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Algorithm for the Management of Intracranial Hypertension in Children with Syndromic Craniosynostosis
Bart Spruijt1, Koen F M Joosten, Caroline Driessen
1Rotterdam, The Netherlands; and Boston, Mass. From the Dutch Craniofacial Center; the Pediatric Intensive Care Unit; and the Departments of Biostatistics, Ophthalmology, Otorhinolaryngology, Oral and Maxillofacial Surgery, and Neurosurgery, Sophia Children's Hospital-Erasmus University Medical Center; and the Departments of Neurology and Anaesthesia (Pediatrics), Harvard Medical School and Boston Children's Hospital.
Insights
Children with syndromic craniosynostosis face risks of intracranial hypertension, particularly linked to impaired head growth and moderate/severe obstructive sleep apnea after surgery.
Area of Science:
- Pediatric Neurosurgery
- Sleep Medicine
- Craniofacial Surgery
Background:
- Syndromic and complex craniosynostosis present unique challenges in pediatric care.
- Intracranial hypertension (ICH) is a significant concern in these patient populations.
- Understanding the interplay between head growth, sleep-disordered breathing, and ICH is crucial for effective management.
Purpose of the Study:
- To investigate the relationship between head growth, obstructive sleep apnea (OSA), and intracranial hypertension (ICH) in patients with syndromic craniosynostosis.
- To evaluate a standardized treatment protocol for managing ICH in this cohort.
- To identify key risk factors for the development of ICH.
Main Methods:
- Prospective observational cohort study of patients with syndromic craniosynostosis.
- Measurements included occipitofrontal head circumference (OFC) growth, sleep studies (dichotomized as no/mild vs. moderate/severe OSA), and funduscopy/OCT/ICP monitoring for ICH.
- Growth arrest defined by OFC trajectory decline or lack of change; ICH identified via papilledema, OCT, or ICP monitoring.
Main Results:
- Out of 62 patients, 33.9% had ICH, 62.9% had OSA, and 32.3% experienced OFC growth arrest.
- Preoperatively, ICH was associated with moderate/severe OSA (p=0.012).
- Postoperatively, ICH was linked to OFC growth arrest within the first year (p=0.006) and a combination of OFC growth arrest (p<0.001) and moderate/severe OSA (p=0.007) beyond one year.
Conclusions:
- Children with syndromic craniosynostosis are at high risk for intracranial hypertension.
- Impaired head growth is a major determinant of ICH after vault expansion, occurring at various ages.
- Moderate/severe obstructive sleep apnea significantly increases the risk of intracranial hypertension in these patients.
Background:
The purpose of this study was to examine the relationship of head growth, obstructive sleep apnea, and intracranial hypertension in patients with syndromic or complex craniosynostosis, and to evaluate the authors' standardized treatment protocol for the management of intracranial hypertension in these patients.
Methods:
The authors conducted a prospective observational cohort study of patients with syndromic craniosynostosis at a national referral center, treated according to a standardized protocol. Measurements included occipitofrontal head circumference, with growth arrest defined as downward deflection in occipitofrontal head circumference trajectory greater than or equal to a 0.5 SD fall from baseline over 2 years, or lack of change in occipitofrontal head circumference growth curve; sleep studies, with results dichotomized into no/mild versus moderate/severe obstructive sleep apnea; and funduscopy to indicate papilledema, supplemented by optical coherence tomography and/or intracranial pressure monitoring to identify intracranial hypertension.
Results:
The authors included 62 patients, of whom 21 (33.9 percent) had intracranial hypertension, 39 (62.9 percent) had obstructive sleep apnea, and 20 (32.3 percent) had occipitofrontal head circumference growth arrest during the study. Age at which intracranial hypertension first occurred was 2.0 years (range, 0.4 to 6.0 years). Preoperatively, 13 patients (21.0 percent) had intracranial hypertension, which was associated only with moderate/severe obstructive sleep apnea (p = 0.012). In the first year after surgery, intracranial hypertension was particularly related to occipitofrontal head circumference growth arrest (p = 0.006). Beyond 1 year after surgery, intracranial hypertension was associated with a combination of occipitofrontal head circumference growth arrest (p < 0.001) and moderate/severe obstructive sleep apnea (p = 0.007).
Conclusions:
Children with syndromic craniosynostosis are at risk of intracranial hypertension. The major determinant of this after vault expansion is impaired head growth, which may occur at varying ages. The presence of moderate/severe obstructive sleep apnea also significantly increases the risk of intracranial hypertension.
Clinicial Question/Level Of Evidence:
Risk, III.
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