We report on mandibulofacial dysostosis in 2 brothers born to normal nonconsanguineous parents, and a girl (F = 1/16) born to normal consanguineous parents. Normal clinical, skeletal, audiologic, and cephalometric studies in the parents, as well as the absence of limb anomalies in these children, exclude the autosomal recessive (Nager and Genée-Widemann) mandibulofacial dysostoses. The data of the present patients associated with the few additional reports on mandibulofacial dysostosis recurring in sibs, suggest the possibility of an autosomal recessive Treacher Collins-like mandibulofacial dysostosis.
Objective
To compare dental arch relationships in children with unilateral cleft lip and palate (UCLP) between two surgical techniques for repair of cleft lip/palate and two ages of palate repair.
Settings and Sample
Dental models were taken for a group of 448 subjects at a mean age of 7 years and were evaluated by means of the Goslon Yardstick. The patients studied consisted of an initial group of 673 infants with complete UCLP randomized into 8 study groups according to lip repair procedures (Millard versus Spina techniques); palate repair procedures (von Langenbeck versus Furlow techniques); and palate repair timing (early: 9 to 12 months versus late: 15‐18 months).
Methods
Four surgeons performed all surgeries. Dependent variables included the following: lip repair technique, palate repair technique, age at time of palate repair and surgeon; with sex as an independent variable. The data were analysed using a general linear model (P < .05).
Results
There were no significant differences for occlusal index scores as a function of lip or palate surgical technique, palatal repair timing and sex. Significant differences were found for occlusal index scores as a function of the surgeon.
Conclusion
Dental arch relationships were not influenced by lip and palatal repair techniques or patient age at palatal repair. The surgeon was the major factor that influenced the dental arch relationship outcome.
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