Document Detail

Raised intracranial pressure is frequent in untreated nonsyndromic unicoronal synostosis and does not correlate with severity of phenotypic features.
MedLine Citation:
PMID:  23096622     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: In a small number of children with unicoronal synostosis, the phenotype is mild and the aesthetic benefit of surgical correction is potentially outweighed by surgical risk. Raised intracranial pressure, however, would necessitate intervention. The authors documented the incidence of raised intracranial pressure in children with mild features and/or parental reluctance to proceed directly to surgery.
METHODS: A retrospective review of all children with (1) a clinical diagnosis of nonsyndromic unicoronal synostosis; (2) unicoronal synostosis confirmed on computed tomographic scanning; (3) negative family history for unicoronal synostosis; and (4) negative genetic screening (including P250R encoded by FGFR3, exons IIIa and IIIc in FGFR2 and TWIST1) was completed.
RESULTS: Of the 80 children studied, seven (9 percent) underwent formal intracranial pressure monitoring because of a combination of mild clinical features (n = 4) and/or parental reluctance to proceed with surgery (n = 3). Intracranial pressure monitoring was reported as high in three (42 percent), borderline in two (29 percent), and normal in two patients (29 percent). The findings did not correlate with the degree of clinical deformation or age at presentation. There was little correlation with clinical and radiographic features of raised intracranial pressure in this genetically screened nonsyndromic group.
CONCLUSIONS: High or borderline raised intracranial pressure was present in five of seven cases. The risk did not correlate with degree of deformity or age at presentation. Formal intracranial pressure monitoring should be considered in all children with unicoronal synostosis not proceeding directly to surgical intervention, in combination with routine long-term follow-up and repeated intracranial pressure monitoring where indicated.
Karen A Eley; David Johnson; Andrew O M Wilkie; Jayaratnam Jayamohan; Peter Richards; Steven A Wall
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Plastic and reconstructive surgery     Volume:  130     ISSN:  1529-4242     ISO Abbreviation:  Plast. Reconstr. Surg.     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-10-25     Completed Date:  2013-01-25     Revised Date:  2014-10-13    
Medline Journal Info:
Nlm Unique ID:  1306050     Medline TA:  Plast Reconstr Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  690e-697e     Citation Subset:  AIM; IM    
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MeSH Terms
Child, Preschool
Craniosynostoses / complications*,  physiopathology
Intracranial Hypertension / complications*
Retrospective Studies

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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