Total external ophthalmoplegia and orbital apex syndrome as first presenting feature of Rhabdomyosarcoma involving petrous part of the temporal bone: A case report.

INTRODUCTION AND IMPORTANCE: Rhabdomyosarcoma (RMS) can have various unusual presentations. We report an unusual presentation of RMS as orbital apex syndrome in a six-year-old boy involving the petrous part of the temporal bone. CASE PRESENTATION: A six-year-old boy presented with drooping of the left eyelid for seven days associated with headache, left-sided hearing loss, and nasal blockage. Contrast-enhanced magnetic resonance imaging (CE-MRI) brain and orbit revealed an ill-defined expansile vascular lesion centered at the petrous part of the left temporal bone with extension to the cavernous sinus, which was confirmed as RMS on histopathology and immunohistochemistry. The patient was managed by chemotherapy and radiotherapy. CLINICAL DISCUSSION: RMS is the most common aggressive malignant soft tissue tumor in the pediatric population. It accounts for 4-8 % of all malignancies in children below 15 years of age, with strong male preponderance. The most common site for RMS is head and neck (45 %), having maximum incidence during the first decade of life. CONCLUSION: Total external ophthalmoplegia in a child is an acute emergency; it should be properly worked up, and neuroimaging should always be advised. Prompt diagnosis and management by a multidisciplinary team can be both life and sight-saving.

The influence of tumor topography on the surgical outcome of craniopharyngiomas.

OBJECTIVE: Various topographical classifications for craniopharyngioma have been proposed based on their relationship with optic chiasm and the third ventricular floor. There is a paucity of literature evaluating the surgical outcome based on tumor topography. This study aims to compare the surgical outcomes of retrochiasmatic craniopharyngiomas (RCPs) and nonretrochiasmatic craniopharyngiomas (non-RCPs). METHODS: This retrospective study includes newly diagnosed patients with craniopharyngioma who underwent surgery between January 2000 and December 2015. Clinical features, the extent of resection (EOR), surgical outcomes, tumor recurrence, and progression-free survival (PFS) of craniopharyngiomas were compared with respect to their relationship to the optic chiasm and third ventricular floor. RESULTS: The authors identified RCPs in 104 and non-RCPs in 33 patients. RCPs were significantly larger and more associated with hydrocephalus than were non-RCPs (p < 0.001) at the time of diagnosis. Puget grade 2 hypothalamic involvement was more frequent with RCPs. EOR and PFS following either subtotal resection (p = 0.07) or gross-total resection (p = 0.7) were comparable between RCPs and non-RCPs. There was no significant difference in the postoperative visual outcome. Resection of RCPs resulted in higher postoperative hypopituitarism (64% vs 42%, p = 0.01) and hypothalamic dysfunction (18% vs 3%, p = 0.02). Location of the tumor, either retrochiasmatic (HR 0.5; 95% CI 0.14-2.2; p = 0.4) or nonretrochiasmatic (HR 1.3; 95% CI 0.3-5.5; p = 0.6), did not show association with recurrence. RCPs with extra- and intraventricular components (type 3b) had a higher incidence of postoperative hypothalamic morbidities (p = 0.01) and tumor recurrence (36% vs 19%; p = 0.05) during follow-up than the extraventricular (type 3a) RCP. Between prechiasmatic and infrachiasmatic/intrasellar craniopharyngiomas, EOR (p = 0.7), postoperative diabetes insipidus (p = 0.4), endocrinological outcome (p = 0.7), and recurrence (p = 0.1) were comparable. The patients with complex multicompartmental tumors had a lower rate of gross-total resection (25%, p = 0.02) and a higher incidence of tumor recurrence (75%, p = 0.004) than the rest. CONCLUSIONS: The tumor topography can influence the postoperative outcome. RCPs can be associated with a higher incidence of hypopituitarism and hypothalamic morbidities postoperatively. The influence of topography on EOR and tumor recurrence is controversial. However, this study did not find a significant difference in EOR and tumor recurrence between RCPs and non-RCPs. PFS and overall mortality are also comparable.

Partial occlusion of left axillary artery in a patient undergoing robot-assisted radical cystectomy.

Robot-assisted surgeries are associated with steep positions which provide free operative field to surgeons; however, it becomes more challenging to the anaesthesiologists. In robot-assisted surgery, the patient is not usually accessible after docking in of robot, so monitors, circuits and tubes should be tightly secured and confirmed before handing over the patient to the surgeons. We report a patient with partial left axillary artery occlusion in a patient posted for robot-assisted radical cystectomy.

Migrating Souvenir Bullet: The Management Dilemma.

The cranio-cerebral trauma following gunshot injuries has high mortality and morbidity, with 66% to 90% victims dying before reaching hospital and only half of those treated in hospital surviving. However, in case of most salvageable patients, the question which poses dilemma to treating physicians is the decision as to when and why remove the retained missile. A 21-year-old man was observing a gunfight in the street from his balcony. Suddenly something struck his forehead and there was a small amount of bleeding toward the medial end of his left eyebrow. He had moderate headache and dizziness. Because of nonresolution of headache over seven days he was hospitalized and underwent X ray of the skull and CT of the head, which showed a retained metallic bullet in left inferior parieto-occipital region without any significant hemorrhage. As there was no neurological deficit or meningeal signs, he was managed conservatively. His symptoms improved gradually within next week and he was discharged home. His most recent follow-up was 28 months since injury and imaging showed migration of the bullet to the right inferior temporal region. As he was completely asymptomatic throughout, no intervention was offered. However, long-term follow-up for potential complications of migration, hydrocephalus, and abscess formation is advisable.

A Systematic Review on the Impact of the COVID-19 Pandemic on Neurosurgical Practice and Indian Perspective.

OBJECTIVE: The study objective was to systematically review the impact of the current pandemic on neurosurgical practice and to find out a safe way of practicing neurosurgery amid the highly infectious patients with COVID-19. MATERIALS AND METHODS: A review of the PubMed and EMBASE databases was performed. The literature was systematically searched using keywords such as "COVID-19" and "Neurosurgery." RESULTS: Among the 425 records, 128 articles were found to be eligible for analysis. These articles described the perspectives of the neurosurgical departments during the pandemic, departmental models, and organizational schemes for triaging emergent and nonemergent neurosurgical cases for the optimal utilization of limited resources, and solutions to continue academic and research activities. Triaging systems help us to optimally utilize the limited resources available. Guidelines have been developed for safe neurosurgical practice and for the continuation of clinical and academic activities during this pandemic by various national and international neurosurgical societies. Key changes in the telemedicine regulatory guidelines would help us to continue to provide neurosurgical care. Videoconferences, online education programs, and webinars could help us to overcome the disadvantages brought upon the neurosurgical education by the social-distancing norms. CONCLUSION: In an unprecedented time like this, no single algorithm is going to clear the ethical dilemma faced by us. Individual patient triage is a way for maintaining our ethical practice and at the same time, for efficiently utilizing the limited resources. As the pandemic progresses, new guidelines and protocols will continue to evolve for better neurosurgical practice.

Anesthetic Management in a Case of MURCS Syndrome.

MURCS syndrome is a more severe form of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, an acronym meaning aplasia/hypoplasia of Müllerian ducts (MU), congenital renal agenesis/ectopia (R), and cervical somite dysplasia (CS). A common presentation is primary amenorrhea in adolescent females. An anesthetist must consider the benefits and limitations of both regional and general anesthesia for these patients based on site of surgery and severity of malformations. We report successful anesthetic management of a 21-year-old female with MURCS syndrome scheduled for a creation of neovagina under spinal anesthesia using ultrasound guidance.

"Locked up inside home" - Head injury patterns during coronavirus disease of 2019 pandemic.

BACKGROUND: As citizens have been forced to stay home during coronavirus disease of 2019 (COVID-19) pandemic, the crisis created unique trends in the neurotrauma patterns with changes in mode, severity, and outcome of head injured patients. METHODS: Details of neurotrauma admissions under the neurosurgery department at our institute since the onset of COVID-19 pandemic in the country were collected retrospectively and compared to the same period last year in terms of demographic profile, mode of injury, GCS at admission, severity of head injury, radiological diagnosis, management (surgical/conservative), and outcome. The patients were studied according to which phase of pandemic they were admitted in - "lockdown" period (March 25 to May 31, 2020) or "unlock" period (June 1 to September 15, 2020). RESULTS: The number of head injuries decreased by 16.8% during the COVID-19 pandemic. Furthermore, during the lockdown period, the number of admissions was 2.7/week while it was 6.8/week during the "unlock" period. RTA was the mode of injury in 29.6% patients during the lockdown, while during the unlock period, it was 56.9% (P = 0.000). Mild and moderate head injuries decreased by 41% and severe head injuries increased by 156.25% during the COVID-19 pandemic (P = 0.000). The mortality among neurotrauma patients increased from 12.4% to 22.5% during the COVID-19 era (P = 0.009). CONCLUSION: We observed a decline in the number of head injury admissions during the pandemic, especially during the lockdown. At the same time, there was increase in the severity of head injuries and associated injuries, resulting in significantly higher mortality in our patients during the ongoing COVID-19 pandemic.

Primary anaplastic lymphoma kinase-negative anaplastic large cell lymphoma of cervical spine presenting with quadriplegia: A case report and literature review.

BACKGROUND: An anaplastic large cell lymphoma (ALCL) involving the cervical spine and leading to quadriplegia is very rare. CASE DESCRIPTION: A 48-year-old immunocompetent male presented with quadriplegia that warranted an anterior cervical corpectomy/fusion. He was previously being presumptively treated for cervical disease attributed to tuberculosis. The histopathology and immunohistochemistry revealed an ALCL that was anaplastic lymphoma kinase (ALK) negative. The patient had a favorable response to surgery followed by CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisolone) chemotherapy. CONCLUSION: ALK-negative ALCL presenting with quadriplegia due to primary involvement of cervical spine is extremely rare, but must be diagnosed and appropriately managed.

Rare Case of Sellar and Suprasellar Metastasis from Ewing's Sarcoma of Tibia.

We hereby report a case of metastatic Ewing's sarcoma presenting with rapid-onset total ophthalmoplegia, optic atrophy, and right temporal hemianopia. Comprehensive ophthalmic and neurological evaluation with targeted radioimaging revealed a tumor mass centered over the sella, compressing optic chiasma, extending to involve the left cavernous sinus and the left orbital apex. Whole-body imaging revealed the evidence of multifocal lung and mediastinal metastasis with focal lytic defect in the left femoral head. Histopathological evaluation of transnasal punch biopsy from the nasopharyngeal extension of the tumor revealed small round-cell tumor with strong CD99 positivity, supporting the diagnosis of Ewing's sarcoma. Rapid, aggressive extensions of the metastatic tumor into vital structures despite the initiation of chemoradiation of the extensive intracranial tumor led to unexpected demise of the patient. Our case is an unusual case of Ewing's sarcoma metastasis manifesting as a sellar mass and mimicking a pituitary adenoma radiologically, with a rapid progression within 2 weeks to cause massive extension of tumor into suprasellar, infrasellar, and left parasellar area, indicative of highly malignant nature of the tumor.

Giant Occipitocervical Encephalocele.

Encephaloceles are cranial defects in which sac contains herniating brain, which is often gliotic. Congenitally, this defect may extend into posterior elements of cervical vertebrae and leads to occipitocervical encephalocele. When the size of this sac is larger than head size, they are termed as giant. Very young age and associated congenital anomalies in these patients pose significant challenges in diagnostic, anesthetic, and surgical techniques. We share a case of giant occipitocervical encephalocele managed at our institute and discuss about its management issues with review of literature.

Spinal tumors: Trends from Northern India.

INTRODUCTION AND METHODS: We retrospectively analyzed 111 patients with spinal tumors operated over a period of 9 years to observe the relative frequency of different lesions, their clinical profile, functional outcome and prognostic factors. 30/111 (27%) were extradural, 40/111 (36.1%) were intradural extramedullary (IDEM) and 41/111 (36.9%) were intramedullary spinal cord tumors (IMSCTs). Mean age at surgery was 30.81 years (range 1-73 years). The average preoperative duration of symptoms was 16.17 months (15 days to 15 years). Major diagnoses were ependymomas and astrocytomas in IMSCT group, schwanommas and neurofibromas in IDEM group, and metastasis, lymphoma in extradural group. The common clinical features were motor weakness in 78/111 (70.27%), sensory loss in 55/111 (49.54%), pain 46/111 (41.44%), and sphincter involvement in 47/111 (42.43%) cases. RESULTS: Totally, 88/111 (79.27%) patients had improvement in their functional status, 17/111 (15.31%) remained same, and 6/111 (5.4%) were worse at time of their last follow-up. The mean follow-up was 15.64 months (1.5 m(-10) years). Totally, 59 out of 79 patients, who were dependent initially, were ambulatory with or without the aid. Most common complication was persistent pain in 10/111 (9%) patients and nonimprovement of bladder/bowel symptoms in 7/111 (6.3%). One patient died 3 months after surgery. CONCLUSIONS: (1) Congenital malformative tumors like epidermoids/dermoids (unrelated to spina bifida) occur more frequently, whereas the incidence of spinal meningioma is less in developing countries than western populations. (2) The incidence of intramedullary tumors approaches to that of IDEM tumors. Intramedullary tumors present at a younger age in developing countries. (3) Rare histological variants like primitive neuroectodermal tumors should also be considered for histological differential diagnosis of spinal tumors. (4) Preoperative neurologic status is the most important factor related to outcome in spinal tumors.