Dural Reconstruction Materials for the Repairing of Spinal Neoplastic Cerebrospinal Fluid Leaks.

Spinal tumors often lead to more complex complications than other bone tumors. Nerve injuries, dura mater defect, and subsequent cerebrospinal fluid (CSF) leakage generally appear in spinal tumor surgeries and are followed by serious adverse outcomes such as infections and even death. The use of suitable dura mater replacements to achieve multifunctionality in fluid leakage plugging, preventing adhesions, and dural reconstruction is a promising therapeutic approach. Although there have been innovative endeavors to manage dura mater defects, only a handful of materials have realized the targeted multifunctionality. Here, we review recent advances in dura repair materials and techniques and discuss the relative merits in both preclinical and clinical trials as well as future therapeutic options. With these advances, spinal tumor patients with dura mater defects may be able to benefit from novel treatments.

Minimally Invasive Surgery for Spinal Metastasis: A Review.

OBJECTIVE: Minimally invasive surgery (MIS) techniques have advanced the treatment of metastatic diseases to the spine. The objective of this review is to describe clinical outcomes, benefits, and complications of these techniques. METHODS: All relevant clinical studies describing the role of MIS, computer-assisted navigation (CAN), robot-assisted (RA) procedures, and laser interstitial thermal therapy (LITT) in the treatment of metastatic spine diseases were identified from PubMed, MEDLINE, and relevant article bibliographies. RESULTS: For MIS articles, we filtered 1480 results and identified 26 studies. For CAN, we searched 464 articles to identify 18 articles for review. For RA, we searched 321 results to identify 7 studies for review. For LITT, we identified 21 articles for review. CONCLUSIONS: MIS for the treatment of spine metastasis has significant potential benefits in reducing surgical site infections, hospital stay, and blood loss without compromising instrument accuracy or overall outcomes. Overall, MIS and its adjuncts have the potential to reduce the risks involved in the treatment of patients with metastatic disease to the spinal column without compromising the benefits of decompression and stabilization of the spine.

Novel clinical phenotype of generalised lymphatic dysplasia in a neonate: a missed diagnosis.

We report a preterm neonate who had a large cervical cystic hygroma and right chylothorax. She was operated on day-21 and a near-complete resection of cystic hygroma was done. She developed refractory hypoxemia and shock post surgery and died after 24 hours. During autopsy, the chest cavity was found to be filled with chyle. Histopathological examination showed dilated lymphatics in the pleura, hepatic capsule, serosa of stomach and intestines, peri-pancreatic regions, peri-renal capsule and peri-adrenal tissues suggestive of generalised lymphatic dysplasia. Clinical exome sequencing did not reveal any pathogenic mutation in the genes involved in primary lymphatic dysplasia, noonan syndrome or rasopathies.

Prevalence of extramedullary hematopoiesis, renal cysts, splenic and hepatic lesions, and vertebral hemangiomas among thalassemic patients: a retrospective study from the Myocardial Iron Overload in Thalassemia (MIOT) network.

We determined the prevalence of incidental extracardiac findings (IEF) at Magnetic Resonance Imaging (MRI) potentially related to anemia and hypoxia in age- and sex-matched populations (N = 318) with thalassemia major (TM) and thalassemia intermedia (TI) enrolled in the Myocardial Iron Overload in Thalassemia network. Overall, IEFs were detected in 33.3% and 25.8% of patients with TI and TM, respectively (P = 0.114). TI and TM patients had elevated but comparable prevalence of renal, splenic and liver cysts, and vertebral hemangiomas while TI patients had a significant higher frequency of extramedullary hematopoiesis (EMH) (15.1% vs 4.4%; P = 0.002). The prevalence of total IEFs increased with advancing age. TI non-transfusion-dependent patients had a significantly lower frequency of renal cysts than TI transfusion-dependent patients (8.8% vs 26.4%; P = 0.005). The prevalence of renal cysts in the thalassemic population was significantly higher than that in the general population (19.2% vs 1.9%; P < 0.0001). Our data on renal cysts indicate a significant higher prevalence of these IEFs compared to the general population, suggesting the role of the inappropriate activation of the hypoxia-inducible factor system linked to the chronic hypoxia. The significant prevalence of IEF in thalassemia patients undergoing MRI for iron quantification should prompt the discussion of the inclusion of IEF in the MRI report.

Intraspinal Inclusion Tumor After Myelomeningocele Repair: A Long-Term Single-Center Experience.

OBJECTIVE: Given the lack of significant responses to many queries regarding behavior, treatment options, and possible prevention of iatrogenic intraspinal inclusion tumors (IITs), we think that further clinical, radiologic, and follow-up data are mandatory to better characterize this entity. We retrospectively reviewed a series of 14 consecutive patients with IIT occurring after myelomeningocele repair. The long follow-up is a mainstay of this series and may help a better understanding of the history of this slow-growing disease. To date, this is the largest series of IITs after myelomeningocele repair reported in the literature. METHODS: A retrospective chart review was performed to identify all cases of surgically repaired myelomeningocele at the Department of Neurosurgery of G. Gaslini Children's Hospital in Genoa, from January 1993 to January 2018. Among them, data regarding patients in whom an IIT developed were recovered. The medical records of these patients and their clinical history, neurologic examination, and diagnostic workup were reviewed. RESULTS: From January 1993 to January 2018, 160 cases of myelomeningocele were surgically treated. Among them, 14 cases of IIT developed. The mean time between myelomeningocele repair and IIT diagnosis was 94.7 months. The mean follow-up time was 60.1 months. CONCLUSIONS: It is well established that IITs may be discovered in patients who have previously undergone myelomeningocele repair. In our series, with a long follow-up time, we describe the possible range of behavior of such lesions and the utility of surgical treatment of primary therapy. Our series confirms that residual IITs showed marked stability during our long follow-up.

MERRF syndrome (Myoclonic Epilepsy with Ragged Red Fibres) presenting with cervicothoracic lipomatosis.

INTRODUCTION: Patients with MERRF syndrome (Myoclonic Epilepsy with Ragged Red Fibres) usually present with encephalomyopathy. However, progressive, recurrent cervicothoracic lipomatosis may be rarely observed. CASE REPORT: The authors report 4 cases of MERRF syndrome associated with lipomatosis. In 3 patients, the diagnosis of MERRF syndrome was established on the basis of the clinical features of the lipomas and clinical interview revealing a personal or family history of lipomas and myopathy. DISCUSSION: In the presence of extensive spinal lipomatosis, the presence of other clinical signs of MERRF syndrome in the patient or the patient's family must be investigated. A diagnosis of MERRF syndrome can guide appropriate genetic counselling.

Benefits of bariatric surgery on spinal epidural lipomatosis: case report and literature review.

Spinal epidural lipomatosis is an abnormal accumulation of unencapsulated epidural fat causing compression of the neural elements. It can be divided into idiopathic and secondary. Secondary is often associated with chronic steroid use and endocrinopathies. Idiopathic has been associated with obesity. We report a 48-year-old man with obesity and a history of chronic back pain who developed idiopathic spinal epidural lipomatosis diagnosed by magnetic resonance imaging, which subsequently resolved completely after sleeve gastroplasty over a 6-month follow-up period.

Chondrosarcoma of the cervical spine.

This case report describes the diagnosis, surgical treatment, and management of a patient with low-grade chondrosarcoma of the cervical spine that initially presented as myositis ossificans. Chondrosarcoma is rare in the cervical spine and in this patient, may have been the result of an injury from a motor vehicle crash. The management of this patient has been unusual in that a complete excision was not performed because the patient refused standard treatment.

Post-traumatic Spinal Hygroma Causing Cord Compression in Type III Odontoid Fracture With Vertical Atlantoaxial Instability.

STUDY DESIGN: Case report. OBJECTIVE: To report the first case in the literature of a traumatic cervical spine subdural cerebrospinal fluid (CSF) collection (hygroma) under tension causing cord compression. We suggest etiopathogenesis and modality of treatment. SUMMARY OF BACKGROUND DATA: Hygromas are subdural cranial CSF collection. A literature review showed no previous published case of post-traumatic spinal hygroma. This was a potential life-threatening sequelae of a high cervical injury that warranted early diagnosis and emergency treatment. METHODS: We present a case of a young adult who sustained a traumatic vertical atlantoaxial dislocation associated with a type III odontoid fracture. He was initially scored C6 ASIA D. Magnetic resonance imaging (MRI) demonstrated cord contusion at the craniocervical junction and a small fluid collection anterior to the cervical cord. On day 5 after his injury he developed complete paraplegia and priapism. An urgent MRI of his spine revealed expansion of the intraspinal fluid collection with distortion of the cord. He was treated with an emergency surgical decompression. The cervical fluid collection was found to be subdural extra-arachnoidal CSF. A subdural-pleural shunt was inserted. The atlantoaxial injury was reduced and fixed with posterior instrumentation. RESULTS: At 1 year from the injury the patient was independent and fully ambulant. MRI and computed tomography images of his spine demonstrated complete resolution of the cervical hygroma, appropriate placement of the cervical-pleural shunt, and stability of the atlantoaxial injury. CONCLUSION: We describe a unique case of post-traumatic spinal hygroma causing cord compression in a patient with an unstable craniocervical injury. The early recognition and correction of this dangerous complication is of paramount importance to savage cord function. LEVEL OF EVIDENCE: 5.

Diagnostic and Therapeutic Strategies for Patients with Malignant Epidural Spinal Cord Compression.

OPINION STATEMENT: Malignant epidural spinal cord compression (MESCC) is an oncologic emergency with the potential for devastating consequences for patients if not promptly diagnosed and treated. MESCC is diagnosed by imaging. MRI is by far the most sensitive test, preferably with gadolinium. Once the diagnosis of MESCC is suspected, patients with neurologic deficits should receive prompt administration of dexamethasone with a 10-mg IV loading dose followed by 4 mg every 6 h. Quick taper is recommended once the definitive treatment is established. Consultation with medical oncology, radiation oncology, and neurosurgery is imperative in order to facilitate a multidisciplinary approach. Although spine surgery is the most effective method for relief of cord compression and is necessary if there is spinal instability, surgery is only used in selected patients because most patients have a poor overall condition and short life expectancy. Radiation therapy, therefore, is the most commonly used therapy for patients with MESCC after surgical decompression or in patients who are not surgical candidates. Conventional fractionated radiation alone can achieve modest neurologic outcomes in selected radiosensitive tumors. Radiosurgery techniques which deliver intense focal irradiation to a delimited area with imaging guidance and contoured radiation delivery to the shape of the tumor have recently emerged as increasing effective treatments in MESCC, especially in radioresistant tumors. Stereotactic radiosurgery and different radiation technologies have been studied in recent clinical trials.

Textiloma: A Case of Foreign Body Mimicking a Spinal Tumor.

STUDY DESIGN: Case report OBJECTIVE.: To report a case of foreign body mimicking spinal tumor. SUMMARY OF BACKGROUND DATA: Gossypiboma or more broadly retained foreign object is a surgical complication resulting from foreign materials such as cotton or gauze pads and surgical sponge, accidentally left inside a patient's body. Such foreign materials cause foreign body reaction in the surrounding tissue. METHODS: A retrospective case report was performed. Informed consent was obtained from patient. RESULTS: We describe a case of textiloma in which the patient presented with low back pain and radiculopathy 12 years after L5\S1 discectomy. Imaging revealed a round mass lesion in the sacral space. CONCLUSION: At the end of surgery, the operative site should be flushed with saline and carefully examined for any foreign materials. LEVEL OF EVIDENCE: 4.

Cervical Osteochondroma Causing Myelopathy in Adults: Management Considerations and Literature Review.

BACKGROUND: Osteochondromas are the most frequent benign bone tumors but only rarely occur along the spinal column and even more rarely induce symptoms from spinal cord compression. CASE DESCRIPTIONS: We report 2 adult patients, both with a history of hereditary multiple exostoses, who presented with cervical myelopathy secondary to osteochondromas. The first patient is a 22-year-old man with numbness and weakness of his right upper limb and neck pain. Radiologic images showed a bony tumor arising from the C3 lamina with evidence of severe spinal cord compression. The second patient is a 20-year-old woman with weakness of her left upper and lower limbs and progressive numbness of the left hand, as well as neck and back pain. Radiologic images showed a bony tumor arising from the C4 lamina with evidence of significant spinal cord compression and cord signal abnormality. Both patients underwent surgical excision of the epidural mass and pathology confirmed a diagnosis of osteochondroma. CONCLUSIONS: We discuss the role of surgical intervention, management, and postoperative follow-up in adult patients with cervical osteochondromas. Recommended management includes radiographic imaging and surgical intervention, particularly when evidence of spinal cord impingement occurs. Consistent postoperative follow-up is necessary to ensure appropriate recovery of neurologic function. Surgical management of cervical osteochondromas typically results in excellent and stable clinical outcomes with rare recurrence.

The relationship between spinopelvic measurements and patient-reported outcome scores in patients with multiple myeloma of the spine.

AIMS: Patients with multiple myeloma (MM) develop deposits in the spine which may lead to vertebral compression fractures (VCFs). Our aim was to establish which spinopelvic parameters are associated with the greatest disability in patients with spinal myeloma and VCFs. PATIENTS AND METHODS: We performed a retrospective cross-sectional review of 148 consecutive patients (87 male, 61 female) with spinal myeloma and analysed correlations between spinopelvic parameters and patient-reported outcome scores. The mean age of the patients was 65.5 years (37 to 91) and the mean number of vertebrae involved was 3.7 (1 to 15). RESULTS: The thoracolumbar region was most commonly affected (109 patients, 73.6%), and was the site of most posterior vertebral wall defects (47 patients, 31.8%). Poorer Oswestry Disability Index scores correlated with an increased sagittal vertical axis (p = 0.006), an increased number of VCFs (p = 0.035) and sternal involvement (p = 0.012). Poorer EuroQol visual analogue scale scores correlated with posterior vertebral wall defects in the thoracolumbar region (p = 0.012). The sagittal vertical axis increased with the number of fractures and kyphosis in the thoracolumbar (p = 0.009) and lumbar (p < 0.001) regions. CONCLUSIONS: In MM, patients with VCFs have poorer clinical scores at presentation in the presence of sagittal imbalance. Outcome is particularly affected by multiple fractures in the thoracolumbar and lumbar regions and by failure to prevent kyphosis. Patients with MM should be screened for spinal lesions early. Cite this article: Bone Joint J 2016;98-B:1234-9.

Spinal Schwannoma and Meningioma Mimicking a Single Mass at the Craniocervical Junction Subsequent to Remote Radiation Therapy for Acne Vulgaris.

BACKGROUND: Schwannomas and meningiomas are relatively common tumors of the nervous system. They have been reported in the literature as existing concurrently as a single mass, but very rarely have they been shown to present at the craniocervical junction. CASE DESCRIPTION: We present a rare and interesting case of a patient previously treated with radiation therapy for acne vulgaris and who presented to us with a concurrent schwannoma and meningioma of the craniocervical junction mimicking a single mass. CONCLUSIONS: These tumors can be solitary or mixed masses, and are known to be associated with certain disease processes such as long-term sequelae of radiation therapy and neurofibromatosis type 2. The precise mechanism behind the formation of these tumors is unknown; however, molecular cues in the tumor microenvironment may play a role.

Spinal metallosis: a systematic review.

PURPOSE: To document a rarely reported complication associated with spinal instrumentation and to evaluate the current literature on spinal metallosis and spinal metalloma. METHODS: A local case report is presented. EBSCOhost, PubMed and ScienceDirect databases were used to conduct a systematic review for articles describing spinal metallosis and spinal metalloma. RESULTS: A total of 836 articles were identified using the terms "metalloma" or "metallosis". Exclusion of arthroplasty-related abstracts retrieved 46 articles of which 3 full text articles presenting spinal metalloma as a causative pathological finding responsible for neurological signs and symptoms in patients with previous spinal fusion instrumentation were reviewed. Our case is the first described with titanium-composed posterior instrumentation and fifth reported, demonstrating the phenomena of neurological symptoms and signs attributed directly to neural tissue compression by spinal metalloma after spinal instrumentation. CONCLUSION: Spinal metallosis can present weeks to years after spinal instrumentation surgery and is a potential cause of neural compression. This process appears to be independent of the instrument composition as metallosis has now been demonstrated in both titanium and stainless steel constructs.

First Report of Recurrent Intramuscular Lipoma after Decompression Surgery of the Lumbar Spine.

Intramuscular or infiltrating lipomas are rare. We present a 58-year-old man with an intramuscular lipoma developing after decompression surgery for lumbar spinal canal stenosis. One year after macroscopically complete lipoma resection, an even bigger recurrent tumor had to be removed. The lumbar paraspinal musculature is a very uncommon site for an intramuscular lipoma. A relation between surgery and the growth and recurrence of an intramuscular lipoma has not been described previously in the literature.

Paraspinal neurofibromas and hypertrophic neuropathy in Noonan syndrome with multiple lentigines.

BACKGROUND: Noonan syndrome with multiple lentigines (NSML), formerly known as LEOPARD syndrome, is an autosomal-dominant disorder characterised by lentigines, EKG abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, growth retardation and deafness. There is significant clinical overlap between NSML and other disorders that result from dysregulated rat sarcoma/mitogen-activated protein kinase pathway (RASopathies). Except for neurofibromatosis type 1, other RASopathies are not known to be typically associated with neurogenic tumours. METHODS AND RESULTS: We evaluated patients from three families with pigmentary skin lesions, progressive neuropathy, enlarged nerves, massive burden of paraspinal tumours (neurofibroma was confirmed in one patient) and a clinical diagnosis of NSML. All patients had a mutation in the protein tyrosine phosphatase catalytic domain of the PTPN11 gene; two unrelated patients had the p.Thr468Met mutation, while the family consisting of two affected individuals harboured the p.Thr279Cys mutation. Molecular analysis performed on hypertrophic nerve tissue did not disclose a second somatic hit in NF1, PTPN11, NF2 or SMARCB1 genes. CONCLUSIONS: Neurogenic tumours and hypertrophic neuropathy are unusual complications of NSML and may be an under-recognised manifestation that would warrant surveillance. Our observation may also have implications for other disorders caused by RAS-pathway dysregulation.