John Mugamba

Long-term outcome for endoscopic third ventriculostomy alone or in combination with choroid plexus cauterization for congenital aqueductal stenosis in African infants.

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 Childrens 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.

Management of Dandy-Walker complex-associated infant hydrocephalus by combined endoscopic third ventriculostomy and choroid plexus cauterization.

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 Childrens 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.

Encephalocele in Uganda: ethnic distinctions in lesion location, endoscopic management of hydrocephalus, and survival in 110 consecutive children

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 Childrens 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.

Shunt survival after failed endoscopic treatment of hydrocephalus.

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 Childrens 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.

Reopening of an obstructed third ventriculostomy: long-term success and factors affecting outcome in 215 infants

OBJECT The role of reopening an obstructed endoscopic third ventriculostomy (ETV) as treatment for ETV failure is not well defined. The authors studied 215 children with ETV closure who underwent successful repeat ETV to determine the indications, long-term success, and factors affecting outcome. METHODS The authors retrospectively reviewed the CURE Childrens Hospital of Uganda database from August 2001 through December 2012, identifying 215 children with failed ETV (with or without prior choroid plexus cauterization [CPC]) who underwent reopening of an obstructed ETV stoma. Treatment survival according to sex, age at first and second operation, time to failure of first operation, etiology of hydrocephalus, prior CPC, and mode of ETV obstruction (simple stoma closure, second membrane, or cisternal obstruction from arachnoid scarring) were assessed using the Kaplan-Meier survival method. Survival differences among groups were assessed using log-rank and Wilcoxon methods and a Cox proportional hazards model. RESULTS There were 125 boys and 90 girls with mean and median ages of 229 and 92 days, respectively, at the initial ETV. Mean and median ages at repeat ETV were 347 and 180 days, respectively. Postinfectious hydrocephalus (PIH) was the etiology in 126 patients, and nonpostinfectious hydrocephalus (NPIH) in 89. Overall estimated 7-year success for repeat ETV was 51%. Sex (p = 0.46, log-rank test; p = 0.54, Wilcoxon test), age (< vs > 6 months) at initial or repeat ETV (p = 0.08 initial, p = 0.13 repeat; log-rank test), and type of ETV obstruction (p = 0.61, log-rank test) did not affect outcome for repeat ETV (p values ≥ 0.05, Cox regression). Those with a longer time to failure of initial ETV (> 6 months 91%, 3-6 months 60%, < 3 months 42%, p < 0.01; log-rank test), postinfectious etiology (PIH 58% vs NPIH 42%, p = 0.02; log-rank and Wilcoxon tests) and prior CPC (p = 0.03, log-rank and Wilcoxon tests) had significantly better outcome. CONCLUSIONS Repeat ETV was successful in half of the patients overall, and was more successful in association with later failures, prior CPC, and PIH. Obstruction of the original ETV by secondary arachnoid scarring was not a negative prognostic factor, and should not discourage the surgeon from proceeding. Repeat ETV may be a more durable solution to failed ETV/CPC than shunt placement in this context, especially for failures at more than 3 months after the initial ETV. Some ETV closures may result from an inflammatory response that is less robust at the second operation.

Effectiveness of the Bactiseal Universal Shunt for reducing shunt infection in a sub-Saharan African context: a retrospective cohort study in 160 Ugandan children.

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, Fishers 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.

SIOP PODC adapted treatment recommendations for standard-risk medulloblastoma in low and middle income settings

Effective treatment of children with medulloblastoma requires a functioning multi‐disciplinary team with adequate neurosurgical, neuroradiological, pathological, radiotherapy and chemotherapy facilities and personnel. In addition the treating centre should have the capacity to effectively screen and manage any tumour and treatment‐associated complications. These requirements have made it difficult for many low and middle‐income countries (LMIC) centres to offer curative treatment. This article provides management recommendations for children with standard‐risk medulloblastoma (localised tumours in children over the age of 3–5 years) according to the level of facilities available. Pediatr Blood Cancer 2015;62:553–564.

Indication for Endoscopic Third Ventriculostomy

BACKGROUND Endoscopic third ventriculostomy (ETV) is increasingly prevalent among pediatric neurosurgeons as the initial treatment for hydrocephalus. The combination of ETV and choroid plexus cauterization (ETV/CPC) has improved the success rate among infants with hydrocephalus for whom ETV alone is much less successful. In parts of the developing world where there are economic and human resource constraints, this mode of treatment may be more appropriate than the routine use of shunts, which are prone to failures that require urgent surgical treatment. Here we review indications for the use of ETV or ETV/CPC as the primary treatment for hydrocephalus. CONCLUSION Primary treatment of hydrocephalus by ETV can avoid shunt-dependence and its complications for many patients. Optimal results depend upon proper patient selection and the use of combined ETV/CPC when treating infants.

Endoscopic Treatment versus Shunting for Infant Hydrocephalus in Uganda

Background Postinfectious hydrocephalus in infants is a major health problem in sub‐Saharan Africa. The conventional treatment is ventriculoperitoneal shunting, but surgeons are usually not immediately available to revise shunts when they fail. Endoscopic third ventriculostomy with choroid plexus cauterization (ETV–CPC) is an alternative treatment that is less subject to late failure but is also less likely than shunting to result in a reduction in ventricular size that might facilitate better brain growth and cognitive outcomes. Methods We conducted a randomized trial to evaluate cognitive outcomes after ETV–CPC versus ventriculoperitoneal shunting in Ugandan infants with postinfectious hydrocephalus. The primary outcome was the Bayley Scales of Infant Development, Third Edition (BSID‐3), cognitive scaled score 12 months after surgery (scores range from 1 to 19, with higher scores indicating better performance). The secondary outcomes were BSID‐3 motor and language scores, treatment failure (defined as treatment‐related death or the need for repeat surgery), and brain volume measured on computed tomography. Results A total of 100 infants were enrolled; 51 were randomly assigned to undergo ETV–CPC, and 49 were assigned to undergo ventriculoperitoneal shunting. The median BSID‐3 cognitive scores at 12 months did not differ significantly between the treatment groups (a score of 4 for ETV–CPC and 2 for ventriculoperitoneal shunting; Hodges–Lehmann estimated difference, 0; 95% confidence interval [CI], ‐2 to 0; P=0.35). There was no significant difference between the ETV–CPC group and the ventriculoperitoneal‐shunt group in BSID‐3 motor or language scores, rates of treatment failure (35% and 24%, respectively; hazard ratio, 0.7; 95% CI, 0.3 to 1.5; P=0.24), or brain volume (z score, ‐2.4 and ‐2.1, respectively; estimated difference, 0.3; 95% CI, ‐0.3 to 1.0; P=0.12). Conclusions This single‐center study involving Ugandan infants with postinfectious hydrocephalus showed no significant difference between endoscopic ETV–CPC and ventriculoperitoneal shunting with regard to cognitive outcomes at 12 months. (Funded by the National Institutes of Health; ClinicalTrials.gov number, NCT01936272.)

Accessory limb with myelomeningocele: a rare case challenging previously held beliefs

IntroductionThere have been previous reports of intra-scapular limbs associated with a closed spina bifida and this has led to a causative theory. It is thought that these dysraphic appendages could not occur with defects of primary neurulation.CaseThe authors present a rare case of this abnormality associated with a large open myelomeningocele in a 6-day-old infant presenting to a paediatric neurosurgical hospital in Uganda. The appendage was removed and the spina bifida closed. There was significant stigma associated with such abnormality in this region.DiscussionThe first reported co-existence of these two lesions challenges previously held beliefs regarding the embryological origin of intra-scapular dysraphic appendage.