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Thirty-Year Experience Treating Syndromic Craniosynostosis: Long-Term Outcomes following Cranial Expansions
Jeffrey A Fearon1, Kanlaya Ditthakasem2, Lucas Harrison3
1From The Craniofacial Center.
Insights
Children with syndromic craniosynostosis typically need two cranial expansion surgeries, with a low 2% complication rate. Factors like early intervention and shunts correlate with more procedures.
Area of Science:
- Craniofacial surgery
- Pediatric neurosurgery
- Genetics
Background:
- Syndromic craniosynostosis necessitates multiple cranial expansion surgeries in children.
- Understanding the typical number of procedures and complication rates is crucial for managing patient care.
Purpose of the Study:
- To determine the average number of cranial expansions required for syndromic craniosynostosis until skeletal maturity.
- To assess the complication rates associated with these procedures.
- To identify factors influencing the number of interventions and potentially reduce the treatment burden.
Main Methods:
- Retrospective chart review of patients with syndromic craniosynostosis undergoing cranial vault enlargement.
- Analysis of long-term records from a single surgeon's practice.
- Data collection on patient demographics, surgical procedures, complications, and follow-up duration.
Main Results:
- 348 patients with Apert, Crouzon, or Pfeiffer syndromes had complete records; mean follow-up was 16.9 years.
- Average of 1.9 expansions per patient at a single center, with Pfeiffer syndrome patients undergoing more (2.7).
- Overall complication rate was 2%; earlier interventions, ventriculoperitoneal shunts, and treatment at outside centers correlated with more procedures.
Conclusions:
- Patients treated at a single center averaged two skull expansion procedures with a 2% complication rate.
- Calvarial distraction, ventriculoperitoneal shunts, and earlier surgical interventions are associated with increased procedure numbers.
- Further research is needed to establish causality for observed correlations.
Background:
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.
Methods:
A retrospective chart review was conducted of all consecutive patients undergoing cranial vault enlargement procedures for syndromic craniosynostosis performed by a single surgeon.
Results:
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).
Conclusions:
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.
Clinical Question/Level Of Evidence:
Therapeutic, IV.
