Remote cerebellar hemorrhage after foramen magnum decompression surgery for Chiari I malformation--case report

A 47-year-old woman underwent decompressive suboccipital craniectomy and C1 laminectomy with duroplasty in the prone position for Chiari malformation type I and syringomyelia. The arachnoid membrane was not injured. Intraoperative echography showed good enlargement of the subarachnoid space. No closed subcutaneous drain was used. The patient complained of repeated nausea and vomiting 3 hours after the operation, and computed tomography revealed remote cerebellar hemorrhage on postoperative day 1. The cerebellar hemorrhage was treated conservatively, and the symptoms continued only for 3 days after surgery. Dural opening with rapid loss of cerebrospinal fluid (CSF) has occurred in every reported case of remote cerebellar hemorrhage complicating intracranial and spinal procedures. Loss of CSF is the main pathogenesis of this condition. In our case, the most probable pathomechanism seems to involve stretching of the infratentorial cerebellar bridging veins due to cerebellar sagging because of dural opening in the prone position and drop in CSF pressure. Such a complication is rare but should be considered after foramen magnum decompression surgery if the patient shows unusual symptoms of repeated vomiting.     

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.     

14 cases of communicating syringomyelia associated with Chiari I malformation in children]

The authors review their experience of 14 children with syringomyelia and associated Chiari I malformation observed during the last 21 years. Initial symptoms were motor weakness (57%), sensory signs (50%), scoliosis (50%) and bladder dysfunction (21.5%), while signs of admission were pyramidal signs (78.5%) with motor deficit (43%), spinal deformities (64.2%), syringomyelic syndrome (36%) and sensory deficit (21.5%). Four children were investigated with myelography, three with myelography and spinal CT Scan and the last seven cases with MRI. Ten children (71.4%) underwent a decompression of the Chiari malformation with or without drainage of the cyst and shunting of an associated hydrocephalus. Syringo-peritoneal shunts were used in 3 children and laminectomy with syringo-subarachnoid shunt in 1 case. The neurological symptoms improved in 57% of the cases, 2 patients deteriorated and 4 patients were lost to follow-up. The authors discuss the clinical symptomatology, the MRI study, the controversies concerning the treatment and concluded with their attitude concerning this pathology in children.     

Surgical technique for cranio-cervical decompression in syringomyelia associated with Chiari type I malformation

Summary Our purpose is to present our results with the surgical treatment of syringomyelia associated with Chiari type I malformation. Between October 1989 and October 1995, twenty-eight patients underwent a sub-occipital craniotomy and a C₁ laminectomy. After dura mater opening the cerebellar tonsils were mobilised. Neither catheter, nor plugging of the obex, nor tonsillar tissue removal was performed. The dura mater was enlarged by means of a wide graft to create a newcisterna magna of adequate size. Postoperative MRI scans showed an ascent of the cerebellum of 4.3±4.8 mm (measured by thefastigium to basal line), as well as of the brainstem (mean migration of the mesencephalon-pons junction of 4.3±3.3 mm). The tonsils emigrated cranially 6.5±4.8 mm. While preoperative mean syringo-cord ratio was 66.3%±13.3, post-operatively was 12.1%±12.7 (p<0.0001). A complete collapse of the syrinx was observed in 39% of the patients. Long-term improvements were obtained in 73% of the cases and 27% were unchanged. No patient got worse. We conclude that in the treatment of syringomyelia associated with Chiari I malformation an artificial cisterma magna of sufficient size must be created. This is achieved by means of an extensive sub-occipital craniotomy and C₁ laminectomy, followed by dural opening. Small bone removal with limited enlargement of theposterior fossa often results in failures of treatment and recurrences. Tonsillar removal is not necessary to obtain a good reconstruction of the cisterma magna.     

Spontaneous resolution of a Chiari I malformation associated syringomyelia in one child

Summary A child with complete spontaneous resolution of a Chiari I malformation associated Syringomyelia without surgical intervention is presented. The child was followed clinically by serial magnetic resonance imaging (MRI) and remains neurologically stable after 8-years of follow-up. To our knowledge, only 6 pediatric cases with spontaneous resolution of a spinal cord syrinx documented by MRI without surgical intervention have been reported. This case is of interest in the light of the postulated theories to explain spontaneous resolution of syringomyelia.     

Chiari I malformation associated with syringomyelia: a retrospective study of 316 surgically treated patients

OBJECTIVES: The objective of this article is to probe the surgical procedures of treatment for Chiari I malformation (CMI) associated with syringomyelia and evaluate their efficacy based on clinical and neuroradiological results. STUDY DESIGN: Retrospective study. SETTING: Department of Neurosurgery in Qilu Hospital of Shandong University in China. METHODS: We retrospectively reviewed 316 surgically treated patients with CMI. We classified our patients after surgery in four global outcome categories as follows: very good (complete remission or marked improvement), good (slight improvement), fair (stability), poor (slight or marked deterioration). We simply describe the postoperative diameter of the syrinx (collapse, decreased, unchanged or increased). RESULTS: At the time of discharge, the result was very good in 163 cases (51.58%), good in 92 cases (29.11%), fair in 49 cases (15.51%) and poor in 12 cases (3.80%). In the follow-up period, neurological status improved and was then sustained in 94.49% of patients (206/218). At more than 2 years after surgery, we were able to compare pre- and postoperative MR images in 218 patients. A collapse of the syrinx was seen in 34.86% (76/218) of patients. The size of the syrinx was decreased in 31.65% (69/218) of patients, remained unchanged in 26.15% (57/218) of patients and increased in 7.34%(16/218) of patients. CONCLUSION: Patients with CMI should be treated using various surgical procedures according to different magnetic resonance imaging types; surgical treatments may fully decompress the medulla oblongata and ameliorate the clinical syndromes.     

Extra-arachnoidal cranio-cervical decompression for syringomyelia associated with Chiari I malformation in adults: technique assessment

Summary Background. The osteo-dural decompression of the cerebellar tonsils at the cranio-cervical junction is generally considered the most effective treatment for syringomyelia-Chiari I complex. However much controversy concerning a great number of surgical adjuvants to the standard bony decompression is still present. In this work an extra-arachnoidal cranio-cervical decompression (CCD) without duroplasty is described and the surgical results are reported. Method. Between 2000 and 2005, 24 adult patients underwent surgery for symptomatic syringomyelia-Chiari I complex not associated with hydrocephalus. In all cases, the surgical procedure consisted of a limited suboccipital craniectomy and laminectomy of C1 (when necessary C2 as well) followed by dural opening leaving the arachnoid membrane intact. The dura mater is left open and stitched laterally to the muscles. Findings. With a mean clinical long term follow-up of 44 months (range, 12–78 mo), neurological disturbances improved in 21 of 24 patients (87.5%) as result of extra-arachnoidal CCD. The postoperative complications occurred when the arachnoid was accidentally violated (4 cases, 16.6%). The complications included aseptic meningitis (one patient), nucal pseudomeningocele (two patients) and postoperative hydrocephalus requiring a ventriculoperitoneal shunt (one patient). Finally, one patient received an additional C2 laminectomy in order to obtain symptoms improvement and syrinx shrinkage. Postoperative MRI studies demonstrated that the syrinx decreased in size or collapsed in 20 patients (83.3%) and stabilized in 4 (16.7%). Conclusions. The extra-arachnoidal CCD is a safe and effective treatment for syringomyelia associated with Chiari I malformation in adults without intraoperative evidence of adhesive arachnoiditis. However a larger number of patients and longer follow-up will be necessary to determine the efficacy of extra-arachnoidal CCD.     

An extramedullary spinal cord tumor associated with syringomyelia: a case report

A 53-year-old woman presenting weakness in the left hand for ten years was diagnosed seven years ago as having juvenile muscular atrophy of the unilateral upper extremity. Afterwards, her left forearm and hand gradually became weaker. A neurologist pointed out syringomyelia in the film of MR imaging. However, the cause of syringomyelia was not demonstrated until gadolinium-DTPA-enhanced MR imaging disclosed a spinal tumor. Through an operation it was found that it was an intradural extramedullary tumor at C7 level. Histological diagnosis of the tumor was meningioma. Only 12 reports of syringomyelia associated with extramedullary intraspinal tumors were found. Among them each tumor in three cases was diagnosed by delayed metrizamide computed tomography, only one case by MR imaging. Though in our case myelography, postmyelographic CT, intravenous enhanced CT and MR imaging were performed, gadolinium-DTPA-enhanced MR imaging was eventually most useful in diagnosing this tumor. The pathophysiologic mechanism by which the syrinx fills, and the differentiation in MR imaging between syrinx and tumor cyst were reviewed.     

Bilateral vocal cord palsy causing stridor as the only symptom of syringomyelia and Chiari I malformation,a case report

IntroductionBilateral vocal cord palsy is a condition which has many causes (Gupta et al., 2012) [1]. Syringomyelia is an uncommon condition which describes the formation of fluid filled cavity, occupying the spinal cord (Chang, 2003) [2]. It rarely manifests itself as subacute onset of stridor.Presentation of caseWe present the case of a three year old female who presented for evaluation of her speech and language delay, when incidentally it was made note of her loud breathing which had previously been managed as bronchiolitis by her general practitioner. In hospital she was found to have a bilateral vocal cord palsy. Further investigation revealed a large syrinx as well as an associated Arnold Chiari 1 malformation, for which she required neurosurgical decompression.ConclusionAlthough uncommon, formation of a syrinx should be considered for patients who present with stridor and reiterates the importance of MRI as an important investigative tool of bilateral vocal cord palsy.     

Chiari I malformation with traumatic syringomyelia and spontaneous resolution: case report and literature review.

A case history of a 28-year-old woman who sustained a moderately severe head injury and then developed acute bilateral arm weakness is presented. Magnetic resonance imaging studies revealed a Chiari I malformation with a large cervical syringomyelia (hydromyelia). The patient's arm weakness almost completely resolved spontaneously as did her syrinx. How this case is interpreted in light of the various theories of pathogenesis will be discussed.     

Cervical spinal motion before and after surgery in patients with Chiari malformation type I associated with syringomyelia

OBJECT: There have been few reports about the cervical spinal motion in patients with Chiari malformation Type I (CM-I) associated with syringomyelia. To investigate this phenomenon, the relationship between the preoperative cervical range of motion (ROM) and the stage of cerebellar tonsillar descent as well as the cervical ROM before and after foramen magnum decompression (FMD) were evaluated. METHODS: Thirty patients who had CM-I associated with syringomyelia and who underwent FMD participated in the study. The ROM and lordosis angle of the cervical spine were measured on x-ray films. In addition, the relationship between preoperative degree of cerebellar tonsillar descent and the ROM between the levels of the occiput (Oc) and C2 was investigated. RESULTS: The mean flexion-extension ROM at Oc-C2 was 15.5 degrees before and 14.1 degrees after surgery, and the mean flexion-extension ROM of C2-7 was 55.1 degrees before and 52.8 degrees after surgery. The mean pre- and postoperative lordosis angles at C2-7 were 16.8 and 19.1 degrees, respectively. There was no significant difference between the values measured before and after surgery. There was no correlation between the degree of cerebellar tonsillar descent and the ROM at Oc-C2. CONCLUSIONS. Foramen magnum decompression is an excellent surgical technique that has no effect on the postoperative cervical ROM and cervical alignment.     

蛛网膜下腔引流治疗Chiari畸形伴脊髓空洞的疗效观察

目的:探讨蛛网膜下腔引流治疗Chiari畸形伴脊髓空洞的手术技巧,总结其临床效果。方法:对56例Chiari畸形伴脊髓空洞的患者进行后颅凹减压和蛛网膜下腔引流术。根据引流部位的不同分为两组,A组行椎板切除术后从正中切开脊髓放置引流;B组从神经根下入路切开脊髓放置引流。结果:两种手术方法术后患者疼痛和感觉减退均明显好转,临床效果满意。1例手术后3周因颈椎不稳猝死,3例手术后上肢麻木加重。结论:Chiari畸形伴脊髓空洞在枕骨大孔减压的同时行空洞蛛网膜下腔引流术效果好,不足之处为手术切口长,椎板打开节段数多,少数患者术后上肢麻木加重。     

Endoscopic third ventriculostomy for treatment of noncommunicating syringomyelia associated with a Chiari I malformation and hydrocephalus: case report and pathophysiological considerations

OBJECTIVE AND IMPORTANCE: A Chiari I malformation associated with syringomyelia and hydrocephalus is a rare condition. We report the successful use of endoscopic third ventriculostomy for the treatment of this pathological entity. The successful use of this technique in such a case has not been previously described, and the results allow us to speculate on the pathophysiological mechanism involved. CLINICAL PRESENTATION: A 34-year-old woman presented with headaches, a motor deficit of the right upper limb, and gait dyspraxia. Magnetic resonance imaging scans demonstrated dilation of all ventricles, compression of the retrocerebellar cerebrospinal fluid space, downward displacement of the tonsils, and syringomyelia. Syringomyelia involved the cervicodorsal cord below C3, with a syrinx-free segment between C1 and C3 and no enlargement of the rostral part of the central canal. INTERVENTION: Endoscopic third ventriculostomy resulted in prompt improvement of the clinical symptoms. Postoperative magnetic resonance imaging scans demonstrated shrinkage of the syrinx and return of the cerebellar tonsils to their physiological positions. CONCLUSION: This experience demonstrates that endoscopic third ventriculostomy, which is a simple, safe technique, may be the treatment of choice for associated Chiari I malformations, hydrocephalus, and syringomyelia (even the noncommunicating type).     

Persistent primitive hypoglossal artery associated with arteriovenous malformation--case report.

A case of persistent primitive hypoglossal artery (PPHA) associated with arteriovenous malformation (AVM) is reported. A 46-year-old male suddenly developed severe headache followed by transient unconsciousness and was admitted to our hospital 2 hours later. A computed tomographic scan showed subarachnoid hemorrhage. Angiograms revealed an AVM in the left cerebellar hemisphere and an ipsilateral PPHA. The AVM was completely removed and he was discharged 1 month after surgery without neurological deficit. Only three cases of PPHA associated with intracranial AVM have been reported in the literature. One patient died of rebleeding from the AVM before surgery, and another was conservatively treated because the AVM was too large for resection. The remaining one was surgically treated only by ligation of the feeding arteries. Ours is the first case treated by total removal of the AVM. Since these four cases, including ours, account for 3.0% of 134 cases of PPHA reported, PPHA associated with AVM is considered rare.     

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.     

Spinal haemangioblastoma associated with syringomyelia and multiple lung lesions

Haemangioblastomas involving the spinal cord are uncommon but by no means unknown. It is well established that haemangioblastomas of the central nervous system are commonly associated with lesions in the retinae and in various abdominal organs. The simultaneous occurrence of similar tumours in the lung is less common and not well recognized. The case of a man with a haemangioblastoma of the spinal cord with many small haemangioblastomas of the lung is reported here.     

Advanced Chiari malformation type I with marked spinal cord atrophy. Case report

The authors discuss the results obtained in patients who underwent foramen magnum decompression for longstanding advanced Chiari I malformation in which marked spinal cord atrophy was present. This 50-year-old woman presented with progressive quadriparesis and sensory disorders. Magnetic resonance imaging revealed the descent of cerebellar tonsils and medulla associated with remarkable C1-L2 spinal cord atrophy. After a C-1 laminectomy-based foramen magnum decompression, arachnoid dissection and duraplasty were undertaken. These procedures resulted in remarkable neurological improvement, even after 40 years of clinical progression. Spinal cord atrophy may be caused by chronic pressure of entrapped cerebrospinal fluid in the spinal canal.