Endovascular Management of Type III Perimedullary Spinal Arteriovenous Malformations in Pediatric Population.

BACKGROUND: Spinal arteriovenous malformations (AVMs) are a cause of 20-30 of all spinal vascular malformation. The treatment option for the AVM depends upon the type of AVM. Here, we present a case series to discuss the type, management, and post-operative conclusion of the spinal AVMs. METHOD: Four patients with spinal AVMs were retrospectively reviewed. All 4 patients were with a nidus-type AVM. Treatment for all patients required embolization. Clinical features, imaging, treatment, and clinical results were observed. All 4 patient's clinical outcome was assessed using the Modified Ranked Scale. RESULT: The follow-up after management showed that all four-patient recovered without any residual deficit. All four-patient scored zero (0) on the Modified Ranked Scale. CONCLUSION: Pediatric spinal AVMs are rare and require complex multimodal approach to achieve favorable outcomes.

Work-life balance amongst residents in surgical and non-surgical specialties in a tertiary care hospital in Karachi.

OBJECTIVE: To assess work-life balance among medical residents at a tertiary hospital. METHODS: The cross-sectional study was conducted from September to December 2016 at a private-sector tertiary care hospital in Karachi, and comprised medical residents working at the facility. A standardised, self-administered questionnaire was developed on the basis of Canadian Mental Health quiz and a study in literature. The questions aimed at assessing satisfaction with work as well as emotional and personal life of residents in various medical and surgical specialties. SPSS 20 was used for data analysis. RESULTS: Of the 275 residents, 129(46.9%) were males and 146(53.1%) were females. The overall mean age was 28.19±2.194 years. Of the total, 13(4.7%) participants thought they had work-life balance; 165(60%) felt their job had negatively affected their private lives; 118(42.9%) felt worn out; 109(39.6%) expressed moderate dissatisfaction with work-related factors; 119(43.3%) were dissatisfied with life outside work; and 93(33.8%) were dissatisfied their health. CONCLUSIONS: There was minimal work-life balance among the residents.

Primary intracranial extraosseous Ewing's sarcoma.

INTRODUCTION: Common sites of occurrence of extraosseous Ewing's sarcoma include the soft tissues and bones of the lower extremity, 12 paravertebral, and retroperitoneal regions. Primary intracranial Ewing's sarcoma/pPNET is usually intraparenchymal located 13 when supratentorially, and an extraaxial epidural tumor radiographically mimicking a meningioma is extremely rare. CASE PRESENTATION: A 20-year14 old male presented to the emergency department with a 1-day history of drowsiness, headache, and fever. Neurological exam15 ination revealed decreased muscle strength (4/5) in the left lower limb. Head computed tomography scan showed an epidural 16 space-occupying lesion in the right temporoparietal region, which was assumed to be a meningioma by radiographic criteria. However, the surgical specimen was diagnosed as Ewing's sarcoma. CONCLUSION: Primary intracranial extraosseous Ewing's sarcoma is a rare condition that may mimic a meningioma on imaging. Physicians must be cognizant of this possibility, particularly in any young individual with a solitary contrast-enhancing dural-based lesion.

Treatment options for paediatric brainstem gliomas.

Paediatric brainstem gliomas (BSGs) can be classified broadly into two distinct categories: focal brainstem gliomas (FBSGs) and diffuse intrinsic pontine gliomas (DIPG). The former account for 20% of paediatric BSGs and are mostly indolent lesions with good prognosis. DIPGs constitute the remaining 80%, and are highly aggressive, malignant tumours having a dismal prognosis; being the foremost cause of death in children with brain tumours. Their poor response to treatment regimens is further complicated by their localization in eloquent brainstem areas, thereby making it difficult to establish a standardized framework of therapeutic intervention. In this review, the authors catalogue and appraise current treatment modalities utilized in the management of paediatric BSGs including steroid use, chemotherapy, radiotherapy, and surgery.

Pre-operative Voriconazole in patients undergoing surgery for Central Nervous System fungal infections: Special Report.

Fungal infections of the central nervous system (CNS) are uncommon. Despite several advancements in diagnosis and treatment of these infections, the mortality rates remain high. The current retrospective study was planned to define the demographic and clinical features of patients with CNS fungal infections. Conducted at Aga Khan University Hospital, Karachi, and comprising CNS fungal infections operated between January 2000 and December 2015. The study analysed whether a short course of pre-operative anti-fungal therapy may improve outcomes in these patients. There were 47 cases confirmed on histopathology and/or microbiology. Outcome measures used were Glasgow coma score (GCS), Glasgow outcome score (GOS) and Karnofsky performance score (KPS). The overall 30-day mortality was 20(42.5%). Fungal infections of the CNS can occur in both immune-compromised and immune-competent patients. Early diagnosis, radical surgery, pre-operative anti-fungal therapy for at least 2 weeks, pre- and postoperative Voriconazole therapy results in more favourable outcomes.

Biological characteristics and outcomes of Gliosarcoma.

Gliosarcoma is a highly aggressive primary brain tumour. It is a relatively rare tumour and comprises of two histological components, glial and sarcomatous. Gliosarcomas carry a poorer prognosis than that of Glioblastoma Multiforme (GBM). The current review highlights important histological and radiological features of gliosarcoma in the light of recent literature, and also touches upon the treatment options and outcomes of various types of gliosarcoma.

Cranioplasty after Craniectomy in a Pediatric Population: Single-Center Experience from a Developing Country.

Cranioplasty is a frequently performed procedure in neurosurgery. The pediatric population for this procedure is distinct from the adult one because of the growing skulls and thinner bones of the calvarium. A paucity of data on the outcomes of this procedure in the pediatric population has been identified repeatedly. We conducted a retrospective cohort study to investigate the outcomes in a pediatric population that underwent cranioplasty after craniectomy at our institute in a developing-world country. Our cohort showed no association of complication rate or cosmetic outcomes with the timing of cranioplasty, area of skull defect, type of implant used, or method of storage.

Paediatric traumatic brain injury: Presentation, prognostic indicators and Outcome analysis from a tertiary care center in a developing country.

Traumatic brain injury (TBI) is the leading cause of morbidity and mortality in children worldwide. This study was conducted to report the presentation, management, outcomes and prognostic indicators in a large series of patients from a tertiary care centre in a developing country. It is a review of prospectively collected data of paediatric patients with TBI admitted at our centre between July 2010 and December 2013. A total of 291 patients with a mean age of 7.2±5.0 years were dichotomised into survivors and non-survivors, and variables were compared between the two groups. The mean post-resuscitation Glasgow coma scale (GCS) score was 11.6±3.9, mean Marshall Score was 2.26±0.95 and the mean revised trauma score at presentation was 10.58±1.7. Younger age, lower GCS score after resuscitation, lower revised trauma score, absent cisterns on imaging, associated subarachnoid haemorrhage (SAH) and intraventricular haemorrhage (IVH) and a lower Marshall score were associated with higher mortality.

Decompressive craniectomy for infants: a case series of five patients.

PURPOSE: Management of cerebral edema in infants is challenging. Decompressive craniectomy in young age has shown favorable outcomes for management of intracranial hypertension, but current literature is scarce and consists of only case reports or small series. The purpose of the current study is to report the challenges faced with this procedure and its complications in this peculiar age group. METHODS: This is a retrospective chart review of infants (less than 1 year of age) undergoing unilateral or bilateral decompressive craniotomy at a tertiary care hospital in Pakistan. Kochi score was used to score outcomes of five infants who underwent the procedure. RESULTS: Five infants were included in this series. Operative time for decompressive craniectomy (DC) ranged from 1 h and 40 min to 4 h. Three infants survived to undergo cranioplasty. Two infants recovered with good Kochi scores of 5a and one infant developed hemiparesis (Kochi score 3b). CONCLUSIONS: Decompressive craniectomy carries good outcomes in selected patients. Risk of bleeding and hemodynamic instability makes this procedure challenging. We found coagulopathy in four of the five patients which poses another challenge to the surgical management of these patients and has not been stressed enough in the previous literature.

Solitary Skull Langerhans Cell Histiocytosis Presenting With a Pus Draining Fistula: An Unusual Presentation and Review of Literature.

Langerhans cell histiocytosis (LCH) is a rare condition in adults, especially when it is limited to a single area of the skull, known as solitary calvarial involvement. In this case report, we present a unique instance of LCH affecting the parietal bone with a pus-draining fistula. This is a rare and unusual presentation at this location, which has been scarcely reported in medical literature. A 30-year-old woman with no prior comorbidity presented with complaints of headache that persisted for a year. She also had swelling on her scalp and a yellowish discharge for 3 weeks, but no neurological problems were observed. Radiology revealed thinning of the calvaria, with ragged margins along the inner table, multiple focal erosions, and involvement of overlying soft tissue and bony sequestrum. The patient underwent biparietal craniotomy and excision of the lesion. The histopathology report showed LCH. After 8 months of follow-up, there was no recurrence. The management of solitary calvarial involvement by LCH with masquerading presentation as a scalp infection can be achieved through complete excision of the lesions, resulting in a favorable outcome.

Comparing redo surgery and stereotactic radiosurgery for recurrent, residual, and/or tumors showing progression in nonfunctioning pituitary adenomas: A systematic review and meta-analysis.

Background: Non-functioning pituitary adenomas (NFPAs) are well-differentiated benign tumors originating from the adenohypophyseal cells of the pituitary gland. They present with headaches, visual disorders, or cranial nerve deficits. NFPAs can recur, progress, or present as residual tumors. We, therefore, conducted this review to compare the effects of both revision surgery and stereotactic surgery on tumor size, visual status, endocrine status, and complications. Methods: A systematic review of published literature on recurrent, residual, or progressing NFPAs that underwent redo surgery or stereotactic radiosurgery from the inception till June 2020 was conducted as per Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Thirteen records (1209 patients) were included, and risk ratio (RR) and 95% confidence intervals (CIs) estimated from each study were pooled using a random-effects meta-analysis model. Results: Redo surgery was the preferred intervention in patients presenting with larger tumor sizes and was more effective in reducing the tumor size as compared to stereotactic radiosurgery (SRS) (risk ratio [RR] 56.14; 95% CI, 16.45-191.58). There was more visual loss with revision surgery as compared to SRS (risk ratio [RR] 0.08; 95% CI, 0.03-0.20). However, SRS was associated with fewer complications, such as new diabetes insipidus, as compared to the redo surgery (risk ratio [RR] 0.01; 95% CI 0.01-0.03). Conclusion: Redo surgery is the superior choice in the treatment of recurrent/residual or progressing NFPAs if the tumor size is large and an immediate reduction in tumor burden through debulking is warranted. However, redo surgery is associated with a higher risk of visual loss, new endocrinopathies, and other complications, in contrast to SRS.

Analysis of factors affecting ventriculoperitoneal shunt survival in pediatric patients.

PURPOSE: Management of hydrocephalus with insertion of ventriculoperitoneal (VP) shunt is associated with significant complications in both adult and pediatric patients. These may be more common in developing countries due to poor economic conditions and a dearth of available resources. We report a 6 years' experience with VP shunt insertion in pediatric patients from a developing country, focusing particularly on factors affecting shunt failure. METHODS: Patients aged below 15 years, who had undergone insertion of VP shunts for hydrocephalus during the years 2006 to 2011, were included. A retrospective analysis of shunt survival was performed using Kaplan-Meier curves and Logrank (Cox-Mantel) test. RESULTS: Among the total 113 patients, the most common etiologies of hydrocephalus were congenital hydrocephalus (19.5 %), brain tumors (14.2 %), and postcranial surgery (13.3 %). Overall shunt failure at a mean follow-up of 11 months was 23 % with the median time to first shunt failure being 68 days. Shunt survival was worse in patients with meningitis (p = 0.024), aqueductal stenosis (p = 0.008), postcranial surgery hydrocephalus (p = 0.006), Caesarean mode of delivery (p = 0.036), congenital abnormalities (p = 0.031), and a past history of surgical excision of mass lesion (p = 0.044).Frequency of shunt failure was also significantly affected by the location of brain tumor (p = 0.015) and prematurity (p = 0.015). CONCLUSION: Premature infants still have a higher rate of shunt failure. Patients with meningitis, aqueductal stenosis, postcranial surgery hydrocephalus, congenital abnormalities, and a past history of surgical excision of mass lesion may have early shunt failure. However, the frequency of shunt failure that we observed (23 %) was much lower than that quoted earlier in the literature and this may be a consequence of rigorous periodic evaluation of patients with VP shunt in situ.

Suicide bomb attack causing penetrating craniocerebral injury.

Penetrating cerebral injuries caused by foreign bodies are rare in civilian neurosurgical trauma, although there are various reports of blast or gunshot injuries in warfare due to multiple foreign bodies like pellets and nails. In our case, a 30-year-old man presented to neurosurgery clinic with signs and symptoms of right-sided weakness after suicide bomb attack. The skull X-ray showed a single intracranial nail. Small craniotomy was done and the nail was removed with caution to avoid injury to surrounding normal brain tissue. At 6 months'follow-up his right-sided power improved to against gravity.

Endocrine Abnormalities in Children With Traumatic Brain Injury at a Tertiary Care Center.

Objective Accidental traumatic brain injury (TBI) can lead to severe complications such as endocrine abnormalities and long-term morbidities and can negatively impact patient lives. These conditions are also associated with a high cost of treatment over a lifetime, a significant concern in low-to-middle-income countries (LMICs). In Pakistan, the prevalence of children with endocrine abnormalities secondary to TBI remains largely unexplored. We conducted a retrospective cross-sectional study to estimate the burden of endocrine abnormalities due to TBI among children in our population. Methods Twenty patients previously admitted with head injury between September and October 2019 were retrospectively reviewed with tests for baseline serum sodium, plasma osmolality, cortisol, adrenocorticotropin (ACTH), free thyroxine (fT4), growth hormone (GH), insulin growth factor-1 (IGF-1), follicle-stimulating hormone (FSH), luteinizing hormone (LH), thyroid-stimulating hormone (TSH), prolactin, estradiol, and testosterone. Data were collated from the electronic Health and Information Management System (HIMS) and analyzed using SPSS v25. Chi-square and t-tests were used to identify associations between variable groups. Outcomes of interest included correlations between hormonal levels and demographic factors, interventions and hormonal levels, and complication rates and hormonal levels. Results Our study reports three (15% of the total cohort) patients with pituitary hormone deficits (two with low IGF-1 and one with low TSH). High serum IGF-1 and ACTH levels were also observed in three (15%) children. High IGF-1 was associated with female gender (p=0.007), mechanical ventilation (p=0.038), and falls (p=0.028). IGF-1 (p=0.035) and GH (p=0.049) levels were associated with improvement in Extended Glasgow Outcome Scale (GOS-E) score. Testosterone was positively correlated with a high percentile for height (p=0.005) and GOS-E scores on follow-up (p=0.030). High testosterone levels (592.12 ± 102.28 ng/dl) were associated with good functional outcomes in post-pubescent patients (p<0.05). Serum fT4 was linked with a high GOS-E score at discharge in prepubescent patients (p=0.034). Neurosurgical decompression was the only risk factor for hormone deficiency, comprising 67% of the group with hormone deficiencies (p=0.028). The learning difficulties were observed exclusively in children with hormonal deficiencies (7 patients, p=0.000).  Conclusion Hormonal dysfunction due to TBI in children can lead to poor outcomes. High serum IGF-1, testosterone, and free T4 levels were associated with improved functional outcomes in children with TBI. Limited follow-up and resources in LMICs are significant barriers to addressing the morbidity associated with these conditions and need to be addressed at a health policy level.

Mature teratoma of the cisterna magna in an adult patient.

Background: Teratomas are a rare subgroup of CNS germ cell tumors and are histologically classified into mature teratomas, immature teratomas, and teratomas with malignant transformation. CNS teratomas are infrequently found in the posterior fossa and pure mature teratomas of posterior fossa are rare especially in adults. We present a case of a young adult female with a mature teratoma in the cisterna magna. Case Description: A 26-year-old female presented to the neurosurgery clinic with headache, nausea and vomiting for the past 1 year. She was found to have dysdiadochokinesia on neurological examination. Brain magnetic resonance imaging scan showed a-well defined lesion, hyperintense on T1 and hypointense on T2-weighted sequences located within the cisterna magna. She underwent a suboccipital craniotomy with resection of lesion. Histopathology confirmed the diagnosis of mature cystic teratoma. Conclusion: Mature teratomas located in the posterior fossa among adults are rare in the literature. We report the second case of mature teratoma in the cisterna magna of an adult patient.

Carotid cavernous fistula in a patient with coronary artery disease.

Carotid cavernous fistulas are abnormal communications between the carotid and cavernous vasculature, with potential for serious neurological and ocular sequelae. There is considerable literature on the neuro-radiological management of carotico-cavernous fistula depending upon their flow status. The coronary artery bypass grafting (CABG) for coronary artery disease (CAD) is a well established method of revascularization. However, the association of carotid cavernous fistula in presence of CAD is an infrequent occurrence. We present a case of coronary artery disease scheduled for bypass surgery, developed spontaneous bilateral carotid cavernous fistulas, highlighting a unique aspect of association between coronary and carotid disease.

Prepontine intracerebral cyst with spontaneous resolution.

BACKGROUND: Intracranial cysts in the prepontine region are rare and can lead to various complications if not managed appropriately. Symptomatic prepontine cysts may require surgical intervention. However, spontaneous resolution of such cysts is rarely reported in literature. CASE DESCRIPTION: We describe the case of a middle-aged lady who presented with headaches and imbalance, with no focal neurological deficits on examination. Magnetic resonance imaging (MRI) of the brain showed a prepontine cyst compressing the brainstem with craniocaudal extension through the foramen magnum. The patient was given symptomatic treatment and followed closely with repeat MRI scans. These scans showed regression and eventual disappearance of the lesion, with complete resolution of symptoms. CONCLUSION: In light of the few reported cases of spontaneous resolution of prepontine cysts, we highlight the possibility of these lesions to self-resolve.

Spinal cord hemangioblastomas with a focus on clinical presentation, diagnosis, and treatment at a tertiary care hospital of Karachi, Pakistan: A retrospective chart review.

BACKGROUND: Hemangioblastomas are benign neoplasms that consist of stromal cells and small blood vessels. They are highly vascular tumors and can arise throughout the central nervous system. This study aims to provide an overview of our experience with this rare tumor's presentation, radiology, histopathology, and outcomes as literature regarding this pathology is sparse from our country. METHODS: The study is a retrospective review of cases that were histopathology proven cases of spinal cord hemangioblastomas. The clinical characteristics of these patients were examined, and their presentation was recorded. The radiology was also reviewed to describe classic appearance on magnetic resonance imaging. A detailed review of immunohistochemistry was also performed and outcome was described. RESULTS: A total of 25 cases of spinal hemangioblastomas were found in our records in the period of 2001-2019. There were 20 males (80%) and only 5 female patients (20%). Gross tumor fragments ranged in size from 0.24 cm2 to 10.5 cm2 (mean 3.28 ± 2.65). Histologically, tumor was composed of nests of large stromal cells with clear to vacuolated cytoplasm separated by thin-walled capillaries. Focal intratumoral hemorrhage was noted. No significant cytological atypia or mitotic figures were noted. Immunohistochemical stains were performed to confirm the diagnosis and exclude other tumors. Inhibin was tested in 20 cases and it was positive in 16 cases (80%). Neuron-specific enolase was positive in 6/8 cases. Cluster of differentiation (CD) CD68 was positive in 6/6 cases and vimentin in 4/4 cases. Glial fibrillary acidic protein (GFAP) and epithelial membrane antigen were performed in 14 and 8 cases, respectively, and all were negative. Cytokeratin AE1/AE3 was negative in 13/13 cases. CD34 highlighted vasculature in the 8 cases in which it was performed and was negative in tumor cells. Follow-up was available in 17 out of 25 cases and ranged from 12 months to 216 months (mean 61.8 ± 60.6 months). Recurrence occurred in 2 out of 17 (11.7%) patients for whom follow-up information was available. CONCLUSION: Our experience shows that spinal cord hemangioblastomas can be surgically removed in most cases with a low risk of recurrence. Most patients in our study were male and unlike other studies, none of our cases showed GFAP positivity.

Arteriovenous malformation with associated multiple flow-related distal anterior cerebral artery aneurysms: A case report with poor outcomes.

BACKGROUND: Low-grade arteriovenous malformations (AVMs) associated with multiple flow-related distal anterior cerebral artery (DACA) aneurysms are rare occurrences. Here, we present a case of a frontal AVM with three associated DACA aneurysms arising from a single feeder. CASE DESCRIPTION: A 36-year-old male presented to us in the ER with acute-onset dysphasia and altered mental status. Head computed tomography and angiogram showed a spontaneous intracerebral hemorrhage with intraventricular extension and revealed a Spetzler Martin Grade II AVM, being fed by two feeders, with the major feeder from the DACA bearing three flow-related aneurysms. As the patient awaited digital subtraction angiography, his Glasgow Coma Scale dropped and he underwent emergency embolization with Onyx. This was followed by external ventricular drainage. The patient's neurological status did not improve, and he died following a complicated clinical course. CONCLUSION: Multiple DACA aneurysms are a case of both clinical and anatomical rarity and to avoid complications in the clinical course, one must be judicious about the time spent between symptom onset and embolization.

Pituitary adenomas: presentations and outcomes in a South Asian country.

OBJECTIVE: The purpose of this retrospective review of all operated cases of pituitary adenomas in the last decade, is to define the demographic patterns and characteristics of such tumors and to assess surgical outcomes with regards to safety and efficacy of trans-sphenoidal tumor removal in our institution. METHODS: Surgically treated pituitary adenomas presenting from 1995 till 2005 were reviewed for different variables. Results were expressed as mean, standard deviation and median for continuous and number with percentage for categorical data. Chi square test was applied to measure differences and significance was taken atp value < 0.05. RESULTS: One hundred and twenty-five patients were operated for pituitary adenoma. Sixty-three percent were male and mean age was 37 years. Sixty percent of the patients presented with headache and/or visual symptoms. Twelve percent presented with pituitary apoplexy and 28% presented with symptoms due to pituitary hyperfunction. Fifty-five percent of patients had functioning and 44% had nonfunctioning adenomas. Mean pre operative tumor diameter from 86 pre op MRI scans was 26.76 mm (3-78 mm). Eighty-four percent of patients underwent trans-sphenoidal tumor resection and three percent had craniotomy. Mean size of post op residual tumor as calculated from 76 available post operative scans was 5.3 mm (range 0-31 mm). 17.6% of the patients required hormone replacement beyond three months and 10% were re-operated. Overall mortality was 1.6%. CONCLUSION: In Pakistan, patients are more likely to present either with apoplexy or with a giant pituitary adenoma than patients reported from developed countries. Overall, our results have been satisfactory and comparable with the literature.