Chiari I-related syringomyelia [surgical management]

ABSTRACT

In this study, ten patients with Chiari I malformation and associated syringomyelia were subjected to surgical suboccipital decompression of the craniocervical junction. Complete neurological recovery with collapse of the syrinx was demonstrated in four patients. Two patients showed good but incomplete recovery along the three- year follow up period in spite of complete collapse of the cavity. Three patients showed stabilization of their neurological condition with no appreciable reversal of the preoperative neurological impairment, but with a reduction of the intramedullary cyst volume. One patient died early postoperatively. A ventriculo-peritoneal shunting of cerebrospinal fluid was done before considering suboccipital decompression for one patient due to the evident hydrocephalus. Postoperatively, one patient developed unilateral bulbar palsy and two patients showed cerebrospinal fluid leak that responded to repeated lumbar puncture satisfactorily. One patient died on the sixth postoperative day due to pulmonary embolism. The progressive destructive nature to the neural tissues by the tense enlarging cyst of syringomyelia makes the surgical intervention inevitable in most of the cases. As a principle, treatment should be directed against the cause of accumulation and intramedullary propagation of the fluid by normalizing the cerebrospinal fluid pathway