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Purpose: Pediatric surgical care in low- and middle-income countries is often hindered by systemic gaps in healthcare resources, infrastructure, training, and organization. This study aims to develop and validate the Global Assessment of Pediatric Surgery (GAPS) to appraise pediatric surgical capacity and discriminate between levels of care across diverse healthcare settings., Methods: The GAPS Version 1 was constructed through a synthesis of existing assessment tools and expert panel consultation. The resultant GAPS Version 2 underwent international pilot testing. Construct validation categorized institutions into providing basic or advanced surgical care. GAPS was further refined to Version 3 to include only questions with a > 75% response rate and those that significantly discriminated between basic or advanced surgical settings., Results: GAPS Version 1 included 139 items, which, after expert panel feedback, was expanded to 168 items in Version 2. Pilot testing, in 65 institutions, yielded a high response rate. Of the 168 questions in GAPS Version 2, 64 significantly discriminated between basic and advanced surgical care. The refined GAPS Version 3 tool comprises 64 questions on: human resources (9), material resources (39), outcomes (3), accessibility (3), and education (10)., Conclusion: The GAPS Version 3 tool presents a validated instrument for evaluating pediatric surgical capabilities in low-resource settings., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The utility and safety of including two neurosurgeons for tumor resections is unknown. This study compares outcomes among pediatric patients with craniopharyngiomas operated on with a dual or single surgeon approach (DSA, SSA)., Methods: A single-center review identified all craniopharyngioma transsphenoidal or craniotomy resections from 2000 to 2020. Surgical years of experience (YOE) and rates of 5-year reoperations, complications, recurrence, and postoperative radiotherapy were analyzed., Results: Twenty-six transsphenoidal and 68 craniotomies were identified among 62 patients. Eleven transsphenoidal (42.3%) utilized DSA and 15 utilized (57.7%) SSA. Eight craniotomies (11.8%) were DSA and 60 (88.2%) were SSA. The surgeon for SSA transsphenoidal procedures had a median of 10.7 YOE (IQR: 9.9-13.7) versus 6.6 (IQR: 2.7-16; p = 0.058) for the lead surgeon in DSAs. The co-surgeon in transsphenoidal DSAs had a median of 27 YOE (IQR: 11.8-35.7). The surgeon for SSA craniotomies had a median of 19.3 YOE (IQR: 12.1-26.4) versus 4.5 years (IQR: 1.3-15.3; p = 0.017) for the lead surgeon in DSA cases. The co-surgeon in DSA craniotomies had a median of 23.2 YOE (IQR: 12.6-31.4). Case complexity was similar across transsphenoidal groups. DSA transsphenoidal resections had fewer complications (18% DSA vs. 33% SSA), reoperations (45% vs. 53%), and radiation therapy (9.1% DSA vs. 33% SSA) than SSA., Conclusion: Lead surgeons in DSAs are frequently junior surgeons while SSAs typically employ senior surgeons. Outcomes did not significantly differ between DSA and SSA. Mentorship through DSAs does not negatively affect patient care., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Few guidelines exist for genetic testing of patients with moyamoya arteriopathy. This study aims to characterize the yield of genetic testing of non-syndromic moyamoya patients given the current pre-test probability., Methods: All pediatric moyamoya patients who received revascularization surgery at one institution between 2018 and 2022 were retrospectively reviewed. Patients with previously diagnosed moyamoya syndromes or therapeutic cranial radiation were excluded., Results: Of 117 patients with moyamoya, 74 non-syndromic patients (44 females, 59%) were eligible. The median age at surgery was 8.1 years. Neurosurgeons referred 18 (24%) patients for neurogenetic evaluation. Eleven (61%) patients subsequently underwent genetic testing. Eight (73%) patients had available testing results. Five (62.5%) of these patients had developmental delay compared to 16 (22%) of the entire cohort. Six (75%) patients who underwent genetic testing were found to have at least one genetic variant. These results led to diagnosis of a new genetic disorder for 1 (12.5%) patient and screening recommendations for 2 (25%) patients. An RNF213 variant in one patient led to recommendations for family member screening and pulmonary hypertension screening. Another patient was diagnosed with CBL disorder and referred for cancer screening. The median age at surgery in patients with clinically actionable findings was 4.6 years compared to 9.2 years in those who were referred for genetic testing. All 3 patients who had an actionable finding had developmental delay., Conclusion: It may be beneficial to refer moyamoya patients under 5 for genetic screening given the high likelihood of discovering actionable mutations., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To describe a surgical technique for posterior cerebral revascularization in pediatric patients with moyamoya arteriopathy. Here, we describe the clinical characteristics, surgical indications, operative techniques, and clinical and radiographic outcomes in a series of pediatric patients with moyamoya disease affecting the posterior cerebral artery (PCA) territory., Methods: A retrospective single-center series of all pediatric patients with moyamoya disease who presented to our institute between July 2009 through August 2019 were reviewed. The clinical characteristics, surgical indications, operative techniques, and long-term clinical and radiographic outcomes of pediatric moyamoya patients with PCA territory ischemia were collected and analyzed., Results: A total of 10 PCA revascularization procedures were performed in 9 patients, 5 female, ages 1 to 11.1 years (average 5.2 years). Complications included 1 stroke, with no infections, hemorrhages, seizures, or deaths. One patient had less than 1 year of radiographic and clinical follow-up. In 8 of 9 patients with at least 1 year of radiographic follow-up, there was engraftment of surgical vessels present in all cases. No new strokes were identified on long-term follow-up despite the radiographic progression of the disease. In the 8 cases available for analysis, the average follow-up was 50.8 months with a range of 12 to 117 months., Conclusions: PCA territory ischemia in patients with progressive moyamoya disease can be surgically treated with indirect revascularization. Here, we describe our experience with PCA revascularization procedures for moyamoya disease, including pial pericranial dural (PiPeD) revascularization and pial synangiosis utilizing the occipital artery. These surgical options may be useful for decreasing the risk of stroke in pediatric moyamoya patients with severe posterior circulation disease., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Arteriovenous malformations (AVMs) represent one of the most challenging diagnoses in pediatric neurosurgery. Until recently, the majority of AVMs was only identified after hemorrhage and primarily treated with surgery. However, recent advances in a wide range of fields-imaging, surgery, interventional radiology, radiation therapy, and molecular biology-have profoundly advanced the understanding and therapy of these complex lesions. Here we review the progress made in pediatric AVMs with a specific focus on innovations relevant to clinical care., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Since the advent of endovascular treatment, the long-term prognosis of vein of Galen malformation (VOGM) has markedly improved; however, the nature of research leading to this point is unclear. The objective of this study was to define the composition of VOGM research to date, by means of a bibliometric analysis of the 100 most cited VOGM articles., Methods: An electronic search of Elsevier's Scopus database was performed to identify the 100 most cited articles on VOGM screened against predetermined criteria. Data were then compared., Results: The 100 most cited VOGM articles were published between 1974 and 2017 in 38 unique journals and originated from 16 unique countries. Mean citation count and rate were 59.4 citations and 2.9 citations/year, respectively. The USA (n = 42); Hôpital de Bicêtre, France (n = 15); and Dr. Pierre Lasjaunias (n = 16) were the largest individual country, institutional, and author contributors. Compared to the older articles (published < 2000), key differences for newer articles were statistically higher citation rates (P < 0.01), more authors (P < 0.01), higher proportion of endovascular treatment descriptions (P = 0.01), and more originating from Asia Pacific (P < 0.01)., Conclusions: From the 100 most cited VOGM articles to date, there has been a noticeable shift from diagnosing VOGM based on the foundational work by Dr. Lasjaunias to understanding how we can model clinical outcomes now that endovascular treatment has become the standard of care. Significant shifts in prognosis are pending, and the current bibliometric data implicate we are on the precipice of more recent works making an impact in the near future., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Congenital conditions comprise a significant portion of the global burden of surgical conditions in children. In Somaliland, over 250,000 children do not receive required surgical care annually, although the estimated costs and benefits of scale-up of children's surgical services to address this disease burden is not known., Methods: We developed a Markov model using a decision tree template to project the costs and benefits of scale-up of surgical care for children across Somaliland. We used a proxy set of congenital anomalies across Somaliland to estimate scale-up costs using three different scale-up rates. The cost-effectiveness ratio and net societal monetary benefit were estimated using these models, supported by disability weights in existing literature., Results: Overall, we found that scale-up of surgical services at an aggressive rate (22.5%) over a 10-year time horizon is cost effective. Although the scale-up of surgical care for most conditions in the proxy set was cost effective, scale-up of hydrocephalus and spina bifida are not as cost effective as other conditions., Conclusions: Our analysis concludes that it is cost effective to scale-up surgical services for congenital anomalies for children in Somaliland., (© 2022. The Author(s) under exclusive licence to Société Internationale de Chirurgie.)

Objectives: To reduce preventable deaths of newborns and children, the United Nations set a target rate per 1000 live births of 12 for neonatal mortality (NMR) and 25 for under-5 mortality (U5MR). The purpose of this paper is to define the minimum surgical workforce needed to meet these targets and evaluate the relative impact of increasing surgeon, anesthesia, and obstetrician (SAO) density on reducing child mortality., Methods: We conducted a cross-sectional study of 192 countries to define the association between surgical workforce density and U5MR as well as NMR using unadjusted and adjusted B-spline regression, adjusting for common non-surgical causes of childhood mortality. We used these models to estimate the minimum surgical workforce to meet the sustainable development goals (SDGs) for U5MR and NMR and marginal effects plots to determine over which range of SAO densities the largest impact is seen as countries scale-up SAO workforce., Results: We found that increased SAO density is associated with decreased U5MR and NMR (P < 0.05), adjusting for common non-surgical causes of child mortality. A minimum SAO density of 10 providers per 100,000 population (95% CI: 7-13) is associated with an U5MR of < 25 per 1000 live births. A minimum SAO density of 12 (95% CI: 9-20) is associated with an NMR of < 12 per 1000 live births. The maximum decrease in U5MR, on the basis of our adjusted B-spline model, occurs from 0 to 20 SAO per 100,000 population. The maximum decrease in NMR based on our adjusted B-spline model occurs up from 0 to 18 SAO, with additional decrease seen up to 80 SAO., Conclusions: Scale-up of the surgical workforce to 12 SAO per 100,000 population may help health systems meet the SDG goals for childhood mortality rates. Increases in up to 80 SAO/100,000 continue to offer mortality benefit for neonates and would help to achieve the SDGs for neonatal mortality reduction., (© 2022. The Author(s) under exclusive licence to Société Internationale de Chirurgie.)

Calciprotein particles (CPP) are nanoscale mineralo-protein aggregates that help stabilize excess mineral in the circulation. We examined the relationship between CPP and bone mineral density in Fabry disease patients. We found an inverse correlation with total hip and femoral neck density, but none with lumbar spine., Purpose: Calciprotein particles (CPP) are colloidal mineral-protein complexes made up primarily of the circulating glycoprotein fetuin-A, calcium, and phosphate. They form in extracellular fluid and facilitate the stabilization, transport, and clearance of excess minerals from the circulation. While most are monomers, they also exist in larger primary (CPP-I) and secondary (CPP-II) form, both of which are reported to be raised in pathological states. This study sought to investigate CPP levels in the serum of patients with Fabry disease, an X-linked systemic lysosomal storage disorder that is associated with generalized inflammation and low bone mineral density (BMD)., Methods: We compared serum CPP-I and CPP-II levels in 59 patients with Fabry disease (37 female) with levels in an age-matched healthy adult cohort (n=28) and evaluated their association with BMD and biochemical data obtained from routine clinical review., Results: CPP-I and CPP-II levels were higher in male Fabry disease patients than female sufferers as well as their corresponding sex- and age-matched controls. CPP-II levels were inversely correlated with BMD at the total hip and femoral neck, but not the lumbar spine. Regression analyses revealed that these associations were independent of common determinants of BMD, but at the femoral neck, a significant association was only found in female patients., Conclusion: Low hip BMD was associated with high CPP-II in patients with Fabry disease, but further work is needed to investigate the relevance of sex-related differences and to establish whether CPP measurement may aid assessment of bone disease in this setting., (© 2022. The Author(s).)

Purpose: To describe the epidemiology and referral patterns of gastroschisis patients in northern Ghana., Methods: A hospital-based retrospective review was undertaken at Tamale Teaching Hospital (TTH) Neonatal Intensive Care Unit (NICU) between 2014 and 2019. Data from gastroschisis patients were compared to patients with other surgical diagnoses. Descriptive and inferential statistics were performed with SAS. Referral flow maps were made with ArcGIS., Results: From a total of 360 neonates admitted with surgical conditions, 12 (3%) were diagnosed with gastroschisis. Around 91% (n = 10) of gastroschisis patients were referred from other hospitals, traveling 4 h, on average. Referral patterns showed gastroschisis patients were admitted from three regions, whereas patients with other surgical diagnoses were admitted from eight regions. Only 6% (12/201) of expected gastroschisis cases were reported during the 6-year period in all regions. All gastroschisis deaths occurred within the first week of life., Conclusions: Improving access to surgical care and reducing neonatal mortality related to gastroschisis in northern Ghana is critical. This study provides a baseline to inform future gastroschisis interventions at TTH. Priority areas may include special management of low birth weight newborns, better referral systems, empowerment of community health workers, and increasing access to timely, affordable, and safe neonatal transport.

Objective: Moyamoya angiopathy (MM) is a chronic, progressive steno-occlusive arteriopathy of the distal internal carotid artery and its proximal branches. MM is recognized as a shared end-pathway common to a broad range of inciting pathologies, suggesting that tailored management is important. Pediatric MM differs from MM in adults. Currently, there are many uncertainties and controversies regarding the diagnosis and management of children with MM. Hence, we conducted an international survey to identify the contemporary management trends followed worldwide., Methods: A survey relating to lifestyle modifications, medical management, diagnosis, surgical management, and follow-up for pediatric MM was circulated across web-based platforms, through various international pediatric neurological and neurosurgical societies. Data collected included geographic region of practice, experience, responses to questions, and comments., Results: One hundred twenty-seven responses were evaluated (104 neurosurgeons and 23 neurologists, from 32 countries, across 6 continents). We found wide variations in the recommendations for management and lifestyle modification, with significant differences between regions of practice. Eighty percent recommend restrictions on physical activity, particularly for symptomatic and non-operated patients. Eighty-four percent prescribe aspirin. Sixty-five percent perform indirect revascularization. Seventy-eight percent recommend performing a staged surgery for bilateral MM. Only 26% perform acetazolamide challenge SPECT to evaluate brain perfusion. Only 15% of responders were from highly experienced centers., Conclusion: This survey reflects the contemporary trends in management of pediatric MM, while highlighting the heterogeneity in the management approach of these patients. There is a need for multicenter, international studies to evaluate the safety, efficacy, and long-term outcome of various aspects of treatment of these patients.

Background: There are complex barriers that increase delays to surgical care in low- and middle-income countries, particularly among the vulnerable population of children. Understanding these barriers to surgical care can result in targeted and strategic intervention efforts to improve care for children. The three-delay model is a widely used framework in global health for evaluating barriers associated with seeking (D1), reaching (D2), and receiving health care (D3). The goal of our study is to evaluate reasons for delays in the surgical care for children in Somaliland using the three-delay framework., Methods: Data were collected in a cross-sectional study in Somaliland from 1503 children through a household survey. Among children with a surgical need, we quantified the number of children seeking, reaching, and receiving care along the surgical care continuum, according to the three-delay framework. We evaluated predictors of the three delays through a multivariate logistic regression model, including the child's age, gender, village type, household income level, region, and household size., Results: Of the 196 children identified with a surgical condition, 50 (27.3%) children had a delay in seeking care (D1), 28 (20.6%) children had a delay in reaching care (D2), and 84 (71.2%) children had a delay in receiving care (D3), including 10 children who also experienced D1 and D2. The main reasons cited for D1 included seeking a traditional healthcare provider, while lack of money and availability of care were main reasons cited for D2. Significant predictors for delays included household size for D1 and D3 and condition type and region for D2., Conclusion: Children in Somaliland experience several barriers to surgical care along the entire continuum of care, allowing for policy guidance tailored to specific local challenges and resources. Since delays in surgical care for children can substantially impact the effectiveness of surgical interventions, viewing delays in surgical care under the lens of the three-delay framework can inform strategic interventions along the pediatric surgical care continuum, thereby reducing delays and improving the quality of surgical care for children.

Background: Delayed access to surgical care for congenital conditions in low- and middle-income countries is associated with increased risk of death and life-long disabilities, although the actual burden of delayed access to care is unknown. Our goal was to quantify the burden of disease related to delays to surgical care for children with congenital surgical conditions in Somaliland., Methods: We collected data from medical records on all children (n = 280) receiving surgery for a proxy set of congenital conditions over a 12-month time period across all 15 surgically equipped hospitals in Somaliland. We defined delay to surgical care for each condition as the difference between the ideal and the actual ages at the time of surgery. Disability-adjusted life years (DALYs) attributable to these delays were calculated and compared by the type of condition, travel distance to care, and demographic characteristics., Results: We found long delays in surgical care for these 280 children with congenital conditions, translating to a total of 2970 attributable delayed DALYs, or 8.4 avertable delayed DALYs per child, with the greatest burden among children with neurosurgical and anorectal conditions. Over half of the families seeking surgical care had to travel over 2 h to a surgically equipped hospital in the capital city of Hargeisa., Conclusions: Children with congenital conditions in Somaliland experience substantial delays to surgical care and travel long distances to obtain care. Estimating the burden of delayed surgical care with avertable delayed DALYs offers a powerful tool for estimating the costs and benefits of interventions to improve the quality of surgical care.

Background: Congenital anomalies have risen to become the fifth leading cause of under-five mortality globally. The majority of deaths and disability occur in low- and middle-income countries including Ghana. This 3-year retrospective review aimed to define, for the first time, the characteristics and outcomes of neonatal surgical conditions in northern Ghana., Methods: A retrospective study was conducted to include all admissions to the Tamale Teaching Hospital (TTH) neonatal intensive care unit (NICU) with surgical conditions between January 2014 and January 2017. Data were collected on demographics, diagnosis and outcomes. Descriptive analysis was performed on all data, and logistic regression was used to predict determinants of neonatal mortality. p < 0.05 was deemed significant., Results: Three hundred and forty-seven neonates were included. Two hundred and sixty-one (75.2%) were aged 7 days or less at presentation, with males (n = 177, 52%) slightly higher than females (n = 165, 48%). The majority were delivered by spontaneous vaginal delivery (n = 247, 88%); 191 (58%) were born in hospital. Congenital anomalies accounted for 302 (87%) of the neonatal surgical cases and 45 (96%) deaths. The most common anomalies were omphalocele (n = 48, 13.8%), imperforate anus (n = 34, 9.8%), intestinal obstruction (n = 29, 8.4%), spina bifida (n = 26, 7.5%) and hydrocephalus (n = 19, 5.5%). The overall mortality rate was 13.5%. Two-thirds of the deaths (n = 30) from congenital anomalies were conditions involving the digestive system with gastroschisis having the highest mortality of 88%. Omphalocele (n = 11, 23.4%), gastroschisis (n = 7, 14.9%) and imperforate anus (n = 6, 12.8%) contributed to the most deaths. On multivariate analysis, low birthweight was significantly associated with mortality (OR 3.59, CI 1.4-9.5, p = 0.009)., Conclusion: Congenital anomalies are a major global health problem associated with high neonatal mortality in Ghana. The highest burden in terms of both caseload and mortality is attributed to congenital anomalies involving the digestive system, which should be targeted to improve outcomes.

Background: Existing data suggest a large burden of surgical conditions in low- and middle-income countries (LMICs). However, surgical care for children in LMICs remains poorly understood. Our goal was to define the hospital infrastructure, workforce, and delivery of surgical care for children across Somaliland and provide policy guidance to improve care., Methods: We used two established hospital assessment tools to assess infrastructure, workforce, and capacity at all hospitals providing surgical care for children across Somaliland. We collected data on all surgical procedures performed in children in Somaliland between August 2016 and July 2017 using operative logbooks., Results: Data were collected from 15 hospitals, including eight government, five for-profit, and two not-for-profit hospitals. Children represented 15.9% of all admitted patients, and pediatric surgical interventions comprised 8.8% of total operations. There were 0.6 surgical providers and 1.2 anesthesia providers per 100,000 population. A total of 1255 surgical procedures were performed in children in all hospitals in Somaliland over 1 year, at a rate of 62.4 surgical procedures annually per 100,000 children. Care was concentrated at private hospitals within urban areas, with a limited number of procedures for many high-burden pediatric surgical conditions., Conclusions: We found a profound lack of surgical capacity for children in Somaliland. Hospital-level surgical infrastructure, workforce, and care delivery reflects a severely resource-constrained health system. Targeted policy to improved essential surgical care at local, regional, and national levels is essential to improve the health of children in Somaliland.

Investing in surgery has been highlighted as integral to strengthening overall health systems and increasing economic prosperity in low-income and middle-income countries (LMICs). The provision of surgical care in LMICs not only affects economies on a macro-level, but also impacts individual families within communities at a microeconomic level. Given that children represent 50% of the population in LMICs and the burden of unmet surgical needs in these areas is high, investing pediatric-specific components of surgical and anesthesia care is needed. Implementation efforts for pediatric surgical care include incorporating surgery-specific priorities into the global child health initiatives, improving global health financing for scale-up activities for children, increasing financial risk protection mechanisms for families of children with surgical needs, and including comprehensive pediatric surgical models of care into country-level plans.

Background: Metrics to measure the burden of surgical conditions, such as disability weights (DWs), are poorly defined, particularly for pediatric conditions. To summarize the literature on DWs of children's surgical conditions, we performed a systematic review of disability weights of pediatric surgical conditions in low- and middle-income countries (LMICs)., Method: For this systematic review, we searched MEDLINE for pediatric surgery cost-effectiveness studies in LMICs, published between January 1, 1996, and April 1, 2017. We also included DWs found in the Global Burden of Disease studies, bibliographies of studies identified in PubMed, or through expert opinion of authors (ES and HR)., Results: Out of 1427 publications, 199 were selected for full-text analysis, and 30 met all eligibility criteria. We identified 194 discrete DWs published for 66 different pediatric surgical conditions. The DWs were primarily derived from the Global Burden of Disease studies (72%). Of the 194 conditions with reported DWs, only 12 reflected pre-surgical severity, and 12 included postsurgical severity. The methodological quality of included studies and DWs for specific conditions varied greatly., Interpretation: It is essential to accurately measure the burden, cost-effectiveness, and impact of pediatric surgical disease in order to make informed policy decisions. Our results indicate that the existing DWs are inadequate to accurately quantify the burden of pediatric surgical conditions. A wider set of DWs for pediatric surgical conditions needs to be developed, taking into account factors specific to the range and severity of surgical conditions.

Purpose: Little is known about the prevalence of pediatric surgical conditions in low- and middle-income countries. Many children never seek medical care, thus the true prevalence of surgical conditions in children in Uganda is unknown. The objective of this study was to determine the prevalence of surgical conditions in children in Uganda., Methods: Using the Surgeons OverSeas Assessment of Surgical Need (SOSAS) survey, we enumerated 4248 individuals in 2315 households in 105 randomly selected clusters throughout Uganda. Children aged 0-18 were included if randomly selected from the household; for those who could not answer for themselves, parents served as surrogates., Results: Of 2176 children surveyed, 160 (7.4 %) reported a currently untreated surgical condition. Lifetime prevalence of surgical conditions was 14.0 % (305/2176). The predominant cause of surgical conditions was trauma (48.4 %), followed by wounds (19.7 %), acquired deformities (16.2 %), and burns (12.5 %). Of 90 pediatric household deaths, 31.1 % were associated with a surgically treatable proximate cause of death (28/90 deaths)., Conclusion: Although some trauma-related surgical burden among children can be adequately addressed at district hospitals, the need for diagnostics, human resources, and curative services for more severe trauma cases, congenital deformities, and masses outweighs the current capacity of hospitals and trained pediatric surgeons in Uganda., Competing Interests: Compliance with ethical standards Funding source Funding was provided by the Duke Global Health Institute, Duke University Department of Neurosurgery, University of Minnesota Department of Surgery, Makerere College of Health Sciences, and Johnson and Johnson Family of Companies. Funding sources played no role in study design, data collection, data analysis, or writing of the manuscript. All authors had full access to the data and had final responsibility for the decision to submit for publication.

Objective: Approximately 10% of patients with neurofibromatosis I (NFI) patients will have central nervous system (CNS) tumors. The most common of these are hypothalamic-optic gliomas, followed by brainstem and cerebellar pilocytic astrocytomas. While isolated pilocytic astrocytomas in NFI are well described, the appearance of multiple pilocytic astrocytomas in an individual patient is less common. The most frequent combination in NFI patients with more than one pilocytic astrocytoma is optic tract/hypothalamic and brainstem. Other combinations are exceedingly rare; multiple pilocytic astrocytomas have only been reported once in the cerebral hemispheres in a patient with NFI. This report presents the first documented case, to our knowledge, of multiple pilocytic astrocytomas in the cerebellum of a patient with NF1., Methods: Case report., Conclusion: The finding of multiple cerebellar pilocytic astrocytomas in a patient with NF1 is important because it expands the spectrum of presentations for patients with NF1 and also highlights specific diagnostic and therapeutic challenges faced by the treating physicians. The genetic and molecular basis of NF1 is reviewed. Strategies of diagnosis and treatment outlined here are relevant to both patients with NF1 and all patients with multiple posterior fossa tumors.