Hydrosyringomyelia in Childhood Clinical Aspects, Pathogenesis and Therapy

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