Fetal Repair of Open Neural Tube Defects: Ethical, Legal, and Social Issues.

Open neural tube defects or myelomeningoceles are a common congenital condition caused by failure of closure of the neural tube early in gestation, leading to a number of neurologic sequelae including paralysis, hindbrain herniation, hydrocephalus and neurogenic bowel and bladder dysfunction. Traditionally, the condition was treated by closure of the defect postnatally but a recently completed randomized controlled trial of prenatal versus postnatal closure demonstrated improved neurologic outcomes in the prenatal closure group. Fetal surgery, or more precisely maternal-fetal surgery, raises a number of ethical issues that we address including who the patient is, informed consent, surgical innovation and equipoise as well maternal assumption of risk. As the procedure becomes more widely adopted into practice, we suggest close monitoring of new fetal surgery centers, in order to ensure that the positive results of the trial are maintained without increased risk to both the mother and fetus.

Choosing Wisely Canada: Pediatric Neurosurgery Recommendations.

OBJECTIVES: Choosing Wisely Canada is an evidence-based, patient-focused, physician-led campaign to improve the delivery of medical care in Canada. The goal of this study was to produce Canadian recommendations for physicians treating patients with selected paediatric neurosurgery issues. METHODS: Paediatric neurosurgeons practicing in Canada were invited to participate. Suggestions were obtained using an anonymous questionnaire, and then ranked anonymously by the participating surgeons. Suggestions that received consensus from participants were discussed at the 2016 annual Canadian Pediatric Neurosurgery Study Group meeting. Suggestions that were not evidence based, or that would not have a substantive population impact were eliminated. All remaining suggestions were anonymously ranked by the group and the top five recommendations were submitted to Choosing Wisely Canada. RESULTS: The final five recommendations include: 1) don't order a computed tomography scan to investigate macrocephaly (order an ultrasound or magnetic resonance imaging scan); 2) don't image a midline dimple related to the coccyx in an asymptomatic infant or child; 3) don't use computed tomography scans for routine imaging of children with hydrocephalus. Fast sequence nonsedated magnetic resonance imaging scans or ultrasounds provide adequate information to assess patients without exposing them to radiation or an anesthetic; 4) don't recommend helmets for mild to severe positional flattening; 5) don't do routine surveillance imaging for incidentally discovered Chiari I malformation. CONCLUSIONS: Five Choosing Wisely Canada recommendations were produced to support care of patients with paediatric neurosurgical issues. While these recommendations will apply to the majority of children with the involved conditions, occasionally, deviation from these recommendations may be clinically indicated.

Motor Cortex Stimulation for Neuropathic Pain: A Randomized Cross-over Trial.

BACKGROUND: Chronic motor cortex stimulation (MCS) has been used to treat medically refractory neuropathic pain over the past 20 years. We investigated this procedure using a prospective multicentre randomized blinded crossover trial. METHODS: Twelve subjects with three different neuropathic pain syndromes had placement of MCS systems after which they were randomized to receive low ("subtherapeutic") or high ("therapeutic") stimulation for 12 weeks, followed by a crossover to the other treatment group for 12 weeks. The primary outcome measure was the pain visual analogue scale (VAS). Secondary outcome measures included McGill Pain Questionnaire (MPQ), Beck Depression Inventory-II, medication log, work status, global impression of change, and SF-36 quality of life scale. RESULTS: The trial was halted early due to lack of efficacy. One subject withdrew early due to protocol violation and five subjects withdrew early due to transient adverse events. Six subjects with upper extremity pain completed the study. There was no significant change in VAS with low or high stimulation and no significant improvement in any of the outcome measures from low to high stimulation. SF-36 role physical and mental health scores were worse with high compared to low stimulation (p=0.024, p=0.005). CONCLUSIONS: We failed to show that MCS is an effective treatment for refractory upper extremity neuropathic pain and suggest that previous studies may have been skewed by placebo effects, or ours by nocebo. We suggest that a healthy degree of skepticism is warranted when considering this invasive therapy for upper extremity pain syndromes.

Temporal trends of intraventricular hemorrhage of prematurity in Nova Scotia from 1993 to 2012.

OBJECT Intraventicular hemorrhage (IVH) and posthemorrhagic hydrocephalus (PHH) are common in premature newborns. The epidemiology of these conditions has been described, but selection bias remains a significant concern in many studies. The goal of this study was to review temporal trends in the incidence of IVH, PHH, and shunt surgery in a population-based cohort of very preterm infants with no selection bias. METHODS All very preterm infants (gestational age ≥ 20 and ≤ 30 weeks) born from 1993 onward to residents of Nova Scotia were evaluated by the IWK Health Centre's Perinatal Follow-Up Program, and were entered in a database. Infants born to residents of Nova Scotia from January 1, 1993, to December 31, 2012, were included in this study. The incidences of IVH, PHH, and shunt surgery were calculated, basic demographic information was described, and chi-square test for trends over time was determined. RESULTS Of 1334 successfully resuscitated very preterm infants who survived to their initial screening ultrasound, 407 (31%) had an IVH, and 149 (11%) had an IVH Grade 3 or 4. No patients with IVH Grade 1 or 2 developed PHH. The percentage of very preterm infants with IVH Grade 3 or 4 has significantly increased over time (p = 0.013), as have the incidence of PHH and shunt surgery (p = 0.001 and p = 0.011, respectively) in infants with Grade 3 or 4 IVH. The proportion of patients with PHH receiving a shunt has not changed over time (p = 0.813). CONCLUSIONS The increasing incidence of high-grade IVH-and PHH and shunt surgery in infants with high-grade IVH-over time is worrisome. This study identifies a number of associated factors, but further research to identify preventable and treatable causal factors is warranted.

Outcomes of intraventricular hemorrhage and posthemorrhagic hydrocephalus in a population-based cohort of very preterm infants born to residents of Nova Scotia from 1993 to 2010.

OBJECT Intraventicular hemorrhage (IVH) is a common complication of preterm birth, and the prognosis of IVH is incompletely characterized. The objective of this study was to describe the outcomes of IVH in a population-based cohort with minimal selection bias. METHODS All very preterm (≥ 30 completed weeks) patients born in the province of Nova Scotia were included in a comprehensive database. This database was screened for infants born to residents of Nova Scotia from January 1, 1993, to December 31, 2010. Among very preterm infants successfully resuscitated at birth, the numbers of infants who died, were disabled, developed cerebral palsy, developed hydrocephalus, were blind, were deaf, or had cognitive/language scores assessed were analyzed by IVH grade. The relative risk of each outcome was calculated (relative to the risk for infants without IVH). RESULTS Grades 2, 3, and 4 IVH were significantly associated with an increased overall mortality, primarily in the neonatal period, and the risk increased with increasing grade of IVH. Grade 4 IVH was significantly associated with an increased risk of disability (RR 2.00, p < 0.001), and the disability appeared to be primarily due to cerebral palsy (RR 6.07, p < 0.001) and cognitive impairment (difference in mean MDI scores between Grade 4 IVH and no IVH: -19.7, p < 0.001). No infants with Grade 1 or 2 IVH developed hydrocephalus, and hydrocephalus and CSF shunting were not associated with poorer outcomes when controlling for IVH grade. CONCLUSIONS Grades 1 and 2 IVH have much better outcomes than Grades 3 or 4, including a 0% risk of hydrocephalus in the Grade 1 and 2 IVH cohort. Given the low risk of selection bias, the results of this study may be helpful in discussing prognosis with families of very preterm infants diagnosed with IVH.