scholarly journals Mucopolysaccharidoses and Orthopedic Management (Focused also on Craniocervical Junction)

2018 ◽  
Vol 08 (01) ◽  
pp. e128-e137
Author(s):  
Christina Lampe ◽  
Christian Lampe
Keyword(s):  
Spinal Cord ◽  
Soft Tissues ◽  
Surgical Intervention ◽  
Cervical Spinal Cord ◽  
Joint Stiffness ◽  
Craniocervical Junction ◽  
Musculoskeletal Symptoms ◽  
Atlantoaxial Instability ◽  
Enzyme Replacement ◽  
Life Threatening

AbstractMucopolysaccharidoses (MPSs) are multisystemic, chronic progressive, heterogeneous, and life-threatening diseases, involving severely the musculoskeletal system, in particular in MPS I, II, IV, VI, VII, and less prevalent in MPS III. Accumulation of glycosaminoglycans (GAGs) in soft tissues, such as ligaments, tendons, and joint capsules, as well as in cartilage, and bone lead to orthopedic complications: joint stiffness, contractures, and skeletal deformities, resulting in hip dysplasia, genua valga, feet deformities, kyphoscoliosis, narrowing of the spinal canal, atlantoaxial instability, carpal tunnel syndrome, trigger finger, and growth retardation. These complications significantly reduce the quality of life due to impaired mobility, loss of independency, and pain. Compression of the cervical spinal cord is also life threatening. According to the progressive nature of MPSs, musculoskeletal symptoms worsen over time and surgical intervention may be inevitable. However, due to the increased anesthesia risk of MPSs, surgical intervention has to be evaluated carefully by a multidisciplinary team. Additionally, due to the rarity of the diseases, not many standards or recommendations are available to determine the indication of a surgical intervention, so each intervention must be decided individually, based on the few data available. In addition, conservative treatment should be taken into consideration. Physiotherapy, pain medication, insoles, orthosis, braces, corsets, and special footwear play a pivotal role. Unfortunately, the skeletal tissues are poorly vascularized and enzyme replacement therapy does not have much effect on them. It is important to detect and observe musculoskeletal complications in the regular follow-up visits, in particular the life-threatening compression of the cervical spinal cord.

CERVICAL CONGENITAL MIDLINE MENINGOCELES IN ADULTS

Neurosurgery ◽  
2008 ◽  
Vol 63 (5) ◽  
pp. 938-945 ◽  
Author(s):  
Bulent Duz ◽  
Erhan Arslan ◽  
Engin Gönül
Keyword(s):  
Spinal Cord ◽  
Cerebrospinal Fluid ◽  
Soft Tissues ◽  
Cervical Spinal Cord ◽  
Surgical Techniques ◽  
Cerebrospinal Fluid Leak ◽  
Neurological Deterioration ◽  
Neurological Deficits ◽  
Cervical Lesions ◽  
Flexion Extension

Abstract OBJECTIVE The clinical presentation, biomechanical evaluation, and surgical techniques for repairing cervical meningoceles in adulthood are presented. Cervical meningoceles are typically diagnosed in childhood and are rarely reported among spinal dysraphic lesions in adulthood. In most cases, the cervical spinal cord is found tethered to the dura and other soft tissues by fibrous or fibroneural elements. Cervical lesions, unlike those that arise more caudally, rarely leak cerebrospinal fluid. METHODS We report 5 male patients with meningoceles, aged 20 to 22 years (mean age, 20.4 years), in whom the primary evolution of the lesion occurred between 1999 and 2006. RESULTS All 5 patients presented to the hospital with cervical pain and mass. One patient had had a cerebrospinal fluid leak from the center of the lesion intermittently since birth. Another patient presented with neurological deficits and hypesthesia of the left hand. All patients underwent surgery. The lesion was excised, a partial laminectomy was performed, the internal tethering fibrous bands were released, and the neck of the structure was ligated. There was no neurological deterioration after surgery. No postoperative complications were observed during the 12-month follow-up period for each patient. CONCLUSION The goals of surgical exploration of these lesions are prevention of neurological deterioration, prevention of infection, and acceptable cosmetic outcome. Cervical meningoceles are tethering lesions of the spinal cord that may cause biomechanical injury with repetitive flexion-extension movements of the head and spine. It is therefore advisable to remove these lesions neurosurgically as soon as the diagnosis is made. Surgical intervention for cervical meningoceles should consist of exploration of the intradural fibrous bands with laminectomy, untethering of the spinal cord, and resection of the stalk and cyst of the meningocele.

scholarly journals Surgical Management of Spinal Disorders in People with Mucopolysaccharidoses

2020 ◽  
Vol 21 (3) ◽  
pp. 1171
Author(s):  
Hidetomi Terai ◽  
Hiroaki Nakamura
Keyword(s):  
Surgical Management ◽  
Bone Growth ◽  
Soft Tissues ◽  
Atlantoaxial Instability ◽  
Lysosomal Storage ◽  
Hematopoietic Stem ◽  
Spinal Disorders ◽  
Enzyme Replacement ◽  
Therapeutic Modalities ◽  
Canal Stenosis

Mucopolysaccharidoses (MPS) are a group of inherited, multisystem, lysosomal storage disorders involving specific lysosomal enzyme deficiencies that result in the accumulation of glycosaminoglycans (GAG) secondary to insufficient degradation within cell lysosomes. GAG accumulation affects both primary bone formation and secondary bone growth, resulting in growth impairment. Typical spinal manifestations in MPS are atlantoaxial instability, thoracolumbar kyphosis/scoliosis, and cervical/lumbar spinal canal stenosis. Spinal disorders and their severity depend on the MPS type and may be related to disease activity. Enzyme replacement therapy or hematopoietic stem cell transplantation has advantages regarding soft tissues; however, these therapeutic modalities are not effective for bone or cartilage and MPS-related bone deformity including the spine. Because spinal disorders show the most serious deterioration among patients with MPS, spinal surgeries are required although they are challenging and associated with high anesthesia-related risks. The aim of this review article is to provide the current comprehensive knowledge of representative spinal disease in MPS and its surgical management, including the related pathology, symptoms, and examinations.

scholarly journals Synovial Cyst of the Atlantoaxial Joint Removed through a Posterior Intradural Approach

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Atsuhiko Toyoshima ◽  
Kiminori Sakurai ◽  
Nobuhiro Sasaki ◽  
Miyuki Fukuda ◽  
Shigeo Ueda ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Spinal Cord ◽  
Synovial Cyst ◽  
Cord Compression ◽  
Atlantoaxial Instability ◽  
Rapid Progression ◽  
Atlantoaxial Joint ◽  
Tumor Lesion ◽  
One Year ◽  
The Right

Introduction. Synovial cysts rarely develop in the atlantoaxial joint. We report a case of posterior C1-2 laminectomy for a synovial cyst of the atlantoaxial joint which passed through the dorsal dura and put pressure on the cervical spinal cord. Case Presentation. A 62-year-old man with rapid progression of pain and weakness in the left upper extremity presented to our hospital. A cervical spine X-ray showed left C5-6 and C6-7 stenoses. A cervical magnetic resonance imaging showed an intradural extramedullary cystic lesion on the right side of the ventral cervical spinal cord at the C1-2 level and severe compression of the cervical spinal cord. Because a cyst was partially enhancing, a tumor lesion was not identifiable. Due to severe spinal cord compression, we performed intradural cyst removal via a posterior intradural approach with C1-2 laminectomy and left-sided C5-6 and C6-7 foraminotomies. One year after surgery, the cyst did not recur, and atlantoaxial instability did not appear. Discussion. A compressive lesion on the cervical spinal cord was not identified preoperatively as a synovial cyst. However, intraoperative and pathological findings suggested that the compressive lesion can be a synovial cyst which passed through the dorsal dura. The surgical treatment strategy for a synovial cyst of the atlantoaxial joint is controversial due to factors, such as the presence of atlantoaxial instability, level of cyst causing compression of the cervical spinal cord, severity of myelopathy, and cyst location. In the present study, the cervical spinal cord was highly compressed and the cyst was located on the right side of the cervical spinal cord; we chose cyst removal through a posterior intradural approach with C1-2 laminectomy.

Acute Traumatic Cervical Central Cord Syndrome—Is Early Intervention Justified?

2018 ◽  
Vol 15 (02/03) ◽  
pp. 094-099
Author(s):  
Rahul Saha ◽  
Sandip Chatterjee
Keyword(s):  
Spinal Cord ◽  
Surgical Intervention ◽  
Cervical Spinal Cord ◽  
Randomized Study ◽  
Japanese Orthopaedic Association ◽  
Fracture Dislocation ◽  
Spine Injury ◽  
Disc Prolapse ◽  
Central Cord Syndrome ◽  
Early Surgical Intervention

AbstractThe clinical features suggestive of traumatic central cord syndrome (TCCS) were presented by Thorburn in his publication in 1887. Later in 1954, Schneider first mentioned the involvement of central cervical spinal cord after an acute cervical spine injury and described it as TCCS. This is generally caused due to an extreme hyperextension injury without vertebral damage and secondary to anteroposterior pinching or squeezing of the spinal cord. There exists a lot of controversy whether early surgical intervention is justified in these patients. It is to assess our own results that we decided to review 40 such cases treated by the senior author in the past 8 years. Twenty-four of these were managed with early surgical intervention (which we defined as within 48 hours), and 16 were treated conservatively. In all cases, the existence of fracture-dislocation, disc prolapse, as well as preoperative and postoperative Japanese Orthopaedic Association (JOA) scores and exact motor and sensory deficit were recorded. The numbers are too few, but there was definite evidence that patients with disc prolapse and fracture dislocation did better with surgery than with conservative treatment. Those with grade 0 power in the upper limbs however did significantly better with surgical treatment. Those with advanced pre-existing cervical spondylotic changes did better than those without these premorbid changes. However, the numbers are too small for meaningful statistical analysis. This is a retrospective study conducted as a pilot study to plan for a future randomized study, which is very necessary in our clinical practice. Currently, we still quote the pros and cons of surgical intervention to the relatives and go by their choice.

scholarly journals Early surgical intervention may facilitate recovery of cervical spinal cord injury in DISH

2019 ◽  
Vol 27 (1) ◽  
pp. 230949901983478 ◽  
Author(s):  
Osahiko Tsuji ◽  
Kota Suda ◽  
Masahiko Takahata ◽  
Satoko Matsumoto-Harmon ◽  
Miki Komatsu ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Surgical Intervention ◽  
Cervical Spinal Cord ◽  
Cervical Spinal Cord Injury ◽  
Cord Injury ◽  
Early Surgical Intervention

scholarly journals Hydatid cyst in the cervical spinal cord complicated by potentially life-threatening difficult airway: a case report

2020 ◽  
Vol 70 (5) ◽  
pp. 553-555
Author(s):  
Dmitriy Viderman ◽  
Aisa Nurpeisov ◽  
Omirzhan Balabayev ◽  
Yermek Urunbayev ◽  
Guilherme de Almeida ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Case Report ◽  
Hydatid Cyst ◽  
Difficult Airway ◽  
Cervical Spinal Cord ◽  
Life Threatening

scholarly journals Emergency surgery for traumatic spinal cord injury in a secondary hospital: A case report

2021 ◽  
Vol 12 ◽  
pp. 375
Author(s):  
Tedy Apriawan ◽  
Pandu Wicaksono ◽  
Rizki Meizikri ◽  
Eko Agus Subagio
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Emergency Surgery ◽  
Cervical Spinal Cord ◽  
Respiratory Arrest ◽  
Cord Injury ◽  
Threatening Condition ◽  
Tertiary Hospitals ◽  
Life Threatening ◽  
Secondary Hospital

Background: Cervical spinal cord injury (SCI) is a life-threatening condition. Prompt surgical intervention is needed to avoid hemodynamic and respiratory catastrophe. In Indonesia, however, spine surgery is more common in tertiary hospitals and thus might prolong the time gap to surgery due to referral waiting time. Case Description: We performed an emergency surgery for a patient with complete SCI due to unstable cervical fracture. The patient was in spinal shock and experienced respiratory arrest after radiological workup. Stability was achieved in the ICU and patient was directly sent to operating theater. Anterior-posterior approach was chosen to decompress and stabilize the cervical spine. The patient was discharged on postoperative day 17 and was seen well at 1-month follow-up. Conclusion: The capability to perform spine procedures should not be exclusive to tertiary hospitals in Indonesia. Satisfying results can be achieved with the presence of capable neurosurgeons or orthopedic surgeons and anesthesiologists in lower-level hospitals.

scholarly journals Motor unit number index detects the effectiveness of surgical treatment in improving distal motor neuron loss in patients with incomplete cervical spinal cord injury

2020 ◽  
Author(s):  
Jun Li ◽  
Yancheng Zhu ◽  
Yang Li ◽  
ShiSheng He ◽  
Deguo Wang
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Surgical Treatment ◽  
Muscle Strength ◽  
Motor Neurons ◽  
Surgical Intervention ◽  
Cervical Spinal Cord ◽  
Injury Site ◽  
Cord Injury ◽  
Motor Neuron Loss

Abstract Background Recovery of motor dysfunction is important for patients with incomplete cervical spinal cord injury (SCI). To enhance the recovery of muscle strength, both research and treatments mainly focus on injury of upper motor neurons at the direct injury site. However, accumulating evidences have suggested that SCI has a downstream effect on the peripheral nervous system, which may contribute to the poor improvement of the muscle strength after operation. The aim of this study is to investigate the impact of early vs. delayed surgical intervention on the lower motor neurons (LMNs) distal to the injury site in patients with incomplete cervical SCI. Methods Motor unit number index (MUNIX) was performed on the tibialis anterior (TA), extensor digitorum brevis (EDB) and abductor hallucis (AH) in 47 patients with incomplete cervical SCI (early vs. delayed surgical-treatment: 17 vs. 30) and 34 healthy subjects approximately 12 months after operation. All patients were further assessed by American spinal injury association (ASIA) motor scales and Medical Research Council (MRC) scales. Results There are no difference of both ASIA motor scores and MRC scales between the patients who accepted early and delayed surgical treatment (P > 0.05). In contrast, the patients undergoing early surgical treatment showed lower MUSIX values in both bilateral EDB and bilateral TA, along with greater MUNIX values in both right-side EDB and right-side TA, compared to the patients who accepted delayed surgical treatment (P < 0.05). Conclusions Cervical SCI has a negative effect on the LMNs distal to the injury site. Early surgical intervention in Cervical SCI patients may improve the dysfunction of LMNs distal to the injury site, reducing secondary motor neuron loss, and eventually improving clinical prognosis.

Remarkable reduction or disappearance of retroodontoid pseudotumors after occipitocervical fusion

2006 ◽  
Vol 5 (2) ◽  
pp. 156-160 ◽  
Author(s):  
Ikuko Yamaguchi ◽  
Sei Shibuya ◽  
Nobuo Arima ◽  
Shiro Oka ◽  
Yoshiaki Kanda ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Spinal Cord ◽  
Symptomatic Improvement ◽  
Cord Compression ◽  
Magnetic Resonance Images ◽  
Mass Lesion ◽  
Atlantoaxial Instability ◽  
Occipitocervical Fusion ◽  
Upper Cervical ◽  
Posterior Laminectomy

✓ Retroodontoid or periodontoid pseudotumor unassociated with rheumatoid arthritis or hemodialysis is clinically rare. The authors report three cases of retroodontoid pseudotumor that they treated surgically. All patients exhibited myelopathy of the upper cervical spinal cord. Plain radiography depicted atlantoaxial instability in two of the three patients. Spinal cord compression caused by a mass lesion in all patients was clearly demonstrated on magnetic resonance images. In two patients, the mass lesion was not limited to the retroodontoid region and expanded continuously to the cranial base. Posterior laminectomy of the atlas and occipitocervical fusion were performed. After surgery, the pseudotumor disappeared in two cases and was clearly reduced in one case, and neurological symptoms also improved. Retroodontoid pseudotumor is a lesion for which symptomatic improvement can be expected with posterior decompression and fusion, even without direct tumor excision.

scholarly journals Respiratory Training and Plasticity After Cervical Spinal Cord Injury

2021 ◽  
Vol 15 ◽  
Author(s):  
Margo Randelman ◽  
Lyandysha V. Zholudeva ◽  
Stéphane Vinit ◽  
Michael A. Lane
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injuries ◽  
Cervical Spinal Cord ◽  
Neural Pathways ◽  
Functional Deficits ◽  
Non Invasive ◽  
Cord Injury ◽  
Life Threatening ◽  
Respiratory Training

While spinal cord injuries (SCIs) result in a vast array of functional deficits, many of which are life threatening, the majority of SCIs are anatomically incomplete. Spared neural pathways contribute to functional and anatomical neuroplasticity that can occur spontaneously, or can be harnessed using rehabilitative, electrophysiological, or pharmacological strategies. With a focus on respiratory networks that are affected by cervical level SCI, the present review summarizes how non-invasive respiratory treatments can be used to harness this neuroplastic potential and enhance long-term recovery. Specific attention is given to “respiratory training” strategies currently used clinically (e.g., strength training) and those being developed through pre-clinical and early clinical testing [e.g., intermittent chemical stimulation via altering inhaled oxygen (hypoxia) or carbon dioxide stimulation]. Consideration is also given to the effect of training on non-respiratory (e.g., locomotor) networks. This review highlights advances in this area of pre-clinical and translational research, with insight into future directions for enhancing plasticity and improving functional outcomes after SCI.

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