French national reference center for Chiari and vertebral and spinal malformations - Chiari and syringomyelia (C-MAVEM)
The pediatric neurosurgery service at Necker-Enfants Malades hospital has been labeled « rare disease reference center » (constitutive center) since 2017. The 
teams in this service take care of Chiari and syringomyelia, on the one hand, and vertebral and spinal anomalies (spina bifida and dysraphisms as well as all malformations of the spine) from birth to the child-adult transition, on the other hand.
Six priority axes structure its action plan:
- improve screening methods and surgical and medical management of the patient;
- facilitate patient and caregiver access to expert care centers;
- lead a policy of fundamental and translational research;
- development of education, training and information;
- organize the child-adult transition;
- encourage the establishment of European reference networks.
The center works very closely with the Bicêtre center for the child-adult transition.
The constituent center of Necker hospital is attached to the CRMR C-MAVEM. This center is made up of a coordinating center (Bicêtre hospital | Pr Fabrice Parker), 5 constituent centers (Necker-Enfants malades, Tenon, Lyon, Marseille, Nancy) and 28 competence centers.
This reference center is affiliated to the NeuroSphinx rare diseases healthcare network, to the eUROGEN European reference network (ERN).
Keywords: Closed dysraphisms, open dysraphisms, Spina Bifida, myelomeningocele, feotoscopy, congenital or acquired malformations of the spine and spinal cord, attached cord, Antenatal surgery for spinal cord malformations, intradural and extradural tumors, spinal nerve sheath tumors, cone lipoma, diastematomyelia, lipomyelomeningocele, tarlov cyst.
Medical
team
Medical leader
Dr Syril James
MD
Contact us
Standard or multidisciplinary post-natal consultation
Phone. +33 (0)1 44 49 42 67
> Send an email
Prenatal consultations
take place in the obstetrics department of Necker hospital
Phone. +33 (0)1 71 19 62 18
> Send an email
In case of emergency
SAMU
15
Hospital switchboard
Phone. +33 (0)1 44 49 40 00
The Chiari anomaly is a congenital malformation of the cerebellum. It is a rare disease, due to the fact that the lower part of the cerebellum, instead of resting on the base of the skull engages in the occipital hole (foramen magnum) normally occupied by the brainstem, thus increasing the pressure on the spinal cord and brain and can cause multiple symptoms.
The term « Chiari anomaly » is preferred to « Arnold Chiari malformation » because of its nature, which is often acquired and not related to a congenital malformation.
Syringomyelia is a rare disease characterized by the abnormal formation of one or more fluid cavities inside the spinal cord. Syringomyelia can be due to a malformation (present from birth that can be isolated or associated with other abnormalities of the nervous system) or appear as a result of a trauma, a hemorrhage, an infection, a tumor of the nervous system etc.
It can be associated with a Chiari anomaly, a dysraphism or be said to be “isolated”.
Source : association APAISER S&C
- Dr Syril James – Pediatric and adult neurosurgeon, Head of the reference center
- Dr Marie Bourgeois – Pediatric neurologist
- Inès Ben Aissa – C-MAVEM reference center project manager
The team also includes neuropsychologists, social workers, school teachers.
- PRIUM study : in utero myelomeningocele repair program
- ENDOSPIN study : endoscopic myelomeningocele repair program
2021
– [Prenatal path of care following the diagnosis of a malformation for which a novel prenatal therapy is available].
S Hautier, E Kermorvant, N Khen-Dunlop, D de Wailly, B Beauquier, R Corroenne, G Milani, D Bonnet, S James, N Vinit, T Blanc, Y Aigrain, C Colmant, L Salomon, Y Ville, J Stirnemann
Gynecol Obstet Fertil Senol, 2021 Mar, PMID: 33166705 DOI: 10.1016/j.gofs.2020.11.003
– [Prenatal ultrasound prognostic of myelomeningocele at the era of fetal surgery].
P Macé, S Ben Miled, R Corroënne, S James, J Stirnemann
Gynecol Obstet Fertil Senol, 2021 Jul-Aug, PMID: 34020095 DOI: 10.1016/j.gofs.2021.05.003
– Craniosynostosis: Monobloc Distraction with Internal Device and Its Variant for Infants with Severe Syndromic Craniosynostosis.
Giovanna Paternoster, Samer Elie Haber, Roman Hossein Khonsari, Syril James, Eric Arnaud
Clin Plast Surg, 2021 Jul, PMID: 34051901 DOI: 10.1016/j.cps.2021.02.008
– The utility of poly(somno)graphy in evaluating children with Chiari malformation type II before and after surgical intervention: a case series.
Foteini Vagianou, Sonia Khirani, Timothée de Saint Denis, Kevin Beccaria, Alessandro Amaddeo, Sylvain Breton, Syril James, Giovanna Paternoster, Eric Arnaud, Michel Zerah, Brigitte Fauroux
Br J Neurosurg, 2021 Nov 8, PMID: 34747686 DOI: 10.1080/02688697.2021.1999392
2020
– An intermediate form of dysraphism: the MyeLDM.
Michel Zerah, Timothé de Saint Denis, Catherine Garel, Jean-Marie Jouannic, Federico Di Rocco
Childs Nerv Syst, 2020 Jul, PMID: 32346789 DOI: 10.1007/s00381-020-04631-5
– Spinal dysraphism as a new entity in V.A.C.TE.R.L syndrome, resulting in a novel acronym V.A.C.TE.R.L.S.
Aymeric Amelot, Célia Cretolle, Timothée de Saint Denis, Sabine Sarnacki, Martin Catala, Michel Zerah
Eur J Pediatr, 2020 Jul, PMID: 32055959 DOI: 10.1007/s00431-020-03609-4
– Cell therapy for prenatal repair of myelomeningocele: A systematic review.
A Dugas, J Larghero, M Zérah, J M Jouannic, L Guilbaud
Curr Res Transl Med, 2020 Nov, PMID: 32624428 DOI: 10.1016/j.retram.2020.04.004
– Severe and progressive neuronal loss in myelomeningocele begins before 16 weeks of pregnancy.
Selima Ben Miled, Laurence Loeuillet, Jean-Paul Duong Van Huyen, Bettina Bessières, Amel Sekour, Brigitte Leroy, Julia Tantau, Homa Adle-Biassette, Houria Salhi, Maryse Bonnière-Darcy, Aude Tessier, Jelena Martinovic, Frédéric Causeret, Julie Bruneau, Yoann Saillour, Syril James, Yves Ville, Tania Attie-Bitach, Ferechte Encha-Razavi, Julien Stirnemann
Am J Obstet Gynecol, 2020 Aug, PMID: 32283072 DOI: 10.1016/j.ajog.2020.02.052
– Prevention of Neural Tube Defects by Folic Acid Supplementation: A National Population-Based Study.
Benoît de la Fournière, Ferdinand Dhombres, Paul Maurice, Sabine de Foucaud, Pauline Lallemant, Michel Zérah, Lucie Guilbaud, Jean-Marie Jouannic
Nutrients, 2020 Oct 16, PMID: 33081287 PMCID: PMC7603060 DOI: 10.3390/nu12103170
2018
– Two-Port Fetoscopic Repair of Myelomeningocele in Fetal Lambs.
Guilbaud L, Garabedian C, Roux N, Friszer S, Dhombres F, Vialle R, Shah Z, Bessières B, Di Rocco F, Zerah M, Jouannic JM.
Fetal Diagn Ther. 2018 Feb 21. doi: 10.1159/000485655. [Epub ahead of print]
PMID: 29466789
– Acid folic and pregnancy: A mandatory supplementation.
Valentin M, Coste Mazeau P, Zerah M, Ceccaldi PF, Benachi A, Luton D.
Ann Endocrinol (Paris). 2018 Apr;79(2):91-94. doi: 10.1016/j.ando.2017.10.001. Epub 2018 Feb 9.
PMID: 29433770
2017
– Intracerebral gene therapy in children with mucopolysaccharidosis type IIIB syndrome: an uncontrolled phase 1/2 clinical trial.
Tardieu M, Zérah M, Gougeon ML, Ausseil J, de Bournonville S, Husson B, Zafeiriou D, Parenti G, Bourget P, Poirier B, Furlan V, Artaud C, Baugnon T, Roujeau T, Crystal RG, Meyer C, Deiva K, Heard JM.
Lancet Neurol. 2017 Sep;16(9):712-720. doi: 10.1016/S1474-4422(17)30169-2. Epub 2017 Jul 14.
PMID: 28713035
– Fetoscopic patch coverage of experimental myelomenigocele using a two-port access in fetal sheep.
Guilbaud L, Roux N, Friszer S, Garabedian C, Dhombres F, Bessières B, Fallet-Bianco C, Di Rocco F, Zerah M, Jouannic JM.
Childs Nerv Syst. 2017 Jul;33(7):1177-1184. doi: 10.1007/s00381-017-3461-7. Epub 2017 May 26.
PMID: 28550526
– Sleep-disordered breathing and its management in children with achondroplasia.
Tenconi R, Khirani S, Amaddeo A, Michot C, Baujat G, Couloigner V, De Sanctis L, James S, Zerah M, Cormier-Daire V, Fauroux B.
Am J Med Genet A. 2017 Apr;173(4):868-878. doi: 10.1002/ajmg.a.38130. Epub 2017 Feb 27.
PMID: 28239978
– Results of the prophylactic surgery of lumbosacral lipomas: the pendulum of management?
Roujeau T, James S, Forin V, Zerah M.
Childs Nerv Syst. 2017 Apr;33(4):561-562. doi: 10.1007/s00381-017-3345-x. Epub 2017 Feb 24. No abstract available.
PMID: 28236066
2016
– Central sleep apnea in children: experience at a single center.
Orlane Felix, Alessandro Amaddeo, Jorge Olmo Arroyo, Michel Zerah, Stephanie Puget, Valerie Cormier-Daire, Genevieve Baujat, Graziella Pinto, Marta Fernandez-Bolanos, Brigitte Fauroux
Sleep Med, 2016 Sep, PMID: 27823711 DOI: 10.1016/j.sleep.2016.07.016
Contact information
Necker-Enfants malades university hospital
> Pediatric neurosurgery department
149 rue de Sèvres
75743 PARIS Cedex 15
In Necker, the C-MAVEM reference center in brief …
* data valid for 2022