ArticleThe Journal of craniofacial surgery
Occult Scaphocephaly: A Forme Fruste Phenotype of Sagittal Craniosynostosis.
Article in The Journal of craniofacial surgery. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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3 citing papers in PubMed.
- Understanding normocephalic craniosynostosis: a case-control study on prevalence, clinical features, and neurodevelopmental challenges.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Article
- Sickle Cell Disease Association with Premature Suture Fusion in Young Children.Plastic and reconstructive surgery. Global open · 2022Article
- Making the Diagnosis in Sagittal Craniosynostosis-It's Height, Not Length, That Matters.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2022Article
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9 authors.
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Abstract
introductionLatent cranial suture fusions may present with mild or absent phenotypic changes that make the clinical diagnosis challenging. Recent reports describe patients with sagittal synostosis and a normal cranial index (CI), a condition termed normocephalic sagittal craniosynostosis (NSC). The goal of this study is to evaluate the shape and intracranial volume (ICV) in a cohort of NSC patients using quantitative cranial shape analysis (CSA).
methodsWe identified 19 patients (7.5 ± 2.28 years) between 2011 and 2016, who presented to our hospital with NSC. Cranial index and CSA were measured from the computed tomography image. Cranial shape analysis calculates the distances between the patient's cranial shape and its closest normal shape. Intracranial volume was measured and compared to an established age-matched normative database.
resultsCranial index revealed 15 (78.9%) patients within the mesocephalic range and 4 patients (21.1%) in the brachycephalic range. Detailed CSA identified 15 (78.9%) patients with subtle phenotypic changes along the scaphocephalic spectrum (ie, subtle anterior and posterior elongation with inter-parietal narrowing) and 1 patient (5.3%) with isolated overdevelopment on the posterior part of the right parietal bone. Three patients (15.8%) had a CSA close to normal. Mean ICV was 1410.5 ± 192.77cc; most patients (78.9%) fell within ±2 standard deviations.
conclusionQuantitative CSA revealed that most of the patients with NSC had cranial shape abnormalities, consistent with a forme fruste scaphocephaly that could not be otherwise recognized by clinical observation or CI. Given these findings, we propose the term occult scaphocephaly to describe this condition. The associated incidence of intracranial hypertension is unknown.
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