Citations

BACKGROUND Hirayama disease (HD), also known as juvenile spinal muscular atrophy, is a rare condition in which flexion of the cervical neck causes compression and ischemic changes to the anterior horns of the spinal cord. Here the authors presented the first reported case of HD in North America that was successfully treated via surgical intervention. OBSERVATIONS The patient was a 15-year boy with insidious onset upper limb weakness and atrophy. His findings were a classic presentation of HD although his complex history and relative rarity of the disease caused him to remain undiagnosed for months. After conservative management via cervical collar failed, the patient was successfully treated via C5-C7 anterior cervical discectomy and fusion. The patient’s symptoms stabilized by the 3-month follow-up. LESSONS The diagnosis of HD is easy to miss because of the lack of reporting and widespread knowledge of this condition in North America. Thus, when presented with a case of insidious onset limb weakness in a juvenile patient, HD should be placed on the differential list and verified with cervical flexion magnetic resonance imaging. Additionally, surgical intervention should be considered a safe and effective option for HD when conservative methods have failed.

BACKGROUND Thoracic spinal epidural lipomatosis (SEL) involves the pathological overgrowth of histologically normal, unencapsulated adipose tissue that can compress the spinal cord and cause myelopathy. SEL has been associated with multiple medical conditions, including Scheuermann kyphosis (SK). Optimal treatment strategies for SEL, especially in the setting of a sagittal spinal deformity, remain unclear. OBSERVATIONS In this report, the authors discussed surgical management of a patient with thoracic SEL and SK using skip hemilaminotomies for resection of the epidural adipose tissue. To the authors’ knowledge, only one other report described a similar surgical technique in a patient who did not have a spinal deformity. LESSONS When conservative efforts fail, thoracic SEL may require surgical treatment. Surgical planning must account for co-medical conditions such as SK. The described approach involving skip laminotomies, which minimizes spine destabilization, is a viable option to treat SEL spanning multiple spinal segments. Prognosis after surgical treatment varies and is impacted by multiple factors, including severity of preoperative neurological deficits.

Background: High-dose chemotherapy followed by autologous stem cell transplantation (HD-ASCT) is a promising alternative to whole brain radiation therapy (WBRT) in the treatment of primary central nervous system lymphoma (PCNSL). The objective of this study was to critically assess current evidence supporting the use of HD-ASCT as first-line consolidative therapy in PCNSL. Methods: The objective was addressed through the development of a critically appraised topic that included a clinical scenario, structured question, literature search strategy, critical appraisal, assessment of results, evidence summary, commentary, and bottom-line conclusions. Participants included consultant and resident neurologists, a medical librarian, clinical epidemiologists, and a content expert in the field of neuro-oncology. Results: A recent, open-label, noncomparative randomized phase II trial was selected for critical appraisal. This trial evaluated the efficacy and toxicity of consolidative therapy with HD-ASCT and WBRT in PCNSL in 2 separate treatment arms. A total of 140 patients with newly diagnosed PCNSL between the ages of 18 and 60 years were included. The primary endpoint of 2-year progression-free survival was met in 63% of patients in the WBRT arm and 87% in the HD-ASCT arm. Notably, an overall improvement in neurocognitive scores was observed following HD-ASCT, while WBRT was associated with worsened cognitive outcomes. Conclusions: In young patients with newly diagnosed PCNSL, consolidative therapy with HD-ASCT appears to be associated with less neurocognitive toxicity and may be more effective than WBRT at preventing relapses, however, at the cost of a higher treatment-related mortality.

Retro-odontoid cysts are a rare cause of cervicomedullary compression. The etiology of these lesions is not completely understood. Previous trauma and instability at the cervicomedullary junction may be the precipitating event in the development of retro-odontoid cysts in rare cases. We discussed the neurosurgical evaluation of a patient who presented with progressive and rapid neurological deterioration secondary to cervicomedullary compression. Posterior occipitocervical fusion was performed. The patient made an excellent neurological recovery, and postoperative imaging studies demonstrated resolution of the compression and intramedullary cyst.

OBJECTIVE The authors aimed to demonstrate the feasibility and advantages of carbon fiber–reinforced PEEK (CFRP) composite implants in patients with both primary and secondary osseous spinal tumors. METHODS Twenty-eight spinal tumor patients who underwent fixation with CFRP hardware were retrospectively identified in a Spine Tumor Quality Database at a single institution. Demographic, procedural, and follow-up data were retrospectively collected. RESULTS The study population included 14 females and 14 males with a mean age of 60 years (range 30–86 years). Five patients had primary bone tumors, and the remaining patients had metastatic tumors. Breast cancer was the most common metastatic tumor. The most common presenting symptom was axial spine pain (25 patients, 89%), and the most common Spine Instability Neoplastic Score was 7 (range 6–14). Two patients in this series had anterior cervical procedures. The remaining patients underwent posterior thoracolumbar fixation. The average fusion length included 4.6 vertebral segments (range 3–8). The mean clinical follow-up time with surgical or oncology teams was 6.5 months (range 1–23 months), and the mean interval for last follow-up imaging (CT or MRI) was 6.5 months (range 1–22 months). Eighteen patients received postoperative radiation at the authors’ institution (16 with photon therapy, 2 with proton therapy). Eleven of the patients (39%) in this series died. At the last clinical follow-up, 26 patients (93%) had stable or improved neurological function compared with their preoperative status. At the last imaging follow-up, local disease control was observed in 25 patients (89%). Two patients required reoperation in the immediate postoperative period, one for surgical site infection and the other for compressive epidural hematoma. One patient was noted to have lucencies around the most cephalad screws 3 months after surgery. No hardware fracture or malfunction occurred intraoperatively. No patients required delayed surgery for hardware loosening, fracture, or other failure. Early tumor recurrence was detected in 3 patients. Early detection was attributed to the imaging characteristics of the CFRP hardware. CONCLUSIONS CFRP spinal implants appear to be safe and comparable to conventional titanium implants in terms of functionality. The imaging characteristics of CFRP hardware facilitate radiation planning and assessment of surveillance imaging. CFRP hardware may enhance safety and efficacy, particularly with particle therapy dosimetry. Larger patient populations with longer-term follow-up are needed to confirm the various valuable aspects of CFRP spinal implants.

The anterior lumbar interbody fusion (ALIF) is a well-established procedure used to treat a multitude of spinal pathologies. When performed at the L5-S1 level, the ALIF is often supplemented with posterior pedicle screw and rod fixation. Because the interbody device can restore disk and foraminal height, one benefit of the ALIF procedure is indirect neural decompression in the spinal canal and neural foramina. If the contour of the posterior rod is not matched to the exact position of the tulip heads on the pedicle screws, spondylolisthesis can be introduced, leading to foraminal stenosis and nerve compression. This concern is particularly germane when the posterior instrumentation is placed percutaneously without any direct foraminal decompression. In this report, we describe a patient who had an L4-S1 ALIF, resulting in new L5-S1 retrolisthesis and worsening L5 radiculopathy. Technical nuances and avoidance strategies are discussed.

BACKGROUND Intramedullary spinal cord tumors represent a minority of intradural tumors. Among intramedullary spinal cord tumors, hemangioblastomas are uncommon, and schwannomas are extremely rare. Collision tumors are histologically distinct tumors that are intermingled and growing together. OBSERVATIONS In this report, the authors describe a patient with a cervical intramedullary collision tumor involving a hemangioblastoma and schwannoma. To the authors’ knowledge, no prior spinal intramedullary collision tumor involving multiple neoplasms has been described. The patient’s presentation and management are described. LESSONS Clinicians should consider the possibility of collision tumors when evaluating intramedullary spinal cord tumors, especially when patient presentation and radiographic findings are atypical. When tumors with similar radiographic characteristics form collision tumors, distinction using preoperative imaging can be extremely challenging. In addition, surgical management of intramedullary collision tumors, like that for all intramedullary spinal cord tumors, should involve meticulous perioperative care and a methodical surgical technique. Maximal safe resection will depend upon histopathological diagnosis, anatomical location of the tumor, presence of distinct dissection planes, and stability of neuromonitoring. Finally, ongoing research on the genetics of intramedullary spinal cord tumors may identify underlying genetic links for intramedullary hemangioblastomas and schwannomas.

BACKGROUND Anterior lumbar fusion procedures have many benefits and continue to grow in popularity. The technique has many potential approach- and procedure-related complications. Symptomatic retroperitoneal fluid collections are uncommon but potentially serious complications after anterior lumbar procedures. Collection types include hematomas, urinomas, chyloperitoneum, cerebrospinal fluid collections, and deep infections. OBSERVATIONS The authors present an unusual case of a patient with persistent symptoms related to a retroperitoneal collection over a 5-year period following anterior lumbar fusion surgery. To the authors’ knowledge, no similar case with such extensive symptom duration has been described. The patient had an infected encapsulated fluid collection. The collection was presumed to be a postoperative lymphocele that was secondarily infected after serial percutaneous drainage procedures. LESSONS When retroperitoneal collections occur after anterior retroperitoneal approaches, clinical clues, such as timing of symptoms, hypotension, acute anemia, urinary tract infection, hydronephrosis, elevated serum creatinine and blood urea nitrogen, low-pressure headaches, anorexia, or systemic signs of infection, can help narrow the differential. Retroperitoneal collections may continue to be symptomatic many years after anterior lumbar surgery. The collections may become infected after serial percutaneous drainage or prolonged continuous drainage. Encapsulated, infected fluid collections typically require surgical debridement of the capsule and its contents.

Background:Among the elderly, chronic subdural hematoma is a relatively common neurosurgical condition. Presenting symptoms range from headache and focal neurological deficits to seizure and coma depending on location and extent of brain compression. Functional recovery following surgery for chronic subdural hematoma is central to quality of life and ongoing health for elderly patients; however, there is a paucity of data regarding functional recovery in this population.Methods:In this study, the physical activity of patients who underwent surgical evacuation of chronic subdural hematoma was surveyed, as well as participation in physical therapy following surgery. In total, 38 patients completed the survey.Results:Of the 30 patients who exercised regularly before surgery, 28 (90.3%) returned to exercise within 1 year after surgery. Of 13 patients who reported playing hobby sports before surgery, 9 (69.2%) returned to those sports. 17/38 (44.7%) patients participated in physical therapy after surgery. 35/38 (92.1%) of patients reported that the surgery improved their quality of life.Conclusion:The majority of patients who underwent surgery for chronic subdural hematoma were able to return to exercise within 1 year. Participation in physical therapy was associated with return to exercise and sports. Further study is needed to determine which factors contribute to a return to baseline levels of physical activity following surgery for chronic subdural hematoma.