Chiari I malformation related syringomyelia: radionuclide cisternography as a predictor of outcome

Summary Background. This prospective study, conducted in patients with Chiari I malformation (C I) related syringomyelia who underwent posterior decompression and duroplasty, utilizes radionuclide cisternography in order to study the cerebrospinal fluid (CSF) dynamics at the foramen magnum and to predict the clinical outcome following surgery.Methods. 17 consecutive patients of C I with syringomyelia (but without hydrocephalus or fixed atlanto-axial dislocation), underwent a detailed neurological examination and were assigned a clinical disability score based on the modified Klekamp and Samii score. A radionuclide cisternography (using Tc99m-DTPA) was performed via the lumbar route and the ascent of the tracer was followed utilizing a gamma camera immediately after injection and then sequentially after 1, 2, 4, 6 and 24 hours. After posterior decompression and duroplasty, the modified Klekamp and Samii score was repeated at follow-up visits (range: 3 months to one year) along with radionuclide cisternography at 3 months, and MR imaging at 6 months.Findings. Three patterns of tracer flow were observed: a) rapid flow (n=7); b) supratentorial subarachnoid delay (n=7); and, c) foramen magnum block (n=3). The patients having foramen magnum block had the poorest clinical scores on admission. At follow up, there was an improvement in the clinical scores so that the mean scores in all three categories reached nearly the same level. Following posterior decompression, the radionuclide cisternography performed in 10 patients showed a rapid flow of the tracer without any obstruction. The syrinx resolved in 4 of the 11 patients in whom an MRI was done.Interpretation. The patients with C I with syringomyelia may often have a free flow of tracer across the FM. Posterior decompression and duroplasty provides maximum clinical relief in patients with a demonstrable foramen magnum block on radionuclide cisternography while those with a normal flow have less relief. The symptomatology related to brain-stem compression immediately responds to the surgical procedure but the syrinx-induced signs and symptoms of spinal cord dysfunction persist.     

Spontaneous resolution of syringomyelia without Chiari malformation

A 30-year-old woman presented with a cervical syrinx manifesting as hemihypesthesia. Neuroimaging found no evidence of Chiari malformation or tight cisterna magna. Serial magnetic resonance imaging studies over a 6-year period demonstrated spontaneous and complete resolution of the syrinx accompanied by an asymptomatic clinical course. The natural history of syringomyelia is highly unpredictable. The outcome of surgical treatment for patients with syringomyelia is not always satisfactory, so the indications for surgery are controversial. Spontaneous resolution of syringomyelia unrelated with foramen magnum lesion has various causes. Close follow up of the patient is necessary to monitor for recurrence.     

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.     

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.     

Anorectal malformation and Down's syndrome in monozygotic twins

Anorectal malformation (ARM) can be divided in high, intermediate, and low forms according to the level of termination of the rectum in relation to the pubococcygeal and ischiatic lines. Patients with Down's syndrome have a high incidence of gastrointestinal anomalies, such as tracheoesophageal fistula, duodenal obstruction, annular pancreas, Hirschsprung's disease, and ARM. In these children, ARM is generally low with or without a fistula. The mode of inheritance of ARM and its genetic relation with Down's syndrome is not known, even if the association (ARM-Down's syndrome) seems not to be coincidental. We describe here a very rare case of monozygotic twins born with the association of ARM and Down's syndrome.     

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.     

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.     

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.     

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.     

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.     

Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia: case report

BACKGROUND: Chiari I malformation, accompanied by superposed bony anomaly of the craniovertebral junction, is comparatively rare. We report a case of Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia. CASE DESCRIPTION: The patient was a 61-year-old woman demonstrating numbness of the extremities, sensory impairment, muscular weakness, and tendon hyper-reflexia. X-ray images and CT scans demonstrated assimilation of the atlas to the occipital bone, C2 and C3 fusion, abnormal passage of the vertebral arteries, and an anomalous bony mass on the right lateral mass of the atlas protruding into the spinal column. The odontoid process was also deviated to the left. Magnetic resonance images demonstrated bilateral descent of the cerebellar tonsils and syringomyelia extending from C6 to T8. Computed tomographic scans with the head rotated to the right demonstrated increased narrowing of the vertebral column caused by the right lateral mass of the atlas, and MR images confirmed exaggerated deformation of the spinal cord at the same region. This deformation manifested no neurologic symptoms, and we therefore performed foramen magnum decompression and duraplasty using Gore-Tex (W.L. Gore & Associates, Inc., Flagstaff, AZ). In the early postoperative period, neurologic symptoms improved. CONCLUSION: We believe it is important that a treatment plan for Chiari I malformation accompanied by bony anomaly of the craniovertebral junction be determined based on morphologic investigation of the region supplemented by dynamic imaging-based evaluation of instability, or a careful inspection for atypical passage of the vertebral arteries, a frequent site of complication.     

脊柱侧凸伴发Chiari畸形和脊髓空洞的影像学特征及临床意义

目的探讨脊髓空洞源性脊柱侧凸的影像学特征以及Chiafi畸形、脊髓空洞与脊柱侧凸影像学模式之间的相关性，并评估其临床意义。方法根据侧凸Cobb角的不同，将87例伴发Chiafi畸形和脊髓空洞的脊柱侧凸患者分为三组：A组（10°≤Cobb角≤30。）13例，B组（300°〈Cobb角≤600）42例，C组（Cobb角〉60°）32例。以特发性脊柱侧凸的影像学特征作为参考，将伴发Chiari畸形和脊髓空洞的脊柱侧凸模式分为典型和不典型两类，并测量胸椎后凸和腰椎前凸的Cobb角。在MRIT。加权像正中矢状面扫描层面上测量小脑扁桃体下移程度、空洞的形态和长度以及空洞与脊髓的最大比值（S／C最大比值），并对以上测量指标进行比较分析。结果87例患者中不典型侧凸的总发生率为43．7％（38／87例），其中左胸弯的发生率为42．5％。典型侧凸模式中不典型特征总发生率为65．3％。三组之间，不典型侧凸的发生率、典型侧凸中不典型特征的发生率差异均无统计学意义（P〉0．05）。三组间胸椎后凸差异有统计学意义（P〈0．05）。小脑扁桃体下移程度、脊髓空洞的长度和形态以及S/C最大比值与脊柱侧凸严重程度、不典型侧凸发生率之间均没有相关性（P〉0．05）。结论对于具有不典型侧凸模式、典型侧凸模式包含不典型特征、胸椎正常后凸或过度后凸的脊柱侧凸患者，术前全脊髓MR扫描应作为常规检查。小脑扁桃体下移程度和脊髓空洞模式对脊柱侧凸的进展和不典型侧凸的发生均未见明显影响。