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Management of secondary turricephaly in craniofacial surgery
W J Sonstein1, C D Hall, R V Argamaso
1Center for Congenital Disorders, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY 10467, USA.
Insights
Secondary turricephaly is a common complication in children with syndromic craniofacial disorders like Crouzon and Apert syndromes. This study reviews surgical management for this deformity, highlighting challenges and outcomes in 11 patients.
Area of Science:
- Craniofacial surgery
- Pediatric plastic surgery
- Syndromic disorders
Background:
- Secondary turricephaly is a challenging complication in surgically managed syndromic craniofacial disorders.
- Theories suggest skull base growth restriction due to suture fusion causes this deformity.
- Primary reconstructions often fail to fully correct the characteristic turricephaly.
Purpose of the Study:
- To analyze the incidence and surgical management of secondary turricephaly in syndromic children.
- To discuss surgical techniques and timing for optimal outcomes.
- To review existing literature on managing this complex deformity.
Main Methods:
- Retrospective review of 11 syndromic children with secondary turricephaly post-craniofacial reconstruction.
- Analysis of surgical interventions, including secondary or tertiary procedures.
- Evaluation of patient outcomes and follow-up duration.
Main Results:
- Six out of 11 patients required additional surgical procedures for turricephaly.
- Only 5 patients achieved a good outcome.
- Mean follow-up was 4.5 years (range 1-8 years).
Conclusions:
- Secondary turricephaly presents a significant challenge in syndromic craniofacial surgery.
- Surgical timing and methods are critical for managing this deformity.
- Further research into optimizing surgical strategies is warranted.
Abstract:
In children with syndromic craniofacial disorders, such as Crouzon and Apert syndromes, who are managed surgically, a difficult problem that can occur is secondary turricephaly. One of the more widely accepted theories as to why this deformity occurs is that a lack of skull base growth results from fusion of the basal and facial sutures. Despite initial adequate forehead and orbital bandeau advancement, many of these patients require subsequent procedures, which do not always correct the characteristics deformity. We have identified a subset of 11 syndromic children who developed this characteristic deformity of turricephaly after primary reconstruction, 6 of whom required either secondary or tertiary procedures. Only 5 patients had a good outcome with a mean follow up of 4.5 years (range 1-8 years). Our surgical methods, and our rationale for the timing of surgery are discussed, and the literature on the management of this problem is reviewed.
