Surgical strategies in the management of aggressive spinal haemangiomas: Retrospective case series with literature review and a practical treatment algorithm.

Purpose: We studied the clinico-radiological features and treatment outcomes of patients with aggressive spinal haemangiomas. Methods: We undertook a retrospective review of 24 patients with aggressive spinal haemangiomas managed at our centre from 2004 to 2016. The cohort was divided into two groups. Group1 included patients managed from 2004 to 2009 while Group 2 was those treated between 2010 and 2016. Clinicoradiological features and treatment outcomes were studied. Results: Back pain (24/24) and myelopathy (18/24) were the most common presenting complaints. Over 80% (20/24) of patients, had involvement of the thoracic spine and more than 50% (13/24) had severe spasticity, being Nurick grade 4&5 at presentation. The various treatment modalities used were laminectomy with or without instrumented posterior fusion (10/24), corpectomy with instrumented fusion (10/24) and alcohol injection alone (4/24). Patients who were treated with surgery had significant clinical improvement at follow-up in both groups. Patients who underwent alcohol injection did not have any improvement in symptoms at follow-up. There was a change in our strategy in the later part of the series from a two staged anterior and posterior approach to a single staged posterior-only approach to address vertebral body disease with preoperative angioembolization. Conclusion: Haemangiomas are benign lesions with locally aggressive behavior in some cases. Results of conservative approaches such as alcohol injection in management of these lesions are discouraging. Aggressive surgical decompression combined with preoperative adjuncts such as angioembolization with or without stabilization reduces intra operative blood loss and results in good neurological recovery even in patients with severe myelopathy.

Clinical Spectrum, Therapeutic Outcomes and Prognostic predictors in Paraneoplastic Neurological Syndromes - Experiences from a Tertiary Care Center in India.

BACKGROUND: Paraneoplastic Neurological Syndromes (PNSs) are a heterogeneous group of immune-mediated disorders that often precede tumor diagnosis. There are few systematic studies on the spectrum and follow-up of PNSs. OBJECTIVE: To analyze the clinical spectrum, associated tumors, antibody profile, outcomes, and prognostic predictors in a cohort of PNSs admitted in a tertiary care center. METHODS: This retrospective study included 97 patients (2008-2019). PNSs were further classified as "classical," "nonclassical," "definite," and "possible." Clinical profile, diagnostic strategies, therapeutic options, and predictors of outcomes were identified. RESULTS: The median age was 54 years (range 17-81). Thirty-nine (40.2%) had classical PNS, and 58 (59.8%) had nonclassical PNS, 74 (76.3%) had "Definite" PNS while 23 (23.7%) had "Possible" PNS. Cerebellar degeneration, peripheral neuropathy, and encephalopathy were the three most common neurological syndromes. Tumors were diagnosed in 66 (68%) patients; Lung cancer was the most common primary tumor. Antibodies were positive in 52 (53.6%). Anti-Yo antibody and anti-Ma2 antibody were the most common antibodies. The majority (57.7%) received immunotherapy in addition to definitive treatment for the tumor. A good outcome was seen in 53 (54.6%). Factors associated with good outcome were: early diagnosis, mRS <3 at presentation, absence of metastatic disease, and adjuvant immunotherapy. CONCLUSION: A high index of clinical suspicion is essential for early diagnosis and prompt management of PNS, especially the nonclassical syndromes. Multimodality diagnostic imaging techniques and antibody profiling play a crucial role in the diagnosis. A favorable prognosis can be expected with the judicious use of immunotherapy and definitive treatment of malignancy.

Neuroimaging of pediatric infratentorial tumors and the value of diffusion-weighted imaging (DWI) in determining tumor grade.

BACKGROUND: Diffusion-weighted imaging (DWI) provides information about the cellular density of tumors. This feature is useful in grading and identifying different tumor types. PURPOSE: To assess the value of diffusion restriction and apparent diffusion coefficient (ADC) values in differentiating pediatric infratentorial tumors. MATERIAL AND METHODS: This was a retrospective review of the magnetic resonance imaging (MRI) of 82 children (age range 1-16 years) with infratentorial tumors. Histopathological grading after surgical excision/biopsy was categorized as low grade (WHO grades I and II) (n = 31; 29 pilocytic astrocytomas, 2 ependymomas) and high grade (WHO grade III and IV) (n = 51; 40 medulloblastomas, 8 anaplastic ependymomas, 1 anaplastic astrocytoma, 2 atypical rhabdoid teratoid tumors [ATRT]). MRI features and ADC values were compared among tumor types and grades using a two-tailed t test, Mann-Whitney U test for continuous data and Chi-square test for categorical variables. RESULTS: Diffusion restriction and low ADC value was a feature of high-grade tumors (P<0.001). The mean ADC values of the low-grade and high-grade tumors were 1.567 × 10-3mm2/s and 0.661 × 10-3mm2/s, respectively. Using 0.9 × 10-3mm2/s as the cut-off value, the sensitivity, specificity, positive and negative predictive values for differentiating the grades was 87%, 100%, 100%, and 81.8%, respectively. Significant differences were found between the mean ADC values of the individual tumor types (P<0.05), except between medulloblastoma and ATRT. CONCLUSION: ADC values and visual assessment of diffusion restriction are useful in tumor grading. The individual tumor types can be identified by an algorithmic approach, using DWI in conjunction with other described MRI features.

Bone Marrow-Derived Mesenchymal Stem Cells Augment Regeneration of Intervertebral Disc in a Reproducible and Validated Mouse Intervertebral Disc Degeneration Model.

BACKGROUND: Back pain and radicular pain due to disc degeneration are probably the most common problems encountered in neurosurgical practice. The experience and results of stem cell therapy in animal disc degeneration model will help us while doing clinical trials. OBJECTIVE: To study the effect of bone marrow-derived mesenchymal stem cells in an established mouse disc degeneration model. METHODS: An easily reproducible mouse coccygeal (Co) 4-5 disc degenerated model by CT-guided percutaneous needle injury was established. The mesenchymal stem cells (MSCs) were cultured from mouse bone marrow and validated. By an established technique, 24 mice disc degenerative models were generated and divided equally into 3 groups (test, placebo, and control). The test group received MSCs with fibrin glue scaffold and placebo group received only scaffold after 6 weeks of degeneration. The control group did not receive any injection. The effects of MSCs were analyzed 8 weeks post injection. RESULTS: The test group showed a significant change in disc height index (%) in micro CT, whereas in the placebo and control groups, there was no change. The Safranin O staining showed an increase in glycosaminoglycan content and the polarized imaging of picrosirius red staining showed restoration of the collagen fibers in annulus fibrosus, which was statistically significant. CONCLUSION: Intradiscal MSC injection restored disc height and promoted regeneration in the discs at the end of 8 weeks. MSC's niche depends on the microenvironment of the host tissue. These findings will be helpful for clinical trials.

The role of imaging in skull base osteomyelitis - Reviewed.

PURPOSE: Skull base osteomyelitis (SBO) is difficult to diagnose due to a wide array of clinical presentations. It can be life threatening if not treated promptly. The objective of this study is to identify the various neck spaces involved in skull base osteomyelitis, correlate them with the possible source of infection and identify the related complications. METHODS: Eighty nine consecutive either culture proven cases of skull base osteomyelitis, or culture negative cases with inflammation on histopathology responding to antibiotic therapy, presenting at a single non-government hospital in south India between January 2016 and December 2018 were included in this study. Images were reviewed by two radiologists and imaging findings were documented by consensus. RESULTS: Involvement of the parotid space, retromastoid space and (temporomandibular) TM joint was associated with otogenic source of infection (p value < 0.05); while, retropharyngeal/prevertebral involvement was associated with sphenoid and nasopharyngeal sources (p value < 0.05). Complications such as cavernous sinus thrombosis (p value = 0.023) and ICA involvement (p value = 0.014) were more commonly seen with central skull base osteomyelitis. Abscess formation was seen in all three groups of patients without a significant difference between the groups. CONCLUSION: Imaging plays an important role in determining the possible source of infection by identifying the involved neck spaces and this in turn can guide the clinician to a site for biopsy. Complications related to SBO can also be identified on imaging and can guide appropriate management.

Dynamic imaging of the craniovertebral junction is mandatory in patients with posterior circulation strokes.

INTRODUCTION: The course of the vertebral artery after exiting from the C1 foramen transversarium and prior to entering the dura lends itself to compression in C1-2 instability. However, atlantoaxial dislocation presenting with vertebrobasilar insufficiency and posterior circulation stroke (PCS) is rare. METHODS: In this retrospective study, we identified 96 patients with PCS who had complete radiological data. Ten (10.4%) patients had craniovertebral junction (CVJ) anomalies, of which six underwent surgery and four were managed conservatively. The clinical and functional outcomes were measured in the two groups. RESULTS: Left-sided strokes were seen in 7/10 patients, the majority of whom had left dominant vertebral arteries. The mean age at presentation in those with CVJ anomalies was 27.2 ± 12.8 years that was significantly lower than those without CVJ anomalies, 52.2 ± 14.5 years (p ≤ 0.001). The etiologies of PCS in those < 50 years were CVJ anomalies (30%), atherosclerosis (30%) and vasculitis (27%); however, the overwhelming majority of strokes in the > 50 year age group was atherosclerosis (91%). Postoperatively, there were no recurrent strokes in the operated patients, who also obtained significant clinical improvement on the modified Rankin Scale, Nurick Scale and modified McCormick Scale as compared to those who did not undergo surgery. CONCLUSION: Early diagnosis and surgical treatment of CVJ instability prevent recurrent strokes and improve outcomes in patients with PCS. Physicians and spine surgeons need to be sensitized regarding CVJ anomalies as a cause of PCS enabling early diagnosis with dynamic imaging particularly in the younger age group. These slides can be retrieved under Electronic Supplementary Material.

Clinical characteristics and complications of skull base osteomyelitis: A 12-year study in a teaching hospital in South India.

CONTEXT: Skull base osteomyelitis (SBO) is an uncommon disease with substantial morbidity and mortality. AIMS: The aim of this study is to characterize clinical features, outcomes, and complications of SBO. We also looked at differences in clinical profile in otogenic and non-otogenic SBO. MATERIALS AND METHODS: This is a single-center retrospective observational study. Patients aged more than 15 years of age with clinical and radiological diagnosis of SBO admitted in general medicine department in a teaching hospital in South India from March 2006 to February 2018 were recruited. RESULTS: A total of 41 patients with SBO were identified and included. Mean age was 56.9 ± 10.7 years. In all, 90% of patients (37/41) had diabetes mellitus and 29% (12/41) had recent head/neck surgery. Only 19% (8/41) needed ICU care, and mortality was 21% (9/41). Most common symptom was headache seen in 73% (30/41) of patients. Majority, 61% (25/41), had otogenic infections. Otogenic infections were associated with longer duration of diabetes mellitus (mean = 11.5 vs. 5 years, P = 0.01), higher creatinine levels (mean = 1.66 vs. 0.9 mg/dL, P = 0.014, odds ratio [OR] = 3.8), and higher incidence of cranial nerve palsy (92% vs. 56%; OR = 8.9) compared to non-otogenic SBO. Cranial nerve palsy (78%), meningitis (63%), and cerebral venous thrombosis (43%) were frequent complications of SBO in this study. The causative organisms for SBO in our cohort was bacterial in 60% (15/25) and fungal in 40% (10/25) of the patients. Surgical debridement for source control was done in 54% of patients (22/41) and was associated with survival at discharge (P = 0.001). CONCLUSIONS: Bacterial infections are the most common cause of SBO. Otogenic SBO is associated with longer duration of diabetes mellitus and higher incidence of cranial nerve palsy. Therapeutic surgical debridement plays an important role in treatment of SBO and is associated with improved survival.

Imaging Spectrum of Cavernous Sinus Lesions with Histopathologic Correlation.

Cavernous sinuses are paired interconnected venous plexuses situated in the floor of the middle cranial fossa on either side of the sella turcica and sphenoid sinus. They are lined by dura mater and consist of multiple venous channels within. The cavernous sinuses are intimately related to the internal carotid artery and its associated sympathetic plexus, the oculomotor nerve, the trochlear nerve, the abducens nerve, and the ophthalmic nerve. Cavernous sinuses are connected to the orbit, the pterygopalatine fossa, the infratemporal fossa, the nasopharynx, and the posterior cranial fossa by various foramina, fissures, and canals in the skull base. A multitude of structures in close relation to the cavernous sinus give rise to a myriad of possible pathologic conditions that can be broadly classified into (a) neoplastic, (b) vascular, (c) infective or inflammatory, or (d) miscellaneous lesions. These pathologic conditions can have overlapping clinical manifestations. Hence, imaging plays a crucial role in identifying the disease, assessing its extent, providing a pertinent differential diagnosis to guide further management, and suggesting a site or route for biopsy. MRI is the modality of choice to depict the cavernous sinuses, with CT and digital subtraction angiography playing supplementary roles in certain situations. In this article, the cavernous sinus lesions encountered in our institution during a 10-year period are reviewed. The purpose of the article is to (a) describe the anatomy of the cavernous sinus; (b) demonstrate the multimodality imaging spectrum of a wide variety of pathologic conditions involving the cavernous sinus, correlating with the histopathologic findings; (c) highlight important imaging clues for differential diagnosis; and (d) help the reader overcome potential pitfalls in interpretation. Online supplemental material is available for this article. ©RSNA, 2019.

Reliability of Imaging-Based Diagnosis of Lateral Ventricular Masses in Children.

OBJECTIVE: We studied the accuracy of the radiologic diagnosis of lateral ventricular masses in children (<20 years of age). METHODS: In this retrospective study, data were collected from children with lateral ventricular masses managed in our unit between 2001 and 2016. There were 43 patients (26 boys and 17 girls; mean age, 12.1 years; range, 8 months to 20 years). Magnetic resonance imaging was available for 36 patients, whereas in 7 patients only a contrast-enhanced computed tomography scan was available. The images were read independently by 3 radiologists, who were blinded to the pathology. Two differential diagnoses were offered for each patient. The agreement between the 3 radiologists was calculated using the Fleiss κ statistic. RESULTS: The common pathologic diagnoses were subependymal giant cell astrocytoma (SEGA) (20.9%), low-grade astrocytoma (16.3%), high-grade astrocytoma (9.3%), choroid plexus papilloma (9.3%), and meningioma (9.3%). The sensitivity, specificity, and accuracy of the radiologic diagnoses were 62%, 96.7%, and 94.2%, respectively. Low-grade tumors such as low-grade gliomas, SEGAs, choroid plexus papillomas, and meningiomas were diagnosed with a high level of accuracy. High-grade gliomas, choroid plexus carcinomas, and other malignant neoplasms were difficult to diagnose on imaging. Cavernous angiomas were also difficult to diagnose. There was only fair agreement between the 3 radiologists (Fleiss κ = 0.24). CONCLUSIONS: The pathologic spectrum of lateral ventricular tumors in children is wide, and identifying the pathology on imaging is difficult for malignant tumors. Benign tumors such as SEGAs, low-grade astrocytomas, and choroid plexus papillomas are relatively easier to diagnose. There is significant interobserver variability in the radiologic diagnosis of these tumors.

Management and Outcome in Patients with Advanced Juvenile Nasopharyngeal Angiofibroma.

Objective To report the management outcome in a series of patients with advanced juvenile nasopharyngeal angiofibroma (JNA). Design Retrospective study. Setting Tertiary care teaching hospital. Participants Forty-five patients classified as Radkowski stage IIIA or IIIB who presented to us over the past 10 years. Main Outcome Measures Surgical approaches used and disease free outcomes in patients with advanced JNA. Results Surgical access for the extracranial component included open (41.9%) and expanded endonasal approaches (58.1%). Craniotomy (16.3%), endoscopy-assisted open approach (7%), or expanded endonasal approach (20.9%) was performed to excise the skull base or intracranial component. Follow up ranged from 4 to 96 months (mean, 20.3 months). Of 35 patients who underwent imaging at the first postoperative follow up, 25 (71.4%) had negative scans. Three symptomatic patients with residual disease underwent endoscopic excision and had negative scans thereafter. Of two others who had radiation therapy, one was disease free and the other lost to follow up. Five others had stable, residual disease. Three patients (8.6%) with recurrent disease underwent surgical excision, of whom two had minimal, stable residual disease. At the last follow-up, 27 (77.1%) patients had negative scans, and 7 (20%) had stable residual disease with one (2.9%) patient lost to follow-up. Conclusions Advanced JNA may be successfully treated in most cases with expanded endonasal/endoscopy assisted ± craniotomy approach after appropriate preoperative evaluation. At follow-up, only symptomatic patients or those with enlarging residue require treatment; periodic imaging surveillance is adequate for those with stable disease.

A randomized controlled trial to determine the role of intraoperative lumbar cerebrospinal fluid drainage in patients undergoing endoscopic transsphenoidal surgery for pituitary adenomas.

BACKGROUND: Intraoperative cerebrospinal fluid (CSF) leaks are a frequent cause of morbidity in patients undergoing transsphenoidal surgery. This prospective study was performed to examine the impact of intraoperative lumbar subarachnoid drainage (LSAD) on the incidence of this complication and on the extent of resection in patients undergoing endoscopic transsphenoidal surgery for pituitary adenomas. MATERIALS AND METHODS: This prospective study was conducted in a single large academic medical center. All patients with pituitary adenomas who had not undergone prior transsphenoidal surgery were eligible for inclusion in the study. Patients were randomly assigned to undergo transsphenoidal surgery with intraoperative lumbar drain insertion (LSAD group) or no lumbar drain insertion (no LSAD group). An otolaryngologist independently determined the occurrence of an intraoperative CSF leak. Extent of tumor resection was determined by volumetric analysis of postoperative magnetic resonance images in patients with nonfunctional tumors or functional adenomas with a large suprasellar component. RESULTS: Sixty patients were eligible for inclusion, of which 30 were assigned to the LSAD group and 30 to the no LSAD group. There were no statistically significant differences in patient demographics, tumor pathology, or radiology between the two groups. The LSAD catheter was successfully inserted in all patients in the LSAD group. Intraoperative CSF drainage significantly reduced the incidence of CSF leak from 46.7% in the no LSAD group to 3.3% in the LSAD group (P < 0.001). However, there were no statistically significant differences in the incidence of postoperative CSF rhinorrhea between the two groups. There were no major catheter-related complications. There was no statistically significant difference in the extent of resection between the two groups. CONCLUSIONS: Controlled intraoperative CSF drainage significantly reduces the incidence of intraoperative CSF leakage in patients undergoing endoscopic transsphenoidal surgery for pituitary adenomas.

Cerebrovascular injury in cryptococcal meningitis.

Background Cryptococcal meningitis continues to be one of the common causes of chronic central nervous system infection worldwide. Individuals with cryptococcal meningitis can occasionally present with small vessel vasculitis causing infarcts primarily in the basal ganglia, internal capsule, and thalamus. Literature regarding patterns of cerebrovascular injury among patients with cryptococcal meningitis is scanty, and outcome following these vascular involvements is unknown. Aim To study the clinical profile, imaging findings, and details of vascular territory involved among patients admitted with cryptococcal meningitis and central nervous system infarct in a tertiary care center from India. And to compare the outcomes of patients of cryptococcal meningitis with or without central nervous system infarcts in terms of mortality and morbidity, Methodology A total of 151 patients with microbiologically proven cryptococcal meningitis over a time span of 11 years were retrospectively enrolled into the study. Of these, 66 patients met the inclusion criteria of having appropriate imaging of the brain. The presence of infarct in the imaging was analyzed by two independent radiologists. Patterns of central nervous system involvement and types of vascular injury were ascertained based on radiological parameters. Clinical parameters and outcomes of patients with and without infarcts were compared. Results Twenty (13%) of these patients had evidence of central nervous system infarcts on imaging. The mean age of patients with and without infarcts was 41 years and 38 years, respectively. Male predominance was present among both the groups. The presence of fever, neck stiffness, positive blood culture, and hydrocephalus in central nervous system imaging was similar among patients with or without infarct. Longer duration of illness, low sensorium at the time of presentation, low Glasgow Coma Scale score, presence of meningeal inflammation, cryptococcomas, and basal exudates in imaging were higher in patients with infarct. All the infarcts were of the lacunar type. Sixty percent of the cerebrovascular infarcts were acute in nature, 50% of these being multiple. Unilateral infarcts were seen in 70% of the patients. The most common site of infarct was the basal ganglia, others being distributed over the thalamus, frontal, temporal, parieto-occipital regions in the descending order. The presence of neurovascular involvement in the form of infarcts to the risk of morbidity and mortality had an odds ratio of 9.1 and 2.6, respectively. Conclusion Neurovascular involvement in chronic cryptococcal meningitis is a rare entity. These tend to present as multiple lacunar infarcts. Mortality and morbidity associated with these patients is higher when compared to patients who do not have infarcts. This result suggests that vascular injury plays a role in predicting outcome of patients with cryptococcal meningitis. Future studies are needed to understand the mechanism by which vascular events (infarcts) occur and result in poor outcome.

Cavernous sinus melanoma: A rare tumor.

Primay melanoma of the cavernous sinus is very rare with only few cases reported in the literature. We present the cross-sectional imaging findings of this rare tumor. The differential diagnosis for cavernous sinus mass lesion is wide as it contains vital neurovascular structures that may be affected by vascular, neoplastic, infective, and infiltrative lesions arising in the cavernous sinus proper or via extension from adjacent intra and/or extracranial regions. Radiologic imaging can narrow the differential diagnosis, however, imaging cannot definitely reach single diagnosis if they present in atypical form with hemorrage and cystic degeneration. This case report illustrates that primary cavernous sinus melanoma may present as a atypical tumor with diagnostic dilemma.

Sonothrombolysis for acute ischemic stroke - Break on through to the other side.

BACKGROUND: Intravenous (IV) tissue plasminogen activator (tPA) infusion combined with transcranial low-frequency ultrasound waves targeted on the occluded arterial segment (sonothrombolysis) can increase recanalization in large artery-acute ischemic stroke (LA-AIS). AIMS: To evaluate the benefits of sonothrombolysis in LA-AIS. SETTINGS AND DESIGNS: An open-labeled observational study done in a quaternary care teaching hospital. METHODOLOGY: Patients with LA-AIS within the window period (<4.5 h) with no contraindications for IV-recombinant tPA were sonothrombolysed. Recanalization was monitored and graded using the transcranial Doppler thrombolysis in brain ischemia (TIBI) flow criteria and also by time of flight magnetic resonance angiography using a modified thrombolysis in myocardial infarction score. Parenchymal changes were assessed using computed tomography (CT) or diffusion-weighted imaging-Alberta Stroke Programme Early CT Score. National Institutes of Health Stroke Scale (NIHSS) and modified Rankin Scale (mRS) were used to assess the outcome. RESULTS: Eighteen patients underwent sonothrombolysis and the mean onset to needle time was 138 min (range 65-256). TIBI residual flow grade of ≥2 was seen in 15 of 18 patients (83%). Immediate dramatic improvement (NIHSS score ≤3 points or improvement by ≥10 points) was seen in 6 of 18 patients (30%) and in 9 of 18 patients (50%) within the next 24 h. Two patients (one with TIBI 0, another with re-occlusion) underwent mechanical thrombectomy post-sonothrombolysis. Symptomatic hemorrhage occurred in 5.5% of the patients. At 6 months, 2 of 18 patients (11%) died and 10 of 16 patients (63%) achieved mRS ≤2. CONCLUSIONS: Sonothrombolysis appears to be a safe way to augment the effect of tPA without increasing the door to needle time with the added advantage of observing flow through the occluded artery in real time.

Three-dimensional computed tomography angiographic study of the vertebral artery in patients with congenital craniovertebral junction anomalies.

PURPOSE: To describe vertebral artery (VA) course at the C0-C1-C2 complex in patients with congenital bony craniovertebral junction (CVJ) anomalies. METHODS: We studied the course of 169 VAs in 86 patients with congenital bony CVJ anomalies [basilar invagination (42), os odontoideum (33), and irreducible atlantoaxial dislocation (11)]. Occipitalized atlas occurred in 41 patients (30 complete and 11 partial). Using axial, coronal and sagittal three-dimensional computed tomography (3D-CT) angiograms, we traced the VA bilaterally at the CVJ and correlated the course to the presence or absence of occipitalization of the atlas. RESULTS: Of the 73 arteries associated with occipitalization of atlas, all had an abnormal course-58 (78.4 %) coursed through a canal within the C0-C1 fused complex and 15 (20.3 %) coursed below the C1 posterior arch, and it was absent unilaterally in one patient. There were 96 arteries associated with a non-occipitalized atlas and only 15 (15.3 %) were abnormal-eight coursed below the C1 posterior arch, four coursed above the C1 arch in the absence of a C1 foramen transversarium, one passed through a canal in C0-C1 and two arteries were absent unilaterally. Sixty vertebral arteries (34 on the right and 26 on the left side) had a redundant loop situated at a distance of ≥5 mm from the C1 lateral mass in patients with os odontoideum and irreducible atlantoaxial dislocation. CONCLUSIONS: In occipitalization of the atlas, the VA course is usually abnormal-typically passing through a canal within the C0-C1 fused complex or below the C1 arch. A redundant VA loop is more likely to be seen in os odontoideum and irreducible atlantoaxial dislocation. Careful study of the vertebral artery course with 3D CT angiography is mandatory while contemplating CVJ realignment surgery in congenital anomalies of the CVJ.

Pure endoscopic transsphenoidal surgery for functional pituitary adenomas: outcomes with Cushing's disease.

BACKGROUND: This study was performed to examine patient outcomes following pure endoscopic transsphenoidal surgery (ETS) for Cushing's disease (CD). METHOD: We studied 64 consecutive patients who underwent 69 endoscopic transsphenoidal procedures. Radiological evaluation comprised detailed examination of preoperative magnetic resonance images (MRI), including positron emission tomography (PET) for select cases. Inferior petrosal sinus sampling (IPSS) was not performed for any patient. Remission was defined by the presence of hypocortisolemia with requirement for steroid replacement therapy or eucortisolemia with suppression to <1.8 µg/dl after 1 mg dexamethasone on evaluation at least 3 months after surgery. RESULTS: Preoperative MRI was abnormal in 87.5 % of cases and included 11 macroadenomas (17.2 %). PET was used to localize the adenoma in four cases. For microadenomas, operative procedures executed were as follows: selective adenomectomy (n = 15), enlarged adenomectomy (n = 21) and subtotal/hemihypophysectomy (n = 17). Overall, pathological confirmation of an adenoma was possible in 58 patients (90.6 %). Forty-nine patients (76.6 %) developed hypocortisolemia (<5 µg/dl) in the early postoperative period. Mean follow-up was 20 months (range 6-18 months). Remission was confirmed in 79.7 % of the 59 cases followed up for >3 months and was superior for microadenomas (86.4 %) versus macroadenomas (55.6 %) and equivocal MRI adenomas (66.7 %). Postoperative CSF rhinorrhea occurred in five patients, and new endocrine deficits were noted in 17.1 % patients. A nadir postoperative cortisol <2 µg/dl in the 1st week after surgery was highly predictive of remission (p = 0.001). CONCLUSION: ETS allows for enhanced intrasellar identification of adenomatous tissue, providing remission rates that are comparable to traditional microsurgery for CD. The best predictor of remission remains induction of profound hypocortisolemia in the early postoperative period.

Recurrent pericardial effusion and tamponade in a patient with Erdheim-Chester disease (ECD).

Erdheim-Chester disease (ECD) is a rare xanthogranulomatous disorder characterised by the proliferation of lipid laden histiocytes along with infiltration of various organs of the body. Although commonly presenting with bone pains secondary to bony infiltration, cardiac involvement in the form of periaortic fibrosis and pericardial involvement may be seen in a subgroup of patients. We report a case of ECD presenting as recurrent pericardial effusion along with pericardial tamponade.