Faculty Bibliography

The present study was undertaken to characterize the intracranial volume in patients with Apert syndrome. Radiographic and CT-based techniques for measuring intracranial volume were found to show close correlation when validated in dry normal adult skulls. A standard-deviation score of intracranial volume, determined from CT scans in 20 patients with Apert syndrome from birth to 30 years of age, was calculated with reference to a normative database of age- and sex-matched controls. Analysis of the data showed no significant difference in standard-deviation score between Apert patients with or without ventriculomegaly, before or after cranial vault surgery, or between male and female patients. The mean standard-deviation score among all Apert CT scans compared with normative data was 3.1, which was highly significant (p < 0.001). However, the growth curves showed no significant difference in intracranial volume between Apert and normal patients below 3.5 months of age. In summary, intracranial volume in Apert syndrome is within normal range at birth but increases to greater than 3 standard deviations above normal after 3.5 months of age, a finding independent of cranial vault surgery

The principle of distraction osteogenesis, well-established in the enchondral bones of the axial skeleton, has recently been applied to the membranous bones (mandible, cranium) of the craniofacial skeleton in the experimental animal and in the human. In the craniofacial skeleton, however, the technique has been used only to lengthen bone in a direction along its major axis, i.e., unidimensional distraction. A canine model is presented to demonstrate the feasibility of distracting membranous bone away from its dominant axis, i.e., multidimensional distraction. Four mongrel dogs, 5 months of age, were the subjects of this study. Two osteotomies were made in the zygomatic arch, and the bone-lengthening device was fixed to the zygoma. After 7 days of external fixation, the osteotomized segment was lengthened 1 mm/day away from the long axis of the bone for 15 days. External fixation was then maintained for a minimum of 4 weeks, after which the dogs were sacrificed. Craniofacial CT with three-dimensional reconstruction documented multidimensional bone lengthening, and histologic analysis of the specimen confirmed the presence of new cortical bone in the expanded areas. Refinement in technique and miniaturization and internalization of the bone-lengthening device may allow for more precise changes in the amount and direction of lengthening, thus making distraction osteogenesis more widely applicable for use in the human craniofacial skeleton