Document Detail


Complex craniosynostoses: a review of the prominent clinical features and the related management strategies.
MedLine Citation:
PMID:  22872268     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
The protocols for clinical evaluation and management of children with complex craniosynostoses are significantly different from those used in single suture forms. The time at which the various anatomical and functional anomalies observed in the affected subjects become clinically relevant varies from patient to patient, consequently requiring a tailored approach. The clinical course is variable and influenced by multiple factors, acting at different steps of the children growth. Intracranial hypertension is a major concern already in the first months of life; active cerebrospinal fluid (CSF) dynamics disorders, venous hypertension, and progressive craniocerebral disproportion are considered the main pathogenetic factors. Cranial vault and skull base sutures synostoses account for the frequently observed increased venous pressure. Skull base abnormalities lead to upper airways obstruction, which, on one side, might create significant upper airways obstructive problems and, on the other, contribute to the increase in the intracranial pressure. Secondary Chiari malformation is common and considered as a progressive disorder, mainly due to progressive craniocerebral disproportion, venous hypertension, and CSF dynamics disorders. Optic nerve and orbit-related eye-globe diseases are also a major concern. Papilledema is mostly related to increased intracranial pressure. The skull base synostotic process is the base of significant abnormalities of the orbital space, ending in the common feature of significant proptosis with the consequent risk of corneal ulcers. Aims of this paper are to analyze the physiopathogenetic mechanisms at the base of the clinical manifestations presented by children with complex craniosynostoses, and the therapeutic options currently available.
Authors:
G Tamburrini; M Caldarelli; L Massimi; G Gasparini; S Pelo; C Di Rocco
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Publication Detail:
Type:  Journal Article     Date:  2012-08-08
Journal Detail:
Title:  Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery     Volume:  28     ISSN:  1433-0350     ISO Abbreviation:  Childs Nerv Syst     Publication Date:  2012 Sep 
Date Detail:
Created Date:  2012-08-08     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8503227     Medline TA:  Childs Nerv Syst     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  1511-23     Citation Subset:  IM    
Affiliation:
Pediatric Neurosurgery, Institute of Neurosurgery, Catholic University Medical School, Largo "A. Gemelli", 8, 00168, Rome, Italy, gianpiero.tamburrini@rm.unicatt.it.
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