Ectrodactyly-ectodermal dysplasia-clefting syndrome-plastic surgeon's considerations.

BACKGROUND

Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome is a rare autosomally dominant disorder phenotypically characterized by specific abnormalities of the hand, feet, and orofacial region coexisting with ectodermal dysplasia features. The purpose of this study was to describe the plastic surgeon's approach to EEC treatment by retrospective analysis of the course of surgical treatment.

METHODS

Medical documentation of 10 patients with EEC syndrome treated in the Department of Plastic Surgery in Polanica Zdroj in the years 1976-2008 was given retrospective analysis.

RESULTS

In the analyzed group of patients, various levels of intensity of ectodermal dysplasia symptoms were confirmed, and in every case, severe forms of clefts were observed. Intensity of cleft malformation, but foremost belated and exacerbated healing, and coexistence of ectodermal dysplasia were the evident cause of more frequent complications and occurrence of greater postcleft deformations. Treatment of this hand and feet malformation is by its nature hampered, and procedures between subsequent stages of cleft surgery are performed. In patients with nasolacrimal duct atresia, dilatation or dacryocystorhinostomy was performed, which effectively improved patients' quality of life.

CONCLUSIONS

In reference to plastic surgery point of view, treatment of coexistence with ectodermal dysplasia malformations in EEC syndrome demands more individual therapeutic approach. The presence of ectodermal dysplasia symptoms increases the risk of complications in cleft surgery and treatment of hand and foot deformities or orbital area malformations. Severity of cleft malformation requires surgery supported by experience and embracing the necessity of performing a greater number of more complex corrective procedures.