diverse, the underlying problem arise from early embryonic ectodermal tissue defects or insults. We report a case of a baby boy who was born at 33 weeks of gestation with EEC syndrome. He died five months later of aspiration pneumonia. The management requires a multidisciplinary approach and proper parental counseling which should include risk of recurrence in future pregnancies. Prenatal diagnosis is possible by antenatal ultrasound scan at 14-16 weeks of gestation.

Keywords: Cleft lip, cleft palate, ectodermal dysplasia, ectrodactyly

Correspondence author: Aftab Ahmed RANA. Department of Paediatric, RIPAS Hospital, Bandar Seri Begawan BA 1710, Brunei Darussalam.

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Brunei Int Med J. 2011; 7 (4): 220-224