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PubMed2001

Surgical treatment of post-traumatic myelopathy associated with syringomyelia.

STUDY DESIGN: Retrospective review. OBJECTIVE: Evaluate the clinical outcome of surgical intervention for post-traumatic syringomyelia. INTRODUCTION: Progressive post-traumatic cystic myelopathy (PPCM), or syringomyelia, can occur after spinal cord injury. The authors present their surgical treatment protocol and treatment outcome of a series of patients with post-traumatic syringomyelia. METHODS: The medical records of 53 patients with PPCM undergoing surgical treatment were reviewed. Laminectomies and intraoperative ultrasonography were performed. For patients with no focal tethering and only a confluent cyst on ultrasonography, a syringosubarachnoid shunt (stent) was inserted. For patients with both tethering and a confluent cord cyst, an untethering procedure was performed first. When a cyst showed significant size reduction (>50%) after untethering, no shunt was placed. When the cyst size persisted on ultrasonographic images, a short syringosubarachnoid shunt was used. The mean follow-up was 23.9 months for the 45 patients available for follow-up (range 12-102 months). RESULTS: The interval between the causative event and the operation was from 5 months to 37 years (mean 6.5 years). Pain was the most frequent manifestation, followed by motor deterioration and spasticity. Postoperative improvements in >50% of the patients were noted in those presenting with worsening motor function or spasticity. In 19 of 28 patients with associated tethered spinal cord, untethering alone caused significant collapse of the cyst. Postoperative MRI demonstrated cyst collapse in 95% of the patients with untethering alone and 93% of the patients with a syringosubarachnoid shunt. CONCLUSION: Post-traumatic syringomyelia can occur with or without cord tethering. Untethering alone for patients with cord tethering and cyst formation can reduce cyst size and alleviate the symptoms and signs of syringomyelia in the majority of these cases. Untethering with expansion of subarachnoid space with an expansile duraplasty may be a more physiologic way of treating a tethered cord with associated syringomyelia, i.e., treating the cause rather than the result.

Adult↗
PubMed2002

Adult scoliosis in syringomyelia associated with Chiari I malformation.

STUDY DESIGN: In adult syringomyelia associated with Chiari I malformation, the spinal deformity, the configuration of cerebellar tonsillar descent, the configuration of syrinx, and the clinical evaluation before and after surgery were investigated. OBJECTIVES: To investigate the characteristics of the scoliosis in syringomyelia associated with Chiari I malformation. SUMMARY OF BACKGROUND DATA: In previous studies, the clinical characteristics of pediatric scoliosis associated with syringomyelia have been reported. METHODS: In this study, 42 patients with syringomyelia were treated. All the patients were 20 years of age or older. They were divided into three groups: Group 1 comprising those without scoliosis, Group 2 composed of those with scoliosis of 10 degrees or more but less than 20 degrees, and Group 3 consisting of those with scoliosis of 20 degrees or more. Investigations conducted with the three groups included determining the curve patterns of scoliosis, the degree of thoracic kyphosis, the configuration of cerebellar tonsillar descent, the configuration of syrinx, the morbidity period, and the clinical evaluation before and after surgery. RESULTS: There were 12 patients in Group 1, 21 patients in Group 2, and 9 patients in Group 3. The concomitant rate of adult syringomyelia with scoliosis was 71.4%. As scoliosis advanced, the kyphotic angle also increased. The concordance in laterality between the cerebellar tonsil and curve convex was 70%. Findings showed that the more advanced the scoliosis was, the more aggravated the neurologic symptoms were, and the poorer the surgical outcomes tended to be. CONCLUSIONS: In adult syringomyelia with scoliosis, the morbidity period is long, the syrinx is long, the neurologic symptoms are aggravated, and the surgical outcomes tend to be poor.

Adult↗
PubMed1999

Focal (segmental) dyshidrosis in syringomyelia.

The features or mechanisms of dyshidrosis have not been sufficiently clarified. Neither has the difference between hyperhidrosis and hypohidrosis. To clarify the features and mechanisms of dyshidrosis (hyperhidrosis and hypohidrosis) in syringomyelia, the clinical features focusing on hidrosis of 30 patients with syringomyelia and Chiari malformation located from a syringomyelia database were prospectively analysed. The patients were classified into three groups: eight patients (26.7%) had segmental hypohidrosis, 10 (33. 3%) had segmental hyperhidrosis, and 12 (40.0%) had normohidrosis. We found that the Karnofsky functional status for the hyperhydrosis and normohidrosis groups were significantly higher than for the hypohidrosis group (p=0.0012), with no significant differences between the hyperhidrosis and normohidrosis groups. The duration from the onset of syringomyelia to the current dyshidrosis was significantly longer in the hypohidrosis group than in the hyperhidrosis group (p=0.0027). A significant correlation was identified between the duration from the onset of syringomyelia to the time at study and the performance score (r=-0.599, p=0.0003). The results substantiate previous hypotheses that in its early stage syringomyelia causes segmental hyperactivity of the sympathetic preganglionic neurons, and hyperactivity of these gradually subsides as tissue damage progresses. Focal hyperhidrosis may be regarded as a hallmark of a relatively intact spinal cord, as well as normohidrosis.

Adolescent↗
PubMed2000

Current opinions for the treatment of syringomyelia and chiari malformations: survey of the Pediatric Section of the American Association of Neurological Surgeons.

INTRODUCTION: A variety of surgical interventions have been recommended for patients with syringomyelia and Chiari malformation. To obtain a better understanding of currently used management strategies and surgical interventions, this study evaluates current opinions in the treatment of these entities. METHODS: Members of the Pediatric Section of the American Association of Neurological Surgeons (AANS) were surveyed in July of 1998. A questionnaire was designed to assess (1) management of asymptomatic and symptomatic cases; (2) signs and symptoms that warrant surgical intervention, and (3) surgical strategies. Seventy-seven of 234 surveys (33%) were returned. Responses were entered in a Microsoft Excel spreadsheet for data analysis. RESULTS: Each year, respondents evaluated on average 10 patients with confirmed syringomyelia and operated on 7. There was substantial agreement to operate on patients with syringomyelia who had progressive scoliosis or progressive motor/sensory loss. For isolated syringomyelia without a Chiari malformation, the majority of respondents recommended shunting of the syrinx. Opinion was mixed concerning physical restrictions for asymptomatic patients with syringomyelia and Chiari malformations. For Chiari malformations, the majority of respondents recommended a minimum cervicomedullary decompression with dural patch grafting. No consensus was obtained on whether dural patch grafting should be done alone or in combination with intradural dissection or manipulation of the tonsils. CONCLUSION: Although many respondents agreed on the management and surgical treatment of symptomatic patients with syringomyelia and/or Chiari malformations, there was disagreement with regard to the optimal surgical procedure. Respondents rejected prophylactic surgery for their asymptomatic patients, choosing to follow them conservatively with serial imaging and neurological examinations.

Arnold-Chiari Malformation↗
PubMed2004

Syringomyelia in twin brothers discordant for Chiari I malformation: case report.

Familial syringomyelia outside of trauma, tumor, or infection has been reported. Cases are presented that highlight the possible connection between familial syringomyelia and the Chiari 0 malformation. We report on 11-year-old twin brothers both with syringomyelia. Magnetic resonance imaging further revealed that one brother had Chiari I malformation and the other had Chiari 0 malformation. Both underwent posterior fossa decompression with radiologic improvement of their syringes. These case reports lend credence to earlier reports of improvement in syringomyelia following posterior fossa decompression in the absence of Chiari I malformation, the so-called Chiari 0 malformation. In addition, these case reports should influence the manner in which familial syringomyelia without tonsillar ectopia is defined and addressed, that is, posterior fossa decompression versus shunting of the syrinx, thereby addressing the potential cause of the syringomyelia and not only the enlarged cavity itself.

Arnold-Chiari Malformation↗
PubMed2001

Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 cases.

OBJECT: The authors describe the results of performing a standard posterior craniovertebral decompression and placement, if indicated, of a syringosubarachnoid shunt for the treatment of patients with Chiari I malformation with and without syringomyelia. METHODS: This is a retrospectively analyzed consecutive series of 66 patients (mean patient age 15 years, range 1-53 years). The uniform posterior craniovertebral decompression consisted of a small suboccipital craniectomy, a C-1 laminectomy, microsurgical reduction of the cerebellar tonsils, and dural closure with a synthetic dural graft to increase the cerebrospinal fluid space at the craniocervical junction. The presence of a large syrinx, with significant thinning of the spinal cord tissue and obliteration of the spinal subarachnoid space, particularly when combined with syrinx-related symptoms, was an indication for the placement of a syringosubarachnoid shunt. In 32 patients Chiari I malformation alone was present, and 34 in patients it was present in combination with syringomyelia. Clinical findings included pain, neurological deficits, and spinal deformity. The presence of syringomyelia was significantly associated with the presence of scoliosis (odds ratio 74.4 [95% confidence interval 8.894-622.4]). All patients underwent a posterior craniovertebral decompression procedure. In 22 of the 34 patients with syringomyelia a syringosubarachnoid shunt was also placed. The mean follow-up period was 24 months (range 3-95 months). Excellent outcome was achieved in 54 patients (82%) and good outcome in 12 (18%). In no patient were symptoms unchanged or worse at follow-up examination, including four patients who initially required a second operation for persistent syringomyelia. Pain was more likely to resolve than sensory and motor deficits after decompressive surgery. Radiological examination revealed normalization of tonsillar position in all patients. The syrinx had disappeared in 15 cases, was decreased in size in 17, and remained unchanged in two. CONCLUSIONS: Posterior craniovertebral decompression and selective placement of a syringosubarachnoid shunt in patients with Chiari I malformation and syringomyelia is an effective and safe treatment. Primary placement of a shunt in the presence of a sufficiently large syrinx appears to be beneficial. The question of if and when to place a shunt, however, requires further, preferably prospective, investigation.

Adolescent↗
PubMed1999

Elucidating the pathophysiology of syringomyelia.

OBJECT: Syringomyelia causes progressive myelopathy. Most patients with syringomyelia have a Chiari I malformation of the cerebellar tonsils. Determination of the pathophysiological mechanisms underlying the progression of syringomyelia associated with the Chiari I malformation should improve strategies to halt progression of myelopathy. METHODS: The authors prospectively studied 20 adult patients with both Chiari I malformation and symptomatic syringomyelia. Testing before surgery included the following: clinical examination; evaluation of anatomy by using T1-weighted magnetic resonance (MR) imaging; evaluation of the syrinx and cerebrospinal fluid (CSF) velocity and flow by using phase-contrast cine MR imaging; and evaluation of lumbar and cervical subarachnoid pressure at rest, during the Valsalva maneuver, during jugular compression, and following removal of CSF (CSF compliance measurement). During surgery, cardiac-gated ultrasonography and pressure measurements were obtained from the intracranial, cervical subarachnoid, and lumbar intrathecal spaces and syrinx. Six months after surgery, clinical examinations, MR imaging studies, and CSF pressure recordings were repeated. Clinical examinations and MR imaging studies were repeated annually. For comparison, 18 healthy volunteers underwent T1-weighted MR imaging, cine MR imaging, and cervical and lumbar subarachnoid pressure testing. Compared with healthy volunteers, before surgery, the patients had decreased anteroposterior diameters of the ventral and dorsal CSF spaces at the foramen magnum. In patients, CSF velocity at the foramen magnum was increased, but CSF flow was reduced. Transmission of intracranial pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was partially obstructed. Spinal CSF compliance was reduced, whereas cervical subarachnoid pressure and pulse pressure were increased. Syrinx fluid flowed inferiorly during systole and superiorly during diastole on cine MR imaging. At surgery, the cerebellar tonsils abruptly descended during systole and ascended during diastole, and the upper pole of the syrinx contracted in a manner synchronous with tonsillar descent and with the peak systolic cervical subarachnoid pressure wave. Following surgery, the diameter of the CSF passages at the foramen magnum increased compared with preoperative values, and the maximum flow rate of CSF across the foramen magnum during systole increased. Transmission of pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was normal and cervical subarachnoid mean pressure and pulse pressure decreased to normal. The maximum syrinx diameter decreased on MR imaging in all patients. Cine MR imaging documented reduced velocity and flow of the syrinx fluid. Clinical symptoms and signs improved or remained stable in all patients, and the tonsils resumed a normal shape. CONCLUSIONS: The progression of syringomyelia associated with Chiari I malformation is produced by the action of the cerebellar tonsils, which partially occlude the subarachnoid space at the foramen magnum and act as a piston on the partially enclosed spinal subarachnoid space. This creates enlarged cervical subarachnoid pressure waves that compress the spinal cord from without, not from within, and propagate syrinx fluid caudally with each heartbeat, which leads to syrinx progression. The disappearance of the abnormal shape and position of the tonsils after simple decompressive extraarachnoidal surgery suggests that the Chiari I malformation of the cerebellar tonsils is acquired, not congenital. Surgery limited to suboccipital craniectomy, C-I laminectomy, and duraplasty eliminates this mechanism and eliminates syringomyelia and its progression without the risk of more invasive procedures.

Adolescent↗
PubMed2002

A novel fibroblast growth factor receptor 2 mutation in Crouzon syndrome associated with Chiari type I malformation and syringomyelia.

OBJECT: It has been reported that due to premature synostosis of the lambdoid suture in the first 24 months of life, more than 70% of patients with Crouzon syndrome concurrently suffer from chronic tonsillar herniation (Chiari Type I malformation) and some (20%) associated syringomyelia. The goal of the present study was to examine mutations in the fibroblast growth factor receptor (FGFR) genes in Crouzon syndrome and its related conditions. METHODS: Five patients were studied: three with Crouzon syndrome (one sporadic and two familial), one with sporadic Chiari I with syringomyelia, and one with unilateral lambdoid synostosis. Deoxyribonucleic acid was screened for FGFR1-3 mutations by using single-strand conformational polymorphism and subsequent direct sequencing. Two types of missense mutations were detected in the FGFR2 gene, Cys342Trp (1205, TGC --> TGG) in a patient with sporadic Crouzon syndrome and Tyr281Cys (1021, TAC --> TGC) in two siblings (brother and sister) with familial Crouzon syndrome, respectively. The former has been reported only in sporadic cases but the latter has not previously been identified. A polymorphism in the FGFR3 gene, Asn294Asn (882, AAT --> AAC), was also detected in three patients. No mutation was found in the patient with sporadic Chiari I with syringomyelia. CONCLUSIONS: The FGFR2 missense mutation was detected in Crouzon syndrome but not in sporadic Chiari I with syringomyelia or lambdoid synostosis. A novel FGFR2 mutation, Tyr281Cys, was found in familial Crouzon syndrome with Chiari I and syringomyelia. It may be informative to look for this in patients with Crouzon syndrome and associated syringomyelia.

Adolescent↗
PubMed2005

Spontaneous regression of syringomyelia in Hajdu-Cheney syndrome with severe platybasia. Case report.

Hadju-Cheney syndrome (HCS) is a rare autosomal-dominant disorder with variable expressivity. It is characterized by facial dysmorphism, premature tooth loss, osteolysis of distal phalanges, and skull abnormalities. In some cases, progressive platybasia can occur and can lead to Chiari malformation with an obstruction of cerebrospinal fluid flow. To the best of the authors' knowledge, only five cases of HCS-associated syringomyelia have been reported in the literature. Because of the rarity of this association, little is known about its natural history. The authors present the case of a 16-year-old boy affected by HCS. On initial magnetic resonance (MR) imaging, a severe basilar invagination with Chiari malformation and cervicothoracic syringomyelia was documented. The syringomyelia had no clinical manifestations. A repeated MR image demonstrated a spontaneous resolution of the syrinx with no changes in the tonsil or the platybasia. The regression of the syringomyelia was confirmed by a control MR imaging examination performed after a 2-year period. No changes in the patient's clinical conditions were found during the follow-up period. This is the first case of spontaneous regression of the syringomyelia despite a severe platybasia in HCS. It did not appear correlated to a modification of the tonsil's structure or position. This observation illustrates one possible evolution of syringomyelia in the natural history of HCS and raises the question of the potential mechanisms involved in the spontaneous drainage of the syringomyelic cavity.

Adolescent↗
PubMed2006

Cordectomy for the treatment of posttraumatic syringomyelia. Report of four cases and review of the literature.

Cordectomy is an effective treatment option in patients in whom posttraumatic syringomyelia develops following complete spinal cord injuries. Since the introduction of cordectomy, numerous approaches to the surgical treatment of posttraumatic syringomyelia have been developed. These newer developments have drawn the attention of surgeons and researchers away from cordectomy. In this report, the authors encourage a reconsideration of cordectomy for the treatment of posttraumatic syringomyelia after complete spinal cord injury. They describe four patients with posttraumatic syringomyelia who were treated successfully with cordectomy and review appropriate literature, examining the effectiveness of cordectomy in the treatment of posttraumatic syringomyelia. The findings of this review indicate that neurological improvement or stabilization occurred in 88% of patients in published reports of posttraumatic syringomyelia treated with cordectomy. The indications for cordectomy as well as factors that may contribute to the procedure's success are discussed.

Adult↗
PubMed1999

[Evoked motor and sensory potentials in syringomyelia].

Seventy nine patients were admitted for syringomyelia. Fifty-nine had a malformative syringomyelia, 15 a post-traumatic syrinx and 5 a syringomyelia related to a meningitis. For all the patients, the diagnosis of syringomyelia was performed by MRI with measures of syrinx extension and transverse diameter. Posterior tibial somato sensory evoked potentials (PT SEP), median (M SEP), trigeminal (V3 SEP), brain stem auditory evoked potentials (BEAP), cortical and cervical motor evoked potentials (MEP) were correlated with clinical and radiological findings. SEP abnormalities were not correlated with the duration of symptoms. PT SEP proved to be more sensitive than M SEP, MEP abnormalities were very frequent (72% of the cases), even without clinical motor deficit. Trigeminal SEP were more sensitive than BAEP which were not related to the presence of associated cranio vertebral abnormalities. No significant relationship between clinical and radiological results was observed. There was a positive relationship between electrophysiological and radiological results. Nevertheless, a good correlation was observed between lateral clinical, MRI and electrophysiological results. Abnormal trigeminal SEP were detected in 46.6% of the patients with high cervical syringomyelia. In all cases, trigeminal SEP and MEP should be done in association with M and PT SEP as both of them detect subclinical evidence of spinal cord dysfunction in syringomyelia.

Adult↗
PubMed2003

Value of treating primary causes of syrinx in scoliosis associated with syringomyelia.

STUDY DESIGN: Retrospective chart and radiologic analysis. OBJECTIVES: To analyze the value and risks of neurosurgical procedures for syringomyelia and of fusion procedures for scoliosis. SUMMARY OF BACKGROUND DATA: There are few reports on the benefit of neurosurgical procedures on scoliosis, as well as the risk of fusion procedures on scoliosis related to syringomyelia. METHODS: The 105 patients who had both scoliosis and syringomyelia were subdivided into three groups: (I) 59 patients without congenital scoliosis or myelomeningocele, (II) 20 patients with congenital scoliosis and syringomyelia, and (III) 26 patients with myelomeningocele and syringomyelia. The study evaluated: (1) The effect of either suboccipital craniectomy or direct shunting on the syrinx, the curvature, and the neurologic status; (2) the neurologic risk of scoliosis fusion surgery; and (3) the curve status after fusion surgery. RESULTS: In Group I (patients without congenital scoliosis or myelomeningocele), suboccipital craniectomy resulted in curve improvement in seven, worsening in three, and no change in two, whereas direct syrinx shunting gave curve improvement in none, curve worsening in six, and no change in two. In Groups II and III, no patient had curve improvement after neurosurgery. Group III had the highest rate of subsequent neurosurgery (50%). The subsequent neurosurgery was nearly always suboccipital craniectomy or detethering of the cord. Three of 38 patients (8%) had neurologic worsening when scoliosis fusion surgery was done without previous syrinx decompression. CONCLUSIONS: In patients without myelomeningocele or congenital scoliosis, but with Arnold-Chiari malformation and syringomyelia, suboccipital craniectomy gave the best chance for syrinx reduction and scoliosis improvement, particularly in children younger than 10 years. Syrinx shunting improved none of the scolioses. For syrinxes in patients with congenital scoliosis or myelomeningocele, neither neurosurgical procedure resulted in curve improvement, as other causes of scoliosis (vertebra anomalies, paralysis) remained untreated. Patients with myelomeningocele require a multipronged surgical approach to address all causes of syrinx, thus minimizing the potential need for repeat neurosurgery. Scoliosis correction without prior syrinx decompression carries a high neurologic risk.

Adolescent↗
PubMed2003

Syringomyelia associated with hydrocephalus and Blake's pouch cyst: case report.

STUDY DESIGN: This is a case report of a 37-year-old woman who sought treatment for a large syringomyelic cavitation extending from C3 to the entire medulla, a tetraventricular hydrocephalus, and a cystic cavitation in the posterior cranial fossa communicating with the fourth ventricle (Blake's pouch cyst). The patient underwent a decompressive craniectomy, a C1 laminectomy, and the opening of the cysts to enable communication with the subarachnoid spaces. After an initial period of symptom remission, reassured by the magnetic resonance images indicating a reduction of the syringomyelia, the patient's neurologic conditions deteriorated because of further dilation of the ventricular cavities, which was resolved by the insertion of a ventriculoperitoneal shunt. OBJECTIVE: To suggest the treatment of choice in a patient with syringomyelia and hydrocephalus caused by Blake's pouch cyst. SUMMARY OF BACKGROUND DATA: Blake's pouch cyst is an entity often poorly understood, deriving from nonperforation of the primitive foramen of Magendie, causing a precarious equilibrium of the cerebrospinal fluid flow resulting from a defect in communication between the fourth ventricle and the encephalic and spinal subarachnoid spaces. Authors report the association of Blake's pouch cyst with cervicodorsal syringomyelia and tetraventricular hydrocephalus. METHODS: A case of syringomyelia associated with hydrocephalus and Blake's pouch cyst is described. RESULTS: Symptoms of syringomyelia and hydrocephalus disappeared only after positioning of a ventriculoperitoneal shunt. CONCLUSIONS: The treatment of choice for a case of syringomyelia associated with Blake's pouch cyst and hydrocephalus is the application of a ventriculoperitoneal shunt or, even better, an endoscopic third ventriculostomy.

Adult↗
PubMed2005

Surgical treatment of craniocervical decompression without Chiari malformation in syringomyelia.

We aimed to describe a patient without Chiari malformation who was treated via craniocervical decompression and by creating cisterna manga with an autologous fascia graft, and who displayed a clinical and radiological improvement in the post-operative period. Syringomyelia is a chronic and progressive disease with cavitation and gliosis in the spinal cord. It is more common in adulthood and often involves the cervical region. Due to the fact that craniocervical decompression therapy is successful in syringomyelia with Chiari malformation, this surgical treatment is currently controversial in syringomyelia without Chiari malformation. A 33-year-old male applied to our clinic with numbness and weakness in his left hand that had lasted for 2 years, but his condition had worsened in recent months, with neck pain before over previous the 8 months and a walking disturbance that had appeared 7 months before. Syringomyelia was found between the C2 and T8 vertebrae levels on magnetic resonance imaging (MRI) scans. The patient was diagnosed with syringomyelia and underwent suboccipital decompression. Cisterna magna was created by duraplasty with a fascia lata graft. Consequently, craniocervical decompression might be accepted as a treatment method of choice in patients with syringomyelia without Chiari malformation.

Adult↗
PubMed1991

[Magnetic resonance imaging of posttraumatic syringomyelia and its surgical treatment].

Posttraumatic syringomyelia is an uncommon but significant late complication of spinal cord injury. It occurs in approximately 1.1 - 3.2% of cases of spinal injuries. With the increasing availability of CT and magnetic resonance imaging (MRI), the incidence of posttraumatic syringomyelia is increasing. The purpose of this report is to show MRI of posttraumatic syringomyelia and to assess the results of surgical treatment. Materials and Methods This series included 16 cases of posttraumatic syringomyelia studied with MRI. 9 out of 16 cases showed delayed deterioration of neurological symptoms following spinal injuries. The interval between the trauma and the delayed symptoms of deterioration was from 2 years 2 months to 32 years (mean, 8 years and 5 months). There were 13 men and 3 women. The age ranged from 22 to 69 years, with a mean age of 42 years. The initial spinal cord injury was located in the lower cervical region in 4 cases, the thoracic region in 8, and the upper lumbar region in 4. All the patients were studied with resistive 0.15T system (Toshiba MRT 15 A) or a superconductive 0.5T system (Toshiba MRT 50 A) or a superconductive 1.5T system (GE Sigma or Siemens Magnetom). Six patients underwent 8 operative procedures for posttraumatic syringomyelia. Syringoperitoneal shunt was performed in 4 patients, syringosubarachnoid shunt in 3 and ventriculoperitoneal shunt in one. Results 1. MRI In all cases, the posttraumatic syringomyelia was easily diagnosed by MRI. The syrinx extended superiorly and/or inferiorly from the area of the old trauma. In 4 out of 16 cases, the syrinx extended into the medulla oblongata.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1993

[Syringomyelia secondary to adhesive arachnoiditis: clinical profiles and efficacy of shunt operations].

The authors report nine cases of syringomyelia secondary to adhesive arachnoiditis treated in our institute from 1982 to 1991. Neurological signs, radiological features, and results of surgical treatment were reviewed. Common initial manifestations in association with syringomyelia were spastic paraparesis in 8 patients (88.9%), regional sensory loss in 4 (44.4%), neurogenic bladder in 4 (44.4%), and somatic pain in 2 (22.2%). All the neurological symptoms or signs progressed gradually for years. All the patients were treated with various modes of shunt operations, including syringo-peritoneal shunt in seven patients, syringo-subarachnoid shunt in one, and ventriculo-peritoneal shunt in one, respectively. Three patients who failed to resolve their major complaints after the first surgery further received syringo-peritoneal shunts. Postsurgical follow-up periods ranged from 18 months to 10 years. Serial MR imaging revealed a significant reduction of the size of syringomyelia in 8 of 9 patients. However only 6 patients showed certain improvement of neurological deficits. Three patients who failed to improve clearly had a long (beyond 15 years) history of syringomyelia before the first surgical treatment. A total of 11 syringo-peritoneal shunts were done in 8 patients of whom 5 patients improved neurologically. It was noticed that 4 of 5 successful syringo-peritoneal shunts were placed in the caudal level of the syrinx. In conclusion, syringo-peritoneal shunt may be at present an optimal mode of surgical management for syringomyelia secondary to adhesive arachnoiditis. In addition, we would like to recommend that follow-up serial MR imaging be done for patients having adhesive arachnoiditis in order to detect syringomyelia as early as possible.

Arachnoiditis↗
PubMed2006

Scoliosis in a child with Chiari I malformation and the absence of syringomyelia: case report and a review of the literature.

BACKGROUND: Scoliosis is a common finding in children with Chiari I malformation and syringomyelia. The development of scoliosis associated with a Chiari malformation typically has been ascribed to the presence of syringomyelia. Decompression of the hindbrain hernia will often alleviate the syringomyelia and, in many cases, stabilize or reverse the scoliosis. CASE REPORT: We report a child with Chiari I malformation and scoliosis who presented with Valsalva-induced headache/neck pain. MRI revealed no syringomyelia or hydrocephalus. Radiographs revealed that her scoliotic curvature was approximately 13 degrees and was a single levoscoliotic curve. This patient underwent a posterior fossa decompression with duraplasty for her Valsalva-induced pain. Postoperatively, she had resolution of her pain and there has been no progression of her scoliosis at 3 years follow-up. PROGNOSIS: Intriguingly, and scattered throughout the medical literature, many have noted cases of scoliosis in patients with only a Chiari I malformation and no syringomyelia. Moreover, experimental studies have induced scoliosis in animals after compression of the dorsal columns. After a review of the medical literature regarding a potential cause and effect of herniated hindbrain-induced scoliosis in the absence of syringomyelia, this association although rare, does seem plausible.

Adolescent↗
PubMed1992

Scoliosis associated with syringomyelia presenting in children.

The clinical presentations and radiological features of scoliosis accompanying syringomyelia were analyzed in 14 cases of syringomyelia associated with a in 14 cases of syringomyelia associated with a Chiari malformation in children. Scoliosis was the initial symptom in 11 out of 16 patients (64%) with syringomyelia and present in 14 (88%) at the initial examination. The scoliosis associated with syringomyelia was characterized by a higher incidence of a single curve (6 cases, 43%) and convexity to the left (7 cases, 50%) than seen in idiopathic scoliosis. The syrinx was shifted to the convex side of scoliosis on the axial section at the middle or lower thoracic level in patients with a single curve, and at the cervical or upper thoracic level in patients with a double curve. The authors think that the scoliosis develops in children as a result of damage done to the anterior horn, which innervates the muscles of the trunk, by an asymmetrically expanded syrinx.

Arnold-Chiari Malformation↗