Anna Lo Presti, Alexander G. Weil and John Ragheb
Anitha Nimmagadda, David I. Sandberg and John Ragheb
✓ The authors report the case of a newborn presenting at birth with macrocephaly and a large pineal region hemorrhagic cyst without neurological deficit. No neurosurgical intervention was performed, and subsequent imaging studies demonstrated complete involution of the cyst.
Michael Ragheb, Ashish H. Shah, Sarah Jernigan, Tulay Koru-Sengul and John Ragheb
Hydrocephalus is recognized as a common disabling pediatric disease afflicting infants and children disproportionately in the developing world, where access to neurosurgical care is limited and risk of perinatal infection is high. This surgical case series describes the Project Medishare Hydrocephalus Specialty Surgery (PMHSS) program experience treating hydrocephalus in Haiti between 2008 and 2015.
The authors conducted a retrospective review of all cases involving children treated for hydrocephalus within the PMHSS program in Port-au-Prince, Haiti, from 2008 through 2015. All relevant epidemiological information of children treated were prospectively collected including relevant demographics, birth history, hydrocephalus etiology, head circumference, and operative notes. All appropriate associations and statistical tests were performed using univariate and multivariate logistic regression analyses.
Among the 401 children treated within PMHSS, postinfectious hydrocephalus (PIH) accounted for 39.4% (n = 158) of cases based on clinical, radiographic, and endoscopic findings. The majority of children with hydrocephalus in Haiti were male (54.8%, n = 197), born in the rainy season (59.7%, n = 233), and born in a coastal/inland location (43.3%, n = 61). The most common surgical intervention was endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) (45.7%, n = 175). Multivariate logistic regression analysis yielded coastal birth location (OR 3.76, 95% CI 1.16–12.18) as a statistically significant predictor of PIH. Increasing head circumference (adjusted OR 1.06, 95% CI 0.99–1.13) demonstrated a slight trend toward significance with the incidence of PIH.
This information will provide the foundation for future clinical and public health studies to better understand hydrocephalus in Haiti. The 39.4% prevalence of PIH falls within observed rates in Africa as does the apparently higher prevalence for those born during the rainy season. Although PIH was the most frequent etiology seen in almost all birth locations, the potential relationship with geography noted in this series will be the focus of further research in an effort to understand the link between climate and PIH in Haiti. The ultimate goal will be to develop an appropriate public health strategy to reduce the burden of PIH on the children of Haiti.
Alexander G. Weil, Aria Fallah, Parthasarathi Chamiraju, John Ragheb and Sanjiv Bhatia
Combining endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has been shown to improve the success rate compared with ETV alone in infants (less than 24 months) with hydrocephalus who were treated in developing countries. The authors sought to evaluate the safety and efficacy of this procedure, using a rigid neuroendoscope, in a single North American center, and to assess whether the ETV success score (ETVSS), the CURE Children’s Hospital of Uganda ETVSS (CCHU ETVSS), and other pre- and intraoperative variables could predict success.
The authors performed a retrospective review of consecutive ETV/CPC procedures performed using a rigid neuroendoscope to treat infantile hydrocephalus. The infants underwent the procedure at Miami Children’s Hospital between January 2007 and 2014, with at least one postoperative follow-up. Duration of follow-up or time to failure of ETV/CPC, the primary outcome measure, was documented. A repeat CSF diversion procedure or death was considered as a failure of ETV/CPC. The time to event was measured using a Kaplan-Meier analysis. The authors analyzed ETVSS, CCHU ETVSS, and pre- and intraoperative variables to determine their suitability to predict success.
Eighty-five patients (45 boys) with a mean age of 4.3 months (range 1 day to 20 months) underwent ETV/CPC. Etiology included intraventricular hemorrhage of prematurity in 44 patients (51.7%), myelomeningocele (MMC) in 7 (8.2%), congenital aqueductal stenosis in 12 (14.1%), congenital communicating hydrocephalus in 6 (7.1%), Dandy-Walker complex in 6 (7.1%), postinfectious hydrocephalus in 5 (5.8%), and other cause in 5 (5.8%). Six procedure-related complications occurred in 5 (5.8%) patients, including 2 hygromas, 1 CSF leak, and 3 infections. There were 3 mortalities in this cohort. ETV/CPC was successful in 42.1%, 37.7%, and 36.8% of patients at 6, 12, and 24 months follow-up, respectively. The median (95% CI) time to ETV/CPC failure was 4.0 months (0.9–7.1 months). In univariate analyses, both the ETVSS (hazard ratio [HR] 1.03; 95% CI 1.01–1.05; p = 0.004) and CCHU ETVSS (HR 1.48; 95% CI 1.04–2.09; p = .028) were predictive of outcome following ETV/CPC. In multivariate analysis, the presence of prepontine scarring was associated with ETV/CPC failure (HR 0.34; 95% CI 0.19–0.63; p < 0.001). Other variables, such as radiological criteria (prepontine interval, prepontine space, aqueductal stenosis, Third Ventricular Morphology Index) and intraoperative findings (ventriculostomy pulsations, extent of CPC), did not predict outcome.
ETV/CPC is a feasible alternative to ETV and ventriculoperitoneal shunt in infants with hydrocephalus. Both the ETVSS and CCHU ETVSS predicted success following ETV/CPC in this single-center North American cohort of patients.
Alexander G. Weil, Andrew L. Middleton, Toba N. Niazi, John Ragheb and Sanjiv Bhatia
Operations on tumors of the posteromedial temporal (PMT) region, that is, on those arising from the posterior parahippocampal, fusiform, and lingual gyri, are challenging to perform because of the deep-seated location of these tumors between critical cisternal neurovascular structures and the adjacent temporal and occipital cortexes. Traditional surgical approaches require temporal or occipital transgression, retraction, or venous sacrifice. These approaches may result in unintended complications that should be avoided. To avoid these complications, the supracerebellar-transtentorial (SCTT) approach to this region has been used as an effective alternative treatment in adult patients. The SCTT approach uses a sitting position that offers a direct route to the posterior fusiform and lingual gyri of the temporal lobe. The authors report the feasibility, safety, and efficacy of this approach, using a modified lateral park-bench position in a small cohort of pediatric patients.
The authors carried out a retrospective case review of 5 consecutive patients undergoing a paramedian SCTT approach between 2009 and 2014 at the authors' institution.
The SCTT approach in the park-bench position was used in 3 boys and 2 girls with a mean age of 7.8 years (range 13 months to 16 years). All patients presented with a seizure disorder related to a tumor in a PMT region involving the parahippocampal and fusiform gyri of the left (n = 3) or right (n = 2) temporal lobe. No procedure-related complications were observed. Gross-total resection and control of seizures were achieved in all cases. Tumor classes and types included 1 Grade II astrocytoma, 1 pleomorphic xanthoastrocytoma, 1 ganglioglioma, and 2 glioneural tumors. None of the tumors had recurred by the mean follow-up of 22 months (range 1–48 months). Outcomes of epileptic seizures were excellent, with seizure symptoms in all 5 patients scoring in Engel Class IA.
The SCTT approach represents a viable option when resecting tumors in this region, providing a reasonable working corridor and low morbidity. The authors' experience in a cohort of pediatric patients demonstrates that complete resection of the lesions in this location is feasible and is safe when involving an approach that involves using a park-bench lateral positioning.
Stacey Quintero Wolfe, Sanjiv Bhatia, Barth Green and John Ragheb
✓The authors report on a 17-year-old boy with cervical myelopathy from dilated epidural veins due to cerebrospinal fluid (CSF) overdrainage. The patient had a long-standing subdural–peritoneal shunt and presented with incapacitating spastic tetraparesis. Magnetic resonance imaging revealed significant cervical spinal cord compression from a markedly dilated epidural venous plexus. The shunt was externalized so that CSF flow dynamics could be assessed, and the patient was found to have low intracranial pressure (ICP). The patient was gradually acclimated to higher ICPs, and a new shunt was placed with an antisiphon device and a programmable valve set at the higher pressure. Postoperatively the child experienced significant clinical improvement, and reduction of spinal cord compression was evident on images. Compensatory engorgement of the epidural venous plexus due to long-term shunt usage should be considered in the differential diagnosis when cervical myelopathy due to a dilated epidural venous plexus is present.
Zachary S. Hubbard, Ashish H. Shah, Michael Ragheb, Shelly Wang, Sarah Jernigan and John Ragheb
Previous models have been utilized in other low- and middle-income countries (LMICs) to explore and assess the cost, sustainability, and effectiveness of infant hydrocephalus treatment. However, similar models have not been implemented in Haiti due to a paucity of data, epidemiology, and outcomes for hydrocephalus. Therefore, the authors utilized previously described economic modeling to estimate the annual cost and benefit of treating hydrocephalus in infants at a neurosurgery referral center, Hospital Bernard Mevs (HBM), in Port-au-Prince, Haiti.
The authors conducted a retrospective review of data obtained in all children treated for hydrocephalus at the HBM from 2008 to 2015. The raw data were pooled with previously described surgical outcomes for hydrocephalus in other LMICs. Modeling was performed to determine outcomes, neurosurgical costs, disability-adjusted life years (DALYs), and economic benefits of Haitian hydrocephalus treatment during this time frame. Standard account methodology was employed to calculate cost per procedure. Using these formulas, the net economic benefit and cost/DALY were determined for hydrocephalus treatment at HBM from 2008 to 2015.
Of the 401 patients treated during the study period, 158 (39.4%) met criteria for postinfectious hydrocephalus, 54 (13.5%) had congenital hydrocephalus, 38 (9.5%) had myelomeningocele, 19 (4.7%) had aqueductal stenosis, and 132 (33%) were not placed into a category. Overall, 317 individuals underwent surgical treatment of their hydrocephalus, averting 3077 DALYs. The total cost of the procedures was $754,000, and the cost per DALY ranged between $86 and $245. The resulting net economic benefit for neurosurgical intervention ranged from $2.5 to $5.5 million.
This work demonstrates the substantial economic benefit of neurosurgical intervention for the treatment of pediatric hydrocephalus at a single hospital in Haiti. Based on DALYs averted, the need for additional centers offering basic neurosurgical services is apparent. A single center offering these services for several days each month was able to generate between $2.5 to $5.5 million in economic benefits, suggesting the need to develop neurosurgical capacity building in Haiti. Ultimately, prevention, screening, and early surgical treatment of these infants represent a public health and socioeconomic requisite for Haiti.
David I. Sandberg, John Ragheb, Catalina Dunoyer, Sanjiv Bhatia, Greg Olavarria and Glenn Morrison
In this study the authors review the outcomes in pediatric patients who presented with seizures and underwent resection of dysembryoplastic neuroepithelial tumors (DNETs). The authors focus on the diagnostic evaluation and surgical techniques that facilitate gross–total tumor resection and subsequent freedom from seizures.
Eighteen patients between the ages of 1 month and 13 years who presented with seizures underwent resection of DNETs between January 1992 and December 2004. Preoperative evaluation included magnetic resonance (MR) imaging and interictal scalp electroencephalography (EEG) in all patients, functional MR imaging in eight patients, video monitoring with ictal scalp EEG in 12 patients, interictal single-photon emission computerized tomography (SPECT) scanning in one patient, and ictal SPECT scanning in two patients. Thirteen patients underwent one-stage procedures, whereas five underwent two-stage procedures (implantation of monitoring electrodes followed by tumor resection), either for functional language mapping (three patients) or due to inconclusive preoperative data (two patients). Intraoperative electrocorticography (ECoG) was performed in 17 patients and led to resection of the cerebral cortex beyond the tumor margins in 10 of them. According to operative reports, gross–total tumor resections were achieved in all patients, but one child had minimal residual tumor on postoperative MR images that has remained stable. The only surgical complication was a transient third cranial nerve palsy. Over a median follow-up duration of 1.6 years, all patients are seizure free and without radiographically detected tumor recurrence.
Dysembryoplastic neuroepithelial tumors are a highly treatable cause of epilepsy in children. Excellent rates of complete tumor resection and seizure control with minimal morbidity can be attained using intraoperative ECoG and two-stage surgical procedures when appropriate.
Mohamed Samy A. Elhammady, David M. Benglis, Sanjiv Bhatia, David I. Sandberg and John Ragheb
Ventriculoatrial (VA) shunts remain the most used alternative to ventriculoperitoneal shunts in infants with hydrocephalus. The authors report a case of an acute VA shunt malfunction as a result of distal catheter displacement in an 18-month-old girl with partial anomalous pulmonary venous return. The child presented with respiratory compromise, and a chest radiograph revealed a lung infiltrate and normal position of the distal shunt catheter tip. Computed tomography demonstrated stable ventricle size in comparison with previous studies. As the patient's respiratory distress progressed, she required intubation, mechanical ventilation with high airway pressures and inspired oxygen concentrations, muscle relaxants, and sedation. A routine morning chest radiograph several days after admission revealed displacement of the distal catheter into the left innominate vein. Later that day the child's pupils were noted to be large and unreactive and a distal shunt malfunction was diagnosed. Complications of VA shunts and the presumed mechanism by which the catheter became displaced are discussed.
Guillermo De Angulo, Sushmita Nair, Vivian Lee, Ziad Khatib, John Ragheb and David I. Sandberg
Solitary eosinophilic granuloma (EG) of the calvaria is most commonly treated with surgical excision. The authors hypothesize that many solitary EGs will resolve without intervention, and observation may be a reasonable option. This study was undertaken to investigate that hypothesis.
The authors reviewed their institutional records and identified 14 cases of solitary calvarial EG. In 6 cases the patients underwent resection based on family and/or neurosurgeon preferences. A strategy of nonoperative management (purposeful observation) was chosen for the other 8 cases. The authors report the clinical course and imaging results in these 8 cases.
One of the 8 patients underwent surgery 2 months after presentation because of slight enlargement of the lesion and increasing pain. After a median follow-up period of 1 year (range 6–19 months), none of the other patients had required surgery. Five of these 7 patients had pain at presentation. Pain resolved completely in all 5. The remaining 2 remained asymptomatic. Complete resolution of pain was reported in the 5 patients who had pain at presentation. There was complete clinical resolution of the palpable soft-tissue lesion in all 7 cases. Complete radiographic resolution of the lesion was observed in 5 cases and near-complete resolution in the remaining 2.
Observation is a safe and reasonable approach in the management of solitary calvarial EG and may prevent unnecessary surgical interventions.