Prenatal and postnatal evaluation for syringomyelia in patients with spinal dysraphism

2014 ◽  
Vol 14 (3) ◽  
pp. 316-321 ◽  
Author(s):  
Ben J. Bixenmann ◽  
Beth M. Kline-Fath ◽  
Karin S. Bierbrauer ◽  
Danesh Bansal
Keyword(s):  
Chiari Malformation ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Fetal Mri ◽  
Clinical History ◽  
Craniocervical Junction ◽  
Csf Flow ◽  
Third Trimester ◽  
Flow Disturbances

Object Syringomyelia can be diagnosed in isolation but is more commonly found in the presence of craniocervical junction anomalies or spinal dysraphism. The origin of syringomyelia has been hypothesized to be either congenital or acquired. The purpose of this study was to determine the incidence of syringomyelia within the fetal and postnatal population with neural tube defects (NTDs). Methods A review was performed of the authors' fetal MRI database of pregnancies with imaging between March 2004 and November 2011 for evaluation of an intrauterine anomaly detected via prenatal ultrasonography. Those cases with an NTD were then selected and a chart review was performed of all prenatal and postnatal imaging as well as available clinical history. Results A total of 2362 fetal MRI examinations were performed, and 109 of these were patients with an NTD. Of the 2362 studies reviewed, 2 cases of fetal syringomyelia were identified. Both fetal syrinxes were identified in fetuses with CSF flow disturbances (1 case each of encephalocele and myelomeningocele). Both fetal MRI examinations were performed late in gestation, at 31 and 38 weeks, respectively. The patient with an encephalocele was excluded from the spinal NTD population; therefore a syrinx was identified in 0.08% (2/2362) of the entire population of fetuses who underwent MRI, or 0.9% (1/109) of fetuses with a spinal NTD. Sixty-three of the 109 patients with an NTD had postnatal clinical data available for review. Twenty-nine (46%) of 63 had a syrinx identified during the follow-up period. Of this group, 50 patients had an open NTD and 27 (54%) of 50 developed a syrinx. Among the patients with an open NTD who developed a syrinx, only 7% did not have or develop hydrocephalus, compared with 35% of the patients who did not develop a syrinx (p < 0.05). There were nonsignificantly more frequent shunt revisions among those patients who developed a syrinx, and a syrinx developed in all patients who required surgical Chiari malformation decompression or tethered cord release. The initial identification of a spinal cord syrinx varied greatly between patients, ranging from 38 weeks gestation to greater than 4 years of age. Conclusions These data suggest that syringomyelia is not a congenital embryonic condition. A syrinx was not identified in fetuses who underwent imaging for other intrauterine anomalies. In the population of patients with NTDs who are known to be at high risk for developing syringomyelia, the pathology was only identified in 2 third-trimester fetuses or postnatally, typically in the presence of hydrocephalus, shunt placement, Chiari malformation decompression, or tethered cord release. The study supports the authors' hypothesis that a syrinx is an acquired lesion, most likely due to the effects of abnormal CSF flow.

Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium

2020 ◽  
Vol 26 (1) ◽  
pp. 53-59 ◽  
Author(s):  
Jennifer M. Strahle ◽  
Rukayat Taiwo ◽  
Christine Averill ◽  
James Torner ◽  
Jordan I. Gewirtz ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Craniocervical Junction ◽  
Curve Progression ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Younger Age ◽  
Curve Magnitude ◽  
The Mean

OBJECTIVEIn patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression.METHODSA large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging.RESULTSOf 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis.CONCLUSIONSIn this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.

Chiari malformation: CSF flow dynamics in the craniocervical junction and syrinx

2005 ◽  
Vol 147 (12) ◽  
pp. 1223-1233 ◽  
Author(s):  
D. E. Sakas ◽  
S. I. Korfias ◽  
S. C. Wayte ◽  
D. J. Beale ◽  
K. P. Papapetrou ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Craniocervical Junction ◽  
Flow Dynamics ◽  
Csf Flow

Treatment and prevention of tethered and retethered spinal cord using a Gore-Tex surgical membrane

1994 ◽  
Vol 80 (4) ◽  
pp. 689-693 ◽  
Author(s):  
Hiroshi K. Inoue ◽  
Satoshi Kobayashi ◽  
Katsumi Ohbayashi ◽  
Hideaki Kohga ◽  
Masaru Nakamura
Keyword(s):  
Spinal Cord ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
New Method ◽  
Resonance Imaging ◽  
Imaging Studies ◽  
Content Type ◽  
Follow Up Study ◽  
Treatment And Prevention

✓ The incidence of the tethered cord syndrome after repair of spinal dysraphism is not insignificant. A retethered spinal cord may also develop after an untethering operation. In order to treat and/or prevent the tethered and retethered spinal cord, the authors developed and successfully used a new method in 12 cases. After complete release and reconstruction of the spinal cord, a Gore-Tex surgical membrane was placed over the cord and fixed to the lateral dural surface with stay sutures. During a postoperative follow-up period ranging from 23 months to 7 years, no further neurological deterioration was observed in the 12 patients and magnetic resonance imaging studies showed no adhesion of the spinal cord to the operative site. It is concluded that this simple new method is effective for the treatment and prevention of tethering and/or retethering of the spinal cord, although a longer follow-up study is required.

Minimally invasive foramen magnum durectomy and obexostomy for treatment of craniocervical junction–related syringomyelia in adults: case series and midterm follow-up

2020 ◽  
Vol 33 (2) ◽  
pp. 148-157
Author(s):  
Mauricio Mandel ◽  
Igor Araújo Ferreira da Silva ◽  
Wellingson Paiva ◽  
Yiping Li ◽  
Gary K. Steinberg ◽  
...  
Keyword(s):  
Minimally Invasive ◽  
Chiari Malformation ◽  
Japanese Orthopaedic Association ◽  
Case Series ◽  
Craniocervical Junction ◽  
Foramen Magnum ◽  
Consecutive Series ◽  
Type I ◽  
Duration Of Surgery

OBJECTIVECraniocervical junction–related syringomyelia (CCJS) is the most common form of syringomyelia. Approximately 30% of patients treated with foramen magnum decompression (FMD) will show persistence, recurrence, or progression of the syrinx. The authors present a pilot study with a new minimally invasive surgery technique targeting the pathophysiology of CCJS in adult patients.METHODSThe authors retrospectively analyzed the clinical and radiological features of a consecutive series of patients treated for CCJS. An FMD and FM durectomy were performed through a 1.5- to 2-cm skin incision. Then arachnoid adhesions were cleared, creating a permanent communication from the fourth ventricle to the new paraspinal extradural cavity (obexostomy) and with the spinal subarachnoid space. The hypothesis was that the new CSF pouch acts like a pressure leak, interrupting the CCJS pathogenesis.RESULTSTwenty-four patients (13 female, 21–61 years old) were treated between 2014 and 2018. The etiology of CCJS was Chiari malformation type I (CM-I) in 20 patients (83.3%), Chiari malformation type 0 (CM-0) in 2 patients (8.3%), and CCJ arachnoiditis in 2 patients (8.3%). Two patients underwent reoperations after failed FMD for CM-I at other institutions. No major surgical complication occurred. One patient had postoperative meningitis with no CSF fistula. On postoperative MRI, shrinkage of the syrinx was seen in all patients. No patients experienced recurrence of the CCJS. No patient required a subsequent operation. The mean duration of surgery was 72 ± 11 minutes (mean ± SD), and blood loss was 35–80 ml (mean 51 ml). Follow-up ranged from 12 to 58 months. The average overall improvement in modified Japanese Orthopaedic Association scores was 10% (p < 0.001). The Odom scale showed that 19 patients (79.1%) were satisfied, 4 (16.7%) remained the same, and 1 (4.2%) reported a poor outcome. All patients experienced postoperative improvement in perception of quality of life (p < 0.001).CONCLUSIONSMinimally invasive FM durectomy and obexostomy is a safe and effective treatment for CCJS and for patients who have not responded to other treatment.

Preuntethering and Postuntethering Courses of Syringomyelia Associated With Tethered Spinal Cord

Neurosurgery ◽  
2012 ◽  
Vol 71 (1) ◽  
pp. 23-29 ◽  
Author(s):  
Ji Yeoun Lee ◽  
Ji Hoon Phi ◽  
Jung-Eun Cheon ◽  
Seung-Ki Kim ◽  
In-One Kim ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Transient Increase ◽  
Tethered Spinal Cord ◽  
Additional Surgery ◽  
Number Of Patients ◽  
Before And After ◽  
Index Ratio

Abstract BACKGROUND: There has been controversy regarding the management of syringomyelia associated with tethered spinal cord. Previous reports on the topic have included only a small number of patients, considered open/closed spinal dysraphism together, or had a short follow-up. OBJECTIVE: To review a uniform group of patients with syringomyelia associated with tethered cord and treated mainly by untethering alone. METHODS: Of the 135 patients operated on for closed spinal dysraphism between 2003 and 2008, 33 patients with preoperative syringomyelia were identified. The preoperative/postoperative clinical data and syrinx index (ratio of the syrinx area and the cord area) were retrospectively reviewed. The syrinx index of each patient was plotted as an individual graph to outline the temporal change of the syrinx before and after untethering surgery. RESULTS: Five patients showed symptom progression during the preoperative period, and 4 of the 5 had an additional magnetic resonance imaging before the operation that showed progression of the syringomyelia. Postoperatively, 31 of 32 patients (97%) who underwent postoperative follow-up imaging showed long-term stability or a decrease in the syrinx index. Four symptomatically stable patients showed a transient increase in the syrinx index during the initial postoperative 6 months, which later decreased spontaneously. In 1 patient with retethering, the syrinx index increased 6 months before the onset of new urinary symptoms. CONCLUSION: Untethering alone may be sufficient for the management of syringomyelia associated with tethered cord. A transient increase in the syrinx index during the initial postoperative period may be observed without additional surgery if patients are symptomatically stable.

scholarly journals Syringomyelia in children with closed spinal dysraphism: long-term outcomes after surgical intervention

2020 ◽  
Vol 25 (3) ◽  
pp. 319-325 ◽  
Author(s):  
Amy K. Bruzek ◽  
Jordan Starr ◽  
Hugh J. L. Garton ◽  
Karin M. Muraszko ◽  
Cormac O. Maher ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Clinical Symptoms ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Presenting Symptoms ◽  
Age At Surgery ◽  
Intradural Lipoma ◽  
The Relationship

OBJECTIVEThe nature of the relationship between spinal cord syrinx and tethered cord is not well known. It is unclear if surgical cord untethering results in resolution or improvement of an associated syrinx. The objective of this study was to report the response of spinal cord syrinx to surgical cord untethering.METHODSThe authors retrospectively reviewed all patients with a syrinx and tethered cord who presented to a single institution over an 11-year interval. Patients with open neural tube defects were excluded. Thirty-one patients were identified, 25 of whom had both clinical and imaging follow-up after surgery. Patients were grouped according to etiology of the tethered cord. Clinical outcomes and syrinx characteristics were recorded.RESULTSOf the 25 patients with tethered cord, 68% (n = 17) were male. The average age at presentation was 2.5 years (0–10.1 years) and age at surgery was 3.7 years (range 1 day to 17 years). Etiologies of tethered cord were lipomyelomeningocele (n = 8), thickened/fatty filum (n = 7), intradural lipoma (n = 5), myelocystocele (n = 2), meningocele (n = 2), and diastematomyelia (n = 1). Twenty-three of the patients underwent primary untethering, whereas 2 patients had received untethering previously at another institution. The average syrinx length and width prior to surgery were 4.81 vertebral levels (SD 4.35) and 5.19 mm (SD 2.55 mm), respectively. Conus level ranged from L1 to S3. Patients were followed for an average of 8.4 years (1.35–15.85 years). Overall there was no significant change in syrinx length or width postoperatively; the average syrinx length increased by 0.86 vertebral levels (SD 4.36) and width decreased by 0.72 mm (SD 2.94 mm). Seven of 25 patients had improvement in at least one presenting symptom, including scoliosis, weakness, bowel/bladder dysfunction, and pain. Eight patients had stable presenting symptoms. Six patients were asymptomatic and 5 patients had new or worsening symptoms, which included scoliosis, pain, or sensory changes.CONCLUSIONSAlthough some syrinxes improved after surgery for tethered cord, radiological improvement was not consistent and did not appear to be associated with change in clinical symptoms. The decision to surgically untether a cord should be focused on the clinical symptoms and not the presence of a syrinx alone. Further studies are needed to confirm this finding.

Resolution of syndromic craniosynostosis-associated Chiari malformation Type I without suboccipital decompression after posterior cranial vault release

2012 ◽  
Vol 9 (2) ◽  
pp. 111-115 ◽  
Author(s):  
Michael R. Levitt ◽  
Toba N. Niazi ◽  
Richard A. Hopper ◽  
Richard G. Ellenbogen ◽  
Jeffrey G. Ojemann
Keyword(s):  
Chiari Malformation ◽  
Watchful Waiting ◽  
Cranial Vault ◽  
Type I ◽  
Crouzon Syndrome ◽  
Csf Flow ◽  
Chiari Malformation Type I ◽  
Syndromic Craniosynostosis ◽  
Suboccipital Decompression

Chiari malformation Type I (CM-I) is associated with syndromic and nonsyndromic craniosynostosis in pediatric patients, and the surgical management of CM-I in such cases is controversial. Previous guidelines have recommended simultaneous cranial vault expansion and suboccipital decompression. However, spontaneous resolution of CM-I has been observed, and the combined procedure carries additional surgical risks. The authors report the case of a 6-month-old boy with Crouzon syndrome, CM-I, and a cervical syrinx who underwent posterior cranial vault release without suboccipital decompression. Imaging at the 3-month follow-up visit demonstrated complete resolution of the CM-I, improvement in CSF flow, and reduction in the size of the syrinx. This case suggests that up-front suboccipital decompression may not be necessary in patients with craniosynostosis and CM-I. A strategy of initial cranial vault release, followed by watchful waiting and radiographic surveillance, is proposed.

scholarly journals Syrinx location and size according to etiology: identification of Chiari-associated syrinx

2015 ◽  
Vol 16 (1) ◽  
pp. 21-29 ◽  
Author(s):  
Jennifer Strahle ◽  
Karin M. Muraszko ◽  
Hugh J. L. Garton ◽  
Brandon W. Smith ◽  
Jordan Starr ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Spine ◽  
Chiari Malformation ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Spinal Tumors ◽  
Cerebellar Tonsil ◽  
Type I ◽  
Chiari Malformation Type I ◽  
The Brain

OBJECT Syrinx size and location within the spinal cord may differ based on etiology or associated conditions of the brain and spine. These differences have not been clearly defined. METHODS All patients with a syrinx were identified from 14,118 patients undergoing brain or cervical spine imaging at a single institution over an 11-year interval. Syrinx width, length, and location in the spinal cord were recorded. Patients were grouped according to associated brain and spine conditions including Chiari malformation Type I (CM-I), secondary CM (2°CM), Chiari malformation Type 0 (CM-0), tethered cord, other closed dysraphism, and spinal tumors. Syringes not associated with any known brain or spinal cord condition were considered idiopathic. Syrinx characteristics were compared between groups. RESULTS A total of 271 patients with a syrinx were identified. The most common associated condition was CM-I (occurring in 117 patients [43.2%]), followed by spinal dysraphism (20 [7.4%]), tumor (15 [5.5%]), and tethered cord (13 [4.8%]). Eighty-three patients (30.6%) did not have any associated condition of the brain or spinal cord and their syringes were considered idiopathic. Syringes in patients with CM-I were wide (7.8 ± 3.9 mm) compared with idiopathic syringes (3.9 ± 1.0, p < 0.0001) and those associated with tethered cord (4.2 ± 0.9, p < 0.01). When considering CM-I–associated and idiopathic syringes, the authors found that CM-I–associated syringes were more likely to have their cranial extent in the cervical spine (88%), compared with idiopathic syringes (43%; p < 0.0001). The combination of syrinx width greater than 5 mm and cranial extent in the cervical spine had 99% specificity (95% CI 0.92–0.99) for CM-I–associated syrinx. CONCLUSIONS Syrinx morphology differs according to syrinx etiology. The combination of width greater than 5 mm and cranial extent in the cervical spine is highly specific for CM-I–associated syringes. This may have relevance when determining the clinical significance of syringes in patients with low cerebellar tonsil position.

Prognostic significance of C1–C2 facet malalignment after surgical decompression in adult Chiari malformation type I: a pilot study based on the Chicago Chiari Outcome Scale

2020 ◽  
pp. 1-7
Author(s):  
Michael Lumintang Loe ◽  
Tito Vivas-Buitrago ◽  
Ricardo A. Domingo ◽  
Johan Heemskerk ◽  
Shashwat Tripathi ◽  
...  
Keyword(s):  
Pilot Study ◽  
Chiari Malformation ◽  
Prognostic Significance ◽  
Foramen Magnum ◽  
Bivariate Analysis ◽  
Type I ◽  
Foramen Magnum Decompression ◽  
Swallowing Function ◽  
Chiari Malformation Type I

OBJECTIVEThe authors assessed the prognostic significance of various clinical and radiographic characteristics, including C1–C2 facet malalignment, in terms of surgical outcomes after foramen magnum decompression of adult Chiari malformation type I.METHODSThe electronic medical records of 273 symptomatic patients with Chiari malformation type I who were treated with foramen magnum decompression, C1 laminectomy, and duraplasty at Mayo Clinic were retrospectively reviewed. Preoperative and postoperative Neurological Scoring System scores were compared using the Friedman test. Bivariate analysis was conducted to identify the preoperative variables that correlated with the patient Chicago Chiari Outcome Scale (CCOS) scores. Multiple linear regression analysis was subsequently performed using the variables with p < 0.05 on the bivariate analysis to check for independent associations with the outcome measures. Statistical software SPSS version 25.0 was used for the data analysis. Significance was defined as p < 0.05 for all analyses.RESULTSFifty-two adult patients with preoperative clinical and radiological data and a minimum follow-up of 12 months were included. Motor deficits, syrinx, and C1–C2 facet malalignment were found to have significant negative associations with the CCOS score at the 1- to 3-month follow-up (p < 0.05), while at the 9- to 12-month follow-up only swallowing function and C1–C2 facet malalignment were significantly associated with the CCOS score (p < 0.05). Multivariate analysis showed that syrinx presence and C1–C2 facet malalignment were independently associated with the CCOS score at the 1- to 3-month follow-up. Swallowing function and C1–C2 facet malalignment were found to be independently associated with the CCOS score at the 9- to 12-month follow-up.CONCLUSIONSThe observed results in this pilot study suggest a significant negative correlation between C1–C2 facet malalignment and clinical outcomes evaluated by the CCOS score at 1–3 months and 9–12 months postoperatively. Prospective studies are needed to further validate the prognostic value of C1–C2 facet malalignment and the potential role of atlantoaxial fixation as part of the treatment.

scholarly journals Reappraising the Role of Trans-Sphenoidal Surgery in Prolactin-Secreting Pituitary Tumors

Cancers ◽  
2021 ◽  
Vol 13 (13) ◽  
pp. 3252
Author(s):  
Pier Paolo Mattogno ◽  
Quintino Giorgio D’Alessandris ◽  
Sabrina Chiloiro ◽  
Antonio Bianchi ◽  
Antonella Giampietro ◽  
...  
Keyword(s):  
Pituitary Tumors ◽  
Female Gender ◽  
Clinical History ◽  
Serum Prolactin ◽  
Cure Rate ◽  
Cross Sectional ◽  
Unique Challenge ◽  
Best Management ◽  
Pituitary Diseases

Background: Prolactinomas represent a unique challenge for endocrinologists and neurosurgeons. Considering recent innovations in surgical practice, the authors aimed to investigate the best management for prolactinomas. Methods: A retrospective, cross-sectional and monocentric study was designed. Consecutive patients affected by prolactinomas were enrolled if treated with a first-line treatment with a dopamine agonist (DA) or trans-sphenoidal surgery (TSS). Patients carried giant prolactinomas, and those with a follow-up <12 months were excluded. Results: Two hundred and fifty-nine patients were enrolled. The first treatment was DA for 140 patients and TS for 119 cases. One hundred and forty-six of 249 patients (58.6%) needed a second therapy. The mean follow-up was 102.2 months (12–438 months). Surgery highly impacted on the cure rate—in particular, in females (p = 0.0021) and in microprolactinomas (p = 0.0020). Considering the multivariate analysis, the female gender and surgical treatment in the course of the clinical history were the only independent positive predictors of a cure at the end of 5 years follow-up (p = 0.0016, p = 0.0005). The evaluation of serum prolactin (24 hours after TSS) revealed that 86.4% of patients with postoperative prolactin (PRL) ≤10 ng/mL were cured at the end of the follow-up (p < 0.0001). Conclusions: According to our experience, surgery allows a high cure rate of prolactinomas, particularly in females with microadenoma, with a good safety profile. TSS for prolactinomas should be considered as a concrete option, during the multidisciplinary evaluation, in centers of reference for pituitary diseases.

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