Syndromic and Systemic Diagnoses Associated With Isolated Sagittal Synostosis.
Journal
Plastic and reconstructive surgery. Global open
ISSN: 2169-7574
Titre abrégé: Plast Reconstr Surg Glob Open
Pays: United States
ID NLM: 101622231
Informations de publication
Date de publication:
Dec 2019
Dec 2019
Historique:
received:
28
08
2019
accepted:
03
10
2019
entrez:
16
6
2020
pubmed:
17
6
2020
medline:
17
6
2020
Statut:
epublish
Résumé
Reports of systemic associations in patients with Isolated Sagittal Synostosis (ISS) are sparse. Craniofacial surgeons, and other providers, should be aware that a significant proportion of patients with ISS may have syndromic or systemic involvement. This study investigates the incidence of systemic disease and syndromic diagnosis in a cohort of patients presenting with ISS (ie, patients with sagittal synostosis without other sutural involvement). This study consists of a retrospective review of patients diagnosed with ISS between 2007 and 2017 at a single institution. Patients were divided according to onset (early <1 year, late >1 year) of ISS. Patient notes were examined for congenital anomalies, systemic conditions, and molecular testing. Only patients with isolated sagittal fusion-meaning, patients with sagittal synostosis and no other sutural involvement-were included. Three hundred seventy-seven patients met the inclusion criteria: systemic conditions were identified in 188/377 (50%) of them. One hundred sixty-one patients with early onset (Group A), and 216 patients with late onset ISS (Group B) were identified. Systemic involvement was identified in 38% of Group A and 60% of Group B, which was statistically significant ( Nearly 50% of the patients initially diagnosed with ISS were found to have some form of systemic involvement. This supports affording full pediatric and genetic evaluation with molecular testing to these children.
Identifiants
pubmed: 32537296
doi: 10.1097/GOX.0000000000002540
pmc: PMC7288895
doi:
Types de publication
Journal Article
Langues
eng
Pagination
e2540Informations de copyright
Copyright © 2019 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons.
Déclaration de conflit d'intérêts
Disclosure: The authors have no financial interest to declare in relation to the content of this article.
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