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Object. The aim of this prospective study was to investigate the causes of hydrocephalus in Uganda, the efficacy of endoscopic third ventriculostomy (ETV) in this environment, and whether existing parameters could be used to guide patient selection. Methods. Three hundred consecutive children, 81.3% of whom were younger than 1 year of age, underwent ventriculoscopy preceding ETV as an initial treatment for hydrocephalus. In 179 patients (60%) the hydrocephalus was caused by a cerebrospinal fluid infection; in 76% of patients the infection had occurred in the 1st month of life. In 229 patients (76.3%) ETV was performed; 2% of patients were lost to follow up after less than 1 month and the surgical mortality rate was 1.8%. The first ETV was successful in 115 patients (52%); the mean follow-up period was 15.2 months. The mean time to repeated operation following a failed ETV was 1.5 months. Sixty-five patients underwent a second endoscopy; 37 underwent a second ETV, of which 14 procedures (38%) were successful (mean follow-up period 12.25 months). The overall success rate for ETV was 59%. Among patients older than 1 year of age, the procedure was successful in 22 (81%) of 27 with postinfectious hydrocephalus (PIHC) and 18 (90%) of 20 with nonpostinfectious hydrocephalus (NPIHC). The success rate of ETV among those patients younger than 1 year of age was 59% (60 of 101) for patients suffering from PIHC and 40% (21 of 52) for those suffering from NPIHC. Age correlated with success for NPIHC (p = 0.0002) and PIHC (p = 0.0421). The success rate of the surgery for pa tients with myelomeningocele and hydrocephalus who were younger than 1 year of age was 40% (eight of 20). The success rate of the surgery for PIHC in infants younger than 1 year of age was 70% (44 of 63) among patients with aqueductal obstruction but 45% (14 of 31) among patients with aqueductal patency (p = 0.0254). Fourth ventricular size as demonstrated on cranial ultrasonography or computerized tomography scanning predicted whether the aqueduct was patent (p = 0.0001). Conclusions. Infection is the most common cause of hydrocephalus in Uganda. In all children older than 1 year of age and in those younger than 1 year of age with PICH and aqueductal obstruction, which was reliably predicted by cranial ultrasonography, ETV was effective.

A 2-month-old male infant presented with macrocephaly and signs of raised intracranial pressure. On physical examination, anterior fontanelle was full, bulging, and tense with increased head circumference. An MRI revealed interhemispheric multiloculated cyst with callosal dysgenesis (figure, A–C). The patient underwent right endoscopic third ventriculostomy with choroid plexus cauterization and endoscopic fenestration of the cyst (figure, D and E). Interhemispheric multiloculated cysts with callosal dysgenesis are relatively rare lesions.1 Neuroendoscopic management is a minimally invasive method creating communication among the cyst, ventricular system, and subarachnoid space thereby avoiding permanent shunting and microsurgery.2

Background: ETV/CPC has become an increasingly common technique for the treatment of infant hydrocephalus. Both flexible and rigid neuroendoscopy can be used, with little empirical evidence directly comparing the two. We, therefore, used a propensity-matched cohort and survival analysis to assess the comparative efficacy of flexible and rigid neuroendoscopy. Methods: Individual data were collected through retrospective review of infants < 2 years of age, treated at one of 2 hospitals: 1) Boston Children’s Hospital, exclusively utilizing flexible neuroendoscopy, and 2) Nicklaus Children’s Hospital, exclusively utilizing rigid neuroendoscopy. Patient characteristics and post-operative outcome were assessed. A propensity score (PS) model was developed to balance patient characteristics in the case mix. Results: A PS model was developed with 5 independent variables: chronological age, sex, hydrocephalus etiology, prior CSF diversion, and prepontine scarring. PS analysis revealed that compared to flexible neuroendoscopy, rigid neuroendoscopy had an ETV/CPC failure OR of 1.43 and 1.31 respectively, compared to unadjusted OR of 2.40. Furthermore, in a Cox regression analysis controlled by propensity score, rigid neuroendoscopy had a HR of 1.10, compared to unadjusted HR of 1.61. Conclusions: Much of the difference in ETV/CPC outcome between endoscopy types is attributed to the case mix. An observational study or randomized controlled trial is required to provide evidence-based guidelines.

Background: Pediatric hydrocephalus is one of the most common neurosurgical conditions and is a major contributor to the global burden of surgically treatable diseases. Methods: The authors conducted a literature review around the topic of pediatric hydrocephalus in the context of global surgery, the unique challenges to creating access to care in low-income countries, and current international efforts to address the problem. Results: Developing countries face the greatest burden of pediatric hydrocephalus due to high birth rates and greater risk of neonatal infections. This burden is related to more general global health challenges, including malnutrition, infectious diseases, maternal and perinatal risk factors, and education gaps. Unique challenges pertaining to the treatment of hydrocephalus in the developing world include a preponderance of postinfectious hydrocephalus, limited resources, and restricted access to neurosurgical care. In the 21st century, several organizations have established programs that provide hydrocephalus treatment and neurosurgical training in Africa, Central and South America, Haiti, and Southeast Asia. These international efforts have employed various models to achieve the goals of providing safe, sustainable, and cost-effective treatment. Conclusions: Broader commitment from the pediatric neurosurgery community, increased funding, public education, surgeon training, and ongoing surgical innovation will be needed to meaningfully address the global burden of untreated hydrocephalus.

Object Combined endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) enhances the likelihood of shunt freedom over ETV alone, and thus avoidance of shunt-related morbidity, in hydrocephalic infants. To date, virtually all published reports describe experiences in Africa, thus hampering generalization to other parts of the world. Here, the authors report the first North American prospective series of this combined approach to treat hydrocephalus of various etiologies in infants. Methods A prospective series of 50 boys and 41 girls (mean and median ages 4.7 and 3.2 months, respectively) with hydrocephalus underwent ETV/CPC performed by the senior author at Boston Children's Hospital from August 2009 through March 2014. Success data were analyzed using the Kaplan-Meier method and Cox proportional hazards model. Results The 91 patients treated included those with aqueductal stenosis (23), myelomeningocele (23), posthemorrhagic hydrocephalus (25), Dandy-Walker complex (6), post-infectious hydrocephalus (6), and other conditions (8). Using Kaplan-Meier survival analysis, 57% of patients required no further hydrocephalus treatment at 1 year. Moreover, 65% remained shunt free to the limit of available follow-up (maximum roughly 4 years). A Cox proportional hazards model identified the following independent predictors of ETV/CPC failure: post-infectious etiology, age at treatment younger than 6 months, prepontine cistern scarring, and prior CSF diversion. Of patients with at least 6 months of follow-up, the overall ETV/CPC success at 6 months (59%) exceeded that predicted by the ETV Success Score (45%). Complications included 1 CSF leak and 1 transient syndrome of inappropriate antidiuretic hormone secretion, and there were no deaths. Conclusions ETV/CPC is an effective, safe, and durable treatment for infant hydrocephalus in a North American population, with 1-year success rates similar to those reported in Africa and equivalent to those for primary shunt placement in North America. These findings underscore the need for prospective multicenter studies of the outcomes, quality of life, and economic impact of the procedure compared with primary shunt insertion.

Several surgical training simulators have been created to improve the learning curve of residents in neurosurgery and plastic surgery. Laboratory training is fundamental for acquiring familiarity with the techniques of surgery and the skill in handling instruments. The aim of this study is to present a novel simulator for training in the technique of craniosynostectomy, specifically for the scaphocephaly type. This realistic simulator was built with a synthetic thermo-retractile and thermo-sensible rubber which, when combined with different polymers, produces more than 30 different formulas. These formulas present textures, consistencies, and mechanical resistance similar to many human tissues. Fiberglass molds in the shape of the skull constitute the basic structure of the craniosynostectomy training module. It has been possible to perform computerized tomography images due to the radiopacity of this simulator and to compare the pre- and postoperative images. The authors present a training model to practice the biparietal remodeling used in scaphocephaly correction. All aspects of the procedure are simulated: the skin incision, the subcutaneous and subperiosteal dissection, the osteotomies, and finally, the skull remodeling with absorbable microplates. The presence of superior sagittal sinus can simulate emergency situations with bleeding. The authors conclude that this training model can represent a fairly useful method to accustom trainees to the required surgical techniques and simulates well the steps of standard surgery for scaphocephaly. This training provides an alternative to the use of human cadavers and animal models. Furthermore, it can represent the anatomical alteration precisely as well as intraoperative emergency situations.

It is increasingly apparent that the standards for surgical training are shifting from time-based to criterion-based parameters that emphasize obtaining and maintaining competencies [24]. The formation of a surgeon demands significant dedication and effort, in addition to time [54]. Current, wellestablished methods of surgical training are being challenged as the environment becomes increasingly competitive and litigious with greater scrutiny of patient outcomes [14, 17, 40, 44, 50]. In order to increase patient safety and improve treatment outcomes, several strategies such as problem-based learning and objective structured clinical examinations have promoted the development of new curricula in surgical education [13, 15, 35, 51, 52]. Some of these changes have been driven by events in the 1980s and 1990s such as medical misconduct and overworked, unsupervised resident staff that contributed to patient morbidity and mortality. This also coincided with a growing medical malpractice crisis. As a result, regulatory bodies began to initiate new standards of work hour restrictions and supervision for residents in training. The New York Health Code of 1989 compiled regulations restricting resident work hours (80 h per week) and one day free a week and placed limits on the number of calls [24, 32]. Concerns arose about the long-established methods of training surgical residents, and solutions were sought to reduce preventable errors and perioperative complications [24, 9]. The airline industry, with the development of flight simulators and pilot coaching methods, proved to be an excellent precedent for innovation in surgical education. Many surgical educators believe such methods are keys to accelerating the acquisition of fundamental skills and the rate of performance improvement among surgical residents. A Yale University study demonstrated that criterion-based simulator training decreased operating time by 30 % and operative errors by 85 % [47, 48]. Neurosurgical trainees in particular face great challenges in learning to plan and perform increasingly complex procedures in which there is little room for error [10]. The educator’s task becomes ever more daunting as the number and complexity of neurosurgical procedures continue to increase in parallel with technological developments such as minimally invasive spine surgery and instrumentation, interventional neuroangiography, image-guided navigation, and endoscopic surgery. The necessity of innovative surgical curriculum development that incorporates safe learning environments and objective skill assessments is thus obvious and needs to be led by trained surgical educators [5]. Adjuvant, non-clinical, surgical training can be grouped into four broad categories

Object Antibiotic-impregnated shunts have yet to find widespread use in the developing world, largely due to cost. Given potential differences in the microbial spectrum, their effectiveness in preventing shunt infection for populations in low-income countries may differ and has not been demonstrated. This study is the first to compare the efficacy of a Bactiseal shunt system with a non–antibiotic-impregnated system in a developing country. Methods The Bactiseal Universal Shunt (BUS) was placed in 80 consecutive Ugandan children who required a shunt. In this retrospective cohort study, the outcome for that group was compared with the outcome for the immediately preceding 80 consecutive children in whom a Chhabra shunt had been placed. The primary end points were shunt failure, shunt infection, and death. Shunt survival was analyzed using the Kaplan-Meier method. Significance of differences between groups was tested using the log-rank test, chi-square analysis, Fisher's exact test, and t-test. Results There was no difference between groups in regard to age, sex, or etiology of hydrocephalus. Mean follow-up for cases of nonfailure was 7.6 months (median 7.8 months, interquartile range 6.5–9.5 months). There was no significant difference between groups for any end point. The BUS group had fewer infections (4 vs 11), but the difference was not significant (p = 0.086, log-rank test). Gram-positive cocci were the most common culturable pathogens in the Chhabra group, while the only positive culture in the BUS group was a gram-negative rod. Conclusions These results provide equipoise for a randomized controlled trial in the same population and this has been initiated. It is possible that the observed trends may become significant in a larger study. The more complex task will involve determining not only the efficacy, but also the cost-effectiveness of using antibiotic-impregnated shunt components in limited-resource settings.

The ability to perform a ‘selective’ dorsal rhizotomy has been challenged. EMG responses are inconsistent and often do not represent reflex responses. We perform nonselective partial dorsal rhizotomy (NSPDR) when reflex response is not evident. Ten children undergoing primarily NSPDR were evaluated preoperatively and postoperatively with the Modified Ashworth Scale, gait lab analyses gross motor function measure and the NSPDR was performed by nonselectively sectioning 50–75% of the dorsal roots not demonstrating a reflex response. Standard selective rhizotomy was performed in the remainder. Only 17 of 106 (16%) dorsal roots demonstrated reflex responses. The results reported in this study demonstrate a benefit to patients undergoing primarily NSPDR which is similar to that reported for patients in whom a selective procedure was intended.

Background. It is reasonable to propose that competence is a multifaceted characteristic defined in part by some minimum level of knowledge and skill. In this study we examined the relationship between surgical faculty's judgment of clinical competence, as measured by a surgical resident objective structured clinical examination (OSCE), and the residents' objective performance on the skills being tested. Methods. Fifty-six general surgery residents at all levels of training participated in a 30-station OSCE. At the completion of each station, the faculty proctor made several overall judgments regarding each resident's performance, including a global judgment of competent or not competent. The competence judgment was applied to the objective percentage performance score in three different ways to construct methods for determining competence based solely upon this objective percentage score. Results. The average mean competent score (MCS) across the stations was 61%, and the average mean noncompetent score (MNCS) was 38%. The difference between MCS and MNCS for each station was very consistent. Upper threshold scores above which a judgment of competent was always made, and lower threshold scores below which a judgment of noncompetent was always made were observed. Overall, the average mean and threshold scores for competent and noncompetent groups were remarkably similar. For performance scores in the range between the threshold competent and noncompetent scores at each station, measures other than objective performance on the skills being evaluated determined the judgment of competent or not competent. Conclusions. Empirically determined minimum acceptable standards for objective performance in clinical skills and knowledge appeared to have been subconsciously applied to the competence judgment by the faculty evaluators in this study. Other factors appeared to have become determinate when the objective performance score fell within a range of uncertainty.

Pygopagus twins were born with a unique spectrum of anomalies including a conjoined distal spinal cord, single kidney (in twin A), single rectum (in twin A), single vagina (in twin B), and severe central nervous system anomalies in twin B that precluded her independent survival. Separation at 10 weeks of age was tailored toward Twin A's survival. This report discusses the surgical modifications necessary in view of the unique anatomy, including salvaging the distal spinal cord and vagina for twin A.

This study reviews the first operative series of pediatric brain tumors from Uganda, the largest series from Sub-Saharan Africa, and explores the challenges to progress in pediatric neuro-oncology in the region. This is a retrospective operative series of brain tumors in 172 children at Cure Children’s Hospital of Uganda over 10 years. Demographics, clinical presentation, lesion location, histopathology, operative management, and outcome were investigated. Survival was assessed using Kaplan–Meier method. Log-rank test and p value with Bonferroni correction were used to determine significance of survival differences. There were 103 males (59.9 %) and 69 females (40.1 %; mean age at diagnosis 6.5 years with 29 %

The traditional bulk flow model of communicating hydrocephalus does not support treatment by endoscopic third ventriculostomy (ETV), but successful treatment is reported in adults. This study is the first to report long-term success of ETV +/- choroid plexus cauterization (CPC) for infants with congenital idiopathic hydrocephalus (CIH). The results are interpreted in the context of current models for hydrocephalus.The CURE Children's Hospital of Uganda prospective database was reviewed for the years 2001-2006 to identify children24 months of age meeting the criteria for CIH who underwent ETV with or without bilateral CPC. Kaplan-Meier method was used to assess treatment success survival. Gehan-Breslow-Wilcoxin and logrank tests were used to determine significance of survival differences. Fisher's exact test was used to determine the significance of differences between groups.Sixty-four infants (mean/median age, 6.1/5.0 months) met the inclusion criteria. Sixteen consecutive patients were treated by ETV alone, and the subsequent 48 by ETV/CPC (mean/median follow-up 34.4/36.0 months). ETV was successful in 20 % and ETV/CPC in 72.4 % at 4 years (p 0.0002, logrank test; p = 0.0006, Gehan-Breslow-Wilcoxin; hazard ratio 6.9, 95 % CI 2.5-19.3).ETV/CPC was significantly more successful than ETV alone in treating CIH. The primary effect of ETV, as a pulsation absorber, and of CPC, as a pulsation reducer, may be to abate the net force of intraventricular pulsations that produce ventricular expansion. ETV alone may be less successful for infants because of greater brain compliance. ETV/CPC should be considered an effective primary treatment option.

Neonatal sepsis in the developing world is incompletely characterized. We seek to characterize the microbial spectrum involved in sepsis and determine the role of maternal transmission by comparing organisms that can be cultured from septic newborn infants and their mothers. From 80 consecutive mother-infant pairs meeting clinical criteria for neonatal sepsis, we collected infant blood and spinal fluid, and maternal blood and vaginal specimens. Identifiable bacteria were recovered from the blood in 32.5% of infants, and from 2.5% of cerebrospinal fluid cultures, for a total of 35% recoverable putative causative agents. Bacteria recovered from vaginal specimens were not concordant with those recovered from infants. Similarly there was no concordance of bacteria recovered from blood and cerebrospinal fluid. We conclude that relying on traditional bacterial culture techniques does not adequately delineate the role of maternal versus environmental sources of neonatal sepsis in this setting. More sensitive molecular approaches will be needed to properly characterize the maternal and environmental microbial community involved in neonatal sepsis in such developing countries.

The progressive neurologic dysfunction caused by occult spinal dysraphism can be prevented with early clinical recognition, radiographic diagnosis, and neurosurgical treatment. However, detection of occult spinal dysraphism in the infant is difficult because neurologic symptoms often are not apparent until the child becomes ambulatory. Occult spinal dysraphism, however, can be suspected in the asymptomatic neonate when cutaneous stigmata, such as hemangiomas, hairy patches, deep and/or eccentric dimples, or subcutaneous masses are seen over the lumbosacral spine. Because of the serious, often irreversible, sequelae of a delayed diagnosis, spinal sonography of high-risk infants with midline, lumbosacral, cutaneous stigmata should be considered as an effective, noninvasive screening method.

Hydrocephalus is one of the most common brain disorders in children throughout the world. The majority of infant hydrocephalus cases in East Africa appear to be postinfectious, related to preceding neonatal infections, and are thus preventable if the microbial origins and routes of infection can be characterized. In prior microbiological work, the authors noted evidence of seasonality in postinfectious hydrocephalus (PIH) cases.The geographical address of 696 consecutive children with PIH who were treated over 6 years was fused with satellite rainfall data for the same time period. A comprehensive time series and spatiotemporal analysis of cases and rainfall was performed.Four infection-onset peaks were found to straddle the twice-yearly rainy season peaks, demonstrating that the infections occurred at intermediate levels of rainfall.The findings in this study reveal a previously unknown link between climate and a neurosurgical condition. Satellite-derived rainfall dynamics are an important factor in driving the infections that lead to PIH. Given prior microbial analysis, these findings point to the importance of environmental factors with respect to preventing the newborn infections that lead to PIH.

Myelomeningocele is the most common neural tube defect. Repair typically involves deep closure with regional muscle flaps (e.g. latissimus dorsi, gluteus maximus) and skin closure with rotation, bipedicle, or rhomboid flaps. We describe the reconstruction of large myelomeningocele defects using (1) local fascial turnover flaps with or without paraspinous muscle flaps for deep coverage of the dural repair followed by (2) linear, midline skin closure.

Neonatal infection is the most common cause of infant hydrocephalus in Uganda. Postinfectious hydrocephalus (PIH) is often accompanied by primary brain injury from the original infection. Since 2001, ETV (with or without choroid plexus cauterization) has been our primary treatment for PIH. The long-term outcome in these children is unknown.We studied the 5-year outcome in a cohort of 149 infants treated for PIH from 2001 to 2005 and who lived in 4 districts close to the hospital. Survival analysis was performed using the Kaplan-Meier method. Statistical significance was determined using the Fisher, Breslow, and log-rank tests.The patients' mean age at presentation was 9.5 months (median 3.0 months). Eighty-four patients (56.4%) were successfully treated without a shunt. Operative mortality was 1.2% for ETV and 4.4% for shunt placement (p = 0.3). Five-year survival was 72.8% in the non-shunt-treated group and 67.6% in the shunt-treated group, with no difference in survival (log rank p = 0.43, Breslow p = 0.46). Of 43 survivors assessed at 5-11 years, those with shunts had significantly worse functional outcomes (p = 0.003-0.035), probably reflecting treatment selection bias since those with the worst sequelae of ventriculitis were more likely to be treated with shunt placement.Nearly one-third of treated infants died within 5 years, and at least one-third of the survivors were severely disabled. There was no survival advantage for non-shunt-treated patients at 5 years. A randomized trial of endoscopic third ventriculostomy versus shunt placement for treating PIH may be indicated. Public health measures that prevent these infections are urgently needed.

Evidence from the CURE Children's Hospital of Uganda (CCHU) suggests that treatment for hydrocephalus in infants can be effective and sustainable in a developing country. This model has not been broadly supported or implemented due in part to the absence of data on the economic burden of disease or any assessment of the cost and benefit of treatment. The authors used economic modeling to estimate the annual cost and benefit of treating hydrocephalus in infants at CCHU. These results were then extrapolated to the potential economic impact of treating all cases of hydrocephalus in infants in sub-Saharan Africa (SSA).The authors conducted a retrospective review of all children initially treated for hydrocephalus at CCHU via endoscopic third ventriculostomy or shunt placement in 2005. A combination of data and explicit assumptions was used to determine the number of times each procedure was performed, the cost of performing each procedure, the number of disability-adjusted life years (DALYs) averted with neurosurgical intervention, and the economic benefit of the treatment. For CCHU and SSA, the cost per DALY averted and the benefit-cost ratio of 1 year's treatment of hydrocephalus in infants were determined.In 2005, 297 patients (median age 4 months) were treated at CCHU. The total cost of neurosurgical intervention was $350,410, and the cost per DALY averted ranged from $59 to $126. The CCHU's economic benefit to Uganda was estimated to be between $3.1 million and $5.2 million using a human capital approach and $4.6 million-$188 million using a value of a statistical life (VSL) approach. The total economic benefit of treating the conservatively estimated 82,000 annual cases of hydrocephalus in infants in SSA ranged from $930 million to $1.6 billion using a human capital approach and $1.4 billion-$56 billion using a VSL approach. The minimum benefit-cost ratio of treating hydrocephalus in infants was estimated to be 7:1.Untreated hydrocephalus in infants exacts an enormous price from SSA. The results of this study suggest that neurosurgical intervention has a cost/DALY averted comparable to other surgical interventions that have been evaluated, as well as a favorable benefit-cost ratio. The prevention and treatment of hydrocephalus in SSA should be recognized as a major public health priority.

Dandy-Walker complex (DWC) is a continuum of congenital anomalies comprising Dandy-Walker malformation (DWM), Dandy-Walker variant (DWV), Blake pouch cyst, and mega cisterna magna (MCM). Hydrocephalus is variably associated with each of these, and DWC-associated hydrocephalus has mostly been treated by shunting, often with 2-compartment shunting. There are few reports of management by endoscopic third ventriculostomy (ETV). This study is the largest series of DWC or DWM-associated hydrocephalus treated by ETV, and the first report of treatment by combined ETV and choroid plexus cauterization (ETV/CPC) in young infants with this association.A retrospective review of the CURE Children's Hospital of Uganda clinical database between 2004 and 2010 identified 45 patients with DWC confirmed by CT scanning (25 with DWM, 17 with DWV, and 3 with MCM) who were treated for hydrocephalus by ETV/CPC. Three were excluded because of other potential causes of hydrocephalus (2 postinfectious and 1 posthemorrhagic).The median age at treatment was 5 months (88% of patients were younger than 12 months). There was a 2.4:1 male predominance among patients with DWV. An ETV/CPC (ETV only in one) was successful with no further operations in 74% (mean and median follow-up 24.2 and 20 months, respectively [range 6-65 months]). The rate of success was 74% for DWM, 73% for DWV, and 100% for MCM; 95% had an open aqueduct, and none required posterior fossa shunting.Endoscopic treatment of DWC-associated hydrocephalus should be strongly considered as the primary management in place of the historical standard of creating shunt dependence.

The survival of infants treated for myelomeningocele (MM) and hydrocephalus in Uganda is unknown. This study investigated 5-year survival and the factors that influenced death in these patients.All 140 children from 16 contiguous districts in southeastern Uganda presenting to CURE Children's Hospital of Uganda for repair of MM prior to 6 months of age between December 2000 and December 2004 comprised the study cohort. Nine patients died within 1 month (6.4% operative mortality) and were excluded from further analysis. Sixty-seven (51%) required treatment for hydrocephalus. Survival status could not be determined for 3 patients (2%). Circumstances of death were ascertained by hospital record or interview. The Kaplan-Meier method was used for survival analysis. Association between survival and district of origin, age at MM closure, MM lesion level, presence of hydrocephalus, and method of hydrocephalus treatment were investigated.The median follow-up was 86 months. Seventy-three children (56%) were alive at the time of the study, and 81 (63%) had survived more than 5 years. The under-5 mortality rate was 37% (2.5 times greater than the general population). Only 4 deaths appeared directly related to hydrocephalus or MM. There was no significant association between survival and age at MM closure, MM lesion level, presence of hydrocephalus, or its method of treatment. Mortality was lower, approaching that for their unaffected peers, in districts with community-based rehabilitation programs (p = 0.001).Community-based support following surgical interventions for MM and hydrocephalus appears essential to the continued survival of these children in Africa.

Infantile hydrocephalus in East Africa is predominantly postinfectious. The microbial origins remain elusive, since most patients present with postinfectious hydrocephalus after antecedent neonatal sepsis (NS) has resolved.To characterize this syndrome in Ugandan infants, the authors used polymerase chain reaction targeting bacterial 16S ribosomal DNA from CSF to determine if bacterial residua from recent infections were detectable. Bacteria were identified based on the relationship of genetic sequences obtained with reference bacteria in public databases. The authors evaluated samples from patients presenting during dry and rainy seasons and performed environmental sampling in the villages of patients.Bacterial DNA was recovered from 94% of patients. Gram-negative bacteria in the phylum Proteobacteria were the most commonly detected. Within this phylum, Gammaproteobacteria dominated in patients presenting after infections during the rainy season, and Betaproteobacteria was most common following infections during the dry season. Acinetobacter species were identified in the majority of patients admitted after rainy season infection.Postinfectious hydrocephalus in Ugandan infants appears associated with predominantly enteric gram-negative bacteria. These findings highlight the need for linking these cases with antecedent NS to develop more effective treatment and prevention strategies.

Post-hemorrhagic hydrocephalus of prematurity (PHHP) is among the most common causes of infant hydrocephalus in developed nations. This population has a high incidence of shunt failure, infection, and slit ventricle syndrome. Although effective for other etiologies of infant hydrocephalus, the efficacy of combined endoscopic third ventriculostomy and choroid plexus cauterization (ETV/CPC) in PHHP has not been investigated. This pilot study reports the initial experience.Ten patients (four grade III and six grade IV intraventricular hemorrhage) requiring definitive treatment for PHHP underwent ETV/CPC within 6 months of birth. Seven had a prior ventriculo-subgaleal shunt. Mean age at birth was -12.8 weeks, or 25.2 weeks gestation (24-28 weeks), and at surgery was -1.6 weeks (-11 to +11 weeks). Mean weight at surgery was 3.3 (1.0-5.5 kg). Each patient had preoperative magnetic resonance imaging (MRI) with fast imaging employing steady-state acquisition (FIESTA).Four of ten (40%) required no further operations related to hydrocephalus (mean follow-up, 29.7 months). Six required another procedure (five ultimately shunted). Prepontine cistern status correlated with outcome (p = 0.033). Procedures in all infants with unobstructed cisterns were successful but failed in six of seven with cisternal obstruction, with the one success having an alternative lamina terminalis endoscopic third ventriculostomy. Preoperative MRI FIESTA images correlated well with intraoperative assessment of the cistern.Results from this small homogenous cohort suggest cistern status is an important determinant of outcome. FIESTA imaging correlated with endoscopic observation. Preliminary analysis suggests ETV/CPC as an effective treatment for PHHP, but only when the cistern is unscarred. This information should guide patient selection for future study protocols.

Surgery for children in developing nations is challenging. Endoscopic third ventriculostomy (ETV) is an important surgical treatment for childhood hydrocephalus and has been performed in developing nations, but with lower success rates than in developed nations. It is not known if the lower success rate is due to inherent differences in prognostic factors.We analyzed a large cohort of children (≤20 years old) treated with ETV in developed nations (618 patients from Canada, Israel, United Kingdom) and developing nations of sub-Saharan Africa (979 patients treated in Uganda). Risk-adjusted survival analysis was performed.The risk of an intra-operative ETV failure (an aborted procedure) was significantly higher in Uganda regardless of risk adjustment (hazard ratio (HR), 95% confidence interval (CI), 11.00 (6.01 to 19.84) P0.001). After adjustment for patient prognostic factors and technical variation in the procedure (the use of choroid plexus cauterization), there was no difference in the risk of failure for completed ETVs (HR, 95% CI, 1.04 (0.83 to 1.29), P=0.74).Three factors account for all significant differences in ETV failure between Uganda and developed nations: patient prognostic factors, technical variation in the procedure, and intra-operatively aborted cases. Once adjusted for these, the response to completed ETVs of children in Uganda is no different than that of children in developed nations.

The neuroepithelial cells of the mammalian neural tube are thought to give rise to all classes of differentiated neurons and macroglial cells in the adult CNS. In most cases, the regulation and timing of commitment of neuroepithelial cells to specific differentiative pathways are unknown. It has been proposed that in developing spinal cord, the macroglial cells--astrocytes and oligodendrocytes--arise either by the direct transformation of radial glial cells in the developing cord or, alternatively, by the differentiation of distinct precursor cells which migrate to presumptive white matter from the region of the central canal during development. In this study, the timing of oligodendrocyte differentiation in different levels of the spinal cord and the capacity of specific regions of the spinal cord to give rise to oligodendrocytes at various ages was tested in vitro. At embryonic day 14, all complete segments, as well as all ventral regions along the rostral-caudal axis of the spinal cord, have the capacity for oligodendrogenesis. By contrast, dorsal regions of the thoracic and lumbar spinal cord do not develop the capacity for oligodendrogenesis until later in development. The capacity of dorsal rat spinal cord to give rise to oligodendrocytes appears to be associated with the ventral- to-dorsal migration of oligodendrocyte precursors. These observations suggest that commitment to an oligodendrocyte differentiative pathway appears to occur in a distinct population of ventrally located glial precursors in the embryonic rat spinal cord.

Since 1980, 22 patients less than 2 years of age have been treated for intracranial tumors at our institution. The most common presentation was elevated intracranial pressure associated with ventriculomegaly (73%). The diagnosis was made by computed tomography or magnetic resonance imaging and confirmed surgically in every case. In 68% of cases, the tumor involved midline structures in the central nervous system. Tumors were infratentorial in 29% of patients less than 1 year of age and in 60% of those more than 1 year of age. Most tumors were either primitive neuroectodermal tumors (41%, 7 of 9 located in midline posterior fossa structures) or astrocytomas (27%, 5 of 6 located in the hypothalamus, chiasm, or optic nerve). Twenty patients had gross or subtotal tumor resection. Surgical mortality and neurological morbidity were 10% and 5%, respectively. Of 5 patients who underwent a second operation for tumor recurrence, 3 demonstrated a change in the pathological features of the tumor from those of the original diagnosis. Of 10 surgical survivors with malignant tumors, 8 received chemotherapy and 5 radiation therapy. Radiation was not administered before 1 year of age. The overall 1-year survival was 70%, and 2-year survival was 58%. Two-year survival for benign and malignant tumors was not significantly different. We think that the interdisciplinary efforts directed toward the treatment of these tumors has improved survival and that all children should be offered entry to a research protocol and aggressive treatment.

Object Shunt dependence is more dangerous for children in less developed countries. Combining endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) was previously shown to treat hydrocephalus more effectively than ETV alone in infants < 1 year of age. The goal of this prospective study was to evaluate the effectiveness of ETV-CPC as primary treatment of hydrocephalus in infants with myelomeningocele. Methods One hundred fifteen consecutive East African infants with myelomeningocele requiring treatment for hydrocephalus were intended for primary management using ETV-CPC. Patient information was prospectively entered into a database. Outcomes were evaluated by life table analysis. Potential predictors for treatment failure were evaluated using multivariate logistic regression. Results Ninety-three patients had a completed ETV-CPC with > 1 month of follow-up. The ETV-CPC procedure was successful in 71 patients (76%), with a mean and median follow-up of 19.0 months. Treatment failures occurred before 6 months in 86% of the patients, and none occurred after 10 months. The operative mortality rate was 1.1%, and there were no infections. Life table analysis suggested that 72% of the patients would be successfully treated using a single ETV-CPC and 78% would remain shunt-independent with reopening of a closed ETV stoma. Multivariate logistic regression showed scarring of the cistern (p = 0.021) or choroid plexus (p = 0.026) as predictors of failure, but age at the time of surgery was not a significant predictor. Conclusions Using ETV-CPC appears to successfully provide a more durable primary treatment of hydrocephalus for infants with spina bifida than does shunt placement. These results support ETV-CPC as the better treatment option for these children in developing countries.

Birth rates several times those in developed countries, the lack of preventive measures, and the rarity of prenatal diagnosis all contribute to the fact that spina bifida is a very common condition among children of developing countries. At the CURE Children’s Hospital of Uganda (CCHU), a center for pediatric neurosurgery established in 2001, 75–100 new infants with myelomeningocele (MMC) present for treatment annually.

Object. The aim of this prospective study was to determine whether, and in which patients, the outcome for bilateral choroid plexus cauterization (CPC) in combination with endoscopic third ventriculostomy (ETV) was superior to ETV alone. Methods. A total of 710 children underwent ventriculoscopy as candidates for ETV as the primary treatment for hydrocephalus. The ETV was accomplished in 550 children: 266 underwent a combined ETV‐CPC procedure and 284 underwent ETV alone. The mean and median ages were 14 and 5 months, respectively, and 443 patients (81%) were younger than 1 year of age. The hydrocephalus was postinfectious (PIH) in 320 patients (58%), nonpostinfectious (NPIH) in 152 (28%), posthemorrhagic in five (1%), and associated with myelomeningocele in 73 (13%). The mean follow up was 19 months for ETV and 9.2 months for ETV‐CPC. Overall, the success rate of ETV‐CPC (66%) was superior to that of ETV alone (47%) among infants younger than 1 year of age (p , 0.0001). The ETV‐CPC combined procedure was superior in patients with a myelomeningocele (76% compared with 35% success, p = 0.0045) and those with NPIH (70% compared with 38% success, p = 0.0025). Although the difference was not significant for PIH (62% compared with 52% success, p = 0.1607), a benefit was not ruled out (power = 0.3). For patients at least 1 year of age, there was no difference between the two procedures (80% success for each, p = 1.0000). The overall surgical mortality rate was 1.3%, and the infection rate was less than 1%. Conclusions. The ETV‐CPC was more successful than ETV alone in infants younger than 1 year of age. In developing countries in which a dependence on shunts is dangerous, ETV‐CPC may be the best option for treating hydrocephalus in infants, particularly for those with NPIH and myelomeningocele.

In their article, “A model for neurosurgical humanitarian aid based on 12 years of medical trips to South and Central America,” Mainthia and coauthors4 have described the methods, evolution, and impact of their sustained endeavor to provide neurosurgical treatment for children in 4 countries of Central and South America. This descriptive report is an unusual yet valuable contribution to the literature, because it highlights the need of children in developing countries for better access to specialized surgical care and demonstrates a model in which an American pediatric neurosurgical team has made a sustained and organized contribution to a solution. Children younger than 15 years of age comprise roughly one-third of the human population, but their distribution is concentrated in regions where the resources for healthcare in general, and neurosurgery in particular, are the most limited. In Guatemala, for instance, where the authors report having performed the majority of their work, 43% of the population is younger than 15 years old compared with 20% in the US.5 Contrasting the 2 countries where I have lived and worked exemplifies the inequities among the children of the world when it comes to accessing neurosurgical care. In the US, the ratio of neurosurgeons to children younger than 10 years of age is about 1:12,000 compared with a ratio of 1:2,600,000 in Uganda.6 Existing models for providing neurosurgical care to these children principally involve importing neurosurgical teams from more developed countries in the North to less developed countries in the South, exporting children from the South to the North, or building neurosurgical capacity within the country through the training and equipping of national surgeons or neurosurgeons. A great strength of the International Hospital for Children model Mainthia and colleagues have reported is that it incorporates all 3 elements. The authors describe a program in which children receive a high level of care from the visiting team and local partners are mentored and included in the enterprise. Transport to the authors’ home institution in the US (Virginia Commonwealth University Health Systems) is arranged for children whose problems are deemed too complex for safe management in the local environment. Senior neurosurgery residents participate in each trip. This not only undoubtedly augments their clinical training, for instance, in having the opportunity to close a number of myelomeningoceles, but also probably proves personally formative through the singular experience of delivering much needed care in a challenging practice environment. Clinical follow-up in a poor rural population is extremely challenging, but the authors have made all reasonable efforts to minimize losing track of patients, and the methods they have established to do this are outlined in their report. Over the course of the program’s 12 years, the authors have noted an increase in the average age of patients and a decline in the fraction of total operations accounted for by myelomeningocele repairs from 58 to 11%. They attribute part of this to their contribution toward increasing the neurosurgical capacity within the country, but they also rightly acknowledge that this could have resulted from the introduction of a national program of folic acid fortification. In 1981, Dr. Richard Bergland eloquently warned of the plight of “neurosurgery in a zero-sum society.”1 I would suggest that, in the context of the 21st-Century global community in an increasingly “flat” world,3 the pediatric surgical disciplines stand on the threshold of a non–zero-sum opportunity. Most of the world’s children live in the southern hemisphere, whereas most of the resources and expertise to treat pediatric surgical diseases are in the northern hemisphere. Conversely, however, the declining fraction of children comprising Northern populations threatens to limit opportunities for training and research in the pediatric surgical disciplines, including in pediatric neurosurgery. It is apparent that innovative strategies to organize and fund the progressive engagement of the North and South could simultaneously provide surgical care to children who would otherwise not receive it, build capacity within the region to provide specialized pediatric surgical services, create opportunities for training North American and European residents, and facilitate globally oriented research that is energized by data derived from large populations. It may be helpful to evolve from the idea of “neurosurgical humanitarian aid” toward the concept of global neurosurgical partnerships in which aid is not delivered but rather via strategies of “social entrepreneurship,”2 non– zero-sum engagements between the North and South are formed in which everyone wins. Pioneering projects such as those described by Mainthia and colleagues, as well as similar endeavors by others in our discipline, serve as guideposts on the road ahead. J Ne osurg Pediatrics 4:1–3

Background. The objective structured clinical examination (OSCE), an established instrument for evaluating resident competence, was used to test the hypothesis that faculty assessment of clinical competence in residents at various levels of training may be influenced more by general skills as a physician and less by competency in the actual skills being specifically tested. In this study, advantage was taken of the anticipated observation that general surgery residents did not demonstrate improvement in their ability to perform a focused neurological assessment over time. Methods. An OSCE, which was administered to 56 general surgery residents at all levels of training, included the assessment of a specific clinical neurosurgical problem (sciatica). Univariate and multivariate analyses were used to evaluate the relationship between the global faculty judgment of competent or noncompetent and the other performance measures that were applied. Results. At different levels of training, there was no observed difference in the specific skills being tested; nevertheless, junior and senior residents were more likely than incoming interns to be judged “competent” and received better evaluations of how well they introduced themselves to the patient. The competence judgment correlated significantly with all of the other performance measures, including the skills being tested. Conclusions. The perception of competence is not solely dependent upon the particular skills under scrutiny. General competence does not guarantee competence in each specific skill set of a medical specialty.

An eight-year-old boy presented with left eye pain, photophobia, proptosis, third nerve paresis and decreased visual acuity. Magnetic resonance imaging revealed a nonenhancing mass filling the cavernous sinus. Using an extradural fronto-orbitozygomatic approach, the cavernous sinus was approached laterally, and a teratoma was removed from within the cavernous sinus. This is the first case of a truly intracavernous teratoma in a child and the fourth case of a teratoma reported in the cavernous sinus region overall. This report outlines the diagnosis and treatment of this unusual cavernous sinus tumor.

A case of ventral brain-stem compression by a congenital subarachnoid ependymal cyst in an infant is presented. Marsupialization of the cyst provided effective decompression and clinical improvement. Immunohistochemical staining demonstrated the glial nature of the epithelium. The fact that these cysts may be congenital, as demonstrated here, supports the hypothesis that they arise from glial elements that have been displaced during development.

Objective There are potentially 100,000 to 200,000 new cases of infant hydrocephalus each year in sub-Saharan Africa alone. The difficulty of accessing urgent neurosurgical care in this region substantially magnifies the risk of serious morbidity and death from shunt malfunction. Endoscopic third ventriculostomy is an excellent treatment alternative to shunt placement, but its effectiveness in young infants is substantially reduced. Methods Combining endoscopic third ventriculostomy with bilateral endoscopic choroid plexus cauterization has emerged as a significantly more successful treatment option for infant hydrocephalus. Results In Uganda, two thirds of infants younger than age 1 year and more than three fourths of children older than age 1 year undergoing the procedure have successfully avoided shunt dependence. Nearly all failures occur in the first 6 months, which, for infants, is a relatively safe time as treatment failure is visible to the mother and the need for intervention is less urgent. Clinical parameters have been identified that help predict the likelihood of treatment failure in a given patient. Conclusions The broader impact of this technique on the management of pediatric hydrocephalus in the developing world will be dependent on demonstration of its success when implemented by other surgeons in different patient populations, and the extent to which this treatment paradigm is ultimately adopted.

Roughly one-third of the world's nearly 7 billion people are covered by approximately 1/20 of its neurosurgeons. Neurosurgeons in the more developed countries have a moral obligation to increase access to neurosurgical care for the rest of the world. This can be achieved most effectively through neurosurgical education. Many neurosurgeons have already contributed greatly in this regard. Because of insufficient access to neurosurgical care, most children with hydrocephalus in Africa go untreated. Possibly as many as 2000 infants per neurosurgeon per year will develop hydrocephalus in sub-Saharan Africa. We have adopted a disease-specific strategy for training and equipping centers to provide evidence-based endoscopic treatment of hydrocephalus to save lives while avoiding the danger of shunt dependence, which is magnified in this context. In Uganda, we have successfully educated one to save a relative few. Our aim is to educate a few to save many. Such a disease-specific approach may provide a useful strategy for increasing access to care for other common, treatable neurosurgical conditions in resource-poor settings.

Vesna Martich Kriss 1,2 Timothy C. Kriss3, Benjamin C. Warf3 . .v ._ n 1972, James and Lassman [1] : coined the term “meningocele * . manque” to describe a form of spinal dysraphism in which aberrant nerve roots and fibrous bands tether normal neural structures. The literature on meningocele manque is sparse [2-5]; to our knowledge, only one radiographic description of this entity has appeared (with the authors using CT and MR imaging) [6]. Our report is unique in that the patient’s condition was revealed by sonography of the spine before surgery. To our knowledge, this is the first sonographic identification of the dorsal tethcling bands of meningocele manque.

OBJECTIVE AND IMPORTANCE : Cavernous sinus thrombophlebitis is a rare, dangerous, and historically difficult condition to diagnose and treat. Knowledge of the imaging findings and the importance of early diagnosis and treatment are emphasized. CLINICAL PRESENTATION : We present a case of cavernous sinus thrombophlebitis caused by sphenoid sinusitis. Previously undescribed magnetic resonance imaging findings of cavernous sinus thrombophlebitis include enlargement of the cavernous sinus, abnormal enhancement and increased T2 signal of the petrous apex and clivus, and marked narrowing of the cavernous portion of the internal carotid artery. Resolution of cavernous sinus thrombophlebitis is also documented by magnetic resonance imaging. INTERVENTION : The anatomy of the cavernous sinus dictates the natural history and diagnosis of cavernous sinus thrombophlebitis. We review the literature regarding the clinical diagnosis, differential diagnosis, and treatment of cavernous sinus thrombophlebitis. CONCLUSION : We emphasize the importance of a high index of suspicion, the potentially rapid and fatal course of the disease process, and the subsequent need for antibiotic therapy and selective surgery.

Concerns have arisen regarding the ability of physiologic techniques to select rootlets mediating spasticity in children undergoing partial dorsal rhizotomy (PDR) for the treatment of spastic gait from cerebral palsy. To determine whether these physiologic responses are reproducible, 60 rootlets in 6 patients were graded from 0 to 4+ according to the system first reported by Phillips and Park, and then retested and graded using a randomized, blinded paradigm. Two thirds of the rootlets had one or more grade difference between the two trials, and 25% had a grade change of 3 or more. Only 4 of 23 rootlets with a "normal' response (grade 0) on one test were also graded normal on the other test. Only 2 of 14 rootlets with a bilateral response (grade 4+) had a bilateral response on both trials. Statistical analysis demonstrated no correlation and poor agreement between trials. Regardless of whether responses are reflex in origin with technical or physiologic variability, or the consequence of stimulus spread to ventral roots, we found inconsistent responses with commonly used methods and the criteria most agreed upon. These findings suggest that currently used techniques are not reliable for rootlet "selection' and result only in a random partial rhizotomy.