Midface growth in patients with ectrodactyly-ectodermal dysplasia-clefting syndrome

Ectrodactyly-ectodermal dysplasia-clefting syndrome is an autosomal dominant disorder involving abnormalities of the hands, feet, skin, and teeth and clefts of the lip and palate. Patients with this syndrome have abnormal facial features--namely, clefts of the lip and palate--and abnormal dentition, which could affect the growth of their midface region. The purpose of this study was to describe characteristics of midface growth in patients with this syndrome.

A retrospective chart review was conducted for eight postoperative patients seen at the cleft clinic at The Children's Hospital of Philadelphia between 1987 and 2004. Clinical notes and operative reports were reviewed. The surgeon's assessment and cephalometric measurements were analyzed. The cephalometric values were compared with Bolton standards for normal facial growth to determine deficiency.

All eight patients displayed midface disproportion. Five of the eight were found to have some degree of midface retrusion. The upper anterior facial height was found to be deficient for half of the patients. Six patients had deficient lower facial height and four had deficient total face height. Two of these patients have undergone midface distraction osteogenesis.

The authors' study identified a subset of patients within the cleft clinic who carry the diagnosis of ectrodactyly-ectodermal dysplasia-clefting syndrome. The patients tend to have poor dentition that, coupled with clefts of the lip and palate, inhibits normal midface growth and can require surgical correction. The need for orthognathic surgery seems to be greater than that seen in patients who have clefts of the lip and palate but do not carry the syndrome.