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.

Primary intracranial pediatric ganglioneuroblastoma-report of two cases and review of an unusual masquerader.

Ganglioneuroblastoma (GNB) is a rare neurogenic tumor with a predilection for extracranial sites. Its primary intracranial occurrence is anecdotal, with less than ten cases reported in pediatric literature. We report two cases of this unusual entity. The first was a 1-year-old boy who presented with a progressive scalp swelling that radiologically appeared to be a mesenchymal tumor. The tumor was resected completely, and its histopathological examination was suggestive of a pigmented GNB. The clinico-radiological presentation and melanin pigmentation of the tumor were unique features of the case. The second case was a 7-year-old girl who presented with left hemiparesis and raised intracranial pressure due to a large right parietal intraparenchymal tumor that histologically proved to be a GNB. The child succumbed to disease progression a month after undergoing near-total resection of the tumor. Analysis of our cases in the light of a literature review reveals that pediatric intracranial GNBs have diverse clinico-radiological features and can easily be confused for commoner pathologies in both intra- and extra-axial locations.

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.

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.

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.

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.