Apert syndrome: quantitative assessment by CT scan of presenting deformity and surgical results after first-stage...

We reviewed our experience with eight infants or young children (less than 2 years of age) who presented sequentially with Apert syndrome. Using a method of 14 measurements taken from the cranio-orbitozygomatic region in preoperative and postoperative CT scans of these patients, we documented their presenting skeletal morphology and the results of surgical correction at least 1 year after surgery. Significant preoperative findings included a wide anterior cranial vault at 110 percent of normal, a maximum cranial length that averaged only 90 percent of normal, a substantially widened anterior interorbital distance of 117 percent of normal, an increased lateral interorbital distance (112 percent of normal), and a widened bitemporal width at 122 percent of normal. Globe protrusion was significant at 121 percent of normal, and the medial orbital wall distance (length) was less than normal at 92 percent. In the upper midface (zygomatic) region, both the distance between the zygomatic buttresses and the interarch distance were found to be increased at 109 percent of normal, while the zygomatic arch lengths were substantially shortened at 79 percent of normal. Results of surgical correction, as documented by CT scan measurements, showed that more than a year after surgery, skeletal morphology had changed from preoperative measurements but that none of the craniofacial measurements had significantly improved (p < 0.05) in comparison with those of age-matched controls. Quantitative measurement of CT scans of the cranio-orbitozygomatic region confirmed clinically observed findings in these patients before cranio-orbital reconstruction in infancy and early childhood and proved useful in assessing the surgical results over time.