Objective: To review the possible craniomaxillofacial deformative consequen
ces associated with ectodermal dysplasias and embryonic malformations, whic
h include dental ageneses.
Setting: Oral and Maxillofacial Surgery Department, University Hospital, Li
lle, France.
Patients: Sixteen patients (seven boys and nine girls, aged 4 to 34 years)
with pure ectodermal dysplasia (no ectodermal dysplasia syndromes).
Interventions: All patients had a clinical examination. Seven (two boys and
five girls, aged 4 to 25 years) had undergone plaster casts and radiograph
ic and Delaire's cephalometric studies before being treated.
Main Outcome Measures: All patients had tooth ageneses (from hypodontia to
anodontla), associated with cutaneous dyshidrosis and hair and nail dystrop
hy. Most of them had a short face, with an unusual facial concavity, a maxi
llary retrusion, and a relative mandibular protrusion.
Management Results and Discussion: Depending on their ages and their orthop
edic abnormalities, patients underwent either dental or prosthodontic, orth
odontic, orthopedic, orthognathic, or implant treatment. So as not to inter
fere with the growth pattern, we preferred to reserve Implant and orthognat
hic surgery for full-grown cases.
Conclusions: Oral and maxillofacial surgeons must undertake a comprehensive
approach to these patients to improve their dental, masticatory, growing,
and orthognathic conditions.