Reduction In Syrinx Size Research Articles

Complications and outcomes of posterior fossa decompression with duraplasty versus without duraplasty for pediatric patients with Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM). The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups. A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD. PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

A Safe and Effective Posterior Intra-Articular Distraction Technique to Treat Congenital Atlantoaxial Dislocation Associated With Basilar Invagination: Case Series and Technical Nuances.

The management of atlantoaxial dislocation (AAD) associated with basilar invagination (BI) is challenging, and traditional posterior-only approaches lack the ability to release the anterior soft tissue resulting in unsatisfactory reduction. Furthermore, vertebral artery anomalies and deformed anatomy increase surgical risks. To introduce a safe and efficient technique to reduce congenital AAD and BI through a single-stage posterior-only approach. A total of 65 patients with AAD and concomitant BI who had congenital osseous abnormalities were retrospectively analyzed. All patients had anterior soft tissue released through a posterior-only approach, followed by intra-facet cages implantation, cantilever correction, and instrumentation. Clinical results were measured using the Japanese Orthopedic Association (JOA) scale, and radiographic measurements included the atlanto-dental interval, the distance of odontoid tip above Chamberlain's line, clivus-canal angle (CCA), and syrinx length. Paired t-tests were used to compare preoperative and postoperative measurements. The mean JOA score increased from 10.98 to 14.40 at 1-yr follow-up. Complete reduction of AAD and BI was achieved in 48 patients (73.8%). The mean CCA improved from 115° preoperatively to 129° postoperatively. Reduction of syrinx size was observed in 14 patients at 1 wk and in 35 patients 1 yr after surgery. All patients achieved bony fusion. Posterior intra-articular distraction followed by cage implantation and cantilever correction can achieve complete reduction in most cases of congenitally anomalous AAD associated with BI.

Timing of syrinx reduction and stabilization after posterior fossa decompression for pediatric Chiari malformation type I.

The aim of this study was to determine the timeline of syrinx regression and to identify factors mitigating syrinx resolution in pediatric patients with Chiari malformation type I (CM-I) undergoing posterior fossa decompression (PFD). The authors conducted a retrospective review of records from pediatric patients (< 18 years old) undergoing PFD for the treatment of CM-I/syringomyelia (SM) between 1998 and 2015. Patient demographic, clinical, radiological, and surgical variables were collected and analyzed. Radiological information was reviewed at 4 time points: 1) pre-PFD, 2) within 6 months post-PFD, 3) within 12 months post-PFD, and 4) at maximum available follow-up. Syrinx regression was defined as ≥ 50% decrease in the maximal anteroposterior syrinx diameter (MSD). The time to syrinx regression was determined using Kaplan-Meier analysis. Multivariate analysis was conducted using a Cox proportional hazards model to determine the association between preoperative, clinical, and surgery-related factors and syrinx regression. The authors identified 85 patients with CM-I/SM who underwent PFD. Within 3 months post-PFD, the mean MSD regressed from 8.1 ± 3.4 mm (preoperatively) to 5.6 ± 2.9 mm within 3 months post-PFD. Seventy patients (82.4%) achieved ≥ 50% regression in MSD. The median time to ≥ 50% regression in MSD was 8 months (95% CI 4.2-11.8 months). Using a risk-adjusted multivariable Cox proportional hazards model, the patients who underwent tonsil coagulation (n = 20) had a higher likelihood of achieving ≥ 50% syrinx regression in a shorter time (HR 2.86, 95% CI 1.2-6.9; p = 0.02). Thirty-six (75%) of 45 patients had improvement in headache at 2.9 months (IQR 1.5-4.4 months). The maximum reduction in syrinx size can be expected within 3 months after PFD for patients with CM-I and a syrinx; however, the syringes continue to regress over time. Tonsil coagulation was associated with early syrinx regression in this cohort. However, the role of surgical maneuvers such as tonsil coagulation and arachnoid veil identification and sectioning in the overall role of CM-I surgery remains unclear.

Reduction in Syrinx Size and Severity After Venous Sinus Stenting in a Patient With Pseudotumor Cerebri and Chiari Malformation: Technical Case Report.

Pseudotumor cerebri and Chiari I malformation are found to coexist in a subset of patients. Surgical cranial decompression has been the mainstay of Chiari malformation management, whereas venous sinus stenting has emerged as an effective treatment modality for pseudotumor cerebri. Gaps in our current understanding of cerebrospinal fluid (CSF) circulation and the pathophysiology behind CSF-related disorders present difficulties in the simultaneous management of these conditions. A case is presented of the successful management of both conditions with venous sinus stenting, with sustained improvement in symptoms and structural abnormalities of the Chiari malformation and associated syrinxes. An obese woman in her 20s with a history of occipital headaches who presented with progressively worsening intermittent visual obscurations was diagnosed with pseudotumor cerebri, Chiari I malformation, and associated spinal syrinxes. Magnetic resonance venography demonstrated right transverse sinus narrowing with an elevated pressure gradient. Successful endovascular intervention with venous sinus stenting achieved normalization of the venous sinus pressure gradient and symptomatic improvement. After stenting, the cervical syrinx was observed to have decreased in size, with a decrease in the extent of cerebellar tonsillar herniation and an increase in CSF signal around the cerebellar tonsils. Venous sinus stenting for treatment of concomitant pseudotumor cerebri and Chiari I malformation can be effective in patients demonstrating transverse sinus stenosis.

Surgical treatment of post-traumatic syringomyelia

A retrospective study. To review the outcome of 40 patients with post-traumatic syringomyelia treated with laminectomy and reconstitution of the spinal subarachnoid channels. Birmingham, United Kingdom. A retrospective study of patients undergoing surgery between 1996 and 2007 was carried out. All 40 patients underwent a laminectomy with the creation of a cerebrospinal fluid (CSF) conduit. A total of 18 (1 in 2) patients had long-term stabilization of symptoms after laminectomy and creation of a CSF conduit alone. Later, 17 patients underwent supplementary procedures. Overall, surgical treatment resulted in 27(2 in 3) patients with continued stabilization of symptoms and 13 (1 in 3) experiencing worsening of symptoms in the long term (mean follow-up was 64 months). Radiological review at 6 months revealed a reduction in the size of the syrinx in 21 out of 33 (2 in 3) patients. Laminectomy for syringomyelia, with reconstruction of the spinal CSF channels alone, resulted in a reduction of syrinx size by 6 months, with half of all patients experiencing stabilization of symptoms in the long term. When additional techniques are taken into account, two in three of patients achieved long-term stabilization of symptoms.

Thecal shunt placement for treatment of obstructive primary syringomyelia

The most commonly reported treatment of primary syringomyelia has been laminectomy with duraplasty or direct shunting from the syrinx cavity. Diversion of cerebrospinal fluid (CSF) from the spinal subarachnoid space to peritoneal, atrial, or pleural cavities has been described previously in only a few case reports. Shunting of the CSF from the subarachnoid space rostral to the level of myelographic blockage may reduce the filling force of the syrinx cavity and avoids myelotomy and manipulation of the spinal cord parenchyma. The authors report on 7 patients who underwent thecal shunt placement for primary spinal syringomyelia. This study is a retrospective review of a consecutive series. The authors reviewed the medical records and neuroimaging studies of 7 adult patients with posttraumatic, postsurgical, or postinflammatory syringomyelia treated with thecoperitoneal, thecopleural, or thecoatrial shunt placement at the University of California Los Angeles Medical Center. Myelographic evidence of partial or complete CSF flow obstruction was confirmed in the majority of patients. The mean duration of follow-up was 33 months (range 6-104 months). Six (86%) of 7 patients showed signs of clinical improvement, whereas 1 remained with stable clinical symptoms. Of the 6 patients with available postoperative imaging, each demonstrated a reduction in syrinx size. Three patients (43%) had > or = 1 complication, including shunt-induced cerebellar tonsillar descent in 1 patient and infections in 2. If laminectomy with duraplasty is not possible for the treatment of primary syringomyelia, placement of a thecoperitoneal shunt (or thecal shunt to another extrathecal cavity) should be considered. Although complications occurred in 3 of 7 patients, the complication rate was outweighed by a relatively high symptomatic and imaging improvement rate.

Post-traumatic syringomyelia: a review.

More than a quarter of spinal cord injured patients develop syringes and many of these patients suffer progressive neurological deficits as a result of cyst enlargement. The mechanism of initial cyst formation and progressive enlargement are unknown, although arachnoiditis and persisting cord compression with disturbance of cerebrospinal fluid flow appear to be important aetiological factors. Current treatment options include correction of bony deformity, decompression of the spinal cord, division of adhesions, and shunting. Long-term improvement occurs in fewer than half of patients treated. Imaging evidence of a reduction in syrinx size following treatment does not guarantee symptomatic resolution or even prevention of further neurological loss. A better understanding of the causal mechanisms of syringomyelia is required to develop more effective therapy.

Primary spinal syringomyelia: a personal perspective

In this paper the author summarizes currently available surgical approaches to spinal syringomyelia that is unrelated to Chiari I malformation or hindbrain descent. Primary spinal syringomyelia is most commonly associated with spinal trauma but is also encountered as a sequela to intradural inflammatory processes (infections or chemical), as a delayed response to surgical procedures, and in association with intra- and extradural neoplasms as well as disc protrusions. The advantages of placing a shunt are its technical simplicity and immediate reduction of syrinx size; its major disadvantages are the high rate of failure observed in long-term follow up and the difficulty in applying this technique in septated cysts. Expansion of the subarachnoid space with resection of scars has better long-term results. Patients in whom a syrinx cavity has caused a kyphotic spinal deformity may need to undergo a procedure in which the kyphotic deformity is corrected to expand the subarachnoid space. Cyst obliteration is an experimental approach that cannot be evaluated at the present time.

Quantitative Cine-mode Magnetic Resonance Imaging of Chiari I Malformations

Quantitative cine-mode magnetic resonance imaging of the craniocervical junction was performed in 17 patients with a Chiari I malformation to evaluate cerebrospinal fluid (CSF) dynamics, including 8 patients who underwent surgery. The cine-mode magnetic resonance images of these patients were compared with those of 12 normal pediatric and adult subjects. The craniocervical junction was imaged by 16 cardiac-gated velocity-encoded images arranged in a cine loop. These images allowed the measurement of both the magnitude and direction of CSF velocity. Velocity measurements were made in four regions of interest--the foramen Magendie, the foramen magnum, and ventral and dorsal to the spinal cord at C2--and were plotted in relation to the cardiac cycle to produce a CSF velocity profile. All patients who underwent surgery had the same procedure: a posterior fossa craniectomy with C1 laminectomy, lysis of arachnoid adhesions, and duraplasty. Normal subjects had unobstructed flow around the craniocervical junction: a short period of cranial CSF flow was followed by a sustained period of caudal CSF flow. Patients with tonsillar herniation of more than 5 mm had obstructed CSF flow, decreased CSF velocity, and shorter periods of caudal CSF flow. These patients also had preferential cranial CSF flow as compared with the controls. Postoperatively, there was a substantial increase in both the velocity of CSF flow and in the period of caudal CSF flow in the foramen magnum. The postoperative changes mirrored the velocity profiles of the normal subjects. These changes in CSF velocity and direction correlated with a more normal-appearing foramen magnum, a reduction in syrinx size, and an improvement in symptoms.