Introduction: Ehlers–Danlos syndrome (EDS) predisposes to craniocervical instability (CCI) with resulting cranial settling and cervicomedullary syndrome due to ligamentous laxity. This study investigates possible differences in radiographic outcomes and operative complication rate between two surgical techniques in patients with EDS and CCI undergoing craniocervical fusion (CCF): occipital bone (OB) versus occipital condyle (OC) fixation. Methods: A retrospective search of the institutional operative database between January 07, 2017, and December 31, 2019, was conducted to identify EDS patients who underwent CCF with either OB (Group OB) or OC (Group OC) fixation. For each patient, pre- and post-operative radiographic measurements and operative complications were extracted and compared between groups (OB vs. OC): pB-C2, clivoaxial angle (CXA), tonsillar descent, C2C7 sagittal Cobb angle, C2 long axis, and operative complications. Results: Of a total of 26 patients, 13 underwent OV and 13 underwent OC fixation. Eighty-five percent of the patients underwent OC underwent fusion from occiput to C2, while the remaining 15% fusion from occiput to C3. Radiographic outcome in the OC versus OB group was preoperative measurements were similar between OC and OB group: pB-C2 8.8 mm (1.5, 6–11) versus 8.3 mm (1.7, 4–9.6), P = 0.43; CXA 128.2° (5.4, 122–136) versus 131.9° (6.8,122–141), P = 0.41; tonsillar descent 6.2 mm (4.8, 0–15) versus 2.9 mm (3.4, 0–8), P = 0.05; C2 long axis 75.2° (6.7, 58–85) versus 67.2° (21.4, 1–80), P = 0.21; postoperative change of CXA + 14.4° (8.8, 0–30) versus 16.2° (12.4, −4–38), P = 0.43; change of pB-C2 − 2.6 mm (1.8, −-5.3 to 0) versus − 1.2 mm (4, −4.6–8), P = 0.26; and postoperative C2C7 sagittal Cobb angle − 2.6° (19.5, −43–39) versus − 2.6° (11.4, −21–12). Operative complications were seen in 1 out of 13 patients (8%) versus 2 out of 13 patients (16%), P = 1. Conclusions: In EDS, patients with CCI undergoing CCF radiographic and clinical outcome were similar between those with OC versus OB fixation. Both techniques resulted in sufficient correction of pB-C2 and CXA measurements with a low complication rate.

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Retroclival epidural hematoma in adults is uncommon. Although most cases are associated with craniocervical trauma, other mechanisms have been reported, such as coagulopathy, vascular lesions, and pituitary apoplexy. We report two adults diagnosed with retroclival epidural hematoma. One patient was an 89-year-old male with leukemia and thrombocytopenia who sustained a fall and developed a traumatic retroclival epidural hematoma with brainstem compression; surgery could not be performed due to his clinical condition and he died 5 days later. The other patient was a 78-year-old female with atrial fibrillation who developed a spontaneous retroclival epidural hematoma as a result of warfarin use; she was treated conservatively with anticoagulant reversal and methylprednisolone and was subsequently discharged without neurological deficit. Retroclival hematomas are primarily treated conservatively due to the difficulty of surgical approach. The bleeding mechanism and dural and venous anatomy of this region tend to limit hematoma expansion.

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Extradural spinal nerve root hemangioblastoma is a rare entity with very few cases reported in the literature. A comprehensive picture of the treatments and outcomes of the same is thus not available. A systematic search was done according to PRISMA guidelines. Search criteria included terms: spinal extradural hemangioblastoma, extradural hemangioblastoma, and spinal root hemangioblastoma. The parameters considered were treatment, motor, and sensory outcome, association with von-Hippel-Lindau (VHL) syndrome. Twenty-two studies (19 full text articles) were available for the review. A total of 39 cases of extradural spinal nerve root hemangioblastoma have been reported. These cases had a median age of 44 years with male predominance (2:1) and up to 48% occur in the thoracic level, similar to our case. Thirty-six percent of patients were associated with VHL syndrome. Surgical resection was the primary modality of treatment with embolization used in selected cases (20%). They had mean follow-up of 23 (±11) months. The prognosis was better than the intradural counterpart with no motor deficit and sensory deficit in only 9%. Preoperative identification of the extradural nature of this pathology and complete excision at the first surgery offers excellent outcomes compared to intradural lesion. Targeted embolization may be used in cases anticipated with high blood loss.

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Background: Anterior cervical osteophytes (ACOs) may rarely cause dysphagia, dysphonia, and dyspnea. Symptomatic ACOs are most commonly located between C3 and C7, whereas those at higher cervical (C1–C2) levels are rarer. We report a case series of 4 patients and discuss the best surgical approach according to the ostheophyte location and size, mainly for those located at C1–C2, and the related surgical problems. Materials and Methods: Four patients (two males and two females) aged from 57 to 72 years were operated on for ACOs, causing variable dysphagia (and dyspnea with respiratory arrest in one). Three patients with osteophytes between C3 and C5 were approached through antero-lateral cervical approach, and one with a large osteophyte between C1 and C3–C4 level underwent a two-stage transcervical and transoral approach. All had significant postoperative improvement of dysphagia. Results: The patient operated on though the transoral approach experienced postoperative flogosis of the prevertebral tissues and occipital muscles and thrombosis of the right jugular vein and transverse-sigmoid sinuses (Lemierre syndrome). Conclusion: The transoral approach is the best surgical route to resect C1 and C2 ACOs, whereas the endoscopic endonasal approach is not indicated. The anterior transcervical approach is easier to resect osteophytes at C3, as well as those located below C3. A combined transoral and anterior cervical approach may be necessary for multilevel osteophytes.

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Introduction: The objective of this study is to propose a novel classification and algorithmic-based management plan for craniovertebral junction osteoarthrosis (CVJOA). Materials and Methods: A retrospective study was done based on prospective database of radiological studies and clinical history. Twenty symptomatic patients (12 females and 8 males) with a mean age of 54.8 years were identified with CVJOA. These patients underwent either nonsurgical treatment only or surgical intervention and had follow-up of at least 14 months. Classification of CVJOA is based on coronal deformity, rigidity, stability, and two modifiers. The main surgical procedures done in the surgical arm of these patients included C1–C2 fusion, C1–C2 facet distraction and fusion, and unilateral subaxial facet distraction, and posterior column osteotomy. Results: All the twenty patients included in this study complained of either sub-occipital or upper neck pain and had radiological evidence of CVJOA. Seven patients improved with nonsurgical management and 13 underwent surgical intervention. Surgical recommendations for each type of CVJOA have been described with case examples, and algorithm for the management of CVJOA has been developed based on this study. Interobserver agreement on CVJOA classification was measured using kappa value statistics which showed moderate strength of agreement (0.467). Conclusion: This study describes a novel classification and management of CVJOA based on algorithm and current surgical recommendations for each type of CVJOA.

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Objective: Larsen syndrome (LS) is characterized by osteo-chondrodysplasia, multiple joint dislocations, and craniofacial abnormalities. Symptomatic myelopathy is attributed to C1–C2 instability and sub-axial cervical kyphosis. In this article, we have analyzed the surgical outcome after posterior fixation in LS with craniovertebral junction instability. Methods: Ten symptomatic pediatric patients, operated between 2011 and 2019, were included, and the clinical outcome was assessed by Nurick grade, neurological improvement, and complications. The requirement of anti-spasticity drugs, the degree of bony fusion, and restriction of neck movement were also noted. At last follow-up, patient satisfaction score (PSS) and back to school status were studied. We also reviewed the literature and categorized two types of presentation of reported LS patients and discussed the pattern of disease progression among both. Results: Ten patients, age range 1.5–16 years, underwent 12 surgeries (6 C1–C2 fixation, 4 long-segment posterior cervical fixation, and 2 trans-oral decompressions as the second stage); the mean follow-up was 23 (range, 6–86 months). All the ten patients in our study had the characteristic “dish-” like face and nine patients had acral anomalies. The median Nurick grade improved from preoperative (median = 4) to follow-up (median = 3). The requirement of anti-spasticity drugs decreased in seven patients and the neck-pain improved in nine patients. The median satisfaction at follow-up was good (median PSS = 2); five patients were going back to school. Conclusion: Craniovertebral junction instability in LS is rare and surgically challenging. Early posterior fixation showed a promising outcome with a halt in the disease progression.

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Aims and Objectives: The human spine degenerates with age. Intervertebral disc degeneration occurs in the cervical spine. The objective of this study is to determine the effects of degenerative disc diseases on the range of motion (ROM) of the human cervical spinal column using a validated finite-element model. Materials and Methods: The validated intact and healthy C2–T1 finite-element model simulated the cortical shell, cancellous core, posterior elements of the vertebrae, and spinal ligaments (longitudinal, capsular, spinous and ligamentum flava, and nucleus and annulus of the discs). Three different stages of the disc disease, that is, mild, moderate, and severe, were simulated at the C5–C6, C6–C7, and C5–C6–C7 discs, respectively, and they were termed as upper single level, lower single level, and bi-level (BL) models, respectively. The material properties and geometry of the disc(s) were altered to simulate the different stages of degeneration. The external mechanical loading was applied in the sagittal mode, via flexion–extension motions and the magnitude was 2.0 Nm for each mode. They were applied to each of the healthy and disc degeneration models, and for each of the three severities of degeneration. The ROM at adjacent and index levels was extracted and normalized with respect to the healthy (baseline) spine. Results: A nonuniform distribution in the ROM was found for different disc degeneration states, segmental levels, and flexion–extension loading modes. The specific results for each and level are reported in the results section of the paper. Conclusion: Closer follow-up times may be necessary in symptomatic patients with progressive disease, especially with BL involvements.

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Objective: Symptomatic lumbar spinal stenosis (LSS) unresponsive to conservative therapy is commonly treated by surgical decompression. In this study, we compared clinical outcomes after decompressive surgery for LSS in patients implanted with interarticular spacers along with microdecompression (MD) with those receiving only MD. Methods: A retrospective study was analyzed 40 patients (Group A) affected by LSS treated by MD and implant of interarticular spacers comparing the outcome with a homogeneous group of 40 patients with LSS treated with MD alone (Group B). Clinical outcome was evaluated using the Oswestry Disability Index (ODI) and visual analog scale (VAS) scores, as well as Macnab's criteria. Results: At 1-year follow-up, ODI improved in both groups with statistically significant differences as compared to baseline and both Groups (P < 0.05). Statistically significant differences were observed at 3-year follow-up (P < 0.05), without further variation at 5-year follow-up. At 1-year follow-up, VAS for back and leg pain scores was significantly better than that of Group B (P < 0.05). At 3-year follow-up, back and leg pain scores were no longer significantly improved (P > 0.01), resulting almost the same at 5-year follow-up. A comparison of functional outcomes between the groups showed significant improvements in Group A as compared to Group B (P < 0.05). The reoperation rate was 10% in Group A and 30% in Group B. In implanted patients, successful fusion was obtained in 90% of the cases. Conclusions: Interarticular spacers showed significant and clinically meaningful improvements in pain and disability, even in a long follow-up.

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Background: The introduction of recent innovations in the field of intraoperative imaging and neuronavigation, such as OArm Stealth Station, allows to obtain crucial intraoperative data by performing safer and controlled surgical procedures. As part of the improvement of surgical visual magnification and wide expansion of surgical corridors, the 3D-4K exoscope (EX) represents nowadays an interesting and useful tool. Transoral approach (TOA) represents the historical gold standard direct microsurgical route to ventral craniovertebral junction (CVJ). Methods: We herein report a preliminary experience on 6 cases of 33 patients operated by TOA concerning the simultaneous application of OArm with Stealth Navigation system (Medtronic, Memphis, TN) and imaging system along with the 3D-4K EXs in TOA for the treatment of CVJ pathologies. Results: Neither intraoperative neurophysiological changes nor postoperative infections occurred, but a neurological improvement was evident in all the patients. A complete decompression along with stable instrumentation and fusion of the CVJ was accomplished in all cases at the maximum follow-up (mean: 16.8 months). Conclusions: With EX, the role of surgeon become self-sufficient with a better individual surgical freedom compared to endoscopic surgery and excellent 3D vision and magnification. OArm allows an absolutely reliable intraoperative support for a more effective CVJ decompression. Nevertheless, with OArm-assisted neuronavigation, it can be difficult to navigate C1 lateral masses and C2 isthmi, and to convert 3D into 2D real-time navigation, it can become quite complicate. Finally, the association of EX and OArm appears more time consuming compared to the old fashion one.

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Introduction: Junctional kyphosis (JK) and junctional failure (JF) are known complications after thoracolumbar spinal deformity surgery. This study aims to define the incidence and possible risk factors for JK/JF following multi-segmental cervicothoracic fusion. Methods: This is a retrospective analysis of 64 consecutive patients undergoing cervicothoracic fusion surgery, including at least five segments. Clinical and radiographic outcome measures were analyzed. A univariate analysis was performed to determine the effect of the level of upper instrumented vertebra (UIV) and lower instrumented vertebra (LIV), fusion status, C2 sagittal vertical axis (SVA), C2–C7 lordotic angle and T1 slope angle on the occurrence of JK/JF. Results: A total of 46 patients were followed up for a median of 1.1 years (range 0.3–4) with a median age of 65.5 years (range 42.2–84.5). Indication for surgery was spinal stenosis in 87%, trauma in 7%, and tumor in 6% of cases. The median number of levels fused was 7; the most frequent UIV was C2, and the most frequent LIV was T2. Solid fusion was achieved in 78% at the last follow-up. Postoperatively, the median C2 SVA was 32 mm (range − 7–75), median T1 slope angle was 33° (range 2°–57°), C2–C7 sagittal cobb angle was 4° (−29°–12°). JK developed in 4% of cases, no case of JF was observed. No statistically significant impact of bone density, level of UIV, level of LIV or postoperative sagittal parameters on the occurrence of JK/JF was observed, even though fusion status and pathologic T1 slope angle showed a trend toward significance. Conclusion: In this cohort of patients with mildly pathologic sagittal balance, JK was a rare event after multi-segmental fusion, observed in only 4% of cases. Neither level of UIV nor LIV had an influence on its occurrence; however, nonunion and pathologic sagittal alignment showed a nonsignificant trend.

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Background: Extraforaminal lumbar disc herniation (ELDH) amounts of 7%–12% of all lumbar disc herniations. Although they have already been widely described, an optimal treatment is still under discussion in the literature. Objective: We describe a novel application of navigation using 2D/3D imaging system to plan an adequate surgical trajectory and performing a neuronavigated microdiscectomy in ELDH that has not been previously described. Methods: This is a retrospective study in a single institution. Between February 2017 and July 2020, a total of 12 patients (7 males and 5 females), with a mean age of 56 years (range 49–71 years), have been treated because of ELDH through a far lateral microdiscectomy using 2D/3D imaging system-assisted neuronavigation (O-arm). Results: No intraoperative and/or postoperative complications were recorded. Patients presented a mean preoperative Visual Analog Scale (VAS) score of 7.83 ± 0.83 (range 7–9). At the day of discharge, leg pain VAS score effectively improved, decreasing to a mean value of 1.83 ± 0.83 (range 1–3). Further, low back and radicular pain improvement was recorded at 1-, 6-, and 12-month follow-up, respectively. Conclusion: We described a novel use of 2D/3D imaging system navigation in the microsurgical treatment of ELDH that has not previously reported. This technique is safe and effective and provides more intraoperative details compared to fluoroscopy, which can be crucial for the success of the procedure and to reduce complications and particularly indicated in complex cases with altered anatomy.

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Biomodels are produced using three-dimensional printers and their use in complex spine surgeries can be quite helpful, especially when complex anatomy is faced. In this case report, we presented a 14-year-old patient who had rigid congenital cervical scoliosis and basilar invagination and abnormalities on a neurological examination. This patient underwent atlantoaxial facet distraction and C1 C2 fusion while using a biomodel of his craniocervical junction in pre-operative planning and also as an anatomical reference per-operatively. Using biomodel in this case helped in achieving favorable surgical outcomes without any perioperative complications. Postoperative assessments including coronal deformity, basilar invagination, and neurological examination showed significant improvements and we recommend using biomodels in complex atlantoaxial distraction procedure to achieve favorable surgical outcomes with minimum complications.

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Horizontal fractures of the atlas are uncommon fractures associated with instability of the craniocervical junction. Most commonly associated with high-speed motor vehicle accidents, these fractures need to be identified and treated appropriately. Due to its relatively benign presentation on bony imaging, magnetic resonance imaging to look for ligamentous instability is important. We present two such cases which were managed by occipitocervical fusion at our institute.

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Context: Bony lesions involving the cranium and spine have a wide range of etiologies, ranging from congenital, traumatic, inflammatory, to neoplastic. Aim: The aim was to analyze the histological spectrum of various bony lesions of cranium and spine received as biopsies from the neurosurgery department in our hospital. Materials and Methods: There were 123 cases of bony lesions of cranium and spine diagnosed over a period of 5 years during 2015–2019 in the neuropathology laboratory. These cases were studied retrospectively. Results: Out of the total 123 cases of bony lesions analyzed, 75 affected the cranium and 48 affected the spine. Overall, neoplastic lesions (83) were more frequent than the nonneoplastic lesions (40). In the cranium, neoplastic lesions (66/75) outnumbered the nonneoplastic ones (9/75), whereas in the spine, nonneoplastic lesions (31/48) were more common. Chordoma (40/83) was the most common neoplasm, whereas tuberculous osteomyelitis (30/40) was the most common nonneoplastic lesion encountered. Majority of the patients were adult males aged between 21 and 50 years. Rare lesions such as spinal osteochondroma, poorly differentiated neoplasm metastatic to the cervical spine from a primary salivary gland neoplasm, spinal metastasis of a glioblastoma, and intraosseous meningioma of cranium were recorded. Conclusions: The study provides epidemiological information regarding the incidence and nature of bone lesions of the spine and cranium.

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