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The syringomyelia of the child

Wednesday 18 February 2009

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The different types syringomyelias of the child are rare pathologies which can occur in two quite different contexts. Those connected with an abnomaly of the flow circulation of cerebrospinal liquid at the level of cranio cervical junction and the others.

Contrary to the adult where the malformation of Chiari suns up the majority of abnomalies of junctions, the children also have many bone malformations which can be linked to syringomyelia (achondroplasy, muccopolysaccharidosis, trisomy 21, Marfan, imperfecta osteogenesis) or more rarely, sequels of neonatal sufferings (basal arachonoïditis). How it appears varies according to the age.

With newborn and during the first months of life, bone abnomalies are more frequent than Chiari and their symptoms are cervical pain, or an axial hypotonia or respiratory disorders and can carry out a true syndrome of apnea of sleep or are sometimes a systematic discovery. In the older child or the teenager Chiari prevails and comes to light through disorders of rachidian statics (scoliosis) sometimes linked to neurological signs with minima (abolition of the abdominal cutaneous reflexes).

The scoliosis is also the revealing sign of the isolated cases concerning the child and the teenager. These cases of syringomyelia are rarely evolutionary and simply modify the scoliosis outcome. Post traumatic syringomyelia are exceptional as far as children are concerned.

Lastly, approximately 20 % of the lombosacral dysraphic state (low attached cord) present syringomyelia. These syringomyelia are regarded as malformation in the vast majority of cases.

They are stable and there is no added extra clinical sign to those of dysraphic states and they usually do not require a surgical intervention.

It should be underlined that contrary to the adult, rebellious painful syndromes to the treatment are very rare with children.

Professor M. ZERAH
Neurosurgery Hospital Necker