Clinical manifestation of syringomyelia is a rarity in childhood. The clinical symptomatology
and the course of the disease in two of our patients and published data of nine other
children are reported. Compared to adults, kyphoscoliosis is a predominant clinical
symptom occuring early and often progressing rapidly. Flaccid or spastic paresis of
both legs constitute a frequent and early neurological symptom in children. The course
of the disease varies widely. The coincidence of syringomyelia and spinal cord tumors
is frequent, some reasons for this will be discussed. The frequent coincidence of
spinal cord tumors and hydrosyringomyelia might indicate a faulty development of embryonic
spongioblasts which transform on the one hand to proliferating gliosis with secondary
disruption of tissue (syringomyelia), and on the other hand to neoplasias. The demonstration
of a hydrocephalus internus should not lead to the immediate inference of a communication
between the syrinx and ventricular system. When such a communication is excluded by
a combined lumbar and suboccipital myelography, e.g. there is a non-communicating
hydromyelia, a decompressive laminectomy with hydromyelostomy is indicated. An enclosed,
non-communicating form of hydrosyringomyelia cannot be explained by Gardner's theory of hydrodynamic etiology of this malformation, but gives reason for a dysraphic
genesis of the disease.
Infantile hydrosyringomyelia - hydrobulbia - hydrocephalus - neurospinal dysraphism
cord lesion - cervico-medullary anomaly - scoliosis - dissociated sensory alterations
