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OBJECTIVE Endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) can avoid ventriculoperitoneal shunt (VPS) dependence in very young hydrocephalic children, although long-term success as a primary treatment in North America has not been previously reported. Moreover, optimal age at surgery, impact of preoperative ventriculomegaly, and relationship to prior cerebrospinal fluid (CSF) diversion remain poorly defined. The authors compared ETV/CPC and VPS placement for averting reoperation, and they evaluated preoperative predictors for reoperation and shunt placement after ETV/CPC. METHODS All patients under 12 months of age who underwent initial hydrocephalus treatment via ETV/CPC or VPS placement at Boston Children’s Hospital between December 2008 and August 2021 were reviewed. Analyses included Cox regression for independent outcome predictors, and both Kaplan-Meier and log-rank rank tests for time-to-event outcomes. Cutoff values for age and preoperative frontal and occipital horn ratio (FOHR) were determined with receiver operating characteristic curve analysis and Youden’s J index. RESULTS In total, 348 children (150 females) were included with principal etiologies of posthemorrhagic hydrocephalus (26.7%), myelomeningocele (20.1%), and aqueduct stenosis (17.0%). Of these, 266 (76.4%) underwent ETV/CPC and 82 (23.6%) underwent VPS placement. Treatment choice largely reflected surgeon preferences before practice shifted toward endoscopy, with endoscopy not considered for > 70% of initial VPS cases. ETV/CPC patients trended toward fewer reoperations, and Kaplan-Meier analysis estimated that 59% of patients would achieve long-term shunt freedom through 11 years (median 42 months of actual follow-up). Among all patients, corrected age < 2.5 months (p < 0.001), prior temporizing CSF diversion (p = 0.003), and excess intraoperative bleeding (p < 0.001) independently predicted reoperation. Among ETV/CPC patients, corrected age < 2.5 months (p = 0.031), prior CSF diversion (p = 0.001), preoperative FOHR > 0.613 (p = 0.011), and excessive intraoperative bleeding (p = 0.001) independently predicted ultimate conversion to VPS. The actual VPS insertion rates remained low in patients who were ≥ 2.5 months old at ETV/CPC either with prior CSF diversion (2/10 [20.0%]) or without prior CSF diversion (24/123 [19.5%]); however, the actual VPS insertion rates increased in patients who were < 2.5 months old at ETV/CPC with prior CSF diversion (19/26 [73.1%]) or without prior CSF diversion (44/107 [41.1%]). CONCLUSIONS ETV/CPC successfully treated hydrocephalus in most patients younger than 1 year irrespective of etiology, averting observed shunt dependence in 80% of patients ≥ 2.5 months of age regardless of prior CSF diversion and in 59% of those < 2.5 months of age without prior CSF diversion. For infants aged < 2.5 months with prior CSF diversion, particularly those with severe ventriculomegaly, ETV/CPC was unlikely to succeed unless safely delayed.

OBJECTIVE Hydrocephalus in infants, particularly that with a postinfectious etiology, is a major public health burden in Sub-Saharan Africa. The authors of this study aimed to determine whether surgical treatment of infant postinfectious hydrocephalus in Uganda results in sustained, long-term brain growth and improved cognitive outcome. METHODS The authors performed a trial at a single center in Mbale, Uganda, involving infants (age < 180 days old) with postinfectious hydrocephalus randomized to endoscopic third ventriculostomy plus choroid plexus cauterization (ETV+CPC; n = 51) or ventriculoperitoneal shunt (VPS; n = 49). After 2 years, they assessed developmental outcome with the Bayley Scales of Infant Development, Third Edition (BSID-III), and brain volume (raw and normalized for age and sex) with CT scans. RESULTS Eighty-nine infants were assessed for 2-year outcome. There were no significant differences between the two surgical treatment arms in terms of BSID-III cognitive score (p = 0.17) or brain volume (p = 0.36), so they were analyzed together. Raw brain volumes increased between baseline and 2 years (p < 0.001), but this increase occurred almost exclusively in the 1st year (p < 0.001). The fraction of patients with a normal brain volume increased from 15.2% at baseline to 50.0% at 1 year but then declined to 17.8% at 2 years. Substantial normalized brain volume loss was seen in 21.3% patients between baseline and year 2 and in 76.7% between years 1 and 2. The extent of brain growth in the 1st year was not associated with the extent of brain volume changes in the 2nd year. There were significant positive correlations between 2-year brain volume and all BSID-III scores and BSID-III changes from baseline. CONCLUSIONS In Sub-Saharan Africa, even after successful surgical treatment of infant postinfectious hydrocephalus, early posttreatment brain growth stagnates in the 2nd year. While the reasons for this finding are unclear, it further emphasizes the importance of primary infection prevention and mitigation strategies along with optimizing the child’s environment to maximize brain growth potential.

OBJECTIVEDespite significant advances in diagnostic and surgical techniques, the surgical management of Chiari malformation type I (CM-I) with associated syringomyelia remains controversial, and the type of surgery performed is surgeon dependent. This study’s goal was to determine the feasibility of a prospective, multicenter, cohort study for CM-I/syringomyelia patients and to provide pilot data that compare posterior fossa decompression and duraplasty (PFDD) with and without tonsillar reduction.METHODSParticipating centers prospectively enrolled children suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression. Clinical data were entered into a database preoperatively and at 1–2 weeks, 3–6 months, and 1 year postoperatively. MR images were evaluated by 3 independent, blinded teams of neurosurgeons and neuroradiologists. The primary endpoint was improvement or resolution of the syrinx.RESULTSEight clinical sites were chosen based on the results of a published questionnaire intended to remove geographic and surgeon bias. Data from 68 patients were analyzed after exclusions, and complete clinical and imaging records were obtained for 55 and 58 individuals, respectively. There was strong agreement among the 3 radiology teams, and there was no difference in patient demographics among sites, surgeons, or surgery types. Tonsillar reduction was not associated with > 50% syrinx improvement (RR = 1.22, p = 0.39) or any syrinx improvement (RR = 1.00, p = 0.99). There were no surgical complications.CONCLUSIONSThis study demonstrated the feasibility of a prospective, multicenter surgical trial in CM-I/syringomyelia and provides pilot data indicating no discernible difference in 1-year outcomes between PFDD with and without tonsillar reduction, with power calculations for larger future studies. In addition, the study revealed important technical factors to consider when setting up future trials. The long-term sequelae of tonsillar reduction have not been addressed and would be an important consideration in future investigations.

Pediatric hydrocephalus is a well-studied and still incompletely understood entity. One of the physiological means by which hydrocephalus and intracranial hypertension evolve is through perturbations to normal vascular dynamics. Here the authors report a unique case of an extracranial vascular anomaly resulting in persistently elevated intracranial pressures (ICPs) independent of CSF diversion in a patient with a Joubert syndrome–related disorder. The patient developed worsening intracranial hypertension after successful CSF diversion of Dandy-Walker malformation–associated hydrocephalus via endoscopic third ventriculostomy–choroid plexus cauterization (ETV/CPC). Vascular workup and imaging revealed an extracranial arteriovenous fistula of the superficial temporal artery at the site of a prior scalp intravenous catheter. Following microsurgical obliteration of the lesion, ICP normalized from > 30 cm H2O preoperatively to 11 cm H2O postoperatively. A repeat lumbar puncture at 4 months postoperatively again demonstrated normal pressure, and the patient remained asymptomatic for 9 months. Recurrent symptoms at 9 months were attributed to inadequate CSF diversion, and the patient underwent ventriculoperitoneal shunt placement. This is the first report of an extracranial-to-extracranial vascular anastomosis resulting in intracranial hypertension. This case report demonstrates the need to consider extracranial vascular anomalies as potential sources of persistently elevated ICP in the syndromic pediatric population.

OBJECTIVEWhile there is a long history of interest in measuring brain growth, as of yet there is no definitive model for normative human brain volume growth. The goal of this study was to analyze a variety of candidate models for such growth and select the model that provides the most statistically applicable fit. The authors sought to optimize clinically applicable growth charts that would facilitate improved treatment and predictive management for conditions such as hydrocephalus.METHODSThe Weibull, two-term power law, West ontogenic, and Gompertz models were chosen as potential models. Normative brain volume data were compiled from the NIH MRI repository, and the data were fit using a nonlinear least squares regression algorithm. Appropriate statistical measures were analyzed for each model, and the best model was characterized with prediction bound curves to provide percentile estimates for clinical use.RESULTSEach model curve fit and the corresponding statistics were presented and analyzed. The Weibull fit had the best statistical results for both males and females, while the two-term power law generated the worst scores. The statistical measures and goodness of fit parameters for each model were provided to assure reproducibility.CONCLUSIONSThe authors identified the Weibull model as the most effective growth curve fit for both males and females. Clinically usable growth charts were developed and provided to facilitate further clinical study of brain volume growth in conditions such as hydrocephalus. The authors note that the homogenous population from which the normative MRI data were compiled limits the study. Gaining a better understanding of the dynamics that underlie childhood brain growth would yield more predictive growth curves and improved neurosurgical management of hydrocephalus.

OBJECTIVEEndoscopic third ventriculostomy (ETV)/choroid plexus cauterization (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. Therefore, the authors used a propensity score–matched cohort and survival analysis to assess the comparative efficacy of flexible and rigid neuroendoscopy.METHODSIndividual data were collected through retrospective review of infants younger than 2 years of age, treated at 1 of 2 hospitals: 1) Boston Children's Hospital, exclusively utilizing flexible neuroendoscopy, and 2) Nicklaus Children's Hospital-Jackson Memorial Hospital, exclusively utilizing rigid neuroendoscopy. Patient characteristics and postoperative outcomes were assessed. A propensity score model was developed to balance patient characteristics in the case mix.RESULTSA propensity score model for neuroendoscope type was developed with 5 independent variables: chronological age, sex, hydrocephalus etiology, prior CSF diversion, and prepontine scarring. Propensity score decile-adjusted and 1-to-1 nearest-neighbor matching analysis revealed that compared with flexible neuroendoscopy, rigid neuroendoscopy had an ETV/CPC failure odds ratio (OR) of 1.43 (p = 0.31) and 1.31 (p = 0.47), respectively, compared with an unadjusted OR of 2.40 (p = 0.034). Furthermore, in a Cox regression analysis controlled by propensity score, rigid neuroendoscopy had a hazard ratio (HR) of 1.10 (p = 0.70), compared with an unadjusted HR of 1.61 (p = 0.031).CONCLUSIONSAlthough unadjusted analysis suggested worse ETV/CPC outcomes for infants treated by rigid neuroendoscopy, much of the difference could be attributed to the case mix and other predictors of outcome. A larger sample observational study or randomized controlled trials are required to provide evidence-based guidelines on ETV/CPC technique.

OBJECTIVE Myelomeningocele (MM) is a neural tube defect complicated by neurological deficits below the level of the spinal lesion and, in many cases, hydrocephalus. Long-term survival of infants treated for MM in a low- and middle-income country has never been reported. This retrospective cohort study reports 10-year outcomes and factors affecting survival for infants undergoing MM repair at CURE Children's Hospital of Uganda. METHODS Patients were traced by telephone or home visit. Survival was estimated using the Kaplan-Meier method. Multivariate survival was analyzed using the Cox proportional hazards model, investigating the following variables: sex, age at surgery, weight-for-age at surgery, motor level, and presence and management of hydrocephalus. RESULTS A total of 145 children underwent MM repair between 2000 and 2004; complete data were available for 133 patients. The probability of 10-year survival was 55%, with 78% of deaths occurring in the first 5 years. Most of the deaths were not directly related to MM; infection and neglect were most commonly described. Lesions at motor level L-2 or above were associated with increased mortality (HR 3.176, 95% CI 1.557–6.476). Compared with repair within 48 hours of birth, surgery at 15–29 days was associated with increased mortality (HR 9.091, 95% CI 1.169–70.698). CONCLUSIONS Infants in low- and middle-income countries with MM can have long-term survival with basic surgical intervention. Motor level and age at surgery were significant factors influencing outcome. Education of local health care workers and families to ensure both urgent referral for initial treatment and subsequent access to basic medical care are essential to survival.

OBJECT The evaluation of hydrocephalus remains focused on ventricular size, yet the goal of treatment is to allow for healthy brain development. It is likely that brain volume is more related to cognitive development than is fluid volume in children with hydrocephalus. This study tests this hypothesis by comparing brain and fluid volumes with neurocognitive outcome in pediatric patients with hydrocephalus. METHODS Warf and colleagues previously acquired CT scans for pediatric patients in Uganda with myelomeningocele, measured frontal–occipital horn ratio (FOHR), and administered the modified Bayley Scales of Infant Development, third edition (BSID-III) to measure neurocognitive outcome that did not correlate with FOHR. In this present study, brain and fluid volumes were measured in 33 of these patients, 26 of whom required surgical treatment for hydrocephalus. Linear discrimination analysis (LDA) was used to test whether age-normalized brain and fluid volumes can discriminate neurocognitive outcome. RESULTS Hydrocephalic patients show normal to small brain volumes and substantially larger fluid volumes compared with normal values. FOHR correlates highly with fluid volume (r = 0.84, p < 0.001) and substantially less with brain volume (r = −0.37, p = 0.03), while brain and fluid volumes do not correlate with each other (p = 0.99). Brain and CSF volumes correlated best with fine motor (p = 0.03, p = 0.01), cognitive (p = 0.05, p = 0.09), and expressive communication (p = 0.08, p = 0.08) scores. A combination of these 3 scores was used as a multivariate measure of neurocognitive outcome. Brain volume alone, unlike fluid volume, could discriminate high from low cognitive outcome (by t-test and ANOVA). It was shown that a combination of age-normalized brain and fluid volumes can discriminate neurocognitive outcome by 2-way LDA (p < 0.01) and 3-way LDA (p < 0.01). The multivariate LDA demonstrated the contribution of large fluid volume to a decrement in cognition. CONCLUSIONS Hydrocephalus is treated by normalizing CSF, but normal brain development depends on brain growth. A combination of brain and CSF volumes appears to be significantly more powerful at predicting good versus poor neurocognitive outcomes in patients with hydrocephalus than either volume alone.

Object It is not known whether previous endoscopic third ventriculostomy (ETV) affects the risk of shunt failure. Different epochs of hydrocephalus treatment at the CURE Children's Hospital of Uganda (CCHU)—initially placing CSF shunts in all patients, then attempting ETV in all patients, and finally attempting ETV combined with choroid plexus cauterization (CPC) in all patients—provided the opportunity to assess whether prior endoscopic surgery affected shunt survival. Methods With appropriate institutional approvals, the authors reviewed the CCHU clinical database to identify 2329 patients treated for hydrocephalus from December 2000 to May 2007. Initial ventriculoperitoneal (VP) shunt placement was performed in 900 patients under one of three circumstances: 1) primary nonselective VP shunt placement with no endoscopy (255 patients); 2) VP shunt placement at the time of abandoned ETV attempt (with or without CPC) (370 patients); 3) VP shunt placement subsequent to a completed but failed ETV (with or without CPC) (275 patients). We analyzed time to shunt failure using the Kaplan-Meier method to construct survival curves, Cox proportional hazards regression modeling, and risk-adjusted analyses to account for possible confounding differences among these groups. Results Shunt failure occurred in 299 patients, and the mean duration of follow-up for the remaining 601 was 28.7 months (median 18.8, interquartile range 4.1–46.3). There was no significant difference in operative mortality (p = 0.07 by log-rank and p = 0.14 by Cox regression adjusted for age and hydrocephalus etiology) or shunt infection (p = 0.94, log-rank) among the 3 groups. There was no difference in shunt survival between patients treated with primary shunt placement and those who underwent shunt placement at the time of an abandoned ETV attempt (adjusted hazard ratio [HR] 1.14, 95% CI 0.86–1.51, p = 0.35). Those who underwent shunt placement after a completed but failed ETV (with or without CPC) had a lower risk of shunt failure (p = 0.008, log-rank), with a hazard ratio (adjusted for age at shunting and etiology) of 0.72 (95% CI 0.53–0.98), p = 0.03, compared with those who underwent primary shunt placement without endoscopy; but this was observed only in patients with postinfectious hydrocephalus (PIH) (adjusted HR 0.55, 95% CI 0.36–0.85, p = 0.007), and no effect was apparent for hydrocephalus of noninfectious etiologies (adjusted HR 0.98, 95% CI 0.64–1.50, p = 0.92). Improved shunt survival after failed ETV in the PIH group may be an artifact of selection arising from the inherent heterogeneity of ventricular damage within that group, or a consequence of the timing of shunt placement. The anticipated benefit of CPC in preventing future ventricular catheter obstruction was not observed. Conclusions A paradigm for infant hydrocephalus involving intention to treat by ETV with or without CPC had no adverse effect on mortality or on subsequent shunt survival or infection risk. This study failed to demonstrate a positive effect of prior ETV or CPC on shunt survival.

Dysplastic structural lesions within the fourth ventricle have been reported in patients with Chiari malformation Type II (CM-II). The authors report the unique case of a 22-year-old patient with myelomeningocele who presented with progressive pain, decreasing hand function, and lower cranial nerve dysfunction in the context of a slowly enlarging intramedullary mass at the cervicomedullary junction. At surgery a multinodular mass attached to caudally displaced fourth ventricle choroid plexus was completely removed from an expanded central canal. The histopathological findings were consistent with dysplastic-reactive choroid plexus. Postoperatively the patient experienced relief of pain and improvement in hand strength. To the authors' knowledge, this is the first reported case in which dysplastic fourth ventricle choroid plexus was displaced caudally through the obex to become an intramedullary lesion at the cervicomedullary junction. Its subsequent slow enlargement with progressive neurological deficits may have been secondary to reactive inflammatory changes. For patients with myelomeningocele and CM-II, intramedullary dysplastic-reactive choroid plexus should be included in the differential diagnosis of mass lesions in this location.

Object 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. Methods 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. Results Four infection-onset peaks were found to straddle the twice-yearly rainy season peaks, demonstrating that the infections occurred at intermediate levels of rainfall. Conclusions 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.

Object The authors have previously reported on the overall improved efficacy of endoscopic third ventriculostomy (ETV) combined with choroid plexus cauterization (CPC) for infants younger than 1 year of age. In the present study they specifically examined the long-term efficacy of ETV with or without CPC in 35 infants with congenital aqueduct stenosis treated at CURE Children's Hospital of Uganda during the years 2001–2006. Methods Infants with congenital aqueductal stenosis were treated during 2 distinct treatment epochs: all underwent ETV alone, and subsequently all underwent ETV-CPC. Prospectively collected data in the clinical database were reviewed for all infants with an age < 1 year who had been treated for hydrocephalus due to congenital aqueductal stenosis. Study exclusion criteria included: 1) a history or findings on imaging or at the time of ventriculoscopy that suggested a possible infectious cause of the hydrocephalus, including scarred choroid plexus; 2) an open aqueduct or an aqueduct obstructed by a membrane or cyst rather than by stenosis; 3) severe malformations of the cerebral hemispheres including hydranencephaly, significant segments of undeveloped brain, or schizencephaly; 4) myelomeningocele, encephalocele, Dandy-Walker complex, or tumor; or 5) previous shunt insertion. The time to treatment failure was analyzed using the Kaplan-Meier method to construct survival curves. Log-rank (Mantel-Cox) and Gehan-Breslow-Wilcoxon tests were used to determine whether differences between the 2 treatment groups were significant. Results Thirty-five patients met the study criteria. Endoscopic third ventriculostomy alone was performed in 12 patients (mean age 4.7 months), and combined ETV-CPC was performed in 23 patients (mean age 3.5 months). For patients without treatment failure, the mean and median follow-ups were, respectively, 51.6 and 48.0 months in the ETV group and 31.2 and 26.4 months in the ETV-CPC group. Treatment was successful in 48.6% of the patients who underwent ETV alone, as accurately predicted by the Endoscopic Third Ventriculostomy Success Score (ETVSS), and in 81.9% of the patients who underwent ETV-CPC (p = 0.0119, log-rank test; p = 0.0041, Gehan-Breslow-Wilcoxon test; HR 6.42 [95% CI 1.51–27.36]). Conclusions Combined ETV-CPC is significantly superior to ETV alone for infants younger than 1 year of age with congenital aqueductal stenosis. The fact that the outcome for ETV alone was accurately predicted by the ETVSS suggests that these results are applicable in developed countries.

Object Although survival for patients with myelomeningocele has dramatically improved in recent decades, the occasional occurrence of sudden, unexplained death in young adult patients with myelomeningocele has been noted by the authors. This study was undertaken to determine risk factors for sudden death in this population. Methods The authors performed a retrospective chart review of patients born between 1978 and 1990 who received care at Children's Hospital Boston. The relationship between sudden death and patient demographics, presence of CSF shunt and history of shunt revisions, midbrain length as a marker for severity of hindbrain malformation, seizures, pulmonary and ventilatory dysfunction, body mass index, scoliosis, renal dysfunction, and cardiac disease was evaluated using the t-test, Fisher exact test, and logistic regression analysis. Results The age range for 106 patients in the study cohort was 19–30 years, with 58 (54.7%) women and 48 (45.3%) men. Six patients, all of whom were young women, experienced sudden death. In multivariate analysis, female sex, sleep apnea, and midbrain elongation ≥ 15 mm on MR imaging remained significantly associated with a higher risk of sudden death. These risk factors were cumulative, and female patients with sleep apnea and midbrain length ≥ 15 mm had the greatest risk (adjusted risk ratio 24.0, 95% CI 7.3–79.0; p < 0.05). No other comorbidities were found to significantly increase the risk of sudden death. Conclusions Young adult women with myelomeningocele are at significantly increased risk of sudden death in the setting of midbrain elongation and sleep apnea. Further investigation is needed to determine the benefit of routine screening to identify at-risk patients for closer cardiopulmonary monitoring and treatment.

Object 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). Methods 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. Results 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. Conclusions 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.

Object 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. Methods 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. Results 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. Conclusions 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.

Object 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. Methods 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). Results 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. Conclusions Endoscopic treatment of DWC-associated hydrocephalus should be strongly considered as the primary management in place of the historical standard of creating shunt dependence.

Object 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. Methods 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. Results 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). Conclusions Community-based support following surgical interventions for MM and hydrocephalus appears essential to the continued survival of these children in Africa.

Object This study characterizes the first clinical series of encephalocele (EC) from East or Central Africa, and is the largest reported from the African continent. The authors explored survival, the efficacy of primary endoscopic management of associated hydrocephalus, and ethnic differences in EC location. Methods One hundred ten consecutive children presented to CURE Children's Hospital of Uganda for treatment of EC over a 9-year period. Clinical data, including patient demographic information, birth date, lesion type (sincipital, parietal, or occipital), operative data, and subsequent course had been entered prospectively into a clinical database. Home visits to update the status of those lost to follow-up were done when possible. With appropriate institutional approvals, the database was reviewed for this retrospective study. Two-tailed probability values calculated using the Fisher exact test were used to assess the significance of differences among groups, with p < 0.05 being considered significant. The Kaplan-Meier method was used for analysis of survival and treatment success probabilities. Results There were 53 (48%) occipital, 33 (30%) sincipital, and 24 (22%) parietal lesions. Occipital lesions were significantly more common among children of Bantu origin (p = 0.02). Nilotes demonstrated a roughly equal distribution among sincipital, parietal, and occipital locations. The female/male ratio was 1.2, with no difference between EC types (range 1.0–1.4, p = 0.6–0.8). Of 110 patients, 108 (98%) underwent surgical repair at a median age of 1 month (mean 15.7 months), whereas 2 had treatment for hydrocephalus only. Wound revision was required in 13% of cases. Surgery-related mortality was 3%. One-year and 5-year survival rates were 87% (95% CI 0.79–0.93) and 61% (95% CI 0.51–0.70), respectively. Hydrocephalus required treatment in 32%, and was equally common among the 3 EC types. Thirteen patients were treated with combined endoscopic third ventriculostomy/choroid plexus cauterization (ETV/CPC) and 2 with ETV alone, whereas 18 patients received primary shunt placement. Predicted treatment success at 1 year was 79% for ETV or ETV/CPC (95% CI 0.50–0.94) and 47% for shunt placement (95% CI 0.24–0.71). Conclusions Analysis of this first EC series from this region suggests that sincipital lesions are 3 times more common in East than in West Africa. Occipital lesions predominate in patients of Bantu origin, but not among those of Nilotic descent. Hydrocephalus incidence was equally common among different EC types, and endoscopic treatment was more successful (79%) than shunting (47%) at 1 year. The 5-year mortality rate was similar to that for infants with myelomeningocele in Uganda, and more than twice that for their unaffected peers.

Object 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. Methods 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. Results 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. Conclusions 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.

Object In Uganda, childhood hydrocephalus is common and difficult to treat. In some children, endoscopic third ventriculostomy (ETV) can be successful and avoid dependence on a shunt. This can be especially beneficial in Uganda, because of the high risk of infection and long-term failure associated with shunting. Therefore, the authors developed and validated a model to predict the chances of ETV success, taking into account the unique characteristics of a large sub-Saharan African population. Methods All children presenting with hydrocephalus at CURE Children's Hospital of Uganda (CCHU) between 2001 and 2007 were offered ETV as first-line treatment and were prospectively followed up. A multivariable logistic regression model was built using ETV success at 6 months as the outcome. The model was derived on 70% of the sample (training set) and validated on the remaining 30% (validation set). Results Endoscopic third ventriculostomy was attempted in 1406 patients. Of these, 427 were lost to follow-up prior to 6 months. In the remaining 979 patients, the ETV was aborted in 281 due to poor anatomy/visibility and in 310 the ETV failed during the first 6 months. Therefore, a total of 388 of 979 (39.6% and [55.6% of completed ETVs]) procedures were successful at 6 months. The mean age at ETV was 12.6 months, and 57.8% of cases were postinfectious in origin. The authors' logistic regression model contained the following significant variables: patient age at ETV, cause of hydrocephalus, and whether choroid plexus cauterization was performed. In the training set (676 patients) and validation set (303 patients), the model was able to accurately predict the probability of successful ETV (Hosmer-Lemeshow p value > 0.60 and C statistic > 0.70). The authors developed the simplified CCHU ETV Success Score that can be used in the field to predict the probability of ETV success. Conclusions The authors' model will allow clinicians to accurately identify children with a good chance of successful outcome with ETV, taking into account the unique characteristics and circumstances of the Ugandan population.

Object In the setting of a developing country where preoperative imaging may be limited, the authors wished to determine whether cisternal scarring or aqueduct patency at the time of surgery was sufficiently predictive of the failure of endoscopic third ventriculostomy (ETV) to justify shunt placement at the time of the initial operation. Methods The status of the prepontine cistern and aqueduct at the time of ventriculoscopy was prospectively recorded in 403 children in whom an ETV had been completed. Kaplan-Meier methods were used to construct survival curves. A Cox proportional hazards model was used to provide estimates of HRs for the time to ETV failure. Several independent variables were tested in a single multivariable model, including those previously shown to be associated with ETV survival, that is, age, hydrocephalus etiology, and extent of choroid plexus cauterization (CPC). In addition, intraoperative variables of particular interest were included in the analysis: status of the aqueduct at surgery (closed vs open) and status of the prepontine cistern at surgery (scarred vs clean/unscarred). Multicollinearity was not a concern since the variance inflation factors for all variables were < 2. The examination of stratified survival curves confirmed the appropriateness of the proportional hazards assumption for each variable. Results Overall actuarial 3-year success was 57%. Consistent with previous results, age, hydrocephalus etiology, and extent of CPC were significantly associated with ETV success. A closed aqueduct and an unscarred cistern were each independently associated with significantly better ETV success (HRs of 0.66 and 0.44, respectively). The presence of cisternal scarring more than doubled the risk of ETV failure, and an open aqueduct increased the risk of failure by 50%. Conclusions Intraoperative observations of the aqueduct and prepontine cistern are independent predictors of the risk of ETV failure and can be used to further refine outcome predictions based on age, hydrocephalus etiology, and extent of CPC. Further studies will test validity in several African centers and determine what threshold of failure risk should prompt shunt placement at the initial operation.

Object Despite lower failure and infection rates compared with shunt placement, it has not been known whether endoscopic third ventriculostomy/choroid plexus cauterization (ETV/CPC) might be inferior in regard to neurocognitive development. This study is the first to describe neurocognitive outcome and ventricle volume in infants with hydrocephalus due to myelomeningocele that was treated primarily by ETV/CPC. Methods The modified Bayley Scales of Infant Development (BSID-III) test was administered to 93 children with spina bifida who were 5–52 months of age. Fifty-five of these children had been treated by ETV/CPC, 19 received ventriculoperitoneal (VP) shunts, and 19 had required no treatment for hydrocephalus. Raw scores were converted to scaled scores for comparison with age-corrected norms. Ventricular volume was assessed by frontal/occipital horn ratio (FOR) calculated from late postoperative CT scans. The mean values between and among groups of patients were compared using independent samples t-test and ANOVA. The comparison of mean values to population normal means was performed using the single-sample t-test. Linear regression analyses were performed using BSID scores as the dependent variables, with treatment group and ventricular size (FOR) as the independent variables. Probability values < 0.05 were considered significant. Results . There was no significant difference in mean age at assessment among groups (p = 0.8). The mean scale scores for untreated patients were no different from normal (all p > 0.27) in all portions of the BSID (excluding gross motor), and were generally significantly better than those for both VP shunt–treated and ETV/CPC groups. The ETV/ CPC-treated patients had nonsignificantly better mean scores than patients treated with VP shunts in all portions of the BSID (all p > 0.06), except receptive communication, which was significantly better for the ETV/CPC group (p = 0.02). The mean FOR was similar among groups, with no significant difference between the untreated group and either the VP shunt or ETV/CPC groups. The FOR did not correlate with performance. Conclusions The ETV/CPC and VP shunt groups had similar neurocognitive outcomes. Neurocognitive outcomes for infants not requiring treatment for hydrocephalus were normal and significantly better than in those requiring treatment. The mean ventricular volume was similar among all 3 groups, and significantly larger than normal. There was no association between FOR and performance. Stable mild-to-moderate ventriculomegaly alone should not trigger intervention in asymptomatic infants with spina bifida.

Object. The author investigated the 1-year outcomes for shunt treatment of hydrocephalic children in Uganda, comparing the results using the inexpensive Chhabra shunt ($35 US dollars), widely used in East Africa, with those using the Codman-Hakim Micro Precision Valve shunt ($650). Methods. The results in 195 consecutive children (mostly infants) in whom shunts were placed were studied prospectively. In Group 1, 90 patients randomly received either the Chhabra or Codman shunt as primary treatment for hydrocephalus. In Group 2, 105 patients received the Chhabra shunt when endoscopic third ventriculostomy could not be performed or had failed. The end points of the study were shunt malfunction, shunt migration, wound complication, death, or no problem at 1 year. Of all patients, 9.7% were lost to follow up and 15.9% died before 1 year. The occurrence of complications in all patients were infection (9.7%), migration/disconnection (6.3%), wound complication (5.7%), valve malfunction (3.4%), ventricular catheter obstruction (2.8%), and peritoneal catheter obstruction (1.1%). There was no statistically significant difference in any outcome category for patients receiving the Codman or Chhabra shunt (p = 0.2463‐1.0000). Conclusions. Ventriculoperitoneal shunt insertion for treatment of hydrocephalus can be performed in a developing country with results similar to those reported in developed countries. No difference in outcome was noted between the two shunt types. No advantage was found in using a shunt system that, in this setting, is prohibitively expensive.

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.

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