Ectrodactyly, ectodermal dysplasia with cleft lip and palate: a case report
Keywords:
ectrodactyly, ectodermal dysplasia, cleft lip, cleft palateAbstract
Ectrodactyly- ectodermal dysplasia-cleft lip or palate syndrome(EEC syndrome) is characterized by the triad of ectrodactyly, ectodermal dysplasia and facial clefting (lip/palate). However, manifestation of all the three anomalies is very uncommon. The incomplete forms, with absence of one or more of the cardinal signs, are often noted. Neither of the two cases, recently reported from India, had all the three anomalies. We report a sporadic case of EEC with all three classical features.ÂReferences
Cockayne EA. Cleft palate-lip, hare lip, dacryocystitis and cleft hand and foot. Biometrika 1936; 28: 60-3.
Robinson GC, Wildervanck LS, Chiang TP. Ectrodactyly, ectodermal dysplasia and cleft lip - palate syndrome: Its association with conducting hearing loss. J Pediatr 1973; 82: 107-9.
Thakkar S, Marfatia Y. EEC syndrome sans clefting: variable clinical presentations in a family. Indian J Dermatol Venereol Leprol 2007; 73: 46-8.
Cyriac MJ, Lashpa E. Lobster-claw hand: A manifestation of EEC syndrome. Indian J Dermatol Venereol Leprol 2006; 72: 54-6.
Buss PW, Hughes HE, Clarke A. Twenty four cases of the EEC syndrome: Clinical presentation and management. J Med Genet 1995; 32: 716-23.
Wallis CE. Ectrodactyly (split hand/split foot) and ectodermal dysplasia with normal lip and palate in four generation kindred. Clin Genet 1988; 34: 252-7.
