Spinal dysraphism

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 (Hôpital Bicêtre | Pr Fabrice Parker), 7 constituent centers (Necker-Enfants Malades, Armand-Trousseau, Ambroise-Paré, Tenon, Tours, Rennes, Marseille) and 34 competence centers . It is one of the three centers in the Neurosphinx rare disease healthcare network.

IN THE SPOTLIGHT !

Illustration article figaro Zerah

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 >>

Medical
team

Michel Zérah

Medical leader
Pr Michel Zerah
MD, PhD

Timothee De Saint Denis

Thimothée de Saint-Denis
MD

Contact us
 

Standard or multidisciplinary post-natal consultation

CRMR C-MAVEM Necker

Phone. +33 (0)1 44 49 42 67

> Send an email

Prenatal consultations
take place at Armand-Trousseau hospital

CRMR C-MAVEM Trousseau
Service d’obstétrique et de médecine fœtale
26 avenue du Dr Arnold Netter
75012 Paris

Phone. +33 (0)1 44 73 52 28

> 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.

En savoir +

Pr Michel Zérah

Dr Timothée de Saint-Denis

Dr Marie Bourgeois

Dr Syril James

The team also includes neuropsychologists, social workers, school teachers.

PRIUM study: in utero myelomeningocele repair program

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

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

Central sleep apnea in children: experience at a single center.

Mars 2020

Friday 6

1st national day of C-MAVEM centers

> agenda

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 >>

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.

Michel Zerah C-MAVEM, Spina Bifida

Contact information

Necker-Enfants malades university hospital
> Pediatric neurosurgery department

149 rue de Sèvres
75743 PARIS Cedex 15

> Pediatric welcome booklet

In Necker, the C-MAVEM reference center in brief …

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patients followed at least once a year*
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medical consultations*
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day hospitalizations*
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patients in full hospitalization*
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* data valid for 2019