Atlantoaxial instability associated with ALDH18A1 mutation.

We report a 10-year-old boy with a de novo pathogenic variant in ALDH18A1, a rare form of metabolic cutis laxa, which was complicated by atlantoaxial instability and spinal cord compression following a fall from standing height. The patient required emergent cervical spine fusion and decompression followed by a 2-month hospitalization and rehabilitation. In addition to the core clinical features of joint and skin laxity, hypotonia, and developmental delays, we expand the connective tissue phenotype by adding a new potential feature of cervical spine instability. Patients with pathogenic variants in ALDH18A1 may warrant cervical spine screening to minimize possible morbidity. Neurosurgeons, geneticists, primary care providers, and families should be aware of the increased risk of severe cervical injury from minor trauma.

Rotational vertebral artery occlusion ("bow hunter syndrome").

PURPOSE: To review the literature, analyze and discuss diagnostic and treatment options for the Bowhunter Syndrome. A clinical case of idiopathic rotatory C1-C2 subluxation causing dynamic vertebral artery occlusion is presented. METHODS: Literature review between 1960 and 2019, discussion of diagnostic methods and treatment options. Description of diagnostic and treatment methods in the aforementioned case. RESULTS: We present a patient with dynamic left vertebral artery occlusion associated with idiopathic rotatory C1-C2 subluxation. A dynamic Angio-CT showed rotatory C1-C2 subluxation with significant flow reduction at the left vertebral artery at the exit of the C2 transverse foramen until the V3 segment when the head rotated towards the right. Due to clinical and radiological worsening in the following months, posterior C1-C2 arthrodesis was performed, with the disappearance of the symptoms. There are 193 cases reported with dynamic vertebral artery occlusion, but in only two, the etiology was primary rotational atlantoaxial instability. The most prevalent etiology was degenerative. CONCLUSION: Rotatory vertebral artery occlusion is a rare condition presented mostly in adults, aged 50-70 years. Vertebrobasilar insufficiency is triggered by the rotation of the head to the contralateral side of the dominant vertebral artery. Dynamic subtraction angiography is considered the diagnostic gold-standard method, but dynamic Angio-CT scan, Angio-MRI, or Doppler ultrasonography are less invasive options. The treatment options are conservative or surgical. Endovascular surgery is another option in specific cases.

Suitability of the Subaxial Cervical Laminae for Laminar Screws in the Pediatric Population: A Radiologic Anatomical Study.

INTRODUCTION: The laminar screw method was popularized during recent years. Since no data exist in the literature on its suitability for subaxial levels in the pediatric population, a radiologic anatomical study was planned to evaluate the suitability of the laminae for laminar screws in children. METHODS: The laminar thicknesses from C3 to C7 were measured in axial sections in 120 pediatric patients using computed tomography. The patients were divided into 3 age-groups: ages 4-6, 7-9, and 10-12. Each age-group consisted of 20 boys and 20 girls. The suitability of the laminae was evaluated for 3.5-mm-thick commercially available screws and also for the 3-mm screws that could be produced in the future. If the height of the base of the spinous process is ≥ 9 mm, the segment was accepted as suitable for the bilateral screw, and ≥5 mm is for the unilateral screw. Additionally, laminar thickness and length were measured for possible short screws longer than 15 mm in the laminae that were distally thin but proximally thick. RESULTS: The C4 and C5 levels had the thinnest (2.77 ± 0.6 mm and 2.81 ± 0.6 mm, respectively) and C7 had the thickest laminae (4.66 ± 0.6 mm) in all age-groups. No significant differences were found between boys and girls and right and left laminae. According to the age-groups, an increase in laminar thickness was possible by growing, but only C7 laminae thickness was statistically different between 10-12 and 4-6 age-groups (p < 0.001). The last rate of the suitability was 9.1% for the 3.5-mm-thick screws and 13.75% for the 3-mm-thick screws after deletion of the bilateral insertion due to the short base of the spinous process and adding the possible short screws in the distally thin but proximally thick laminae. The rates increased with age, but the only statistically significant difference was found between 4-6- and 10-12-year-old age-groups (p < 0.001). CONCLUSIONS: Laminar screws may be suitable for some levels of C7, C6, and C3 even in the young pediatric population. The use of thinner screws (3 mm) may increase the suitability rate. Therefore, laminar screw choice may be considered as a salvage method in pediatric patients, and all laminae may be evaluated individually for suitability. This study did not evaluate the safety and efficacy of the method in children, and these issues must be studied further.

Does the C3/4 disc play a role in cervical spondylosis with dizziness? A retrospective study.

PURPOSE: To investigate the effect of C3/4 disc degeneration on cervical spondylosis with dizziness (CSD) and to assess the curative effect of anterior cervical decompression and fusion (ACDF) in patients with CSD. METHOD: Four hundred nineteen patients who underwent ACDF for treatment of myelopathy or radiculopathy were divided into dizziness and non-dizziness group. The visual analog scale (VAS) score and Japanese Orthopaedic Association (JOA) score were used to determine the intensity of dizziness and neurological symptoms, respectively. Cervical disc degeneration was evaluated using Miyazaki's classification system. Some parameters were measured using cervical radiographs. The surgical effects on CSD were compared between surgery with and without C3/4 level. Multivariate logistic regression analysis was used to determine the risk factors for CSD. RESULTS: The pre-operative incidence of CSD was 33.9%. Women were more likely to develop dizziness than men (p < 0.05), CSD was significantly associated with C3/4 disc degeneration (69.7%, p < 0.001), and smokers were more subject to dizziness (p < 0.05). Regression analysis showed that female (OR = 1.611, p = 0.031), smoking (OR = 1.719, p = 0.032), Miyazaki grade of C3/4 ≥ IV (OR = 2.648, p < 0.001), and instability on C3/4 (OR = 1.672, p = 0.024) were risk factors for CSD. Treatment of CSD by ACDF involving C3/4 was more effective than not involving C3/4 (efficacy rate, 73.2% vs 51.7%, p < 0.05). CONCLUSION: The CSD is a common clinical manifestation in elderly patients, especially patients with cervical spondylosis at the C3/4 level. Female, smoking, instability on C3/4, and C3/4 Miyazaki grade ≥ IV could be considered significant risk factors for CSD. CSD is more likely to be alleviated by ACDF involving C3/4.

Two-level cervical corpectomy-long-term follow-up reveals the high rate of material failure in patients, who received an anterior approach only.

In contrast to a one-level cervical corpectomy, a multilevel corpectomy without posterior fusion is accompanied by a high material failure rate. So far, the adequate surgical technique for patients, who receive a two-level corpectomy, remains to be elucidated. The aim of this study was to determine the long-term clinical outcome of patients with cervical myelopathy, who underwent a two-level corpectomy. Outcome parameters of 21 patients, who received a two-level cervical corpectomy, were retrospectively analyzed concerning reoperations and outcome scores (VAS, Neck Disability Index (NDI), Nurick scale, modified Japanese Orthopaedic Association score (mJOAS), Short Form 36-item Health Survey Questionnaire (SF-36)). The failure rate was determined using postoperative radiographs. The choice over the surgical procedures was exercised by every surgeon individually. Therefore, a distinction between two groups was possible: (1) anterior group (ANT group) with a two-level corpectomy and a cervical plate, (2) anterior/posterior group (A/P group) with two-level corpectomy, cervical plate, and additional posterior fusion. Both groups benefitted from surgery concerning pain, disability, and myelopathy. While all patients of the A/P group showed no postoperative instability, one third of the patients of the ANT group exhibited instability and clinical deterioration. Thus, a revision surgery with secondary posterior fusion was needed. Furthermore, the ANT group had worse myelopathy scores (mJOASANT group = 13.5 ± 2.5, mJOASA/P group = 15.7 ± 2.2). Patients with myelopathy, who receive a two-level cervical corpectomy, benefitted from surgical decompression. However, patients with a sole anterior approach demonstrated a very high rate of instability (33%) and clinical deterioration in a long-term follow-up. Therefore, we recommend to routinely perform an additional posterior fusion after two-level cervical corpectomy.

Radiographic analysis of the correlation between ossification of the nuchal ligament and sagittal alignment and segmental stability of the cervical spine in patients with cervical spondylotic myelopathy.

BACKGROUND: Ossification of the nuchal ligament (ONL) caused by chronic injury to the nuchal ligament (NL) is very common in instability-related cervical disorders. PURPOSE: To determine possible correlations between ONL, sagittal alignment, and segmental stability of the cervical spine. MATERIAL AND METHODS: Seventy-three patients with cervical spondylotic myelopathy (CSM) and ONL (ONL group) and 118 patients with CSM only (control group) were recruited. Radiographic data included the characteristics of ONL, sagittal alignment and segmental stability, and ossification of the posterior longitudinal ligament (OPLL). We performed comparisons in terms of radiographic parameters between the ONL and control groups. The correlations between ONL size, cervical sagittal alignment, and segmental stability were analyzed. Multivariate logistic regression was used to identify the independent risk factors of the development of ONL. RESULTS: C2-C7 sagittal vertical axis (SVA), T1 slope (T1S), T1S minus cervical lordosis (T1S-CL) on the lateral plain, angular displacement (AD), and horizontal displacement (HD) on the dynamic radiograph increased significantly in the ONL group compared with the control group. The size of ONL significantly correlated with C2-C7 SVA, T1S, AD, and HD. The incidence of ONL was higher in patients with OPLL and segmental instability. Cervical instability, sagittal malalignment, and OPLL were independent predictors of the development of ONL through multivariate analysis. CONCLUSION: Patients with ONL are more likely to have abnormal sagittal alignment and instability of the cervical spine. Thus, increased awareness and appreciation of this often-overlooked radiographic finding is warranted during diagnosis and treatment of instability-related cervical pathologies and injuries.

Traumatic Occipitocervical Distraction Injuries in Children: A Systematic Review.

BACKGROUND: Occipitocervical distraction injuries (OCDI) in children occur on a wide spectrum of severity, and decisions about treatment suffer from a lack of rigorous guidelines and significant inter-institutional variability. While clear cases of frank atlanto-occipital dislocation (AOD) are treated with surgical stabilization, the approach for less severe cases of OCDI is not standardized. These patients require a careful assessment of both radiographic and clinical criteria, as part of a complex risk-benefit analysis, to establish whether occipitocervical fusion (OCF) is indicated. Here, we performed a systematic review of the literature that describes traumatic OCDI in children < 18 years of age. SUMMARY: We performed a systematic review, according to PRISMA guidelines, of children < 18 years of age presenting with traumatic etiologies of OCDI. We searched PubMed to identify papers congruent with these criteria. Exclusion criteria included (1) reports on atraumatic causes of OCDI and (2) studies with insufficient clinical and radiographic details on individual patients. We identified 16 reports describing a total of 144 patients treated for pediatric traumatic OCDI. Based on the synthesis of these findings and the collective experience of the authors, we present the demographic, clinical, and radiographic factors that underlie OC instability, which we hope will serve as components of a grading system in the future. We considered various clinical and radiographic findings including: (1) the mechanism of injury, (2) the patient's age, (3) CT/CT angiography of head and neck findings and parameters, (4) MRI findings, and (5) neurological exam, for the purpose of determining the severity of the OCDI and offering treatment guidelines based on the summative risk of underlying OC instability. Key Messages: OCDI is a potentially devastating injury, especially in children. Although missing the diagnosis can have potentially catastrophic consequences, reverting to surgical fixation in less severe cases can subject children to unnecessary operative risk and permanently reduce their range of motion. After reviewing all the available reports of pediatric traumatic OCDI in the neurosurgical literature, we propose an outline of clinical and radiographic factors influencing underlying OC instability that could be incorporated into a grading scale to guide treatment. We hope this study stimulates discussion on the standardization of treatment for pediatric OCDI.

Myelopathy associated with instability consequent to resection of ossification of anterior longitudinal ligament in DISH.

PURPOSE: The presence of prominent OALL (ossification of anterior longitudinal ligament) in the anterior cervical spine has been implicated as a cause of dysphagia. Surgical resection of the OALL is considered effective for the management of diffuse idiopathic skeletal hyperostosis (DISH)-related dysphagia. Although many reports have been published on DISH-related dysphagia, no cases of postoperative cervical instability have been reported thus far. We present a case in which the patient developed myelopathy associated with instability consequent to resection of OALL in DISH. METHODS: A 62-year-old man presented with progressive dysphagia that persisted for a year. The patient's symptoms were successfully resolved by resection of OALL. Five years after the surgery, the dysphagia resurfaced and was found to be caused by the regrowth of the OALL. A repeat surgery was performed, and the dysphagia disappeared. Eleven months after the second surgery, he visited the hospital with progressive quadriparesis and pain in the cervical region. RESULTS: Nine-month follow-up radiologic study revealed cervical instability at the level of C5-6 resulting in myelopathy. The patient underwent decompressive laminectomy and posterior fusion surgery. CONCLUSION: Surgical resection of DISH-related dysphagia typically yields excellent outcomes, but our experience in this case highlights the possibility of OALL regrowth and subsequent cervical instability after resection of OALL.

[A traumatic cervical spine injury in children?] / Comment j'explore Un traumatisme du rachis cervical chez l'enfant.

Cervical spine injuries are a regular reason for consultation in general practice or pediatric emergencies. In many cases, it is difficult to distinguish between whether or not a radiological assessment is necessary. We propose in this article a management strategy based on clinical scores simple to achieve in order to avoid unnecessary radiological examinations for children.

Les traumatismes du rachis cervical sont un motif régulier de consultation en médecine générale ou aux urgences pédiatriques. Bien souvent, il est difficile de faire la part des choses pour savoir si un bilan radiologique est ou non nécessaire. Nous proposons, dans cet article, une prise en charge basée sur des scores cliniques simples à réaliser, permettant d'éviter ainsi des examens radiologiques inutiles pour les enfants.

Do Modic changes affect cervical sagittal alignment and motion in symptomatic patients?

PURPOSE: The cervical segmental instability often occurs simultaneously with Modic changes (MCs). However, it is unknown whether there is a relation between the two diseases. The aim of this study was to evaluate the relationship between MCs and cervical segmental instability, cervical curvature and range of motion (ROM) in the cervical spine. METHODS: A total of 464 patients with neck pain or cervical neurologic symptoms who underwent imaging examination were analyzed retrospectively. Based on MRI imaging cervical MCs were diagnosed, and patients were divided into with or without MCs groups. The cervical curvature and range of motion were measured. We compared the incidence of cervical instability, cervical curvature and ROM between the two group patients and their relationships with MCs were studied. Logistic regression was used to study the risk factors associated with MCs. RESULTS: MCs were observed in 94 of 464 patients and 122 of total 2320 cervical segments and were most frequent at C5-6 segment. The incidence of the cervical instability was significantly higher in patients with MCs than those without MCs at cervical level C3-7. In addition, cervical curvature and ROM in patients with MCs were less than those without MCs. Logistic regression analysis showed that the occurrence of cervical spine instability, less cervical curvature and ROM were risk factors for MCs. CONCLUSIONS: Patients with MCs were prone to have cervical instability at the same cervical level and may have a higher possibility of less cervical curvature and ROM.

Upper cervical instability associated with rheumatoid arthritis: a case report.

Rheumatoid arthritis (RA) affects between 1 and 2 million individuals in the United States. Atlantoaxial instability (AAI) has been shown to have 40-85% prevalence among individuals with RA. Despite the high incidence of craniovertebral involvement, overt symptoms of instability are rare. The high risk of AAI and limited symptomology should increase therapist suspicion of potential contraindications and precautions to initiation of therapy for the cervical spine without prior diagnostic imaging. The purpose of this case study is to describe the historical, clinical, and diagnostic imaging complexity of AAI associated with RA, and to illustrate the use of these factors in the clinical reasoning within a patient case.

NBAS mutations cause a multisystem disorder involving bone, connective tissue, liver, immune system, and retina.

We report two unrelated patients with a multisystem disease involving liver, eye, immune system, connective tissue, and bone, caused by biallelic mutations in the neuroblastoma amplified sequence (NBAS) gene. Both presented as infants with recurrent episodes triggered by fever with vomiting, dehydration, and elevated transaminases. They had frequent infections, hypogammaglobulinemia, reduced natural killer cells, and the Pelger-Huët anomaly of their granulocytes. Their facial features were similar with a pointed chin and proptosis; loose skin and reduced subcutaneous fat gave them a progeroid appearance. Skeletal features included short stature, slender bones, epiphyseal dysplasia with multiple phalangeal pseudo-epiphyses, and small C1-C2 vertebrae causing cervical instability and myelopathy. Retinal dystrophy and optic atrophy were present in one patient. NBAS is a component of the synthaxin-18 complex and is involved in nonsense-mediated mRNA decay control. Putative loss-of-function mutations in NBAS are already known to cause disease in humans. A specific founder mutation has been associated with short stature, optic nerve atrophy and Pelger-Huët anomaly of granulocytes (SOPH) in the Siberian Yakut population. A more recent report associates NBAS mutations with recurrent acute liver failure in infancy in a group of patients of European descent. Our observations indicate that the phenotypic spectrum of NBAS deficiency is wider than previously known and includes skeletal, hepatic, metabolic, and immunologic aspects. Early recognition of the skeletal phenotype is important for preventive management of cervical instability.

Risk factors for cervical instability in rheumatoid arthritis: a meta-analysis.

Introduction: The aim of the study was to evaluate the risk factors for cervical instability in rheumatoid arthritis (RA). Material and methods: Computer searches were conducted in PubMed, Embase, Cochrane Library, the China National Knowledge Infrastructure (CNKI) database, the Wan Fang database, the Chinese Scientific Journal Databases (VIP) database, and the Chinese Biomedical Literature database (CBM) from their establishment until November 2022. Results: A total of 8 articles were included in this study, including 1 cross-sectional study, 5 case-control studies, and 2 cohort study, including 3078 patients with RA. Meta-analysis results showed that: male sex (OR = 1.70, 95% CI: 1.19-2.42), course of disease (OR = 1.72, 95% CI: 1.29-2.28), long-term glucocorticosteroid use (OR = 2.84, 95% CI: 1.97-2.40), Steinbrocker staging (OR = 2.30, 95% CI: 1.61-3.28), disability at baseline (OR = 24.57, 95% CI: 5.51-109.60), peripheral joint destruction (OR = 2.24, 95% CI: 1.56-3.21), Steinbrocker stage I-IV progression to disability (OR = 20.08, 95% CI: 4.18-96.53), and previous joint surgery (OR = 1.54, 95% CI: 1.06-2.26) are the main risk factors for cervical instability in RA. Conclusions: There are many risk factors for cervical instability in RA. In clinical practice, special attention should be paid to patients who are male, have a longer course of disease, have long-term glucocorticosteroid use, have previous joint surgery, have peripheral joint damage, and develop disability in Steinbrocker stage I-IV. Attention should be paid to the high-risk groups mentioned above, and effective measures such as early screening and full monitoring should be taken to prevent the occurrence of cervical instability in RA.

Natural history of craniovertebral abnormalities in a single-center study in 54 patients with Hurler syndrome.

OBJECTIVE: Craniovertebral junction (CVJ) abnormalities are common and well documented in mucopolysaccharidosis type I-Hurler syndrome (MPS IH), often causing severe spinal canal narrowing. However, the requirement for surgical decompression and/or fusion is uncommon. Although hematopoietic cell transplant (HCT) has been shown to prolong the lives of patients with MPS IH, its effect in halting or reversing musculoskeletal abnormalities is less clear. Unfortunately, there are currently no universal guidelines for imaging or indication for surgical interventions in these patients. The goal of this study was to track the progression of the CVJ anatomy in patients with MPS IH following HCT, and to examine radiographic features in patients who needed surgical intervention. METHODS: Patients with MPS IH treated at the University of Minnesota with allogeneic HCT between 2008 and 2020 were retrospectively reviewed. Patients who underwent CVJ surgery were identified with chart review. All MPS IH cervical scans were examined, and the odontoid retroflexion angle, clivoaxial angle (CXA), canal width, and Grabb-Oakes distance (pB-C2) were measured yearly for up to 7 years after HCT. Longitudinal models based on the measurements were made. An intraclass correlation coefficient was used to measure interrater reliability. Nine children without MPS IH were examined for control CVJ measurements. RESULTS: A total of 253 cervical spine MRI scans were reviewed in 54 patients with MPS IH. Only 4 (7.4%) patients in the study cohort required surgery. Three of them had posterior fossa and C1 decompression, and 1 had a C1-2 fusion. There was no statistically significant difference in the spinal parameters that were examined between surgery and nonsurgery groups. Among the measurements, canal width and CXA varied drastically in patients with different neck positions. Odontoid retroflexion angle and CXA tended to decrease with age. Canal width and pB-C2 tended to increase with age. CONCLUSIONS: Based on the data, the authors observed an increase in canal width and pB-C2, whereas the CXA and odontoid retroflexion angle became more acute as the patients aged after HCT. The longitudinal models derived from these data mirrored the development in children without MPS IH. Spinal measurements obtained on MR images alone are not sufficient in identifying patients who require surgical intervention. Symptom monitoring and clinical examination, as well as pathological spinal cord changes on MRI, are more crucial in assessing the need for surgery than is obtaining serial imaging.

Whether the potential degree of cervical instability and cervical muscle degeneration in patients with cervical spondylosis radicular affect the efficacy of cervical traction.

To explore whether the potential instability of the cervical spine and cervical muscle degeneration in patients with cervical spondylotic radiculopathy (CSR) affect the efficacy of cervical traction, and whether cervical traction can aggravate the potential instability of the cervical spine. We divided the 113 recruited CRS patients into three groups based on the differences in horizontal displacement and abnormal angle, and measured the degree of cervical muscle degeneration in the patients through MRI. Considering functional scores, VAS, NDI and PCS scores of the three groups post-treatment were significantly improved. Through the intergroup analysis, we found that the improvement in functional scores in the mild and moderate instability trend groups was better than that in the severe group. Through MRI measurements, we found that the degree of cervical muscle degeneration was significantly increased in the severe instability trend group. Regarding the changes in X-Ray imaging parameters pre- and post-treatment, no significant differences were observed pre- and post-treatment. For patients with CSR, the more serious their predisposition for cervical instability was, the more severe the degree of cervical muscle degeneration was, which means the worse the curative effect was, but cervical traction did not aggravate the potential degree of cervical instability.

Corrigendum: Anatomical variations of the atlas arches: prevalence assessment, systematic review and proposition for an updated classification system.

[This corrects the article DOI: 10.3389/fnins.2024.1348066.].

Anatomical variations of the atlas arches: prevalence assessment, systematic review and proposition for an updated classification system.

Objective and background: This study focuses on the atlas, a pivotal component of the craniovertebral junction, bridging the cranium and spinal column. Notably, variations in its arches are documented globally, necessitating a thorough assessment and categorization due to their significant implications in clinical, diagnostic, functional, and therapeutic contexts. The primary objective is to ascertain the frequency of these anatomical deviations in the atlas arches among a Colombian cohort using cone-beam computed tomography (CBCT). Methodology: Employing a descriptive, cross-sectional approach, this research scrutinizes the structural intricacies of the atlas arches in CBCT scans. Analytical parameters included sex distribution and the nature of anatomical deviations as per Currarino's classification. Statistical analyses were conducted to identify significant differences, including descriptive statistics and Chi-square tests. A systematic review of the literature was conducted in order to enhance the current Currarino's classification. Results: The study examined 839 CBCT images, with a nearly equal sex distribution (49.7% female, 50.3% male). Anatomical variations were identified in 26 instances (3%), displaying a higher incidence in females (X2 [(1, N = 839) = 4.0933, p = 0.0430]). The most prevalent variation was Type A (2.5%), followed by Type B (0.4%), and Type G (0.2%) without documenting any other variation. The systematic review yielded 7 studies. A novel classification system for these variations is proposed, considering global prevalence data in the cervical region. Conclusion: The study highlights a statistically significant predominance of Type A variations in the female subset. Given the critical nature of the craniovertebral junction and supporting evidence, it recommends an amendment to Currarino's classification to better reflect these clinical observations. A thorough study of anatomical variations of the upper cervical spine is relevant as they can impact important functional aspects such as mobility as well as stability. Considering the intricate anatomy of this area and the pivotal function of the atlas, accurately categorizing the variations of its arches is crucial for clinical practice. This classification aids in diagnosis, surgical planning, preventing iatrogenic incidents, and designing rehabilitation strategies.

Co-occurrence of tethered cord syndrome and cervical spine instability in hypermobile Ehlers-Danlos syndrome.

The Ehlers-Danlos Syndromes (EDS) represent a group of hereditary connective tissue disorders, with the hypermobile subtype (hEDS) being the most prevalent. hEDS manifests with a diverse array of clinical symptoms and associated comorbidities spanning the musculoskeletal, neurological, gastrointestinal, cardiovascular, and immunological systems. hEDS patients may experience spinal neurological complications, including cervico-medullary symptoms arising from cranio-cervical and/or cervical instability/hypermobility, as well as tethered cord syndrome (TCS). TCS is often radiographically occult in nature, not always detectable on standard imaging and presents with lower back pain, balance issues, weakness in the lower extremities, sensory loss, and bowel or bladder dysfunction. Cervical instability due to ligament laxity can lead to headaches, vertigo, tinnitus, vision changes, syncope, radiculopathy, pain, and dysphagia. TCS and cervical instability not only share clinical features but can also co-occur in hEDS patients, posing challenges in diagnostics and clinical management. We present a review of the literature and a case study of a 20-year-old female with hEDS, who underwent surgical interventions for these conditions, highlighting the challenges in diagnosing and managing these complexities and underscoring the importance of tailored treatment strategies to improve patient outcomes.

Safe intubation in Morquio-Brailsford syndrome: A challenge for the anesthesiologist.

Morquio-Brailsford syndrome is a type of mucopolysaccharidoses. It is a rare disease with features of short stature, atlantoaxial instability with risk of cord damage, odontoid hypoplasia, pectus carinatum, spine deformities, hepatomegaly, and restrictive lung disease. Neck movements during intubation are associated with the risk of quadriparesis due to cervical instability. This, along with the distortion of the airway anatomy due to deposition of mucopolysaccharides makes airway management arduous. We present our experience in management of difficult airway in a 3-year-old girl with Morquio-Brailsford syndrome posted for magnetic resonance imaging and computerized tomography scan of a suspected unstable cervical spine. As utmost sagacity during intubation is required, the child was intubated inside operation theatre in the presence of experienced anesthesiologists and then shifted to the peripheral location. Intubation was done with an endotracheal tube railroaded over a pediatric fibreoptic bronchoscope passed through the lumen of a classic laryngeal mask airway, keeping head in neutral position.

[Analysis of clinical efficacy and risk factors of relapse in young patients with cervical instability treated by manipulation].

OBJECTIVE: To determine the clinical efficacy of different manipulation in the treatment of cervical instability in young people, and to analyze the risk factors of relapse of cervical instability in young people. METHODS: From March 2021 to June 2022, the clinical data of 120 young patients with cervical instability were retrospectively analyzed. According to the different treatment methods, they were divided into rotation group (60 cases, 3 cases of loss) and tendon group (60 cases, 5 cases of loss). There were 25 males and 32 females in rotation-traction manipulation group;age ranged from 22 to 44 years old with a median of 28 years old;course of disease ranged from 0.17 to 120 months with amedian of 22 months. There were 22 males and 33 females in tendon-regulating manipulation group;age ranged from 21 to 42 years old with a median of 27 years old;course of disease ranged from 0.23 to 180 months with a median of 24 months. Both groups were treated for 2 weeks, once every other day for 7 times, and were followed up for 1 year. The clinical efficacy of the two groups was evaluated, and the visual analogue scale (VAS), neck disability index (NDI) were observed before and after treatment. One year after the course of treatment, patients with effective treatment were followed up to make statistics on recurrence. Patients with recurrence were included in the recurrence group, while those without recurrence were included in the non-recurrence group. Factors that may affect symptom recurrence were analyzed, and univariate and multivariate Logistic regression analysis were performed. RESULTS: The 13 patients who failed the treatment (4 cases in the rotation-traction manipulation group and 9 cases in the tendon-regulating manipulation group) were not followed up. All the 99 patients who were effective in treatment were followed up ranged from 303 to 406 days with a median of 359 days. No complications occurred in all patients. There were significant differences in VAS and NDI between the two groups after treatment and before treatment (P<0.05), and there were significant differences in VAS and NDI between the two groups after treatment (P<0.05). Ninety-nine patients achieved follow-up, 56 (56.57%) relapsed and 43 (43.43%) did not. Univariate correlation analysis showed that NDI index, the time spent at the desk every day, the time spent using electronic products every day and angular displacement of anterior flexion before treatment in the relapse group were significantly higher than those in the non-relapse group (P<0.05). Logistic regression analysis showed that the time spent at the desk every day [OR=2.447, 95%CI(1.255, 4.771)], the time spent using electronic products every day [OR=1.892, 95%CI(1.066, 3.358)] and the angular displacement of anterior flexion of the cervical before treatment [OR=1.246, 95%CI(1.045, 1.485) ]were the risk factors for relapse. CONCLUSION: Both rotation-traction manipulation and tendon-regulating manipulation can effectively treat cervical instability in young people, and rotation-traction manipulation has more advantages than tendon-regulating manipulation in improving cervical pain and cervical dysfunction in patients. The time spent at the desk every day, the time spent using electronic products every day, and the increase of cervical flexion angle displacement will increase the risk of relapse in patients.