14 CASES OF COMMUNICATING SYRINGOMYELIA ASSOCIATED WITH CHIARI-I MALFORMATION IN CHILDREN

The authors review their experience of 14 children with syringomyelia and associated Chiari I malformation observed during the last 21 years. Initial symtoms were motor weakness (57 %), sensory signs (50 %), scoliosis (50 %) and bladder dysfunction (21,5 %), while signs of admission were pyramidal signs (78,5 %) with motor deficit (43 %), spinal deformities (64,2 %), syringomyelic syndrome (36 %) and sensory deficit (21,5 %). Four children were investigated with myelography, three with myelography and spinal CT Scan and the last seven cases with MRI. Ten children (71,4 %) underwent a decompression of the Chiari malformation with or without drainage of the cyst and shunting of an associated hydrocephalus. Syringo-peritoneal shunts were used in 3 children and laminectomy with syringo-subarachnoid shunt in 1 case. The neurological symptoms improved in 57 % of the cases, 2 patients deteriorated and 4 patients were lost to follow-up. The authors discuss the clinical symptomatology, the MRI study, the controversis concerning the treatment and concluded with their attitude concerning this pathology in children.