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Jörg Klekamp, Giorgio Iaconetta, Ulrich Batzdorf and Madjid Samii

Object. Syringomyelia is often linked to pathological lesions of the foramen magnum. The most common cause is hindbrain herniation, usually referred to as Chiari I or II malformation. Foramen magnum arachnoiditis without either Chiari I or II malformation is a rare cause of syringomyelia. The authors undertook a retrospective analysis of 21 patients with foramen magnum arachnoiditis (FMA) and syringomyelia treated between 1978 and 2000 to determine clinical course and optimum management.

Methods. In the review of records, 21 patients with FMA and syringomyelia were documented. A stable clinical course was demonstrated in three patients in whom surgery was not performed, and one patient refused surgical intervention. Seventeen patients underwent 23 operations to treat progressive neurological disease. Of these 23 operations, 18 involved opening of the foramen magnum, arachnoid dissection, and placement of a large dural graft. One patient underwent insertion of a ventriculoperitoneal shunt for treatment of accompanying hydrocephalus, one patient received a cystoperitoneal shunt for an accompanying arachnoid cyst; two syringoperitoneal and one syringosubarachnoid shunts were also inserted. Hospital and outpatient files, neuroimaging studies, and intraoperative photographic and video material were analyzed. Additional follow-up information was obtained by telephone interview and questionnaires.

Standard and cardiac-gated magnetic resonance imaging studies are the diagnostic procedures of choice in these patients. Sensory disturbances, dysesthesias, and pain were the only symptoms likely to improve after foramen magnum surgery. Motor weakness and gait disturbances, which were severe in a considerable number of patients, and swallowing disorders tended to remain unchanged. As a consequence of the rather severe arachnoid lesions in most patients, clinical recurrences were observed in 57% over a 5-year period.

Conclusions. Surgery for FMA and syringomyelia has to provide clear cerebrospinal fluid pathways between the cerebellopontine cisterns, spinal canal, and fourth ventricle. If this can be achieved successfully, the syrinx decreases in size and the clinical course of the patient may even improve. In patients with severe and widespread areas of arachnoiditis, however, multiple operations may be required at least to stabilize the clinical course.

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Jörg Klekamp, Ulrich Batzdorf, Madjid Samii and Hans Werner Bothe

✓ The authors conducted a retrospective study of 107 patients treated for syringomyelia associated with arachnoid scarring between 1976 and 1995 at the Departments of Neurosurgery at the Nordstadt Hospital in Hannover, Germany, and the University of California in Los Angeles, California. Twenty-nine patients have not been surgically treated to date because of their stable neurological status. Seventy-eight patients with progressive neurological deficits underwent a total of 121 surgical procedures and were followed for a mean period of 32 (±37) months. All patients demonstrated arachnoid scarring at a level close to the syrinx. In 52 patients the arachnoid scarring was related to spinal trauma, whereas 55 had no history of trauma and developed arachnoid scarring as a result of an inflammatory reaction. Of these, 15 patients had undergone intradural surgery, eight had suffered from spinal meningitis, three had undergone peridural anesthesia, and one each presented with a history of osteomyelitis, spondylodiscitis, and subarachnoid hemorrhage. No obvious cause for the inflammatory reaction resulting in arachnoid scarring could be ascertained for the remaining 26 patients.

The postoperative neurological outcome correlated with the severity of arachnoid pathology and the type of surgery performed. Shunting of the syrinx to the subarachnoid, pleural, or peritoneal cavity was associated with recurrence rates of 92% and 100% for focal and extensive scarring, respectively. Successful long-term management of the syrinx required microsurgical dissection of the arachnoid scar and decompression of the subarachnoid space with a fascia lata graft. This operation stabilized the preoperative progressive neurological course in 83% of patients with a focal arachnoid scar. For patients with extensive arachnoid scarring over multiple spinal levels or after previous surgery, clinical stabilization was achieved in only 17% with this technique.