CoChat
Sign Up
Cited by 115Craniofacial Disorders and TreatmentsTraumatic Brain Injury and Neurovascular DisturbancesPectus Deformity Diagnosis and Treatment

Progressive Postnatal Craniosynostosis and Increased Intracranial Pressure

Connolly, John P., Gruss, Joseph, Seto, Marianne L., Whelan, Michael F., Ellenbogen, Richard, Weiss, Avery, Buchman, Steven R., Cunningham, Michael L.·2004·Plastic and Reconstructive Surgery
DOI 10.1097/01.prs.0000111593.96440.30
Open this paper in CoChat — free account

Abstract

Since its first description by Virchow in 1851, craniosynostosis has been known as a potentially serious condition resulting in premature fusion of skull sutures. Traditionally, craniosynostosis has been regarded as an event that occurs early in fetal development, resulting in a skull shape at birth that is determined by the suture or sutures involved. In recent years, a different form of craniosynostosis has been observed. Patients initially come to the attention of physicians because they exhibit midface hypoplasia or occasionally hypertelorism. The affected individuals all have a normal skull shape and open sutures in infancy but develop multiple-suture craniosynostosis postnatally, ultimately requiring surgical correction. These cases are significant because, although the patients do not initially display the physical manifestations of craniosynostosis, they frequently develop increased intracranial pressure, which can have devastating consequences. Unless these patients are recognized and vigilant follow-up monitoring is instituted at an early age, permanent impairment can result. A retrospective chart review study was conducted, and patients with multiple-suture craniosynostosis who developed symptoms of increased intracranial pressure were selected. The patients were divided into two groups, namely, those with normal sutures and/or head shape at birth (progressive craniosynostosis) (n = 15) and those with abnormal head shapes at birth (classic syndromic craniosynostosis) (n = 12). Clinical and radiological findings typically used to monitor the development of increased intracranial pressure were reviewed for both groups and compared. In addition, mutational analyses were performed. All patients with progressive postnatal craniosynostosis demonstrated clinical, radiological, or ophthalmological evidence of increased intracranial pressure, requiring skull expansion. Those patients displayed papilledema, anterior fontanelle bulge, and thumbprinting more often than did the patients with classic craniosynostosis. Thirteen of 15 patients were given the clinical diagnosis of Crouzon syndrome, which raises the question of whether such patients represent a subset of patients with this syndrome. Mutational analyses for the patients with progressive craniosynostosis demonstrated that, of 13 patients tested, 11 had mutations in exon 7 or 9 of FGFR2, which is a common site of mutations in Crouzon syndrome. The traditional indications of increased intracranial pressure used to monitor patients with classic craniosynostosis can be used to monitor patients with progressive postnatal craniosynostosis, particularly anterior fontanelle bulge, papilledema, and thumbprinting. It is thought that regular monitoring of these characteristics may lead to earlier diagnosis and allow for surgical intervention before the development of undesirable outcomes. It is important for clinicians to be aware of this group of patients, because any delay in diagnosis and treatment can result in severe consequences for the patients.

Integrity signals

Registry

Crossref · 10.1097/01.prs.0000111593.96440.30

Cites

9 of 9 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

  • Crouzon syndrome can present with late-onset pansynostosis, although bicoronal synostosis is most common.

    “Although bicoronal synostosis is most common, Crouzon syndrome can present with late-onset pansynostosis.18,19”

    From Craniosynostosis (2011) · cites this paper as [19]

Cites

Flagged first, then by citations

Cited by

Sorted by citations

Related

Authors
Connolly, J. P., Gruss, J., Seto, M. L., Whelan, M. F., Ellenbogen, R., Weiss, A., Buchman, S. R., & Cunningham, M. L.
Affiliations
Seattle Children's Hospital (US)
ORCID
Venue
Plastic and Reconstructive Surgery
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2004-04
Volume
113
Issue
5
Pages
1313-1323
ISSN
0032-1052 (ISSN-L)
DOI
10.1097/01.prs.0000111593.96440.30
OpenAlex
W2005565398
Cited by
115
References
8
Citations by year
2025: 8 · 2024: 7 · 2023: 3 · 2022: 7 · 2021: 5 · 2020: 7 · 2019: 8 · 2018: 9 · 2017: 4 · 2016: 6 · 2015: 13 · 2014: 6 · 2013: 5 · 2012: 4
FWCI
1.78
Type
journal article
Language
en
Open access
closed
Locations
  • Plastic & Reconstructive Surgery (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • March of Dimes Foundation
  • National Institute of Dental and Craniofacial Research (P60-DE13061-03, P60DE013061)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Craniofacial Disorders and Treatments
  • Health Sciences › Medicine › Neurology › Traumatic Brain Injury and Neurovascular Disturbances
  • Health Sciences › Medicine › Surgery › Pectus Deformity Diagnosis and Treatment
Keywords
intracranial hypertension, Crouzon syndrome, FGFR2 mutations, papilledema, midface hypoplasia, hypertelorism
MeSH terms
Child, Child, Preschool, Craniofacial Dysostosis, Craniosynostoses, Female, Humans, Infant, Intracranial Pressure, Male, Retrospective Studies, Disease Progression

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review