The two-hit hypothesis of neural injury in the wake of open neural tube defects suggests an opportunity for preservation of function and potential reversibility of early morphological changes in the fetus diagnosed with myelomeningocele. The Management of Myelomeningocele Study (MOMS) demonstrated reduced need for shunting and improved neurological function in patients treated in utero relative to postnatally, thereby offering level 1 evidence supporting fetal repair. Subsequent studies have offered additional information about urological, orthopedic, radiological, and maternal factors surrounding fetal repair. The quest for robust long-term neurocognitive and motor function data is underway and poised to shape the future of fetal repair. In addition, technical innovations such as fetoscopic surgery aim to minimize maternal morbidity while conferring the beneficial effects observed with open intrauterine intervention.
Fetal surgery for spina bifida
JNSPG 75th Anniversary Invited Review Article
Michael C. Dewan and John C. Wellons III
Management of Dandy-Walker complex–associated infant hydrocephalus by combined endoscopic third ventriculostomy and choroid plexus cauterization
Benjamin C. Warf, Michael Dewan, and John Mugamba
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.
Letter to the Editor. Endoscopic third ventriculostomy with choroid plexus cauterization
Daniel H. Fulkerson
The durability of endoscopic third ventriculostomy and ventriculoperitoneal shunts in children with hydrocephalus following posterior fossa tumor resection: a systematic review and time-to-failure analysis
Michael C. Dewan, Jaims Lim, Chevis N. Shannon, and John C. Wellons III
Up to one-third of patients with a posterior fossa brain tumor (PFBT) will experience persistent hydrocephalus mandating permanent CSF diversion. The optimal hydrocephalus treatment modality is unknown; the authors sought to compare the durability between endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunt (VPS) therapy in the pediatric population.
The authors conducted a systematic review of articles indexed in PubMed between 1986 and 2016 describing ETV and/or VPS treatment success/failure and time-to-failure rate in patients < 19 years of age with hydrocephalus related to a PFBT. Additionally, the authors conducted a retrospective review of their institutional series of PFBT patients requiring CSF diversion. Patient data from the systematic review and from the institutional series were aggregated and a time-to-failure analysis was performed comparing ETV and VPS using the Kaplan-Meier method.
A total of 408 patients were included from 12 studies and the authors' institutional series: 284 who underwent ETV and 124 who underwent VPS placement. The analysis included uncontrolled studies with variable method and timing of CSF diversion and were subject to surgeon bias. No significant differences between cohorts were observed with regard to age, sex, tumor grade or histology, metastatic status, or extent of resection. The cumulative failure rate of ETV was 21%, whereas that of VPS surgery was 29% (p = 0.105). The median time to failure was earlier for ETV than for VPS surgery (0.82 [IQR 0.2–1.8] vs 4.7 months [IQR 0.3–5.7], p = 0.03). Initially the ETV survival curve dropped sharply and then stabilized around 2 months. The VPS curve fell gradually but eventually crossed below the ETV curve at 5.7 months. Overall, a significant survival advantage was not demonstrated for one procedure over the other (p = 0.21, log-rank). However, postoperative complications were higher following VPS (31%) than ETV (17%) (p = 0.012).
ETV failure occurred sooner than VPS failure, but long-term treatment durability may be higher for ETV. Complications occurred more commonly with VPS than with ETV. Limited clinical conclusions are drawn using this methodology; the optimal treatment for PFBT-related hydrocephalus warrants investigation through prospective studies.
Letter to the Editor. Posterior fossa tumor resection and the failure of endoscopic third ventriculostomy and ventriculoperitoneal shunts
Chiazor U. Onyia
Surgical outcomes for pediatric moyamoya: a systematic review and meta-analysis
Krishnan Ravindran, John C. Wellons, and Michael C. Dewan
The optimal revascularization for pediatric moyamoya for reducing the incidence of future stroke events remains to be determined.
The authors conducted a search of PubMed, MEDLINE, Embase, and Web of Science electronic databases from inception until January 2019. The primary endpoint was the presence of a future ischemic stroke event. Secondary endpoints were angiographic outcomes as measured by postoperative Matsushima grade and clinical symptom persistence. Patients who underwent either direct or combined direct/indirect revascularization were classified into the direct cohort. Data from each study on presence of postoperative stroke events were used to generate standardized mean differences and 95% confidence intervals, which were combined using inverse variance-weighted averages of standardized mean differences in a random effects model.
Twenty-nine studies met the inclusion criteria for analysis, comprising 2258 patients (1011 males, mean age 8.3 ± 1.8 years) who were followed up clinically for an average of 71.4 ± 51.3 months. One hundred fifty-four patients underwent direct bypass alone, 680 patients underwent either direct or combined direct/indirect revascularization procedures, while 1424 patients underwent indirect bypass alone. The frequencies of future stroke events in patients undergoing direct bypass alone, combined bypass, or indirect bypass alone were 1 per 190.3 patient-years, 1 per 108.9 patient-years, and 1 per 61.1 patient-years, respectively, in each cohort. The estimated stroke rates were 9.0% with indirect revascularization, 4.5% with direct revascularization alone, and 6.0% with combined revascularization. A forest plot did not reveal any significant differences in the incidence of future stroke events or angiographic outcomes following direct-only, combined, or indirect-only revascularization.
Direct, indirect, and combined indirect/direct bypass techniques are all effective revascularization options for pediatric moyamoya disease. A relative paucity of granular studies—and inherent surgical selection bias—limits direct comparison between interventions. Suitably designed prospective cohort studies may be useful in identifying patients likely to receive benefit from specific procedures.
Scoliosis in myelomeningocele: epidemiology, management, and functional outcome
Nishit Mummareddy, Michael C. Dewan, Michael R. Mercier, Robert P. Naftel, John C. Wellons III, and Christopher M. Bonfield
The authors aimed to provide an updated and consolidated report on the epidemiology, management, and functional outcome of cases of myelomeningocele (MMC) in patients with scoliosis.
A comprehensive literature search was performed using MEDLINE, Embase, Google Scholar, and the Cochrane Database of Systematic Reviews on cases of MMC in patients with scoliosis between 1980 and 2016. The initial search yielded 670 reports. After removing duplicates and applying inclusion criteria, we included 32 full-text original articles in this study.
Pooled statistical analysis of the included articles revealed the prevalence of scoliosis in MMC patients to be 53% (95% CI 0.42–0.64). Slightly more females (56%) are affected with both MMC and scoliosis than males. Motor level appears to be a significant predictor of prevalence, but not severity, of scoliosis in MMC patients. Treatment options for these patients include tethered cord release (TCR) and fusion surgeries. Curvature improvement and stabilization after TCR may be limited to patients with milder (< 50°) curves. Meanwhile, more aggressive fusion procedures such as a combined anterior-posterior approach may result in more favorable long-term scoliosis correction, albeit with greater complication rates. Quality of life metrics including ambulatory status and sitting stability are influenced by motor level of the lesion as well as the degree of the scoliosis curvature.
Scoliosis is among the most common and challenging comorbidities from which patients with MMC suffer. Although important epidemiological and management trends are evident, larger, prospective studies are needed to discover ways to more accurately counsel and more optimally treat these patients.
Resection of a ventral intramedullary spinal cord ependymoma through an anterior cervical approach: illustrative case
Hani Chanbour, Patrick D. Kelly, Michael C. Topf, Michael C. Dewan, Peter J. Morone, and Scott L. Zuckerman
Although posterior myelotomy leaves patients with dorsal column deficits, few reports have explored the anterior cervical approach for cervical intramedullary tumors. The authors describe the resection of a cervical intramedullary ependymoma through an anterior approach with a two-level corpectomy and fusion.
A 49-year-old male presented with a C3–5 ventral intramedullary mass with polar cysts. Because of the ventral location of the tumor and the added benefit of avoiding a posterior myelotomy and dorsal column deficits, an anterior C4–5 corpectomy offered a direct route and excellent visualization of the ventrally located tumor. After a C4–5 corpectomy, microsurgical resection, and C3–6 anterior fusion with a fibular allograft filled with autograft, the patient remained neurologically intact. Magnetic resonance imaging (MRI) on postoperative day (POD) 1 confirmed gross-total resection. The patient was extubated on POD 2 and was discharged home on POD 4 with a stable examination. At 9 months, the patient developed mechanical neck pain refractory to conservative treatment and underwent a posterior fusion to address pseudarthrosis. MRI at 15 months showed no evidence of tumor recurrence with the resolution of neck pain.
An anterior cervical corpectomy provides a safe corridor to access ventral cervical intramedullary tumors and avoids posterior myelotomy. Although the patient required a three-level fusion, we believe the tradeoff of decreased motion compared to dorsal column deficits is preferred.
Cerebral hemorrhage in monozygotic twins with hereditary hemorrhagic telangiectasia: case report and hemorrhagic risk evaluation
Abbas Rattani, Michael C. Dewan, Vickie Hannig, Robert P. Naftel, John C. Wellons III, and Lori C. Jordan
The authors present a case of monozygotic twins with hereditary hemorrhagic telangiectasia (HHT) who experienced cerebral arteriovenous malformation (AVM) hemorrhage at a very young age. The clinical variables influencing HHT-related AVM rupture are discussed, and questions surrounding the timing of screening and intervention are explored. This is only the second known case of monozygotic HHT twins published in the medical literature, and the youngest pair of first-degree relatives to experience AVM-related cerebral hemorrhage. Evidence guiding the screening and management of familial HHT is lacking, and cases such as this underscore the need for objective and validated protocols.
Endoscopic third ventriculostomy with choroid plexus cauterization outcome: distinguishing success from failure
Michael C. Dewan, Jaims Lim, Clinton D. Morgan, Stephen R. Gannon, Chevis N. Shannon, John C. Wellons III, and Robert P. Naftel
Endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) offers an alternative to shunt treatment for infantile hydrocephalus. Diagnosing treatment failure is dependent on infantile hydrocephalus metrics, including head circumference, fontanel quality, and ventricle size. However, it is not clear to what degree these metrics should be expected to change after ETV/CPC. Using these clinical metrics, the authors present and analyze the decision making in cases of ETV/CPC failure.
Infantile hydrocephalus metrics, including bulging fontanel, head circumference z-score, and frontal and occipital horn ratio (FOHR), were compared between ETV/CPC failures and successes. Treatment outcome predictive values of metrics individually and in combination were calculated.
Forty-four patients (57% males, median age 1.2 months) underwent ETV/CPC for hydrocephalus; of these patients, 25 (57%) experienced failure at a median time of 51 days postoperatively. Patients experiencing failure were younger than those experiencing successful treatment (0.8 vs 3.9 months, p = 0.01). During outpatient follow-up, bulging anterior fontanel, progressive macrocephaly, and enlarging ventricles each demonstrated a positive predictive value (PPV) of no less than 71%, but a bulging anterior fontanel remained the most predictive indicator of ETV/CPC failure, with a PPV of 100%, negative predictive value of 73%, and sensitivity of 72%. The highest PPVs and specificities existed when the clinical metrics were present in combination, although sensitivities decreased expectedly. Only 48% of failures were diagnosed on the basis all 3 hydrocephalus metrics, while only 37% of successes were negative for all 3 metrics. In the remaining 57% of patients, a diagnosis of success or failure was made in the presence of discordant data.
Successful ETV/CPC for infantile hydrocephalus was evaluated in relation to fontanel status, head growth, and change in ventricular size. In most patients, a designation of failure or success was made in the setting of discordant data.