Optico-chiasmatic distortions in pituitary adenomas: correlation between postoperative morphological reversal and follow-up functional visual recovery.

PURPOSE: To objectively correlate distortions of optic apparatus morphology in patients with visual outcomes in patients with pituitary adenomas (PAs) undergoing trans-sphenoidal surgery. METHODS: In this retrospective analysis, visual acuity (VA), visual field (VF) and total visual (TV) function was objectively scored in patients selected from an institutional cohort of PAs The following imaging measures of optico-chiasmatic morphology were recorded preoperatively, and at 3 months after surgery: chiasm area (CA), mid-chiasm height (CH), optic nerve-canal bending angle (BA), and optic nerve kinking angle (ONKA). Receiver operator characteristic (ROC) analysis was performed to establish optimal thresholds for achieving a 'normal' TV score of 200 at 1-year follow-up. RESULTS: Seventy-one eyes were individually studied. VA, VF and TV scores significantly improved after surgery, both at the 3-month and at the 1-year follow-up visits (p<0.001). Cranio-caudal tumor dimension was significantly associated with VA, VF and TV scores, both pre- and post-operatively. There were significant changes in CH (p<0.001), BA (p<0.001) and ONKA (p<0.001) at 3 months after surgery, but not in CA (p=0.061). Baseline VA, VF and TVS scores were positively correlated with preoperative CH and ONKA, while VF and TV scores also demonstrated significant negative correlations with preoperative BA. VF scores at 1-year follow-up were significantly correlated with preoperative and change in BA values, as well as with preoperative and change in ONKA values. ROC analysis revealed that only the preoperative ONKA was found to have acceptable discrimination (AUC>0.7) for predicting 'normal' TV score. Chiasm sag was noted in 45.8% of patients at one year follow-up, but was not associated with delayed visual deterioration in any case. CONCLUSIONS: Anatomic realignment of the optic apparatus 3 months following trans-sphenoidal surgery predicts VF scores, but not VA or TV scores at 1 year follow-up. Patients with preoperative ONKA values of more than 139.3° have a 76% chance of achieving normal TV scores one year after surgery. Postoperative chiasm sag appears to be clinically irrelevant at short term follow-up.

Radiological evolution of pituitary hyperplasia in primary hypothyroidism and its differentiation from nonfunctioning pituitary adenoma coexisting with primary hypothyroidism.

PURPOSE: In a patient with elevated thyroid stimulating hormone (TSH, >50 µIU/ml) with sellar mass, it is crucial to differentiate isolated pituitary hyperplasia (IPH) from primary hypothyroidism coexisting with nonfunctioning pituitary adenoma (PHCNFPA) pre-operatively to avoid unwarranted surgery in the former condition. Here, we describe patients having pituitary mass/enlargement with markedly elevated TSH (>50 µIU/ml) and attempt to find the differentiating features between IPH and PHCNFPA. METHODS: This is a retrospective study conducted at a tertiary care center. Case records of patients presenting between January 2020 and December 2022 with elevated TSH (>50 µIU/ml) for whom magnetic resonance imaging (MRI) of the sella was available were reviewed. Demographic details, symptomatology, clinical examination findings, thyroid function tests, data on pituitary hormonal excess and deficiencies, MRI findings, and details regarding levothyroxine supplementation were noted. Based on the final diagnosis, the patients were categorized into two groups: PHCNFPA and IPH. RESULTS: Five and 11 patients were diagnosed with PHCNFPA and IPH, respectively. The median (IQR) age at presentation of patients with PHCNFPA was significantly higher than that of IPH patients [37 (28-60.5) vs. 21 (10-21.5) years, p: 0.002]. A longer duration of hypothyroid symptoms was noted in the IPH group whereas visual field defects and corticotropin deficiency were more frequent and the pituitary lesion size was greater in PHCNFPA. Thyroid function tests were not different between the two groups. The pituitary enlargement in IPH was initially an increase in pituitary height that progressed to symmetrical nipple-, dome- or tent-shaped enlargement. Besides this characteristic enlargement pattern, isointense appearance on T1-weighted and T2-weighted images, homogeneous contrast enhancement, and prompt regression of pituitary lesion with levothyroxine replacement were characteristic of IPH whereas heterogeneous enhancement, cystic/hemorrhagic change, and ≥Knosp III invasion were characteristic of PHCNFPA. Peripheral rim enhancement and Knosp I-II parasellar extension were not uncommon in patients with IPH and did not distinguish it from PHCNFPA. CONCLUSIONS: The present study reports the radiological evolution of IPH and a unique series of PHCNFPA along with the distinguishing characteristics between them.

Cervical intraosseous arteriovenous malformation: report of a rare entity and its management dilemmas.

Intraosseous occurrence of a spinal AVM is anecdotal, with only four such cases reported previously. This is the first report of a spinal intraosseous AVM in the cervical vertebrae. A 44-year-old male patient presented with a 2-month history of progressive quadriparesis and bladder dysfunction. Magnetic resonance imaging showed multiple flow voids within the C4 and C5 vertebral bodies, and an extradural component causing cord compression. CT showed extensive bony destruction at both levels. The diagnosis of an intraosseous AVM was confirmed with spinal angiography. The AVM was noted to be fed by branches from the ascending cervical arteries and the vertebral artery. The nidus was draining into the vertebral venous plexus and thence into the jugular vein through the marginal sinus. The patient underwent partial embolization of the AVM. Surgical resection was attempted but found to be unfeasible due to torrential bleeding. A 360-degree stabilization along with decompressive laminectomies was performed, resulting in clinical improvement and disease stabilization at one year follow-up. The case and its management dilemmas are discussed in light of a brief literature review.

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.

Dural sinus malformation presenting with proptosis: report and review of a rare entity.

Dural sinus malformation (DSM) is a rare vascular malformation characterized by the dilatation of a dural venous sinus with or without an anomalous jugular bulb. Its presentation with venous-reflux-related secondary proptosis is anecdotal, with only six such cases reported so far. We report a 17-month-old boy who presented with a progressive proptosis of the right eye secondary to a DSM of the transverse sinus and torcula. Following endovascular embolization of the arterio-venous fistula, complete thrombosis of the venous lake and improvement in proptosis was noted at 6-month follow-up. Prognosis of this rare malformation is variable and dependent on specific angio-architectural features.

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.

Cervical Flexor-Extensor Muscle Disparity in Monomelic Amyotrophy (Hirayama Disease): Evidence from a Comprehensive Morphometric Evaluation of Subaxial Paraspinal Musculature.

Background Monomelic amyotrophy (Hirayama disease) has been established to have accompanied biomechanical abnormalities such as flexion hypermobility and sagittal imbalance. Paraspinal muscles, the major contributor to cervical biomechanics, have, however, not been comprehensively evaluated in the disease. The objective of this study was to compare the morphology of the subaxial cervical paraspinal musculature in patients with and without Hirayama disease. Materials and Methods A retrospective case-control study of 64 patients with Hirayama disease and 64 age- and sex-matched controls was performed . Cross-sectional areas (CSAs) of the superficial and deep flexors and extensors from C3 to C7 were measured on T2-weighted magnetic resonance imaging sequences. Student's t -test was used to compare differences between the paraspinal muscle CSAs in the study and control groups. Results Compared with controls, patients with Hirayama disease were found to have larger flexors and smaller extensors at all levels. The overall subaxial muscle area values for superficial flexors and deep flexors were significantly larger ( p < 0.0001) in patients, while the corresponding superficial extensor and deep extensor area values were significantly smaller than in controls ( p = 0.01 and < 0.0001, respectively). The patient group demonstrated stronger subaxial deep flexor-deep extensor, superficial flexor-superficial extensor, and total flexor-total extensor ratios ( p < 0.0001). Conclusion Patients with Hirayama disease have morphometric alterations at all levels of their subaxial cervical paraspinal musculature. These patients have abnormally large flexors and small extensors compared with controls. This flexor-extensor muscle disparity could be utilized as a potentially modifiable factor in the management of the disease.

Are Two-Dimensional Morphometric Measures Reflective of Disease Severity in Adult Chiari I Malformation?

BACKGROUND: Two-dimensional (2D) morphometric measures have been previously used to diagnose Chiari I malformation (CMI) and distinguish patients from healthy subjects. There is, however, a paucity of literature regarding whether morphometric differences exist across the range of CMI disease severity. We evaluated whether 2D morphometrics demonstrate significant changes across standardized grades of CMI severity in adults. METHODS: This retrospective observational study comprised 76 patients with symptomatic CMI with or without syringomyelia. Patients matched for age, sex, and body mass index were selected from each of the 3 severity grades of the Chiari Severity Index (CSI). The study used 17 2D craniocervical and posterior fossa morphometric variables that were previously found to differentiate patients with CMI from healthy subjects. The measurements were performed on midsagittal T1-weighted magnetic resonance imaging sequences using 2 custom in-house software tools, MorphPro and CerePro, and compared across CSI grades. RESULTS: Analysis of variance showed that none of the 17 morphometric variables were significantly different across the 3 CSI grades (P > 0.003). Bayes factor 10 scores ranged from 0.11 to 0.82. Of variables, 9 had Bayes factor 10 scores between 0.10 and 0.30, while 8 had scores between 0.30 and 1. None of the Bayes factor 10 scores provided support for the alternative hypothesis that the morphometric measures differed across the CSI grades. CONCLUSIONS: Our study indicated that 2D morphometrics are not reflective of CMI disease severity as indicated by the CSI grading system. The findings of our study support the need for further investigation into whether non-2D morphometric variables can be used as markers of disease severity.

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.

Clinical and Laboratory Markers of Brain Abscess in Tetralogy of Fallot ('BA-TOF' Score): Results of a Case-Control Study and Implications for Community Surveillance.

Background Cardiogenic brain abscess (CBA) is the commonest noncardiac cause of morbidity and mortality in cyanotic heart disease (CHD). The clinical diagnosis of a CBA is often delayed due to its nonspecific presentations and the scarce availability of computed tomography (CT) imaging in resource-restricted settings. We attempted to identify parameters that reliably point to the diagnosis of a CBA in patients with Tetralogy of Fallot (TOF). Methods From among 150 children with TOF treated at a tertiary care institute over a 15-year period from 2001 to 2016, 30 consecutive patients with CBAs and 85 age- and sex-matched controls without CBAs were included in this retrospective case-control study. Demographic and clinical features, laboratory investigations, and baseline echocardiographic findings were analyzed for possible correlations with the presence of a CBA. Statistical Analysis Variables demonstrating significant bivariate correlations with the presence of a CBA were further analyzed using multivariate logistic regression (LR) analysis. Various LR models were tested for their predictive value, and the best model was then validated on a hold-out dataset of 25 patients. Results Among the 26 variables tested for bivariate associations with the presence of a CBA, some of the clinical, echocardiographic, and laboratory variables demonstrated significant correlations ( p < 0.05). LR analysis revealed elevated neutrophil-lymphocyte ratio and erythrocyte sedimentation rate values and a lower age-adjusted resting heart rate percentile to be the strongest independent biomarkers of a CBA. The LR model was statistically significant, (χ 2 = 23.72, p = <0.001), and it fitted the data well. It explained 53% (Nagelkerke R 2 ) of the variance in occurrence of a CBA, and correctly classified 83.93% of cases. The model demonstrated a good predictive value (area under the curve: 0.80) on validation analysis. Conclusions This study has identified simple clinical and laboratory parameters that can serve as reliable pointers of a CBA in patients with TOF. A scoring model-the 'BA-TOF' score-that predicts the occurrence of a CBA has been proposed. Patients with higher scores on the proposed model should be referred urgently for a CT confirmation of the diagnosis. Usage of such a diagnostic aid in resource-limited settings can optimize the pickup rates of a CBA and potentially improve outcomes.

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.