Background: Klippel–Feil syndrome (KFS) includes craniocervical anomalies, low posterior hairline, and brevicollis, with limited cervical range of motion; however, there remains no consensus on inheritance pattern. This study defines incidence, characterizes concurrent diagnoses, and examines trends in the presentation and management of KFS. Methods: This was a retrospective review of the Kid's Inpatient Database (KID) for KFSpatients aged 0–20 years from 2003 to 2012. Incidence was established using KID-supplied year and hospital-trend weights. Demographics and secondary diagnoses associated with KFS were evaluated. Comorbidities, anomalies, and procedure type trends from 2003 to 2012 were assessed for likelihood to increase among the years studied using ANOVA tests. Results: Eight hundred and fifty-eight KFS diagnoses (age: 9.49 years; 51.1% females) and 475 patients with congenital fusion (CF) (age: 8.33 years; 50.3% females) were analyzed. We identified an incidence rate of 1/21,587 discharges. Only 6.36% of KFS patients were diagnosed with Sprengel's deformity; 1.44% with congenital fusion. About 19.1% of KFS patients presented with another spinal abnormality and 34.0% presented with another neuromuscular anomaly. About 36.51% of KFS patients were diagnosed with a nonspinal or nonmusculoskeletal anomaly, with the most prevalent anomalies being of cardiac origin (12.95%). About 7.34% of KFS patients underwent anterior fusions, whereas 6.64% of KFS patients underwent posterior fusions. The average number of levels operated on was 4.99 with 8.28% receiving decompressions. Interbody devices were used in 2.45% of cases. The rate of fusions with <3 levels (7.46%) was comparable to that of 3 levels or greater (7.81%). Conclusions: KFS patients were more likely to have other spinal abnormalities (19.1%) and nonnervous system abnormalities (13.63%). Compared to congenital fusions, KFS patients were more likely to have congenital abnormalities such as Sprengel's deformity. KFS patients are increasingly being treated with spinal fusion. Level of Evidence: III

Objectives: The aim of this study was to determine the reliability of a C1:C2 ratio in a cohort of patients with atlas fractures. Second, we aimed to consider the utility of the C1:C2 ratio with regard to diagnosis of transverse ligament (TL) injury. Design: This is a retrospective analysis. Methods: Patients with atlas fractures in the Waikato region between 2008 and 2010 were identified retrospectively through clinical coding and collated radiology trauma database. Main Outcome Measurements: The maximal width of C1 and C2 was measured using the first-taken trauma radiograph series. Combined overhang of lateral masses (△mm) and a C1:C2 ratio was then calculated. Final ratio and atlanto-dens interval (ADI) were measured at the last clinical follow-up. Results: A total of 24 patients with full radiographic records were included. Of these, five patients (21%) had TL injuries confirmed on computed tomography or magnetic resonance imaging. No patient with a ratio 1.15 had an intact TL, whereas a ratio of >1.10 captured 80% of TL injuries. The ratio ( P < 0.001) and delta values ( P < 0.001) were statistically significantly different between TL-injured and TL-intact cohorts. Two patients in the TL injury group demonstrated increased ADI on final follow-up with a ratio of >1.10. Conclusions: A C1:C2 ratio >1.10 on plain radiographs showed a sensitivity of 80% in detecting atlas fractures with associated TL injury. All patients with a ratio of ≥1.15 had TL rupture subsequently confirmed by an advanced modality. A ratio calculation on radiographs is a potentially useful method of describing atlas lateral mass displacement. Level of evidence: Level III

Background: Surgical strategy of multisegmental spinal fixation that includes atlantoaxial joint for patients having cervical spondylosis-related symptoms of severe myelopathy is analyzed. Objective: Surgical outcome of patients presenting with “severe” symptoms of cervical myelopathy having multisegmental degenerative cervical spondylosis and treated by multisegmental spinal fixation is analyzed. Atlantoaxial joint was included in the fixation construct in majority of patients. No bone, soft tissue, osteophyte, or disc resection for decompression was done. Materials and Methods: Sixty-four patients having multisegmental cervical spondylosis who presented with symptoms of severe myelopathy were surgically treated during the period from March 2013 to December 2018. On the basis of the concept that instability is the primary cause of spinal degeneration, multisegmental spinal fixation was done in all patients. Atlantoaxial joint was included in the fixation construct in 48 patients. The levels of spinal fixation were determined on the basis of direct observation of facet joints and by manual manipulation and were guided by the presenting clinical features and radiological information. Clinical monitoring was done using Goel clinical grading, modified Japanese Orthopedic Association Score, and visual analog score parameters. Patient satisfaction index assessed the functional and symptomatic improvement. Results: During the follow-up that ranged from 6 to 75 months, all patients improved in their clinical status. Fifty-five (85.9%) patients could walk independently or with mild support. Conclusions: Multisegmental spinal fixation that includes atlantoaxial joint in most patients forms a rational treatment strategy for patients of cervical spondylosis presenting with severe symptoms of myelopathy.

Hypothesis: Reciprocal changes in the upper cervical spine correlate with adult TL deformity modifiers. Design: This was a retrospective review. Introduction: The upper cervical spine has remarkable adaptability to wide ranges of thoracolumbar (TL) deformity. Methods: Patients >18 years with adult spinal deformity (ASD) and complete radiographic data at baseline (BL) and 1 year were identified. Patients were grouped into component types of the Roussouly classification system (Type 1: Pelvic incidence [PI] <45° and lumbar lordosis [LL] apex below L4; Type 2: PI <45° and LL apex above L4; Type 3:45°r assessed changes in BL upper cervical parameters (C0-2, C0 slope, McGregor's Slope [MGS], and CBVA) across groups. Results: A total of 343 ASD patients were analyzed. When grouped by BL Schwab and Roussouly, Group P had the lowest BL disability compared to other Groups, while Roussouley Type 1 correlated with higher BL disability compared to Type 2. Moving cranially up the spine, Group P, Group LP, and Group TL did not differ in C0-2 angle, C0 slope, MGS, or CBVA. Group C had a significantly smaller C0-C2, and more negative MGS, C0 slope, and CBVA than noncervical groups. Type 1 trended slightly higher CBVA and MGS than types 2–4, but no differences in cervical lordosis, C0-C2, or C0S were found. MGS (r = −0.131, P = 0.015), CBVA (r = −0.473, P < 0.001), and C0S (r = −0.099, P = 0.042) correlated most strongly with sagittal vertical axis (SVA) compared to other Schwab modifiers. We found SVA > 34 mm predicted a 1 unit (°°) decrease in MGS (odds ratio [OR]: 0.970 [0.948–0.993], P = 0.010), while cervical SVA >51 mm predicted a 1 unit increase in MGS (OR: 1.25 [1.12–1.38], P < 0.001). Conclusions: Our study suggests that upper cervical alignment remains relatively stable through most broad variations of adult TL deformity. Changes in SVA correlated most with upper cervical changes.

Introduction: Correction of cervical deformity (CD) often involves different types of osteotomies to address sagittal malalignment. This study assessed the relationship between osteotomy grade and vertebral level on alignment and clinical outcomes. Methods: Retrospective review of a multi-center prospectively collected CD database. CD was defined as at least one of the following: C2–C7 Cobb >10°, cervical lordosis (CL) >10°, C2–C7 sagittal vertical axis (cSVA) >4 cm, and chin-brow vertical angle > 25°. Patients were evaluated for level and type of cervical osteotomy. Results: 86 CD patients were included (61.4 ± 10.6 years, 66.3% female, body mass index 29.1 kg/m²). 141 osteotomies were in the cervical spine and 79 were in the thoracic spine. There were 19 major osteotomies performed, with 47% at T1. Patients with an osteotomy in the cervical spine improved in T1 slope minus CL (TS − CL), CL, and C2 slope (all P < 0.05). Patients with upper thoracic osteotomies improved in TS − CL, cSVA, C2–T3, C2–T3 sagittal vertical axis (SVA), and C2 slope (all P < 0.05). Minor osteotomies in the upper thoracic spine showed improvement in cSVA (63 mm to 49 mm, P = 0.022), C2–T3 ( P = 0.007), and SVA (−16 mm to 27 mm, P < 0.001). The greatest amount of C2–T3 angular change occurred for patients with a major osteotomy at T2 (39.1° change), then T3 (15.7°), C7 (16.9°°), and T1 (13.5°°). Patients with a major osteotomy in the upper thoracic spine showed similar radiographic changes from pre- to post-operative as patients with three or more minor osteotomies, although C2–T3 SVA trended toward greater improvement with a major osteotomy (−22.5 mm vs. +5.9 mm, P = 0.058) due to lever arm effect. Conclusions: CD patients undergoing osteotomies in the cervical and upper thoracic spine experienced improvement in TS–−CL and C2 slope. In the upper thoracic spine, multiple minor osteotomies achieved similar alignment changes to major osteotomies at a single level, while a major osteotomy focused at T2 had the greatest overall impact in cervicothoracic and global alignment in CD patients.

Background: Most of the literature on role of spinopelvic parameters in various lumbar spine pathologies has been based on studies done on Caucasian population. Aims and Objectives: The present study attempts to establish a database of measurements of the sagittal profile of spine in asymptomatic Indian population and their comparison with subjects having various lumbar spine pathologies. Materials and Methods: We performed a prospective case control study at All India Institute of Medical Sciences, New Delhi in which we enrolled 109 patients and 22 healthy asymptomatic subjects in 2 years from 2015 to 2017. All patients underwent standing lateral radiographs of the pelvis and the entire spine and various spino-pelvic parameters were measured using Surgimap software. Results: The mean Pelvic incidence (PI) in the asymptomatic individuals was 49.29 ± 5.95° which was significantly lower when compared with patients of chronic low backache (53.96 ± 9.47, P-<0.001), lumbar listhesis (59.4 ± 21.33, P-<0.001) and failed back surgery syndrome (56.7 ± 8.21, P-<0.001). The mean Pelvic Tilt (PT) in healthy subjects was 14.3±4.08° which was significantly lower when compared with patients of lumbar listhesis (23.35 ± 14.03, P-<0.001) and failed back surgery syndrome (22.8 ± 8.09, P-<0.001). Sacral slope (SS) and sagittal vertical axis (SVA) offset did not show any statistically significant difference. The mean Lumbar lordosis (LL) measured in healthy individuals was 42.5 ± 7.89° which was significantly lower when compared with patients of lumbar listhesis (46.24 ± 19.24, P-0.04) and failed back surgery syndrome (45.12 ± 6.87, P-0.05). Conclusion: PT and PI showed statistically significant difference in subjects having lumbar spondylolisthesis and failed back surgery syndrome as compared to healthy asymptomatic subjects.

Background: Scoliosis is the most frequent spinal deformity related to Marfan syndrome (MFS). Treatment with a brace is often ineffective, and surgical treatment is very challenging; many instrumentations were used along the years. Our retrospective study has the purpose of identifying the reliability of different devices in three-dimensional correction of the spine deformities in MFS. Materials and Methods: We reviewed retrospectively the records of patients surgically treated, in a single institution between 1999 and 2016, for spinal deformities in MFS. X-rays were reviewed for analyzing the magnitude of the curves in preoperative time (T0), the amount of correction in the immediate after surgery period (T1), and it's stability at follow-up (FU) (T2). The clinical outcomes were also evaluated with the Scoliosis Research Society 24. Results: A total of 21 patients with a mean age at surgery of 16 years met inclusion and exclusion criteria. Four different construct types were identified: hooks with sublaminar wires (G1), hooks and pedicle screws (G2), pedicle screws (G3), and pedicle screws with sublaminar wires (G4). The mean FU time was 8 years. The average major scoliosis curve had a mean value of 63.48° at T0 and was corrected to 28.81° at T2. Furthermore, minor curve, thoracic lordosis, and lumbar kyphosis (when associated to scoliosis) were also corrected. Student t-test showed significative differences ( P < 0.05) for all curves between T0–T1 and T0–T2 while between T1 and T2, no differences were found. We also evaluated separately the results of each instrumentation, and G3 obtained the best performances. Conclusions: Our results shows that screws may guarantee a better correction of the deformities. Level of Evidence: III

Introduction: The aim of this study was to evaluate the association of available cervical alignment components through the Ames cervical deformity (CD) classification parameters with the Patient-Reported Outcomes Measurement Information System (PROMIS) physical health domain metrics. Methods: Surgical CD patients (C2–C7 Cobb >10° or C2–C7 sagittal vertical axis [cSVA] >4 cm or T1 slope minus cervical lordosis (TS-CL) >15°) ≥18 years with available baseline (BL) radiographic and PROMIS were isolated in a single-center spine database. Patients were classified according to the Ames CD modifiers for cSVA and TS-CL (low deformity [Low], moderate deformity [Mod], and severe deformity [Sev]). Descriptives and univariate analyses compared population-weighted PROMIS scores for Pain Intensity (PI), Physical Function (PF), and Pain Interference (Int) across CD modifiers. Conditional tree analysis with logistic regression sampling determined the threshold of PROMIS scores for which the correlation with Ames radiographic cutoffs was most significant. Reported cutoff values for Mod (cSVA: 4–8 cm; TS-CL: 15–20°) and Sev (cSVA: >8 cm; TS-CL: >20°) disabilities were used. Results: Two hundred and eight patients (58.8 years, female: 51%, 29.6 kg/m², Charlson Comorbidity Index: 1.19). BL cSVA modifier by severity: 83.2% Low, 16.8% Mod. No patients met criteria for severe cSVA. BL TS-CL modifier by severity: 18.8% Low, 22.1% Mod, 59.1% Sev. Mean baseline PROMIS scores were as follows: PI score: 89.6 ± 15.4, PF score: 11.9 ± 13.1, Int score: 56.9 ± 6.8. PI did not differ between cSVA and TS-CL severity. Mod cSVA patients and Mod/Sev TS-CL modifier groups trended toward lower PF scores and higher Int scores. A PI score of >96 (odds ratio [OR]: 0.658 [0.303–1.430]), a PF score of <14 (OR: 1.864 [0.767–4.531]), and an Int score of > 57.4 (OR: 1.878 [0.889–3.967]) were predictors of Mod cSVA. A PI score of >87 (OR: 1.428 [0.767–2.659]), a PF score of <14 (OR: 1.551 [0.851–2.827]), and an Int score of >56.5 (OR: 1.689 [0.967–2.949]) were predictors of Sev TS-CL. Conclusions: PROMIS physical health domains were related to the Ames CD classification. Certain BL PROMIS thresholds can be connected to the severity of CD.

The authors report a case of a 19-year-old female patient having basilar invagination with complex musculoskeletal abnormalities wherein atlantoaxial fixation was done with a combination of Goel and Magerl techniques on a single articulation on one side and two transarticular screws (Magerl technique) were deployed on the contralateral side articulation. The combination of Goel and Magerl techniques used in a novel fashion resulted in strong fixation and provided an environment for bone fusion. The special joint architecture and location of facet of atlas anterior and rostral to the facet of axis in the form of facetal-spondyloptosis were used to advantage as it provided a direct screw trajectory for transarticular screw insertion. The patient recovered after surgery in her neurological function. Craniovertebral junction realignment could be observed. Solid bone fusion was observed after 8 months of the surgical procedure.

The clinical presentation of spinal or extraspinal neurofibroma is radiculopathy or myelopathy, pain, and motor weakness. Extraspinal neurofibroma presenting with acute-onset monoparesis and Horner's syndrome is very rare. We report the case of a 55-year-old female who presented with acute-onset monoparesis of the left upper limb along with left-side drooping of the eyelid. Imaging revealed C6–D2 extraspinal solitary mass lesion lateral to spinous process with bleed without intraspinal component. The patient underwent an anterior cervical approach and excision of the tumor. Final biopsy report was a neurofibroma. At 3-year follow-up, she recovered from motor weakness, and Horner's syndrome subsided. Extraspinal neurofibroma can present with acute bleed, and surgical outcome is superior in early intervention.

Localized proliferation of atypical plasma cells, either at bony or extramedullary, forms a rare subset of multiple myeloma (MM) disorders. The patients usually present with intractable pain and pathological fractures and respond well to radiotherapy. The clinical presentation is variable and radiologically is nonspecific. The spinal location is rare, and the solitary plasmacytoma in the intradural extramedullary (IDEM) region is unusual. Herein, we report the second case of solitary plasmacytoma at the lumbar IDEM region. A 54-year-male patient was referred to our institute with complaints of radicular pain in bilateral lower limbs along L5 dermatome for the past 3 months. The neurological examination was normal with power 5/5 and reflexes 2+, except for bilateral straight leg raising test restricted at 30. Magnetic resonance imaging of spine showed a well-defined eccentrically placed (left Side) spherical lesion at the level of L2 vertebrae. The lesion had foraminal extension and showed minimal contrast enhancement. The underlying ventral vertebral body was irregular and hyperosteotic. Our radiological impression was primarily neurofibroma, but the features were slightly atypical. The patient underwent L1–L2 laminectomy and excision of IDEM tumor. The histopathological features were consistent with plasmacytoma. The histopathology was a surprise to us. We further evaluated the patient for MM. He received adjuvant radiotherapy and is currently asymptomatic. An index of suspicion for SP must be kept among differentials of intradural lesions, especially when adjacent bony changes are present. The diagnosis of plasmacytoma warrants further workup according to the recommendations of the International Myeloma Working Group.