Dynamic cranioplasty for brachycephaly in apert syndrome: long-term follow-up study

Brachycephaly is a characteristic feature of Apert syndrome. Traditional techniques of cranioplasty often fail to produce an acceptable morphological outcome in patients with this condition. In 1996 a new surgical procedure called "dynamic cranioplasty for brachycephaly" (DCB) was reported. The purpose of the present study was to analyze perioperative data and morphological long-term results in patients with the cranial vault deformity of Apert syndrome who were treated with DCB.

Twelve patients have undergone surgery performed using this technique since its introduction in 1991 (mean duration of follow-up review 60.2 months). Eleven patients had bicoronal synostosis and one had a combined bicoronal-bilambdoid synostosis. Perioperative data and long-term evolution of skull shape visualized on serial cephalometric radiographs were analyzed and compared with normative data. Changes in mean skull proportions were evaluated using a two-tailed paired-samples t-test, with differences being considered significant for probability values less than 0.01. The mean operative blood transfusion was 136% of estimated red cell mass (ERCM) and the mean postoperative transfusion was 48% of ERCM. The mean operative time was 218 minutes. The duration of stay in the intensive care unit averaged 1.7 days and the mean hospital stay was 11.8 days. There were no incidences of mortality and few complications. An improvement in skull shape was achieved in all cases, with a change in the mean cephalic index from a preoperative value of 90 to a postoperative value of 78 (p = 0.000254).

Dynamic cranioplasty for brachycephaly is a safe procedure, yielding high-quality morphological results in the treatment of brachycephaly in patients with Apert syndrome.