Novel Risk Calculator for Suboccipital Decompression for Adult Chiari Malformation

Gorham-Stout disease (GSD) is an intractable disease characterized by massive osteolysis caused by abnormal lymphangiogenesis in bone. In rare cases of GSD, CSF abnormalities develop. The authors present the case of a 19-year-old woman with GSD presenting with orthostatic headache due to intracranial hypotension (5 cm H2O). The clinical course of this case was very unusual. Orthostatic headache was associated with a CSF leak from the thigh after pathological fractures of the femur and pelvis. The chronic CSF leak led to acquired Chiari malformation (CM) with syringomyelia. After an epidural blood patch, her neurological status improved; however, after the complete arrest of the CSF leak from the thigh, she presented with severe nonpostural headache and progressive visual acuity loss with optic papilledema. A ventriculoperitoneal shunt was placed to treat intracranial hypertension (50 cm H2O). Headache improved and optic papilledema decreased after shunt surgery. This case shows that dynamic CSF abnormalities may lead to reversible CM in patients with GSD. Sealing a CSF leak rather than performing suboccipital decompression is recommended for acquired CM resulting from a CSF leak.

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Suboccipital decompression during posterior cranial vault remodeling for selected cases of Chiari malformations associated with craniosynostosis

Journal of Neurosurgery Pediatrics ◽

10.3171/2013.4.peds12463 ◽

2013 ◽

Vol 12 (2) ◽

pp. 166-170 ◽

Cited By ~ 17

Author(s):

William W. Scott ◽

Jeffrey A. Fearon ◽

Dale M. Swift ◽

David J. Sacco

Keyword(s):

Chiari Malformation ◽

Demographic Data ◽

Foramen Magnum ◽

Cranial Vault ◽

Direct Result ◽

Suboccipital Decompression ◽

Combined Technique ◽

Cranial Vault Remodeling ◽

Chiari Malformations

Object The optimal management of Chiari malformations in the setting of craniosynostosis is not well established. In this report the authors describe their outcomes with the combined technique of simultaneous suboccipital decompression (SOD) during posterior cranial vault remodeling (PCVR). Methods A retrospective review was performed of all patients undergoing PCVR and simultaneous SOD. Demographic data, diagnosis, imaging studies, operative intervention, and clinical follow-up were evaluated. Results Thirty-four patients were identified as having undergone a simultaneous PCVR/SOD for Chiari malformation associated with craniosynostosis. Eighty-eight percent of these patients had syndromic, multisutural craniosynostosis, and the remaining patients had unilateral lambdoid craniosynostosis. There were no postoperative complications as a direct result from this combined procedure. Two patients required a subsequent direct approach for decompression of the Chiari malformation. The interval between these subsequent surgeries was 3 years and 19 months. Conclusions Chiari malformations are commonly associated with syndromic, complex craniosynostosis and isolated lambdoid craniosynostosis. In appropriately selected patients, a combined posterior cranial vault enlargement and SOD of the foramen magnum is associated with a low complication rate and appears to be an effective procedure.

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External validation of current prediction systems of improvement after decompression surgery in Chiari malformation type I patients: can we do better?

Journal of Neurosurgery ◽

10.3171/2020.2.jns20181 ◽

2020 ◽

pp. 1-6 ◽

Cited By ~ 1

Author(s):

James Feghali ◽

Yangyiran Xie ◽

Yuxi Chen ◽

Sean Li ◽

Judy Huang

Keyword(s):

Chiari Malformation ◽

Receiver Operating Curve ◽

Symptom Improvement ◽

Type I ◽

Chiari Malformation Type I ◽

Meaningful Improvement ◽

Point Score ◽

Significant Difference ◽

Suboccipital Decompression ◽

Prediction Systems

OBJECTIVEThe Chiari Severity Index (CSI) and points-based algorithm of Thakar et al. are two prognostic tools that have been developed to predict the likelihood of improvement after suboccipital decompression in adult patients with Chiari malformation type I (CM-I). This study aimed to externally validate and critically evaluate these algorithms in the interest of guiding the development of improved prediction systems.METHODSA consecutive cohort of CM-I patients undergoing suboccipital decompression between September 2006 and September 2018 were included. The CSI and Thakar point score were computed for all patients, and associations with improvement were analyzed. The ability of both prediction systems to predict improvement as measured by different Chicago Chiari Outcome Scale (CCOS) cutoffs was assessed using receiver operating curve analysis. Post hoc correlations between the algorithms and different CCOS subcomponents were also assessed.RESULTSThe surgical cohort was composed of 149 adult CM-I patients, of whom 39 (26%) had a syrinx. Most patients experienced improvement after surgery (80% CCOS ≥ 13; 96% CCOS ≥ 11). The proportion of patients improving decreased with increasing CSI, but the results were not statistically significant (p = 0.246). No statistically significant difference in the mean Thakar point score was identified between improved and nonimproved patients using both CCOS cutoffs (p = 0.246 for a cutoff of 13 and p = 0.480 for a cutoff of 11). The CSI had a poor ability in identifying improved patients at a CCOS cutoff of 13 (area under the curve [AUC] 0.582) and 11 (AUC 0.646). The Thakar point score similarly had poor discrimination at a cutoff of 13 (AUC 0.467) and 11 (AUC 0.646). Neither algorithm had significant correlation with any of the CCOS subcomponents except for CSI and nonpain symptom improvement (coefficient = −0.273, p = 0.001).CONCLUSIONSPreviously published algorithms failed to provide prediction value with regard to clinically meaningful improvement following suboccipital decompression in adult CM-I patients. Future models and practical scoring systems are still required to improve the decision-making process.

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Effect of symptomatic pseudomeningocele on improvement in pain, disability, and quality of life following suboccipital decompression for adult Chiari malformation Type I

Journal of Neurosurgery ◽

10.3171/2013.8.jns122106 ◽

2013 ◽

Vol 119 (5) ◽

pp. 1159-1165 ◽

Cited By ~ 14

Author(s):

Scott L. Parker ◽

Saniya S. Godil ◽

Scott L. Zuckerman ◽

Stephen K. Mendenhall ◽

Noel B. Tulipan ◽

...

Keyword(s):

Quality Of Life ◽

Chiari Malformation ◽

Type I ◽

Complication Rates ◽

Chiari Malformation Type I ◽

Long Term Outcome ◽

Pain Disability ◽

Patient Reported ◽

Suboccipital Decompression

Object Suboccipital decompression is a common procedure for patients with Chiari malformation Type I (CMI). Published studies have reported complication rates ranging from 3% to 40%, with pseudomeningocele being one of the most common complications. To date, there are no studies assessing the effect of this complication on long-term outcome. Therefore, the authors set out to assess the effect of symptomatic pseudomeningocele on patient outcomes following suboccipital decompression for CM-I. Methods The study comprised 50 adult patients with CM-I who underwent suboccipital craniectomy and C-1 laminectomy with or without duraplasty. Clinical presentation, radiological studies, operative variables, and complications were assessed for each case. Baseline and 1-year postoperative patient-reported outcomes were assessed to determine improvement in pain, disability, and quality of life. The extent of improvement was compared for patients with and without development of a postoperative symptomatic pseudomeningocele. Results A symptomatic pseudomeningocele developed postoperatively in 9 patients (18%). There was no difference with regard to clinical, radiological, or operative variables for patients with or without a postoperative pseudomeningocele. Patients without a pseudomeningocele had significant improvement in all 9 patient-reported outcome measures assessed. On the other hand, patients with pseudomeningocele only had significant improvement in headache (as measured on the Numeric Rating Scale) and headache-related disability (as measured on the Headache Disability Index) but no improvement in quality of life. Twenty-nine (71%) of 41 patients without a pseudomeningocele reported improvement in health status postoperatively compared with only 3 (33%) of 9 patients with a postoperative pseudomeningocele (p = 0.05). Conclusions Surgical management of CM-I in adults provides significant and sustained improvement in pain, disability, general health, and quality of life. Development of a postoperative symptomatic pseudomeningocele has lingering effects at 1 year, and it significantly diminishes the overall benefit of suboccipital decompression for CM-related symptoms. Further research is needed to accurately predict which patients may benefit from decompression alone without duraplasty.

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Time to Resolution of Symptoms After Suboccipital Decompression with Duraplasty in Children with Chiari Malformation Type I

World Neurosurgery ◽

10.1016/j.wneu.2018.06.073 ◽

2018 ◽

Vol 117 ◽

pp. e544-e551 ◽

Cited By ~ 5

Author(s):

Eveline Teresa Hidalgo ◽

Yosef Dastagirzada ◽

Cordelia Orillac ◽

Svetlana Kvint ◽

Emily North ◽

...

Keyword(s):

Chiari Malformation ◽

Type I ◽

Chiari Malformation Type I ◽

Suboccipital Decompression

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Experience with Arnold-Chiari malformation, 1960 to 1970

Journal of Neurosurgery ◽

10.3171/jns.1976.45.4.0416 ◽

1976 ◽

Vol 45 (4) ◽

pp. 416-422 ◽

Cited By ~ 127

Author(s):

Ruben J. Saez ◽

Burton M. Onofrio ◽

Takehiko Yanagihara

Keyword(s):

Retrospective Study ◽

Neurological Deficit ◽

Chiari Malformation ◽

Prognostic Significance ◽

Cerebellar Dysfunction ◽

Content Type ◽

Arnold Chiari Malformation ◽

Clinical Syndromes ◽

Suboccipital Decompression ◽

Fine Print

✓ A retrospective study of 60 adult patients with Arnold-Chiari malformation revealed that certain presenting clinical syndromes, although not pathognomonic, seemed to have definite prognostic significance. Surgical management by suboccipital decompression led to remarkable and enduring improvement in 65% of patients followed for as long as 14 years. In some patients, however, the initial postoperative benefit tended to fade into an insidious progression of neurological deficit. Despite operation, 18.6% of patients eventually experienced progressive neurological deterioration. Patients who presented with paroxysmal intracranial hypertension or cerebellar dysfunction had the best prognosis. Evidence of central cord involvement was the single most detrimental factor to neurological recovery.

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Suboccipital decompression for Chiari malformation–associated scoliosis: risk factors and time course of deformity progression

Journal of Neurosurgery Pediatrics ◽

10.3171/ped/2008/1/6/456 ◽

2008 ◽

Vol 1 (6) ◽

pp. 456-460 ◽

Cited By ~ 35

Author(s):

Frank J. Attenello ◽

Matthew J. McGirt ◽

April Atiba ◽

Muraya Gathinji ◽

Ghazala Datoo ◽

...

Keyword(s):

Cobb Angle ◽

Spinal Deformity ◽

Chiari Malformation ◽

Time Course ◽

Thoracolumbar Junction ◽

Type I ◽

Chiari Malformation Type I ◽

Severe Scoliosis ◽

Suboccipital Decompression ◽

Deformity Progression

Object Chiari malformation Type I (CM-I) is often associated with scoliosis. It remains unclear which subgroups of patients are most likely to experience progression of spinal deformity after cervicomedullary decompression. The authors' goal was to determine the time frame of curvature progression and assess which patient subgroups are at greatest risk for progression of spinal deformity after surgery. Methods The authors retrospectively reviewed the records of all pediatric patients with significant scoliosis in whom suboccipital decompression was performed to treat for CM-I during a 10-year period at a single academic institution. Clinical, radiological, and operative variables were assessed as independent factors for failure (worsening of scoliosis) by using a univariate regression analysis. Results Twenty-one children (mean age 9 ± 3 years; 4 male) underwent hindbrain decompression for CM-I–associated scoliosis and were followed for a mean of 39 months. All patients harbored a syrinx. Eight patients (38%) experienced improvement in scoliosis curvature, whereas 10 (48%) suffered a progression. Thoracolumbar junction scoliosis (p = 0.04) and failure of the syrinx to improve (p = 0.05) were associated with 5- and 4-fold respective increases in the likelihood of deformity progression. Each increasing degree of preoperative Cobb angle was associated with an 11% increase in the likelihood of scoliotic curve progression (p < 0.05). Conclusions Over one third of patients with CM-I–associated scoliosis will improve after cervicomedullary decompression alone. Cervicomedullary decompression is a good first-line option, particularly in children with concordant posterior fossa symptoms. Patients presenting with more severe scoliosis (increasing Cobb angle) or scoliosis that crosses the thoracolumbar junction may benefit from earlier orthopedic involvement and should be monitored regularly for curvature progression after cervicomedullary decompression. In cases in which there is a failure of the syrinx to show improvement after suboccipital decompression, the patients are also more likely to develop curvature progression.

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Symptomatic Outcome after Bone-only Suboccipital Decompression in Adult Patients with Chiari Type I Malformations in the Absence of Hydromyelia or Hydrocephalus

Journal of Neurological Surgery Part A Central European Neurosurgery ◽

10.1055/s-0037-1599841 ◽

2017 ◽

Vol 78 (04) ◽

pp. 344-349 ◽

Cited By ~ 2

Author(s):

Hazem Abuhusain ◽

Manuri Gunawardena ◽

Tyler Auschwitz ◽

Charles Teo ◽

Nicolas Koechlin

Keyword(s):

Chiari Malformation ◽

Length Of Hospital Stay ◽

Adult Patients ◽

Common Symptom ◽

Type I ◽

Tonsillar Herniation ◽

Adult Type ◽

Duration Of Symptoms ◽

Suboccipital Decompression ◽

Pain Symptoms

Background Type I Chiari malformation presents without an associated hydromyelia in 30 to 70% of cases, yet there is no agreement regarding the optimal surgical treatment for these patients. We review our experience for treating symptomatic adult type I Chiari malformation without hydromyelia using a suboccipital bone decompression of the hindbrain and no duraplasty in 12 adult patients. Outcome was measured according to the Chicago Chiari Outcome Scale (CCOS). Results Nine of 12 patients were female; average age at surgery was 34.4 years (range: 17–67 years). Average duration of symptoms prior to surgery was 9.6 years (2 months–29 years). The most common symptom was head and/or neck pain (11/12 patients). All patients additionally presented with at least one non-pain symptom. Mean degree of tonsillar herniation on magnetic resonance imaging was 6.8 mm (range: 5–12 mm) below McRae's line. Operative time was on average 68 minutes (range: 47–120 minutes). No surgical complications were noted in any patient. Length of hospital stay was 2 days (1 overnight) for all patients. Mean follow-up was 167 weeks (range: 13–378 weeks). CCOS for all patients on average was 14.50 (range: 12–16). Pain symptoms underwent improvement (7/11 [63.6%]) or complete resolution (4/11 [36.4%]) in all affected patients. A shorter duration of preoperative symptoms significantly correlated with a better CCOS (p = 0.03). Degree of tonsillar herniation had no significant effect on CCOS (p = 0.67). Of non-pain symptoms, paresthesias/dysesthesias and visual symptoms improved or resolved completely in all affected patients. No patient experienced a worsening of either pain or non-pain symptoms. Conclusion In the subset of adult patients with a type I Chiari malformation and no associated hydromyelia, a craniectomy without an additional opening of the dura may achieve good overall results according to the CCOS.

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Brainstem hypertrophy, acquired Chiari malformation, syringomyelia, and hydrocephalus: diagnostic dilemma

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.5.peds10380 ◽

2011 ◽

Vol 8 (2) ◽

pp. 184-188 ◽

Cited By ~ 1

Author(s):

Rohan Ramakrishna ◽

Jeffrey C. Mai ◽

Tanya Filardi ◽

Samuel R. Browd ◽

Richard G. Ellenbogen

Keyword(s):

Present Article ◽

Posterior Fossa ◽

Ventriculoperitoneal Shunt ◽

Chiari Malformation ◽

Scar Tissue ◽

Diagnostic Dilemma ◽

Cerebellar Hemorrhage ◽

Csf Shunting ◽

Neural Axis ◽

Suboccipital Decompression

This 18-year-old woman presented with symptoms of right upper-extremity ataxia and imaging evidence of syringomyelia and an acquired Chiari malformation after a previous suboccipital decompression for cerebellar hemorrhage. The patient underwent posterior fossa reexploration to detether any adhesions and release scar tissue in the fourth ventricular outlet. Her symptoms of syringomyelia resolved but she then developed symptoms of lethargy, confusion, and amnesia in addition to ataxia. Repeat neural axis imaging revealed resolution of the syrinx but prominent brainstem hypertrophy. Eventually, the placement of a ventriculoperitoneal shunt resulted in the resolution of both symptoms and brainstem hypertrophy. In the present article, the authors elaborate on this first reported case of a reversible brainstem hypertrophy responsive to CSF shunting.

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