The EEC syndrome (ectrodactyly, ectodermal dysplasia and cleft lip-palate) is a rare disorder inherited as an autosomal dominant trait or can occur sporadically. We describe a case of this syndrome with chronic ocular surface disorder secondary to abnormalities of ocular adnexia.
This is a study on 124 patients who were treated by the authors over a 19-year period. There were 48 male and 76 female patients. The age range of these patients at the time of treatment was between 4 months and 32 years. There was no family history of similar deformity. All of these patients come from a socially low-income group. Fourteen patients had accompanying congenital amputation of fingers, toes, or limbs. Two had oral cleft lip and palate. The surgical treatment was medial orbital wall osteotomy and excision of encephalocele. There were two mortalities and five patients who had complications that needed secondary surgical intervention. Thirty-eight patients are still under follow up without any complaints.