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Neurological deficits are present in syndromic craniosynostosis patients with and without tonsillar herniation
P N Doerga1, B F M Rijken1, H Bredero-Boelhouwer1
1Department of Plastic and Reconstructive Surgery and Hand Surgery, Sophia Children's Hospital, Dutch Craniofacial Center, Erasmus MC, University Medical Center, Doctor Molewaterplein 40, 3015 GD, Rotterdam, the Netherlands.
Insights
Neurological deficits are common in children with syndromic craniosynostosis (sCS), regardless of cerebellar tonsillar position. These findings may be developmental, indicating that surgical intervention timing needs careful consideration beyond tonsillar herniation (TH) measurements.
Area of Science:
- Pediatric Neurosurgery
- Craniofacial Anomalies
- Neurology
Background:
- Children with syndromic craniosynostosis (sCS) exhibit a higher prevalence of cerebellar tonsillar herniation (TH) compared to the general population.
- The threshold of TH (≥5 mm below the foramen magnum) associated with neurological deficits in the general population is not well-defined for sCS patients.
Purpose of the Study:
- To investigate the association between neurological assessment findings and cerebellar tonsillar position in pediatric patients with sCS.
- To clarify the clinical significance of cerebellar tonsillar herniation (TH) in the context of neurological deficits in syndromic craniosynostosis.
Main Methods:
- Prospective cohort study utilizing magnetic resonance imaging (MRI) to assess cerebellar tonsillar herniation (TH) ≥5 mm and syringomyelia.
- Neurological deficits were systematically categorized, evaluating cerebellar function, cranial nerve abnormalities, and sensory or motor dysfunction.
Main Results:
- Cerebellar tonsillar herniation (TH) ≥5 mm and/or syringomyelia were identified in 32% of the 63 evaluated sCS patients.
- Neurological deficits were highly prevalent (73%) in sCS patients, occurring with similar frequency in those with and without TH ≥5 mm and/or syringomyelia.
- No significant differences in TH prevalence were observed across different subtypes of sCS, including Crouzon, Muenke, Apert, and Saethre-Chotzen syndromes.
Conclusions:
- Neurological deficits in syndromic craniosynostosis (sCS) patients appear to be largely independent of cerebellar tonsillar herniation (TH) ≥5 mm.
- The high prevalence of neurological deficits suggests they may represent developmental and syndrome-specific central nervous system features in sCS.
- These findings emphasize the need to consider developmental factors and syndrome characteristics, rather than solely TH measurements, when determining the necessity of surgical intervention.
Background:
Children with syndromic craniosynostosis (sCS) have a higher incidence of cerebellar tonsillar herniation (TH) than the general population. In the general population, TH ≥ 5 mm below the foramen magnum is associated with typical neurological deficits but, in sCS, we do not know whether this degree of TH is required before such deficits occur.
Objective:
This prospective cohort study aimed to determine the association between findings on neurological assessment and cerebellar tonsillar position.
Methods:
Magnetic resonance imaging (MRI) was used to determine TH ≥ 5 mm and the presence of syringomyelia. In regard to the outcome of neurological deficits, these were categorized according to: A, cerebellar function; B, cranial nerve abnormalities; and C, sensory or motor dysfunction.
Results:
Twenty of 63 patients with sCS (32% [95% confidence interval 21-45%]) had TH ≥ 5 mm and/or syringomyelia. There was no significant difference in proportion between individual forms of sCS: 16/34 Crouzon, 2/11 Muenke, 2/12 Apert, and 0/7 Saethre-Chotzen patients. Neurological deficits were prevalent (73% [95% confidence interval 60-83%]), and as frequent in patients with TH ≥ 5 mm and/or syringomyelia as those without. Surgery occurred in 3 patients overall, and only in Crouzon patients.
Conclusion:
Determining the effect of TH ≥ 5 mm on neurologic functioning in sCS patients is used to better determine when surgical intervention is warranted. However, we have found that neurological deficits are prevalent in sCS patients, irrespective of cerebellar tonsillar position, suggesting that such findings are developmental and, in part, syndrome-specific central nervous system features.
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