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Long-term outcomes of foramen magnum decompression with duraplasty for Chiari malformation type I in adults: a series of 297 patients

Bo Wang, Chong Wang, Yao-Wu Zhang, Yu-Chao Liang, Wei-Hao Liu, Jun Yang, Yu-Lun Xu, Yong-Zhi Wang, and Wen-Qing Jia

OBJECTIVE

Foramen magnum decompression with duraplasty (FMDD) is one of the most frequently utilized surgical treatments for Chiari malformation type I (CMI) in adults. However, its long-term outcomes remain controversial. The object of this study was to evaluate the long-term outcomes of FMDD in adults with CMI.

METHODS

In total, 297 adults with CMI who had undergone FMDD at the authors’ institution between 2011 and 2020 were included in this retrospective study. Long-term (> 1 year) outcomes were evaluated using the Chicago Chiari Outcome Scale (CCOS), visual analog scale (VAS), and Japanese Orthopaedic Association (JOA) scale.

RESULTS

The median patient age was 44 years (range 18–65 years). The mean clinical follow-up period was 67 months (range 14–123 months). Compared with preoperative conditions, the postoperative syringomyelia regression rate was 91.3% (242/265), and the cerebellar tonsil ascended in 18.2% of patients (54/297), was stable in 64.3% (191/297), and continuously descended in 17.5% (52/297). Long-term clinical follow-up data were acquired from 267 patients. According to the CCOS score, the patient’s condition improved in 62.5% of cases (167/267), was stable in 31.8% (85/267), and worsened in 5.6% (15/267). According to the VAS score, the patient’s condition improved in 59.5% of cases (110/185), remained unchanged in 27.6% (51/185), and worsened in 13.0% (24/185) among the follow-up patients with relevant data. According to the JOA score, the patient’s condition improved in 40.1% of cases (107/267), remained unchanged in 50.2% (134/267), and worsened in 9.7% (26/267). Notably, there was no association between clinical outcomes and syringomyelia regression (p = 0.227) or changes in the cerebellar tonsillar position (p = 0.323).

CONCLUSIONS

FMDD is a simple, safe, and effective surgical procedure for adult CMI that yields significant and sustained improvement in clinical and radiological outcomes. However, clinical improvement does not always correlate with syringomyelia regression and cerebellar tonsillar shift.

In Neurosurgical Focus Volume 54 (2023): Issue 3 (Mar 2023): Management of Chiari Malformation and Craniocervical Anomalies

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Minimally invasive modification of the Goel-Harms atlantoaxial fusion technique: a case series and illustrative guide

Aaron Gelinne, Martin Piazza, and Deb A. Bhowmick

OBJECTIVE

The Goel-Harms atlantoaxial screw fixation technique for the treatment of atlantoaxial instability and unstable odontoid fractures is reliable and reproducible for a variety of anatomies. The drawbacks of the technique are the potential for significant bleeding from the C2 nerve root venous plexus and the risks associated with posterior midline exposure and retraction, such as pain and wound complications. The authors developed a minimally invasive surgical (MIS) modification of the Goel-Harms technique using intra-articular grafting to facilitate placement of percutaneous lateral mass and pars screws with extended tabs for minimally invasive subfascial rod placement. The objective of this study was to present the authors’ first series of 5 patients undergoing minimally invasive modification in comparison with 51 patients undergoing open atlantoaxial fusion.

METHODS

A retrospectiveanalysis of patient comorbid conditions, blood loss, length of surgery, and length of stay was performed on patients undergoing Goel-Harms instrumented fusion (GHIF) for unstable odontoid fractures performed between 2016 and 2021.

RESULTS

Patients undergoing the minimally invasive procedure showed significantly less blood loss than those undergoing the open atlantoaxial fusion procedure, with a median blood loss of 30 ml compared with 150 ml using the open technique (p < 0.01). The patients showed no significant differences in length of stay (2 days for MIS vs 4 days for open atlantoaxial fusion, p = 0.25). There were no significant differences in length of surgery for MIS, but a possible trend toward increased operative duration (234 vs 151 minutes, p = 0.112).

CONCLUSIONS

In this small pilot study, it was shown that MIS-GHIF can be performed with decreased blood loss in atlantoaxial instability and odontoid fractures. This technique may allow for greater and safer application of the procedure in the elderly and infirm.

In Neurosurgical Focus Volume 54 (2023): Issue 3 (Mar 2023): Management of Chiari Malformation and Craniocervical Anomalies

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Prognostic analysis of posterior fossa decompression with or without cerebellar tonsillectomy for Chiari malformation type I: a multicenter retrospective study

Yuankun Cai, Chaobin Wang, Songshan Chai, Guo Li, Tingbao Zhang, Zheng Liu, Dongye Yi, Jincao Chen, Juntao Hu, Kui Liu, and Nanxiang Xiong

OBJECTIVE

The purpose of this study was to compare the prognosis of patients with Chiari malformation type I (CM-I) treated with posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression with resection of tonsils (PFDRT).

METHODS

The clinical data of patients with CM-I treated using these two procedures in three medical centers between January 2016 and June 2021 were retrospectively analyzed and divided into PFDD and PFDRT groups according to the procedures. The Chicago Chiari Outcome Scale (CCOS) was used to score the patients and compare the prognosis of the two groups.

RESULTS

A total of 125 patients with CM-I were included, of whom 90 (72.0%) were in the PFDD group, and 35 (28.0%) were in the PFDRT group. There was no significant difference in the overall essential characteristics of the two groups. Moreover, there was no significant difference in complication rates (3.3% vs 8.6%, p = 0.348), CCOS scores (13.5 ± 1.59 vs 14.0 ± 1.21, p = 0.111), and the probability of poor prognosis (25.6% vs 11.4%, p = 0.096) between the two groups. Nevertheless, a subgroup of patients who had CM-I combined with syringomyelia (SM) revealed higher CCOS scores (13.91 ± 1.12 vs 12.70 ± 1.64, p = 0.002) and a lower probability of poor prognosis (13.0% vs 40.4%, p = 0.028) in the PFDRT than in the PFDD group. Also, SM relief was more significant in patients in the PFDRT compared to the PFDD group. A logistic multifactor regression analysis of poor prognosis in patients with CM-I and SM showed that the PFDRT surgical approach was a protective factor compared to PFDD. Furthermore, by CCOS analysis, it was found that the main advantage of PFDRT in treating patients with CM-I and SM was to improve patients’ nonpain and functionality scores.

CONCLUSIONS

Compared with PFDD, PFDRT is associated with a better prognosis for patients with CM-I and SM and is a protective factor for poor prognosis. Therefore, the authors suggest that PFDRT may be considered for patients with CM-I and SM.

In Neurosurgical Focus Volume 54 (2023): Issue 3 (Mar 2023): Management of Chiari Malformation and Craniocervical Anomalies

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Reappraisal of intradural findings in Chiari malformation type I

Belinda Shao, Daniel Wojcik, Kevin L. Ma, Natalie Amaral-Nieves, Jonathan A. Poggi, Owen P. Leary, and Petra M. Klinge

OBJECTIVE

Management of Chiari malformation type I (CM-I) requires the functional restoration of an obstructed cisterna magna. In posterior fossa decompression with duraplasty (PFDD), various intradural pathologies are suggested to alter CSF flow at the craniocervical junction and require surgical correction. However, reports of the spectrum of intraoperative intradural findings and their nuances are scarce, especially those characterizing rarer findings pertaining to the vascular structures and vascular compression.

METHODS

The authors conducted a retrospective cohort analysis of adults and children who underwent first-time PFDD for CM-I (2011–2021), with and without syringomyelia. The surgical reports and intraoperative videos were reviewed, and the frequency and nature of the intradural observations in regard to the tonsils, arachnoid, and vasculature were analyzed along with the clinical findings and surgical outcomes.

RESULTS

All 180 patients (age range 1–72 years; median [interquartile range] 24 (14–38) years; 37% of patients were children < 21 years of age) exhibited multiple intradural findings, with a median of 7 distinct concurrent observations in each patient. Novel findings not previously reported included posterior inferior communicating artery (PICA) branches compressing the neural elements at the cervicomedullary junction (26.7%). Other common findings included arachnoid adhesions (92.8%), thickening (90.6%), webs at the obex (52.2%), tonsillar gliosis (57.2%), tonsillar hypertrophy (18.3%), adhesions obstructing the foramen of Magendie (FoM) (62.2%), PICA obstruction of the FoM (17.2%), and dural scarring (87.8%). Tonsillar gliosis and intertonsillar adhesions obstructing the FoM were more common in children than adults. Tonsillar gliosis and arachnoid webs were more common among syringomyelia patients. After multivariable adjustment, none of the observed findings were independently associated with syringomyelia, preoperative symptoms, or postoperative improvement. The vast majority of patients improved postoperatively. The complication rate was low: 1.2% of patients required revision PFDD at > 3 years postoperatively, 3.6% experienced other operative complications, and 0% had CSF leaks.

CONCLUSIONS

The diversity of intradural findings and observations revealed in this study suggests that obstructive and compressive structural anomalies may be more common than previously reported among CM-I patients, both those patients with and those without syringomyelia and especially those with obstructive and compressive PICA branches. Although the authors cannot conclude that all these findings are necessarily pathological, further study may determine how they contribute to CM-I pathology and symptomatology in the setting of a compromised cisterna magna.

In Neurosurgical Focus Volume 54 (2023): Issue 3 (Mar 2023): Management of Chiari Malformation and Craniocervical Anomalies

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Risk factors for clinical and radiological worsening following Chiari malformation type I surgery in the pediatric population

Nida Fatima, Dale M. Swift, Eric Y Montgomery, Bradley E. Weprin, Angela V. Price, Brett A. Whittemore, and Bruno P. Braga

OBJECTIVE

Foramen magnum (FM) decompression with or without duraplasty is considered a common treatment strategy for Chiari malformation type I (CM-I). The authors’ objective was to determine a predictive model of risk factors for clinical and radiological worsening after CM-I surgery.

METHODS

A retrospective review of electronic health records was conducted at an academic tertiary care hospital from 2001 to 2019. A multivariable Cox proportional hazards regression model was used to determine the risk factors. The Kaplan-Meier estimate was plotted to delineate outcomes based on FM size. FM was measured as the preoperative distance between the basion and opisthion and dichotomized into < 34 mm and ≥ 34 mm. Syrinx was measured preoperatively and postoperatively in the craniocaudal and anteroposterior directions using a T2-weighted MRI sequence.

RESULTS

A total of 454 patients (231 females [50.9%]) with a median (range) age of 8.0 (0–18) years were included in the study. The median duration of follow-up was 21.0 months (range 3.0–144.0 years). The model suggested that patients with symptoms consisting of occipital/tussive headache (HR 4.05, 95% CI 1.34–12.17, p = 0.01), cranial nerve symptoms (HR 3.46, 95% CI 1.16–10.2, p = 0.02), and brainstem/spinal cord symptoms (HR 3.25, 95% CI 1.01–11.49, p = 0.05) had higher risk, whereas those who underwent arachnoid dissection/adhesion lysis had 75% lower likelihood (HR 0.25, 95% CI 0.10–0.64, p = 0.004) of clinical worsening postoperatively. Similarly, patients with evidence of brainstem/spinal cord symptoms (HR 7.9, 95% CI 2.84–9.50, p = 0.03), scoliosis (HR 1.04, 95% CI 1.01–2.80, p = 0.04), and preoperative syrinx (HR 16.1, 95% CI 1.95–132.7, p = 0.03) had significantly higher likelihood of postoperative worsening of syrinx. Patients with symptoms consisting of occipital/tussive headache (HR 5.44, 95% CI 1.86–15.9, p = 0.002), cranial nerve symptoms (HR 2.80, 95% CI 1.02–7.68, p = 0.04), and nonspecific symptoms (HR 6.70, 95% CI 1.99–22.6, p = 0.002) had significantly higher likelihood, whereas patients with FM ≥ 34 mm and those who underwent arachnoid dissection/adhesion lysis had 73% (HR 0.27, 95% CI 0.08–0.89, p = 0.03) and 70% (HR 0.30, 95% CI 0.12–0.73, p = 0.008) lower likelihood of reoperation, respectively. The Kaplan-Meier curve showed that patients with FM size ≥ 34 mm had significantly better clinical (p = 0.02) and syrinx (p = 0.03) improvement postoperatively when the tonsils were resected.

CONCLUSIONS

These results showed that preoperative and intraoperative factors may help to provide better clinical decision-making for CM-I surgery. Patients with FM size ≥ 34 mm may have better outcomes when the tonsils are resected.

In Neurosurgical Focus Volume 54 (2023): Issue 3 (Mar 2023): Management of Chiari Malformation and Craniocervical Anomalies

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Single-institution comparative analysis for odontoid resection: posterior transaxis versus anterior transnasal approach

İhsan Doğan, Eyüp Bayatli, Halit Anil Eray, Koral Erdogan, Macit Terzi, Savas Hasimoglu, Ozan Tekneci, Suha Beton, Umit Eroglu, Onur Ozgural, Gokmen Kahilogullari, and Yusuf Sukru Caglar

OBJECTIVE

The resection of an upwardly migrated odontoid is most widely performed via an anterior endoscopic endonasal approach after the addition of posterior occipitocervical instrumentation. In patients with craniovertebral junction (CVJ) anomalies like basilar invagination (BI), surgery is usually achieved in two separate stages. However, the authors have recently introduced a novel posterior transaxis approach in which all the therapeutic goals of the surgery can be safely and effectively accomplished in a single-stage procedure. The aim of the current study was to compare the widely used anterior and the recently introduced posterior approaches on the basis of objective clinical results in patients who underwent odontoid resection for BI.

METHODS

Patients with BI who had undergone odontoid resection were retrospectively reviewed in two groups. The first group (n = 7) consisted of patients who underwent anterior odontoidectomy via the standard anterior transnasal route, and the second group (n = 6) included patients in whom the novel transaxis approach was performed. Patient characteristics, neurological conditions, and modified Rankin Scale (mRS) scores at admission were evaluated. Operative time, changes in intraoperative neurophysiological monitoring, blood loss during surgery, odontoid resection rate, postoperative complications, and mortality were compared between the patient groups.

RESULTS

Data were retrospectively reviewed for 13 patients who underwent odontoid resection, posterior CVJ decompression, and occipitocervical instrumentation at the Ankara University School of Medicine Department of Neurosurgery between 2009 and 2022. In the first group (n = 7), patients who underwent anterior odontoidectomy via the standard endonasal route, two serious complications were observed, pneumocephaly and basilar artery injury. In the second group (n = 6), patients in whom the novel transaxis approach was performed, only one complication was observed, occipital plate malposition.

CONCLUSIONS

This study represents the results of what is to the authors’ knowledge the first comparison of a novel approach with a widely used surgical approach to odontoid resection in patients with BI. The preliminary data support the successful utility of the transaxis approach for odontoid resection that meets all the operative therapeutic demands in a single-stage operation. Considering the diminished surgical risks and operative time, the transaxis approach may be regarded as a primary approach for the treatment of BI.

In Neurosurgical Focus Volume 54 (2023): Issue 3 (Mar 2023): Management of Chiari Malformation and Craniocervical Anomalies

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Surgery for basilar invagination with and without Chiari I malformation

Jörg Klekamp

OBJECTIVE

The surgical treatment for Chiari I malformation and basilar invagination has been discussed with great controversy in recent years. This paper presents a treatment algorithm for these disorders based on radiological features, intraoperative findings, and analyses of long-term outcomes.

METHODS

Eight-five operations for 82 patients (mean ± SD age 40 ± 18 years; range 9–75 years) with basilar invagination were evaluated, with a mean follow-up of 57 ± 55 months. Apart from the radiological features and intraoperative findings, findings on neurological examinations before and after surgery were analyzed. Long-term outcomes were evaluated with Kaplan-Meier statistics. All 77 patients with a Chiari I malformation underwent foramen magnum decompression with arachnoid dissection and duraplasty. Patients with ventral compression by the odontoid peg were managed with posterior realignment and C1–2 fusion. Patients without ventral compression did not undergo C1–2 fusion unless radiological or clinical signs of instability were detected.

RESULTS

Thirty-three patients without ventral compression or instability underwent foramen magnum decompression without additional fusion, whereas 52 operations on 49 patients involved posterior fusion at C0–2 or C1–2 after realignment of ventral compression and/or treatment of C1–2 instability. Postoperatively, gait ataxia, swallowing functions, and suboccipital pain improved significantly in both treatment groups. In total, 79% and 73% of patients reported that their condition improved after foramen magnum decompression alone and after fusion with or without foramen magnum decompression, respectively. Progression-free survival rates at 10 years were 83% and 81%, respectively.

CONCLUSIONS

Among the patients with basilar invagination, a subgroup consisting of 40.2% of the included patients underwent successful long-term treatment with foramen magnum decompression alone and without additional fusion. This subgroup was characterized by the absence of a ventral compression and no atlantoaxial dislocation or other signs of craniocervical instability. The remainder of patients underwent C1–2 fusion with posterior realignment of ventral compression if required. In the presence of basilar invagination, Chiari I malformation should be treated with foramen magnum decompression and duraplasty.

In Neurosurgical Focus Volume 54 (2023): Issue 3 (Mar 2023): Management of Chiari Malformation and Craniocervical Anomalies

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Thirty-day outcomes for suboccipital decompression in adults with Chiari malformation type I: a frailty-driven perspective from the American College of Surgeons National Surgical Quality Improvement Program

Alexander J. Kassicieh, Emily M. Estes, Kavelin Rumalla, Syed Faraz Kazim, Rohini G. McKee, Meic H. Schmidt, and Christian A. Bowers

OBJECTIVE

When indicated, patients with symptomatic Chiari malformation type I (CM-I) may benefit from suboccipital decompression (SOD). Although SOD is considered a lower-risk neurosurgical procedure, preoperative risk assessment and careful surgical patient selection remain critical. The objectives of the present study were twofold: 1) describe 30-day SOD outcomes for CM patients with attention to the impact of preoperative frailty and 2) design a predictive model for the primary endpoint of nonhome discharge (NHD).

METHODS

There were 1015 CM-I patients who underwent SOD in the 2011–2020 American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) database, as specified by diagnostic and procedural codes (Current Procedural Terminology code 61343). Descriptive statistics were used to analyze total cohort baseline demographics, preoperative comorbidities, and postoperative outcomes within 30 days of surgery. Univariate cross-tabulation was used to compare baseline demographics and preoperative characteristics across the NHD and home discharge (HD) cohorts. Receiver operating characteristic (ROC) curve analysis was used to assess the discriminative ability of the revised Risk Analysis Index (RAI-rev) on NHD.

RESULTS

The study cohort had a median age of 36 years, and 80.6% of patients were female. Race distribution was categorized as White (69.9%), Black (16.6%), and other groups (13.6%). The most common preoperative comorbidities were active smoking (24.4%), hypertension (19.2%), and diabetes mellitus (4.7%). The primary outcome of NHD occurred in 4.6% of patients (n = 47). Increasing frailty (measured by the RAI-rev) was associated with a stepwise increase in the rate of NHD: 2.3% for RAI-rev scores 0–10, 5.8% for RAI-rev scores 11–15, 7.6% for RAI-rev scores 16–20, 18.2% for RAI-rev scores 21–25, and 77.8% for RAI-rev scores ≥ 26 (p < 0.001). Other preoperative factors associated with NHD included older age, nonelective surgery, diabetes, hypertension, and elevated creatinine (all p < 0.01). The other most common 30-day complications included unplanned readmission (9.3%), unplanned reoperation (5.3%), return to the operating room (5.8%), Clavien-Dindo grade IV (life-threatening) (1.5%), organ space surgical site infection (SSI) (1.5%), superficial SSI (1.4%), and reoperation for a CSF leak (1.1%). Surgical mortality (within 30 days) was extremely rare (1/1015, 0.1%). ROC curve analysis demonstrated that RAI-rev predicted NHD with significant discriminatory accuracy among CM-I patients who received SOD treatment (C-statistic 0.731, 95% CI 0.648–0.814).

CONCLUSIONS

This decade-long analysis of a multicenter surgical registry provides internationally representative, modern rates of 30-day outcomes after suboccipital decompression (with or without duraplasty) for adult CM-I patients. Preoperative frailty assessment with the RAI-rev may help identify higher-risk surgical candidates.

In Neurosurgical Focus Volume 54 (2023): Issue 3 (Mar 2023): Management of Chiari Malformation and Craniocervical Anomalies

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Delayed open treatment of aortic penetration by a thoracic pedicle screw: illustrative case

Hannah A. Levy, Zachariah W. Pinter, Sandra L. Hobson, and Michael J. Yaszemski

BACKGROUND

Iatrogenic aortic injury from pedicle screw malpositioning or anterior prominence in posterior spinal fusion represents a rare but potentially devasting complication. While intraoperative aortic injury is associated with hemodynamic instability, delayed presentations of pedicle screw aortic impingement or violation often present insidiously with pseudoaneurysm or vascular remodeling in clinically asymptomatic patients. Currently, there is a lack of guidance in the field for the recommended surveillance, urgency of operative intervention, and optimal surgical management of delayed pedicle screw aortic injuries.

OBSERVATIONS

The following case study discusses the open treatment of delayed thoracic aortic penetration from an excessively long T12 pedicle screw in an asymptomatic adolescent patient with idiopathic scoliosis. The pedicle screw prominence anteriorly was corrected by burring the screw tip until it was flush with the vertebral body. The associated aortic injury was addressed with open vascular repair via primary anastomosis supplemented with a bovine pericardial patch.

LESSONS

Complete aortic wall penetration from an excessively long thoracic pedicle screw with otherwise stable screw positioning may be addressed most effectively with a single anterior surgical approach for open aortic repair and screw tip burring.

In Journal of Neurosurgery: Case Lessons Volume 5 (2023): Issue 9 (Feb 2023)

Open access

Middle meningeal artery embolization for chronic subdural hematoma in a young patient with refractory thrombocytopenia secondary to leukemia: more evidence for a paradigm shift? Illustrative case

Emalee J. Burrows, Seng Chye Lee, Omar K. Bangash, Timothy J. Phillips, and Sharon Lee

BACKGROUND

Chronic subdural hematoma (CSDH) is one of the most common neurosurgical presentations, with an increasing number now also presenting with concurrent thrombocytopenia. Although middle meningeal artery (MMA) embolization has been considered in elderly patients with high comorbidities, it may permit treatment of CSDH in patients who are at high risk for recurrence or deemed unsuitable for surgical management due to thrombocytopenia.

OBSERVATIONS

A 35-year-old man who had severe thrombocytopenia due to blast cell crisis with chronic myeloid leukemia developed an atraumatic CSDH. The patient developed severe headaches in the hospital while being treated for febrile neutropenia. He remained neurologically intact. MMA embolization was undertaken due to the morbidity and mortality risks associated with surgery and the high risk of recurrence due to severe thrombocytopenia. At 2 months post-procedure the patient was asymptomatic and there was almost complete resolution of the hematoma.

LESSONS

Thrombocytopenia in the presence of a CSDH is becoming increasingly common. This case highlights the particular role of MMA embolization in patients with severe thrombocytopenia where surgery carries high morbidity and increased mortality.

In Journal of Neurosurgery: Case Lessons Volume 5 (2023): Issue 9 (Feb 2023)