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Syndromic Craniosynostosis: Objective and Parent-Reported Outcome Measurements after Cranio-Facial Remodelling
Alok Mohan Uppar1, Dhaval Shukla1, Nitish Nayak1
1Department of Neurosurgery, NIMHANS, Bangalore, India.
Insights
Reconstructive cranio-facial surgery significantly improves cosmetic outcomes and quality of life for children with syndromic craniosynostosis (SC). Parent-reported outcomes show good results in cosmesis, cognition, and ophthalmological issues following surgical intervention.
Area of Science:
- Craniofacial Surgery
- Pediatric Plastic Surgery
- Medical Genetics
Background:
- Syndromic craniosynostosis (SC) is a rare condition requiring multidisciplinary care.
- Early surgical intervention in SC is crucial for optimal outcomes.
- This study reviews reconstructive cranio-facial surgery outcomes in children with SC.
Purpose of the Study:
- To evaluate the outcomes of reconstructive cranio-facial surgery in children with syndromic craniosynostosis.
- To assess both objective and subjective improvements following surgical intervention.
- To determine the impact of surgery on quality of life.
Main Methods:
- Retrospective study analyzing hospital records and imaging.
- Comparison of pre- and post-operative cephalic index (CI).
- Assessment of cosmetic outcomes using Sloan and Whitaker scales, and parent-reported outcome measurements (PROMs) for quality of life (QOL).
Main Results:
- Nineteen of 21 SC patients underwent cranio-facial remodelling.
- Maximum improvement in CI observed at 6 months post-surgery.
- High rates of satisfactory cosmetic outcomes (Sloan class 1 & 2, Whitaker category 1) and significant improvements in QOL, cosmesis, and ophthalmological issues reported by parents.
Conclusions:
- Cranio-facial remodelling surgery in SC yields favorable cosmetic and functional results.
- PROMs indicate substantial improvements in cosmesis, cognition, and ophthalmological concerns.
- Surgery significantly enhances the overall quality of life for children with syndromic craniosynostosis.
Introduction:
Syndromic craniosynostosis (SC) is a rare entity compared to the non-syndromic variant. Treatment involves a multidisciplinary approach towards a multitude of problems. Early intervention is known to be better for optimum results. We reviewed outcomes of children with SC who underwent reconstructive cranio-facial surgery.
Materials And Methods:
A retrospective study was conducted using data from hospital case files and the picture archival communication system. Objective data like the cephalic index (CI), both preoperatively and post-operatively, were compared. Subjective data for the cosmesis outcome - "Sloan and Whitaker outcome class" - following surgery were assessed. Also, parent-reported outcome measurement (PROM) was performed with various parameters to assess quality of life (QOL).
Results:
We had 21 operated cases of SC, with 19 needing cranio-facial remodelling. The male to female ratio was 11:10. Crouzon's syndrome was the most common syndromic association followed by Apert's syndrome. Nineteen patients underwent cranio-facial remodelling surgeries and 2 underwent the ventriculo-peritoneal shunt only - for raised intracranial pressure (ICP). Nine patients underwent cranial remodelling with fronto-orbital advancements, and 3 of these patients also received le-fort's type 3 osteotomy and advancement later. Ten patients underwent fronto-orbital advancement with parieto-occipital barrel-stave osteotomies.
Outcomes:
Improvement in the CI was maximum at the 6-month follow-up. Six (37.5%) cases had Sloan class 1 outcome, 9 (56.25%) had class 2 outcomes, and 1 patient had a class 6 outcome. Whitaker cosmesis outcomes - 14 out of 16 cases (87.5%) had Category 1 outcomes. PROM was assessed. All parents reported at least a moderate improvement in cosmesis following surgery. Out of 15 cases, 10 (66%) reported significant improvement, while 4 (26.6%) cases reported moderate improvement with respect to eye and visual problems. Four parents reported snoring as a significant problem even after surgery. Most parents felt that the children were doing well, attending regular school, and social well-being was normal and had an overall good QOL.
Conclusions:
SC cases may have a multitude of other problems like raised ICP, ophthalmological problems, poor intelligence, and cognition apart from cosmetic concerns. PROMs revealed good outcomes in terms of cosmesis, cognition, and ophthalmological and oral cavity-related problems. Significant improvement in overall QOL was seen in most patients following cranio-facial remodelling surgery.
