CROUZON SYNDROME - QUANTITATIVE ASSESSMENT OF PRESENTING DEFORMITY AND SURGICAL RESULTS BASED ON CT SCANS

We reviewed our experience with 14 children who presented sequentially with untreated Crouzon syndrome and whose cranial vault presentation was with bilateral coronal synostosis. Using a method of 14 measuremen ts in the cranio-orbitozygomatic region taken from preoperative and po stoperative CT scans in these patients, we documented their presenting skeletal morphology and the results of surgical correction at least 1 year after operation. Our preoperative measurements confirmed a widen ed anterior cranial vault at 108 percent of normal and a cranial lengt h averaging only 92 percent of normal. In comparison with age-matched controls, orbital measurements revealed a widened anterior interorbita l distance at 122 percent of normal, an increased intertemporal width at 121 percent of normal, globe protrusion at 119 percent of normal, a nd a short medial orbital wall distance at only 86 percent of normal. The distance between the zygomatic buttresses and the interarch distan ce were found to be increased at 106 and 103 percent of normal, respec tively. The zygomatic arch lengths were substantially shortened at onl y 87 percent of age-matched control values. These findings confirmed c linical observations of brachycephalic anterior cranial vaults with sh allow, hyperteloric orbits and globe proptosis. Generally, in these pa tients the midface is horizontally retrusive and transversely wide, re flected in wide and shortened zygomas. Assessment of the postoperative results at least 1 year later showed no significant changes in any cr aniofacial measurements. Our findings indicate that early surgical att empts to decompress and reshape the cranio-orbital regions may limit t he effects of increased intracranial pressure but do not correct the d eformity as judged by CT scan evaluation at least 1 year later. Over t he period of the study, the Crouzon deformity did not worsen after sur gery, but the measurements remained far from normal.