The mean age at onset of symptoms in the Masri et al. cohort of 19 children was 6 years (range: 7 months to 12 years). Most patients (90%) were under 11 years old and (84.2%) symptomatic. The probable cause was identified in 7/19 (37.0%) patients. The most common cause was vitamin D deficiency (26.3%). Other associated probably coincidental comorbidities included sinusitis (5/19, 26.3%), hypophosphatasia (1/19), Pyle disease (1/19), and measles vaccine (1/19). Apart from 2 patients who required lumboperitoneal shunt, the cerebrospinal fluid pressure returned to normal in all patients within a period of 6 weeks to 1 year (average, 5 months). Of those who followed up with the authors' ophthalmologist, 30.7% developed optic nerve atrophy or pallor; 75% of these patients had previous ocular comorbidities 1).

Raised intracranial pressure (ICP) is recognized to occur in patients with nonsyndromic isolated sagittal craniosynostosis (SC) prior to surgery.


1)
Masri A, Jaafar A, Noman R, Gharaibeh A, Ababneh OH. Intracranial Hypertension in Children: Etiologies, Clinical Features, and Outcome. J Child Neurol. 2015 Mar 11. pii: 0883073815574332. [Epub ahead of print] PubMed PMID: 25762586.
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