Thirty-Year Experience Treating Syndromic Craniosynostosis: Long-Term Outcomes following Cranial Expansions

Children with syndromic craniosynostosis require multiple cranial expansion procedures. The purpose of this study was to determine how many expansions are typically performed through maturity, to assess complication rates, and to identify trends that might reduce the burden of care.

A retrospective chart review was conducted of all consecutive patients undergoing cranial vault enlargement procedures for syndromic craniosynostosis performed by a single surgeon.

Of 444 patients treated with Apert or Crouzon/Pfeiffer syndromes, 348 had complete long-term records. The mean age at last follow-up was 16.9 years. Initial cranial expansions were performed at a significantly younger age for those beginning care at outside centers: 7.7 months versus 25.7 months in Dallas; 26.4% had ventriculoperitoneal shunts (syndrome-specific incidences: Apert syndrome, 16%; Crouzon syndrome, 27%; and Pfeiffer syndrome, 54%). Of 498 procedures, 80% were anterior, 18% were posterior, and 2% were midvaults. The overall complication rate was 2%, with no differences found between anterior and posterior procedures. Those treated solely in Dallas underwent a mean of 1.9 expansions. Children with Pfeiffer syndrome underwent more expansions (2.7) than those with Apert or Crouzon syndrome. Factors correlating with more expansions included earlier surgical interventions, use of distraction, ventriculoperitoneal shunts, and treatment begun at outside centers (3.1 procedures).

Children with syndromic craniosynostosis treated at a single center underwent an average of 2 skull expansion procedures with a 2% complication rate. Calvarial distraction, ventriculoperitoneal shunts, and earlier surgical interventions were all associated with higher numbers of expansion procedures. Further research is needed to determine whether these correlations are causal.

Therapeutic, IV.