Ruptured pial-pial collateral aneurysm associated with left internal carotid artery occlusion: nuances of surgical management. Illustrative case.

BACKGROUND: Carotid occlusion often leads to the formation of a collateral network. On rare occasions, due to hemodynamic influence, aneurysms can occur. Here, the authors describe a 69-year-old male presenting with intracerebral hemorrhage secondary to a ruptured aneurysm within such a network. OBSERVATIONS: The patient presented to the emergency department with an altered level of consciousness. Imaging showed a left temporal lobe hemorrhage extending into the ventricle, subdural hematoma, and evidence of contrast extravasation. Digital subtraction angiography revealed an occluded left internal carotid artery with the left middle cerebral artery territory reconstituted by flow through an external carotid artery-internal carotid artery anastomosis. The latter was formed by the superficial temporal artery-superior orbital artery, as well as pial-pial collaterals from the posterior temporal artery. Notably, a 4-mm aneurysm arising from the pial-pial collateral network was identified. Surgical intervention involved a left temporal craniectomy and aneurysm excision, with special attention paid to preserving the anastomotic flow through the superficial temporal artery. LESSONS: This case underscores the importance of recognizing and preserving collateral vascular pathways in cases of carotid occlusion with an associated aneurysm. It emphasizes the necessary balance between managing aneurysm risk and maintaining cerebral perfusion, highlighting the need for careful preoperative planning and intraoperative caution.

Transcranial magnetic resonance-guided focused ultrasound pallidothalamic tractotomy for patients with X-linked dystonia-parkinsonism: a study protocol.

Transcranial magnetic resonance-guided focused ultrasound (MRgFUS) is a noninvasive method for controlling tremor and has recently been used in patients with X-linked dystonia-parkinsonism (XDP). This study aims to determine the improvement in dystonia and parkinsonism in patients with XDP after MRgFUS pallidothalamic tractotomy. This prospective study will be conducted at the Philippine General Hospital, University of the Philippines Manila. The primary outcome measure is the change in the pre- and post-treatment XDP-Movement Disorder Society of the Philippines Scale scores. In addition, demographic and clinical data will be collected, including the Burke-Fahn-Marsden Dystonia Rating Scale, Part III of the Movement Disorder Society-Unified Parkinson's disease Rating Scale score, XDP clinical and functional stage, the five-level EuroQol five-dimensional questionnaire, Montreal Cognitive Assessment scores, MRgFUS treatment parameters, and adverse events. Patients will be assessed within 24 hours of treatment, then at 2 weeks, 3 months, 6 months, 9 months, and 12 months post-treatment. This protocol was approved by the University of the Philippines Manila Research Ethics Board (UPMREB 2022-0271-01). Data collection began in January 2023. This protocol has been registered with ClinicalTrials.gov: Trial Registration number: NCT05592028.

Missed pituitary microadenoma during endoscopic transsphenoidal surgery for short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing with symptom relief: illustrative case.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is a rare headache disorder that has been associated with pituitary adenomas. Resection has been posited to be curative. OBSERVATIONS: A 60-year-old female presented with a 10-year history of SUNCT, which had been medically refractory. Sellar magnetic resonance imaging (MRI) showed a 2 × 2 mm nodule in the right anterolateral aspect of the pituitary. Endoscopic endonasal transsphenoidal resection of the pituitary microadenoma with neuronavigation was performed. The patient felt immediate relief from the headaches. Postoperative MRI showed persistence of the pituitary microadenoma and the resection tract to be inferomedial to the lesion. The right middle and partial superior turbinectomy site was close to the sphenopalatine foramen (SPF). The patient was discharged on postoperative day 1 and remained headache-free without any medications at the 4-month follow-up. LESSONS: Resection of pituitary lesions associated with SUNCT may not necessarily be the cause of SUNCT resolution. Manipulation of the middle and superior turbinate close to the SPF may lead to a pterygopalatine ganglion block. This may be the mechanism of cure for SUNCT in patients with related pituitary lesions who undergo endonasal resection.

Time to Care: Analysis of Time Differences and Outcomes in the Management of Pediatric Traumatic Brain Injury for a High-Income and Lower-Middle-Income Country.

BACKGROUND: Pediatric cranial trauma is the leading cause of acquired death and disability in children worldwide. However, trauma resources vary widely among countries. We sought to compare management and timely access to care between a level 1 U.S. pediatric trauma center and a tertiary referral hospital in a lower-middle-income country to assess whether system and resource differences influence care and outcomes. METHODS: We compared data from 214 pediatric head trauma admissions to Philippine General Hospital (Manila) with 136 children from the TRACK-TBI pediatrics study cohort at Massachusetts General Hospital (MGH). Admitted MGH patients were compared with the Philippine cohort regarding demographics; mechanism of injury; times to neurosurgical consult, imaging, and surgery; in-hospital mortality; and length of hospitalization. RESULTS: Age (9 years), gender distribution (67% male), and presenting Glasgow Coma Scale scores were similar (P = 0.10) between sites. More children had intracranial injury in the Philippine cohort (73% vs. 60%; n = 319) and more underwent neurosurgery (27% vs. 4%). Times to consult, imaging, and surgery were longer in the Philippines (12.3 vs. 6.5, 12.0 vs. 2.8, and 45.4 vs. 5.6 hours, respectively). In-hospital mortality across all admissions was similar between cohorts (3% vs. 0%; P = 0.09), but significantly higher in the most severe Philippines cases (31% vs. 0%, P=0.04). Length of stay was longer (5 vs. 2 days; P < 0.001) in the Philippine cohort. CONCLUSIONS: High-income country status correlated with faster care, shorter hospitalizations, and better outcomes among severe cases. Prompt care through sophisticated trauma system implementation may improve pediatric health in resource-limited settings.

Cortical mapping in multilinguals undergoing awake brain surgery for brain tumors: Illustrative cases and systematic review.

BACKGROUND: Awake brain mapping in multilingual patients with brain tumors presents unique challenges to the neurosurgeon. Knowledge of potential eloquent sites is vital to preserve language function. METHODS: We present two cases of pars opercularis glioma and perform a systematic review in accordance with PRISMA guidelines. RESULTS: Our review yielded 7 studies, with a total of 25 multilingual brain tumor patients who underwent awake brain mapping. The age ranged from 25 to 62 years. Majority were female (56.5%). Most (52%) were trilingual, while 20% were quadrilingual and 28% were pentalingual. All tumors were left-sided, mostly in the frontal lobe. These were predominantly gliomas. Extent of resection was gross total in 61%. The brain mapping findings were heterogeneous. Some authors reported a greater number of cortical sites for the first language compared to others. Others found that the first and second languages shared cortical sites whereas the third and subsequent languages were located in distant sites. The peri-Sylvian area was also found to be involved in language that was learned at an earlier age. Subsequent languages thus involved more distant sites. A larger number of cortical areas were also activated for languages that were learned later in life. In terms of language disturbance and recovery, there were mixed results. CONCLUSION: Cortical mapping in multilingual brain tumor patients showed heterogeneity in terms of the location and number of language areas in the face of pathology. These findings may influence neurosurgical and oncological management of tumors in the speech area but emphasize the need to tailor surgical approaches and intraoperative testing to the patient.

The need to consider return to work as a main outcome in patients undergoing surgery for diffuse low-grade glioma: a systematic review.

OBJECTIVE: For a long time, return to work (RTW) has been neglected in patients harboring a diffuse low-grade glioma (LGG). However, a majority of LGG patients worked at time of diagnosis. Moreover, these patients now live longer given current treatment paradigms, especially thanks to early maximal surgery. METHODS: We systematically searched available medical databases for studies that reported data on RTW in patients who underwent resection for LGG. RESULTS: A total of 30 studies were selected: 19 considered RTW (especially rate and timing) as an outcome and 11 used scales of health-related quality of life (HRQoL) which included work-related aspects. Series that considered RTW as a main endpoint were composed of 1014 patients, with postoperative RTW rates ranging from 31 to 97.1% (mean 73.1%). Timing to RTW ranged from 15 days to 22 months (mean 6.3 months). Factors related to an increased proportion of RTW were: younger age, better neurologic status, having a white-collar occupation, working pre-operatively, being the sole breadwinner, the use of awake surgery, and greater extent of resection. Female sex, older age, poor neurologic status, pre-operative history of work absences, slow lexical access speed, and postoperative seizures were negatively related to RTW. No studies that used HRQoL scales directly investigated RTW rate or timing. CONCLUSIONS: RTW was scarcely analyzed in LGG patients who underwent resection. However, because they are usually young, with no or only mild functional deficits and have a longer life expectancy, postoperative RTW should be assessed more systematically and accurately as a main outcome. As majority (61.5-100%) of LGG patients were working at time of surgery, the responsibility of neurosurgeons is to bring these patients back to their previous activities according to his/her wishes. RTW might also be included as a critical endpoint for future prospective studies and randomized control trials on LGGs.

Patient-reported intraoperative experiences during awake craniotomy for brain tumors: a scoping review.

Awake craniotomy (AC) is a neurosurgical procedure that may be used to excise tumors located in eloquent areas of the brain. The techniques and outcomes of AC have been extensively described, but data on patient-reported experiences are not as well known. To determine these, we performed a scoping review of patient-reported intraoperative experiences during awake craniotomy for brain tumor resection. A total of 21 articles describing 534 patients were included in the review. Majority of the studies were performed on adult patients and utilized questionnaires and interviews. Some used additional qualitative methodology such as grounded theory and phenomenology. Most of the evaluation was performed within the first 2 weeks post-operatively. Recollection of the procedure ranged from 0 to 100%, and most memories dealt with the cranial fixation device application, cranial drilling, and intraoperative mapping. All patients reported some degree of pain and discomfort, mainly due to the cranial fixation device and uncomfortable operative position. Most patients were satisfied with their AC experience. They felt that participating in AC gave them a sense of control over their disease and thought that trust in the treatment team and adequate pre-operative preparation were very important. Patients who underwent AC for brain tumor resection had both positive and negative experiences intraoperatively, but overall, majority had a positive perception of and high levels of satisfaction with AC. Successful AC depends not only on a well-conducted intraoperative course, but also on adequate pre-operative information and patient preparation.

Resources for Operative Neurosurgical Education Among Trainees in the Philippines.

BACKGROUND: Operative neurosurgical skills education is a vital part of neurosurgical training, and these skills are usually obtained through operating room experience and supplemented by textbooks and other resources. We aimed to determine the resources used by trainees in the Philippines, both prior to and after the onset of the coronavirus disease 2019 pandemic. METHODS: An online survey was sent to neurosurgical trainees in the Philippines from January to March 2021. Data on demographics, educational resources used, and weekly hours spent on each were collected, for both the pre- and post-coronavirus disease 2019 periods. RESULTS: A total of 37 neurosurgical trainees (60% response rate) participated in the survey. Most respondents were female (70%), in their senior levels (58%), and undergoing training in a public institution (65%). The main resources for operative neurosurgical education were operative experience, online academic resources, and neurosurgical textbooks. After the onset of the pandemic, the overall time spent decreased to 61.2 hours/week from 67.7 hours/week, with a significant reduction in the hours spent on operative experience (27.3 vs. 21.3 hours/week, P < 0.0001) and a significant increase in the time spent on webinars (0 vs. 3.2 hours/week, P < 0.0001) and online resources as a whole (14.9 vs. 16.4 hours/week, P = 0.0003). CONCLUSIONS: Operative experience, online academic resources, and neurosurgical textbooks were the main resources for operative neurosurgical education among trainees in the Philippines. After the onset of the pandemic, the hours spent on operative experience decreased and online academic resources increased significantly. New avenues of neurosurgical education, particularly webinars, also became available locally.

A meta-analysis on the prevalence of anxiety and depression in patients with unruptured intracranial aneurysms: exposing critical treatment gaps.

Unruptured intracranial aneurysms (UIAs) are a significant cause of anxiety and depression. Though the annual rupture rate is relatively low, ensuing mortality and morbidity may be high. Most published studies have focused on functional outcomes; however, limited studies have explored and reported on psychiatric outcomes, which are equally important. We aimed to review existing data on anxiety and depression in patients with UIAs. We systematically searched the databases of Pubmed, Cochrane, Scopus, EBSCOHOST, and ClinicalTrials.gov for studies that reported on anxiety and depression in patients with UIAs. Where available, we also reported data on aneurysm characteristics, treatment modalities, and functional outcomes of these populations. We performed a meta-analysis of proportions by random-effects modeling to compute the prevalence of anxiety and depression in patients with UIAs. Eighteen studies reporting a total of 1413 patients with UIAs were included in the systematic review. The mean age was 57.8 (range 27-79); 64% of whom were female. Random-effect modeling analysis showed an overall estimated prevalence of 28% [95% CI: 0.17-0.42] for anxiety and 21% [95% CI: 0.13-0.33] for depression among patients with UIAs. No significant difference was found in the prevalence of these conditions between treated vs untreated aneurysms. Our review highlights the heterogeneity of data from existing studies and the lack of standardized methodologies in determining psychiatric outcomes in patients with UIAs. It was also limited by the small sample sizes and patient counseling bias in the included studies. Larger, well-designed epidemiologic studies on patients with UIA should include more representative samples, assess for predictors of psychological outcomes, and explore the most optimal psychiatric assessment tools.

Extensive neuritis ossificans of the sciatic nerve: Systematic review and illustrative case.

BACKGROUND: Neuritis ossificans is a rare disease described as heterotopic calcification that occurs in neural tissue such as nerves. METHOD: A systematic review of the literature was done in accordance with PRISMA guidelines. An illustrative case of neuritis ossificans of the sciatic nerve in an 18 year old man was also reported. RESULTS: The review yielded 17 previously reported cases of neuritis ossificans. There was a male predominance and the most common locations were the median and tibial nerves. Only 2 previous cases involved the sciatic nerve. Most were treated with excision with positive outcomes. Our case was treated initially with a biopsy, then partial excision and symptomatic management with NSAIDs and GABA inhibitors. He had a good outcome at 13 months follow-up. CONCLUSION: Neuritis ossificans is a rare disease with unclear pathophysiology that has been theorized to involve neuroinflammation. Treatment consists of excision, if deemed possible, and symptom control.

Predictive Value of Hematologic Inflammatory Markers in Delayed Cerebral Ischemia After Aneurysmal Subarachnoid Hemorrhage.

BACKGROUND AND OBJECTIVE: Our study aimed to determine the ability of hematologic markers (neutrophil/lymphocyte ratio [NLR], platelet/lymphocyte ratio, and red cell distribution width [RDW]) in predicting delayed cerebral ischemia (DCI), modified Rankin Scale scores, and mortality in patients with aneurysmal subarachnoid hemorrhage (aSAH). METHODS: A retrospective observational 6-year review of medical records was conducted to identify all consecutive patients with aSAH admitted to the largest training hospital in the Philippines. Univariable and multivariable regression analyses were performed to determine the association of the biomarkers with the respective outcomes. Receiver operating characteristic curves were used to detect overall predictive accuracy. RESULTS: A total of 222 patients with aSAH were included, of whom 11.71% developed DCI. Most patients with NLR ≥5.9 subsequently died (77 vs. 52%; P = 0.03). DCI was also associated with poor functional outcomes with higher modified Rankin Scale scores (3-6) on discharge (92% vs. 49%; P < 0.01), and longer duration of hospitalization (median, 20 vs. 13 days; P = 0.01). In receiver operating characteristic analyses, the value of RDW was predictive for DCI (area under the curve, 0.70; 95% confidence interval, 0.62-0.79; P < 0.01). The values of NLR (area under the curve, 0.67; 95% CI, 0.59-0.74) potentially predict functional outcome. RDW, NLR, and their combinations were poor discriminators of mortality. CONCLUSIONS: Our study showed that some hematologic parameters analyzed could be of potential value as prognostic biomarkers in patients with aSAH. Hematologic biomarkers are widely available and practical parameters that may be of considerable clinical value in aSAH management, especially in lower-middle-income countries such as the Philippines.

Practice Patterns in Surgical Neuro-Oncology Among Low- and Middle-Income Countries During the Coronavirus Disease 2019 Pandemic: A Scoping Review and Situational Report from the Philippines.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic has negatively affected the outcomes of surgical neuro-oncology patients worldwide. We aimed to review the practice patterns in surgical neuro-oncology in low- and middle-income countries (LMICs). We also present a situational report from our own country. METHODS: A scoping review was performed following the PRISMA-ScR (Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews) guidelines. RESULTS: Twelve studies were included in the review. Most of the studies were from Asia (India, China, Iran, and Turkey), and 1 was from Brazil. Quantitative reports showed a decrease in the number of surgical neuro-oncology operations between pre-COVID-19 and post-COVID-19 time frames, but similar proportions of neuro-oncology procedures. Qualitative review showed similar practice patterns between LMICs and high-income countries, except for limitations in resources such as negative-pressure operating rooms and intensive care units, and maintenance of face-to-face consults despite the adoption of telemedicine. Limited data on adjuvant therapy were available in LMICs. CONCLUSIONS: In our review, we found that the practice patterns in surgical neuro-oncology in LMICs during the COVID-19 pandemic are similar to those in high-income countries, except for a few modifications because of resource limitation and patient preferences.

Intraoperative radiotherapy for glioblastoma: A systematic review of techniques and outcomes.

BACKGROUND: Despite multimodality treatment, the prognosis of glioblastoma (GBM) has remained poor. Intraoperative radiation therapy (IORT) offers additional local control by directly applying a radiation source to the resection margin, where most recurrences occur. METHODS: We performed a systematic review on the oncologic outcomes and toxicities of IORT for GBM in the era of modern external beam radiation therapy (EBRT) and chemotherapy with temozolamide. RESULTS: Four studies representing 123 patients were included. Majority (81%) were newly diagnosed, and gross total resection was reported in 13-80% of cases. IORT modalities included electrons from a linear accelerator (LINAC) and photons from a 50-kV x-ray device. Median doses were from 12.5 to 20 Gy for electron-based studies and 10-25 Gy for photon-based studies. Adjuvant treatment consisted of 46-60 Gy post-operative EBRT in electron-based studies and the Stupp protocol in photon-based studies. Complications included radiation necrosis (2.8-33%), infection, hematoma, perilesional edema, and wound dehiscence. Median time to local recurrence was 9.9-16 months and the reported overall progression-free survival was 11.2-12.2 months. Median overall survival was 13-14.2 months for the electron-based studies and 13.8-18 months for the photon-based studies. CONCLUSION: IORT resulted in improved local control and comparable overall survival rates with the Stupp protocol. Although photon-based IORT had better results than electron IORT, this may be due to improvements in other forms of adjuvant treatment rather than the IORT modality itself. The overall effect of IORT on GBM treatment is still inconclusive due to the small number of patients and heterogeneous reporting of data.

Paving the Path to Wellness: A Systematic Review of Wellness Programs for Neurosurgery Trainees.

BACKGROUND: Neurosurgical trainees have a heavy workload and poor quality of life, resulting in high rates of burnout and attrition. Consequently, wellness programs have been used by various training institutions to combat this situation. OBJECTIVE: We aimed to identify and describe wellness programs available for neurosurgical trainees in their training institutions, the outcome measures used to assess them, and their efficacy. METHODS: A systematic review of the literature was made following PRISMA guidelines. RESULTS: Six studies were included in the review, describing wellness programs from 9 institutions. All programs except 1 used exercise as the core component. The other components included physical and mental well-being lectures, team-building activities, and cultural excursions. Most institutions used piloted satisfaction and perception questionnaires to assess efficacy. Trainee perceptions of wellness programs were generally positive, but the responses on validated questionnaires and surveys were mixed. Barriers to the program included lack of institutional support, time constraints, fatigue, and feelings of guilt in prioritizing wellness over patient care. CONCLUSIONS: There is a paucity of literature regarding trainee wellness in neurosurgery. A few training programs have instituted wellness initiatives for trainees, and the feedback was generally positive. However, objective measures of efficacy such as validated questionnaires and scales yielded mixed results.

Type A Behavior Patterns Among Neurosurgeons and Neurosurgical Trainees.

BACKGROUND: The busy and demanding work schedule of neurosurgeons suggests that they might have type A behavior patterns (TABPs). TABPs are also associated with multiple diseases; hence, their early identification could lead to behavioral changes and disease prevention. We aimed to determine the presence of TABPs among neurosurgeons and trainees in the Philippines and the association between demographic and clinical factors and TABPs. METHODS: An online survey was sent to neurosurgeons and neurosurgical trainees in the Philippines. Data on age, sex, comorbid conditions, smoking, alcohol use, training institution, level of residency training, and place of practice were obtained. The modified Bortner scale was used to determine TABPs. Univariate and multiple regression analyses were used for analysis. RESULTS: A total of 102 individuals (41 neurosurgeons and 61 trainees) completed the survey. Most of the respondents were men (74%), with a mean age of 36 years. The Bortner scores were normally distributed. TABPs were significantly associated with female sex (P = 0.0006) in the entire cohort. However, the trainee cohort also exhibited a significant association between TABPs and training in a public institution (P = 0.0027). CONCLUSION: The presence of TABPs followed a normal distribution among neurosurgeons and neurosurgical trainees in the Philippines. A significant association was found between TABPs and female sex for both neurosurgeons and trainees, and training in a public institution was significantly associated with TABPs only among the trainees.

Awake Craniotomy in Low-Resource Settings: Findings from a Retrospective Cohort in the Philippines.

BACKGROUND: Awake craniotomy is a technique used to maximize resection of lesions in eloquent areas of the brain and preserve function. Although its use in high-income centers is well documented for tumors and vascular lesions, reports of its use in low-middle-income countries are limited. There are no published series from the Philippines. METHODS: We performed a retrospective review of all patients who underwent awake craniotomy at a tertiary referral center in Manila, Philippines from 2010 to 2019. Data on demographics, clinical features, diagnoses, intraoperative and postoperative complications, and outcomes were collected. Regression analyses were performed to correlate use of intraoperative adjuncts with outcome measures (extent of resection, complication rate, neurologic status after surgery and on last follow-up, and in-hospital mortality). RESULTS: A total of 65 patients were included in the cohort, who had a male predilection (60%) and a mean age at diagnosis of 40.4 years. The most common indication was tumor excision (90%), followed by excision of arteriovenous malformations (5%) and cavernomas (3%). Of the tumors, the most common histopathologic diagnosis was low-grade glioma (48%). The intraoperative complication rate was 13.8%, with the most common complication being patient intolerance. Gross total excision rate for tumors was 78.3%. Univariate analysis showed that use of a cortical stimulator was associated with improved neurologic status on last follow-up (P = 0.0471). CONCLUSIONS: Our experience shows that awake craniotomy is feasible in low-middle-income country settings and is safe and effective for excision of tumors, arteriovenous malformations, and cavernomas.

Pediatric choroid plexus papilloma arising from the cerebellopontine angle: systematic review with illustrative case.

INTRODUCTION: Choroid plexus tumors are uncommon intraventricular tumors that develop from the choroid plexus of the central nervous system. Choroid plexus papillomas arising from the cerebellopontine angle have been reported to almost exclusively occur in adults and are rarely found in children. METHODS: We report a systematic review conducted in accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines of SCOPUS and PubMed databases for case reports and case series of choroid plexus papillomas arising in the cerebellopontine angle in the pediatric population and discuss clinical presentation, imaging features, management options, and outcomes. We also report a case managed at our center. RESULTS: Ten cases of pediatric choroid plexus papillomas arising in the cerebellopontine angle were identified from the systematic review in addition to the case reported here, resulting in a total of eleven cases. The patients' median age was 8 years with a slight female sex predilection (1.2:1). Patients most commonly presented with headache, cerebellar signs, and cranial nerve palsies with median duration of symptoms at 4 months. All patients underwent surgical treatment with majority achieving gross total excision. No deaths were reported at median follow-up of 12 months. Complete neurologic recovery was attained in seven cases while partial recovery was seen in two cases. CONCLUSION: Choroid plexus papillomas found in the cerebellopontine angle in the pediatric population are extremely rare but they should be considered in the differential diagnosis. Complete surgical resection is the mainstay of treatment with excellent outcomes achievable in majority of patients.

Giant Cerebellar Cavernomas in Pediatric Patients: Systematic Review with Illustrative Case.

INTRODUCTION: Giant cavernomas (GCMs) are angiographically occult vascular malformations of the central nervous system measuring at least 4 cm in diameter. These are rare lesions, especially in the cerebellum. As such, the clinical and radiologic features, surgical management, and outcomes of treatment for this condition are unknown. METHODS: We performed a systematic review of SCOPUS and PubMed databases for case reports and case series of histopathologically proven GCMs arising from the cerebellum in the pediatric population, and included a case managed at our center. Data on clinical presentation, imaging features, surgical treatment and outcomes of management were collected. RESULTS: A total of 10 cases were identified from the systematic review, including our case report. The patients' median age was 6 months, with a slight male sex predilection (1.5:1). The most common neurologic manifestations were bulging fontanelle in infants, and headache and focal cerebellar deficits in older children. Surgical excision was done in all cases, while 50% of the cases had CSF diversion surgery done prior to excision. In cases with reported outcome, complete neurologic recovery was achieved in seven while partial recovery was reported in one case. No deaths were reported at median follow-up of 12 months. CONCLUSION: Our case report and systematic review show that giant cerebellar cavernoma is a rare differential diagnosis for symptomatic hemorrhagic cerebellar masses in the pediatric age group. Mainstay of treatment is surgical excision, which can be associated with favorable outcome in most cases.