Craniofacial morphology in patients with sickle cell disease: a cephalometric analysis

The aim of the study was to evaluate the craniofacial morphology in Caucasian patients with sickle cell disease (SCD) by comparing them with a healthy group paired for gender and age, by means of lateral cephalometric radiographs. Thirty-six Sicilian patients with SCD (17 females and 19 males), including 14 beta(s)beta(s) (mean age 28 +/- 5.9 years), 13 beta(s)beta(0 th) (mean age 27.5 +/- 8 years), and nine beta(s)beta(+th) (mean age 32.8 +/- 9.9 years) were examined. The control group consisted of 36 subjects (mean age 28.9 +/- 8 years) without recognized haematological abnormalities. The means and standard deviations were calculated for each cephalometric variable. A two-sample t-test was used to compare the means between the study and control groups. One-way analysis of variance and Dunnet's multiple comparison test were used in order to analyse the differences between the control group and the subgroups divided according to genotype. The level of significance used was P<0.05. The cephalometric findings indicated a posterior rotation of the mandible and a tendency towards a vertical pattern (clockwise), with lower (P=0.000) and total (P=0.002) face heights increased in comparison with the control sample. These findings were more pronounced in subjects with SCD (beta(s)beta(s)). In all patients, there was a significantly greater maxillary incisor proclination than in the control group. The upper first molar position to the PTV line was significantly increased but only in patients with compound heterozygosis beta(s)beta(th). The SCD patients did not exhibit the craniofacial abnormalities noted in black American patients with SCD; the craniofacial features observed, reflecting the degree of clinical expression of SCD in Sicilian patients, were of moderate severity.