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Achondroplasia causes narrowing of the foramen magnum and the spinal canal leading to increased mortality due to cervicomedullary compression in infants and significant morbidity due to spinal stenosis later in adulthood. Vosoritide is a C-natriuretic peptide analogue that has been shown to improve endochondral ossification in children with achondroplasia. The objective of this trial is to evaluate the safety of vosoritide and whether vosoritide can improve the growth of the foramen magnum and spinal canal in children that may require decompression surgery. An Achondroplasia Foramen Magnum Score will be used to identify infants at risk of requiring decompression surgery. This is a 2-year open label randomized controlled trial of vosoritide in infants with achondroplasia ages 0 to ≤12months. Approximately 20 infants will be randomized 1:1 to either open label once daily subcutaneous vosoritide combined with standard of care or standard of care alone. The primary and secondary aims of the study are to evaluate the safety and efficacy of vosoritide in children with cervicomedullary compression at risk of requiring decompression surgery. The trial will be carried out in specialized skeletal dysplasia treatment centers with well established multidisciplinary care pathways and standardized approaches to the neurosurgical management of cervicomedually compression. After 2 years, infants randomized to standard of care alone will be eligible to switch to vosoritide plus standard of care for an additional 3 years. This pioneering trial hopes to address the important question as to whether treatment with vosoritide at an early age in infants at risk of requiring cervicomedullary decompression surgery is safe, and can improve growth at the foramen magnum and spinal canal alleviating stenosis. This in turn may reduce compression of surrounding structures including the neuraxis and spinal cord, which could alleviate future morbidity and mortality.

Lumbar radicular pain often results from lumbar disc herniation, spinal stenosis, or degenerative spondylolisthesis. Minimally invasive disc decompression procedures, such as nucleo-annuloplasty or epiduroscopic neural decompression by laser, have been devised to treat such pain. The short-term outcomes of disc decompression by endoscopic epidural laser decompression (EELD) or transforaminal epiduroscopic laser annuloplasty (TELA) were compared in patients with lumbar radicular pain due to disc herniation. A randomized, prospective trial. The Department of Anesthesiology and Pain Medicine at Spine Health Wooridul Hospital in Daegu, Korea. A total of 97 patients were enrolled in this study; 48 patients underwent EELD and 49 underwent TELA. The pain relief was evaluated at baseline and at 1, 3, and 6 months post-procedure via the numeric rating scale (NRS). The Oswestry Disability Index (ODI) was recorded at baseline and at the final follow-up. Postoperative wound pain was assessed over a 24-hour period. Complications and side effects were also recorded, as were operative times (from local anesthetic infiltration at entry sites to suturing of skin). At post-treatment months 1, 3, and 6 the mean pain scores of patients were significantly lower (relative to pre-treatment baseline) regardless of the procedure used. However, the mean pain scores did not differ significantly by procedure (EELD vs TELA). As well, the number of patients who obtained relief from their pain and needed analgesics was not statistically significant. The irrigation volume was significantly higher in the TELA group. Two patients undergoing TELA procedures experienced headache during the procedures; however, no serious complications such as bleeding, dural/neural injuries, or infection were recorded for either group. The observed significant reductions in pain (from baseline) lacked secondary outcome substantiation and given the mid follow-up period, no long-term follow-up results were monitored. Both EELD and TELA provide similar outcomes and are reasonable treatment options for carefully selected patients with lower back or radicular pain. Epiduroscopy, laser, annuloplasty, disc, herniation, TELA.

Anterior foramen magnum (AFM) meningiomas are difficult to treat because they are situated in anatomically complex regions. One of the most common approaches for AFM meningiomas is the far lateral approach. We describe our exo- and endoscopic two-step approach (EETA) through suboccipital craniotomy. An exoscope is used to remove the tumour’s lateral part, whereas an endoscope is used to remove the residual tumour in the exoscope’s blind spots, including the brainstem’s ventral side. EETA is a viable option for treating AFM meningiomas because it provides an adequate surgical field of view and is minimally invasive.

PurposeProximity to critical neurovascular structures can create significant obstacles during surgical resection of foramen magnum meningiomas (FMMs) to the detriment of treatment outcomes. We propose a new classification that defines the tumor’s relationship to neurovascular structures and assess correlation with postoperative outcomes.MethodsIn this retrospective review, 41 consecutive patients underwent primary resection of FMMs through a far lateral approach. Groups defined based on tumor-neurovascular bundle configuration included Type 1, bundle ventral to tumor; Type 2a-c, bundle superior, inferior, or splayed, respectively; Type 3, bundle dorsal; and Type 4, nerves and/or vertebral artery encased by tumor.ResultsThe 41 patients (range 29–81 years old) had maximal tumor diameter averaging 30.1 mm (range 12.7–56 mm). Preoperatively, 17 (41%) patients had cranial nerve (CN) dysfunction, 12 (29%) had motor weakness and/or myelopathy, and 9 (22%) had sensory deficits. Tumor type was relevant to surgical outcomes: specifically, Type 4 demonstrated lower rates of gross total resection (65%) and worse immediate postoperative CN outcomes. Long-term findings showed Types 2, 3, and 4 demonstrated higher rates of permanent cranial neuropathy. Although patients with Type 4 tumors had overall higher ICU and hospital length of stay, there was no difference in tumor configuration and rates of postoperative complications or 30-day readmission.ConclusionThe four main types of FMMs in this proposed classification reflected a gradual increase in surgical difficulty and worse outcomes. Further studies are warranted in larger cohorts to confirm its reliability in predicting postoperative outcomes and possibly directing management decisions.

BackgroundDural arteriovenous fistulas (dAVFs) in the foramen magnum region (FMR) are rare entity of dAVFs. There is no established treatment for FMR-dAVFs owing to their rarity and anatomical complexity. Herein, we report cases of high-flow dAVFs located at the posteromedial part of the FMR that were successfully treated by surgical interruption.MethodsWe demonstrated the surgical procedures for the interruption of high-flow FMR-dAVF with representative images and videos. In both cases, endovascular transarterial embolization was performed prior to surgical interruption.ConclusionAs this type of FMR-dAVF has high-risk clinical features, curative surgical treatment is highly desirable.

Background Chiari I malformation is defined by tonsillar herniation through the foramen magnum. There is no consensus on the treatment of Chiari malformation. A simple follow-up is recommended for asymptomatic cases. The classic approach is the midline sub-occipital craniotomy. Methods For four years, we operated on six patients with Chiari malformation I using our endoscopic minimally invasive sub-occipital approach. We compared the results with six other patients operated by the classical sub-occipital approach. Results Patients operated by endoscopic approach had shorter hospital stays, and wounds healed faster and smoother. Mid-term results were similar in the two groups. Conclusion This paper proposes a new endoscopic Minimally invasive paramedian sub-occipital approach for Chiari malformation I. Although the number of cases is limited, the results look promising. We need to gather more cases to have significant numbers to perform a global comparison between the two approaches and assess the advantages and disadvantages of each technique.

BackgroundMastoid emissary vein is especially important from the neurosurgical point of view, because it is located in variable number in the area of the occipitomastoid suture and it can become a source of significant bleeding in surgical approaches through the mastoid process, especially in retrosigmoid craniotomy, which is used for approaches to pathologies localized in the cerebellopontine angle. Ideal imaging method for diagnosis of these neglected structures when planning a surgical approach is high-resolution computed tomography. The aim of this work was to provide detailed information about this issue.MethodsWe studied a group of 295 skulls obtained from collections of five anatomy departments and the National Museum. Both quantitative and qualitative parameters of the mastoid foramen were evaluated depending on side of appearance and gender. Individual distances of the mastoid foramen from clearly defined surface landmarks (asterion, apex of mastoid process, foramen magnum) and other anatomical structures closely related to this issue (width of groove for sigmoid sinus, diameters of internal and external openings of mastoid foramen) were statistically processed.ResultsThe most frequently represented type of the mastoid foramen is type II by Louis (41.2%). The differences between right and left sides were not statistically significant. In men there was a higher number of openings on the right side and in qualitative parameters the type III and IV predominated, whereas in women the types I and II were more frequent. In men, greater distances from the mastoid foramen were observed when evaluating qualitative parameters for defined surface landmarks. Mean size of the external opening diameter was 1.3 mm; however, several openings measured up to 7 mm.ConclusionsDespite excellent knowledge of anatomy, however, good pre-operative examination using imaging methods and mastering of microsurgical techniques create the base for successful treatment of pathological structures in these anatomically complex areas.

Purpose Dural arteriovenous fistulas (AVFs) draining to medullary bridging vein (MBV) are located at foramen magnum (FM) and craniocervical junction (CCJ). Such fistulas are rare but pose a challenge to endovascular management. This study was undertaken to assess clinical manifestations, angiographic features, and outcomes of endovascular treatment in patients with MBV dural AVFs. Methods A number of our patients ( N  = 22) were diagnosed with MBV dural AVF and treated by endovascular means. There were 9 FM lesions and 13 CCJ lesions. We reviewed clinical records and imaging studies to define clinical characteristics, vascular anatomic details, and treatment outcomes, comparing FM- and CCJ-level subsets. Results Subjects ranged from 37 to 74 years of age (mean, 57.7 years) with male predominance (2.7:1). They presented with intracranial hemorrhage (11/22, 50%), myelopathy (8/22, 36%), or nonspecific symptoms (3/22, 14%). In 17 patients (77.3%), the shunts showed complete or near-complete occlusion following endovascular treatment (FM, 100%; CCJ, 61.5%). However, seven patients experienced ischemic events (FM, 11.1%; CCJ, 46.2%) and one patient sustained a hemorrhagic complication. No hemorrhages recurred during follow-up monitoring, and myelopathic symptoms abated. Conclusion MBV dural AVFs are highly aggressive lesions for which proper diagnosis and treatment are of utmost importance. Although transarterial embolization proved highly successful in FM lesions, shunt occlusion was less frequent in the CCJ subset, with greater risk of ischemic complications.

Introduction Chiari 1 malformation and hind brain hernia can be associated with skull base and craniocervical anomalies. One of the more recently associated anomalies is a retroverted or retroflexed odontoid process or dens. We conducted a retrospective study of our operated symptomatic and conservatively managed asymptomatic Chiari cohort to assess the impact of dens retroflexion on rate of revision or cerebrospinal fluid diversion following primary foramen magnum decompression (FMD). Methods We undertook a retrospective study of all foramen magnum decompression (FMD) cases for Chiari type 1 malformation performed over a 15-year period in a single tertiary paediatric neurosurgical unit. For comparison, non-operated asymptomatic Chiari cases were considered as reference cohort. Information gathered included: demographics, age, sex, length of cerebellar tonsils below McRae’s line, pB-C2 distance (a line drawn perpendicular to one drawn between the basion and the posterior aspect of the C2 body), angle of retroflexion (angle formed between a line drawn through the odontoid synchondrosis and its intersection with a line drawn from the tip of the odontoid process) and angle of retroversion (angle formed between the line drawn from the base of C2 and its intersection with a line drawn from the tip of the odontoid process). Grade of retroflexion was measured using pre-operative mid-sagittal MR images and classified as grade 0 (> 90°), grade 1 (85°–89°); grade 2 (80°–84°) and grade 3 (< 80°). The rates for redo surgery or need for cerebrospinal fluid (CSF) diversion were obtained from clinical records and compared in the operated and non-operated groups. Results One hundred twenty-six Chiari 1 patients were included in this study with adequate imaging. Sixty-five patients were in the non-operated asymptomatic cohort with 61 patients in the operated symptomatic cohort. Mean age of non-operated cohort was 10.2 years with M:F ratio (30:35). Mean cerebellar tonsillar length below McRae’s line was 10.3 mm. 7.7% of this cohort had associated syrinx. Mean angles of retroversion and retroflexion were 76 and 78°, respectively. Retroflexion grades included (9.2% grade 1, 35% grade 2 and 52.3% grade 3). pB-C2 distance was 6.8 mm. Mean age of operated cohort was 11.3 years, with M:F ratio (21:40). Mean cerebellar tonsillar length below McRae’s line was 15 mm. 45.9% of this cohort had associated syrinx. Mean angles of retroversion and retroflexion were 73 and 74.5°, respectively. Retroflexion grades included (4.9% grade 1, 16.5% grade 2 and 78.6% grade 3). pB-C2 distance was 6.9 mm. No association was identified between retroflexion grade and rate of revision or CSF diversion following primary foramen magnum decompression. Conclusion The operated Chiari 1 cohort had more retroflexed dens, longer tonsils and associated syrinx compared to the non-operated asymptomatic cohort.