Primary intradural extramedullary lesions: a longitudinal study of 212 patients and analysis of predictors of functional outcome.

BACKGROUND: Primary intradural extramedullary (IDEM) lesions are rare, with an incidence of about 1/100,000 person-years. The aim of this study was to investigate their demographic, clinical, imaging, management, histopathological and outcome parameters. Another objective was to evaluate the various predicting factors leading to long-term favorable outcomes, thereby answering the controversial question: when to operate? METHODS: This study observed 212 patients of primary IDEM lesions and followed-up for a mean of 53.80 months. The patient's outcome using McCormick grade at follow-up was correlated with age, sex, duration of symptoms, preoperative McCormick grade, tumor location and extent, extradural spread, extent of excision, vascularity, WHO grade and histopathological tumor type. RESULTS: Benign nerve sheath tumors were the commonest lesions (47.17% schwannoma, 4.72% Neurofibroma), followed in incidence by meningioma (19.34%). There was predominance of males (57.08%), except in meningiomas (male: female ratio 1:2.15). Pain was the commonest initial symptom (51.88%). Limb weakness was the most common presenting complaint (88.68%). Gross total excision was achieved in 81.60% of cases and 70.75% of patients improved following surgery. The significant factors predicting favorable outcome included preoperative McCormick grade (P=0.001), the vertical extent of the tumor (P=0.027), histopathological tumor type (P=0.023) and WHO grading (P=0.015); and extent of excision had an odds ratio of 1: 2.5. CONCLUSIONS: Significant predictors of functional outcome following surgery in IDEM lesions included preoperative McCormick grade, extent of the tumor, tumor type, WHO grading and extent of resection. The authors recommend surgery with the intent of complete tumor excision, before the onset of substantial symptoms, for better outcome.

Intracranial Meningeal Hemangiopericytomas: An Analysis of Factors Affecting Outcome in 39 Cases Managed with Multimodality Treatment.

AIM: To retrospectively evaluate the outcome of patients with intracranial meningeal hemangiopericytomas (MHPCs) and to analyze various factors for recurrence and survival in these patients. MATERIALS AND METHODS: We retrospectively reviewed the clinical data of 39 patients undergoing microsurgical resection for MHPCs at our institute from 2009 to 2015. RESULTS: Gross total excision (GTE) was achieved in 27 (69.2%) patients, whereas 12 (30.8%) underwent subtotal excision (STE). A total of 25 patients received radiotherapy (RT) (Conventional RT-15; GKT-10), 21 patients had a low grade tumor, while 18 had an anaplastic variant. Twenty patients (51.3%) developed recurrences and the average recurrence-free survival (RFS) was 56 months (range: 12-180 months). Eight patients (20.5%) died during the study period. The average overall survival (OS) was 77.2 months (range: 36-192 months). Two patients (5.1%) developed systemic metastases during follow-up. Patient age was not found to affect RFS or OS. GTE was associated with prolonged RFS and OS but the impact was not statistically significant (P-values = 0.160 and 0.414, respectively). Low tumor grade was associated with statistically significant longer RFS as well as OS (P-values = 0.049 and 0.013, respectively). Addition of adjuvant RT was associated with statistically significant prolongation of RFS (P value = 0.016); however, it was not associated with statistically significant OS benefits (P-value = 0.758). CONCLUSIONS: Our study suggests that a greater extent of excision, lower tumor grade, and addition of adjuvant RT have a positive impact on both RFS and OS; however, low grade and adjuvant RT were the only factors associated with statistically significant prolongation of RFS and only tumor grade was associated with statistically significant OS benefits.

Intracranial aspergillosis amongst immunocompetent patients: An experience with combined surgical and medical management of 18 patients.

OBJECTIVE: Fungal infections of central nervous system (CNS) commonly affect immunocompromised patients, however, recently such cases have been reported even amongst immunocompetent patients. PATIENTS & METHODS: In this study, we retrospectively analyzed outcome of 18 immunocompetent patients with histopathologically proven intracranial Aspergillosis undergoing combined surgical and medical management. RESULTS: The age of patients ranged from 5-65 years. Fourteen out of 18 patients had well defined lesions while 4 had diffuse disease. Paranasal sinuses were involved in 8 & cavernous sinus in 3 patients. Six patients had hydrocephalus. Four patients developed infarcts during their clinical course. Surgical interventions included gross (n = 4) or subtotal excision (n = 8), decompressive craniectomy & biopsy of lesion (n = 4), biopsy only (n = 2) and ventriculoperitoneal shunt placement (n = 6). All patients received postoperative antifungal therapy. The duration of follow up ranged from 10-60 months. Overall mortality was 44.4%. Mortality amongst patients undergoing gross total and subtotal excision was 25% & 50% respectively. Patients undergoing DC had a mortality of 25%. Both patients undergoing only biopsy died. Hydrocephalus was associated with a very high mortality (83.3%). Amongst surviving patients (n = 10), 6 patients became disease free & rest 4 had stable disease at last follow up. CONCLUSIONS: Intracranial aspergillosis is associated with high morbidity & mortality even amongst immunocompetent patients. An aggressive multidisciplinary management is thus needed to improve outcome. Our study shows that a combination of surgical excision or decompressive craniectomy and antifungal therapy can be helpful in improving prognosis of these patients.

Traumatic Atlantoaxial Spondyloptosis Associated with Displaced Odontoid Fracture: Complete Reduction via Posterior Approach Using "Joint Remodeling" Technique.

BACKGROUND: Atlantoaxial spondyloptosis (AAS), which is defined as complete displacement of facets of atlas anterior to the facets of axis such that there is no contact between the 2 articulating surfaces, is an extremely rare manifestation of atlantoaxial instability. The reason for an extreme rarity of traumatic AAS is probably the severity of injury in traumatic AAS that is usually incompatible to life. It represents the most severe form of atlantoaxial dislocation, and complete reduction in such a case presents a real technical challenge because of the interlocking of C1-C2 facets. Cranial traction fails to achieve reduction in such cases. CASE DESCRIPTION: In this report, we describe a case of traumatic odontoid fracture associated with AAS and discuss our technique of complete reduction of deformity via posterior approach. An 11-year-old child presented to us 4 months after a road traffic accident with progressive spastic quadriparesis. On evaluation, displaced type II odontoid fracture with AAS was detected. The patient underwent surgery via posterior approach. The techniques of "joint manipulation" and "joint remodeling" were used to achieve complete reduction of spondyloptosis. Postoperative imaging showed complete reduction of deformity. The patient also improved neurologically after surgery. CONCLUSION: This case report aims to present the ability of "joint manipulation" and "joint remodeling" techniques in achieving excellent reduction in even one of the most difficult post-traumatic deformities that affect the craniovertebral junction.

Diencephalic Syndrome as Presentation of Giant Childhood Craniopharyngioma: Management Review.

Diencephalic syndrome (DES) is an extremely uncommon occurrence, and approximately 100 cases have been reported. It presents as a failure to thrive in infants and children but rarely occurs in adult population. The characteristic clinical features of DES include severely emaciated body, normal linear growth and normal or precocious intellectual development, hyperalertness, hyperkinesis, and euphoria usually associated with intracranial sellar-suprasellar mass lesion, usually optico-chiasmatic glioma or hypothalamic mass. DES as a presentation of craniopharyngioma is extremely uncommon but can also occur with brain stem mass. Detailed PubMed and MEDLINE search for craniopharyngioma associated with DES yielded only six cases in children below 6 years of age. Thus, we reviewed a total of seven cases including previously published six cases and added additional our own case. Overall, the mean age at diagnosis was 4.15 years with male:female ratio of 4:3, the mean time interval between symptom of DES appearance and final diagnosis was 6.6 months. The most commonly observed symptom of DES was weight loss (85%). The clinical feature, imaging, and management of such rare syndrome along with pertinent literature are briefly reviewed.

Traumatic spondyloptosis: a series of 20 patients.

OBJECT Spondyloptosis represents the most severe form of spondylolisthesis, which usually follows high-energy trauma. Few reports exist on this specific condition, and the largest series published to date consists of only 5 patients. In the present study the authors report the clinical observations and outcomes in a cohort of 20 patients admitted to a regional trauma center for severe injuries including spondyloptosis. METHODS The authors performed a retrospective chart review of patients admitted with spondyloptosis at their department over a 5-year period (March 2008-March 2013). Clinical, radiological, and operative details were reviewed for all patients. RESULTS In total, 20 patients with spondyloptosis were treated during the period reviewed. The mean age of the patients was 27 years (range 12-45 years), and 17 patients were male (2 boys and 15 men) and 3 were women. Fall from height (45%) and road traffic accidents (35%) were the most common causes of the spinal injuries. The grading of the American Spinal Injury Association (ASIA) was used to assess the severity of spinal cord injury, which for all patients was ASIA Grade A at the time of admission. In 11 patients (55%), the thoracolumbar junction (T10-L2) was involved in the injury, followed by the dorsal region (T1-9) in 7 patients (35%); 1 patient (5%) had lumbar and 1 patient (5%) sacral spondyloptosis. In 19 patients (95%), spondyloptosis was treated surgically, involving the posterior route in all cases. In 7 patients (37%), corpectomy was performed. None of the patients showed improvement in neurological deficits. The mean follow-up length was 37.5 months (range 3-60 months), and 5 patients died in the follow-up period from complications due to formation of bedsores (decubitus ulcers). CONCLUSIONS To the authors' best knowledge, this study was the largest of its kind on traumatic spondyloptosis. Its results illustrate the challenges of treating patients with this condition. Despite deformity correction of the spine and early mobilization of patients, traumatic spondyloptosis led to high morbidity and mortality rates because the patients lacked access to rehabilitation facilities postoperatively.

Mount meningioma with tumor cap.

The association between meningioma and hyperostosis of adjacent calvarium is well established. However, the cause of hyperostosis is still not certain. Various authors in the past have tried to explain this phenomenon by preceding trauma or by bone irritation by the tumor without bone invasion, while some believe it to be because of stimulation of osteoblasts in normal bone by factors secreted by tumor cells. There are still others who believe it to be secondary to production of bone by the tumor itself, while some have hypothesised that vascular disturbances secondary to the tumor result in hyperostosis. The authors wish to report a case of parasagittal meningioma in which the bone overlying the meningioma was so hyperostotic that it appeared to look like a 'mountain'. In addition, the tumor appeared to invade through the bone to form a 'tumor cap' over the hyperostotic bone.

Congenital spinal lipomas: Role of prophylactic surgery.

INTRODUCTION: Congenital spinal lipomas constitute an important group of lesions causing tethered cord syndrome. Management of these lesions is challenging and role of prophylactic surgery for these lesions is still controversial. Hence, current study was undertaken with the aim to evaluate the role of prophylactic surgery in the management of these lesions. MATERIALS AND METHODS: A total of 147 consecutive patients, treated over a period of 10 years (January 2001-December 2010), are retrospectively analyzed. RESULTS: In our study, 93 patients had conus lipomas, 26 had filum lipomas and 28 had only lipomeningomyelocele. Boys and girls were almost equally represented. The age of patients at the time of surgery ranged from 15 days to 34 years with an average of 62 months (5.2 years). Neurological deficits were present in 101 (68) patients. The patients with neurological deficits were older in comparison to those neurologically intact (average age 6.2 versus 2.8 years, respectively). Difference in age between the two groups was statistically significant (P value 0.03). Neurological deterioration was observed in 8 (5) patients following surgery, out of which six patients developed transient deterioration and only two, had persisting deficits. None of the neurologically asymptomatic patients developed persistent neurological deficits. In the symptomatic group, nine patients (9) showed improvement in neurological status after surgery. CONCLUSIONS: In author's view, prophylactic surgery for congenital spinal lipomas is safe and effective. However, a well designed randomized controlled trial, to definitely and objectively prove the usefulness of prophylactic surgery is needed.