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 (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.
Pr Michel Zerah
Thimothée de Saint-Denis
take place at Armand-Trousseau hospital
CRMR C-MAVEM Trousseau
Service d’obstétrique et de médecine fœtale
26 avenue du Dr Arnold Netter
Phone. +33 (0)1 44 73 52 28
> Send an email
In case of emergency
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”.
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]
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.
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.
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.
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.
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.
In Necker, the C-MAVEM reference center in brief …
* data valid for 2019