Patients with “benign” Chiari I malformations require surgical decompression at a low rate

2019 ◽  
Vol 23 (4) ◽  
pp. 498-506 ◽  
Author(s):  
Tofey J. Leon ◽  
Elizabeth N. Kuhn ◽  
Anastasia A. Arynchyna ◽  
Burkely P. Smith ◽  
R. Shane Tubbs ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Posterior Fossa ◽  
Multivariate Logistic Regression Analysis ◽  
Large Cohort ◽  
Initial Evaluation ◽  
Published Data ◽  
Posterior Fossa Decompression ◽  
Signal Abnormality ◽  
Chiari I

OBJECTIVEThere are sparse published data on the natural history of “benign” Chiari I malformation (CM-I)—i.e., Chiari with minimal or no symptoms at presentation and no imaging evidence of syrinx, hydrocephalus, or spinal cord signal abnormality. The purpose of this study was to review a large cohort of children with benign CM-I and to determine whether these children become symptomatic and require surgical treatment.METHODSPatients were identified from institutional outpatient records using International Classification of Diseases, 9th Revision, diagnosis codes for CM-I from 1996 to 2016. After review of the medical records, patients were excluded if they 1) did not have a diagnosis of CM-I, 2) were not evaluated by a neurosurgeon, 3) had previously undergone posterior fossa decompression, or 4) had imaging evidence of syringomyelia at their first appointment. To include only patients with benign Chiari (without syrinx or classic Chiari symptoms that could prompt immediate intervention), any patient who underwent decompression within 9 months of initial evaluation was excluded. After a detailed chart review, patients were excluded if they had classical Chiari malformation symptoms at presentation. The authors then determined what changes in the clinical picture prompted surgical treatment. Patients were excluded from the multivariate logistic regression analysis if they had missing data such as race and insurance; however, these patients were included in the overall survival analysis.RESULTSA total of 427 patients were included for analysis with a median follow-up duration of 25.5 months (range 0.17–179.1 months) after initial evaluation. Fifteen patients had surgery at a median time of 21.0 months (range 11.3–139.3 months) after initial evaluation. The most common indications for surgery were tussive headache in 5 (33.3%), syringomyelia in 5 (33.3%), and nontussive headache in 5 (33.3%). Using the Kaplan-Meier method, rate of freedom from posterior fossa decompression was 95.8%, 94.1%, and 93.1% at 3, 5, and 10 years, respectively.CONCLUSIONSAmong a large cohort of patients with benign CM-I, progression of imaging abnormalities or symptoms that warrant surgical treatment is infrequent. Therefore, these patients should be managed conservatively. However, clinical follow-up of such individuals is justified, as there is a low, but nonzero, rate of new symptom or syringomyelia development. Future analyses will determine whether imaging or clinical features present at initial evaluation are associated with progression and future need for treatment.

Worsening or development of syringomyelia following Chiari I decompression

2013 ◽  
Vol 12 (4) ◽  
pp. 351-356 ◽  
Author(s):  
Robert P. Naftel ◽  
R. Shane Tubbs ◽  
Joshua Y. Menendez ◽  
John C. Wellons ◽  
Ian F. Pollack ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Posterior Arch ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Persistent Symptoms ◽  
Chiari I ◽  
Age Range

Object The effects of posterior fossa decompression on Chiari malformation Type I–induced syringomyelia have been well described. However, treatment of worsening syringomyelia after Chiari decompression remains enigmatic. This paper defines patient and clinical characteristics as well as treatment and postoperative radiological and clinical outcomes in patients experiencing this complication. Methods The authors performed a retrospective review of patients at the Children's Hospital of Pittsburgh and Children's of Alabama who developed worsening syringomyelia after Chiari decompression was performed. Results Fourteen children (age range 8 months to 15 years), 7 of whom had preoperative syringomyelia, underwent posterior fossa decompression. Aseptic meningitis (n = 3) and bacterial meningitis (n = 2) complicated 5 cases (4 of these patients were originally treated at outside hospitals). Worsening syringomyelia presented a median of 1.4 years (range 0.2–10.3 years) after the primary decompression. Ten children presented with new, recurrent, or persistent symptoms, and 4 were asymptomatic. Secondary Chiari decompression was performed in 11 of the 14 children. The other 3 children were advised to undergo secondary decompression. A structural cause for each failed primary Chiari decompression (for example, extensive scarring, suture in the obex, arachnoid web, residual posterior arch of C-1, and no duraplasty) was identified at the secondary operation. After secondary decompression, 8 patients' symptoms completely resolved, 1 patient's condition stabilized, and 2 patients remained asymptomatic. Radiologically, 10 of the 11 children had a decrease in the size of their syringes, and 1 child experienced no change (but improved clinically). The median follow-up from initial Chiari decompression was 3.1 years (range 0.8–14.1 years) and from secondary decompression, 1.3 years (range 0.3–4.5 years). No patient underwent syringopleural shunting or other nonposterior fossa treatment for syringomyelia. Conclusions Based on the authors' experience, children with worsening syringomyelia after decompression for Chiari malformation Type I generally have a surgically remediable structural etiology, and secondary exploration and decompression should be considered.

scholarly journals Retrospective study of patients with Chiari: malformation submitted to surgical treatment

2008 ◽  
Vol 66 (2a) ◽  
pp. 184-188 ◽  
Author(s):  
Mário Augusto Taricco ◽  
Luiz Ricardo Santiago Melo
Keyword(s):  
Retrospective Study ◽  
Surgical Treatment ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Posterior Fossa Decompression ◽  
Basilar Impression ◽  
Iii Ventricle ◽  
Complementary Techniques ◽  
Cerebellar Tonsils

The Chiari malformation (CM) is characterized by variable herniation of one or both cerebellar tonsils, associated or not with displacement of the hindbrain structures into the vertebral canal. This is a retrospective study of 29 patients with CM submitted to surgical procedure between 1990 and 2003. There were 14 females and 15 males whose ages ranged from 16 to 65 years. There were seven patients with isolated CM, 12 associated with syringomyelia (SM), three associated with basilar impression (BI) and seven associated with SM and BI. The surgery was based on posterior fossa decompression. In seven patients a catheter was introduced from the subarachnoid space into the III ventricle and five were submitted to tonsillectomy. Twenty-one patients improved, one worsened, one remained unchanged, four missed follow up and two died. We conclude that the best results with CM surgery are obtained by an effective posterior fossa decompression. Those CM cases associated with other abnormalities, such as SM and BI, probably need complementary techniques which will be the theme for new prospective studies.

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 Type I Malformation Presenting as Hemifacial Spasm: Case Report

Neurosurgery ◽  
2005 ◽  
Vol 57 (2) ◽  
pp. E371-E371 ◽  
Author(s):  
Mustafa Efkan Colpan ◽  
Zeki Sekerci
Keyword(s):  
Facial Nerve ◽  
Posterior Fossa ◽  
Hemifacial Spasm ◽  
Chiari I Malformation ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Type I Malformation ◽  
Downward Displacement ◽  
Facial Spasm ◽  
Chiari I

ABSTRACT OBJECTIVE AND IMPORTANCE: We report on a patient with a Chiari I malformation presenting with right hemifacial spasm. Clinicians should consider the downward displacement of the hindbrain as a rare cause of hemifacial spasm in Chiari I malformation. CLINICAL PRESENTATION: An 18-year-old man was admitted with right hemifacial spasm. The results of the neurological examination were normal except for the facial spasm. Magnetic resonance imaging demonstrated a Chiari I malformation without syringomyelia. After surgery, the hemifacial spasm completely resolved. INTERVENTION: Posterior fossa decompression, C1 laminectomy, and duraplasty were performed. CONCLUSION: The hemifacial spasm could be attributed to compression and/or traction of the facial nerve because of downward displacement of the hindbrain in Chiari I malformation. Compression and/or traction might create irritation of the facial nerve that causes hemifacial spasm. Resolution of the hemifacial spasm after posterior fossa decompression could explain the facial nerve irritation in Chiari I malformation. Clinicians should consider Chiari malformation as a cause of hemifacial spasm and posterior fossa decompression as a potential treatment.

Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation Type I in pediatric patients: a meta-analysis

2008 ◽  
Vol 2 (1) ◽  
pp. 42-49 ◽  
Author(s):  
Susan R. Durham ◽  
Kristina Fjeld-Olenec
Keyword(s):  
Cerebrospinal Fluid ◽  
Surgical Treatment ◽  
Clinical Improvement ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Meta Analysis ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Patient Age

Object Surgery for Chiari malformation Type I (CM-I) is one of the most common neurosurgical procedures performed in children, although there is clearly no consensus among practitioners about which surgical method is preferred. The objective of this meta-analysis was to compare the outcome of posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression without duraplasty (PFD) for the treatment of CM-I in children. Methods The authors searched Medline–Ovid, The Cochrane Library, and the conference proceedings of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons (2000–2007) for studies meeting the following inclusion criteria: 1) surgical treatment of CM-I; 2) surgical techniques of PFD and PFDD being reported in a single cohort; and 3) patient age < 18 years. Results Five retrospective and 2 prospective cohort studies involving a total of 582 patients met the criteria for inclusion in the meta-analysis. Of the 582 patients, 316 were treated with PFDD and 266 were treated with PFD alone. Patient age ranged from 6 months to 18 years. Patients undergoing PFDD had a significantly lower reoperation rate (2.1 vs 12.6%, risk ratio [RR] 0.23, 95% confidence interval [CI] 0.08–0.69) and a higher rate of cerebrospinal fluid–related complications (18.5 vs 1.8%, RR 7.64, 95% CI 2.53–23.09) than those undergoing PFD. No significant differences in either clinical improvement (78.6 vs 64.6%, RR 1.23, 95% CI 0.95–1.59) or syringomyelia decrease (87.0 vs 56.3%, RR 1.43, 95% CI 0.91–2.25) were noted between PFDD and PFD. Conclusions Posterior fossa decompression with duraplasty is associated with a lower risk of reoperation than PFD but a greater risk for cerebrospinal fluid–related complications. There was no significant difference between the 2 operative techniques with respect to clinical improvement or decrease in syringomyelia.

Syrinx shunt to posterior fossa cisterns (syringocisternostomy) for bypassing obstructions of upper cervical theca

1992 ◽  
Vol 77 (6) ◽  
pp. 871-874 ◽  
Author(s):  
Thomas H. Milhorat ◽  
Walter D. Johnson ◽  
John I. Miller
Keyword(s):  
Posterior Fossa ◽  
Symptomatic Improvement ◽  
Foramen Magnum ◽  
Content Type ◽  
Results Of Treatment ◽  
Spinal Subarachnoid Space ◽  
Upper Cervical ◽  
Spinal Arachnoiditis ◽  
Chiari I

✓ Syrinx shunts to the spinal subarachnoid space are likely to fail if the cerebrospinal fluid pathways rostral to the syrinx are blocked. To bypass obstructions at or below the level of the foramen magnum, a technique was developed for shunting the syrinx to the posterior fossa cisterns, termed “syringocisternostomy.” Syrinxes were shunted to the cisterna magna in two patients with spinal arachnoiditis and to the cerebellopontine angle cistern in four patients with Chiari I malformations. There was symptomatic improvement and collapse of the syrinx in each case, with no complications or recurrences over a follow-up interval of 14 to 27 months (average 20.3 months). The surgical technique and results of treatment are described.

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