Evaluating compliance with the best practice guidelines for wrong-level surgery prevention in high-risk pediatric spine surgery.

PURPOSE: In 2018, Best Practice Guidelines (BPGs) were published for preventing wrong-level surgery in pediatric spinal deformity, but successful implementation has not been established. The purpose of this study was to evaluate BPG compliance 5 years after publication. We hypothesized higher compliance among BPG authors and among surgeons with more experience, higher caseload, and awareness of the BPGs. METHODS: We queried North American and European surgeons, authors and nonauthors, and members of pediatric spinal study groups on adherence to BPGs using an anonymous survey consisting of 18 Likert scale questions. Respondents provided years in practice, yearly caseload, and guideline awareness. Mean compliance scores (MCS) were developed by correlating Likert responses with MCS scores ("None of the time" = no compliance = MCS 0, "Sometimes" = weak to moderate = MCS 1, "Most of the time" = high = MCS 2, and "All the time" = perfect = MCS 3). RESULTS: Of the 134 respondents, 81.5% reported high or perfect compliance. Average MCS for all guidelines was 2.4 ± 0.4. North American and European surgeons showed no compliance differences (2.4 vs. 2.3, p = 0.07). Authors and nonauthors showed significantly different compliance scores (2.8 vs 2.4, p < 0.001), as did surgeons with and without knowledge of the BPGs (2.5 vs 2.2, p < 0.001). BPG awareness and compliance showed a moderate positive correlation (r = 0.48, p < 0.001), with non-significant associations between compliance and both years in practice (r = 0.41, p = 0.64) and yearly caseload (r = 0.02, p = 0.87). CONCLUSION: Surgeons reported high or perfect compliance 81.5% of the time with BPGs for preventing wrong-level surgery. Authorship and BPG awareness showed increased compliance. Location, study group membership, years in practice, and yearly caseload did not affect compliance. LEVEL OF EVIDENCE: Level V-expert opinion.

Fusion mass to pelvis internal distraction technique using multiple-hook fixation for scoliosis correction: illustrative case.

BACKGROUND: Internal distraction rods have been described as an alternative to halo gravity traction for the treatment of severe scoliosis. Distraction rods can be challenging to use in patients with existing fusion masses. The authors report an internal distraction, construct-to-construct rod technique using multiple-hook fixation in a patient with a sharply angulated cervicothoracic scoliosis fusion mass. OBSERVATIONS: A 12-year-old female with previously diagnosed congenital scoliosis who had undergone cervical fusion in situ at age 2 presented to the clinic with shortness of breath exacerbated by increased levels of activity. Standing anteroposterior and lateral scoliosis radiographs revealed a left >150° cervicothoracic curve, right 140° thoracolumbar curve, and left 28° lumbosacral fractional curve with pelvic obliquity. The authors indicated this patient for a 3-stage all-posterior approach for spinal fusion and deformity correction. In the final fusion surgery, the authors set up a construct-to-construct internal distraction configuration connecting the left hemipelvis to the cervicothoracic fusion mass to aid in deformity correction. LESSONS: A construct-to-construct internal distraction rod technique connecting a fusion mass to the pelvis can assist with curve correction in severe scoliosis.

Evaluating compliance with the 2013 best practice guidelines for preventing surgical site infection in high-risk pediatric spine surgery.

PURPOSE: Best Practice Guidelines (BPGs) were published one decade ago to decrease surgical site infection (SSI) in pediatric spinal deformity. Successful implementation has not been established. This study evaluated surgeon compliance with items on the BPG. We hypothesized that BPG authors and surgeons with more experience, higher caseload, and awareness of the BPG would have higher compliance. METHODS: We queried North American and European surgeons, authors and non-authors, and members of various spine study groups on adherence to BPGs using an anonymous survey. Mean compliance scores (MCSs) were developed by correlating Likert responses with MCSs ("None of the time" = no compliance = MCS 0, "Sometimes" = weak to moderate = MCS 1, "Most of the time" = high = MCS 2, "All the time" = perfect = MCS 3). RESULTS: Of the 142 respondents, 73.7% reported high or perfect compliance. Average compliance scores for all guidelines was 2.2 ± 0.4. There were significantly different compliance scores between North American and European surgeons (2.3 vs 1.8, p < 0.001), authors and non-authors (2.5 vs. 2.2, p = 0.023), and surgeons with and without knowledge of the BPGs (2.3 vs. 1.8, p < 0.001). There was a weak correlation between BPG awareness and compliance (r = 0.34, p < 0.001) and no correlation between years in practice (r = 0.0, p = 0.37) or yearly caseload (r = 0.2, p = 0.78) with compliance. CONCLUSIONS: Compliance among our cohort of surgeons surveyed was high. North American surgeons, authors of the BPGs and those aware of the guidelines had increased compliance. Participation in a spine study group, years in practice, and yearly caseload were not associated with compliance. LEVEL OF EVIDENCE: Level V-expert opinion.

Thoracic pediculectomy for acute spinal cord decompression in high-risk spinal deformity correction: illustrative case.

BACKGROUND: Neurological complications are higher in patients with severe spinal deformities (Cobb angle >100°). The authors highlight a known technique for thoracic concave apical pedicle resection that is useful for spinal cord decompression in patients with high-risk spinal deformities in the setting of intraoperative neuromonitoring (IONM) changes. OBSERVATIONS: A 14-year-old female with progressive idiopathic scoliosis presented for evaluation of her clinical deformity. Scoliosis radiographs showed a double major curve pattern comprising a 107° right main thoracic curve and a compensatory 88° left thoracolumbar curve. She underwent 2 weeks of halo-gravity traction that reduced her major thoracic curve to 72°. During thoracic posterior column osteotomies, the authors were alerted to decreases in IONM signals that were not responsive to increases in mean arterial pressure, traction weight reduction, and convex compression maneuvers. The dural surface was tightly draped over the two thoracic apical pedicles of T7 and T8, so emergent pediculectomies were performed at both levels for spinal cord decompression. IONM signals gradually improved and eventually became even better than baseline. The patient woke up without any neurological deficits. LESSONS: Pediculectomy of the concave apical pedicle(s) should be considered for spinal cord decompression if there are IONM changes during high-risk spinal deformity surgery.

Three-stage correction of severe idiopathic scoliosis with limited skeletal traction during a humanitarian surgical mission: illustrative case.

BACKGROUND: Underprivileged and underserved patients from developing countries often present late with advanced, untreated spinal deformities. We report a three-stage all-posterior approach using limited skeletal traction with Gardner-Wells tongs (GWTs) for the management of severe idiopathic scoliosis during a humanitarian surgical mission trip. OBSERVATIONS: A 17-year-old high-school female was previously diagnosed with juvenile idiopathic scoliosis (diagnosed at age 8) and progressed to a severe 135° kyphoscoliosis. Procedural stage 1 involved spinal instrumentation and posterior releases via posterior column osteotomies from T3 to L4. She then underwent 7 days of skeletal traction with GWTs in the intensive care unit as stage 2. In stage 3, rod engagement, posterior spinal fusion, and partial T10 vertebral column resection were performed. There were no changes in intraoperative neuromonitoring during either surgery and she woke up neurologically intact after both stages of the surgical procedure. LESSONS: Skeletal traction with GWTs is a viable alternative to traditional halo-gravity traction in settings with limited resources. Three-stage spinal deformity correction using limited skeletal traction is a feasible and effective approach for managing severe scoliosis during humanitarian surgical mission trips.

Construct-to-construct biplanar cantilever technique for spinal deformity correction: illustrative case.

BACKGROUND: There is a continued trend toward posterior-only approaches for achieving spinal deformity correction of idiopathic scoliosis. We present a posteriorly based correction technique and en bloc translation reduction maneuver that can be useful in the management of kyphoscoliosis. OBSERVATIONS: A 50-year-old female with a past medical history of untreated adolescent idiopathic scoliosis since she was 12 years old presented to the clinic for evaluation of progressive thoracolumbar spinal deformity and worsening mid-to-low back pain. Standing scoliosis radiographs shows an 85° left lumbar curve with an apex at the L1-2 disk. There was a compensatory 58° right thoracic curve with an apex at T9, a -1.4 cm central vertical axis, and a focal kyphotic deformity of 86° from T11-L3 with a corresponding apex at the L1-2 disk. She was diagnosed with adult idiopathic scoliosis and indicated for a T9-L4 posterior spinal fusion with T11-L4 Smith-Peterson osteotomies. A simple en bloc reduction maneuver was used to translate the apex of the coronal deformity toward the midline and simultaneously correct the patient's focal kyphosis. LESSONS: A construct-to-construct biplanar cantilever technique is ideal for the treatment of kyphoscoliosis and can provide effective deformity correction in both the sagittal and coronal planes.

Step-by-step guide to robotic-guided minimally invasive transforaminal lumbar interbody fusion (MI-TLIF).

Robotics in spinal surgery offers a promising potential to refine and improve the minimally invasive transforaminal lumbar interbody fusion (MI-TLIF) technique. Suitable surgeons for this technique include those who are already familiar with robotic-guided lumbar pedicle screw placement and want to advance their skillset by incorporating posterior-based interbody fusion. We provide a step-by-step guide for robotic-guided MI-TLIF. The procedure is divided into 7 practical and detailed techniques. The steps in sequential order include: (I) planning trajectories for pedicle screws and the tubular retractor; (II) robotic-guided pedicle screw placement; (III) placement of tubular retractor; (IV) unilateral facetectomy using the surgical microscope; (V) discectomy & disc preparation; (VI) interbody implant insertion; and (VII) percutaneous rod placement. We standardize surgeon training in robotic MI-TLIF by teaching our spine surgery fellows these 7 key technical steps highlighted in this guide. Current-generation robotics offers integrated navigation capability, K-wireless placement of pedicle screws through a rigid robotic arm, compatibility with tubular retractor systems to perform facetectomy, and allows for placement of interbody devices. We have found robotic-guided MI-TLIF to be a safe procedure that allows for accurate and reliable pedicle screw placement, less collateral damage to the soft tissues of the low back, and decreased radiation exposure.

Female Sex and Supine Proximal Lumbar Lordosis Are Associated With the Size of the LLIF "Safe Zone" at L4-L5.

STUDY DESIGN: Retrospective chart review. OBJECTIVE: Identify demographic and sagittal alignment parameters that are independently associated with femoral nerve position at the L4-L5 disk space. SUMMARY OF BACKGROUND DATA: Iatrogenic femoral nerve or lumbar plexus injury during lateral lumbar interbody fusion (LLIF) can result in neurological complications. The LLIF "safe zone" is the anterior half to two third of the disk space. However, femoral nerve position varies and is inconsistently identifiable on magnetic resonance imaging. The safe zone is also narrowest at L4-L5. METHODS: An analysis of patients with symptomatic lumbar spine pathology and magnetic resonance imaging with a visibly identifiable femoral nerve evaluated at a single large academic spine center from January 1, 2017, to January 8, 2020, was performed. Exclusion criteria were transitional anatomy, severe hip osteoarthritis, coronal deformity with cobb >10 degrees, > grade 1 spondylolisthesis at L4-L5 and anterior migration of the psoas.Standing and supine lumbar lordosis (LL) and its proximal (L1-L4) and distal (L4-S1) components were measured. Femoral nerve position on sagittal imaging was then measured as a percentage of the L4 inferior endplate. A stepwise multivariate linear regression of sagittal alignment and LL parameters was then performed. Data are written as estimate, 95% CI. RESULTS: Mean patient age was 58.2±14.7 years, 25 (34.2%) were female and 26 (35.6%) had a grade 1 spondylolisthesis. Mean femoral nerve position was 26.6±10.3% from the posterior border of L4. Female sex (-6.6, -11.1 to -2.1) and supine proximal lumbar lordosis (0.4, 0.1-0.7) were independently associated with femoral nerve position. CONCLUSIONS: Patient sex and proximal LL can serve as early indicators of the size of the femoral nerve safe zone during a transpsoas LLIF approach at L4-L5.

Acute development of spinal lumbar synovial facet cyst within 1 week after lumbar decompression: illustrative case.

BACKGROUND: Synovial facet cysts can sometimes develop in patients with lumbar spinal stenosis after decompressive laminectomy. The etiology of spinal lumbar synovial cysts is still unclear, but their formation is associated with underlying spinal instability, facet joint arthropathy, and degenerative spondylolisthesis. OBSERVATIONS: A 61-year-old-male patient presented with neurogenic claudication due to lumbar spinal stenosis. Radiographic studies showed grade I spondylolisthesis and radiological predictors of delayed spinal instability. He underwent lumbar decompression and shortly thereafter developed spinal instability and recurrent symptoms, with formation of a new spinal lumbar synovial facet cyst. He required revisional decompression, cyst excision, and posterolateral spinal fusion for definitive treatment. LESSONS: The literature reports postoperative spinal instability in up to one-third of patients with lumbar spinal stenosis and stable degenerative spondylolisthesis who undergo decompressive laminectomy. Close radiographic monitoring and early advanced imaging may be prudent in this patient population if they develop new postoperative neurological symptoms and show radiographic predictors of instability on preoperative imaging. Posterolateral spinal fusion with instrumentation should be considered in addition to lumbar decompression in this select group of patients who demonstrate radiographic predictors of delayed spinal instability if they are medically capable of tolerating a spinal fusion procedure.

Anterior Cervical Foraminotomy for Radiculopathy After Cervical Artificial Disc Replacement: Technique Description and Case Report.

BACKGROUND: Patients may occasionally have persistent or recurrent radicular symptoms after cervical artificial disc replacement (ADR) for cervical spondylotic radiculopathy. We describe our approach using anterior cervical foraminotomy (ACF) to provide symptom relief in such patients without the need to convert to a fusion or remove the ADR implant. METHODS: Our operative technique for ACF after cervical ADR begins by starting at the lateral edge of the ADR at the superior end plate of the inferior vertebral body. The ipsilateral uncovertebral joint is drilled with a combination of a high-speed burr and diamond-coated burr to minimize the risk of injury to the vertebral artery. The neuroforamen is entered after drilling through the posterior aspect of the uncinate process. The exiting cervical nerve root should be directly visualized, and a Kerrison rongeur may be used to trace along the nerve root laterally to remove any remaining uncinate osteophyte or process. We queried our internal database for patients with recurrent or new radicular pain following cervical ADR who underwent ACF. Clinical characteristics and outcomes were reported. RESULTS: Five patients with recurrent radicular symptoms after ADR were reviewed. Two ACFs were performed at C5-6, and 2 were performed at C6-7. Four patients developed ipsilateral recurrent radicular symptoms, and only 1 patient developed contralateral new radicular symptoms. Three patients reported complete resolution of their new or recurrent radicular symptoms following ACF, and 2 patients reported only partial resolution. No patients required conversion to a fusion. CONCLUSIONS: In patients with recurrent symptoms of cervical spondylotic radiculopathy following ADR, ACF with uncovertebral joint resection can be used to provide direct foraminal decompression without the need for implant removal. This approach also preserves motion at the affected level, preserves cervical spinal stability, and prevents the need for spinal fusion. CLINICAL RELEVANCE: Patients with persistent or recurrent radicular symptoms after cervical ADR may achieve resolution of symptoms through a modified ACF technique.

Occipitocervical fusion of traumatic atlanto-occipital dissociation in a patient with autofused cervical facet joints: illustrative case.

BACKGROUND: Patients who survive traumatic atlanto-occipital dissociation (AOD) may present with normal neurological examinations and near-normal-appearing diagnostic images, such as cervical radiographs and computed tomography (CT) scans. OBSERVATIONS: The authors described a neurologically intact 64-year-old female patient with a degenerative autofusion of her right C4-5 facet joints who presented to their center after a motor vehicle collision. Prevertebral soft tissue swelling and craniocervical subarachnoid hemorrhage prompted awareness and consideration for traumatic AOD. An abnormal occipital condyle-C1 interval (4.67 mm) on CT and craniocervical junction ligamentous injury on magnetic resonance imaging (MRI) confirmed the diagnosis of AOD. Her autofused right C4-5 facet joints were incorporated into the occipitocervical fusion construct. LESSONS: Traumatic AOD can be easily overlooked in patients with a normal neurological examination and no associated upper cervical spine fractures. A high index of suspicion is needed when evaluating CT scans because normal values for craniocervical parameters are significantly different from the accepted ranges of normal on radiographs in the adult population. MRI of the cervical spine is helpful to evaluate for atlanto-occipital ligamentous injury and confirm the diagnosis. Occipitocervical fusion construct may need to be extended to incorporate spinal levels with degenerative autofusion to prevent adjacent level degeneration.

Evaluation of tortuous vertebral arteries before cervical spine surgery: illustrative case.

BACKGROUND: Cervical spine surgery sometimes necessitates complex ventral/dorsal approaches or osteotomies that place the vertebral artery (VA) at risk of inadvertent injury. Tortuosity of the VA poses increased risk of vessel injury during anterior decompression or placement of posterior instrumentation. OBSERVATIONS: In this report, the authors describe a patient with degenerative cervical spondylotic myelopathy and focal kyphotic deformity requiring corrective surgery via a combined ventral/dorsal approach. Computed tomography (CT) and CT angiography (CTA) of the spine identified a left medially enlarged C4 transverse foramen and tortuous VA V2 segment forming a potentially dangerous medial loop into the vertebral body, respectively. The patient's presentation and management are described. LESSONS: The course of the VA is variable, and a tortuous VA with significant medial or lateral displacement may be dangerous during ventral and dorsal approaches to the cervical spine. CTA of the cervical spine is warranted in cases in which atlantoaxial fixation is needed or suspicious transverse foramen morphology is identified to understand the course of the VA and identify anatomical variations that would put the VA at risk during cervical spine surgery.

A phase I trial of surgical resection with Gliadel Wafer placement followed by vaccination with dendritic cells pulsed with tumor lysate for patients with malignant glioma.

High grade gliomas are associated with poor prognosis and high mortality. Conventional treatments and management of high grade gliomas have shown little improvement in 5-year overall survival. This phase I trial evaluated the safety, immunogenicity, and potential synergy of surgical resection with Gliadel Wafer implantation, followed by autologous tumor lysate-pulsed dendritic cell (DC) vaccine in patients with malignant glioma. Primary end points of this study were safety and surrogate markers of immunogenicity, overall survival, and progression free survival. Following surgical resection, Gliadel Wafers were placed along the resection cavity. Patients subsequently received intradermal injections of autologous tumor lysate-pulsed DC vaccines 3 times at 2 week intervals. Treatment response was evaluated clinically and through MRI at regular intervals. Twenty-eight patients received Gliadel Wafers and DC vaccination: 11 newly diagnosed (8 glioblastoma [GBM], 2 anaplastic astrocytoma [AA], and 1 anaplastic oligodendroglioma [AO]) and 17 recurrent (15 GBMs, 1 AA, and 1 AO) high grade gliomas. Immunogenicity data was collected for 20 of the 28 patients. Five of 20 patients showed elevated IFN-γ responses following vaccination. Median progression-free survival and overall survival for all GBM patients in the trial from the start of vaccination were 3.6 months and 16.9 months respectively. Comparisons between vaccine responders and non-vaccine responders were not statistically significant. Adjuvant autologous dendritic cells pulsed with tumor-lysate following resection and Gliadel Wafer placement is safe, elicits modest immunogenicity and shows similar clinical outcomes in patients who had DC vaccination in previous studies.

Resection of Cervical Juxtacortical Chondroma and Circumferential Spinal Stabilization for Kyphotic Deformity.

Chondromas are rare, benign tumors composed of cartilaginous tissue that mainly affect the metaphases of long tubular bones. Juxtacortical (periosteal) chondromas arise from the surface of periosteum and rarely affect the cervical spine. We present a patient with a spinal juxtacortical chondroma causing spinal cord compression and a cervical deformity treated with surgical resection and circumferential spinal fixation and stabilization. A 55-year-old female with past medical history of Crohn's disease with years of neck pain, balance issues, and left upper extremity radicular symptoms. Cervical spine x-rays show kyphosis with an apex at C5, degenerative changes of the endplates and facet joints, and grade 2 anterolisthesis C4 on C5 with no abnormal motion with flexion/extension. MRI showed a left sided C5-6 extramedullary mass measuring 11 x 11 x 15 mm causing spinal cord compression and neural foraminal narrowing. Her pain is worsening and refractory to physical therapy, gabapentin and methocarbamol. A C4-5 & C5-6 anterior cervical discectomy and fusion, C4-5 & C5-6 laminectomy for tumor resection, and C4-5 & C5-6 posterior fusion with instrumentation was performed. The tumor was completely removed in piecemeal fashion. Microscopic findings showed bland well differentiated cartilaginous neoplasm consistent with juxtacortical chondroma. Postoperative X-rays show partial reduction of C4-5 anterolisthesis and partial reversal of cervical kyphosis. The patient's radicular pain resolved and neck pain improved postoperatively but she still has some left sided neck pain and hand dysesthesias that are controlled with oral medication one year following surgery. Cervical chondromas are rare, benign cartilaginous tumors that may present with spinal cord or nerve root compression. They are more complex when they present in patients with co-existing spinal deformities. Maximal safe resection followed by spinal re-alignment and fixation without adjuvant chemotherapy or radiation is recommended in most cases. Close follow-up is recommended to monitor for recurrence.

L5 Osteoid Osteoma Treated with Partial Laminectomy and Cement Augmentation.

Osteoid osteoma is a benign primary bone tumor of unknown etiology that occurs most commonly in males during adolescence and early adulthood. Osteoid osteoma affects the spine in 20% of cases, and may cause spinal deformity, stiffness, and pain that may sometimes be worst at night. We present a novel description of a partial laminectomy with cement augmentation after resection of an osteoid osteoma. A 22-year-old male with a past medical history of Hodgkin's lymphoma status post chemotherapy and radiation to the mediastinum, and right hip osteoblastoma treated with surgery and radiofrequency ablation presented with low back pain for five years with a recent onset of severe radicular symptoms. The pain was described as shooting and radiating laterally down the right leg to the mid-calf without bowel or bladder incontinence. He has a known right L5 laminar sclerotic lesion measuring 11 x 10 mm causing neuroforaminal narrowing and it kept increasing in size despite previous treatment with stereotactic radiosurgery and radiofrequency ablation. This lesion was metabolically active on positron emission tomography-computed tomography (PET-CT) imaging. His pain was worsening and was refractory to physical therapy, non-steroidal anti-inflammatory drugs (NSAIDs), aspirin, and radiation therapy. A right L5 partial laminectomy was performed to resect the abnormality in an en-bloc fashion. The lesion did not involve the inner cortex of the bone. Lamina reconstruction was achieved with bone cement augmentation for the preservation of vertebral column strength. Pathology was consistent with osteoid osteoma with marrow edema. Microscopic findings include bony trabeculae associated with prominent rimming and hypercellular fibroblastic stroma. No nuclear atypia, necrosis or appreciable mitotic activity was observed. The patient remains neurologically intact with significantly improved radicular symptoms and low back pain. Osteoid osteoma of the lamina may be resected using a partial laminectomy and cement augmentation done to preserve the integrity of the posterior ligamentous complex, prevent potential fracture of the pars interarticularis, and avoid the need for lumbar fusion in younger patients in whom this pathology is commonly found.

Whole brain radiation therapy (WBRT) alone versus WBRT and radiosurgery for the treatment of brain metastases.

BACKGROUND: Historically, whole brain radiation therapy (WBRT) has been the main treatment for brain metastases. Stereotactic radiosurgery (SRS) delivers high-dose focused radiation and is being increasingly utilized to treat brain metastases. The benefit of adding SRS to WBRT is unclear. This is an updated version of the original Cochrane Review published in Issue 9, 2012. OBJECTIVES: To assess the efficacy of WBRT plus SRS versus WBRT alone in the treatment of adults with brain metastases. SEARCH METHODS: For the original review, in 2009 we searched the following electronic databases: CENTRAL, MEDLINE, Embase, and CancerLit in order to identify trials for inclusion in this review. For the first update the searches were updated in May 2012.For this update, in May 2017 we searched CENTRAL, MEDLINE, and Embase in order to identify trials for inclusion in the review. SELECTION CRITERIA: We restricted the review to randomized controlled trials (RCTs) that compared use of WBRT plus SRS versus WBRT alone for upfront treatment of adults with newly diagnosed metastases (single or multiple) in the brain resulting from any primary, extracranial cancer. DATA COLLECTION AND ANALYSIS: We used the generic inverse variance method, random-effects model in Review Manager 5 for the meta-analysis. MAIN RESULTS: We identified three studies and one abstract for inclusion but we could only include two studies, with a total of 358 participants in a meta-analysis. This found no difference in overall survival (OS) between the WBRT plus SRS and WBRT alone groups (hazard ratio (HR) 0.82, 95% confidence interval (CI) 0.65 to 1.02; 2 studies, 358 participants; moderate-quality evidence). For participants with one brain metastasis median survival was significantly longer in the WBRT plus SRS group (6.5 months) versus WBRT group (4.9 months; P = 0.04). Participants in the WBRT plus SRS group had decreased local failure compared to participants who received WBRT alone (HR 0.27, 95% CI 0.14 to 0.52; 2 studies, 129 participants; moderate-quality evidence). Furthermore, we observed an improvement in performance status scores and decrease in steroid use in the WBRT plus SRS group (risk ratio (RR) 0.64 CI 0.42 to 0.97; 1 study, 118 participants; low-quality evidence). Unchanged or improved Karnofsky Performance Scale (KPS) at six months was seen in 43% of participants in the combined therapy group versus only 28% in the WBRT-alone group (RR 0.78 CI 0.61 to 1.00; P value = 0.05; 1 study, 118 participants; low-quality evidence). Overall, risk of bias in the included studies was unclear. AUTHORS' CONCLUSIONS: Since the last version of this review we have identified one new study that met the inclusion criteria. However, due to a lack of data from this study we were not able to include it in a meta-analysis. Given the unclear risk of bias in the included studies, the results of this analysis have to be interpreted with caution. In our analysis of all included participants, SRS plus WBRT did not show a survival benefit over WBRT alone. However, performance status and local control were significantly better in the SRS plus WBRT group. Furthermore, significantly longer OS was reported in the combined treatment group for recursive partitioning analysis (RPA) Class I patients as well as patients with single metastasis. Most of our outcomes of interest were graded as moderate-quality evidence according to the GRADE criteria and the risk of bias in the majority of included studies was mostly unclear.

Rubinstein-Taybi Syndrome Associated with Pituitary Macroadenoma: A Case Report.

Rubinstein-Taybi Syndrome (RSTS) is an autosomal dominant disorder that is classically characterized by prenatal and postnatal growth restriction, microcephaly, dysmorphic craniofacial features, broad thumbs and toes, and intellectual disability. We describe the first reported case of a pituitary macroadenoma associated with RSTS. A 39-year-old Caucasian female with a past medical history of RSTS diagnosed at age two was found to have a gadolinium-enhancing pituitary mass on magnetic resonance imaging (MRI) of the brain three years ago during workup for migraine-like headaches. Subsequent serial imaging showed radiographic evidence of growth up to 11.5 x 14.0 x 10.0 mm in size. The pituitary sellar lesion was resected through an endoscopic transnasal transsphenoidal approach and was found to be a thyrotroph adenoma. RSTS is a rare, neurodevelopmental genetic disease where most patients with disabilities survive into adulthood. The disorder is associated with an increased predisposition for development of nervous system tumors, including pituitary adenomas.

Proposed Diagnostic Criteria, Classification Schema, and Review of Literature of Notochord-Derived Ecchordosis Physaliphora.

Ecchordosis physaliphora (EP) is a benign notochordal remnant derived from ectopic nests found along the craniospinal axis. It typically presents asymptomatically and is diagnosed using classic radiologic features, particularly location, T1-hypointensity, T2-hyperintensity, and lack of enhancement following gadolinium (Gd) contrast administration. Distinguishing EP from its malignant counterpart, chordoma, is of paramount importance, given the aggressive nature of the latter. Advances in imaging and immunohistochemistry have aided in diagnosis to an extent but, to our knowledge, identification of the genetic fingerprint of EP has yet to take place. Further cytological analysis of these lesions in search of a genetic link is warranted. We propose here a set of diagnostic criteria based on features consistently cited in the literature. In this literature review, 23 case reports were identified and collated into a summary of symptomatic cases of ecchordosis physaliphora. An illustrative case report of two patients was also included.

A national perspective of adult gangliogliomas.

Gangliogliomas (GG) are rare tumors of the nervous system. Patient characteristics and clinical outcomes of low and high-grade GG have been difficult to elucidate in the adult population. This study aims to further elaborate on GG treatment and overall survival utilizing a larger cohort than previously published. The USA National Cancer Database was utilized to evaluate adult (age 18years and older) patients diagnosed with GG between 2004 and 2006. Descriptive statistics and Kaplan-Meier overall survival estimates were provided. A total of 198 adult GG patients were diagnosed between 2004 and 2006. Of these, 181 (91.4%) were low-grade and 17 (8.6%) high-grade GG. Overall, the median age was 36years; approximately 50% of patients were female, and 86.5% Caucasian. Most patients (59%) had near/gross total resection. Radiation and chemotherapy were prescribed in 18 (9.1%) and 11 (5.7%) patients, respectively. Radiation (64.7% versus 3.9%, p<.0001) and chemotherapy (47.1% versus 1.7%, p<.0001) were more frequently given to patients with high-grade tumors than low-grade. The median overall survival of high-grade GG was 44.4months (95% confidence interval [CI]: 10.5-92.5) while the corresponding estimate for low-grade tumors was not reached. Older age (hazard ratio [HR] 1.72, 95% CI: 1.26-2.34) and high tumor grade (HR 3.91, 95% CI: 1.43-10.8) were found to be associated with poor survival. Adult GG have a temporal lobe predilection and overall gross total resection rate of 59%. Older patients with high-grade tumors had an increased hazard of mortality. High-grade GG were significantly more likely to be treated with radiation therapy and chemotherapy.

Do Long-Term Survivor Primary Glioblastoma Patients Harbor IDH1 Mutations?

BACKGROUND: Approximately 3 to 16% of glioblastoma multiforme (GBM) patients are considered long-term survivors (LTS: 3+ years). OBJECTIVE: Given the improved survival conferred by IDH1 mutations and the fact that these mutations are detected in 12% of newly diagnosed GBM cases, could long-term survivorship be explained by IDH1 mutation status? Our aim was to describe GBM LTS with IDH1 mutations and explore its association with overall survival (OS). METHODS: Records of 453 newly diagnosed adult GBM patients treated at a single institution from 2004 to 2010 were reviewed retrospectively for patients who survived at least 36 months postsurgery. Descriptive statistics for clinical characteristics, treatments received, and tumor biomarkers were reported. Estimates for progression-free survival (PFS) and OS were provided. RESULTS: Forty (8.8%) LTS GBM patients were identified, with a median age of 50 years and a median preoperative Karnofsky Performance Score (KPS) of 80. Most patients underwent near-total/gross-total resection (72.5%), postoperative radiation (97.5%), and adjuvant temozolomide (95%). PFS rates at 12, 36, 48, and 72 months were 67.5%, 40%, 32.7%, and 26.2%, respectively. Median OS has not yet been reached; however, the survival rate at 48 months was 62.1%. Among 35 patients with available tumor samples, only 8 (22.9%) had IDH1 mutations. No significant difference in median PFS was found between IDH1 mutation and wild-type patients (46.6 versus 26.3 months; p =0.45). CONCLUSIONS: Less than a quarter of our patients' long-term survivorship was associated with favorable IDH1 status. Therefore, IDH1 status does not explain most of the long-term survivorship in the temozolomide era.