Synostosis of the lambdoid suture: a spectrum

Vinchon, Matthieu; Guerreschi, Pierre; Karnoub, Melodie-Anne; Wolber, Alexis

Type de document :

Article dans une revue scientifique: Article original

DOI :

10.1007/s00381-020-05003-9

PMID :

33404727

URL permanente :

http://hdl.handle.net/20.500.12210/73617

Titre :

Synostosis of the lambdoid suture: a spectrum

Auteur(s) :

Vinchon, Matthieu [Auteur]

Maladies Rares du Développement : Génétique, Régulation et Protéomique (RADEME) - ULR 7364
Guerreschi, Pierre [Auteur]

Advanced Drug Delivery Systems (ADDS) - U1008
Karnoub, Melodie-Anne [Auteur]
Protéomique, Réponse Inflammatoire, Spectrométrie de Masse (PRISM) - U1192
Wolber, Alexis [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]

Titre de la revue :

Child's Nervous System

Nom court de la revue :

Childs Nerv Syst

Numéro :

Pagination :

1991–2000

Date de publication :

2021-01-06

ISSN :

1433-0350

Mot(s)-clé(s) en anglais :

Syndromic synostosis
Faciostenosis
Dystocic birth
Transverse sinus agenesis

Discipline(s) HAL :

Sciences du Vivant [q-bio]

Résumé en anglais : [en]

Lambdoid synostosis (LS) is a rare condition, which is either isolated; associated with sagittal synostosis, the "Mercedes-Benz" syndrome (MBS); or with synostosis of the coronal sutures (oxycephalic form). In addition, ...
Lire la suite >Lambdoid synostosis (LS) is a rare condition, which is either isolated; associated with sagittal synostosis, the "Mercedes-Benz" syndrome (MBS); or with synostosis of the coronal sutures (oxycephalic form). In addition, LS is part of the phenotype of a growing number of genetic diseases. The nosology, pathophysiology, and management are controversial. We decided to review our experience with LS. We reviewed retrospectively pediatric cases of LS proved on CT-scanner, isolated or associated with other conditions, followed in our craniofacial center during the last 15 years, regarding clinical presentation, anatomical lesions, syndromic associations, surgical management, and outcome. We reviewed 48 cases: 6 isolated LS, 22 MBS, and 20 oxycephalic. A syndromic context was present in 72% (up to 80% of oxycephalic cases), and faciostenosis was present in 23%, mostly oxycephalic cases (40%). Transverse sinus agenesis was found in 61% of documented patients. A total of 31% of children had a dystocic birth, up to 45% of MBS. Decompressive craniectomy or cranioplasty was needed in a majority of patients, often young infants, while posterior fossa decompression was mostly performed in older children. LS is rarely isolated and non syndromic; most cases are found in a wide spectrum of diseases, and LS is often associated with sagittal or coronal synostosis. Genetic evaluation is mandatory for LS; conversely, geneticists may require neurosurgical advice for LS in an increasing number of very rare diseases. The surgical management of LS should be tailored according to clinical presentation, age, and syndromic context.Lire moins >

Langue :

Anglais

Audience :

Internationale

Vulgarisation :

Non

Établissement(s) :

CHU Lille
Inserm
Université de Lille

Collections :

Équipe(s) de recherche :

U1008

Date de dépôt :

2022-04-13T07:12:06Z
2024-02-19T10:04:15Z

Numéro de version	Lien	Date de modification
2	20.500.12210/73617*	2024-02-19T10:01:54Z
1	20.500.12210/73617.1	2022-04-13T07:12:06Z

Synostosis of the lambdoid suture: a spectrum

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Synostosis of the lambdoid suture: a spectrum

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