Segmental neurofibromatosis with compression of the spinal cord at the cervical level. Literature review and case report

Background. Neurofibromatosis is a fairly rare disease (1/3000). In 1992, V. Riccardi described seven types of neurofibromatosis. Segmental neurofibromatosis (sh), also known as type V neurofibromatosis, is an extremely rare variant characterized by the development of typical cutaneous manifestations or one body segment neurofibromas. Clinical case. Currently, the literature describes about 100 cases of sh and only one of them with compression of the spinal cord. We present our first case of this nosological form with spinal cord compression in a Russian patient. A 70-year-old patient, due to an increasing paresis in the left extremities, underwent mri of the cervical spine, which revealed solid tumors located extramedullary intra-extradurally at the level of c2-c3 vertebrae with pronounced compression of the spinal cord. At the time of hospitalization, clinical presentation was characterized by deep spastic tetraparesis (1–2 points), impairment of all types of sensitivity from the c4 level by the conductive type, and dysfunction of the pelvic organs by the type of delay. Karnofsky index was 50 %, 2 points on the Fim scale. Standard c2-c3 vertebrae laminectomy was performed. Spinal cord compression was eliminated due to the removal of intradural tumors. Subsequently, extradural tumors were removed step by step. On histological examination, tumors were represented by intertwining bundles of elongated schwann cells with wavy nuclei with pointed ends and ileogenic fibers. Mucin present in the stroma separated cells and fibers. Conclusion. Sn is a rare type of neurofibromatosis. However, from the point of view of genetics, it is most likely incorrect to attribute it to a separate type of neurofibromatosis, since the cause of its development is mosaicism of somatic cells due to mutation of the NF 1 gene. Sn is rarely manifested by the development of spinal nerves multiple neurofibromas, however, it can be accompanied by a gross neurological deficit caused by compression of the spinal cord such neurofibromas. Surgical treatment is based on basic and special surgical principles that determine the anatomical and morphological characteristics of the area of intervention, the compliance of which allows for good treatment results.

Extradural thoracic nerve root hemangioblastoma approached by a combined posterior thoracic spine and video-assisted thoracoscopic surgery: A case report

Background: Hemangioblastomas commonly occur in the posterior fossa and are typically attributed to sporadic or familial Von Hippel–Lindau disease. Spinal hemangioblastomas, found in 7–10% of patients, are usually located within the cord (i.e., intramedullary). Here, a 58-year-old male presented with a purely extradural hemangioblastoma involving a spinal root that was surgically excised. Case Description: A 58-year-old male was admitted with a progressive paraparesis and incomplete sensory deficit. The magnetic resonance imaging documented a solid dumbbell-shaped lesion that extended through the left T3-T4 foramen resulting in nerve root and spinal cord compression. Following arterial embolization and lesion excision by both neurosurgeons and thoracic surgeons, the patient’s deficits improved. The postoperative computed tomography scan documented complete tumor removal, and the neuropathology revealed a hemangioblastoma. Conclusion: Here, we describe a 58-year-old male with a purely extradural thoracic foraminal T3-T4 dumbbell-shaped hemangioblastoma successfully treated by both embolization and surgical excision.

HISTORY OF EVOLUTION OF POSTERO-LATERAL APPROACHES TO THE THORACIC SPINE: FROM CURE OF POTT’S DISEASE TO EPIDURAL TUMOR RESECTION

Since the end of the nineteenth century, the wide dissemination of Pott’s disease has ignited debates about which should be the ideal route to perform ventrolateral decompression of the dorsal rachis in case of paraplegia due to spinal cord compression in tuberculosis spondylitis. It was immediately clear that the optimal approach should be the one minimizing the surgical manipulation on both neural and extra-neural structures, while optimizing the exposure and surgical maneuverability on the target area. The first attempt was reported by Victor Auguste Menard in 1894, who described, for the first time, a completely different route from traditional laminectomy, called costotransversectomy. The technique was conceived to drain tubercular paravertebral abscesses causing paraplegia without manipulating the spinal cord. Over the following decades many other routes have been described all over the world, thus demonstrating the wide interest on the topic. Surgical development has been marked by the new technical achievements and by instrumental/technological advancements, until the advent of portal surgery and endoscopy-assisted techniques. Authors retraced the milestones of this history up to the present days, through a systematic review on the topic.

Hydrated Nucleus Pulposus Extrusion in Dogs: Thoracolumbar Compared to Cervical Cases

Objective The aim of this study was to review and describe cases of thoracolumbar (TL) hydrated nucleus pulposus extrusion (HNPE) diagnosed with magnetic resonance imaging and surgery, and compare them to cases of cervical (C) HNPE. Study Design Retrospective, single-center study. Results Thirty-six dogs met the inclusion criteria. Fifteen cases were C and 21 TL. Thirteen dogs were chondrodystrophic breeds, mean body weight was 13 kg, median age was 7.5 years, and 30/36 were male. Fewer dogs were chondrodystrophic in the C group compared with the TL group (p = 0.022). More than 90% had an acute onset, and strong activity was more often reported in the TL group. TL HNPE was more often painful, and extruded disc material more often lateralized (p = 0.017). Median Modified Frankel Score at presentation was 3 and 72.2% were non-ambulatory. More TL HNPE (11/21) were treated surgically compared with C HNPE (4/15). Treatment choice was correlated with spinal cord compression (p = 0.0075). Median Modified Frankel Score improved during hospitalization (p = 0.002) and there was no difference in outcome between C and TL HNPE or conservative and surgical treatment. Mean follow-up time was 33 days. All patients were ambulatory at follow-up. Conclusion This study suggests that the HNPE is not limited to the C vertebral column of dogs and can occur in the TL vertebral column as well. Dogs with TL HNPE show spinal hyperesthesia more often and extruded nucleus material is more often lateralized. Outcome is similar to what has previously been described for C HNPE.

Scoping review of existing funding sources for DCM research: opportunities and challenges for targeting the AO Spine RECODE-DCM research priorities (Preprint)

BACKGROUND Degenerative Cervical Myelopathy (DCM) is a common, disabling condition of symptomatic cervical spinal cord compression that requires significant research advances to improve patient outcomes. AO Spine RECODE-DCM recently identified the top research priorities for DCM. To effectively address these priorities, appropriate funding of DCM research is essential. OBJECTIVE This review characterises current funding in DCM research to consider its significance and highlight future opportunities. METHODS A systematic review of Web of Science for “cervical” AND “myelopathy” was conducted. Papers exclusively studying DCM, with declared funding, and published between January 1, 1995 and March 21, 2020 were considered eligible. Funding sources were classified by country of origin and organisation type. A grant search was also conducted using Dimensions.ai (Digital Science Ltd, London, United Kingdom). RESULTS A total of 621 papers were included, with 300 unique funding bodies. The top funders were AO Spine (n=87), National Institutes of Health, USA (n=63) and National Natural Science Foundation, China (n=63). The USA (n=242) funded the most DCM research, followed by China (n=209) and Japan (n=116). Funding in the USA was primarily provided by corporate or non-profit organisations (60.3%); in China, by institutions (99.5%). Dimensions.ai data showed 180 DCM research grants explicitly awarded, with a total value of US$45.6 million since 1996. CONCLUSIONS DCM funding appears to be predominantly from USA, China and Japan, aligning with areas of high DCM research activity and underpinning the importance of funding to increasing research capacity. The existing funding sources differ from medical research in general, representing opportunities for future investment in DCM.

The Importance of Lamina Size Measurement and Proper Implants Selection before Laminoplasty : Two Case Reports

Open door laminoplasty using plates is a safe and effective procedure for multi-level cord compression. To achieve stable laminar arch, various types of plate have been developed and used. Now, we introduce two rare complications related to the laminar shelf of plate. In the first case, we used the wider laminar shelf plate because the elevated lamina did not fit well into the usual laminar shelf. During follow-up, cord compression due to laminar shelf was observed. And in the second case, the laminar shelf of plate did not fit into the elevated lamina, so we inserted it with a little bit of force. But the patient’s symptom was not improved. On CT image, the inner cortical bone of the lamina was fractured. To prevent these complications, surgeons need to consider the thickness of the lamina and the size of the laminar shelf before surgery.

Management and outcome of spontaneous cervical epidural hematoma: Case report and review of the literature

Spontaneous spinal epidural hematomas are rare and potentially disabling neurological emergencies. Its lead to devastating neurologic outcomes and most patient does not recover completely. The clinical presentation is diverse and includes a severe acute attack, radiating pain at the back, interscapular, or neurological deficits. We report a case of a young woman, 24-year-old, that was admitted to our department for sudden non-traumatic cervical spinal cord compression syndrome (Type A of the American Spinal Cord Injury Association “ASIA A”) including intense cervical back pain, sensory loss, and tetraplegia. Her past medical history was unremarkable. The MRI confirmed a cervical mass responsible for the spinal cord compression and the emergent surgical intervention allow us to evacuate acute C3-C7 hematoma. The patient never recovers from the neurologic deficit despite the emergent management of her case followed by functional musculoskeletal rehabilitation for two years.

Editorial Comment on the Article by Mushkin M.A. et al. “Effect of Prehospital Pause on the Outcomes of Emergency Decompression and Stabilization Procedures in Patients with Tumor and Infectious Spine Diseases”

The editorial comment evaluates the current state of issue of medical care at urgent states caused by pathological vertebral fractures and spinal cord compression in patients with spine destructive diseases. The rare occurrence of pathology and the deficiency of objective data determine the lack of consensus on the medical care for this category of patients. The article by M.A. Mushkin et al helps to understand how the prehospital pause affects the outcomes of emergency decompression and stabilization procedures in patients with tumor and infectious diseases of the spine, as well as to determine how much time the surgeon has at his disposal. The author of the comment believes that close cooperation of orthopedic surgeons, neurosurgeons, oncologists is necessary to solve this problem. Even despite the absence of oncologists in the staff of emergency hospitals, telemedicine allows to receive methodological support in a timely manner for making a correct decision for each patient. Interaction between clinicians, radiologists and pathologists is equally important. An important aspect is continuity in patient care. An urgently performed spinal cord decompression is only a stage of the complex treatment. Such patients should be provided with accurate routing depending on the diagnosis and the treatment early outcome.