Craniofacial and dental dysmorphology in patients with median facial dysplasia: long-term follow-up.

摘要

Median facial dysplasia affects a subset of patients with cleft lip and palate exhibiting certain characteristics of median facial structure deficiencies without definable gross abnormalities of the brain. The aim of this study was to describe the craniofacial and dental morphology of almost skeletally mature patients with median facial dysplasia. Patients were selected for this retrospective study if they were diagnosed with median facial dysplasia and >/=15 years old. The craniofacial and dental morphology was evaluated by analysing cephalometric and panoramic radiographs. This sample of median facial dysplasia patients (9 males and 11 females; 6 unilateral and 14 bilateral clefts) had a mean age of 16.7+/-1.9 years. Controls were age-, sex-, cleft type-matched, and nonsyndromic patients. The results showed that in patients with median facial dysplasia, the anterior cranial base and midface were shorter than in controls. The median facial dysplasia inter-orbital distance was shorter and the nasal bone was more retrusive than in controls. All patients with median facial dysplasia had several missing permanent teeth. These features require extensive surgical, orthodontic, and dental rehabilitation procedures.