Validation of the Penn Acoustic Neuroma Quality of Life (PANQOL) Scale for Hindi-Speaking Patients Recently Diagnosed with Vestibular Schwannoma.

Background: The Penn Acoustic Neuroma Quality-of-Life (PANQOL) scale is a disease specific quality-of-life instrument for vestibular schwannoma, developed in English and subsequently validated in four major languages of the world. No such validated version of PANQOL exists for Hindi-speaking population. Objective: To translate and examine the validity of the PANQOL scale in a sample of Hindi-speaking patients recently diagnosed with vestibular schwannoma. Materials and Methods: A cross-sectional study was done at a corporate tertiary care hospital. The PANQOL questionnaire was translated into Hindi by a language expert according to the accepted rules of forward-backward translation. In total, 30 consecutive patients (n = 30) diagnosed with vestibular schwannoma between September 2017 and March 2018 were included in the study. Quality-of-life at the time of diagnosis was measured with the generic Short Form -36 (SF-36) and the disease-specific PANQOL questionnaires. The internal consistency and reliability of the PANQOL domains, as well as the relationship with SF-36 dimensions, were statistically analyzed.The PANQOL scores of the patients in the current study were compared with those of similar such published studies worldwide. Results: Mean age of the patients was 46.3 ΁ 13.25 years. The sex ratio of male to female patients was 19:11. The mean PANQOL total score was 71.6. All its domains had good internal consistency except pain. Most PANQOL domains were correlated with those of the SF-36 dimensions, with the exception of facial dysfunction domain. Conclusions: Good internal consistencies and strong correlations between PANQOL domains and SF-36 dimensions in the current study support the validity of the PANQOL Hindi version.

Quality of life in patients of unilateral vestibular schwannoma treated with microsurgery: A South-Asian tertiary care Hospital experience.

Majority of the published literature on the impact of microsurgery over quality-of-life (QOL) in vestibular schwannoma (VS) patients is retrospective in nature, with variable results, and there has been very little contribution from South-Asia. The authors designed this study to investigate the role of microsurgery in QOL alterations in VS patients, and to compare obtained QOL scores with that of control population. Between February 2017 and March 2018, a prospective/retrospective study was done at our institute. Prospective and retrospective cohorts included, 30 and 34 consecutive patients, respectively, of unilateral VS undergoing microsurgery. QOL assessment was done using Short Form-36 (SF-36), and the Penn Acoustic Neuroma Quality-Of-Life (PANQOL) questionnaires. Prospective cohort underwent QOL assessment at 3 junctures - pre-surgery, 3-months & 1-year post-surgery; while retrospective cohort was assessed only once, at 1-year post-surgery. Age- & sex- matched normal population and diabetic patients were used as controls. In the prospective cohort, patients showed drop in all PANQOL domain/ SF-36 dimension scores at 3-month post-surgery, exception being vitality and general health in SF-36; showed improvement at 1-year follow-up, with majority either levelling or improving their respective pre-surgery scores. Gross total resection, tumors ≤ 3 cm, and age ≤ 45 years had better PANQOL scores at 1-year post-surgery across all the domains. Patients in both the cohorts showed worse QOL compared to normal population, but the gap got narrower at 1-year post-surgery. Our study adds a South-Asian perspective to the pool of prospective knowledge available on QOL following microsurgery in VS patients.

Meningioma in a patient with Werner syndrome.

Werner syndrome (WS), also known as adult progeria, is extremely rare, with about 1300 known cases in the world, with over 1000 of these in Japan. It occurs due to loss of function mutations in the WRN gene located on chromosome 8p12. WS is characterized by premature aging and increased risk of neoplasms, with meningiomas being the commonest intracranial tumor. We report the case of a 39-year-old male patient, who presented with occasional numbness in right arm for three weeks. The patient had developed signs and symptoms of premature aging which started in his adolescence. MRI brain done was suggestive of left frontal convexity extra-axial lesion, suggestive of meningioma. Genetic analysis performed has identified an autosomal recessive, apparently homozygous c.3383+3A>G mutation, a mutation not previously reported. As per the existing literature, this is the index case of meningioma in Werner syndrome from India. A new mutation has been identified.

Microsurgery of Vestibular Schwannoma Post-radiosurgery.

OBJECTIVES: Microsurgery of vestibular schwannoma (VS) after radiosurgery is not common. In this study, we analyze nerve functional outcomes and technical challenges associated with the microsurgery following radiosurgery of VS. MATERIALS AND METHODS: Sixteen patients underwent microsurgery at an average of 37 months (range 3-96 months) after radiosurgery. Indications of surgery included tumour progression or clinical worsening. Twelve of them had never undergone microsurgery before radiosurgery, and three had partial excision before radiosurgery. Maximum tumour diameter of all patients ranged from 23 to 51 mm, of which four were giant tumours. Operative findings and post-microsurgery clinical course, imaging and outcome were analysed. RESULTS: The tumours found at surgery were firmer, with an avascular core but increased vascularity near the surface. There were thickened arachnoid and often adhesions with surrounding structures making total excision difficult. Near total excision was employed in such cases. Eleven patients underwent gross total/near total resection. No major morbidity or death was reported. Post-surgery, eight patients had grade I/II HB facial function, four had grades III and three had grades IV/V. Of the last three, two had the same deficit preoperatively. At the last follow-up (average 50 months), all patients had a stable facial function. None of the patients have undergone any further radiosurgery/microsurgery since the last intervention. CONCLUSION: For the few patients who require microsurgery after radiosurgery, excellent tumour resection with an acceptable outcome can be achieved with proper techniques and neuromonitoring. Leaving tiny residue attached to critical structures is the key to an optimal outcome.

Correlation of Preoperative Cranial Nerve Diffusion Tensor Tractography with Intraoperative Findings in Surgery of Cerebellopontine Angle Tumors.

OBJECTIVE: Imaging to visualize nerves around tumors preoperatively could help the surgeon to plan the surgery and excise the tumor with preservation or improvement in patients' quality of life. In the present prospective study, we attempted to correlate the preoperative cranial nerves (i.e., trigeminal, abducens, facial, and vestibulocochlear diffusion tensor tractography and anatomic magnetic resonance imaging [MRI] findings) with intraoperative findings during cerebellopontine angle (CPA) tumor surgery. METHODS: The 40 enrolled patients had CPA tumors (31 vestibular schwannomas, 5 epidermoid tumors, 2 meningiomas, 2 trigeminal schwannomas). All the patients were undergoing microsurgery for the first time. They underwent preoperative MRI for anatomic and diffusion tensor imaging (DTI) data. The imaging series were postprocessed, and 3-dimensional images were obtained. The location of the involved nerves around the tumors was recorded during surgery by the senior surgeon, who was kept unaware of the results of the fiber tracking, and a correlation was performed. RESULTS: We evaluated the correlation between the DTI and intraoperative findings for the nerve location in relation to the tumor in the CPA. The correlation between the DTI and intraoperative findings for the facial, trigeminal, and vestibulocochlear nerves was 85% (34 of 40), 85% (34 of 40), and 75% (12 of 16), respectively. CONCLUSIONS: The modified technical parameters proposed could lead to better preservation of cranial nerve function, especially for patients with large tumors. To the best of our knowledge, the present study is the first systematic study of relevant cranial nerves in 40 patients with demonstration of the vestibulocochlear bundle by preoperative MRI in the most common CPA tumors using advanced MRI sequences such as diffusion tensor tractography and capturing the data in a multiplanar format.

Newer advances in lesional surgery for movement disorders.

An analysis of the existing literature on lesioning for movement disorders was undertaken to review lesion therapy and its advances. Advances in imaging technology and electrophysiological techniques used for localization of brain structures and its functions, such as microelectrode recordings and macrostimulation, have greatly improved the ability to accurately identify the target nuclei such as the ventrointermediate nucleus (Vim), globus pallidus interna (GPi) and subthalamic nucleus (STN). Many important changes are happening in the understanding of lesion making. Its application as a cheaper modality of treatment; being less cumbersome; having a wider geographical appeal; and more options to create a lesion, appeals to the clinician. The procedure is undergoing a revival.

Application of fluorescein sodium videoangiography in surgery for spinal arteriovenous malformation.

We present our recent experience with fluorescein sodium videoangiography (FLVA) in the intra-operative evaluation of a patient with conus medullaris arteriovenous malformation (AVM). To our knowledge this is the first report in the literature of use of FLVA in the surgery of spinal AVM. Intra-operative FLVA was done to identify an early filling vessel and to obliterate the site of fistulous connection. This was correlated and confirmed with simultaneous indocyanine green videoangiography (ICGVA). The conus and cauda equina roots could be appreciated and manipulated in relation to this fluorescence. Obliteration was confirmed with FLVA and correlated with ICGVA. There was no untoward reaction to the dye injection. We conclude that FLVA is a useful adjunct in the surgical treatment of conus medullaris AVMs since it is a real time, noninvasive, radiation-free, easily reproducible technique allowing surgical manipulation through the operating oculars with simultaneous visualization of surrounding critical structures.

Anterior petrosal approach for brainstem cavernoma.

Brainstem cavernomas (BC) comprise about 5-18% of intracranial vascular malformations. The annual hemorrhage rate varies depending on the study design ranging from as low as 0.25% per patient-year in a retrospective study[2] to 1.6-3.1% per patient-year in prospective studies.[45] The annual event rate is significantly higher in deep (brainstem, diencephalon) and infratentorial cavernomas when compared to their counterparts in other locations.[5] The management of BC can be conservative or surgical depending upon the mode of clinical presentation. Surgical excision of a BC is a challenge because of critical anatomy. We present a case of BC, which was totally excised with anterior petrosal approach. Anterior petrosal approach has been used for excision of BC in only 17 cases until now.[6] The use of preoperative diffusion tensor imaging, tractography, intra-operative navigation, and cranial nerve monitoring will help in reducing the morbidity.