Category: Developmental Malformations

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Posterior Fossa Midline Epidermoid Tumors: Role of the Inferior Medullary Velum and a Proposed New Imaging Classification

Neurosurgery 93:112–119, 2023

Posterior fossa midline epidermoid tumors (PFMETs) include the epidermoid tumors of the cisterna magna (CM) and fourth ventricle (FV).

OBJECTIVE: To report tumor epicenter-based classification of PFMETs and its clinical and surgical implications with outcome. METHODS: On retrospective analysis of operated cases of intracranial epidermoid tumors, 19 (N = 19) patients having tumor epicenter within FV, CM, or both were included. Cerebellopontine and prepontine cistern epidermoid were excluded. Tumor location was decided based on preoperative MRI and intraoperative findings. Major complication was defined as new onset or worsening of cranial nerve (CN) deficit, sensory motor impairment, or tracheostomy.

RESULTS: The mean (±SD) age of the patients was 42.0 ± 11.6 years (range 25-61 years), with no sex predilection (male: female: 1:0.9). The most common symptoms were cerebellar dysfunction, headache, vomiting, and diplopia. Common CNs affected were VII, V, lower cranial nerve, and VI. The PFMETs were classified based on tumor epicenter as type 1 (tumor epicenter in CM, n = 4/21.1%), type 2 (FV, n = 5/26.3%), and type 3 (involved CM and FV, n = 10/52.6%). Type 2 tumors had a higher incidence of raised intracranial pressure and only facial nerve palsy as preoperative CN deficit. Type 1 tumors had the least incidence of postoperative major complications. Type 3 tumors were the largest and had a greater incidence of brainstem adhesion and postoperative complications. The tumor size, duration of symptoms, and patient age were higher in patients with brainstem adhesion (5.3 ± 1.0 cm, 21 ± 16 months, 44.1 ± 9.2 years) as against its absence (4.8 ± 1.3 cm, 11.2 ± 7.3 months, 38.2 ± 11.7 years). Inferior medullary velum and tela choroidea have a critical role in tumorogenesis, tumor extension, and brainstem adhesion.

CONCLUSION: PFMETs can be classified into 3 subtypes based on tumor epicenter having clinical and surgical implications. Less aggressive dissection and near total excision in the presence of brainstem adhesion yield favorable outcomes.

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Exploring the Pathogenesis of Atlanto-Occipital Instability in ChiariMalformationWith Type II Basilar Invagination: A Systematic Morphological Study

Neurosurgery 92:837–853, 2023

Our previous study suggested that atlanto-occipital instability (AOI) is common in patients with type II basilar invagination (II-BI).

OBJECTIVE: To further understand the pathogenesis of AOI in Chiari malformations (CM) and CM + II-BI through systematic measurements of the bone structure surrounding the craniocervical junction.

METHODS: Computed tomography data from 185 adults (80 controls, 63 CM, and 42 CM + II-BI) were collected, and geometric models were established for parameter measurement. Canonical correlation analysis was used to evaluate the morphological and positional relationships of the atlanto-occipital joint (AOJ).

RESULTS: Among the 3 groups, the length and height of the condyle and superior portion of the lateral masses of the atlas (C1-LM) were smallest in CM + II-BI cases; the AOJ had the shallowest depth and the lowest curvature in the same group. AOJs were divided into 3 morphological types: type I, the typical ball-and-socket joint, mainly in the control group (100%); type II, the shallower joint, mainly in the CM group (92.9%); and type III, the abnormal flat-tilt joint, mainly in the CM + II-BI group (89.3%). Kinematic computed tomography revealed AOI in all III-AOJs (100%) and some II-AOJs (1.5%) but not in type I-AOJs (0%). Morphological parameters of the superior portion of C1-LM positively correlated with those of C0 and the clivus and significantly correlated with AOI.

CONCLUSION: Dysplasia of the condyle and superior portion of C1-LM exists in both CM and II-BI cases yet is more obvious in type II-BI. Unstable movement caused by AOJ deformation is another pathogenic factor in patients with CM + II-BI.

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Extended endoscopic transsphenoidal approach for suprasellar craniopharyngiomas

Acta Neurochirurgica (2023) 165:677–683

Craniopharyngiomas are benign sellar lesions. Surgical excision of craniopharyngiomas is difficult because of the surrounding important neurovascular structures. The choice of surgery depends on the histological type, location, hormonal status, and size of the craniopharyngioma, surrounding neurovascular structures, and invasion of the brain parenchyma.

Methods We describe the resection of an adamantinomatous craniopharyngioma using an extended endoscopic endonasal approach and discuss the relevant surgical anatomy, indications, limitations, and possible complications.

Conclusions The extended endoscopic endonasal approach allows successful removal of the craniopharyngioma and poses little risk to surrounding neurovascular structures.

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Spinal Intradural Arachnoid Cysts in Adults

Neurosurgery 92:450–463, 2023

Adult spinal intradural arachnoid cysts are rare pathologic entities with an unclear etiopathogenesis. These lesions can be dichotomized into primary (idiopathic) or secondary (related to inflammation, intradural surgery, or trauma) etiologies. Limited series have depicted optimal management strategies and clinical outcomes.

OBJECTIVE: To illustrate our experience with spinal intradural arachnoid cysts and to present a literature review of surgically treated cysts to elucidate the clinical and anatomic differences between etiologies.

METHODS: Institutional review revealed 29 patients. Various data were extracted from the medical record. Initial and follow-up symptomatologies of the surgical cohort were compared. The literature review included case series describing cysts managed surgically.

RESULTS: From patients treated surgically at our institution (22), there was a significant reduction in thoracic back pain postoperatively (P = .034). A literature review yielded 271 additional cases. Overall, primary and secondary lesions accounted for 254 and 39 cases, respectively. Cysts of secondary origin were more likely localized ventral to the spinal cord (P = .013). The rate of symptomatic improvement after surgical intervention for primary cysts was more than double than that of secondary cysts (P < .001). Compared with primary etiologies, the rates of radiographic progression (P = .032) and repeat surgery (P = .041) were each more than double for secondary cysts.

CONCLUSION: Surgical intervention for spinal intradural arachnoid cysts improves thoracic back pain. The literature supports surgical intervention for symptomatic primary spinal intradural arachnoid cysts with improved clinical outcomes. Surgery should be cautiously considered for secondary cysts given worse outcomes.

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Pineal cyst: results of long-term MRI surveillance and analysis of growth and shrinkage rates

J Neurosurg 138:113–119, 2023

OBJECTIVE Pineal cyst (PC) is a relatively common true cyst in the pineal gland. Its long-term natural course remains ill defined. This study aims to evaluate the long-term natural history of PC and examine MRI risk factors for cyst growth and shrinkage to help better define which patients might benefit from surgical intervention.

METHODS The records and MRI of 409 consecutive patients with PC were retrospectively examined (nonsurgical cohort). Cyst growth and shrinkage were defined as a ≥ 2-mm increase and decrease in cyst diameter in any direction, respectively. In addition to size, MRI signal intensity ratios were analyzed.

RESULTS The median radiological follow-up period was 10.7 years (interquartile range [IQR] 6.4–14.3 years). The median change in maximal diameter was −0.6 mm (IQR −1.5 to 1.3 mm). During the observation period, cyst growth was confirmed in 21 patients (5.1%). Multivariate logistic regression analysis revealed that only age (odds ratio [OR] 0.96, 95% confidence interval [CI] 0.93–0.99, p < 0.01) was significantly associated with cyst growth. No patient required resection during the observation period. Cyst shrinkage was confirmed in 57 patients (13.9%). Multivariate analysis revealed that maximal diameter (OR 1.22, 95% CI 1.12–1.35, p < 0.01) and cyst CSF T2 signal intensity ratio (OR 9.06, 95% CI 1.38–6.62 × 101, p = 0.02) were significantly associated with cyst shrinkage.

CONCLUSIONS Only 5% of PCs, mainly in patients younger than 50 years of age, have the potential to grow, while cyst shrinkage is more likely to occur across all age groups. Younger age is associated with cyst growth, while larger diameter and higher signal intensity on T2-weighted imaging are associated with shrinkage. Surgery is rarely needed for PCs, despite the possibility of a certain degree of growth.

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Dura Management Strategies in the Surgical Treatment of Adult Chiari Type I Malformation

Operative Neurosurgery 23:304–311, 2022

Symptomatic Chiari I malformation is treated with suboccipital decompression and C1 laminectomy. However, whether the dura should be opened (durotomy) or enlarged with a graft (duraplasty) remains unclear.

OBJECTIVE: To compare outcomes in adult Chiari I malformation patients treated with duraplasty, durotomy, or without dural opening (“mini-decompression”).

METHODS: A retrospective, multicenter, population-based cohort study was performed of all adult patients surgically treated for a Chiari I malformation at 3 regional neurosurgical centers between 2005 and 2017. Three different dura management strategies were favored by the participating hospitals, with data stratified accordingly. The primary outcome was measured using the Chicago Chiari Outcome Scale (CCOS), dichotomized into favorable (CCOS ≥13) or unfavorable (CCOS ≤12). Propensity score matching was used to adjust for potential confounders in outcome comparisons.

RESULTS: In total, 318 patients were included, of whom 52% were treated with duraplasty, 37% with durotomy, and 11% with mini-decompression. In total, 285 (90%) showed a favorable surgical outcome (CCOS ≥13). Duraplasty was associated with more favorable CCOS and shorter hospital stay compared with durotomy, both in unadjusted (93% vs 84%. P = .018 and 6.0 vs 8.0 days, P < .001) and adjusted analyses (92% vs 84%, P = .044 and 6.0 vs 8.0 days, P < .001). Mini-decompression was excluded from the adjusted analyses because of its small sample size.

CONCLUSION: In this study of adult Chiari I malformation, posterior fossa decompression with duraplasty was associated with more favorable postoperative outcome, as determined by the CCOS, compared with posterior fossa decompression with durotomy alone.

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Transseptal interforniceal endoscopic removal of superiorly recessed colloid cysts

J Neurosurg 137:813–819, 2022

Transforaminal endoscopic colloid cyst resection is well described. However, some anatomical colloid cyst variants may warrant a modified approach. Rarely, colloid cysts separate the forniceal columns and grow superiorly within the leaflets of the septum pellucidum. Thus, the authors’ goal was to characterize the imaging features, clinical presentation, surgical strategy, and outcomes of patients with this superiorly recessed colloid cyst variant.

METHODS A retrospective evaluation of patients who underwent endoscopic resection of colloid cysts from 1999 to 2020 was performed. The patients were dichotomized depending on whether the cyst was located predominately below the forniceal columns or was superiorly recessed (forniceal column separation with variable intraseptal extension). This comparative cohort study focused on clinical presentation, imaging features, operative technique, and patient outcome.

RESULTS In total, 182 patients were identified. Seventeen patients had colloid cysts that were defined as superiorly recessed and underwent transseptal interforniceal removal, and 165 patients underwent a standard transforaminal approach. Patients had similar demographic characteristics. However, transseptal cysts were on average larger (17.8 mm vs 11.4 mm, p < 0.0001), and these patients had a greater frontal-occipital horn ratio (0.45 vs 0.41, p = 0.012). They were also more likely to have undergone a previous resection (p = 0.02). The two cohorts had similar surgical outcomes, with no differences in extent of resection, recurrence, or complications.

CONCLUSIONS Superiorly recessed intraseptal colloid cysts are larger and tend to splay the bodies of the fornix, thus requiring a parasagittal transseptal interforniceal endoscopic approach. This achieves complete removal with comparatively negligible morbidity or rare recurrence (5.9%).

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Surgical management of cerebellopontine angle epidermoid cysts: an institutional experience of 10 years

BRITISH JOURNAL OF NEUROSURGERY 2022, VOL. 36, NO. 2, 203–212

Cerebellopontine angle (CPA) epidermoids, although of benign nature, are of considerable neurosurgical interest because of their close proximity and adherence to the cranial nerves and brain stem. In this paper, we describe our experience and attempt to correlate the final outcomes with the extent of surgical removal. The main objectives were to study various modes of surgical management of CPA epidermoids with regard to removal and preservation of the cranial nerves and also to evaluate the role of endoscopic assisted microsurgical excision thereby minimizing recurrences. This case series is one of the largest series reported so far worldwide.

Materials and methods: From 2006 to 2016, 139 patients with CPA epidermoids were operated at Grant Medical College and J. J. Hospital, Mumbai. All patients underwent detailed magnetic resonance imaging (MRI) of brain. Lesions were classified according Rogelio Revuelta-Gutierrez et al. with respect to their anatomic extent: grade I- within the boundaries of the CPA, grade II- extension to the suprasellar and perimesencephalic cisterns, and grade III-parasellar and temporomesial region involvement. Retrosigmoidal and subtemporal approaches were taken to excise the lesions. Endoscopic assisted microsurgical excision was done in cases with extensions beyond the CPA. Patient follow-up was based on outpatient repeated brain MRI studies.

Results: The mean duration of symptoms before surgery was 42 months (range, 2 months to 6 years). The mean follow-up period was 27 months (range, 2–60 months). The main presenting symptom was headache in 69% (96/139) of the cases and trigeminal neuralgia in 30% cases was the second most common cause of consultation. Seventy-five percent of patients had some degree of cranial nerve (CN) involvement. Retrosigmoid approach was taken in 92% patients and 7 patients with supratentorial extension were operated by combined retrosigmoidal and subtemporal approach. Endoscopic assisted microsurgical excision was done in 40% cases. Use of angled views by an endoscope helped to excise residual tumor in 47 (83%) patients. Complete excision was achieved in 67% of cases. In 33% patients, small capsular remnants could not be removed completely because of their adherence to vessels, brainstem and cranial nerves. Compared with their preoperative clinical status, 74% improved and 20% had persistent cranial nerve deficits in the first year of follow up.

Conclusions: Epidermoid cysts are challenging entities in current neurosurgery practice due to tumor adhesions to neurovascular structures. Meticulous surgical technique with the aid of neurophysiological monitoring is crucial to achieve safe and effective total or subtotal removal of these lesions. A conservative approach is indicated for patients in whom the fragments of capsule is adhered closely to blood vessels, nerves, or the brainstem, in order to avoid risk of serious neurological deficits related to an inadvertent damage of these structures. Use of angled views by endoscope at the conclusion of the surgery may assure the surgeon of total removal of the tumor.

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Management of Chiari type I malformation: a retrospective analysis of a series of 91 children treated surgically

Acta Neurochirurgica (2021) 163:3065–3073

The diagnosis of Chiari I malformation, its symptomatology, and the results of its surgical management are still discussed. We report a pediatric series of CMI without associated skull base malformations or cerebellar growth anomalies operated between 2001 and 2018.

Material and methods Ninety-one children out of 146 surgically treated cases have been included in the study. Age at surgery ranged from 5 months to 17 years clinical data, and complementary examinations leading to the surgical indication have been analyzed together with the surgical outcomes. The average follow-up duration was of 4 years. The occipito-cervical decompression with duraplasty without opening the arachnoid was the procedure of election. Three quarters of patients presented with headaches, 12% with cerebellar syndrome, 13% with vertigo, 26% with nausea or vomiting, 24% with sensorimotor deficits, 11% with cranial nerve deficits, and 29% with other symptoms. Eighteen percent of patients suffered from scoliosis, 47% had an associated syrinx and 16% a ventricular dilation.

Results After the treatment, the clinical symptomatology improved in about three-quarters of the patients: headache (69.4%), nausea or vomiting (66.7%), sensorimotor deficits (55.6%), and other symptoms (78.3%). Syringomyelic cavities diminished partially in size or disappeared in 58.3% of patients, remained stable in 29.2%, and worsened in 12.5%. Only one-third of children with preoperative scoliosis benefited from the surgical treatment. No clinical signs or symptoms were found to be reliable predictors of surgical success, neither the extent of the cerebellar tonsil descent.

Conclusion Occipito-cervical decompression allows to improve the clinical condition in the majority of children with symptomatic CMI in the absence of associated cervico-spinal junction alterations, craniosynostosis, or cerebellar growth anomalies. No clinical signs or symptoms neither radiological criterion appear to be a specific finding for the surgical indication.

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Posterior cranial fossa and cervical spine morphometric abnormalities in symptomatic Chiari type 0 and Chiari type 1 malformation patients with and without syringomyelia

Acta Neurochirurgica (2021) 163:3051–3064

To better understand how anatomical features of Chiari malformation type 0 (CM0) result in the manifestation of Chiari malformation type 1 (CM1) signs and symptoms, we conducted a morphometric study of the posterior cranial fossa (PCF) and cervical canal in patients with CM1 and CM0.

Methods This retrospective study had a STROBE design and included 120 adult patients with MRI evidence of a small PCF (SPCF), typical clinical symptoms of CM1, and a diagnosis of CM1, CM0, or SPCF-TH0-only (SPCF with cerebellar ectopia less than 2 mm and without syringomyelia). Patients were divided by MRI findings into 4 groups: SPCF-TH0-only, SPCF-TH0-syr (CM0 with SPCF and syringomyelia), SPCF-CM1-only (SPCF with cerebellar ectopia 5 mm or more without syringomyelia), and SPCF-CM1-syr (CM1 with syringomyelia). Neurological examination data and MRI parameters were analyzed.

Results All patient cohorts had morphometric evidence of a small, flattened, and overcrowded PCF. The PCF phenotype of the SPCF-TH0-only group differed from that of other CM cohorts in that the length of clivus and supraocciput and the height of the PF were longer, the upper CSF spaces of PCF were taller, and the area of the foramen magnum was smaller. The SPCF-TH0 groups had a more significant narrowing of the superior cervical canal and a smaller decrease in PCF height than the SPCF-CM1 groups.

Conclusions Patients with SPCF-TH0 with and without syringomyelia developed Chiari 1 symptoms and signs. Patients with SPCF-TH0-syr (Chiari 0) had more constriction of their CSF pathways in and around the foramen magnum than patients with SPCF-TH0-only.

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Tethered spinal cord syndrome in adults in the MRI era: recognition, pathology, and long-term objective outcomes

J Neurosurg Spine 34:942–954, 2021

Tethered cord syndrome (TCS) has been well described in pediatric patients. Many recent reports of TCS in adult patients have grouped retethering patients with newly diagnosed ones without separately analyzing each entity and outcome. The authors reviewed their experience of newly diagnosed adult TCS patients to identify and explore TCS misdiagnosis, recognition, subtype pathology, and individual objective outcomes.

METHODS This study included 24 adult patients (20 female and 4 male) who fit the criteria of being newly diagnosed and aged 20 years and older (age range 20–77 years). Preexisting dermal sinus was present in 6 patients, hypertrichosis in 5, skin tag/cleft/dimple and fatty subcutaneous masses in 5, scoliosis in 2, and neurological abnormalities in 4 patients. The pathology consisted of TCS with taut filum in 8 patients, conus lipoma with TCS in 7, diastematomyelia in 7, and cervical cord tethering in 2 patients. Of the 24 study patients, nondermatomal low-back or perineal pain occurred in 19 patients, bladder dysfunction in 21, and motor, sensory, and reflex abnormalities in 21 patients. Aggravating factors were repeated stretching, multiple pregnancies, heavy lifting, and repeated bending. Urological evaluation included bladder capacity, emptying, postvoid residuals, detrusor function, pelvic floor electromyography (EMG), bladder sensitivity, and sphincter EMG, which were repeated at 6 months and 1 year postoperatively. The follow-up was 1 to 30 years. Detailed postoperative neurological findings and separate patient outcome evaluations were recorded. Four of the 24 patients did not have an operation.

RESULTS Resolution of pain occurred in 16 of the 19 patients reporting low-back or perineal pain. Motor and sensory complaints resolved in 17 of 20 patients. Regarding bladder dysfunction, in the 20 patients with available data, bladder function returned to normal in 12 patients, improved in 3 patients, and was unchanged in 5 patients. If the symptom duration was less than 6–8 months, there was recovery of all parameters of pain, bladder dysfunction, and neurological deficit, and recovery from hyperreflexia matched that from neurological deficit. Fifteen patients were employed preoperatively and returned to work, and an additional 3 others who were unable to work preoperatively were able to do so postoperatively.

CONCLUSIONS Most adults with newly diagnosed TCS have unrecognized neurocutaneous abnormalities and neurological deficits. The triad of nondermatomal sacral or perineal pain, bladder dysfunction, and neurological deficit should not be confused with hip or degenerative lumbosacral disease. Addressing the primary pathology often leads to successful results.

 

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Posterior Vertebral Column Subtraction Osteotomy for Recurrent Tethered Cord Syndrome

Neurosurgery 88:637–647, 202

Few have explored the safety and efficacy of posterior vertebral column subtraction osteotomy (PVCSO) to treat tethered cord syndrome (TCS).

OBJECTIVE: To evaluate surgical outcomes after PVCSO in adults with TCS caused by lipomyelomeningocele, who had undergone a previous detethering procedure(s) that ultimately failed.

METHODS: This is a multicenter, retrospective analysis of a prospectively collected cohort. Patients were prospectively enrolled and treated with PVCSO at 2 institutions between January 1, 2011 and December 31, 2018. Inclusion criteria were age ≥18 yr, TCS caused by lipomyelomeningocele, previous detethering surgery, and recurrent symptom progression of less than 2-yr duration. All patients undergoing surgerywith a 1-yr minimum follow-up were evaluated.

RESULTS: A total of 20 patients (mean age: 36 yr; sex: 15F/5M) met inclusion criteria and were evaluated. At follow-up (mean: 23.3±7.4 mo), symptomatic improvement/resolution was seen in 93% of patients with leg pain, 84% in back pain, 80% in sensory abnormalities, 80% in motor deficits, 55% in bowel incontinence, and 50% in urinary incontinence. Oswestry Disability Index improved froma preoperative mean of 57.7 to 36.6 at last followup (P < .01). Mean spinal column height reduction was 23.4 ± 2.7 mm. Four complications occurred: intraoperative durotomy (no reoperation), wound infection, instrumentation failure requiring revision, and new sensory abnormality.

CONCLUSION: This is the largest study to date assessing the safety and efficacy of PVCSO in adults with TCS caused by lipomyelomeningocele and prior failed detethering. We found PVCSO to be an excellent extradural approach that may afford definitive treatment in this particularly challenging population.

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Histological and intraoperative evaluations of the arachnoid in patients with Chiari I malformation

Acta Neurochirurgica (2021) 163:219–225

Arachnoid dissection for decompression of Chiari I malformation is controversial. Whether arachnoid changes have an impact on the clinical course is not established. This paper documents the histological spectrum of arachnoid changes and evaluates correlations with preoperative, intraoperative, and postoperative data.

Method Arachnoid samples of 162 consecutive foramen magnum decompressions from 2006 to 2016 were studied. Arachnoid thickness and degrees of fibrosis and cellularity were determined with the examiner blinded for clinical data. Based on 145 first time decompressions, a histological classification for arachnoid features was developed.

Results The arachnoid was thicker in secondary compared with primary decompressions (176.1 ± 158.2 μm vs. 35.9 ± 43.5 μm; p = 0.0026) and in adults compared to children (37.3 ± 45.3 μm vs. 21.8 ± 7.7 μm; p = 0.0007). In primary decompressions, arachnoid thickness, degrees of fibrosis, and cellularity followed a normal distribution with all features shifted significantly to higher grades in secondary decompressions. The histological classification correlatedwith the preoperative severity of gait ataxia, motor weakness, and sensory deficits, whereas it had no predictive power for postoperative short- or long-term results. By comparison, the intraoperative evaluation of arachnoid changes accounting for relationships between arachnoid and surrounding tissues showed higher correlations with preoperative symptoms and had significant predictive power for postoperative short- and long-term results.

Conclusions Histological changes of the arachnoid correlate with preoperative symptoms. Relationships between arachnoid and surrounding tissues show even higher correlations with predictive power for short- and long-term outcomes. These findings suggest a pathophysiological role for the arachnoid in Chiari I malformation.

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Syringomyelia Resolution Following Chiari Surgery: A Novel Scale for Communication and Research

Neurosurgery 88( 1) 2021: 131–139

The pathophysiological connection between Chiari malformation and syringomyelia is accepted. Debate remains, however, how can we best define changes in syringomyelia following surgery.

OBJECTIVE: To introduce a grading system focusing on syrinx reduction based on routinely and reproducible radiological information, and provide a suggestion of the application of this scale for prediction of patient’s prognoses.

METHODS: Data from 48 patients with Chiari malformation and syringomyelia were compiled. We calculated syrinx cross-sectional area by approximating an ellipse in the largest axial plane. We compared the percentage of reduction or enlargement following surgery. The percentage change was grouped into four grades: Grade 0 = Increasing size, grade I ≤ 50% reduction, grade II = 50% to 90% reduction, grade III ≥ 90% reduction.

RESULTS: A total of 89.6% of patients had syrinx improvement after surgery. A total of 5 patients were grade 0, 14 were grade I, 20 patients were grade II, and 9 patients met criteria for grade III. The mean postoperative syrinx area was 24.1 mm2 (0-169 mm2) with a mean syrinx reduction of 62.7%.

CONCLUSION: Radiological improvement of syringomyelia can be mathematically defined and standardized to assist in communication in outcome-based trials. Radiological resolution is expected most patients.

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Chiari I malformation and pregnancy: a comprehensive review of the literature to address common questions and to guide management

Acta Neurochirurgica (2020) 162:1565–1573

The optimal management of Chiari I malformation during pregnancy remains uncertain. Labor contractions, which increase intracranial pressure, and neuraxial anesthesia both carry the theoretical risk of brainstem herniation given the altered CSF dynamics inherent to the condition. Mode of delivery and planned anesthesia, therefore, require forethought to avoid potentially lifethreatening complications. Since the assumed potential risks are significant, we seek to systematically review published literature regarding Chiari I malformation in pregnancy and, therefore, to establish a best practice recommendation based on available evidence.

Methods The English-language literature was systematically reviewed from 1991 to 2018 according to PRISMA guidelines to assess all pregnancies reported in patients with Chiari I malformation. After analysis, a total of 34 patients and 35 deliveries were included in this investigation. Additionally, a single case from our institutional experience is presented for illustrative purposes but not included in the statistical analysis.

Results No instances of brain herniation during pregnancy in patients with Chiari I malformation were reported. Cesarean deliveries (51%) and vaginal deliveries (49%) under neuraxial blockade and general anesthesia were both reported as safe and suitable modes of delivery. Across all publications, only one patient experienced a worsening of neurologic symptoms, which was only later discovered to be the result of a previously undiagnosed Chiari I malformation. Several patients underwent decompressive suboccipital craniectomy to treat the Chiari I malformation during the preconception period (31%), during pregnancy (3%), and after birth (6%). Specific data regarding maternal management were not reported for a large number (21) of these patients (60%). Aside from one abortion in our own institutional experience, there was no report of any therapeutic abortion or of adverse fetal outcome.

Conclusions Although devastating maternal complications are frequently feared, very few adverse outcomes have ever been reported in pregnant patients with a Chiari I malformation. The available evidence is, however, rather limited. Based on our survey of available data, we recommend vaginal delivery under neuraxial blockade for truly asymptomatic patients. Furthermore, based on our own experience and physiological conceptual considerations, we recommend limiting maternal Valsalva efforts either via Cesarean delivery under regional or general anesthesia or by choosing assisted vaginal delivery under neuraxial blockade. There is no compelling reason to offer suboccipital decompression for Chiari I malformation during pregnancy. For patients with significant neurologic symptoms prior to conception, decompression prior to pregnancy should be considered.

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Chiari I malformation—neuropsychological functions and quality of life

Acta Neurochirurgica (2020) 162:1575–1582

Objective To assess the neuropsychological (NP) functioning and quality of life (QOL) before and 3 months after surgery on adults with Chiari I malformation (CMI).

Patients and methods All adult patients who had been diagnosed with CMI were invited to participate. Those who participated were assessed using a Hospital Anxiety and Depression scale (HAD) and NP examinations. Their QOL was assessed using the self-reported life satisfaction checklist, LiSat-11 and the five-dimensional EuroQol measurement of health outcome, EQ-5D-5L. All assessments were carried out both before and 3 months after surgery was performed. Demographic data and comorbidities were also registered.

Results Of the 11 patientswho underwentNP assessment, the majority demonstrated cognitive functioning within the normal range. However, postoperatively, their performance in verbal learning, psychomotor speed, colour naming speed and the ability to manage interference through response selection and inhibition (aspects of executive functioning) was significantly improved. Thirteen patients completed QOL assessments. When LiSat-11 item domains were compared with those of healthy subjects, patients reported a lower level of satisfaction with their life quality both before and after surgery. However, the EQ-5D-5L measurements, i.e., the descriptive system and the visual analogue, indicated that their QOL of life was significantly improved after surgery.

Conclusion There is scarcely any literature documenting effects of surgery on the QOL of CMI patients. The study we present here breaks new ground by comparing pre- and postoperative NP functions in CMI. We also examine the value of surgery for improving both NP functions and QOL in CMI.

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Operative findings and surgical outcomes in patients undergoing Chiari 1 malformation decompression: relationship to the extent of tonsillar ectopia

Acta Neurochirurgica (2020) 162:1539–1547

The diagnosis of Chiari 1 malformation is based on the extent of tonsillar ectopia.

Objective To examine the relationship between the extent of tonsillar ectopia and the intra-operative findings and clinical outcome following Chiari decompression surgery.

Methods Patients were divided into four groups depending on the position of the cerebellar tonsil (T): group 1: 0 < T < 3; group 2: 3 ≤ T ≤ 5; group 3: 5 < T ≤ 10; and group 4: T > 10. Intra-operative observations were recorded with regard to compression of the brain stem by posterior inferior cerebellar artery (pica), neuroma formation along the first cervical (C1), and accessory spinal nerves (XI), and pallor of the cerebellar tonsils. Brain stem auditory evoked potentials, (BAEP), were monitored in each case. One hundred sixty-eight patients accrued between 2009 and 2013 agreed to participate in an outcome study to determine the effectiveness of foramen magnum decompression. Findings across the four groups were compared using one-way ANOVA. Observed differences were further subjected to paired analysis. Intra-group comparisons were made using the paired t test. A P value less than 0.05 was considered statistically significant.

Results There were 98 patients in group 1, 147 patients in group 2, 180 patients in group 3, and 63 patients in group 4. The mean extent of tonsillar ectopia was 0.4, 4.0, 7.1, and 14.3 mm in the four groups respectively. The prevalence of tonsillar pallor was greatest in group 4. Otherwise, there was no difference observed in the operative findings. A reduction of > 0.1 msec in the wave III–wave V latency of the BAEP was noted in all four groups with equal frequency. One hundred ten patients complied with at least 6 months follow-up. There was no difference in the prevalence of symptoms between the four groups at the time of initial evaluation and at 6 weeks and 6 months following surgery. There was a statistically significant reduction in the intensity of individual symptoms 6 months following surgery regardless of the extent of tonsil ectopia.

Conclusion Other than the finding of tonsillar pallor, there was no relationship between the extent of tonsillar ectopia and the intraoperative anatomical and physiological observations, nor was there any relationship to the likelihood of symptomatic improvement following surgery. These observations call into question the focus on the extent of tonsillar of ectopia in assessing the patient who presents with symptoms of the Chiari malformation.

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Is there a relationship between the extent of tonsillar ectopia and the severity of the clinical Chiari syndrome?

Acta Neurochirurgica (2020) 162:1531–1538

Chiari 1 malformation is diagnosed if the cerebellar tonsils extend at least 5 mm below the opisthion-basion line.

Objective To examine the correlation of the extent of tonsillar ectopia with the prevalence and severity of the symptoms associated with the Chiari malformation.

Methods Patients (N = 428) were grouped according to the extent of tonsillar ectopia on the mid-sagittal MRI image (group 1, 0–< 3 mm; group 2, 3–5 mm; group 3, > 5 mm). Groups were compared regarding demographics, symptoms, neurological signs, pain score, and response to HADS and sf-36 questionnaires.

Results were analyzed using one-way ANOVA, chi-square, and two sample Z test, and Student’s t test for pairwise comparison, (statistical significance p < 0.05). A logistic regression analysis was performed to determine the relationship between tonsillar ectopia and the probability of a patient reporting any particular symptom. Results There were 97,148 and 183 patients in groups 1, 2, and 3 respectively. Groups did not differ with regard to antecedent trauma or female preponderance. Patients in group 1 were more symptomatic than those in groups 2 and 3 with regard to some symptoms, (p = 0.04–p = 0.000). Regression analysis confirmed an inverse relationship between the extent of tonsillar ectopia and the likelihood of many symptoms. The pain score was greatest in group 1, (p = 0.006). Prevalence of objective signs of myelopathy did not differ between groups except for Hoffmann sign which was more prevalent in group 1, (p = 0.034). HADS and sf-36 scores did not differ between groups.

Conclusion The severity of the symptoms associated with the Chiari malformation does not correlate directly with the extent of tonsillar ectopia. The extent of tonsillar ectopia should be re-evaluated as the threshold for diagnosis of Chiari 1 malformation.

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Tonsillectomy with modified reconstruction of the cisterna magna with and without craniectomy for the treatment of adult Chiari malformation type I with syringomyelia

Acta Neurochirurgica (2020) 162:1585–1595

In light of the controversies regarding the surgical treatment of adult Chiari malformation type I (CM-I) with syringomyelia, a retrospective study was conducted to evaluate the safety and efficacy of tonsillectomy followed by modified reconstruction of the cisterna magna with or without craniectomy.

Methods Between 2008 and 2017, 78 adult CM-I patients (36 males and 42 females, mean age 40.6 years old) with syringomyelia were treated with posterior fossa decompression (PFD) with tonsillectomy and modified reconstruction of the cisterna magna. Patients were divided into two study groups: group A (n = 40) underwent cranioplasty with replacement of the bone flap; group B (n=38) underwent suboccipital craniectomy. Neurological outcomes were evaluated by traditional physician assessment (improved, unchanged, and worsened) and the Chicago Chiari Outcome Scale (CCOS). Syringomyelia outcomes were assessed radiologically.

Results The procedure was successfully performed in all patients, and restoration of normal cerebrospinal fluid (CSF) flow was confirmed by intraoperative ultrasonography. The median postoperative follow-up was 20.3 months (range 18– 60 months). Clinical improvement was evident in 66 (84.6%) patients, with no significant differences between the two groups (85.0% vs. 84.2%, P = 0.897). According to the CCOS, 36 patients (90.0%) in group A were labeled as “good” outcome, compared with that of 34 (86.8%) in group B (P = 0.734). Improvement of syringomyelia was also comparable between the groups, which was observed in 35 (87.5%) vs. 33 (86.8%) patients (P = 0.887). The postoperative overall (7.5% vs. 23.7%, P = 0.048) and CSF-related (2.5% vs. 18.4%, P = 0.027) complication rates were significantly lower in group A than group B.

Conclusions Tonsillectomy with modified reconstruction of the cisterna magna without craniectomy seems to be a safe and effective surgical option to treat adult CM-I patients with syringomyelia, though future well-powered prospective randomized studies are warranted to validate these findings.

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Chiari malformation type I and basilar invagination originating from atlantoaxial instability

Acta Neurochirurgica (2020) 162:1553–1563

Recently, a novel hypothesis has been proposed concerning the origin of craniovertebral junction (CVJ) abnormalities. Commonly found in patients with these entities, atlantoaxial instability has been suspected to cause both Chiari malformation type I and basilar invagination, which renders the tried and tested surgical decompression strategy ineffective. In turn, C1-2 fusion is proposed as a single solution for all CVJ abnormalities, and a revised definition of atlantoaxial instability sees patients both with and without radiographic evidence of instability undergo fusion, instead relying on the intraoperative assessment of the atlantoaxial joints to confirm instability.

Methods The authors conducted a comprehensive narrative review of literature and evidence covering this recently emerged hypothesis. The proposed pathomechanisms are discussed and contextualized with published literature.

Conclusion The existing evidence is evaluated for supporting or opposing sole posterior C1-2 fusion in patients with CVJ abnormalities and compared with reported outcomes for conventional surgical strategies such as posterior fossa decompression, occipitocervical fusion, and anterior decompression. At present, there is insufficient evidence supporting the hypothesis of atlantoaxial instability being the common progenitor for CVJ abnormalities. Abolishing tried and tested surgical procedures in favor of a single universal approach would thus be unwarranted.

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