Foramen magnum decompression for the treatment of Arnold Chiari malformation type I with associated syringomyelia in an elderly patient

STUDY DESIGN: Report of a rare case of an elderly patient with late onset of Arnold Chiari malformation type I with associated syringomyelia that was successfully treated with foramen magnum decompression. OBJECTIVE: To report this rare case along with a literature review. SETTING: Gifu, Japan. METHODS: A 69-year-old woman with a 4-year history of dull pain in her right arm was referred to the clinic. After physical and radiographical examinations, she was diagnosed with Arnold Chiari malformation type I with associated syringomyelia. A foramen magnum decompression by the removal of the outer layer of the dura mater was performed. RESULTS: At 2 years postoperatively, MRI revealed a decrease in the size of the syringomyelia. Her symptoms had also remarkably improved. CONCLUSIONS: A rare case of symptomatic Arnold Chiari malformation type I with associated syringomyelia in an elderly woman was successfully treated with foramen magnum decompression by the removal of the outer layer of the dura mater.     

Hydrosyringomyelia and its management in childhood

Syringomyelia, once regarded as a degenerative disease of adults, is now recognized to be a disorder usually associated with the Chiari malformation and occurring in patients of all ages. We have reviewed 47 patients with syringomyelia treated on the Neurosurgical Service at the Hospital for Sick Children during the years 1977 to 1985. Twelve of these patients had a Chiari I malformation, 30 had a Chiari II malformation, and 5 had an acquired Chiari malformation. Thirty-one of these patients were treated by decompression of the Chiari malformation and plugging of the obex, 5 were treated by a simple posterior fossa decompression, 9 were treated by shunting of the syringomyelic cavity, and 2 were treated by a combined decompression of the posterior fossa and shunting of the syrinx. The Gardner procedure (decompression of the Chiari malformation and plugging of the obex) was the procedure most commonly used in managing our group of patients and resulted in improvement in over 70% of patients.     

Hiccups attributable to syringobulbia and/or syringomyelia associated with a Chiari I malformation: case report

OBJECTIVE AND IMPORTANCE: Approximately 20 to 50% of patients with syringomyelia associated with Chiari malformations exhibit cranial nerve or cerebellar symptoms. However, hiccups represent a rare clinical manifestation of this disorder. We report a case of intractable hiccups resulting from syringobulbia associated with a Chiari I malformation, which was successfully treated with foramen magnum decompression. CLINICAL PRESENTATION: We report the case of a patient who presented with syringomyelia and syringobulbia associated with a Chiari I malformation, manifested as intractable hiccups and neurological deficits. Magnetic resonance imaging scans demonstrated syringobulbia in the dorsal medullary region and a large cervical syrinx from C2 to C6-C7, associated with a Chiari I malformation. INTERVENTION: Foramen magnum decompression and a C1 laminectomy were performed. One month later, the intractable hiccups disappeared and the neurological symptoms demonstrated improvement. CONCLUSION: Postoperative magnetic resonance imaging scans demonstrated enlargement of the subarachnoid space in the posterior fossa and disappearance of the syringobulbia. There has been no recurrence of intractable hiccups and syringobulbia in 6 months after surgery. Magnetic resonance imaging of the brainstem is an important diagnostic procedure for intractable hiccups, because syringobulbia associated with a Chiari malformation represents a surgically treatable disorder, although the incidence is low.     

Foramen magnum decompression for syringomyelia associated with basilar impression and Chiari I malformation--report of three cases.

Anterior or posterior decompression of the foramen magnum was performed in three patients with syringomyelia associated with basilar impression and Chiari I malformation. The operative results were evaluated using the pre- and postoperative magnetic resonance (MR) images. Two patients with combined anterior and posterior cervicomedullary compression due to basilar impression and tonsillar descent received suboccipital craniectomy, upper cervical laminectomy, and dural plasty without any intradural manipulations via the posterior approach. One patient with prominent anterior cervicomedullary compression due to basilar impression and a sharp clivoaxial angle was operated on by the transoral anterior approach. Postoperatively, all patients showed a sustained shrinkage of the syrinx and rounding of the flattened cerebellar tonsils. Two patients showed upward movement of the herniated tonsils. All patients had improved symptoms during 2-4 years follow-up. Treatment of syringomyelia associated with basilar impression and Chiari I malformation requires more efficient decompressive procedures at the foramen magnum based on neurological and MR findings.     

Transverse Microincisions of the Outer Layer of the Dura mater Combined with Foramen Magnum Decompression as Treatment for Syringomyelia with Chiari I Malformation

Summary Numerous surgical procedures have been proposed for treatment of syringomyelia associated with Chiari I malformation, but the optimal treatment has not yet been uniformly standardised. The main aim of the surgical treatment of syringomyelia/Chiari I complex is directed toward restoration of physiological cerebrospinal fluid dynamic at the craniovertebral junction. We report the surgical results of eight patients, affected by syringomyelia and Chiari I malformation, age range from 18 to 62 years, treated by bony foramen magnum decompression combined with transverse microincisions of the outer layer of the dura mater. In an average postoperative follow-up period of two years neurological symptoms and signs improved in seven patients. Postoperative Magnetic Resonance showed a decrease in size of the syrinx in seven patients. These results suggest that foramen magnum decompression combined with transverse microincisions of the outer layer of the dura 1) is an effective and safe treatment option for syringomyelia and Chiari I malformation, 2) corrects the circulatory disturbances of cerebrospinal fluid dynamic, 3) leads to a decrease in size of the syrinx and to a significant improvement in neurological signs and symptoms, 4) avoids complications of intradural approaches and syringosubarachnoid shunting.     

Pathophysiology of headache associated with cough in patients with Chiari I malformation

OBJECT: The aim of this study was to evaluate the pathophysiology underlying headache associated with cough in patients with Chiari I tonsillar abnormality. The authors hypothesized that peak intrathecal pressure during coughing is higher in patients with headache aggravated by cough than in patients without or in healthy volunteers. In addition, the authors evaluated the use of intrathecal pressure during cough as a means of assessing obstruction to the free flow of cerebrospinal fluid (CSF) at the craniocervical junction. METHODS: Twenty-six adult patients with Chiari I malformation and syringomyelia, four adult patients with Chiari I malformation without syringomyelia, and 15 healthy volunteers were prospectively studied. Testing before surgery included the following: 1) clinical evaluation for the presence of headache associated with cough; and 2) evaluation of lumbar subarachnoid pressure at rest, during three to five coughs, while performing the Valsalva maneuver, during jugular compression, and after removal of CSF. Patients underwent suboccipital craniectomy, C-1 laminectomy, and duraplasty. Testing was repeated 6 months after surgery. CONCLUSIONS: Peak intrathecal pressures during cough and at baseline were elevated in patients with headache associated with cough compared with either patients without headache or healthy volunteers. After surgery, intrathecal pressures during cough were significantly lower than preoperative values and headache aggravated by cough was resolved partially or completely. Headache linked to coughing in patients with Chiari I malformation is associated with sudden increased intrathecal pressure caused by obstruction to the free flow of CSF in the subarachnoid space.     

Decompression of the spinal subarachnoid space as a solution for syringomyelia without Chiari malformation

STUDY DESIGN: Review and analysis of seven cases of syringomyelia treated surgically. OBJECTIVE: To demonstrate the beneficial role of decompressive surgery for the altered cerebrospinal fluid (CSF) flow dynamics in syringomyelia not associated with Chiari I malformation. A comparison between the pre- and post-operative syrinx size and CSF flow in the subarachnoid space was made using cine-mode magnetic resonance imaging (cine-MRI) and then correlated with clinical improvement. SETTING: University Hospital, Seoul, Korea. METHODS: Conventional spinal MRI and cine-MRI were performed in the region of CSF flow obstruction preoperatively in seven patients with syringomyelia not associated with Chiari I malformation. The group consisted of one case of syrinx with post-traumatic compression fracture, one case of post-traumatic arachnoiditis, two cases of holocord syrinx associated with hydrocephalus without Chiari malformation, one case of syrinx with post-traumatic pseudomeningeal cyst, one case of post-laminectomy kyphosis-associated syringomyelia and one case of post-tuberculous arachnoiditis syringomyelia. Based on the preoperative cine-MRI, the types of surgery appropriate to correct the CSF flow obstruction were chosen: decompressive laminectomy-adhesiolysis and augmentation duraplasty in arachnoiditis cases, ventriculoperitoneal shunt for hydrocephalus, cyst extirpation in pseudomeningeal cyst and both anterior and posterior decompression-fusion in the case of post-laminectomy kyphosis. A syrinx-draining shunt operation was performed in three cases; where the syringomyelia was associated with post-traumatic compression fracture refractory to a previous decompression, where hydrocephalus was present in which the decompression by ventriculoperitoneal shunt was insufficient and where post-traumatic arachnoiditis was present in which the decompression was impossible due to diffuse adhesion. Change in syrinx size was evaluated with post-operative MRI in all seven cases and restoration of flow dynamics was evaluated with cine-MRI in three of the cases, two patients with clinical improvement and one patient with no change of clinical status, respectively. RESULTS: Four out of seven patients showed symptomatic improvement after each decompressive operation. In the remaining three cases, reconstruction of the spinal subarachnoid space was not possible due to diffuse adhesion or was not the main problem as in the patient with syrinx associated with hydrocephalus who had to undergo a shunt operation. One of these three patients showed clinical improvement after undergoing syringosubarachnoid shunt. A decrease of syrinx size was observed in only two out of the five patients who showed clinical improvement after treatment. Of these five patients, two patients underwent post-operative cine-MRI and the restoration of normal CSF flow dynamics was noted in both patients. Of the remaining two patients, one underwent post-operative cine-MRI and there was no change in the CSF flow dynamics evident. CONCLUSION: These results suggest that the restoration of CSF flow dynamics between the syrinx and the subarachnoid space by decompressive operation is more effective than simple drainage of the syrinx cavity itself in the treatment of syringomyelia without Chiari malformation.     

Magnetic resonance imaging of Chiari malformations.

The authors describe the features of magnetic resonance (MR) images of Chiari type I and II malformations and present four illustrative cases. Downward displacement of the posterior fossa was more pronounced with type II than type I. A variety of intracranial anomalies were associated with the Chiari type II malformation, whereas type I was mainly associated with syringomyelia and craniovertebral changes. MR imaging is useful in the diagnosis of intracranial anomalies and those situated at the craniovertebral junction, including Chiari malformations.     

Results of the section of the filum terminale in 20 patients with syringomyelia,scoliosis and Chiari malformation

Summary Background. Spinal cord traction caused by a tight filum terminale may be considered a pathogenic mechanism involved in the development of syringomyelia, the Chiari malformation (type I) and scoliosis. Section of the filum terminale is proposed as a useful surgical approach in these conditions.Methods. Between April 1993 and July 2003, a total of 20 patients (8 men and 12 women) with a mean age of 33.5 years underwent section of the filum terminale with or without opening of the dural sac through a standard sacrectomy. Eight patients suffered from scoliosis, 5 from syringomyelia, 2 from Chiari malformation and 5 with a combination of these conditions.Finding. After section of the filum terminale, patients with syringomyelia showed an early clinical improvement of dysaesthesia, thermo-anaesthesia, hypo-aesthesia and walking difficulties. Rising of the medullary conus was also observed. In patients with scoliosis, back pain improved dramatically and a curve reduction was noticed, although progression of the curve was observed in one case. In patients with Chiari malformation, headache, dysaesthesia and paraparesis disappeared.Conclusions. Section of the filum terminale is a useful strategy in the treatment of scoliosis, syringomyelia and the Chiari malformation, and offers a new aetiological basis for the understanding of these three disorders.     

Chiari malformation (type I) associated with bilateral abducens nerve palsy: case report

There are still many questions concerning the pathology and therapeutic strategy for Chiari malformation. We recently encountered a case of Chiari malformation associated with only bilateral abducens nerve palsy which was successfully treated by foramen magnum decompression. The pathology and mechanisms of Chiari malformation (type I) were evaluated in this case. The patient was a 12-year-old girl who had visited our institute 1 month previously complaining of diplopia. She had no particular past or family history and her general appearance was also normal. Bilateral abducens nerve palsy was the only neurological deficit. Her cerebrospinal pressure was 9 cm H2O. Neuroradiological findings revealed basilar impression, C4-6 syringomyelia and Chiari malformation (type I), but no hydrocephalus or meningocele. Foramen magnum decompression with lateral extension, upper cervical laminectomy and open duraplasty were performed. The position of both eyes recovered fully shortly after surgery, and diplopia occurred only when the patient looked sideways. Eye movements also returned completely to normal 3 months after surgery. MRI showed no syringomyelia 4 months after surgery, the upward shift of the pendulous portion of the cerebellar tonsil, and the expansion of the subarachnoidal space of the pons-oblongata junction. In conclusion, it was thought that the mechanism of bilateral abducens nerve palsy in this case was due to the strangulation and the downward traction of the pons-oblongata junction. The nerve was relieved from this traction by foramen magnum decompression.     

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.     

Syringo-subarachnoid shunt for syringomyelia associated with Chiari malformation (type 1)

Summary The authors report the surgical results of 28 patients with syringomyelia associated with Chiari malformation (type 1). 28 patients underwent 34 operative procedures. Syringo-subarachnoid shunt was performed in 28 patients, foramen magnum decompression with syringo-subarachnoid shunt in three, ventriculo-peritoneal shunt in one, terminal syringostomy in one, and foramen magnum decompression with terminal syringostomy in one. In an average postoperative follow-up period of 3 years and 9 months ranging from one year to 7 years and one month, neurological symptoms and signs improved in 24 out of 28 patients (82%). Some improvement was noted in sensory deficit and motor weakness. In 3 patients, the symptoms did not change. In 3 patients whose symptoms were unchanged, preoperative studies demonstrated atrophy of the spinal cord, in which irreversible changes were shown. The symptoms deteriorated in one patient. In one patient whose symptoms increased 3 months after syringo-subarachnoid shunt, shunt insufficiency due to postoperative adhesive arachnoiditis was responsible for neurological deterioration.The authors propose that syringo-subarachnoid shunt is effective as a surgical procedure for syringomyelia associated with Chiari malformation (type 1) if the patient does not have symptoms due to Chiari malformation or has only mild signs and symptoms which do not require foramen magnum decompression, such as nystagmus or atrophy of sternocleidomastoid muscle.     

Spontaneous resolution of Chiari I malformation and syringomyelia: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Indications for surgery and the surgical technique of foramen magnum decompression for patients with Chiari I malformation and syringomyelia are controversial issues. This case report supports the view that observation may be adequate for patients without progressive symptoms or with mild clinical symptoms. CLINICAL PRESENTATION: A 37-year-old woman presented with a 3-month history of burning dysesthesias and hypesthesia in her right arm. A neurological examination revealed hypesthesia in the right trigeminal distribution. A magnetic resonance imaging scan revealed a Chiari I malformation with syringomyelia between C2 and T2. No hydrocephalus was observed. CLINICAL COURSE: Because the patient's symptoms regressed spontaneously, surgery was not performed. Thirty-two months after her initial examination, the patient was asymptomatic. A second magnetic resonance imaging scan was obtained, which demonstrated complete spontaneous resolution of the Chiari I malformation and syringomyelia. CONCLUSION: We attribute the regression of the patient's symptoms to spontaneous recanalization of cerebrospinal fluid pathways at the foramen magnum, which most likely was due to rupture of the arachnoid membranes that had obstructed cerebrospinal fluid flow.     

"特发性"胸椎左侧凸患者并发脊髓病变及其临床意义

目的 探讨对临床上无明显神经损害的"特发性"胸椎左侧凸患者存在的脊髓病变情况,并分析其临床意义.方法 1997年10月至2003年10月共诊治"特发性"胸椎左侧凸59例,其中男性31例,女性28例;年龄7～44岁,平均15岁;Cobb角15°～108°,平均56°.所有病例经临床及影像学检查,排除神经纤维瘤及先天性脊椎发育异常等病因.对临床诊断为"特发性"胸椎左侧凸的患者行全脊髓磁共振成像(MRI)检查,对脊髓病变的发生率进行统计.结果 59例"特发性"胸椎左侧凸患者经全脊髓MRI检查后,共有33例发现髓内病变,髓内病变发生率为56%,其中Chiari畸形合并脊髓空洞24例,脊髓空洞5例,Chiari畸形、脊髓空洞、脊髓裂1例,Chiari畸形、脊髓空洞、脊髓栓系1例,脊髓空洞合并栓系1例,Dandy-Walker畸形1例.其余26例未发现脊髓病变.分析伴有脊髓病变的胸椎左侧凸患者中男性比例及平均Cobb角均显著大于无脊髓病变的胸椎左侧凸(P＜0.05).结论 对于胸椎左侧凸,尤其是男性、Cobb角较大的患者需高度怀疑伴有脊髓病变.对于此类患者术前常规全脊髓MRI检查具有重要意义.     

Clinical outcomes in patients with Chiari I malformation: a review of 27 cases

BACKGROUND: Chiari I malformation is a congenital maldevelopment defined as downward herniation of the cerebellar tonsils through the foramen magnum. It has been treated using a variety of surgical procedures. Surgical outcomes have been reported with varying results throughout the literature. METHODS:We retrospectively reviewed 27 patients with Chiari I malformation operated at the University Hospital during a 9-year period from 1988 to 1997. We assessed preoperative and postoperative signs and symptoms in all the patients including headache, neck pain, nystagmus, vertigo, weakness, spasticity, atrophy, numbness, pain and temperature dissociation, diplopia, dysphagia, and sphincter dysfunction. Each patient was analyzed to determine if there was an arrest in the progression of the disease after surgical intervention. RESULTS: Syringomyelia was present in 59% of the patients. Only 1 patient who presented with neck pain improved. One patient reported new onset headache, and one patient described his headache resolved. Vertigo resolved in three patients; two patients stated mild improvement, and one patient reported worsening. Nystagmus improved or resolved in six patients. Weakness improved in only two patients who did not have syringomyelia. Dysphagia improved in two patients, and in the others it remained unchanged. Diplopia, spasticity, atrophy, and numbness remained unchanged. CONCLUSIONS: Our study provides evidence that the main benefit of the surgical management in patients with Chiari I malformation with or without syringomyelia is to arrest the progression of the disease.     

Surgical treatment of syringomyelia. Selection of surgical procedures

The surgical approach to syringomyelia is controversial. In this study, the authors evaluated the results of various operative procedures applied in 31 patients with syringomyelia. Syringomyelia was associated with Chiari malformation in 17 cases, with spinal adhesive arachnoiditis in nine, with trauma in three, and with epidural arachnoid cyst in one. One case was idiopathic. A total of 38 operations were performed. Syringosubarachnoid shunting was applied in 17 patients, syringoperitoneal shunting in 11, terminal syringostomy in three, ventriculoperitoneal shunting in three, lumboperitoneal shunting in two, foramen magnum decompression (suboccipital craniectomy plus upper cervical laminectomy) and terminal syringostomy in one, and foramen magnum decompression with syringosubarachnoid shunting in one. The postoperative follow-up period ranged from 2 to 55 months (average, 26 months). Of the 31 patients, 23 showed neurological improvement, five were unchanged, and three deteriorated. Among the last three, one patient with Chiari malformation developed shunt malfunction due to arachnoiditis after syringosubarachnoid shunting. In two patients with syringomyelia secondary to adhesive arachnoiditis, the spinal cord was damaged by extensive separation of the arachnoid membrane at surgery. On the basis of the results in these 31 cases, the authors conclude that syringosubarachnoid shunting is effective for syringomyelia associated with Chiari malformation if syringomyelia is responsible for the clinical symptoms. Post-traumatic syringomyelia and syringomyelia secondary to adhesive arachnoiditis should be treated by syringoperitoneal shunting. If hydrocephalus is present, ventriculoperitoneal shunting is indicated. Finally, terminal syringostomy is no more effective than syringosubarachnoid or syringoperitoneal shunting.     

Radiological outcome after surgical treatment of syringomyelia-Chiari I complex in adults: a systematic review and meta-analysis

Neurosurgical Review - Foramen magnum decompression (FMD) is widely accepted as the standard treatment for syringomyelia associated with Chiari type I malformation (CMI). Despite extensive clinical...     

Spontaneous resolution of syringomyelia and Chiari malformation Type I in a patient with cerebrospinal fluid otorrhea. Case report

The spontaneous resolution of syringomyelia in the setting of a Chiari malformation Type I (CM-I) has been reported infrequently. Several theories about the pathogenesis and spontaneous resolution of syringomyelia associated with CM-I have been proposed. The authors present the case of a patient with spontaneous resolution of a CM-I and syringomyelia coinciding with the development of cerebrospinal fluid (CSF) otorrhea. Although cases of spontaneous resolution of syringomyelia have been reported, this is the first reported case of spontaneous resolution of syringomyelia and a CM-I associated with the simultaneous development of CSF otorrhea.     

Presentation of type I Chiari malformation after head trauma

An unusual case of Type I Chiari malformation that became symptomatic after closed head injury is reported. The patient manifested transient upper extremity weakness, persistent lower cranial nerve dysfunction, and cerebellar signs that slowly resolved. Magnetic resonance images showed tonsillar ectopia but no displacement of the brain stem or syringomyelia. Type I Chiari malformation should be included in the differential diagnosis of patients who present with upper extremity weakness, lower cranial nerve palsies, or cerebellar signs after trauma.     

Malformations of the craniocervical junction (chiari type I and syringomyelia: classification,diagnosis and treatment)

Chiari disease (or malformation) is in general a congenital condition characterized by an anatomic defect of the base of the skull, in which the cerebellum and brain stem herniate through the foramen magnum into the cervical spinal canal. The onset of Chiari syndrome symptoms usually occurs in the second or third decade (age 25 to 45 years). Symptoms may vary between periods of exacerbation and remission. The diagnosis of Chiari type I malformation in patients with or without symptoms is established with neuroimaging techniques. The most effective therapy for patients with Chiari type I malformation/syringomyelia is surgical decompression of the foramen magnum, however there are non-surgical therapy to relieve neurophatic pain: either pharmacological and non-pharmacological. Pharmacological therapy use drugs that act on different components of pain. Non-pharmacological therapies are primarly based on spinal or peripheral electrical stimulation.