French national reference center for Chiari and vertebral and spinal malformations - spinal dysraphism (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 collaborates very closely with the CRMR C-MAVEM of Armand-Trousseau AP-HP Hospital (fetal medicine) coordinated by Pr Jean-Marie Jouannic and the Bicêtre center for the transition from child to adult.
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).
IN THE SPOTLIGHT !
Preparing for pregnancy: a reflex still insufficient
Le Figaro | 10/05/2020 | Professor Michel Zérah
Healthcare professionals in particular recommend taking vitamin B9 before conception in order to avoid serious disabilities.
Read the article in French >>
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
Spinal dysraphism
The term « Spina Bifida » formerly used tends gradually to be replaced by that of « dysraphism (spinal) » which includes all forms of this pathology, from the most minimal to the most extensive. The Spina Bifida (thorny bifid) only concerns the localized non-closure of the posterior part of the spine.
Classically, closed dysraphisms are separated from open dysraphisms according to the skin covering with regard to the vertebral-medullary anomaly.
Many of these defects can be prevented by adequate prevention with folic acid (vitamin B9).
Open dysraphisms (spina bifida aperta) are the most severe malformations:
myelocele,
myelomeningocele,
rachischisis.
The consequences of this spinal-medullary development disorder are:
paraplegia (paralysis of the lower limbs),
hydrocephalus
and a malformation of Arnold Chiari (consequence of the spinal cord attached and cerebrospinal fluid leakage during intrauterine life),
urinary and anorectal incontinence.
These disorders vary in intensity depending on the level of the lesion and its extent.
Occult or closed dysraphisms (also called spina bifida occulta) are composed of a very heterogeneous group of malformations.
Their anatomical forms and clinical expressions are very variable and, as a rule, are less severe than open dysraphisms:
filum lipomas,
spinal lipoma (spina lipoma, cone lipoma, lipomyelocele, lipomyelocystocele),
LDM (Limited Dorsal Myeloschisis), dermal sinus,
diastematomyelia,
neurenteric cyst,
syndomic forms (Currarino, VACTERL, pallister Hall, cloaca …).
Vertebral malformations
The C-MAVEM-Necker constitutive center takes care of the neurosurgical part of vertebral malformations. These malformations are taken care of by the team of Professor Thierry Odent (Tours). This service is part of the CRMR for constitutional bone diseases (MOC) affiliated with the OSCAR rare diseases health sector and the CRMR for chiari and vertebral and spinal malformations (C-MAVEM) affiliated to the Neurosphinx rare diseases health sector.
These malformations are, among others:
- Constitutional bone diseases affecting the hinge and the spine: …
Spasticity
The C-MAVEM-Necker constitutive center is implementing a specific spasticity management program in collaboration with the Établissement national de convalescence.
- 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.
- Management of Filum terminale lipomas (2021)
- Management of dysraphism in the perinatal period (2021)
- Spina Bifida – Dysraphism – Managing intestinal disability (2021)
- Spinal dysraphism (Spina Bifida) – urological management in adulthood (2020)
- Management of the patient with Spina Bifida in physical medicine and rehabilitation (2015)
- PRIUM study : in utero myelomeningocele repair program
- ENDOSPIN study : endoscopic myelomeningocele repair program
2022
– Laparotomy-Assisted 2-Port Fetoscopic Repair of Spina Bifida Aperta: Report of a Single-Center Experience in Paris, France
Chloé Arthuis, Syril James, Laurence Bussieres, Shushanik Hovhannisyan, Romain Corroenne, Yves Ville, Julien Stirnemann
Fetal Diagn Ther 2022;49(9-10):377-384. Aug 31.
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
Mars 2020
Friday 6
1st national day of C-MAVEM centers
Preparing for pregnancy: a reflex still insufficient
Le Figaro | 10/05/2020 | Professor Michel Zerah
Healthcare professionals in particular recommend taking vitamin B9 before conception in order to avoid serious disabilities.
Read the article in French >>
Spina bifida: management and prevention
Paris Match | 07/16/2018 | Professor Michel Zerah
Professor Michel Zerah, head of the pediatric neurosurgery department at the Necker-Enfants malades hospital in Paris, provides an update on this spinal cord malformation, which often has serious consequences.
Read the article in French >>
In utero surgery is progressing but remains an exception
Le Figaro santé | 01/27/2015 | Professor Michel Zerah
Si elle se développe pas à pas avec de nouvelles technologies, la chirurgie fœtale reste avant tout un type d’intervention d’exception.
Read the article in French >>
A baby operated on… before he was even born
Paris Match | 11/19/2014 | Professor Michel Zerah
The Assistance Publique – Hôpitaux de Paris hospital group announced Tuesday the success of an in utero intervention, which limited the after-effects in a baby with a malformation called spina bifida.
Read the article in French >>
Surgery on a fetus: « We limit the handicap but the baby is not cured », says Professor Zerah
RTL | 11/19/2014 | Professor Michel Zerah
Professor of pediatric neurosurgery at Necker hospital, Michel Zerah performed the first intrauterine operation to repair a congenital malformation on a five-month-old fetus.
Read the article in French >>
Spina bifida: première chirurgie réparatrice sur un foetus en France
Le Point | 11/18/2014 | Professor Michel Zerah
An operation on a fetus to repair a congenital malformation called « spina bifida » has been performed for the first time in France, specialists from the Paris public hospital group Assistance Publique-Hôpitaux de Paris (AP-HP) said Tuesday.
Read the article in French >>
On the occasion of world Spina Bifida day, which took place on October 25, 2020, professor Michel Zerah (pediatric neurosurgeon) wishes to raise awareness among expectant mothers about the good reflexes to adopt before and during their pregnancies. Indeed, a simple regular intake of vitamin B9 two months before pregnancy and then in the first two months of gestation would reduce by 70% the risks of appearance of this malformation which today affects 1 woman out of 1000.
The 7th edition of the national days of the NeuroSphinx network, in partnership with the reference center for chiari and vertebral and spinal malformations – spinal dysraphism (C-MAVEM), the reference center for rare anorectal and pelvic malformations (MAREP) and the reference center for rare urinary tract malformations (MARVU), was held on Thursday 2 and Friday 3 March 2023, at the Institut Imagine, These two days of program, gathering coordinators, medical (and paramedical) teams of the rare disease centers, project team of the network, patient associations and institutions (DGOS and BNDMR), allowed you to be informed about the current events and the dynamism of the actors of our network, dedicated to rare spinal and pelvic malformations.
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 2021