Syringomyelia: The Fluid Cavity in the Spinal Cord
Table of Contents
- What a Syrinx Is
- How a Syrinx Forms: Competing Theories
- Causes
- How Common It Is
- Symptoms in Plain Words
- How It Is Found on Magnetic Resonance Imaging
- Small and Incidental Cavities Followed Without Surgery
- Surgery: Outcomes, Recurrence and Complications
- Post-Traumatic Syringomyelia
- Pain Treatment Evidence
- Pregnancy, Delivery and Anesthesia
- Signs Hospitals Treat as Urgent
- What Remains Unknown
- Key Research Papers
- Connections
- Featured Videos
What a Syrinx Is
Syringomyelia is a rare disorder in which a fluid-filled cavity, called a syrinx, forms inside the spinal cord. The cord is normally solid tissue with a tiny central channel. A syrinx widens or tunnels through that tissue and damages the nerve fibers around it. It can stay short or run the length of many vertebrae, and it can stay still for years or grow.
The Chiari Malformation page covers the most common companion condition, its types and the operation at the skull base. This page is about the cavity itself, whatever caused it.
Not every fluid space in the cord is the same thing
A widened central channel is often called hydromyelia or a slit-like syrinx, and it is treated in the literature as a separate finding. One specialist center sorted 2,276 people whose spinal cords showed cystic features between 1991 and 2015. It diagnosed syringomyelia in 1,535, a widened central canal in 635, glioependymal cysts (a rare developmental cyst) in 52, softened cord tissue after damage (myelomalacia) in 52 and cystic tumors in 2. Its author proposes keeping the word syringomyelia for a cavity tied to disturbed cerebrospinal fluid flow, a tethered cord or a tumor inside the cord.
Shape also carries information. In 115 people with cavities not caused by tumors, scans showed three patterns: evenly widened central cavities (28), central cavities bulging to one side (36) and off-center cavities (51). Evenly widened cavities caused no symptoms or only vague ones. Off-center cavities typically appeared with disorders that damage cord tissue, such as injury, meningitis or radiation, and they produced symptoms that matched their position in the cord.
How a Syrinx Forms: Competing Theories
No one has proven how a syrinx forms. The prevailing idea is that disturbed cerebrospinal fluid flow in the space around the cord leads to the cavity, but many theories have been argued and each explains some findings better than others. Cerebrospinal fluid is the clear fluid that bathes the brain and cord and pulses with every heartbeat. The theories below are stated as theories.
The piston theory
Dynamic imaging and ultrasound during surgery in seven people with Chiari I malformation showed the lower cerebellum (the tonsils) thrusting downward with each heartbeat, in step with a sudden squeeze of the cord and syrinx. The authors proposed that the tonsils act as a piston on a partly closed-off fluid space, sending a pressure wave against the surface of the cord. After decompression the thrust stopped, and the syrinx resolved within 1 to 6 months in all seven. A later study of 20 adults and 18 healthy volunteers measured raised pressure waves in the neck region and concluded that the cord is compressed from outside, with syrinx fluid pushed downward at each heartbeat. The same group found the same pattern in 16 people whose cavity persisted after a first operation; after a wider repair the cavity shrank in 15 of them.
The fluid-from-within theories
One critical review calls the fluid-driven theories biophysically implausible, because none names a pump strong enough to force fluid into the cord and none explains why syrinx fluid differs from cerebrospinal fluid. It proposes instead that pressure swings below a blockage strain the small blood vessels and partly break the blood-spinal cord barrier, so that protein-poor fluid leaks out of the blood. Another theory holds that a syrinx is made of extracellular fluid, the fluid between cells, rather than cerebrospinal fluid. In this account, faster fluid flow through a narrowed channel lowers the pressure there and creates a suction effect on the cord with each heartbeat. A third describes syringomyelia as chronic swelling of the cord from trapped extracellular fluid, which begins with blocked cerebrospinal fluid flow or tethering. A fourth points to poor drainage of that fluid through the veins of the cord.
Where the evidence conflicts
The measurements themselves disagree. One review states that syrinx pressure runs higher than cerebrospinal fluid pressure, while another summarizes pressure studies as showing almost identical values. A computer model suggested that the cisterna magna, a fluid pocket at the skull base, works as a shock absorber whose loss raises pressure along the central canal, but that is a simulation, not an observation in patients. For cavities with a tethered cord, a further idea is that pulling forces open the cavity and low pressure then draws fluid in.
Causes
A syrinx is usually a result of something else, so the search for that cause drives the workup. In the specialist series of 1,535 people above, 604 had a problem at the junction of the skull and neck, 543 of them a Chiari I malformation. Another 931 had a problem in the spinal canal: 533 had scarring of the membranes around the cord, 152 had a tumor inside the cord and 69 had a tethered cord. A referral center sees a skewed mix, so these counts do not describe the general population. In a Japanese national survey, Chiari I malformation came first and unexplained (idiopathic) cavities second.
Chiari malformation and other crowding at the skull base
Syrinx cavities are most commonly linked to Chiari malformations. The same crowding can be acquired. A systematic review found 44 patients in whom a mass, most often a meningioma (36%) or arachnoid cyst (32%), had pushed the tonsils down; 82% had a syrinx. After surgery on the cause, the cavity improved or resolved in all but one, and none needed a shunt into the cavity. Draining fluid from the lower spine can also pull the tonsils down: 11 of 70 people (15.7%) given a lumbar shunt for raised brain pressure developed an acquired Chiari malformation, 3 with symptoms, and 1 of the others also had a syrinx. The authors call the figures for cases without symptoms tentative.
Spinal cord injury
A cavity can appear years after an injury. This has its own section below.
Arachnoiditis and other scarring
The arachnoid is one of the thin membranes around the cord. When it scars, cerebrospinal fluid cannot pass freely. Of 107 people with a syrinx and arachnoid scarring, 52 had a history of spinal trauma. The other 55 included 15 with earlier surgery inside the spinal covering, 8 with spinal meningitis and 3 with epidural (peridural) anesthesia; one each had osteomyelitis, spondylodiscitis and subarachnoid hemorrhage, and 26 had no cause found. In a smaller series of 15, the cavity sat mainly at thoracic levels where the scarring was heaviest, and every patient arrived with weakness in two or four limbs.
Tumors
Of 100 tumors inside the spinal cord operated on at one hospital, 45% came with a syrinx, most often with ependymomas and hemangioblastomas. In a series of 38 spinal hemangioblastomas, 21 (55.26%) had a marked syrinx, which collapsed or shrank between two weeks and 15 months after surgery. A review states that a tumor-related syrinx resolves on its own after the tumor is removed. On scans, a mass was found behind 23 of 87 cavities in one radiology series.
Tethered cord
A tethered cord is held down at its lower end, usually by a developmental defect such as those in spina bifida. A syrinx is reported in 21% to 67% of people with spinal defects of this type, depending on the form, and about 60% of them are likely to have symptoms from it. One surgical series found a syrinx in 33 of 135 people with closed defects, and another found a cavity in the lower cord in 32 of 132 people with tethered cord syndrome.
Idiopathic cavities
Idiopathic means that no cause was found. Modern imaging has made these cavities more common as an incidental finding, and their exact origin is unclear. Some have a hidden cause. Of 320 people scanned with cerebrospinal fluid flow imaging, 125 had no obvious cause, and flow blockage was detected in 33. Surgery in the patients with blockage who had no finding on myelography revealed arachnoid cysts or webs. Another author notes that scarring of the arachnoid is hard to diagnose, so these cavities often get labeled idiopathic.
How Common It Is
Measured figures come from only a few places, and they differ by method.
- Japan: a two-stage national postal survey in 2009 collected 1,215 patients (543 men, 672 women) and estimated 1.94 outpatients per 100,000. In the second stage, 22.7% had no symptoms.
- Northwest Italy: a 2011 regional registry census of 436 people with syringomyelia or Chiari syndrome (292 female) estimated a syringomyelia prevalence of 4.84 per 100,000 and an incidence of 0.82 new cases per 100,000.
- Western countries: a 2025 review quotes 8.4 per 100,000.
The numbers depend on who gets scanned. The Japanese authors credit widespread magnetic resonance imaging for finding both cavities without symptoms and idiopathic ones, and a 2023 review states that the prevalence of syringomyelia linked to Chiari I malformation is likely overestimated in the literature. Symptoms of paralysis, sensory loss and chronic pain commonly develop in the third or fourth decade of life.
Symptoms in Plain Words
The fibers that carry pain and temperature feeling cross the cord right next to its center, so a central cavity tends to cut them first. Fibers for light touch, vibration and joint position run farther out and are often spared. This selective loss has a name: dissociated sensory loss.
Lost feeling for pain and heat, in a cape shape
The classic pattern is a cape: loss of feeling across the nape of the neck, shoulders and upper arms. An early sign can be as small as not sensing the heat of a coffee mug. The pattern is typical but not universal. In 210 cases, dissociated sensory loss occurred in 49%, so its absence does not exclude the condition.
Painless burns and injuries
When the arms and hands are affected, people often come for treatment of burns, cuts or blisters on the numb areas. One published case describes a 30-year-old woman whose repeated painless finger infections led to the loss of a fingertip and to shortening of the others.
Weak, thinning hands
When the cavity reaches the motor nerve cells in the front of the cord, muscles waste away. This often shows first in the hands or stays there, then can spread up the forearm toward the shoulder. Reflexes in the arms fade early. Damage to longer pathways produces stiffness in the legs, such as difficulty climbing stairs.
Pain that burns and tingles
In an older series, the commonest first symptom was pain in the head, neck, trunk or limbs, and headache or neck ache made worse by straining was singled out as significant. In 157 adults with Chiari-related cavities, pain and sensory disturbance were the most frequent first symptoms, and symptoms had lasted an average of 8.2 years before surgery. Burning pain with oversensitive skin was described by 51 of 137 people (37%) at presentation.
Curved spine in children
Scoliosis is reported in 25% to 74% of people with syringomyelia, and a syrinx is found in up to 9.7% of people with scoliosis scanned before surgery. In one pediatric series, 19 of 25 children with Chiari I and a syrinx had scoliosis, and 13 of those 19 came in for the curve rather than for nerve symptoms. In 12 children first thought to have ordinary scoliosis who had a missing belly-skin reflex on routine examination, scans showed a syrinx in 10.
Curves can change with the cavity. Among 27 children with scoliosis followed by imaging, the cavity shrank by half or more on its own in 14, and the curve improved by 5 degrees or more in six of them. In 20 people followed for an average of 6.6 years, draining the syrinx improved or stabilized the curve in those under ten with a flexible curve, while most over ten needed scoliosis surgery. Spinal fusion carries added nerve risk: in 36 people with Chiari I and a syrinx matched with 36 with idiopathic scoliosis, neurological complications occurred in 11% vs. 0% and nerve-monitoring difficulties in 28% vs. 3%, with similar curve correction (58% vs. 64%).
Destroyed joints
A joint without protective feeling can break down, a condition called neuropathic or Charcot arthropathy. In 12 people with this complication from a syrinx, 16 joints were involved: 10 elbows, 3 shoulders, 2 interphalangeal joints and 1 wrist, always on the same side as the cavity. A review of 65 people with 71 affected shoulders found that 75% had complete or nearly complete destruction of the upper arm bone head, and described the condition as commonly unilateral.
Size does not predict severity
In a small study of 12 people, muscle wasting, sensory loss and disability bore no significant relationship to the dimensions of the cavity. The authors note that this bears on surgical decisions.
How It Is Found on Magnetic Resonance Imaging
Magnetic resonance imaging is the test that shows a syrinx, its length and its width. A full evaluation pairs standard pictures with dynamic sequences that track cerebrospinal fluid velocity. CT scanning after dye is placed in the spinal fluid (myelography) can help find a focal blockage that surgery might address. In one series, however, flow imaging detected blockages in 6 of 8 patients whose myelography had missed them, all confirmed at surgery.
Ruling out a tumor
Contrast dye is often given to rule out a hidden mass. In 87 cavities, readers using only T2-weighted images (a sequence without dye) detected every underlying mass, a sensitivity of 100% with a specificity of 86%. Larger cavities were more likely to have a mass. The authors conclude contrast may not be required in every case.
Size thresholds differ
There is no single agreed definition. A pediatric study counted a dilated central canal of at least 1 mm across and at least 2 vertebral levels long as a syrinx, while a large pediatric surgical registry study required at least 3 mm. A 2025 review describes a width over 4 mm as commonly regarded as the threshold for neurosurgical evaluation. An international panel of 32 invited experts and patient representatives wrote 63 consensus statements for adults, published in 2022, starting from the observation that international diagnostic criteria were missing.
The slit-like finding
Thin cavities are often incidental findings. In 32 adults with slit-like cavities averaging 2 mm wide and three vertebral levels long, none changed in size over a mean of 32 months of imaging follow-up, and 16 turned out to have another explanation for their symptoms. The authors regard these as remnants of the central canal rather than true syringomyelia.
Small and Incidental Cavities Followed Without Surgery
Most cavities found by chance have stayed quiet in the studies that followed them, though the studies are small. A 2021 review states that there is no evidence for surgery on an incidental cavity without symptoms.
- Adults, Chiari-related, no symptoms: of 9 people followed for more than 10 years, 1 had surgery at 7 years for neurological change and the other 8 showed no change.
- Adults, no cause found: of 12 followed for a mean of 39.9 months, none worsened neurologically, and 11 (91.7%) had no change on scans.
- Widened central canal without deficits: 40 people followed for a mean of 36.9 months had no neurological or imaging worsening.
- Children, idiopathic: among 48 at two centers, symptoms improved in 34%, worsened in 9% and stayed stable in 57%. In the 32 with detailed follow-up imaging, cavities shrank in 25%, grew in 12.5% and were unchanged in 62.5%, with no link between size change and symptoms.
- Children, minimal symptoms: of 17, the cavity was unchanged or smaller in 15 (88%). The 2 that grew both had Chiari malformations and went on to decompression.
- Children, isolated cavity: of 14 found incidentally, 11 stayed without symptoms and with no change; the other 3 were lost to follow-up.
Cavities that do progress
The reassuring results do not cover everyone. In 156 people with mild Chiari-related cavities managed without surgery and followed over more than 1,401 patient-years, 55 clearly worsened, an annual rate of 3.9%. Worsening was linked to obstructive sleep apnea, a positive Babinski sign on examination, and a cavity that did not shrink by itself (hazard ratio 20.3, with a wide confidence interval of 4.8 to 85.8). Among 218 children with an incidentally found Chiari malformation, 36 (16.5%) had decompression, and the presence of a syrinx was the only factor that significantly predicted surgery.
Scan schedules are disputed
Authors reading similar data reach different conclusions. One pediatric group concluded repeated imaging may not be necessary for idiopathic cavities, while another, having seen 2 of 17 cavities enlarge, judged periodic imaging likely appropriate. The studies found for this page did not compare schedules.
Surgery: Outcomes, Recurrence and Complications
The main goal of surgery is to reopen cerebrospinal fluid flow across the point of blockage, not to treat the hole directly. The evidence is almost entirely case series, with one randomized trial in young people. Most figures below come from selected patients at specialist centers.
Decompression at the skull base
The operation itself is described on the Chiari Malformation page. In a 2021 meta-analysis of 276 adults in 13 studies, the cavity improved on imaging in 81.1%. Rates were 90% when the arachnoid was left intact, 79.8% when it was dissected, and 55.6% when only the outer layer of the dura (the tough outer covering) was split. A 2025 systematic review reports about 80% syrinx resolution after decompression with a dura patch. In a pediatric registry of 380 patients, younger age at surgery and a dura patch were both independently linked to cavity resolution, and resolution went with better headache and neck pain.
A 2026 multicenter trial randomized 38 centers and 162 people aged 21 or younger to decompression with a dura patch (78) or without one (84). Complications within 6 months were 14% vs. 6%, a difference that did not reach statistical significance (p=0.11). At 24 months, clinical improvement was 58% vs. 46%, the cavity had narrowed by a mean of 3.08 mm vs. 1.22 mm, and repeat decompression was 3% vs. 14%. Quality of life changed similarly. The authors state that larger trials are needed to weigh the benefits and risks of the two operations.
Long-term results in 157 adults, followed a median of 88 months: 63.06% improved, 30.58% stabilized, 5.73% worsened and 1 died after surgery. Younger age predicted better outcomes; older age and arachnoid scarring predicted worse ones.
Persistent or returning cavities after decompression
A systematic review found residual or returning cavities in 0 to 22% of adults, averaging 6.7%, and found no studies on how best to manage them. It adds that a large cavity running the full length of the cord may leave permanent symptoms even after adequate decompression. Another paper cites failure in 10% to 40% of patients. Reopening and enlarging the repair shrank the cavity in 15 of 16 people in that study. In 14 young people with a stent placed from the fourth ventricle to the neck, the cavity disappeared or shrank by at least 75% in 13 (93%), but the stent dislodged late in 2, 1 had a new neurological deficit and 1 had a fluid leak.
Graft choice and complications of decompression
In 781 children and young people who had a dura patch, overall complications within 6 months were similar with the patient's own tissue (28.7%) and other materials (33.9%), but other materials brought more fluid pockets under the wound (pseudomeningocele) and more meningitis, particularly chemical meningitis. A meta-analysis of 1,404 patients put the meningitis rate at 1% for the patient's own tissue, 2% for donor tissue, 5% for synthetic grafts and 8% for animal-derived grafts, with reoperation at 4%, 9%, 10% and 5% respectively.
Shunts into the cavity
A shunt is a small tube that drains the syrinx into the fluid space around the cord (syringosubarachnoid), the abdomen (syringoperitoneal) or the chest lining (syringopleural). Pooled data on 473 shunts, with a median follow-up of 44 months:
- Syringosubarachnoid: 13% revision, 61% improved, 13% deteriorated.
- Syringoperitoneal: 28% revision, 64% improved, 13% deteriorated.
- Syringopleural: 10% revision, 71% improved, 10% deteriorated.
In 44 people with chest-lining shunts followed a mean of 9.1 years, cavities collapsed early in 43 and 88.6% improved significantly. There were 9 minor complications and 7 serious ones, including permanent deficit, shunt migration and misplacement, and 5 worsened over the long term, 2 of whom later died. After a failed decompression, a shunt into the space around the cord reduced the syrinx in 70% to 85%, with variable long-term results.
Cutting scar tissue (arachnolysis)
Results depend heavily on how far the scarring extends. In 107 people with scarring, shunting alone was followed by recurrence in 92% with focal scars and 100% with extensive ones. Dissecting the scar and widening the space stabilized 83% with focal scars but only 17% with extensive ones. In 288 later patients, 10-year progression-free survival after surgery was 78% when scarring covered no more than 2 spinal segments and 31% when it was extensive. Among 79 unoperated people with follow-up of up to 8 years, 2 worsened. A review of 12 case series (410 people) linked arachnolysis to longer time before symptoms returned, while noting the evidence was all low level. In the 15-patient series on arachnoiditis mentioned above, 8 needed further shunting between 2 months and 12 years after the first operation.
Untethering
In 33 people with a closed spinal defect treated mainly by untethering alone, 31 of the 32 with follow-up scans (97%) had a stable or smaller cavity. Four had a brief increase in the first 6 months that later fell on its own. In one patient whose cord tethered again, the cavity grew 6 months before new urinary symptoms began. In 16 children with tethered cord symptoms and few other findings, all 11 with urinary symptoms and follow-up improved, while the cavity stayed unchanged in 12 and improved in 4. Symptom relief was not necessarily tied to the cavity shrinking. One older series reported better results when the cavity collapsed. A review describes the order in pediatric care as untethering first, with direct surgery on the syrinx only if it worsens after complete untethering.
Post-Traumatic Syringomyelia
A cavity can form at an old injury site and climb the cord years later, taking function that survived the original accident. The reported frequency depends on whether symptoms or scans are counted:
- 30 of 951 people with spinal cord injury (3.2%) developed cervical cavities, about 8% in complete tetraplegia.
- 20 of 449 (4.45%) in a prospective series had symptomatic cavities.
- 28 of 815 (3.43%) in a third series, twice as often after complete as after incomplete injuries. The time from injury to diagnosis ran from six months to 34 years, with a mean of 8.6 years.
- A review states that more than a quarter of injured patients develop syringes, well above these diagnosed series.
Among 138 people with symptomatic cavities at one center, the diagnosis came a median of 15 years after injury, 78.3% had complete injuries, and more than 57% had cavities reaching the neck. Older age and complete injury predicted earlier formation. Less familiar signs include autonomic changes, sensory levels that shift with posture, and weakness that progresses without pain. Bladder problems are among the presenting symptoms; the Neurogenic Bladder page covers that topic. The cause of the first cavity is unknown, though arachnoid scarring and ongoing cord compression appear to matter.
What happens without surgery
Among 76 people who were not operated on, neurological symptoms stayed stable for 10 years in 84% of those for whom the surgeons did not propose an operation because they showed no progression. In contrast, 60% of those who declined a proposed operation worsened within 5 years. In the prospective series above, 10 unoperated people stayed clinically stable for an average of 37 months, and 10 others worsened after a mean of 97 months. One early series saw no benefit from operating on a small cavity.
What happens with surgery
A systematic review of 43 observational studies and 1,803 people reported a surgical complication rate of 26%. Symptoms improved in 43% after surgery and in 2% of those treated without it. They remained stable in 50% and 88%, and worsened in 16% (0.8% died) and 10%. Motor function worsened in around 25% in the surgical analysis. The authors state that no satisfactory standard treatment exists. These groups were not randomized, so they may differ in ways other than treatment. A 2026 review reports an overall improvement range of 58.02% to 93.5% from before-and-after studies. An earlier review put long-term improvement at fewer than half, and warned that a smaller cavity on scans does not guarantee relief or even prevent further loss.
Scar release compared with shunting
- In 137 people, operated cavities shrank in 61%, and 47% of those affected reported less pain. Neurological deterioration at 5 years was 6% in those with trauma but no cord injury, 14% with complete injuries and 39% with incomplete injuries, where scar release is limited by the need to protect remaining function.
- In a non-randomized comparison of 15 shunts and 19 scar releases, scar release stabilized or improved 94%, and shunting brought more recurrence and repeat surgery.
- In 27 people, 13 needed two or more operations. Revisions followed 10 of 16 shunts and 3 of 11 dura patches, a difference that did not reach statistical significance (p=0.0718).
- Older data showed only 49% of drains still working at 6 years.
- In 404 people who had untethering and dura expansion, sometimes with a shunt, sensory and motor scores did not change significantly when one surgery sufficed, but over 90% reported that loss of function had stopped.
Experimental cell therapy
A small phase 2 trial placed 300 million of the patient's own mesenchymal stromal cells inside the cavity in six paraplegic people, with no control group. Scans showed a clear reduction in the cavity at 6 months in all six, and some bladder and bowel measures improved.
Pain Treatment Evidence
Pain is often the symptom that lasts. A 2021 review calls chronic pain the main long-lasting symptom, and an earlier review states that effective treatment remains elusive and that there is no consensus on the best treatment. The searches for this page found no randomized drug trial limited to people with syringomyelia. General background is on the Neuropathic Pain page.
Why it is hard to predict
In 46 people, those with and without pain had no significant difference in the size of their sensory deficits, so damage to the pain pathway alone does not explain the pain. In a second study, people with pain could not be told apart from those without on testing. But among those with pain, higher daily intensity went with more structural cord damage, the first direct link of its kind according to the authors.
Surgery and pain
- Of 37 people with burning pain who had surgery, 22 (59%) improved, and 15 (41%) had no improvement or worse pain despite collapse of the cavity. This pain often responded poorly to analgesics, sedatives, antiepileptics, antispasmodics and anti-inflammatory drugs.
- In 25 people, a cavity sitting toward the back and side of the cord at the level of the pain tended to stay put after surgery, with poor pain relief.
- In 13 people followed prospectively after decompression, pain improved in 11. Longer-lasting symptoms went with worse results.
Drugs tested in related central pain
The following trials studied spinal cord injury or stroke, not syringomyelia, so they apply only indirectly. A 2023 review lists antidepressants and gabapentinoids as first-line drugs for central neuropathic pain in general, alongside self-management, exercise and nerve stimulation.
- Pregabalin, 137 people with spinal cord injury: the trial gave 150 to 600 mg a day for 12 weeks. Mean pain scores ended at 4.62 vs. 6.27 on placebo (from baselines of 6.54 and 6.73, p<0.001). Sleepiness and dizziness were the most common side effects.
- Pregabalin, 220 people with spinal cord injury: the trial gave the same dose for 17 weeks, with significant pain improvement over placebo.
- Duloxetine, 48 people with injury or stroke: the trial gave 60 to 120 mg a day for eight weeks. It did not significantly change pain intensity (p=0.056), though touch- and cold-triggered pain improved.
Procedures
Evidence here is anecdotal. Sympathetic nerve blocks gave prompt but brief relief in two of two people, and cutting the stellate ganglion gave prolonged relief in one. A single case report describes relief after spinal cord stimulation combined with decompression, adhesion release and a surgical lesion at the nerve root entry zone.
Pregnancy, Delivery and Anesthesia
A review of 43 pregnancies in 39 people found no maternal or newborn complications tied to spinal or epidural anesthesia. Three cases raised concerns with general anesthesia: a difficult intubation, a temporary postpartum worsening of neurological symptoms and prolonged muscle paralysis after atracurium. Vaginal delivery has never been documented as causing lasting neurological worsening, according to the authors, who describe all anesthesia techniques as having been performed without major lasting complications.
Signs Hospitals Treat as Urgent
Syringomyelia is usually slow, but it can turn sudden. A review found only 41 reported cases of abrupt onset with Chiari I or a syrinx over three decades. About 87% had no earlier symptoms, onset was frequently set off by a minor injury, and sudden or early death occurred in 19.5%. Most survivors did well. Published emergencies include:
- Sudden or fast-worsening weakness in the legs or arms, or sudden paralysis: motor deficits made up 36.5% of abrupt cases.
- Trouble breathing or a change in the voice: respiratory failure made up 29% of abrupt cases, and paralyzed vocal cords caused acute respiratory distress in one report.
- Trouble swallowing with a hoarse or weak voice: this pairing predicted breathing disturbances during sleep in people with a cavity reaching the brainstem (syringobulbia).
- Fainting or collapse: cardiac arrest made up 14.5% of abrupt cases.
- New loss of bladder control, urine retention or worsening headache in someone with a brain fluid shunt: one woman developed headaches, leg weakness and urine retention over 8 days, with a new full-length cavity on scans that had been absent 3 days before onset. Her shunt had failed, and revising it reversed everything. In people with spinal defects and hydrocephalus, a new or larger cavity may signal shunt blockage.
- After surgery: meningitis, cerebrospinal fluid leak and hydrocephalus are among the complications tracked in the first 6 months.
Breathing during sleep
Among 30 people studied with sleep testing, those with syringobulbia commonly had very prolonged pauses in breathing with low oxygen. Five died of severe respiratory complications within a 10-year follow-up. These abnormalities did not track the size of the cavity. In an earlier report of three patients, two died during sleep and the third was resuscitated during a nap. A review of 13 adults with acute respiratory failure found that eight needed a tracheostomy.
What Remains Unknown
- How the cavity forms. Several theories remain in conflict, and some disagree even about basic pressure measurements.
- Which cavities will grow. Scan measurements did not separate people with and without symptoms in the 10-year Chiari study, and clinical predictors come from a single recent cohort.
- How to manage a cavity that returns. A systematic review found no studies addressing it directly.
- The best operation for scarring or injury. Reviews call for better-designed studies.
- How to treat the pain. Drug evidence is borrowed from other causes of central pain, and pain can persist or worsen after a cavity collapses.
- How many people have it. Figures vary with imaging habits, and no agreed case definition was available until recent consensus work.
Key Research Papers
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- Chang HS, Nakagawa H. Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics. J Neurol Neurosurg Psychiatry. 2003;74(3):344-7. PubMed PMID: 12588922
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