Cephalometric Features of Moyamoya Disease: A case control study

Background:Moyamoya disease is highly prevalent among patients with syndromes that haveunique cephalometric characteristics such as Down syndrome. We performed a casecontrol study to investigate the relationship between cephalometric parametersand Moyamoya disease. Methods:Patients [aged 16-82 years] with angiographically confirmed Moyamoya diseasewho underwent cranial CT scan were analyzed. We identified three controls foreach patient who were matched for age (±1 year), gender, and race (white orAfrican American). The fronto-occipital diameter, bi-parietal diameter, anddistance between bregma and occiput were measured from the head CT scans ofcases and controls. The cephalic index was calculated by determining the ratiobetween bi-parietal diameter and fronto-occipital diameter and multiplying thevalue by 100.  Results: A total of13 cases of Moyamoya disease and 39 controls were analyzed. The stage ofMoyamoya disease in cases was as follows: stage 1 (n=0), stage 2 (n=1), stage 3(n=4), stage 4 (n=2), stage 5 (n=5) and stage 6 (n=1).  There was a significantly greater bi-parietaldiameter in Moyamoya disease patients compared with controls (141.5±3.7 mm versus 136.9±5.4 mm,p=0.007). Therewas a significantly greater fronto-occipitaldiameter in Moyamoya disease patients compared with controls (186.5±6.5 mm versus 180.2±8.7mm, p=0.02).The distance betweenbregma and occiput was shorter among cases comparedwith controls (81.1±6.2versus 87.5±7.0, p=0.01).  Conclusions: We observed an association between cephalometricparameters and Moyamoya disease. Further study of the unique cephalometriccharacteristics among Moyamoya disease patients may provide additional insightinto disease occurrence in white and African American populations.