Moving Beyond Morphometrics and Alignment: Prospective Longitudinal Study on Cognition, Quality of Life, and Diffusion Metrics in Congenital Craniovertebral Junction Anomalies.

BACKGROUND AND OBJECTIVES: Chiari type I malformation (CMI) has been implicated to cause cognitive impairment. Unusual for a craniovertebral junction (CVJ) anomaly, this phenomenon is postulated to be related to microstructural changes across the brain. However, this has not been confirmed in a longitudinal study, in bony CVJ anomalies, or in the context of quality of life (QOL). This study aimed to analyze cognition, QOL, and diffusion tensor imaging (DTI) metrics before and after surgery for congenital CVJ anomalies and evaluate their correlations with conventional clinico-radiological factors. METHODS: Fifty-seven patients with congenital CVJ anomalies-CMI, atlantoaxial dislocation (AAD), and basilar invagination (BI)-were evaluated in this prospective longitudinal study over 3 years with a neuropsychological battery, QOL and functional scores, and DTI metrics. A total of 43 patients (23 with CMI; 20 with AAD or BI) with 1-year postsurgical data were included in the final analysis. RESULTS: Cognitive dysfunction in one or more domains was noted in 15 (65%) of the patients with CMI and 16 (80%) of the patients with AAD/BI. Memory and visual motor perception were the most-affected domains in both the cohorts. At the 1-year follow-up, the proportion of patients demonstrating an overall cognitive dysfunction did not change significantly (P > .05). All patients demonstrated significant postoperative improvement in functional and QOL scores at follow-up. DTI metrics improved in many regions postoperatively, but did not correlate with functional or cognitive changes (P > .05). CONCLUSION: Cognition is affected in a majority of patients with CMI, AAD, or BI. These patients experience good functional and QOL outcomes after surgery but most of them continue to exhibit cognitive impairment. DTI metrics improve after surgery, but do not correlate with cognitive changes. These findings underline the multidimensional nature of the pathology of these anomalies that complicate achieving the concept of a "successful" surgery.

Giant Tumefactive Perivascular Spaces: An Unusual Cause of Holmes Tremor.

A 34-year-old man presented with a 2-year history of medically refractory Holmes tremor in the right upper limb. Magnetic resonance imaging revealed a large, nonenhancing, multiseptate cystic lesion of cerebrospinal fluid intensity in the left thalamopeduncular region causing brainstem compression and hydrocephalus. A diagnosis of giant tumefactive perivascular spaces was made after a biopsy ruled out an infectious or neoplastic etiology. Significant clinicoradiologic improvement was noted following a ventriculoperitoneal shunt. Giant tumefactive perivascular spaces should be included as one of the rare differentials of a large, nonenhancing cystic lesion situated along the course of perforator vessels. Treatment options include cerebrospinal fluid diversion with or without cyst fenestration.

Primary intracranial sarcoma masquerading as a chronic subdural haematoma: illustrative case and review of an unusual phenomenon.

It is very unusual for a primary intracranial malignancy to present as a chronic subdural hematoma. This case report describes one such case in a 3-year-old girl who presented with raised intracranial pressure following a mild head injury. Imaging of her brain revealed bilateral chronic subdural hematomas with an enhancing subdural mass and multiple nodular lesions infiltrating the brain parenchyma. She underwent a craniotomy, drainage of the subdural collections and resection of the subdural mass. Histopathology revealed an undifferentiated sarcoma, and she was referred for adjuvant therapy. This case underlines the importance of evaluating paediatric subdural collections for a possible underlying malignancy, even in the background of a preceding head injury. The cause-effect relationship of sarcomas with subdural collections remains unclear because of the scarcity of available literature on the subject.

Changes in Sinonasal and Overall Quality of Life Following Endoscopic Endonasal Surgery for Non-functioning Pituitary Adenomas: Results of A Prospective Observational Study.

Introduction: Quality of life (QOL) assessment is increasingly being recognized as an important parameter while evaluating outcomes after endoscopic endonasal transsphenoidal surgery (EETS). There has been no study that has evaluated this in the Indian context. Objectives: We aimed to analyze sinonasal and overall QOL before and after EETS for non-functioning pituitary adenomas (NFPAs), and to evaluate possible correlations between changes in QOL and various clinicoradiological, hormonal, and surgical factors. Methods: This prospective observational study included 62 patients who underwent EETS for NFPAs. The Anterior Skull Base Nasal Inventory-12 (ASK-12) and Short Form-12 (SF-12) were used to assess QOL. Changes in QOL were recorded at 2 weeks, 3 months, and 1-year follow-up periods. Results: While the nasal crusting and nasal discharge components of the ASK-12 worsened 2 weeks after EETS, the headache component demonstrated significant improvement (P < 0.05). The overall ASK-12 score (6.69+/-7.98) did not change significantly at follow-up. The SF-12 physical component summary (PCS) score (42.14+/-8.54) improved progressively after surgery, while the SF-12 mental component summary (MCS) score (42.48+/-7.95) improved 1 year later. The SF-12 PCS correlated with visual field improvement at 3-month follow-up (P = 0.04). Conclusions: EETS affects some components of the ASK-12 without affecting the overall sinonasal QOL in patients with NFPAs. These patients demonstrate progressive postoperative improvement in physical well-being, while their mental functioning improves only a year later. Improvement in physical functioning correlates with visual field improvement at a 3-month follow-up. Hormonal dysfunction and surgical factors do not have any impact on QOL in these patients.

Posterior Reversible Encephalopathy Syndrome Complicating the Postoperative Clinical Course in a Child with a Suprasellar Craniopharyngioma.

Posterior reversible encephalopathy syndrome (PRES) is an unusual neurological condition known to occur in the setting of various risk factors such as acute hypertension, renal failure, sepsis, multiorgan failure, autoimmune disease, and immunosuppression. Twenty cases of PRES have been previously reported in neurosurgical literature, almost all of which were attributed to either hypertension or chemotherapeutic drugs. Herein, we report a case of PRES in a neurosurgical patient in the absence of the commonly described risk factors for the syndrome. The patient was a 9-year-old boy who underwent uneventful transcranial resection of a suprasellar craniopharyngioma. His postoperative clinical course was complicated by hepatic failure and hypothalamic dysfunction, both of which were managed conservatively. Ten days after the onset of these complications, he developed clinical and radiological features suggestive of PRES which eventually resolved at follow-up. We discuss the pathogenesis of this unusual neurological syndrome in our patient in the light of a literature review.

Spinal Morphometry As A Novel Predictor For Recurrent Lumbar Disc Herniation Requiring Revision Surgery: Results of A Case Control Study.

Introduction: There is conflicting data on the risk factors for recurrent lumbar disc herniation (rLDH). Most of the predictors for rLDH identified so far are acquired risk factors or radiological factors at the level of the herniation. Whole lumbar spine (WLS) morphometry has not been evaluated as a possible predictor of rLDH. Objectives: We aimed to evaluate if preoperative spinal morphometry can predict the occurrence of rLDH requiring revision surgery. Methods: This retrospective case-control study on 250 patients included 45 patients operated for rLDH, 180 controls without rLDH who had previously undergone microdiscectomy for a single level lumbar disc prolapse, and a holdout validation set of 25 patients. Morphometric variables related to the WLS were recorded in addition to previously identified predictors of rLDH. Logistic regression (LR) analysis was performed to identify independent predictors of rLDH. Results: LR yielded four predictors of which two were WLS morphometric variables. While increasing age and smoking positively predicted rLDH, increasing WLS interfacet distance and WLS dural-sac circumference negatively predicted rLDH. The LR model was statistically significant, χ2 (4) =15.98, P = 0.003, and correctly classified 80.3% of cases. On validation, the model demonstrated a fair accuracy in predicting rLDH (accuracy: 0.80, AUC: 0.70). Conclusions: Larger mean lumbar bony canals and dural sacs protect from the occurrence of symptomatic rLDH. These WLS morphometric variables should be included in future risk stratification algorithms for lumbar disc disease. In addition to the previously recognized risk factors, our study points to an underlying developmental predisposition for rLDH.

Occipital Interhemispheric Transtentorial Approach for Tumors of Posterior Third Ventricular Region: Review of Surgical Results.

Background: Controversies exist regarding the ideal surgical approach for tumors in posterior third ventricular region (PTV). Objective: To evaluate the results of occipital interhemispheric transtentorial (OITT) approach for tumors in PTV. Materials And Methods: Thirty-three patients underwent surgery via OITT approach for PTV tumors at Sri Sathya Sai Institute of Higher Medical Sciences during the study period of 5 years (June 2011-May 2016). Ideal trajectory for OITT approach was determined by neuronavigation. Endoscope was used for removing any residual lesion at the blind spots. Results: Postoperative magnetic resonance imaging (MRI) performed in all the patients revealed gross total or near-total (>95%) excision of tumor in 31 patients (93.9%). Preoperative neurological deficits improved either completely or significantly following excision of the tumor in 73.3% (11/15) of the patients. Outcome was good (modified Rankin scale ≤2) at discharge in 93.9% (31/33) and at a final follow-up of 3 months or more in 96.8% (30/31) of the patients. None of the patients died during the postoperative period. Complications included upgaze palsy (transient- 6.1% [2/33], persisting- 3% [1/33]), visual field defects (transient- 3% [1/33], persisting- 3% [1/33]), transient third nerve paresis (1/33-3%), transient hemiparesis (1/33-3%), operative site hematoma (1/33-3%), small posterior cerebral artery (PCA) territory infarct (1/33-3%), and small venous infarct (1/33-3%). At least one follow-up MRI could be performed in 23 patients. Final follow-up MRI revealed no recurrence or increase in the size of the residual lesion compared to postoperative images in 20 patients (20/23-87%) and large recurrence in 3 patients (3/23-13%) with high-grade lesions. Conclusion: Gross total/near-total excision can be performed in majority of the PTV tumors through OITT approach with minimal morbidity and mortality.

CNS paraganglioma: 15-year experience in a tertiary care hospital with literature review.

Paraganglioma that involves the CNS may mimic clinico-radiologically many other commoner entities. The current study presents a wide view of the clinical, radiological, and histomorphological spectrum along with rare associations that can occur concurrently with this lesion. The most common site of infliction in CNS is the spine and, in the current series, involvement of the lumbar spine was most frequent. Both clinical and radiological features point towards other more common differentials, including neurofibroma/schwannoma and ependymoma. Some studies suggest rich vascularity (cap sign) and salt pepper appearance in T2-weighted images to serve as soft pointers towards diagnosing it on magnetic resonance imaging, however, in our series we did not encounter the same.

Outcomes after Cervical Duraplasty for Monomelic Amyotrophy (Hirayama Disease): Results of a Case-Control Study of 60 Patients.

Background The optimal management and the role of surgery in monomelic amyotrophy, also known as Hirayama disease (HD), remain controversial. In the largest series of patients with HD managed by cervical duraplasty (CD), this study compares the outcomes between conservative and surgical management. Methods A retrospective case-control study was performed on 60 patients with HD. The cases consisted of 30 patients who underwent CD, and 30 age- and sex-matched controls who managed with long-term collar wear. Clinical improvement was recorded using two patient-reported outcome measures-the Odom's scale and a self-rated hand grip strength score. Median and ulnar compound muscle action potential (CMAP) amplitudes and various magnetic resonance imaging parameters were recorded. Results None of the controls experienced clinical improvement at follow-up; 90% remained status quo at 24.33 ± 9.38 months. Seventy-six percent of the operated patients demonstrated clinical improvement at 29.61 ± 12.78 months, and a majority of them reported moderately better grip strength. The preoperative anteroposterior diameter (APD) of the surgical group improved from 0.74 ± 0.13 to 0.86 ± 0.21 ( p = 0.01), and the cord demonstrated a significant decrease in forward migration on flexion (0.20 ± 0.11 and 0.03 ± 0.07 preoperatively and at follow-up, respectively, p < 0.0001). Patients with clinical improvement demonstrated significantly better mean APD and median and ulnar CMAP values than those who did not improve ( p < 0.0001). The mean cervical alignment did not change significantly at follow-up ( p = 0.13). Conclusions This study shows that CD can significantly alter the clinical course of patients with progressive symptoms of HD. This approach successfully arrests the forward migration of the cervical cord on flexion and promotes morphological recovery of the cord. A majority of the patients undergoing CD demonstrate clinical and electrophysiological improvement at mid-term follow-up.

An Unusual Case of a Conus Hanging by a Thread.

We report a rare cause of paraparesis in a 2-year-old girl. Along with lower limb weakness, she presented with a neurogenic bladder and lower limb deformities. Her magnetic resonance imaging showed multiple dorsolumbar segmentation defects and a dysgenetic spinal cord segment between T10 and S1. A thin septum connected the lower end of the normal cord to a bulky conus at S1-2. These features were suggestive of type 1 segmental spinal dysgenesis. The findings were confirmed at surgery, and the child was referred for supportive care.

Outcome of Surgery for Congenital Craniovertebral Junction Anomalies with Atlantoaxial Dislocation/Basilar Invagination: A Retrospective Study of 94 Patients.

OBJECTIVE: To evaluate the results of surgery for congenital craniovertebral junction (CVJ) anomalies with atlantoaxial dislocation (AAD)/basilar invagination (BI) and compare the results of transoral odontoidectomy and posterior fusion (TOO+PF) with only posterior fusion (PF) in patients with irreducible AAD/BI. METHODS AND RESULTS: All 94 patients with congenital CVJ anomalies with AAD/BI operated on during the 3-year study period (June 2013-May 2016) were included. Of these patients, 55 had irreducible AAD/BI and the remaining 39 had reducible AAD/BI. TOO+PF was restricted to patients (34/94; 36.2%) with irreducible AAD/BI when reduction and realignment by intraoperative C1-C2 facet joint manipulation were considered technically difficult and risky. The remaining patients with irreducible AAD/BI and all the patients with reducible AAD/BI (60/94; 63.8%) were managed with only posterior fusion. Poor preoperative Nurick grade, preoperative dyspnea/lower cranial nerve deficits, and syringomyelia were associated with significantly higher incidence of postoperative pulmonary complications. Follow-up ≥3 months (final follow-up) was available for 87 patients. Good outcome (Nurick grade 0-3) at final follow-up was noted in 90% (45/50) of the patients with irreducible AAD/BI and 91.9% (34/37) of the patients with reducible AAD/BI. Preoperative poor Nurick grade (4-5) was the only factor associated with poor outcome. No significant difference in perioperative complications, outcome, and fusion was noted between patients who underwent TOO+PF or only PF for irreducible AAD/BI. CONCLUSIONS: Many of the patients with congenital AAD/BI showed remarkable recovery after surgery. Preoperative poor Nurick grade (4-5) is associated with poor outcome. TOO+PF is a safe alternative treatment option for irreducible AAD/BI when only PF techniques are technically difficult/risky.

Fatal holocord recurrence of a pregnancy-related, low-grade spinal ependymoma: case report and review of an unusual clinical phenomenon.

INTRODUCTION: Pregnancy-related spinal tumors (PRSTs) are unusual tumors that present during pregnancy or within a year after delivery. We describe a fatal holocord recurrence of a spinal ependymoma, which, to the best of our knowledge, is one of the most extensive PRSTs reported thus far. CASE PRESENTATION: A 21-year-old primigravida presented at 6 months of gestation with urinary incontinence for 2 months and spastic paraparesis for 1 month. MRI showed a conus intramedullary lesion from T10 to 12. Near-total resection of the lesion was performed. The histopathological diagnosis was that of a cellular ependymoma (WHO grade II). The patient presented 6 months later with progressive quadriparesis and breathing difficulty. MRI demonstrated holocord recurrence of the tumor with edema extending to the pontomedullary junction. The patient succumbed to respiratory failure before decompression of the tumor could be performed. DISCUSSION: This case highlights an unusual clinical course of a pregnancy-related, low-grade spinal ependymoma. The possible hormonal and genetic mechanisms underlying the aggressive involvement of the entire spinal cord by the recurrent tumor are discussed in the light of a literature review. Future studies may shed light on the possibility of utilizing these mechanisms as therapeutic targets to alter the clinical course of aggressive spinal ependymomas.

Far lateral approach without occipital condylar resection for intradural ventral/ventrolateral foramen magnum tumors and aneurysms of V4 segment of vertebral artery: Review of surgical results.

BACKGROUND: Controversies exist regarding the need and extent of condylar resection for safe surgical management of intradural ventral/ventrolateral foramen magnum (VFM) tumors and aneurysms of V4 segment of vertebral artery (VA) by far lateral approach. This retrospective study was conducted to evaluate the results of basic far lateral approach(retrocondylar approach) without upfront occipital condylar resection. METHODS AND RESULTS: Twenty one patients underwent surgery via far lateral approach for intradural VFM tumors and aneurysms of V4 segment of VA at Sri Sathya Sai Institute of Higher Medical Sciences during 9 years(2008-2016) study period. Eight patients had VA aneurysms and 13 patients had intradural VFM tumors. After basic far lateral approach(retrocondylar approach), dura was opened and checked if the exposure was adequate for safe surgery. Retrocondylar approach provided adequate exposure for all these lesions and resection of occipital condyle/jugular tubercle was not required in any of these cases. Skeletonization or transposition of VA was not done in any of these cases. Gross total resection of the tumor could be done in 9 patients(9/13-69.2 %) and near total excision (>95 %) in 4 patients (4/13-30.8 %). Seven of the 8 VA aneurysms were successfully clipped. Outcome at a final follow up of 3 months or more was good(mRS<2) in 19 patients(19/21-90.5 %) and poor in 2 patients. Complications included lower cranial nerve deficits [transient-2/21(9.5 %), persisting-2/21(9.5 %)], motor deficits(2/21-9.5%), seventh nerve paresis(1/21-4.8%), sixth nerve paresis(2/21-9.5%) and pseudomeningocele(1/21-4.8%). CONCLUSION: Basic far lateral (retrocondylar) approach provides excellent exposure for majority of VFM tumors and aneurysms of V4 segment of VA. Condylar resection(transcondylar approach), drilling of jugular tubercle (transtubercular approach), skeletonization/transposition of VA might not be required for safe surgical management of majority of these lesions.

Morphometric Alterations of the Sphenoid Ostium and other Landmarks in Acromegaly: Anatomical Considerations and Implications in Endoscopic Pituitary Surgery.

BACKGROUND: The sphenoid ostium (SO) is an important landmark for the endoscopic surgeon. Changes in size and position of the SO and variations in other skull base landmarks in acromegalics have not been adequately evaluated. AIMS: The authors evaluated the morphometry and location of the SO and other landmarks in acromegaly and compared these findings with those in nonfunctioning pituitary adenomas (NFPAs). METHODS: In this retrospective case-control study, the dimensions and location of the SO and other skull base landmarks were radiologically evaluated in 18 patients with growth hormone (GH)-secreting adenomas. These findings were analyzed in relation to preoperative GH levels and compared with 18 age- and sex-matched controls with NFPAs. RESULTS: The dimensions of the SO were significantly larger in the GH-adenoma group (P < 0.05). The SO was further from the midline (P = 0.04) and closer to the sphenopalatine foramen (SPF) (P = 0.02) in the GH-adenoma group, and this finding correlated with increasing preoperative GH levels. Acromegalics demonstrated larger intracavernous carotid diameters (P = 0.05) and smaller intercarotid distances than the patients with NFPAs (P = 0.02). CONCLUSION: The SO is larger and located higher up in the sphenoid face and closer to the SPF in patients with GH adenomas. Increasing GH levels in these patients correlate with the upward and lateral displacement of the SO. These patients demonstrate larger intracavernous carotid diameters and smaller intercarotid distances than patients with NFPAs. These morphological alterations are of particular relevance to the pituitary surgeon.

Sellar-Suprasellar Paraganglioma: Report of 2 Cases and Review of Literature.

BACKGROUND: Intracranial paragangliomas are infrequent and those occurring in the sellar-suprasellar region are rare, with only 31 cases described in literature. CASE DESCRIPTION: We describe 2 cases of sellar-suprasellar paragangliomas in the light of a literature review. The first patient was a 13-year-old boy who presented with an intensely enhancing lesion in the sellar-suprasellar region with multiple flow voids within. Resection of the lesion was limited to a biopsy in view of its hypervascular nature. A second attempt at resection following partial embolization of the lesion was also unsuccessful. The tumor showed progressive reduction in size following radiotherapy. The second case was a 20-year-old man who presented with a similar tumor in the same location. He also had a probable metastatic deposit in the foramen of Magendie. An attempted surgical resection of the suprasellar lesion was abandoned after a biopsy. The patient improved symptomatically after radiotherapy. CONCLUSIONS: We report 2 cases of paraganglioma occurring in a rare location. Presence of flow voids within tumors in the sellar-suprasellar location should alert the surgeon to this entity. The hypervascular nature of these tumors may limit the extent of resection. In cases of inadequate tumor decompression, or if there is evidence of growth of residual tumor, radiotherapy can help to stabilize the disease.