Resolution Of Syringomyelia Research Articles

Spontaneous resolution of Chiari I malformation and associated syringomyelia following parturition

Dear Editor, Spontaneous resolution of Chiari malformation and syringomyelia following normal delivery has not been reported to date. We are reporting one such rare case and discuss the putative mechanisms of this event. Case report: A 24-year-old woman presented in March 2009 with a 3-month history of headache and 2-month history of hoarseness of voice. On examination, her higher functions were normal. The gag reflex was decreased on the left side and there was left vocal cord palsy. There was no other neurological deficit. MRI revealed Chiari I malformation with peg-shaped tonsils herniating up to C 2 (arrows in Fig. 1a, b) with associated syringomyelia extending from C 3 to T 4. The risks and benefits of surgical treatment were explained to the patient and she chose a conservative line of management. Her headaches resolved with drugs for migraine. Three years later, she underwent normal vaginal delivery of a baby on March 14th, 2012. Neurological examination before and after delivery did not reveal any change. Two weeks following delivery, at the request of the patient, MRI was repeated. MRI showed ascent of the cerebellar tonsils (arrows in Fig. 1c, d) up to the foramen magnum with significant resolution of syringomyelia. The following theories have been postulated for spontaneous resolution:

Spontaneous resolution of syringomyelia in an adult patient with tight cisterna magna

Spontaneous resolution of syringomyelia in adult patients with Chiari malformation is exceptionally rare, with only 10 cases having been reported. A 21-year-old man working as a carpenter presented with a 1-year history of paresthesias in his right arm. A magnetic resonance imaging scan disclosed a cervicothoracic syrinx associated with tight tonsillar impaction of the cisterna magna without herniation. The patient left the carpentry job and underwent close monitoring with serial clinical and neuroradiological controls. The patient's symptoms gradually disappeared and magnetic resonance imaging studies revealed progressive shrinkage of the syrinx despite persistence of crowding of posterior fossa structures at the level of the foramen magnum. This case suggests that spontaneous resolution of syringomyelia can occasionally be triggered by the cessation of daily physical strain in patients with tight cisterna magna. Health care professionals should be aware that strenuous physical activities could affect the natural history of syringomyelia.

Craniovertebral junction abnormalities with hindbrain herniation and syringomyelia: regression of syringomyelia after removal of ventral craniovertebral junction compression

Hindbrain herniation syndrome, or Chiari malformation Type I (CM-I), occurs frequently with craniovertebral junction (CVJ) abnormalities when there is reduction in the posterior fossa volume. Syringomyelia is often present. Posterior fossa dorsal decompression (PFDD) is typically performed but has adverse results when ventral bone abnormality exists. This paper presents the results of a prospective study on CVJ abnormalities in patients with CM-I and syringomyelia. Between 1984 and 2008 (the MR imaging era), 298 patients with CVJ abnormalities and CM-I underwent ventral cervicomedullary decompression. Eighty-four patients had associated syringomyelia (15 with secondary invagination and 69 with primary basilar invagination, os odontoideum, or malunion of fractures). Of these 84 patients with CVJ abnormalities, CM-I, and syringomyelia, 46 had previously undergone PFDD, and 28 had previously undergone PFDD combined with fusion procedures or shunt placements. Of the 84 patients, a cervicothoracic syrinx was observed in 57, thoracic syrinx in 14, and holocord syrinx in 13. Studies included CT, MR imaging, and cine flow studies. All 298 patients who underwent ventral CVJ decompression had irreducible or partially reducible pathology. All 84 with syringomyelia showed brainstem dysfunction, lower cranial nerve symptoms, or myelopathy. Brainstem signs improved in 66 of the 84 patients, myelopathy improved in 58, and syringomyelia regressed in 64. Neurological improvement and syringomyelia resolution can occur using only ventral cervicomedullary junction decompression in patients with basilar invagination and basilar impression. This is likely due to the relief of neural encroachment and reestablishment of CSF pathways.

Volumetric analysis of syringomyelia following hindbrain decompression for Chiari malformation Type I: syringomyelia resolution follows exponential kinetics

Resolution of syringomyelia is common following hindbrain decompression for Chiari malformation, yet little is known about the kinetics governing this process. The authors sought to establish the volumetric rate of syringomyelia resolution. A retrospective cohort of patients undergoing hindbrain decompression for a Chiari malformation Type I with preoperative cervical or thoracic syringomyelia was identified. Patients were included in the study if they had at least 3 neuroimaging studies that detailed the entirety of their preoperative syringomyelia over a minimum of 6 months postoperatively. The authors reconstructed the MR images in 3 dimensions and calculated the volume of the syringomyelia. They plotted the syringomyelia volume over time and constructed regression models using the method of least squares. The Akaike information criterion and Bayesian information criterion were used to calculate the relative goodness of fit. The coefficients of determination R(2) (unadjusted and adjusted) were calculated to describe the proportion of variability in each individual data set accounted for by the statistical model. Two patients were identified as meeting inclusion criteria. Plots of the least-squares best fit were identified as 4.01459e(-0.0180804)(x) and 13.2556e(-0.00615859)(x). Decay of the syringomyelia followed an exponential model in both patients (R(2) = 0.989582 and 0.948864). Three-dimensional analysis of syringomyelia resolution over time enables the kinetics to be estimated. This technique is yet to be validated in a large cohort. Because syringomyelia is the final common pathway for a number of different pathological processes, it is possible that this exponential only applies to syringomyelia related to treatment of Chiari malformation Type I.

Resolution of syringomyelia after release of tethered cord

Background Syringomyelia is an abnormal cystic dilatation of the spinal cord caused by excessive accumulation of CSF. Patients can develop various neurologic deficits secondary to untreated syringomyelia, some of which can be permanent despite surgical intervention. Case Description The authors present a patient with syringomyelia, aortic coarctation, and tethered cord syndrome. Serial radiographic imaging demonstrated initial significant reduction of the thoracic syrinx after coarctation repair and release of tethered cord. However, subsequent follow-up imaging revealed partial recurrence. Conclusion This case provides evidence of a possible cause-effect relationship between syringomyelia and tethered cord. It demonstrates the indication of surveillance imaging of the entire spine to ensure that all potential etiologies of syringomyelia are identified and treated. Furthermore, it illustrates the complex dynamic nature of syrinx physiology and reinforces the importance of serial follow-up studies after surgical intervention.

Resolution of syringomyelia and Chiari malformation after growth hormone therapy

The association between hypopituitarism, Chiari I malformation, and syringomyelia has been recently recognized. Most of the reported patients suffered perinatal injury or asphyxia. We present the case of a premature child without identifiable perinatal injury, who was recognized to have growth hormone (GH) deficiency, Chiari I malformation, and syringohydromyelia. There was a resolution of syrinx and tonsillar herniation after GH replacement.

Time course of syringomyelia resolution following decompression of Chiari malformation Type I

To better understand syrinx pathophysiology, the authors performed a prospective study in which they used findings from serial clinical and magnetic resonance (MR) imaging examinations performed before and after craniocervical decompression to establish the time course of syrinx narrowing. Serial clinical examinations and cervical MR imaging were performed in 29 consecutive patients with Chiari malformation Type I (CM-I) and syringomyelia before surgery, 1 week, and 3-6 months after surgery, and then annually. Time to narrowing of the syrinx (>50% decrease in maximal anteroposterior diameter) following surgery was calculated using the Kaplan-Meier method. All syringes decreased in diameter and length (number of segments) on MR images at 3-6 months, 1 year, and 2 years or later. The syrinx diameter decreased from 6.9+/-2.1 mm (mean+/-standard deviation) preoperatively to <1.5 mm at last evaluation (p<0.0001). The median time to syrinx narrowing was 3.6 months following CM-I decompression (95% confidence interval 3.0-6.5 months). After surgery 94% of patients had improved symptoms, but symptoms resolved incompletely in 68% of patients; 52 and 59% of patients had residual dysesthesias and sensory loss, respectively. Clinical improvement occurred before partial or complete disappearance of the syrinx on MR images. Patient age, duration of symptoms, sex, preoperative syrinx diameter, and length of syrinx were unrelated to time to syrinx narrowing. Most patients improve after decompression for CM-I, but many have residual symptoms. Syringes may continue to diminish for months to years after surgical decompression. A collapsed syrinx (absence of distention of the spinal cord) indicates that the pathophysiology has been reversed by treatment regardless of the completeness of elimination of the cavity on MR images.

Time Course of Syringomyelia Resolution after Decompression of Chiari I Malformation

Time Course of Syringomyelia Resolution after Decompression of Chiari I Malformation

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

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.

Spontaneous Resolution of Syringomyelia Without Chiari Malformation-Case Report-

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.

An unusual presentation and resolution of syringomyelia after cervical spine injury

The authors describe a case of noncommunicating syringomyelia associated with Chiari malformation Type I in a patient in whom acute symptomatic exacerbation occurred following cervical spine trauma. Surgical stabilization and realignment of the spine resulted in marked resolution of the neurological abnormalities, and subsequent magnetic resonance imaging demonstrated persistent collapse of the syrinx. The authors review the various factors in the pathogenesis of this unusual sequence of events.

Arachnoid veils and the Chiari I malformation.

The literature contains scant data regarding variations in anatomy at the level of the foramen of Magendie in patients with Chiari I malformation and syringomyelia. Based on their operative experience and hospital data, the authors detailed the incidence of arachnoid veils found in juxtaposition to the foramen of Magendie in patients with hindbrain herniation. Additionally, radiological studies were retrospectively reviewed in cases in which such an anomaly was noted intraoperatively. Of 140 patients with Chiari I malformation who underwent decompressive surgery, an associated syrinx was demonstrated in 80 (57%). The foramen of Magendie was obstructed by an arachnoid veil in 10 (12.5%) of these patients; once the lesion was punctured, the cerebrospinal fluid drained freely from this median aperture. On retrospective review of imaging studies, none of these anomalous structures was evident. In all patients with an arachnoid veil and syringomyelia resolution of syringomyelia was revealed on postoperative imaging. In the absence of a clear pathophysiology of syrinx production, the authors would recommend that patients with syringomyelia and Chiari I malformation undergo duraplasty so that, if present, these veils can be fenestrated.

Spontaneous resolution and recurrence of a Chiari I malformation and associated syringomyelia

The spontaneous resolution of syringomyelia has been reported infrequently. In patients with Chiari I malformations, resolution of the syringomyelia has sometimes been associated with improvement of their malformation. The authors present a case of spontaneous resolution followed by recurrence of syringomyelia and a corresponding change in the Chiari malformation. This case is of interest in light of the theories postulated to explain spontaneous resolution of syringomyelia.

Resolution of syringomyelia and Chiari I malformation by ventriculoatrial shunting in a patient with pseudotumor cerebri and a lumboperitoneal shunt

Resolution of syringomyelia and Chiari I malformation by ventriculoatrial shunting in a patient with pseudotumor cerebri and a lumboperitoneal shunt