RESUMEN
BACKGROUND: Nigeria has an inadequate number of neurosurgeons to meet the population's demand for neurosurgical care. Furthermore, few Nigerian neurosurgeons are female. This study sought to evaluate perceived barriers to pursuing neurosurgery among Nigerian trainees. METHODS: A 60-question survey was distributed electronically to medical students at the College of Medicine, University of Ibadan, and unspecialized intern physicians at the University College Hospital, Ibadan, Nigeria. Participation was voluntary. RESULTS: One hundred fifty-seven respondents participated in the survey. A greater proportion of males indicated an interest in neurosurgery than females (40% vs. 18%, P = 0.010). Over 75% of respondents identified decreased family and personal time, long work hours, and limited access to maternity or paternity leave as potential barriers to neurosurgery, with no differences by gender. Respondents overall saw being female and low-income as disadvantageous to pursuing neurosurgery in Nigeria. Although they universally viewed research as important in neurosurgery, 59% of respondents reported inadequate access to research opportunities; this did not vary by gender. However, 65% of female respondents reported that having a female neurosurgery mentor would increase their interest in neurosurgery (vs. 37% of males, P = 0.001). CONCLUSIONS: Nigerian medical trainees perceived the time commitment of neurosurgery as a major barrier to pursuing the specialty. Regardless of gender, they also reported low exposure to neurosurgery and inadequate access to research and mentorship opportunities. However, we found that enhanced female representation among neurosurgery mentors and improved work-life balance could increase interest in neurosurgery and help expand Nigeria's neurosurgical workforce.
Asunto(s)
Selección de Profesión , Neurocirugia , Estudiantes de Medicina , Humanos , Nigeria , Femenino , Masculino , Neurocirugia/educación , Estudiantes de Medicina/psicología , Estudios Transversales , Adulto , Encuestas y Cuestionarios , Médicos/psicología , Neurocirujanos , Adulto JovenRESUMEN
Pediatric low-grade gliomas constitute the most common brain tumors worldwide, though with some peculiarities in the presentation and surgical care in different parts of the world. The symptomatology in developing countries is likely to be over longer periods with a tendency to delayed diagnosis due to cultural, religious beliefs, manpower, and infrastructural deficits. Thus, the children present with large tumors with attendant morbidities and an increased risk of mortalities from surgery. Surgery is mainly by "general" neurosurgeons due to the paucity of trained pediatric neurosurgeons. The pre-operative imaging may be limited to anatomic MR imaging, and in some cases, CT scans, without expansive neuropsychological evaluation. The armamentarium available to the neurosurgeon may warrant large openings to access the tumor, and there may be limited possibility for intra-operative mapping of "eloquent" brain functions when this is deemed necessary. Complicating pre-operative acute hydrocephalus can result in two operations that further worsen the catastrophic spending associated with brain tumor surgeries in these climes. While these challenges appear daunting but certainly have not been enough to deter the "can do" spirit of neurosurgeons in developing countries, it is essential to strengthen the training of pediatric neurosurgeons in LMICs and provide a platform for the advocacy of better infrastructure for the surgical management of these tumors.
RESUMEN
OBJECTIVE: Brain tumors are a global problem, leading to higher cancer-related morbidity and mortality rates in children. Despite the progressive though slow advances in neuro-oncology care, research, and diagnostics in sub-Saharan Africa (SSA), the epidemiological landscape of pediatric brain tumors (PBTs) remains underestimated. This study aimed to systematically analyze the distribution of PBT types in SSA. METHODS: Ovid Medline, Global Index Medicus, African Journals Online, Google Scholar, and faculty of medicine libraries were searched for literature on PBTs in SSA published before October 29, 2022. A proportional meta-analysis was performed. RESULTS: Forty-nine studies, involving 2360 children, met the inclusion criteria for review; only 20 (40.82%) were included in the quantitative analysis. South Africa and Nigeria were the countries with the most abundant data. Glioma not otherwise specified (NOS) was the common PBT in the 4 SSA regions combined. However, medulloblastoma was more commonly reported in Southern SSA (p = 0.01) than in other regions. The prevalence and the overall pooled proportion of the 3 common PBTs was estimated at 46.27% and 0.41 (95% CI 0.32-0.50, 95% prediction interval [PI] 0.11-0.79), 25.34% and 0.18 (95% CI 0.14-0.21, 95% PI 0.06-0.40), and 12.67% and 0.12 (95% CI 0.09-0.15, 95% PI 0.04-0.29) for glioma NOS, medulloblastoma, and craniopharyngioma, respectively. Sample size moderated the estimated proportion of glioma NOS (p = 0.02). The highest proportion of craniopharyngiomas was in Western SSA, and medulloblastoma and glioma NOS in Central SSA. CONCLUSIONS: These findings provide insight into the trends of PBT types and the proportion of the top 3 most common tumors across SSA. Although statistical conclusions are difficult due to the inconsistency in the data, the study identifies critical areas for policy development and collaborations that can facilitate improved outcomes in PBTs in SSA. More accurate epidemiological studies of these tumors are needed to better understand the burden of the disease and the geographic variation in their distribution, and to raise awareness in their subsequent management.
Asunto(s)
Neoplasias Encefálicas , Humanos , Neoplasias Encefálicas/epidemiología , África del Sur del Sahara/epidemiología , Niño , Glioma/epidemiología , Preescolar , Adolescente , Prevalencia , Meduloblastoma/epidemiología , Meduloblastoma/terapia , Craneofaringioma/epidemiología , LactanteRESUMEN
OBJECTIVE: To assess the status of brain tumor programs in Asia and Africa and propose comprehensive evidence-based short- and long-term measures for improving the existing systems. METHODS: A cross-sectional analytical study was conducted in June 2022 by the Asia-Africa Neurosurgery Collaborative. A 27-item questionnaire was designed and distributed to gain insight into the status and future directions of brain tumor programs in Asia and Africa. Six components of brain tumor programs were identified-surgery, oncology, neuropathology, research, training, and finances-and assigned scores of 0-14. The total scores allowed subclassification of each country into levels of brain tumor program from I to VI. RESULTS: A total of 110 responses from 92 countries were received. These were subdivided into 3 groups: group 1, countries with response from neurosurgeons (73 countries); group 2, countries with no neurosurgeons (19 countries); and group 3, countries without a neurosurgeon response (16 countries). The components associated with the highest level of brain tumor program were surgery, neuropathology, and oncology. Most countries in both continents had level III brain tumor programs with a mean surgical score of 2.24. The major lag between each group was with respect to the advances in neuropathology and financial support. CONCLUSIONS: There is an urgent need to improve and develop existing and nonexistent neuro-oncology infrastructure, personnel, and logistics in countries across the continents, especially for the countries with no neurosurgeons.
Asunto(s)
Neoplasias Encefálicas , Neurocirugia , Humanos , Estudios Transversales , África/epidemiología , Asia , Neurocirugia/educación , Neoplasias Encefálicas/cirugíaRESUMEN
BACKGROUND: Accidental falls are a common cause of disability and trauma-related death in the pediatric population, accounting for a large number of pediatric emergency hospital admissions. This multicenter study assesses the clinical characteristics, management outcomes of pediatric falls-related traumatic brain injury and associated factors in Nigeria. METHODS: A retrospective study of pediatric patients (age less than 18-years) with falls over a 2-year period. Data was extracted from clinical records and neurosurgical data sheets from three major centres in Nigeria, and was analyzed using descriptive statistics, Chi square test and multinomial regression with significance set at p < 0.05. RESULTS: 128 children presented with fall-related TBI, with a median age of 96 months (age range of 5-216 months) and M:F was 6:1. 40.6 % had moderate head injury and 55.5 % of the falls were building related. There were 52(42.6 %) children with skull fractures. Older children more commonly fell from trees and had more cases of severe head injury (14.1 %) compared with those who fell from buildings (5.5 %). Children who fell from buildings presented early (64.1 %). The 10 % mortality was mainly in children older than 5-years. Children aged 0-5 years were three times likely to fall inside/outside a building (OR: 3.3, CI: 3.06 to 243.44). Also, those who fell from trees were 6 times more likely to have a long bone fracture (OR: 6.1, CI: 1.2 to 32.6). CONCLUSIONS: In the Nigerian population Traumatic Brain Injury from falls is common among children, with older children falling more from trees and associated with high mortality.
Asunto(s)
Lesiones Traumáticas del Encéfalo , Traumatismos Craneocerebrales , Niño , Humanos , Adolescente , Accidentes por Caídas , Estudios Retrospectivos , Lesiones Traumáticas del Encéfalo/complicaciones , Lesiones Traumáticas del Encéfalo/epidemiología , HospitalizaciónRESUMEN
Background: Antiseizure medications, such as phenytoin sodium, have been shown in some reports to reduce the incidence of early post-traumatic seizure. These medications, however, are not without side effects which may be dose related or duration related. The risks associated with short-term therapy are minimal and often dose related (and hence avoidable). This study intends to determine the efficacy of a short-course (48-hour dose) of phenytoin in prevention of early post-traumatic seizure. Methods: This was a prospective randomised double-blind clinical intervention study. Head injured patients presenting within the first 24 hours were randomly assigned to either 48-hour dose of phenytoin or control groups, and were observed for clinical seizure over a week. The difference in the incidences of early post-traumatic seizure between the two groups was determined by χ2 test. A p<0.05 was considered as statistically significant. Results: A total of 94 patients were included in the study, 47 each in the control group and the phenytoin group. There were 77 males and 17 female (M:F 4.5:1). Both groups had similar demographic and clinical profile. The incidence of seizure was 21.3% in the control but 2.1% in the treatment arm (p<0.01). All seizures occurred within 24 hours of trauma in the control, while the only episode of seizure in the treatment group occurred later. Conclusion: A short-course (48-hour dose) of phenytoin might be an effective prophylactic treatment to reduce the incidence of early post-traumatic seizure.
RESUMEN
BACKGROUND: Sub-Saharan African (SSA) neuro-oncologists report high workloads and challenges in delivering evidence-based care; however, these reports contrast with modeled estimates of adult neuro-oncology disease burden in the region. This scoping review aimed to better understand the reasons for this discrepancy by mapping out the SSA adult brain tumor landscape based on published literature. METHODS: Systematic searches were conducted in OVID Medline, Global Index Medicus, African Journals Online, Google Scholar, and faculty of medicine libraries from database inception to May 31, 2021. The results were summarized quantitatively and narratively. English and French peer-reviewed articles were included (title, abstract, and full text). RESULTS: Of the 819 records identified, 119 articles by 24 SSA countries (42.9%) were included in the final review. Odeku published the first article in 1967, and nine of the ten most prolific years were in the 21st century. The greatest contributing region was Western Africa (nâ =â 58, 48.7%) led by Nigeria (nâ =â 37, 31.1%). Central Africa had fewer articles published later than the other SSA regions (P = .61). Most studies were nonrandomized (nâ =â 75, 63.0%) and meningiomas (nâ =â 50, 42.0%) were the most common brain tumors reported. Less than 30 studies reported on adjuvant treatment or patient outcomes. CONCLUSIONS: Most publications were hospital-based, and there was significant heterogeneity in the quality of evidence and reporting. This study highlights the need for rapid and sustainable investments and brain tumor research capacity in SSA.
Asunto(s)
Neoplasias Encefálicas , África del Sur del Sahara/epidemiología , Neoplasias Encefálicas/epidemiología , Neoplasias Encefálicas/terapia , HumanosRESUMEN
Adolescents and Young Adults (AYA), have distinct endocrine and psychosocial peculiarities. Brain tumors occur less among AYAs, compared to other age groups and with better prognosis. There is however a paucity of literature about brain tumors in AYA in sub-Saharan Africa. We aim to describe the clinical characteristics of brain tumors in AYA across five neurosurgical centers in Nigeria and the associated factors. We report results for older children (10-14 years), adolescents (15-19 years) and young adults (20-24 years). This was a retrospective review of AYA with brain tumors over a 10-year period (2010-2019). Data analysis was by descriptive statistics, Chi square test and multinomial regression at α0.05. There were 104 AYAand the male to female ratio was 1.2:1. Headache (79.8%) and visual symptoms (65.4%) were the most common presenting symptoms. Focal limb weakness (44.1%) occurred less frequently. Median duration of symptoms prior to presentation was 9 months. Glioma was the most common tumor (31, 29.8%) while pituitary adenoma and craniopharyngioma constituted 30.8% of the tumors. Patients with symptom duration of ≤one year were more likely to have infratentorial tumors. There was no significant association between the KPS following intervention and the AYA characteristics. Age group was not significantly associated with any of the presenting symptoms except ataxia, which was significantly higher among the 10 to 14 years group.We have described the epidemiology of brain tumors within AYA in Nigeria and highlighted a need to maximize their care and meet their special needs.
Asunto(s)
Neoplasias Encefálicas , Glioma , Adolescente , Neoplasias Encefálicas/epidemiología , Niño , Femenino , Humanos , Masculino , Nigeria/epidemiología , Pronóstico , Estudios Retrospectivos , Adulto JovenRESUMEN
Introduction: Africa has many untreated neurosurgical cases due to limited access to safe, affordable, and timely care. In this study, we surveyed young African neurosurgeons and trainees to identify challenges to training and practice. Methods: African trainees and residents were surveyed online by the Young Neurosurgeons Forum from April 25th to November 30th, 2018. The survey link was distributed via social media platforms and through professional society mailing lists. Univariate and bivariate data analyses were run and a P-value < 0.05 was considered to be statistically significant. Results: 112 respondents from 20 countries participated in this study. 98 (87.5%) were male, 63 (56.3%) were from sub-Saharan Africa, and 52 (46.4%) were residents. 39 (34.8%) had regular journal club sessions at their hospital, 100 (89.3%) did not have access to cadaver dissection labs, and 62 (55.4%) had never attended a WFNS-endorsed conference. 67.0% of respondents reported limited research opportunities and 58.9% reported limited education opportunities. Lack of mentorship (P = 0.023, Phi = 0.26), lack of access to journals (P = 0.002, Phi = 0.332), and limited access to conferences (P = 0.019, Phi = 0.369) were associated with the country income category. Conclusion: This survey identified barriers to education, research, and practice among African trainees and young neurosurgeons. The findings of this study should inform future initiatives aimed at reducing the barriers faced by this group.
RESUMEN
Background The management of severe traumatic brain injury is directed at avoidance of secondary brain injuries. The intensive care unit (ICU) provides the ideal environment to achieving improved survival and functional outcome. The study sets out to identify the factors that determine the access of patients with severe head injury presenting at our hospital, to the ICU and their impact on outcome. Materials and Methods his was a longitudinal study at the University College Hospital, Ibadan. Data of all consecutive severe head injury patients over a 9-month period, presenting to the accident and emergency department, was collected and analyzed using descriptive statistics and chi-squared test. The level of significance was p < 0.05. Result There were 36 males (80.0%) in our study, with road crashes (25; 79.5%) as the most common mechanism of injury. Most patients (33; 73.3%) were transferred to our center after initial care in another hospital. Though 31(68.9%) patients had access to the ICU, they were all delayed, with the most common reason for the delay being lack of ICU space. More patients who got admitted into ICU (14; 45.2%) were alive at 28 days into admission ( p = 0.04). The females (6; 13.3%) significantly survived till 28 days on admission compared with males ( p = 0.03), but there was no difference in the survival rates between children and adults. Conclusion Our study underscores the need for ICU admission in these patients to optimize outcome and identify the nonavailability of beds, as the most important cause of delayed access, as well as the need for increased manpower capacity and organized resource utilization.
RESUMEN
BACKGROUND: Surgical intervention in patients with hemoglobinopathies has been extensively reviewed in the literature, but information on the outcome of cranial surgery in this patient population in sub-Saharan Africa is limited. METHODS: This is a retrospective study of patients with hemoglobinopathies, who underwent brain surgery in our facility. The review covered a 5-year period. We examined patient- and surgery-related variables and described the surgical complications as well as the 60-day mortality. RESULTS: A total of nine procedures (eight under general anesthesia and one under local anesthesia) were performed on seven patients with hemoglobinopathy during the study period. Eight (88.9%) of these were done in female patients and one (11.1%) in a male patient. Six (66.7%) were performed in patients with no previous history of blood transfusion. Hb SC accounted for five (55.6%), Hb SS for three (33.3%), and Hb CC for one (11.1%) procedure, respectively. Three (33.3%) of these procedures were brain tumor-related, three (33.3%) trauma-related, one (11.1%) cosmetic, one (11.1%) vascular, and one for a postoperative complication. Only one (11.1%) procedure was associated with preoperative blood transfusion, whereas there was a need for blood transfusion following five (55.6%) of the procedures. There was a mortality rate of 11.1% (1 case). Other complications were recorded after three (33.3%) of the procedures and none with five (55.6%) of the procedures. CONCLUSION: Neurosurgery is possible and safe in patients with hemoglobin disorders. Adequate preoperative preparation, proper anesthetic techniques, meticulous surgery, and excellent postoperative care can help optimize outcome of surgical intervention in this patient population.
RESUMEN
BACKGROUND: The deficit in access to neurosurgical care worldwide remains a challenge, particularly in the developing economies such as Nigeria, where the neurosurgeon density is low. The concern that arguably subsists in sub-Saharan Africa is the decline in the number of recruited candidates into surgical specialty training programs, including neurosurgery, despite increasing burden of surgical cases. This study assessed the perception and willingness of medical undergraduates regarding neurosurgery career and determined the factors influencing their choice. This understanding will be critical to advancing ways of motivating them toward a neurosurgery career. METHODS: A cross-sectional study was conducted among 256 consenting final-year medical students of the College of Medicine, University of Ibadan, Nigeria. Data on the perception (of) and willingness to choose neurosurgery career were obtained. Data were analyzed with descriptive statistics and χ2 test at P < 0.05. RESULTS: The mean age of respondents was 24.9 ± 1.9 years. Within the duration of their clinical trainings, almost all (96.1%) the respondents had 1-2 neurosurgical postings. Although surgery was the most preferred specialty, only 7.8% of the respondents reported a willingness to choose neurosurgery as a career. Proffered reasons for dissatisfaction with neurosurgical postings were unfriendly teaching environment (52.6%) and incessant industrial strikes, coupled with stress among trainers and residents (27.7%). More male than female students were willing to choose neurosurgery career (P = 0.027). CONCLUSIONS: Our study indicated that the proportion of final-year medical students willing to choose neurosurgery career was low and there was sex disparity in the willingness to pursue a neurosurgical career.
Asunto(s)
Selección de Profesión , Neurocirugia , Estudiantes de Medicina , Estudios Transversales , Femenino , Humanos , Masculino , Nigeria , Adulto JovenRESUMEN
BACKGROUND: Nigeria has the largest population in Africa and has suboptimal access to neurooncology care. It has been estimated that there is approximately 1 neurosurgeon for every 2.4 million people in the country, with only few of these trained in the neurooncology subspecialty and no dedicated medical or radiation neurooncologists. There is a paucity of information on the field of neurooncology in Nigeria. This manuscript aims to provide an overview of the current state of neurooncology literature in Nigeria. METHODS: A systematic literature review was performed, using Google Scholar, PubMed, and African Journals Online, to search for articles related to neurooncology in Nigeria, from 1963-2018. Articles were reviewed and categorized. RESULTS: Sixty-three relevant articles were identified. They comprised original research in basic science (N = 1), clinical science (N = 59), and reviews (N = 3). Retrospective case series were the most common type of publication. Categorizing according to histology, articles focused on meningioma (N = 12), pituitary tumors (N = 10), glioma (N = 7), central nervous system metastases (N = 6), multiple histologic types (N = 25), and other types of tumors (N = 3). Eight pediatric neurooncology publications were among these. Two manuscripts, focusing on surgical subjects, specifically addressed issues on neurooncology clinical practice in Nigeria. Of the total manuscripts, 26 were published in Nigerian-based journals and 37 in journals outside Nigeria. The majority of the journals were low-impact factor journals. An increasing number of publications over time was noted. CONCLUSIONS: There is a small but growing amount of scholarly literature on neurooncology from Nigeria. However, there continues to be room for growth in neurooncology research output. With Nigeria's large patient population, there is potential to learn and add to the academic literature. Although there are logistical obstacles to both patient care and research in neurooncology in Nigeria, there is promise for favorable advancement.
RESUMEN
BACKGROUND: Meningiomas are the second commonest intracranial tumors in many places worldwide. They are rare in the pediatric age group, however, and most studies have been able to document only a few patients. Meningiomas in pediatric patients have also been shown to behave differently from those in the adult population. This study was done to examine histologic types of meningiomas seen in pediatric patients from a predominantly African population using the 2016 World Health Organization (WHO) grading system for intracranial tumors. METHODS: Data from the operating logs of patients and histology reports of the samples sent to the pathology department during the study period were extracted. The data obtained were the age, sex, location of the intracranial tumor, histologic diagnosis, WHO grade, and tumor recurrence. RESULTS: Nine pediatric age patients were found among the 166 surgically excised meningiomas received at the pathology department in our institution over a 19-year period. The age range was from 8 months to 17 years. There was a male-to-female ratio of 1:2 with a female predominance. Six tumors were basally located. All tumors were WHO grade I with transitional meningiomas being the commonest followed by meningothelial. There was no history of recurrence in any of the tumors after complete surgical excisions. CONCLUSIONS: Our study showed the rarity of meningiomas in the study population, and there was a predominance of basally located tumors.
Asunto(s)
Neoplasias Meníngeas/cirugía , Meningioma/cirugía , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Neoplasias Meníngeas/epidemiología , Neoplasias Meníngeas/patología , Meningioma/epidemiología , Meningioma/patología , Recurrencia Local de Neoplasia/epidemiología , Recurrencia Local de Neoplasia/patología , Nigeria/epidemiología , Prevalencia , Estudios RetrospectivosRESUMEN
BACKGROUND: Medical error (ME) remains central to discussions regarding patient's safety and its frequency appears high in surgical specialties because of some peculiarities. We set out to study the perception of surgical residents about medical errors, their ability to recognize them and predisposition to disclosing their errors. METHODS: This was a cross-sectional study among surgical residents at the University College Hospital, Nigeria. Data about their knowledge, perception and recognition of medical errors were obtained. Knowledge and practice of medical error disclosure was also examined. Each of these was scored on Likert scale and scores categorized. Chi-square test and logistic regression were used for analysis with p at <0.05. RESULTS: 92 residents participated and 11(12.0%) were females. 32.6% of the respondents had less knowledge about medical errors and these were significantly junior residents. Residents with poor perception about ME were 43.5% and recent involvement with ME was significantly associated with good perception about ME. Delay in obtaining consultation and delay in diagnosis were identified respectively as MEs by only 40(43.5%) and 31(33.7) of the participants. While 82(89.1%) agreed that all errors should be reported to the consultant, only 20(21.7%) believed patients/relatives should be informed of all errors, while 49(53.3%) were well disposed to disclosing ME. Only 4(4.3%) residents had a formal training on ME. CONCLUSIONS: Knowledge of ME was low among junior residents and residents are less likely to disclose error to patients/relatives. A formal training on ME will impact on their recognition, practice, and disclosure of ME.
Asunto(s)
Internado y Residencia , Errores Médicos , Especialidades Quirúrgicas/educación , Revelación de la Verdad , Adulto , Estudios Transversales , Femenino , Hospitales Universitarios , Humanos , Masculino , Nigeria , Centros de Atención TerciariaRESUMEN
BACKGROUND: Epidermoid tumors are rare, benign slow-growing congenital tumors, most frequently located in the cerebellopontine angle of the intracranial cavity. They usually grow to a large size before patients become symptomatic. Although these tumors are amenable to surgery, their adherence to neurovascular structures poses a surgical challenge that results in subtotal resection, thus increasing the risk of recurrence. CASE DESCRIPTION: We report 2 adult patients whose imaging studies revealed epidermoid tumors located in the cerebellar vermis, an uncommon site for such tumors. The patients presented with variable symptomatology. We highlight the imaging features and challenges of surgery. Both patients had good outcomes, with resolution of symptoms and neurologic deficits. CONCLUSIONS: A safe complete excision of epidermoid tumor and its capsule is possible with a good understanding of their clinical and radiologic features and a high index of suspicion. To the best of our knowledge, this is the first report of cerebellar vermian epidermoid tumors from sub-Saharan Africa.
Asunto(s)
Neoplasias Cerebelosas/cirugía , Vermis Cerebeloso/cirugía , Adulto , Neoplasias Cerebelosas/diagnóstico por imagen , Neoplasias Cerebelosas/patología , Vermis Cerebeloso/diagnóstico por imagen , Vermis Cerebeloso/patología , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Procedimientos Neuroquirúrgicos , Resultado del TratamientoRESUMEN
BACKGROUND: Delleman-Oorthuys syndrome, also known as oculocerebrocutaneous syndrome, is a rare congenital anomaly with ocular, cerebral and cutaneous manifestations. So far, only 40 cases have been described. CLINICAL CASE: A 3-year-old female Nigerian child with no identifiable left eyeball, multiple left-sided facial skin defects and delayed developmental milestones but otherwise uneventful medical and family history was evaluated at the Ophthalmology and Paediatric Neurosurgery in Ibadan, Nigeria. Besides the mentioned defects that were present since birth, brain imaging revealed several brain abnormalities including intracranial cysts. Global hyperreflexia and bilateral flexor plantar response were observed upon clinical examination. Left micro-ophthalmia and orbital mass were detected. A histological assessment of the orbital mass revealed it to be rudimentary ocular tissue. The diagnosis of Delleman-Oorthuys syndrome was made based on the clinico-radiological features. The patient underwent a left-sided posterior fossa cystoperitoneal shunt. The left orbital mass was enuclated and the patient is currently awaiting left eyelid reconstruction and an orbital implant and repair of the left alar nasi cleft. CONCLUSION: To our knowledge, this is the first published report of Delleman-Oorthuys syndrome in a female child of West African descent. Given the variable manifestations of Delleman-Oorthuys syndrome, and overlap with other syndromes, the Delleman-Oorthuys syndrome may be underreported. Neuroimaging of patients with cutaneous tags, orbital cysts and micro-ophthalmia could reveal more cases.
RESUMEN
Gliomas are the most common type of brain cancer in the pediatric patients, constituting about 50% of all childhood intracranial tumors. This is a highly heterogeneous group, varying from the benign WHO histopathological grades I and II to malignant WHO grades III and IV. The histology and location are significant prognostic factors, which influence the decision for surgical intervention, as well as the extent of possible tumor removal. In low-grade gliomas, surgery remains the initial option and should be directed at gross total resection in favorable locations, such as the cerebral hemispheres and the cerebellum. Management of high-grade gliomas (HGG), which are less common in children compared to adults, continue to pose a significant challenge. In non-brainstem HGG, the goal is safe maximal tumor removal, while it generally does not play any role in diffuse intrinsic pontine gliomas. Treatment must, thus, be individualized in the majority of cases of HGG. Surgery for gliomas in children continues to be aided by technological advancements facilitating tumor resection and improving patient safety and outcomes.