Treatment of apert syndrome: a long-term follow-up study.

BACKGROUND: Patients with Apert syndrome have severe malformations of the skull and face requiring multiple complex reconstructive procedures. The authors present a long-term follow-up study reporting both surgical results and psychosocial status of patients with Apert syndrome.

METHODS: A retrospective study was performed identifying patients with Apert syndrome treated between 1975 and 2009. All surgical procedures were recorded and a review of psychosocial and educational status was obtained when patients reached adulthood.

RESULTS: A total of 31 patients with Apert syndrome were identified; nine with long-term follow-up had complete records for evaluation. The average patient age was 30.4 years. Primary procedures performed included strip craniectomy and fronto-orbital advancement. Monobloc osteotomy and facial bipartition were performed in eight patients, and all underwent surgical orthognathic correction. Multiple auxiliary procedures were also performed to achieve better facial symmetry. Mean follow-up after frontofacial advancement was 22.5 years. Psychosocial evaluation demonstrated good integration of patients into mainstream life.

CONCLUSIONS: This report presents one of the longest available follow-up studies for surgical correction of patients with Apert syndrome. Although multiple reconstructive procedures were necessary, they play an important role in enhancing the psychosocial condition of the patients, helping them integrate into mainstream life.