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Syringomyelia: a Look at Surgical Therapy Syringomyelia: A Look at Surgical Therapy J. GRAFTON LOVE, M.D. AND RICHARD A. OLAFSON, M.D. Mayo Clinic and Mayo Foundation, Section of Neurologic Surgery, and Mayo Graduate School of Medicine, University of Minnesota, Rochester, Minnesota YRINGOMYELIA and syringobulbia are had complete follow-up for ~ years or more. characterized by an accumulation of Thirty-five patients with syringomyelia S fluid in a cyst within the substance of did not have associated anomalies and were the spinal cord or medulla or both. These without evidence of other pathologic pro- cysts arise from various pathological pro- cesses related to the central nervous system. cesses and have been treated by surgical Three of these, however, had minimal platy- incision and drainage and by irradiation3 ,s basia, but this did not contribute to the Different materials have been inserted be- patients' symptoms. The remaining 5 pa- tween the syrinx and the subarachnoid space tients had syringomyelia or syringobulbia to provide permanent drainage. and syringomyelia associated with Arnold- In 1953, while attending a meeting of the Chiari malformations. These 5 eases will be Society of Neurological Surgeons in New discussed separately. Orleans, one of us heard Dr. Dean Echols explain his technique of using a tantalum Symptoms wire to maintain a fistula between a syrinx The common complaint in syringomyelia of the spinal cord and the spinal subaraeh- is one of an asymmetrical diminution of noid space and saw his patients who had had sensation plus muscular weakness at and this operative procedure. The results were below the level of the cyst. Because of the impressive, and we have employed this preference of the syringomyelic process for technique at our clinic since that time. the cervical and cervicothoracic region, In an effort to determine whether results diminution of sensation in the hands and have improved since the introduction of this forearms is a common early finding. Nine- technique, we have reviewed the records of teen of our 40 patients said that radicular patients with syringomyelia or syringobulbia pain, corresponding to the level of the and syringomyelia but without associated syrinx, had been an early symptom. The neoplasm who were operated on at the Mayo pain, as described, was indistinguishable Clinic between 1948 and 196~. from radicular pain produced by other Evaluation space-occupying intraspinal masses. The symptom-complex most typical of syringo- Forty patients underwent 44 operative myelia includes bilateral diminution of procedures for syringomyelia or syringo- temperature perception and asymmetrical myelia and syringobulbia. These patients hypalgesia. Proprioception and vibratory do not represent all those with syringomyelia sensation are generally reduced to a moder- who were seen at the Mayo Clinic, as only ate degree, whereas diminution of touch and surgically verified cases were studied. We pressure may be minimal. Characteristi- have analyzed the subjective complaints and cally, the motor disturbances in syringo- objective neurological findings both before myelia are those of weakness at or below the and after operation and throughout the fol- upper level of disturbed sensation. The low-up period. In addition we have reviewed weakness at the upper level of sensory dis- diagnostic studies, operative findings, and turbance is generally associated with atrophy surgical methods employed. All patients con- and hyporeflexia indicative of denervation, sidered as having adequate follow-up studies whereas in the lower extremities there are had neurological re-examinations at least 1 hyperreflexia, pathologic reflexes, and year after operation; the longest follow-up spasticity indicative of corticospinal-tract was 10 years. The majority of our patients involvement. The major motor disturbances Received for publication June ~8, 1965. distally are generally opposite the side of Revision received Octoher ~5, 1965. major sensory disturbance. 714 Surgical Treatment of Syringomyelia 715 Sex and Age of 900 mg. In the remaining 9 patients, the In syringomyelia without complications, protein values of the fluid from the cysts males tend to be affected more frequently was at least 20 rag. below the values of the than females (23 males to 12 females). Of cerebrospinal-fluid protein. The range of the 5 patients with associated Arnold-Chiari values of syrinx-fluid protein in these 9 malformation, 3 were males and 2 were fe- patients was 10 to 40 rag./100 ml. males. Twenty-one of the 40 patients were between 20 and 40 years of age. The ages of Surgical Methods patients at the time of surgery ranged from The surgical procedures were perfolnned 12 to 60 years. A few patients did not have by several neurological surgeons, providing onset of symptoms prior to the age of 40 various approaches for comparison. The pro- years. One of the 5 patients with syringo- cedures included the following: laminectomy myelia-syringobulbia and associated Arnold- alone, laminectomy with simple evacuation Chiari malformation had a Klippel-Feil of the syrinx, laminectomy with syringostomy anomaly with fusion of the C-1 and C-2 for drainage by the use of various materials vertebrae as well. Of the other 35 patients, to maintain cyst and subarachnoid commu- 30 patients had cervical syrinxes, 4 patients nication (rubber drain, polyethylene tubing, had thoracic syrinxes, and 1 patient had a cotton wicks, silk sutures, and tantalum-wire cervical and thoracic syrinx. sutures), and suboccipital craniectomies with cervical laminectomies alone and with both Diagnosis simple drainage of cysts and syringostomies In all 35 patients with uncomplicated with permanent-drain materials. In all but 15 syringomyelia, the history and neurological of the 44 operative procedures, some form of examination suggested the diagnosis. In this permanent drainage of the syrinx was at- group, 27 lumbar punctures were pe~5~ormed. tempted. Two of these operations were re- Jugular compression done at the time of the operations in which the spinal cyst was lumbar puncture revealed no evidence of found to be collapsed. The initial procedure subarachnoid-space block in 19 cases. There in 1 patient had been simple drainage of a was evidence suggestive of partial block in 5 thoracic cyst; and the second patient had had and of complete block in 3 cases. Seventeen a tantalum-wire syringostomy for a cervical contrast-medium (Pantopaque) myelograms syrinx previously. and 1 air myelogram were done in this group of patients. Of these 1~ demonstrated widen- Results of Surgical Treatment ing of the spinal cord at the level of the Results of surgical therapy were classified syrinx, and 2 showed no abnormal changes. as excellent, good, or poor. Results were Three of the contrast-medium myelograins considered excellent when detailed examina- and the 1 air myelogram revealed complete tion revealed definite objective improvement block at the level of the spinal-cord cyst. In in neurological status and when subjective the majority of patients with syringomyelia improvement was noted by the patients or syringobulbia and syringomyelia, the themselves. Results were considered good level of the lesion can be determined by when the neurological deficit did not increase neurological methods, and thus contrast or decrease and no subjective increase in myelography is not necessary. deficit was noted by the patients them- The value for cerebrospinal-fluid protein selves. However, most patients of this second was elevated above the normal of 45 mg./100 group believed that they had definitely been nil. in 18 of the 27 patients in whom it improved by operation. Results were classi- was studied. Four patients had values of 750 fied as poor if the neurological deficit in- rag. or greater; 2 of these had thoracic cysts creased after surgery even though the pa- and 2 had cervical syrinxes. Values for pro- tients themselves may have considered their tein content in fluid removed from syringo- condition improved. myelic cavities ranged from 10 to 180 nlg. Of the 35 patients with syringomyelia /100 ml. in 10 recorded specimens. The 1 alone, 5 were lost to follow-up. The neuro- specimen of 180 mg. was taken from a patient logical status of these 5 patients 7 to 10 days who had a cerebrospinal-fluid protein value after operation was essentially unchanged .
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