ArticleChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2019
Chiari 1 malformation and untreated sagittal synostosis: a new subset of complex Chiari?
Article in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers, 1 of them a synthesis that pooled it.
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8 citing papers in PubMed, 1 synthesis or guideline pooled it, 16 citations in OpenAlex.
- The management of Chiari malformation type 1 and syringomyelia in children: a review of the literature.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2021Pooled it
- Chiari malformation type I with an associated syrinx presenting as acute central cord syndrome in a child: illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- Endoscopic third ventriculostomy for hydrocephalus after craniovertebral decompression for Chiari malformation type I: technical nuances and surgical pitfalls.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2023Article
- Amyotrophic Lateral Sclerosis Mimic Syndrome in a 24-Year-Old Man with Chiari 1 Malformation and Syringomyelia: A Clinical Case.Journal of clinical medicine · 2023Article
- Evaluation of Adult and Pediatric Chiari Type 1 Malformation Patients: Do Consensus Documents Fit Everyday Practice?Acta neurochirurgica. Supplement · 2023Article
- Multisuture and Syndromic Craniosynostoses: Simplifying the Complex.Journal of pediatric neurosciences · 2022Review
- Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2022Article
- Acute presentation of Chiari 1 malformation in children.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2020Review
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5 authors at 1 institution in 1 country.
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Abstract
purposeChiari 1 malformation (CM1) is a well-known association with complex craniosynostosis (CC), while it has been rarely reported in association with monosynostosis. The aim of the present study is to investigate on the association between CM1 and untreated sagittal synostosis (USS).
methodThe study included 48 cases of sagittal synostosis (SS), untreated for misdiagnosis and associated with CM1. The children were firstly diagnosed for CM1 by MRI (mean age 9) than for SS (mean age 10.5) by three-dimensional computerized tomography (3D-CT), which documented the absence of the sagittal suture, in the presence of residual indentation of all the other sutures. Syndromic cases were diagnosed by clinical evaluation and molecular studies.
resultsOf the 48 children harboring CM1 plus USS, 21 were asymptomatic for CM1 and are still on follow-up, while 27 children were operated for syringomyelia and scoliosis and/or occurrence of symptoms, three of them had an acute presentation (two papilledema and one sleep apneas) and 11 children had a documented increase of preoperative ICP. Craniovertebral decompression (CVD) was the first-line surgery in 24 children, 16 with duroplasty and five without and eight had also cerebellar (CBL) tonsil coagulation. A cranial vault remodelling was firstly performed in three children. Fifteen percent of children submitted to CVD needed a revision for cerebrospinal fluid (CSF) collection, while two needed both the supra- and infratentorial decompressive procedure and another two needed a treatment for the associated hydrocephalus.
conclusionsThe present study identified an USS in 27 (15.5%) of 174 CM1 children operated for a symptomatic CM1. We suggest to define this association CM1 plus USS, a new subtype of complex CM1. For the high percentage of complications and multiple procedures needed to solve the CM1, we advise to identify by 3D-CT scan these children before performing CVD. Our finding suggests also that, if left untreated, SS may lead to the delayed occurrence of a challenging subset of CM1.
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