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OBJECTIVE The primary purpose of selective dorsal rhizotomy (SDR) is to ameliorate spasticity in the lower extremities of children with cerebral palsy (CP). In correctly selected patients, this neurosurgical procedure has been shown to have a beneficial effect on many aspects of the child’s life. However, given the challenges faced by adults with CP, it would be valuable to document the status of this population compared to their peers later in adulthood. Therefore, the aim of this study was to determine the physical status, mental health, and health-related quality of life (HRQoL) of adults with CP who underwent SDR at least 25 years ago, compared to matched typically developing (TD) individuals. The second aim was to investigate relationships between physical status and the other outcome measures. METHODS Adults with CP were recruited from a database of children who had undergone SDR performed using the technique introduced by Professor Warwick Peacock at Red Cross War Memorial Children’s Hospital in Cape Town, South Africa, between 1981 and 1991. These individuals were matched for age, sex, body mass index, and socioeconomic status to a TD adult cohort from a similar background. The parameters assessed were lower-extremity muscle tone, passive range of motion, muscle strength, selectivity, functional mobility and dynamic balance (Timed Up and Go [TUG] test), HRQoL (SF-36), and anxiety and depression levels. RESULTS Twenty-six adults with CP who had a median age of 35.8 years (interquartile range 34.2–41.4 years) (female/male: n = 10/16; Gross Motor Function Classification System level I/II/III: n = 13/10/3), were compared to 26 TD adults. Muscle tone was similar, whereas passive range of motion, muscle strength, selectivity, TUG, and SF-36 physical functioning (concept and summary) scores differed between the cohorts. Other SF-36 parameters, anxiety levels, and depression levels were not different. Strong correlations were found between the muscle strength and TUG scores. CONCLUSIONS Normalized lower-extremity muscle tone was sustained 25–35 years after SDR. Whereas the lower scores for physical assessments are in line with findings in other CP populations, remarkably, relatively good mental health and HRQoL scores were reported in this CP group despite their physical limitations. The strong correlation between muscle strength and TUG suggests that strength training after SDR may have value in improving functional mobility and balance.

OBJECTIVEThe main purpose of selective dorsal rhizotomy (SDR) is to reduce spasticity in the lower extremities of children diagnosed with cerebral palsy (CP) and spastic diplegia. The potential for developing spinal abnormalities and pain is a concern, especially in the aging CP population. Therefore, the aim of this study was to evaluate spinal abnormalities, level of pain, and disability (due to back or leg pain) in adults with CP, and associations with participant characteristics, more than 25 years after SDR.METHODSThis is a 9-year follow-up study with data collection conducted in 2008 and 2017. Radiographs were assessed for the degree of scoliosis, thoracic kyphosis and lumbar lordosis curvatures, and prevalence of spondylolysis and spondylolisthesis, while level of pain and disability was determined with a self-developed questionnaire and the Oswestry Disability Index (ODI) questionnaire, respectively.RESULTSTwenty-five participants were included (15 males; median age 35.9 years, IQR 34.3–41.5 years), with a follow-up time after SDR ranging from 25 to 35 years. No clinically relevant changes were found for spinal curvatures, spondylolysis and spondylolisthesis, perceived pain frequency, and ODI scores between 2008 and 2017. While the prevalence of spondylolysis was 44%, spondylolisthesis was found in 20% (of whom 15% were grade I and 5% grade II), lumbar hyperlordosis was found in 32%, thoracic hyperkyphosis in 4%, and scoliosis in 20%. The Cobb angle was < 25°, and no patient required surgery for scoliosis. In addition, the low back was reported as the most common site of pain, with 28% of the adults with CP having daily pain. This resulted in 80% of the cohort indicating none or minimal disability due to pain based on the ODI. The only correlation found was between hyperkyphosis and female gender.CONCLUSIONSAt follow-up more than 25 years after SDR, no progression in spinal abnormalities, level of pain, and disability was found when compared with findings 15 years after SDR. The prevalence of scoliosis, thoracic hyperkyphosis, and lumbar hyperlordosis was within the range reported for adults with CP, while spondylolysis and spondylolisthesis occurred more often than would be expected. It is difficult, however, to establish the role of SDR in this finding, given the limited data on the natural history of CP. Despite the encouraging outcome of this long-term follow-up study after SDR, it is important to continue monitoring adults with CP during the aging process.

Background Hydrocephalus is a common, life threatening complication of human immunodeficiency virus (HIV)-related central nervous system opportunistic infection which can be treated by insertion of a ventriculoperitoneal shunt (VPS). In HIV-infected patients there is concern that VPS might be associated with unacceptably high mortality. To identify prognostic indicators, we aimed to compare survival and clinical outcome following VPS placement between all studied causes of hydrocephalus in HIV infected patients. Methods The following electronic databases were searched: The Cochrane Central Register of Controlled Trials, MEDLINE (PubMed), EMBASE, CINAHL Plus, LILACS, Research Registry, the metaRegister of Controlled Trials, ClinicalTrials.gov, African Journals Online, and the OpenGrey database. We included observational studies of HIV-infected patients treated with VPS which reported of survival or clinical outcome. Data was extracted using standardised proformas. Risk of bias was assessed using validated domain-based tools. Results Seven Hunderd twenty-three unique study records were screened. Nine observational studies were included. Three included a total of 75 patients with tuberculous meningitis (TBM) and six included a total of 49 patients with cryptococcal meningitis (CM). All of the CM and two of the TBM studies were of weak quality. One of the TBM studies was of moderate quality. One-month mortality ranged from 62.5–100% for CM and 33.3–61.9% for TBM. These pooled data were of low to very-low quality and was inadequate to support meta-analysis between aetiologies. Pooling of results from two studies with a total of 77 participants indicated that HIV-infected patients with TBM had higher risk of one-month mortality compared with HIV non-infected controls (odds ratio 3.03; 95% confidence-interval 1.13–8.12; p = 0.03). Conclusions The evidence base is currently inadequate to inform prognostication in VPS insertion in HIV-infected patients. A population-based prospective cohort study is required to address this, in the first instance.

Background: Research in high income countries shows that people with cerebral palsy (CP) are less physically active than typically developing (TD) peers, but less is known regarding physical activity (PA) in those with CP in low-to-middle income countries. The aim of this study was to determine daily step count and levels of PA in adolescents and adults with CP living in urban South Africa, compared to TD peers, and to determine associations with sex, Gross Motor Function Classification System (GMFCS) level, body mass index and socio-economic status.Materials and Methods: This case–control study included 26 adolescents and 22 adults with CP (GMFCS Level I-V) and matched TD peers (25 and 30, respectively). Participants wore an ActiGraph GT3X for 7 consecutive days to determine step count and time (minutes per hour) spent in PA levels, including sedentary (SED), low physical activity (LPA) and moderate to vigorous physical activity (MVPA).Results: The daily step count and PA levels for ambulant adolescents with CP (GMFCS level I-III) were similar to TD peers, while this was less for adolescents classified in GMFCS level IV-V. Daily step count, SED and MVPA were similar for adults classified in GMFCS level I-II compared to TD adults, while all parameters were lower for adults using assistive devices (GMFCS level III) and non-ambulant adults (GMFCS level IV-V) compared to TD peers. Daily step count and PA levels were inversely associated with GMFCS, while no other associations were found.Conclusion: People with CP who were more mobile dependent (higher GMFCS level) were less active. However, adolescents and adults with CP classified as GMFCS levels I-II living in urban South Africa recorded similar step count and PA levels as their TD peers. This was also the case for adolescents using assistive devices, though not for those in the adult group (GMFCS level III). Furthermore, it was apparent that even the ambulant individuals with CP and TD cohorts were relatively inactive. Intervention programs for CP and TD adolescents should be aimed at finding strategies to keep adolescents physically active well into adulthood, in order to promote physical health, social and emotional well-being and independence.

Background Although the short-term outcomes of selective dorsal rhizotomy (SDR) in children with cerebral palsy (CP) have been well documented, less is known about the long-term benefits of SDR, especially while aging. Research question Does the gait of adults with CP, who underwent SDR in childhood, change during a nine-year aging follow-up period? Do associations exists between the gait deviation index (GDI) and contextual factors at follow-up? Methods Three-dimensional motion data was captured (Vicon system) of 26 adults (10 female) with CP and spastic diplegia at baseline (17-to-26 years post-SDR) and at nine-year follow-up (26-to-35 years post-SDR), as well as 41 matched typically developed (TD) adults used for reference data. Kinematic, non-dimensional temporal distance and GDI parameters were determined, and associations with contextual factors were studied. Results At follow-up the adults with CP had a (median [interquartile ranges]) age of 35.8 [34.2–41.2] years and classified as Gross Motor Function Classification System (GMFCS) level I (n = 13), level II (n = 10) and level III (n = 3). Overall no clinically relevant change in gait quality (GDI) was detected over the nine-year follow-up period. However, small changes were found in hip and knee range of motion, peak knee flexion and walking speed. In line with the baseline study, a number of gait parameters were different to TD adults. The only association found was between GDI and GMFCS at follow-up (r = 0.64, p Significance The overall walking pattern of the adults with CP who underwent SDR in childhood was characterised by mild crouch gait with minimal signs of spasticity, confirming former physical examination findings. Some small changes in certain gait parameters were determined, though clinically the gait quality remained stable during the nine-year aging period. This finding supports the stability of the gait pattern during mid-life in adults with CP, who met the strict SDR selection criteria.

OBJECTIVEWorldwide disparities in the provision of surgical care result in otherwise preventable disability and death. There is a growing need to quantify the global burden of neurosurgical disease specifically, and the workforce necessary to meet this demand.METHODSResults from a multinational collaborative effort to describe the global neurosurgical burden were aggregated and summarized. First, country registries, third-party modeled data, and meta-analyzed published data were combined to generate incidence and volume figures for 10 common neurosurgical conditions. Next, a global mapping survey was performed to identify the number and location of neurosurgeons in each country. Finally, a practitioner survey was conducted to quantify the proportion of disease requiring surgery, as well as the median number of neurosurgical cases per annum. The neurosurgical case deficit was calculated as the difference between the volume of essential neurosurgical cases and the existing neurosurgical workforce capacity.RESULTSEvery year, an estimated 22.6 million patients suffer from neurological disorders or injuries that warrant the expertise of a neurosurgeon, of whom 13.8 million require surgery. Traumatic brain injury, stroke-related conditions, tumors, hydrocephalus, and epilepsy constitute the majority of essential neurosurgical care worldwide. Approximately 23,300 additional neurosurgeons are needed to address more than 5 million essential neurosurgical cases—all in low- and middle-income countries—that go unmet each year. There exists a gross disparity in the allocation of the surgical workforce, leaving large geographic treatment gaps, particularly in Africa and Southeast Asia.CONCLUSIONSEach year, more than 5 million individuals suffering from treatable neurosurgical conditions will never undergo therapeutic surgical intervention. Populations in Africa and Southeast Asia, where the proportion of neurosurgeons to neurosurgical disease is critically low, are especially at risk. Increasing access to essential neurosurgical care in low- and middle-income countries via neurosurgical workforce expansion as part of surgical system strengthening is necessary to prevent severe disability and death for millions with neurological disease.

Background: Single-event multilevel surgery (SEMLS) approach is regarded as the golden standard in developed countries to improve gait and functional mobility in children with cerebral palsy (CP). However, this approach is not always feasible in developing countries. Therefore, orthopedic surgery based on an interval surgery approach (ISA) is still commonly used in developing countries, although little is known about the long term outcomes of an ISA. Therefore, the aim of this study was to describe the gait patterns of adults with CP, who have been treated with ISA, which started more than 15 years ago. Materials and Methods: Thirty adults with CP and spastic diplegia, who received ISA treatment 21.6–33.7 years ago, were recruited for this study and participated in three-dimensional gait analysis. Twenty kinematic and nondimensional temporal-distance parameters were captured, while the overall gait deviation index (GDI) was also calculated. Data of the adults with CP were compared to normative data of typically developing (TD) adults. Results: Although all adults with CP were still ambulant, their gait parameters significantly differed from TD adults, with a lower GDI in the adults with CP. The CP gait patterns were characterized by excessive hip flexion and hip internal rotation as well as a stiff-knee gait. Conclusion: Although different to TD adults, the gait patterns observed in the adult with CP treated with ISA is in line with other studies. Gait patterns suggest that derotation osteotomies potentially could have improved the long term gait patterns. Although SEMLS might be the preferred treatment method, potentially resulting in better outcomes, ISA can also be used to treat children with CP in developing countries as India and South Africa, where a SEMLS approach is not always feasible.

Charles F M Saint, a 33-year-old graduate from the University of Durham, Newcastle upon Tyne, was appointed to establish the first department of surgery in South Africa (SA) at the University of Cape Town (UCT) in 1920. A mentee of the celebrated British surgeon, Prof. James Rutherford Morison, Saint's distinguished surgical pedigree and exceptional academic and clinical achievements underpinned his astute leadership and legendary ability to inspire, essential qualities necessary for the founding professor of SA surgery. Saint's imprimatur gave primacy to teaching and a priority to skilled, rigorous and fundamental undergraduate instruction, expounding the Morison-Saint philosophy, which made the department the seedbed of SA surgery. He was the first to introduce basic research programmes in clinical departments. During his tenure, Saint received wide international recognition and honours and when he retired in 1946, he had taught more than 1 300 students, trained 7 professors of surgery and over 40 specialist surgeons, instilling his distinctive brand of disciplined, caring surgery. In his 26 years at UCT and Groote Schur Hospital, Saint laid the foundations and built a department of surgery with a global reach and an enduring legacy at the southern tip of Africa.

Introduction COVID-19 has affected the global provision of neurosurgical services. We sought to review the impact of COVID-19 on the neurosurgical services in Africa. Methods A cross-sectional survey was distributed to African neurosurgeons seeking to review demographics, national and neurosurgical preparedness, and change in clinical services in April 2020. Results A total of 316 responses from 42 countries were received. Of these, 81.6% of respondents were male and 79.11% were under the age of 45 years. In our sample, 123 (38.92%) respondents were in training. Most (94.3%) respondents stated they had COVID-19 cases reported in their country as of April 2020. Only 31 (41.50%) had received training on managing COVID-19. A total of 173 (54.70%) respondents were not performing elective surgery. There was a deficit in the provision of personal protective equipment (PPE): surgical masks (90.80%), gloves (84.80%), N95 masks (50.80%), and shoe covers (49.10%). Health ministry (80.40%), World Health Organization (74.50%), and journal papers (41.40%) were the most common sources of information on COVID-19. A total of 43.60% had a neurosurgeon in the COVID-19 preparedness team; 59.8% were concerned they may contract COVID-19 at work with a further 25.90% worried they may infect their family. Mental stress as a result of COVID-19 was reported by 14.20% of respondents. As of April 2020, 73.40% had no change in their income. Conclusions Most African countries have a national COVID-19 policy response plan that is not always fully suited to the local neurosurgery services. There is an ongoing need for PPE and training for COVID-19 preparedness. There has been a reduction in clinical activities both in clinic and surgeries undertaken.

Background Containment measures for COVID-19 have affected surgical training globally. We sought to assess how neurosurgical training has been affected across Africa in April 2020. Methods A cross-sectional survey was distributed to African Neurosurgical trainees seeking to review demographics and effects of COVID on training. Results A total of 123 neurosurgery trainees responded from 23 African countries and a further 6 were abroad. A total of 91.80% were men, and 96.70% were training in public institutions. Only 41% had received training in COVID-19 with 61.79% worried that they would contract COVID-19 while performing their clinical duties. There was a marked reduction in clinical activities including a median reduction of elective surgery (−80%), clinics (−83%), and emergency surgery (−38.50%). A total of 23.58% of residents did not receive a formal salary, with 50% on less than $1000 USD gross per month. Conclusions This is the first continental survey of neurosurgery trainees in Africa. COVID-19 has significantly affected clinical and learning opportunities. There are concerns of the long-term effects on their training activities for an uncertain period of time during this pandemic. Although there has been a global increase in e-learning, there is need to evaluate if this is accessible to all trainees.

Tumours are known to increase the risk of infections, especially those occurring in the central nervous system where insertion of surgical hardware/shunts such as in craniopharyngiomas may be required. However, infections are surprisingly scarce in craniopharyngioma cases. In this study, we explored the possibility of antimicrobial effects of craniopharyngioma cystic fluid. The antibacterial effect of craniopharyngioma cystic fluid samples against selected human pathogens: Escherichia. coli, S. aureus and S. pneumoniae were determined using the agar disc diffusion method. Streptomycin and ampicillin were used as controls. The test organisms were cultured in Mueller-Hinton broth overnight at 37 °C. McFarland standard was used as a reference to adjust the inoculum size of each test organism to a concentration of 1 × 106 CFU/ml using sterile broth. The craniopharyngioma cystic fluid inhibited growth of Gram-positive bacteria S. aureus and S. pneumoniae, but not the Gram-negative bacteria, E. coli. The samples showed the highest zones of S. pneumoniae growth inhibition of up to 20.0 ± 1.0 mm compared with 18.0 ± 1.0 mm of streptomycin and 9.0 ± 0.0 mm of ampicillin. Craniopharyngioma cystic fluid showed significant antibacterial properties against Gram-positive bacteria. This novel finding has implications in the way we view infections in craniopharyngioma patients. More studies need to be carried out to further elucidate this unique finding and possibly exploit these antimicrobial properties.

ObjectivesThe optimal size of the health workforce for children’s surgical care around the world remains poorly defined. The goal of this study was to characterise the surgical workforce for children across Brazil, and to identify associations between the surgical workforce and measures of childhood health.DesignThis study is an ecological, cross-sectional analysis using data from the Brazil public health system (Sistema Único de Saúde).Settings and participantsWe collected data on the surgical workforce (paediatric surgeons, general surgeons, anaesthesiologists and nursing staff), perioperative mortality rate (POMR) and under-5 mortality rate (U5MR) across Brazil for 2015.Primary and secondary outcome measuresWe performed descriptive analyses, and identified associations between the workforce and U5MR using geospatial analysis (Getis-Ord-Gi analysis, spatial cluster analysis and linear regression models).FindingsThere were 39 926 general surgeons, 856 paediatric surgeons, 13 243 anaesthesiologists and 103 793 nurses across Brazil in 2015. The U5MR ranged from 11 to 26 deaths/1000 live births and the POMR ranged from 0.11–0.17 deaths/100 000 children across the country. The surgical workforce is inequitably distributed across the country, with the wealthier South and Southeast regions having a higher workforce density as well as lower U5MR than the poorer North and Northeast regions. Using linear regression, we found an inverse relationship between the surgical workforce density and U5MR. An U5MR of 15 deaths/1000 births across Brazil is associated with a workforce level of 5 paediatric surgeons, 200 surgeons, 100 anaesthesiologists or 700 nurses/100 000 children.ConclusionsWe found wide disparities in the surgical workforce and childhood mortality across Brazil, with both directly related to socioeconomic status. Areas of increased surgical workforce are associated with lower U5MR. Strategic investment in the surgical workforce may be required to attain optimal health outcomes for children in Brazil, particularly in rural regions.

Background: Neurosurgical task shifting and task sharing (TS/S), delegating clinical care to non-neurosurgeons, is ongoing in many hospital systems in which neurosurgeons are scarce. Although TS/S can increase access to treatment, it remains highly controversial. This survey investigated perceptions of neurosurgical TS/S to elucidate whether it is a permissible temporary solution to the global workforce deficit. Methods: The survey was distributed to a convenience sample of individuals providing neurosurgical care. A digital survey link was distributed through electronic mailing lists of continental neurosurgical societies and various collectives, conference announcements, and social media platforms (July 2018-January 2019). Data were analyzed by descriptive statistics and univariate regression of Likert Scale scores. Results: Survey respondents represented 105 of 194 World Health Organization member countries (54.1%; 391 respondents, 162 from high-income countries and 229 from low- and middle-income countries [LMICs]). The most agreed on statement was that task sharing is preferred to task shifting. There was broad consensus that both task shifting and task sharing should require competency-based evaluation, standardized training endorsed by governing organizations, and maintenance of certification. When perspectives were stratified by income class, LMICs were significantly more likely to agree that task shifting is professionally disruptive to traditional training, task sharing should be a priority where human resources are scarce, and to call for additional TS/S regulation, such as certification and formal consultation with a neurosurgeon (in person or electronic/telemedicine). Conclusions: Both LMIC and high-income countries agreed that task sharing should be prioritized over task shifting and that additional recommendations and regulations could enhance care. These data invite future discussions on policy and training programs. Keywords: Global health; Global neurosurgery; HIC, High-income country; LMIC; LMIC, Low- and middle-income country; NSOAP, National Surgical Anesthesia and Obstetric Plan; Neurotrauma; TS/S, Task shifting and task sharing; Task sharing; Task shifting; WHO, World Health Organization; Workforce.

Emeritus Professor Jonathan Clemence Peter passed away in Wynberg, Cape Town, on April 02, 2018, after an illness confronted with quiet courage and dignity. A beloved father, husband, neurosurgeon, leader, teacher, mentor and friend, he is remembered with deep respect and affection throughout the world of paediatric neurosurgery.

In recent years, much progress has been made in our understanding of traumatic brain injury (TBI). Clinical outcomes have progressively improved, but evidence-based guidelines for how we manage patients remain surprisingly weak. The problem is that the many interventions and strategies that have been investigated in randomized controlled trials have all disappointed. These include many concepts that had become standard care in TBI. And that is just for adult TBI; in children, the situation is even worse. Not only is pediatric care more difficult than adult care because physiological norms change with age, but also there is less evidence for clinical practice. In this article, we discuss the heterogeneity inherent in TBI and why so many clinical trials have failed. We submit that a key goal for the future is to appreciate important clinical differences between patients in their pathophysiology and their responses to treatment. The challenge that faces us is how to rationally apply therapies based on the specific needs of an individual patient. In doing so, we may be able to apply the principles of precision medicine approaches to the patients we treat.

Purpose: To determine changes in level of accomplishment and satisfaction in daily activities and social participation, and functional mobility in adults with cerebral palsy (CP) more than 25 years after selective dorsal rhizotomy (SDR). Materials and methods: This long-term observational nine-year follow-up study included 26 adults (median age 35 years) with CP and spastic diplegia, and 26 matched typically developing adults. Assessment tools used were the Life-Habits questionnaire and the Functional Mobility Scale. Results: Most of the adults with CP were independent and satisfied with accomplishing life habits and no changes were determined, except for a small change in the Housing accomplishment level. Compared to typically developing adults, the CP cohort was more dependent in accomplishing Mobility and Recreation. However, the level of satisfaction was similar for most life habits except for Mobility. Functional mobility did not change, but correlated with Life-Habits results. Conclusions: Adults with CP showed high and stable levels of accomplishment and satisfaction in daily activities and social participation more than 25 years after SDR. This is in contrast with the literature, where functional decline was shown for individuals with CP as they age. The relation with functional mobility highlights the importance to focus the rehabilitation on maintaining walking ability in order to enable high level of daily activities and social participation in adults with CP.Implications for rehabilitation Selective dorsal rhizotomy (SDR) is a valuable treatment option for a selective group of children with cerebral palsy (CP) in order to reduce spasticity. The long-term outcomes of SDR on level of accomplishment and satisfaction in daily activities and social participation as well functional mobility in adults with CP are not clear. More than 25 years after SDR adults with CP experienced stable and lasting high levels of functioning regarding daily activities and social participation and were satisfied with the way they accomplished life habits. Functional mobility was correlated to level of accomplishment and satisfaction in daily activities and social participation, which highlights the importance to focus rehabilitation programs on maintaining functional mobility in order to enable daily activities and social participation in adults with CP.

The purpose of this study was to determine the incidence of spinal deformities in ambulant adults with cerebral palsy (CP) and spastic diplegia, more than 15 years after orthopaedic interval surgery approach (ISA) treatment, and its relationship to contextual factors, level of pain and physical status. Spinal X-rays, pain (Oswestry Disability Index (ODI) and location/frequency) questionnaires and physical examination assessing lower extremity muscle strength (Medical Research Council scale), motor control (selectivity scale) and muscle tone (Ashworth score) were conducted in 30 adults with spastic diplegic CP. Mild scoliosis (curve 12–22°) was determined in eight (28%) participants. Hyperkyphosis (> 50°) was reported in two (7%) and lumbar hyperlordosis (> 60°) in five (17%) participants. Pain was most commonly reported at cervical (n = 19, 63%) and lumbosacral (n = 18, 60%) area, resulting in ‘moderate disability’ for six (20%) and ‘severe disability’ for one (3%) participant. Most apparent physical abnormalities determined were hip abduction weakness and increased rectus femoris muscle tone. Regarding correlations, no relations were found for scoliosis curvature, but kyphosis curvature was related to females, ODI scores (lifting and sitting) and increased muscle tone of ankle plantar flexor muscles, lordosis curvature to passive hip extension mobility, and hip flexors and ankle plantar flexors muscle tone. Adults with spastic diplegic CP who received their first orthopaedic intervention more than 15 years ago (based on ISA) showed similar incidence of spinal deformities as reported in the younger CP population, suggesting stability of spinal curvature into adulthood. These slides can be retrieved under Electronic Supplementary Material.

© 2016, Springer-Verlag Berlin Heidelberg. Purpose: To analyse the diagnostic accuracy of age-related optic nerve sheath diameter (ONSD) cut-off values in children for detecting raised intracranial pressure (ICP) and to assess the benefit of using patency of the anterior fontanelle in describing a different set of cut-off values. Methods: The ONSD measurement was performed prior to invasive ICP measurement in children under general anesthesia. The diagnostic accuracy of the ONSD measurement was compared to ICP at thresholds of 20, 15, 10, and 5 mmHg. This was further analysed in children above and below the age of 1 year, with a subgroup analysis of age at 4 years, and assessment of the anterior fontanelle (AF) as a reliable physiological marker in part II of this study. Results: Data from 174 children were analysed. In children ≤1 year old, the ONSD measurement with the best diagnostic accuracy for detecting ICP ≥ 20 mmHg was 5.16 mm, compared to 5.75 mm in children >1 year old (p

Assessment of intracranial pressure (ICP) remains a fundamental tool in the care of patients with certain neurological disorders. Invasive monitoring of ICP, using microsensor devices placed within the brain parenchyma or transduced external ventricular drains remain the gold standard. These techniques provide valuable diagnostic information, but have specific limitations, with the most significant of these being the risk of infection and hemorrhage.1-3 The indications for ICP monitoring beyond some of the guidelines for severe traumatic brain injury, however, still remain unclear.4,5 This results in unnecessary invasive procedures being performed and highlights the need for a reliable noninvasive technique to estimate ICP. Numerous noninvasive surrogate markers of ICP have been described,4-7 but none of these have yet been able to replace invasive monitoring as the criterion standard technique. The optic nerve sheath (ONS) is a continuation of the intracranial meninges, and the perineural subarachnoid space surrounding the optic nerve is a septated, trabeculated region filled with cerebrospinal fluid (CSF).8 The ONS compartment represents a cul-de-sac space, which is in communication with the intracranial subarachnoid space, and an increase in CSF transmission to the ONS occurs when ICP is increased.9-11 Consequently, the accumulation of CSF within the perineural space due to increased ICP can result in distension of the ONS and the optic nerve protrusion or flattening of the posterior globe.12 The relationship between ICP and the optic nerve sheath diameter (ONSD) has been described by several authors with varying accuracy.13 The most distinct limitation of these studies is the difficulty in defining the stiffness of the ONS, a parameter that would be extremely helpful in interpreting the ONSD.

Background: Because nearly 23,000 more neurosurgeons are needed globally to address 5 million essential neurosurgical cases that go untreated each year, there is an increasing interest in task-shifting and task-sharing (TS/S), delegating neurosurgical tasks to nonspecialists, particularly in low- and middle-income countries (LMICs). This global survey aimed to provide a cross-sectional understanding of the prevalence and structure of current neurosurgical TS/S practices in LMICs. Methods: The survey was distributed to a convenience sample of individuals providing neurosurgical care in LMICs with a Web-based survey link via electronic mailing lists of continental societies and various neurosurgical groups, conference announcements, e-mailing lists, and social media platforms. Country-level data were analyzed by descriptive statistics. Results: The survey yielded 127 responses from 47 LMICs; 20 countries (42.6%) reported ongoing TS/S. Most TS/S procedures involved emergency interventions, the top 3 being burr holes, craniotomy for hematoma evacuation, and external ventricular drain. Most (65.0%) believed that their Ministry of Health does not endorse TS/S (24.0% unsure), and only 11% believed that TS/S training was structured. There were few opportunities for TS/S providers to continue medical education (11.6%) or maintenance of certification (9.4%, or receive remuneration (4.2%). Conclusions: TS/S is ongoing in many LMICs without substantial structure or oversight, which is concerning for patient safety. These data invite future clinical outcomes studies to assess effectiveness and discussions on policy recommendations such as standardized curricula, certification protocols, specialist oversight, and referral networks to increase the level of TS/S care and to continue to increase the specialist workforce. Key words: Capacity, Global health, Global neurosurgery, LMIC, Task-sharing, Task-shifting, Workforce

Prevention and management of opportunistic infections in children is particularly relevant in an era demonstrating an increased prevalence of immunocompromising conditions. The presence of an unusual organism which results in serious infection in a child should therefore always raise the consideration of immune compromise. The more common opportunistic infections have become easier to recognize in recent times due to improved awareness and more refined diagnostic testing. Targeted treatment is usually followed by long-term prophylactic medication. The impact of these conditions on patient outcome is of clear significance and certainly warrants further discussion.

Pediatric spinal infection includes spinal epidural abscess, spondylodiscitis and intradural (extramedullary and intramedullary) spinal infections. However, these entities are extremely rare and no clear guidelines exist for their management. Pertinent literature was searched and a detailed narrative review of this topic is presented.

Background Measurement of optic nerve sheath diameter (ONSD) is a promising technique for noninvasive assessment of intracranial pressure (ICP), but has certain limitations. A recent study showed that the deformability index (DI), a dynamic parameter quantifying the pulsatile nature of the optic nerve sheath, could differentiate between patients with high vs normal ICP. Objective To further evaluate the diagnostic accuracy of the DI, when interpreted together with ONSD. Methods This prospective study included children undergoing invasive ICP measurement as part of their clinical management. Ultrasound images of the optic nerve sheath were acquired prior to measuring ICP, the images were further processed to obtain the DI. Patients were dichotomized into high (≥20 mm Hg) or normal ICP groups and compared using the Mann-Whitney U-test. Diagnostic accuracy was described using area under the receiver operating characteristic curve (AUC), sensitivity and specificity, correlation between DI, ONSD, and ICP was investigated using linear regression. Results A total of 28 patients were included (19 high ICP). The DI was lower in the high ICP group (0.105 vs 0.28, P = .001). AUC was 0.87, and a cut-off value of DI ≤ 0.185 demonstrated sensitivity of 89.5% and specificity of 88.9%. Diagnostic accuracy improved when combining DI with ONSD (AUC 0.98, sensitivity 94.7%, specificity 88.9%) and correlation with ICP improved when combined analysis of DI and ONSD was performed (Pearson correlation coefficient: 0.82 vs 0.42, respectively, P = .012). Conclusion The DI was significantly lower for patients with high vs normal ICP. This relationship improved further when the DI and ONSD were interpreted together.

Background Hydrocephalus is a recognised complication of human immunodeficiency virus (HIV)-related opportunistic infections. Symptomatic raised cerebrospinal fluid pressure can be treated with ventriculoperitoneal shunt insertion (VPS). In HIV-infected patients however, there is a concern that VPS might be associated with unacceptably high rates of mortality. We aim to systematically review and appraise published literature to determine reported outcomes and identify predictors of outcome following VPS in relevant subgroups of HIV-infected adults. Methods The following electronic databases will be searched: The Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE (PubMed), EMBASE, CINAHL (EBSCOhost), LILACS (BIREME), Research Registry (www.researchregistry.com), the metaRegister of Controlled Trials (mRCT) (www.controlled-trials.com), ClinicalTrials.gov (www.clinicaltrials.gov) and OpenSIGLE database. Any randomised studies, cohort studies, case-control studies, interrupted time series or sequential case series reporting survival following VPS in HIV-infected individuals will be included. If high-quality homogenous studies exist, meta-analysis will be conducted to determine 1-, 6- and 12-month mortality with comparison made between underlying aetiologies of hydrocephalus. Discussion and conclusion This study will generate a comprehensive review of VPS in HIV-infected patients for publication. The primary outcome of meta-analysis is 12-month survival. If only low-quality, heterogeneous studies are available, this study will demonstrate this deficiency and will be of value in justifying and aiding the design of future studies. Systematic review registration PROSPERO CRD42016052239 Electronic supplementary material The online version of this article (10.1186/s13643-017-0603-7) contains supplementary material, which is available to authorized users.

OBJECTIVEHydrocephalus is one of the most common brain disorders, yet a reliable assessment of the global burden of disease is lacking. The authors sought a reliable estimate of the prevalence and annual incidence of hydrocephalus worldwide.METHODSThe authors performed a systematic literature review and meta-analysis to estimate the incidence of congenital hydrocephalus by WHO region and World Bank income level using the MEDLINE/PubMed and Cochrane Database of Systematic Reviews databases. A global estimate of pediatric hydrocephalus was obtained by adding acquired forms of childhood hydrocephalus to the baseline congenital figures using neural tube defect (NTD) registry data and known proportions of posthemorrhagic and postinfectious cases. Adult forms of hydrocephalus were also examined qualitatively.RESULTSSeventy-eight articles were included from the systematic review, representative of all WHO regions and each income level. The pooled incidence of congenital hydrocephalus was highest in Africa and Latin America (145 and 316 per 100,000 births, respectively) and lowest in the United States/Canada (68 per 100,000 births) (p for interaction < 0.1). The incidence was higher in low- and middle-income countries (123 per 100,000 births; 95% CI 98–152 births) than in high-income countries (79 per 100,000 births; 95% CI 68–90 births) (p for interaction < 0.01). While likely representing an underestimate, this model predicts that each year, nearly 400,000 new cases of pediatric hydrocephalus will develop worldwide. The greatest burden of disease falls on the African, Latin American, and Southeast Asian regions, accounting for three-quarters of the total volume of new cases. The high crude birth rate, greater proportion of patients with postinfectious etiology, and higher incidence of NTDs all contribute to a case volume in low- and middle-income countries that outweighs that in high-income countries by more than 20-fold. Global estimates of adult and other forms of acquired hydrocephalus are lacking.CONCLUSIONSFor the first time in a global model, the annual incidence of pediatric hydrocephalus is estimated. Low- and middle-income countries incur the greatest burden of disease, particularly those within the African and Latin American regions. Reliable incidence and burden figures for adult forms of hydrocephalus are absent in the literature and warrant specific investigation. A global effort to address hydrocephalus in regions with the greatest demand is imperative to reduce disease incidence, morbidity, mortality, and disparities of access to treatment.

Tuberculous (TB) hydrocephalus is in many ways unique among the various causes of hydrocephalus. It is an ancient infection that has plagued patients and their clinicians for millennia. Yet in the twenty-first century, mortality still remains high, survivors are often left with significant disabilities, and the management of TB hydrocephalus remains nonstandardized across the world. Each center has its own preference for interventions that range from medical treatment, temporary ventricular drainage, endoscopy, shunt surgery, and various combinations of each. Similarly, the patient selection criteria for each of these interventions are variably applied. In this chapter we discuss the key pathophysiological aspects of the disease and how these may influence the way we select the best way to treat our patients from the various protocols described.

Effective treatment of children with medulloblastoma requires a functioning multi-disciplinary team with adequate neurosurgical, neuroradiological, pathological, radiotherapy and chemotherapy facilities and personnel. In addition the treating centre should have the capacity to effectively screen and manage any tumour and treatment-associated complications. These requirements have made it difficult for many low and middle-income countries (LMIC) centres to offer curative treatment. This article provides management recommendations for children with standard-risk medulloblastoma (localised tumours in children over the age of 3-5 years) according to the level of facilities available.

AIM Information regarding the selection procedure for selective dorsal rhizotomy (SDR) in children with spastic cerebral palsy (CP) is scarce. Therefore, the aim of this study was to summarize the selection criteria for SDR in children with spastic CP. METHOD A systematic review was carried out using the following databases: MEDLINE, CINAHL, EMBASE, PEDro, and the Cochrane Library. Additional studies were identified in the reference lists. Search terms included 'selective dorsal rhizotomy', 'functional posterior rhizotomy', 'selective posterior rhizotomy', and 'cerebral palsy'. Studies were selected if they studied mainly children (

Purpose: The aim of this study was to investigate the relationship between optic nerve sheath diameter (ONSD) measurement and invasively measured intracranial pressure (ICP) in children. Methods: ONSD measurement was performed prior to invasive measurement of ICP. The mean binocular ONSD measurement was compared to the ICP reading. Physiological variables including systolic blood pressure (SBP), diastolic blood pressure (DBP), mean arterial pressure (MAP), pulse rate, temperature, respiratory rate and end tidal carbon dioxide (ETCO2) level were recorded at the time of ONSD measurement. Diagnostic accuracy analysis was performed at various ICP thresholds and repeatability, intra- and inter-observer variability, correlation between measurements in different imaging planes as well the relationship over the entire patient cohort were examined in part I of this study. Results: Data from 174 patients were analysed. Repeatability and intra-observer variability were excellent (α = 0.97–0.99). Testing for inter-observer variability revealed good correlation (r = 0.89, p

ince the first recorded use of the “ultrasonoscope” for localizing subcortical intracerebral neoplasms in postmorS tem specimens in 1950 (4), technological advances in ultrasound have seen a transformation from poor-quality, A-mode wave forms to superb-quality, real-time images in 2 (2D) and 3 (3D) dimensions. As a result, intraoperative ultrasonography (IOUS) has undergone remarkable development, particularly in the last 2 decades.

Neural tube defects (NTDs) are one of the greatest causes of childhood mortality and disability-adjusted life years worldwide. Global prevalence at birth is approximately 18.6 per 10,000 live births, with more than 300,000 infants with NTDs born every year. Substantial strides have been made in understanding the genetics, pathophysiology, and surgical treatment of NTDs, yet the natural history remains one of high morbidity and profound impairment of quality of life. Direct and indirect costs of care are enormous, which ensures profound inequities and disparities in the burden of disease in countries of low and moderate resources. All indices of disease burden are higher for NTDs in developing countries. The great tragedy is that the majority of NTDs can be prevented with folate fortification of commercially produced food. Unequivocal evidence of the effectiveness of folate to reduce the incidence of NTDs has existed for more than 25 years. Yet, the most comprehensive surveys of effectiveness of implementation strategies show that more than 100 countries fail to fortify, and consequently only 13% of folate-preventable spina bifida is actually prevented. Neurosurgeons harbor a disproportionate, central, and fundamental role in the management of NTDs and enjoy high standing in society. No organized group in medicine can speak as authoritatively or convincingly. As a result, neurosurgeons and organized neurosurgery harbor disproportionate potential to advocate for more comprehensive folate fortification, and thereby prevent the most common and severe birth defect to impact the human nervous system. Assertive, proactive, informed advocacy for folate fortification should be a central and integral part of the neurosurgical approach to NTDs. Only by making the prevention of dysraphism a priority can we best address the inequities often observed worldwide.

Click on the link to view.S Afr Med J 2018;108(8):607

Object Cerebral pressure autoregulation is an important neuroprotective mechanism that stabilizes cerebral blood flow when blood pressure (BP) changes. In this study the authors examined the association between autoregulation and clinical factors, BP, intracranial pressure (ICP), brain tissue oxygen tension (PbtO2), and outcome after pediatric severe traumatic brain injury (TBI). In particular we examined how the status of autoregulation influenced the effect of BP changes on ICP and PbtO2. Methods In this prospective observational study, 52 autoregulation tests were performed in 24 patients with severe TBI. The patients had a mean age of 6.3 ± 3.2 years, and a postresuscitation Glasgow Coma Scale score of 6 (range 3–8). All patients underwent continuous ICP and PbtO2 monitoring, and transcranial Doppler ultrasonography was used to examine the autoregulatory index (ARI) based on blood flow velocity of the middle cerebral artery after increasing mean arterial pressure by 20% of the baseline value. Impaired autoregulation was defined as an ARI < 0.4 and intact autoregulation as an ARI ≥ 0.4. The relationships between autoregulation (measured as both a continuous and dichotomous variable), outcome, and clinical and physiological variables were examined using multiple logistic regression analysis. Results Autoregulation was impaired (ARI < 0.4) in 29% of patients (7 patients). The initial Glasgow Coma Scale score was significantly associated with the ARI (p = 0.02, r = 0.32) but no other clinical factors were associated with autoregulation status. Baseline values at the time of testing for ICP, PbtO2, the ratio of PbtO2/PaO2, mean arterial pressure, and middle cerebral artery blood flow velocity were similar in the patients with impaired or intact autoregulation. There was an inverse relationship between ARI (continuous and dichotomous) with a change in ICP (continuous ARI, p = 0.005; dichotomous ARI, p = 0.02); that is, ICP increased with the BP increase when ARI was low (weak autoregulation). The ARI (continuous and dichotomous) was also inversely associated with a change in PbtO2 (continuous ARI, p = 0.002; dichotomous ARI, p = 0.02). The PbtO2 increased when BP was increased in most patients, even when the ARI was relatively high (stronger autoregulation), but the magnitude of this response was still associated with the ARI. There was no relationship between the ARI and outcome. Conclusions These data demonstrate the influence of the strength of autoregulation on the response of ICP and PbtO2 to BP changes and the variability of this response between individuals. The findings suggest that autoregulation testing may assist clinical decision-making in pediatric severe TBI and help better define optimal BP or cerebral perfusion pressure targets for individual patients.

OBJECTIVE Most physicians rely on conventional treatment targets for intracranial pressure, cerebral perfusion pressure, systemic oxygenation, and hemoglobin to direct management of traumatic brain injury (TBI) in children. In this study, we used brain tissue oxygen tension (PbtO2) monitoring to examine the association between PbtO2 values and outcome in pediatric severe TBI and to determine the incidence of compromised PbtO2 in patients for whom acceptable treatment targets had been achieved. METHODS In this prospective observational study, 26 children with severe TBI and a median postresuscitation Glasgow Coma Scale score of 5 were managed with continuous PbtO2 monitoring. The relationships between outcome and the 6-hour period of lowest PbtO2 values and the length of time that PbtO2 was less than 20, 15, 10, and 5 mmHg were examined. The incidence of reduced PbtO2 for each threshold was evaluated where the following targets were met: intracranial pressure less than 20 mmHg, cerebral perfusion pressure greater than 50 mmHg, arterial oxygen tension greater than 60 mmHg (and peripheral oxygen saturation > 90%), and hemoglobin greater than 8 g/dl. RESULTS There was a significant association between poor outcome and the 6-hour period of lowest PbtO2 and length of time that PbtO2 was less than 15 and 10 mmHg. Multiple logistic regression analysis showed that low PbtO2 had an independent association with poor outcome. Despite achieving the management targets described above, 80% of patients experienced one or more episodes of compromised PbtO2 (< 20 mmHg), and almost one-third experienced episodes of brain hypoxia (PbtO2 < 10 mmHg). CONCLUSION Reduced PbtO2 is associated with poor outcome in pediatric severe TBI. In addition, many patients experience episodes of compromised PbtO2 despite achieving acceptable treatment targets.

OBJECTIVE: Tuberculous meningitis (TBM) is a massive global problem. The mortality and morbidity associated with the severe form of the disease are exceptionally high. Even when increased intracranial pressure is treated and full conventional therapy is commenced, cerebral ischemia can develop and is associated with a particularly poor prognosis. We sought to evaluate our experience with two patients with severe TBM and cerebral oxygenation monitoring. DESIGN: Case report. SETTING: Red Cross Children's Hospital, Cape Town. PATIENTS: Two comatose patients with TBM. INTERVENTIONS: Targeted interventions against low cerebral oxygenation in one patient. MEASUREMENTS AND MAIN RESULTS: Cerebral tissue oxygenation (Ptio2) was measured. In both patients, Ptio2 monitoring demonstrated delayed cerebral ischemia despite the institution of full conventional therapy and the control of intracranial pressure. These data confirm that the vascular involvement in TBM is potentially progressive and that failure to diagnose infarction initially is not merely due to a delay in the radiologic appearance. The first patient developed extensive infarction, consistent with Ptio2 readings, and subsequently died after treatment withdrawal. Intervention in the second patient successfully reversed a precipitous decline of the Ptio2 readings and may have prevented infarction in this patient. CONCLUSIONS: The development of delayed cerebral ischemia in TBM despite treatment is confirmed in these two patients. The reversal of a decline in Ptio2 readings suggests a possible benefit for cerebral oxygenation monitoring in selected patients with severe TBM.

Object Selective dorsal rhizotomy (SDR) has been widely performed for the reduction of spasticity in patients with cerebral palsy during the past 2 decades. The objective of this study was to determine whether the surgery has yielded long-term functional benefits for these patients. Methods The authors present results from a prospective 20-year follow-up study of locomotor function in 13 patients who underwent an SDR in 1985. For comparison, we also present gait data for 48 age-matched healthy controls (12 at each of 4 time points). Patients were studied preoperatively and then at 1, 3, 10, and 20 years after surgery. Study participants were recorded in the sagittal plane while walking using a digital video camera, and 6 standard gait parameters were measured. Results In this group of patients 20 years after surgery, knee range of motion (ROM) was on average 12° greater than preoperative values (p < 0.001). Hip ROM before surgery was no different from that in the healthy control group. This parameter increased markedly immediately after surgery (p < 0.001) but had returned to normal after 20 years. The knee and hip midrange values—a measure of the degree of “collapse” due to muscle weakness after surgery—had returned to preoperative levels after 20 years, although they were respectively 11 and 8° greater than those in healthy controls. Both temporal-distance parameters (dimensionless cadence and dimensionless step length) were significantly greater at 20 years than preoperative values (cadence, p = 0.003; step length, p = 0.02), leading to improved walking speed. Conclusions Twenty years after undergoing SDR, our patients showed improved locomotor function compared with their preoperative status.

Given the large number of cerebral palsy patients who have undergone selective dorsal rhizotomy in the past two decades, it is clearly imperative that the clinical community be provided with objective and compelling evidence of the long-term sequelae of the procedure.In the early 1980s, Peacock in Cape Town shifted the site of the rhizotomy from the conus medullaris to the cauda equina, and in the past 25 years, more than 200 children have been operated on. We have studied the incidence of spinal deformities after multiple-level laminectomy and recorded a 20% incidence of isthmic spondylolysis or grade-I spondylolisthesis. We have also conducted a long-term prospective gait analysis study on a cohort of 14 ambulatory patients who were operated on in 1985.Ten years after surgery, our patients had increased ranges of motion that were within normal limits. Step length was significantly improved, although cadence was unchanged postoperatively and was significantly less than normal age-matched control subjects.We have recently tracked down all 14 patients from the original cohort and are currently completing a 20-year prospective follow-up analysis of their neuromuscular function and gait. Our preliminary data suggest that selective dorsal rhizotomy is not only an effective method for alleviating spasticity but it also leads to long-term functional benefits.

Aim Tuberculous meningitis (TBM) is a lethal and commonly occurring form of extra-pulmonary tuberculosis in children, often complicated by hydrocephalus which worsens outcome. Despite high mortality and morbidity, little data on the impact on neurodevelopment exists. We examined the clinical characteristics, and clinical and neurodevelopmental outcomes of TBM and hydrocephalus. Method Demographic and clinical data (laboratory and radiological findings) were prospectively collected on children treated for probable and definite TBM with hydrocephalus. At 6 months, clinical outcome was assessed using the Paediatric Cerebral Performance Category Scale and neurodevelopmental outcome was assessed with the Griffiths Mental Development Scale – Extended Version. Results Forty-four patients (median age 3y 3mo, range 3mo–13y 1mo, [SD 3y 5mo]) were enrolled. The mortality rate was 16%, three patients (6.8%) were in a persistent vegetative state, two were severely disabled (4.5%), and 11 (25%) suffered mild–moderate disability. All cases demonstrated neurodevelopmental deficits relative to controls. Multiple or large infarcts were prognostic of poor outcome. Interpretation Neurological and neurodevelopmental deficits are common after paediatric TBM with hydrocephalus, and appear to be related to ongoing cerebral ischaemia and consequent infarction. The impact of TBM on these children is multidimensional and presents short- and long-term challenges.

Introduction Prolactinomas in childhood and adolescence are rare. However, in male patients in particular they may become extremely large and invasive, resulting in visual impairment without necessarily producing endocrine symptoms. Case reports We report on two adolescent males who presented with deteriorating vision over a long period of time and who had optic atrophy on examination. Magnetic resonance imaging identified tumours with extensive anterior skull base invasion and suprasellar extension. Prolactin levels in both patients were markedly elevated, and a diagnosis of prolactinoma was made. Bromocriptine treatment was started resulting in lowered prolactin levels, improved vision and tumour shrinkage on imaging. Conclusion A male child or adolescent presenting with diminished vision and found to have suprasellar or anterior skull base tumour should have their prolactin levels checked to rule out a prolactinoma, which can be successfully managed with medical therapy.

Forty-eight children with neurofibromatosis 1 presenting between 2000 and 2004 were reviewed for their clinical phenotype, and data were compared with published reports. The median age at presentation was 4 years (range 10 days to 12 years). The male to female ratio was similar (22 male:26 female). There were frequencies of café au lait spots, axillary freckling, Lisch nodules, and new mutations comparable to those cited in the literature. Fewer patients had neurofibromas (4%), but more patients had plexiform neurofibromas of the head and neck (16%). Three patients of the 22 who had neuroimaging had optic gliomas (14%). The most consistent disability, with maximum impact, related to the patient's cognitive level of functioning. School problems, defined as learning and behavioral problems observed in the classroom, were reported in 70% of school-aged children ( n = 21), compared with international figures of 29.8% to 45%. This high prevalence has reinforced the clinic service policy of formal neuropsychology assessments in all children with reported school problems. In addition, earlier referral of children to the service (preschool n = 18) has enabled formal developmental assessments and planning of specific educational placement to optimize learning. This is the first description of the neurofibromatosis 1 phenotype from the African continent. The multidisciplinary approach to management has proved beneficial in the South African context. The combined clinic has resulted in a holistic approach to patient care, early detection of pathology, consistent therapies across the specialties, and better patient attendance and compliance. ( J Child Neurol 2006;21:63—70).