Neurological deficits are present in syndromic craniosynostosis patients with and without tonsillar herniation.

Autor: Doerga PN; Department 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. Electronic address: p.doerga@erasmusmc.nl., Rijken BFM; Department 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., Bredero-Boelhouwer H; Department 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., Joosten KFM; Pediatric Intensive Care Unit, Sophia Children's Hospital, Erasmus MC, University Medical Center, Doctor Molewaterplein 40, 3015 GD, Rotterdam, the Netherlands., Neuteboom RF; Department of Pediatric Neurology, Sophia Children's Hospital, Erasmus MC, University Medical Center, Doctor Molewaterplein 40, 3015 GD, Rotterdam, the Netherlands., Tasker RC; Department of Anaesthesia (Pediatrics) and Division of Critical Care Medicine, Harvard Medical School and Boston Children's Hospital, 300 Longwood Avenue, Boston, Massachusetts, 02115, USA., Dremmen MHG; Department of Neurosurgery, Sophia Children's Hospital, Erasmus MC, University Medical Center, Doctor Molewaterplein 40, 3015 GD, Rotterdam, the Netherlands., Lequin MH; Department of Radiology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, the Netherlands., van Veelen MLC; Department of Neurosurgery, Sophia Children's Hospital, Erasmus MC, University Medical Center, Doctor Molewaterplein 40, 3015 GD, Rotterdam, the Netherlands., Mathijssen IMJ; Department 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.
Jazyk: angličtina
Zdroj: European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society [Eur J Paediatr Neurol] 2020 Sep; Vol. 28, pp. 120-125. Date of Electronic Publication: 2020 Jul 21.
DOI: 10.1016/j.ejpn.2020.06.018
Abstrakt: 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.
Competing Interests: Declaration of competing interest None.
(Copyright © 2020. Published by Elsevier Ltd.)
Databáze: MEDLINE
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