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Single Suture Synostosis and Isolated Cleft Palate in Non-Apert Syndrome Patients
Theodore Pezas1, Rona Slator, Nicholas White
1Birmingham Children's Hospital, Birmingham, UK.
Insights
Single suture craniosynostosis (SSC) and isolated cleft palate (ICP) rarely co-occur. This study found that managing both conditions together does not increase risks or negatively impact outcomes for either SSC or ICP.
Area of Science:
- Craniofacial Surgery
- Pediatric Surgery
- Genetics
Background:
- Single suture craniosynostosis (SSC) and isolated cleft palate (ICP) are rare in non-Apert syndrome patients.
- Management requires careful airway assessment, surgical timing, and evaluation of cranial aesthetics and speech.
- This study aimed to compare treatment and outcomes for patients with both SSC and ICP against isolated cases.
Observation:
- Six patients with both SSC and ICP were identified over 18 years.
- Cranial surgery occurred between 4-16 months; cleft surgery between 6-34 months.
- Two patients had genetic syndromes (Fragile X, Emanuel syndrome); one had hemi-palatal absence requiring an obturator.
Findings:
- No increased perioperative airway risk was noted.
- Aesthetic outcomes (Whitaker grading) were predominantly grade 1 (4/6 patients).
- Half of the patients experienced developmental delays, while the other half did not.
Implications:
- The co-occurrence of SSC and ICP does not appear to complicate management or negatively affect outcomes.
- Standardized treatment pathways for each condition are likely effective even when present together.
- Further research with larger cohorts could validate these findings and refine management protocols.
Introduction:
Single suture craniosynostosis (SSC) and isolated cleft palate (ICP) in non-Apert syndrome patients rarely occur together. Management includes airway optimization, timing surgery appropriately, and assessing both cranial vault aesthetics and speech outcomes. The aim of this study was to compare treatment pathways and outcomes in patients with both conditions to standard treatment for these conditions in isolation.
Methods:
Patient hospital medical records were retrospectively reviewed for demographic data, timing of surgery, aesthetic outcome (using the Whitaker grading system for head shape), and speech outcome (from speech therapy records of general development and speech assessment).
Results:
Six patients with SSC and ICP were identified over an 18-year period. Cranial surgery was performed between 4 and 16 months and cleft surgery between 6 and 34 months of age in all cases. Documentation of cleft surgery and genetic testing was not available for 1 patient. One patient with hemi-palatal absence had an obturator inserted at 34 months. Two patients were found to have Fragile X and Emanuel syndrome, respectively.No increased perioperative airway risk was highlighted in any case. Four cases were designated Whitaker grade 1, 1 case was designated grade 2, and 1 case was not graded as no cranial surgery was performed. Half of the patients had general and speech developmental delay, while the other half demonstrated no signs of developmental delay.
Discussion/Conclusion:
Examination of 6 patients with SSC and ICP suggests the presence of both conditions does not adversely impact management or outcome for each condition, or increase perioperative risk.
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