Syringomyelia is a condition caused by a fluid-filled cavity, or syrinx, which forms within the spinal cord.
A syrinx can refer to an abnormal gliotic-lined fluid-filled cavity located within the spinal cord parenchyma (syringomyelia) or a focal dilation of the central canal (hydromyelia).1,2 These two terms have been used interchangeably in the literature and occasionally are combined as syringohydromyelia. There are conflicting viewpoints regarding distinguishing these two entities based on clinical and radiographical features2 versus classifying all cases under syringomyelia and distinguishing based on proposed pathogenesis.3
The syrinx can span one or more spinal levels or even the entire length of the spinal cord, causing a variety of motor, sensory, and autonomic symptoms based on location.
In the pediatric population, syringomyelia usually occurs in the setting of congenital abnormalities, which may result in complex medical and rehabilitation needs that require a multidisciplinary approach to care.
Syringomyelia may be congenital or acquired:
- Neural tube defects:
- Tethered cord syndrome
- Chiari malformations (Type I most common)
- Meningitis, arachnoiditis
- Transverse myelitis, sarcoidosis, multiple sclerosis
- Spinal cord injury, postsurgical, arachnoid scarring
- Spinal cord tumors (ependymomas and hemangioblastomas)
- Extramedullary tumors
The most common etiology of syringomyelia in both adult and pediatric patients is Chiari I malformation, representing 48-50% of adult cases4,5 and 43.2% of pediatric cases.6 In adults, the next most common etiologies include idiopathic (15.7%), Chiari II malformation (8.1%), post-traumatic (7.5%), spinal cord tumor (5.2%), and arachnoiditis (4.9%). 4 In pediatric populations with syrinx, the next most commonly associated conditions are idiopathic (30.6%), spinal dysraphism (7.4%), tumor (5.5%), and tethered cord (4.8%).6
Epidemiology including risk factors and primary prevention
Syringomyelia most commonly presents in young adults ages 20 to 40, although all ages may be affected.7 Estimates of prevalence vary between 1.94-8.4 per 100,000.4,5 Men and women are equally affected.4,7
Risk factors for developing syringomyelia include a family history of syringomyelia as well as specific factors related to predisposing conditions:
Chiari I malformation (CM-I): CM-I is commonly associated with spinal cord syrinx. An estimated 12-22.9% of pediatric patients with CM-I also have a syrinx.8,9 Estimates are higher in patients undergoing surgery for CM-I, in which syrinx is seen in as high as 60-85% of patients.10 CM-I associated syringes are more common in the cervical spinal cord.6 There is an increased likelihood of spinal syrinx with lower tonsil position.10
Myelomeningocele: The presence of syringomyelia in patients with myelomeningocele varies from 25 to 45%.11 There is an association between untreated or inadequately treated hydrocephalus and symptomatic syringomyelia in patients with myelomeningocele.12
Trauma-associated: The incidence of post-traumatic syringomyelia ranges from <1 to 7% in patients with clinical symptoms and is as high as >50% on imaging studies.13 Increased risk of development of syringomyelia within 5 years after spinal cord injury may be associated with complete SCI and age greater than 30 years.13 Dislocated spine fractures and spinal stenosis may also increase risk of development of posttraumatic syrinx.14
Tumor-associated: Syrinx occurs in 25 to 58% of patients with intramedullary spinal cord tumors and are typically seen in the lower cervical and upper thoracic regions. Ependymomas are most frequent, followed by hemangioblastomas and cavernomas.15
Familial: Familial syringomyelia is rare. Familial cases of both autosomal dominant and recessive inheritance patterns have been reported and are felt to be due to gene abnormalities in spinal cord development.16,17 Some cases have been associated with Chiari I malformation while others have revealed isolated thoracic syringomyelia with only mild symptoms.16
The pathophysiology of syringomyelia is not completely understood. Several theories have emerged over time, most of which focus on altered cerebrospinal fluid flow dynamics. Theories regarding pathogenesis of syringomyelia can be divided into three categories as outlined by Blegvad, et al:3
- Altered cerebrospinal fluid flow dynamics.
- Direct communication between the fourth ventricle and syrinx.
- Transmedullary infiltration of CSF through the perivascular space.
- Parenchymal formation due to blood plasma ultrafiltration.
- Intramedullary tissue damage due to hemorrhage or infarction.
- Intramedullary tumor with secretory capabilities.
Specific theories related to altered CSF flow dynamics are categorized below:
(a) Direct communication between the fourth ventricle and syrinx.
- Gardner’s “water hammer” theory (1965): Gardener hypothesized that anatomic abnormalities in the hindbrain cause blockage at the craniocervical junction and/or cisterna magna. Arterial systolic pulsations generated by the choroid plexus transmit CSF via a “water hammer” effect from the fourth ventricle into the central canal, leading to its dilation. The central canal may rupture at various points producing syringomyelia.18
- Williams’ “suck and slosh” theory (1969): Williams proposed that an obstruction at the foramen magnum could act as a valve, allowing CSF to cross the foramen magnum rostrally more effectively than caudally. Valsalva-like maneuvers force CSF from the spinal into the intracranial cavity, causing prolonged elevation of intracranial CSF pressure at which time CSF may be “sucked” from the fourth ventricle into the central canal.18 The syrinx is maintained by longitudinal fluid motions that “slosh” inside the cavity during valsalva maneuvers.3
(b) Transmedullary infiltration of CSF through the perivascular space.
- Ball and Dayan’s infiltration theory (1972): In patients with foramen magnum obstruction who perform valsalva like maneuvers, increased spinal subarachnoid pressure forces CSF into the spinal parenchyma via the perivascular space (Virchow-Robin space), which becomes dilated. CSF subsequently accumulates from the perivascular entrances into the spinal parenchyma, forming a syrinx.3
- Oldfield’s piston theory (1994): During the cardiac cycle, the cerebellar tonsils are rhythmically compressed in a piston-like motion creating a spinal CSF pulse wave that forces CSF into the parenchyma of the spinal cord. The fluid in the syrinx is forced longitudinally in the spinal cord similar to Williams’ “slosh” effect.3
- Heiss’s theory (2012): In primary spinal syringomyelia, blockage in the subarachnoid space increases spinal subarachnoid pulse pressure above the block, which propagates CSF pressure waves into the spinal cord during cardiac systole and creates a syrinx. After the syrinx is formed, it continues to progress through subarachnoid pressure waves that compress the external surface of the spinal cord and propel the syrinx fluid.19
(c) Parenchymal formation due to blood plasma ultrafiltration.
- Levine’s theory (2004): Pulsatile fluctuations of CSF pressure during the cardiac cycle and valsalva maneuvers produce transiently higher CSF pressure above the area of subarachnoid obstruction. Mechanical stress and venous and capillary dilation partially disrupt the blood–spinal cord barrier, which allows ultrafiltration of crystalloids and accumulation of a protein-poor fluid, resulting in a syrinx.18
- Koyanagi and Houkin’s theory (2010): Decreased compliance of the spinal subarachnoid space as well as reduced compliance of the posterior spinal veins result in disturbed absorption of the extracellular fluid through the intramedullary venous channels, leading to formation of a syrinx.20
Other than CSF flow dynamics, an additional potential mechanism for syrinx formation is intramedullary tissue damage from hemorrhage and infarction in the cases of trauma, spinal cord tumors, and tethered cord.3 In syringomyelia due to spinal cord tumors, tumor syringes have been found to have an elevated protein concentration compared to normal CSF and syrinx fluid associated with other etiologies, suggesting a possible unique mechanism related to the secretory ability of the tumor itself.15
Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)
The natural history of syringomyelia is variable and dependent on etiology. Some patients remain asymptomatic with syringomyelia found incidentally, while others present with progression in symptoms. Syringomyelia often remains stable with nonoperative treatment.
CM-I: Syringomyelia has been shown to improve in 78% of patients with CM-I who undergo surgery. Clinically, 75% of patients experience improvement, 17% have no change, and 9% experience worsening of neurological outcomes post-operatively.21
Idiopathic: In one study of 48 pediatric patients with idiopathic syrinx managed conservatively, clinical symptoms improved in 34%, 57% remained stable, and 9% worsened over a 24 month follow-up. Syrinx size decreased in 25% of patients and remained stable in 62.5% without intervention. There was no correlation between clinical symptoms and changes in syrinx size.22 In idiopathic cases, patients often have no symptoms attributable to the syrinx, and only about 9% have progression.22 These data suggest that the natural history of idiopathic syrinx is benign; however, further studies are needed.
Myelomeningocele: Syringomyelia usually develops around age four to seven after surgical repair of the defect during the neonatal period.11 There is a lack of data regarding the natural history of syringomyelia in patients with myelomeningocele. Although there is a high estimate for the prevalence of syringomyelia based on imaging studies (25-45%), only an estimated 2.3% of patients with myelomeningocele will develop clinically active syringomyelia.12
Trauma-associated: Most patients with post-traumatic syringomyelia who are asymptomatic or with mild symptoms will remain stable over time. A recent study revealed that 84% of patients with post-traumatic syringomyelia without neurological progression remained stable over 10 years. In patients with neurological progression who did not undergo surgical intervention, 60% experienced further neurological progression within 5 years.14 Deterioration of motor and sensory function, pain and increased spasticity are the most commonly observed symptoms in patients with post-traumatic syringomyelia.13 In patients who underwent surgical intervention, 52% had improvement in their symptoms, 37% remained stable, and 11% had progression.23 Many patients (51%) required more than one surgical intervention.23
When syringomyelia presents symptomatically, progressive neurologic deterioration may appear, but it may take years from onset to diagnosis. Presymptomatic diagnosis can be made by magnetic resonance imaging (MRI). Syringomyelia involving the cervical spinal cord usually progresses slowly.
With extension of the syrinx, progression of clinical features may include the following:
- Dissociated sensory loss due to injury to the spinothalamic fibers, which mediate pain and temperature, with preservation of light touch, vibration, and proprioception. This can occur in either or both arms or across the upper torso in a shawl-like distribution.
- Enlargement involving the posterior column causes lower extremity loss of position and vibration sense.
- Dysesthesias, defined as severe deep aching pain involving neck and shoulders, sometimes following a radicular distribution in the upper extremities and trunk.
- Motor loss with extension to anterior horns with diffuse muscle atrophy beginning in the hands progressing proximally to the forearm and shoulder girdle.
- Impaired bowel, bladder, and sexual dysfunction are uncommon until end-stage spinal cord dysfunction.
- Horner’s syndrome, reflecting damage to sympathetic fibers in the intermediary cell columns.
- With extension into the medulla (syringobulbia), symptoms may include dysphagia, nystagmus, pharyngeal and palatal weakness, tongue atrophy, and sensory loss in the trigeminal nerve distribution.
- Lumbar syringomyelia can cause leg muscle atrophy with dissociated sensory loss in lumbosacral dermatomes, with decreased or absent reflexes.
Specific secondary or associated conditions and complications
- Scoliosis may be seen in 25% to 85% of pediatric cases of syringomyelia associated with CM-I.24 The presence of syrinx has been found to be independently associated with scoliosis in this subset of patients suggesting that low tonsillar position is not itself a risk factor for development of scoliosis.8 Left thoracic curve, young age, severity, and rapid progression may raise suspicion for intraspinal anomaly in a patient presenting with scoliosis.11
- Painless ulcers of the hands (aphthous ulcers) caused by decreased peripheral sensation.
- Neurogenic arthropathies of shoulder, elbow, and wrist (Charcot joints); painful enlargement of the shoulder because of humeral destruction. The most common cause of upper extremity Charcot joint is syringomyelia.
- Pain complaints are more common in adults than younger children.
2. ESSENTIALS OF ASSESSMENT
Symptoms range from asymptomatic to severe disability and are variable depending on location of the syrinx within the spinal cord. Onset of symptoms and progression over time are important to determine as symptoms often progress slowly.
Key components of history:
- Ask about pain and stiffness in the back, shoulders and extremities.
- Symptoms are often aggravated by postural changes or Valsalva maneuver.
- Inquire about weakness, gait changes or clumsiness.
- Sensory symptoms:
- Classically described as “cape-like” loss of pain and temperature sensation in the back and arms, although this is rare in clinical practice.1
- May complain of loss of ability to feel temperature extremes of hot/cold in the hands.
- Sensory deficits may be asymmetric and not always follow a dermatomal distribution.11
- Autonomic symptoms:
- Dry skin or hyperhidrosis may be seen.11
- Inquire about bowel and bladder function.
- Brainstem symptoms (syringobulbia):
- Inquire about facial numbness, dysarthria, dysphagia, vision, hearing, and respiratory function.11
- Associated symptoms:
- Scoliosis may be the only presenting sign of newly developing syringomyelia.
- Ask about upper or lower extremity joint pain or swelling.
- Weight loss, anorexia, fatigue, history of cancer and back pain exacerbated by the supine position may be indicative of tumor.
- Previous or family history of brain or spinal cord malformations may increase suspicion of syringomyelia.
Factors related to specific patient populations:
- History of spinal cord injury or myelomeningocele:
- It is critical to establish a baseline level of neurological function.
- New neurologic deficits in the lower extremities, pain, bowel or bladder changes, and scoliosis may suggest development of syringomyelia. If the syrinx extends upward to the cervical spinal cord, patients can present with new hand weakness and sensory symptoms.1
- Tethered cord versus syringomyelia
- New bowel/bladder dysfunction is more frequently associated with tethered cord than syringomyelia.
- Upper extremity symptoms are more likely due to syringomyelia.
- Tethered cord pain is often unilateral lower extremity pain exacerbated by forward spinal flexion.
- Syringomyelia pain is more often bilateral and widespread.11
Detailed neurologic examination is required.
- Cranial Nerves: Evaluate for any cranial nerve dysfunction.
- Motor: Look for any weakness or asymmetries.
- Sensory: Determine if there is any loss of pain, temperature, light touch, or proprioception.
- Tone: Evaluate for any spasticity or increased tone.
- Reflexes: Evaluate for hyporeflexia, hyperreflexia, clonus, Hoffman reflex, Babinski reflex.
- Cerebellar: Look for any dysfunction in coordination or ataxia.
- Gait/Balance: Assess fluidity of gait and balance.
- Autonomic findings: Evaluate for any evidence of bowel/bladder dysfunction, blood pressure fluctuations, hyperhidrosis or dry skin, or Horner’s syndrome.
- Musculoskeletal: Determine if there is any evidence of atrophy, scoliosis, or Charcot joints.
- Skin: Assess for any aphthous ulcers.
Findings are dependent on spinal cord level:
- Cranial nerve dysfunction and cerebellar signs
- Upper extremity hyporeflexia
- Lower extremity hyperreflexia, spasticity with paraparesis, and Babinski reflex
- Horner’s syndrome or other evidence of dysautonomia
- Lower extremity hyporeflexia and paresis
The following should be documented because any changes may indicate progression of syrinx:
- Spasticity (using the Ashworth Scale)
- Deep tendon reflexes
- Sensory level
Patients must be evaluated for development of the following:
- Gross motor skills
- Bed mobility
- Ambulation with/without assistive devices
- Static/dynamic balance
- Active/passive range of motion
- Fine motor skills
- Eye-hand coordination
- Activities of daily living status
- Speech/language and other oral skills
- Indicators of dysphagia (cough, hypoxia, or dysphonia may require additional studies)
- Nutritional status
- Cognitive skills
- Executive functioning, at school and home
- Short- and long-term memory
- Safety awareness, judgment
Contrast-enhanced spinal MRI is the gold standard for detection of syringomyelia. Contrast-enhanced images help to differentiate between tumor and inflammatory causes. Three-dimensional constructive interference in steady state (CISS) sequences may be helpful for determining anatomical details of syringes with multiple septa and for detecting extramedullary arachnoid adhesions or membranes.1,11 Four-dimensional phase contrast MRI is a new technique that can be used to study CSF flow dynamics.1
Additional imaging studies:
- Computed tomography (CT) myelogram can be considered if MRI is unavailable or unsafe.
- Spine radiographs can evaluate for coexisting scoliosis.
- MRI of the head and craniocervical junction can be considered to evaluate for hydrocephalus and Chiari malformation.1
- MRI of the lumbosacral spine should be included if there is concern for tethered cord1.
Supplemental assessment tools
- Swallowing videofluoroscopy, fiberoptic endoscopic evaluation of swallowing, or other studies for dysphagia; laryngoscopy for dysphonia and vocal cord dysfunction.25
- Electrodiagnosis for decreased hypothenar muscle action potentials or abnormal tibial nerve somatosensory evoked potentials related to impaired proprioception in the lower extremities.26
- Urodynamic studies can be considered pre and post spinal cord untethering to evaluate for retethering or progression of syringomyelia.11
Early predictions of outcomes
Morbidity/mortality and assessment of treatment results are difficult because of rarity, variable presentation, and limited follow-up with small numbers in most studies. They may be lower in modern studies, given neurosurgical intervention, better imaging techniques, and treatment of complications.
CM-I: Successful surgery for CM-I depends on early detection, which is facilitated by MRI. Studies show that a shorter duration of symptoms before decompression favorably influences clinical outcome.27 Syrinx shape also influences pain and symptoms. Recovery is slower in patients with deviated or dorsal horn involvement compared to enlarged or central syrinx.28
Idiopathic syringomyelia: Children with idiopathic syrinx remain asymptomatic, stable, or improved in 91% of cases without surgical intervention.22 Orthopedic and neurologic studies reviewing pain, neurologic symptoms, and rate of scoliosis progression found no correlation between location or size of syrinx and magnitude of major curve or severity of neurologic deficit.24
Myelomeningocele: Patients with myelomeningocele who are asymptomatic with syringomyelia identified on imaging are likely to remain asymptomatic.12 Only a small proportion require surgical treatment. In patients with hydrocephalus, shunt obstruction can lead to deterioration in syringomyelia.11
Trauma-associated: During the acute phase of spinal cord injury, restoration of spinal alignment with surgery and instrumentation, when appropriate, may decrease the risk of development of post-traumatic syringomyelia and may improve the chance of successful treatment.14,23 The extent of intradural scarring and arachnoiditis leads to more difficult surgical intervention and may result in recurrence or worsening of syringomyelia and need for surgical revision.23 This may be especially problematic in patients with motor incomplete injuries (ASIA C or D) who have been shown to have higher rates of neurological deterioration after 5 years postoperatively (39%) compared to 14% in ASIA A or B and 6% in ASIA E.14 This may be due to the intraoperative difficulty in performing complete arachnolysis and untethering while trying to preserve as much residual cord function as possible in patients with partially preserved motor function at baseline.14
Tumor-associated: Tumors with associated syringomeylia tend to be noninfiltrative with distinct cleavage planes and patients may recover from surgery more rapidly than those with diffuse infiltrative tumors. After tumor removal, the syrinx will typically resolve.15
Inquire about home environment particularly regarding stairs, width of corridors/doors, and accessibility for durable medical equipment if there is paralysis or gait dysfunction. Barrier-free school environment should also be assessed.
Social role and social support system
Providers need comprehensive understanding of the living situation, including the number of primary caretakers and their roles in daily management of the patient’s functional and medical needs. Direct questions must be asked of the caretaker to ensure they can meet the patient’s needs (eg, who will provide bladder, bowel, and skin care management).
3. REHABILITATION MANAGEMENT AND TREATMENTS
Available or current treatment guidelines
No current treatment guidelines are available.
At different disease stages
Asymptomatic patients require expectant management with monitoring of MRI and clinical symptoms.
With Chiari malformation and neurologic symptoms, expanding syrinx, or progressive scoliosis, treatment is neurosurgical. Patients who begin to decline in function at any stage require emergent neurosurgical referral.
When surgery is indicated, the goal is restoration of normal CSF flow dynamics with different techniques dependent on etiology:1
CM-I: Craniocervical decompression with or without augmentation duraplasty and an outlet made for the fourth ventricle if necessary (fourth ventricular-spinal or vetnriculopleural catheter). Recent studies have found that posterior fossa decompression without duraplasty is associated with similar outcomes and a lower risk of complications compared to posterior fossa decompression with duraplasty.29,30
Chiari II malformation: Decompression of the foramen magnum and upper cervical laminectomy until the level of the normal spinal cord and possible fourth ventricular spinal catheter.
Myelomeningocele: Exclude shunt malfunction. Consider surgery for cord untethering prior to specific treatment of syringomyelia.
Arachnoid scarring (due to infection, inflammation or trauma): Adhesiolysis of fibrotic bands if localized. If widespread, direct drainage of syrinx with shunt (syringoarachnoid, syringoperitoneal, or syringopleural).1 Duraplasty and arachnolysis is the treatment of choice for post-traumatic syringomyelia as shunting procedures alone are associated with less desirable outcomes and require more revisions.23
Tumor-associated: Surgical tumor resection. May require radiation therapy if complete removal is impossible.
Postoperative complications include the following:1,21
- General surgical complications: anesthetic complications, infection, postoperative hematoma, deep venous thrombosis, pneumonia, urinary tract infection
- Wound leakage of CSF
- Aseptic meningitis
- Neurological deficit
- Scar formation around spinal cord or shunt
- Shunt obstruction
Pain management is multi-modal:
- Medications: Analgesics, nonsteroidal anti-inflammatories, muscle relaxants, anticonvulsants, antidepressants
- Topical agents
- Behavioral pain coping strategies
- Alternative and complementary medicine (acupuncture, massage, etc.)
Rehabilitation assessment and intervention include the following:
- Physical therapy
- Occupational therapy
- Speech and language therapy (if deficits in brainstem function)
Short- and long-term goals include the following:
- Injury prevention
- Contracture prevention
- Strengthening and conditioning
- Balance: static/dynamic
- Fine motor skills
- Activities of daily living with/without assistive devices
- Swallowing, phonation, and respiration
- Verbal and written communication
- Family education/training
Coordination of care
Care of this complex condition requires coordination with the following:
- Primary physician
- Orthopedic surgeon
- Social worker/case manager
- Third-party payers
Parents require a supportive team approach and development of a support network including the following:
- Social workers
- Respite care
Parents must learn how to advocate effectively for their child while promoting independence and must understand all issues involved in their child’s diagnosis and be involved in all treatment decisions. When able, the child should be involved in their own personal care.
Early developmental and neuropsychologic assessment and periodic re-evaluation are needed to ascertain ability to make decisions regarding care. A legal guardian should be appointed if capacity as a legal adult is lacking.
Patient & family education
Families must be aware of symptoms to monitor and bring to medical attention, which could indicate progression and the need for intervention. Education and training in skin protection and bowel/bladder care are essential. If primary caretakers are unable to provide adequate support, referral to social services is required.
External resources include the following:
- Chiari and Syringomyelia Foundation (http://csfinfo.org/)
- American Syringomyelia & Chiari Alliance Project (http://asap.org/)
There are no specific syringomyelia outcome measures. Functional scales (eg, WeeFIM) that address mobility, self-care, and cognition can be used for children ages 3 to 8 years. There is presently no valid, specific, reliable outcome scale for syringomyelia.
Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills
Syringomyelia is a life-long disease requiring complex management over the life span. Vigilant monitoring of patients with a history of Chiari malformation, spinal cord injury, or myelomeningocele is necessary. Declines in neurologic function mandate evaluation for the presence of syrinx or spinal instability.
4. CUTTING EDGE/EMERGING AND UNIQUE CONCEPTS AND PRACTICE
Cutting edge concepts and practice
Dynamic 4D phase contrast-enhanced MRI to analyze CSF flow dynamics within the syrinx.
5. GAPS IN THE EVIDENCE-BASED KNOWLEDGE
Gaps in the evidence-based knowledge
A survey of the pediatric section of the American Association of Neurological Surgeons favors surgical intervention for cranial nerve dysfunction, motor/sensory loss, and scoliosis associated with syringomyelia.31 Treatment of asymptomatic patients with CM-I and syringomyelia with conservative management versus early surgical intervention is unclear.32 Although posterior fossa decompression is the most common treatment modality for children with CM-I and syringomyelia, optimal surgical technique has not been determined.32 Consideration may also be given to the timing of neurosurgical intervention before scoliosis surgery.
Current research in syringomyelia (www.clinicaltrials.gov):
- Genetics of Chiari Type I malformation
- Refinements of surgical techniques: surgery to open spinal subarachnoid space versus syrinx shunt, posterior fossa decompression with or without duraplasty in patients with CM-I and syringomyelia.
- Establishing the physiology of syringomyelia: further investigations of cerebrospinal fluid dynamics through theoretic models and imaging techniques
- Prospective studies on the natural history of syringomyelia
Determining efficacy of stem cell therapy in patients with post-traumatic syringomyelia
- Vandertop WP. Syringomyelia. Neuropediatrics. 2014;45(1):3-9.
- Roser F, Ebner FH, Sixt C, Hagen JM, Tatagiba MS. Defining the line between hydromyelia and syringomyelia. A differentiation is possible based on electrophysiological and magnetic resonance imaging studies. Acta Neurochir (Wien). 2010;152(2):213-9; discussion 219.
- Blegvad C, Grotenhuis JA, Juhler M. Syringomyelia: A practical, clinical concept for classification. Acta Neurochir (Wien). 2014;156(11):2127-2138.
- Sakushima K, Tsuboi S, Yabe I, et al. Nationwide survey on the epidemiology of syringomyelia in japan. J Neurol Sci. 2012;313(1-2):147-152.
- Brickell KL, Anderson NE, Charleston AJ, Hope JK, Bok AP, Barber PA. Ethnic differences in syringomyelia in new zealand. J Neurol Neurosurg Psychiatry. 2006;77(8):989-991.
- Strahle J, Muraszko KM, Garton HJ, et al. Syrinx location and size according to etiology: Identification of chiari-associated syrinx. J Neurosurg Pediatr. 2015;16(1):21-29.
- Syringomyelia. https://rarediseases.org/rare-diseases/syringomyelia/. Updated 2017. Accessed May 31, 2018.
- Strahle J, Smith BW, Martinez M, et al. The association between chiari malformation type I, spinal syrinx, and scoliosis. J Neurosurg Pediatr. 2015;15(6):607-611.
- Aitken LA, Lindan CE, Sidney S, et al. Chiari type I malformation in a pediatric population. Pediatr Neurol. 2009;40(6):449-454.
- Kahn EN, Muraszko KM, Maher CO. Prevalence of chiari I malformation and syringomyelia. Neurosurg Clin N Am. 2015;26(4):501-507.
- Tsitouras V, Sgouros S. Syringomyelia and tethered cord in children. Childs Nerv Syst. 2013;29(9):1625-1634.
- Piatt JH,Jr. Syringomyelia complicating myelomeningocele: Review of the evidence. J Neurosurg. 2004;100(2 Suppl Pediatrics):101-109.
- Krebs J, Koch HG, Hartmann K, Frotzler A. The characteristics of posttraumatic syringomyelia. Spinal Cord. 2016;54(6):463-466.
- Klekamp J. Treatment of posttraumatic syringomyelia. J Neurosurg Spine. 2012;17(3):199-211.
- Samartzis D, Gillis CC, Shih P, O’Toole JE, Fessler RG. Intramedullary spinal cord tumors: Part I-epidemiology, pathophysiology, and diagnosis. Global Spine J. 2015;5(5):425-435.
- Koc K, Anik I, Anik Y, Ceylan S. Familial syringomyelia in two siblings: Case report. Turk Neurosurg. 2007;17(4):251-254.
- Zakeri A, Glasauer FE, Egnatchik JG. Familial syringomyelia: Case report and review of the literature. Surg Neurol. 1995;44(1):48-53.
- Levine DN. The pathogenesis of syringomyelia associated with lesions at the foramen magnum: A critical review of existing theories and proposal of a new hypothesis. J Neurol Sci. 2004;220(1-2):3-21.
- Heiss JD, Snyder K, Peterson MM, et al. Pathophysiology of primary spinal syringomyelia. J Neurosurg Spine. 2012;17(5):367-380.
- Koyanagi I, Houkin K. Pathogenesis of syringomyelia associated with chiari type 1 malformation: Review of evidences and proposal of a new hypothesis. Neurosurg Rev. 2010;33(3):271-84; discussion 284-5.
- Arnautovic A, Splavski B, Boop FA, Arnautovic KI. Pediatric and adult chiari malformation type I surgical series 1965-2013: A review of demographics, operative treatment, and outcomes. J Neurosurg Pediatr. 2015;15(2):161-177.
- Magge SN, Smyth MD, Governale LS, et al. Idiopathic syrinx in the pediatric population: A combined center experience. J Neurosurg Pediatr. 2011;7(1):30-36.
- Karam Y, Hitchon PW, Mhanna NE, He W, Noeller J. Post-traumatic syringomyelia: Outcome predictors. Clin Neurol Neurosurg. 2014;124:44-50.
- Yeom JS, Lee CK, Park KW, et al. Scoliosis associated with syringomyelia: Analysis of MRI and curve progression. Eur Spine J. 2007;16(10):1629-1635.
- Tubbs RS, Bailey M, Barrow WC, Loukas M, Shoja MM, Oakes WJ. Morphometric analysis of the craniocervical juncture in children with chiari I malformation and concomitant syringobulbia. Childs Nerv Syst. 2009;25(6):689-692.
- Veilleux M, Stevens JC. Syringomyelia: Electrophysiologic aspects. Muscle Nerve. 1987;10(5):449-458.
- Dyste GN, Menezes AH, VanGilder JC. Symptomatic chiari malformations. an analysis of presentation, management, and long-term outcome. J Neurosurg. 1989;71(2):159-168.
- Nakamura M, Chiba K, Nishizawa T, Maruiwa H, Matsumoto M, Toyama Y. Retrospective study of surgery-related outcomes in patients with syringomyelia associated with chiari I malformation: Clinical significance of changes in the size and localization of syrinx on pain relief. J Neurosurg. 2004;100(3 Suppl Spine):241-244.
- Chotai S, Medhkour A. Surgical outcomes after posterior fossa decompression with and without duraplasty in chiari malformation-I. Clin Neurol Neurosurg. 2014;125:182-188.
- Jiang E, Sha S, Yuan X, et al. Comparison of clinical and radiographic outcomes for posterior fossa decompression with and without duraplasty for treatment of pediatric chiari I malformation: A prospective study. World Neurosurg. 2018;110:e465-e472.
- Haroun RI, Guarnieri M, Meadow JJ, Kraut M, Carson BS. Current opinions for the treatment of syringomyelia and chiari malformations: Survey of the pediatric section of the american association of neurological surgeons. Pediatr Neurosurg. 2000;33(6):311-317.
- Rocque BG, George TM, Kestle J, Iskandar BJ. Treatment practices for chiari malformation type I with syringomyelia: Results of a survey of the american society of pediatric neurosurgeons. J Neurosurg Pediatr. 2011;8(5):430-437.
Original Version of the Topic
Meg A. Krilov, MD, Jennifer Gomez, MD. Pediatric Syringomyelia. Original Publication Date: 9/20/2018
Amanda Kole Morrow, MD
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Frank Pidcock, MD
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