Congenital Heart Disease and Myelomeningocele in the Newborn: Prevalence and Mortality.

Myelomeningocele (MMC) and congenital heart disease (CHD) are independent risk factors for increased morbidity and mortality in the newborn period and each can require significant operations shortly after birth. Few studies have examined the impact of these combined lesions. We sought to examine the incidence of CHD in patients with MMC, and to evaluate length of stay (LOS), hospital charges, and mortality. Using the Texas Inpatient Public Use Data File, ~ 6.9 million newborn records between 1/1999 and 12/2016 were examined. Hospitalizations were classified as MMC without CHD (n = 3054), CHD without MMC (n = 72,266), and MMC with CHD (n = 171). The birth prevalence of CHD with MMC was 0.3/10,000 live hospital births, with 5% of patients with MMC having CHD, and 0.2% of those with CHD having MMC. There was increased LOS in patients with both MMC and CHD (median 15 days, IQR 5-31), compared to CHD without MMC (median 6 days, IQR 2-20) and MMC without CHD (median 8 days, IQR 1-14) and higher total hospital charges (median $95,007, IQR $26,731-$222,660) compared to CHD without MMC (median $27,726, $6463-$118,370) and MMC without CHD (median $40,066, IQR $5744-$97,490). Mortality was significantly higher in patients with MMC and CHD (22.2% compared to 3.1% in MMC without CHD and 4.1% in CHD without MMC). Significance remained when limiting for patients without genetic conditions or additional major birth defects. MMC with CHD in the newborn compared to either CHD or MMC alone is associated with longer LOS, higher charges, and increased mortality.

Factors Associated with Early Neonatal and First-Year Mortality in Infants with Myelomeningocele in California from 2006 to 2011.

OBJECTIVE: The aim of this study is to examine factors associated with early neonatal (death within first 7 days of birth) and infant (death during the first year of life) mortality among infants born with myelomeningocele. STUDY DESIGN: We examined linked data from the California Perinatal Quality Care Collaborative, vital records, and hospital discharge records for infants born with myelomeningocele from 2006 to 2011. Survival probability was calculated using Kaplan-Meier Product Limit method and 95% confidence intervals (CI) using Greenwood's method; Cox proportional hazard models were used to estimate unadjusted and adjusted hazard ratios (HR) and 95% CI. RESULTS: Early neonatal and first-year survival probabilities among infants born with myelomeningocele were 96.0% (95% CI: 94.1-97.3%) and 94.5% (95% CI: 92.4-96.1%), respectively. Low birthweight and having multiple co-occurring birth defects were associated with increased HRs ranging between 5 and 20, while having congenital hydrocephalus and receiving hospital transfer from the birth hospital to another hospital for myelomeningocele surgery were associated with HRs indicating a protective association with early neonatal and infant mortality. CONCLUSION: Maternal race/ethnicity and social disadvantage did not predict early neonatal and infant mortality among infants with myelomeningocele; presence of congenital hydrocephalus and the role of hospital transfer for myelomeningocele repair should be further examined. KEY POINTS: · Mortality in myelomeningocele is a concern. · Social disadvantage was not associated with death. · Hospital-based factors should be further examined.

A Decade of Experience with the Ovine Model of Myelomeningocele: Risk Factors for Fetal Loss.

INTRODUCTION: The ovine model is the gold standard large animal model of myelomeningocele (MMC); however, it has a high rate of fetal loss. We reviewed our experience with the model to determine risk factors for fetal loss. METHODS: We performed a retrospective review from 2009 to 2018 to identify operative factors associated with fetal loss (early fetal demise, abortion, or stillbirth). Operative risk factors included gestational age at operation, operative time, reduction of multiple gestations, amount of replaced amniotic fluid, ambient temperature, and method of delivery. RESULTS: MMC defects were created in 232 lambs with an overall survival rate of 43%. Of the 128 fetuses that died, 53 (42%) had demise prior to repair, 61 (48%) aborted, and 14 (11%) were stillborn. Selective reduction of multiple gestations in the same uterine horn was associated with increased fetal demise (OR 3.03 [95% CI 1.29-7.05], p = 0.01). Later gestational age at MMC repair and Cesarean delivery were associated with decreased abortion/stillbirth (OR 0.90 [95% CI 0.83-0.90], p = 0.03, and OR 0.37 [95% CI 0.16-0.31], p = 0.02), respectively. CONCLUSION: Avoiding selective reduction, repairing MMC later in gestation, and performing Cesarean delivery decreases the rate of fetal loss in the ovine MMC model.

The Impact of Prenatal Diagnosis of Selected Central Nervous System Anomalies for Prenatal Counselling Based on Significant Pregnancy Morbidity and Neonatal Outcomes.

BACKGROUND & OBJECTIVES: Prenatal screening and diagnostic imaging advances have led to an increased detection of CNS anomalies, including ventriculomegaly/congenital hydrocephalus (HCP), Dandy-Walker malformation (DWM), and myelomeningocele (MMC). Data on pregnancy outcomes and the impact of prenatal diagnosis on neonatal outcomes is limited. Our study aimed to provide data on obstetric and neonatal outcomes following prenatal diagnosis of one of three CNS anomalies. METHODS: A retrospective search of two databases in Alberta, Canada and NICU chart review of cases between 2001 and 2011was completed. Primary outcomes for each group were pregnancy outcome (live birth, stillbirth, and termination) and detection rate. Secondary outcomes were live and total birth prevalence, mode of delivery, GA at delivery, and length of NICU stay for inborn versus outborn patients. RESULTS: Prenatal detection rates were 91.6% (HCP), 83.4% (DWM), and 92.9 % (MMC). Termination rates were 30.2% (DWM), 34.2% (HCP), and 48.5% (MMC). Median GA (weeks, range) at diagnosis were 22 (17-38), 20 (12-37), and 20.5 (18-34) for HCP, DWM, and MMC, respectively. Rate of Caesarean section for fetal indication was 50.0%, 44.4%, and 42.9% for HCP, DWM, and MMC, respectively. Median NICU length of stay was longer for outborn patients than inborn patients and were as follows: (range) 33.0 (21-38) versus 8.5 (1-49) d (HCP), and 29 (29-57) versus 14 (2-75) d (DWM). CONCLUSION: This study provides termination rates, obstetric interventions, and NICU length of stay for prenatally-identified CNS anomalies. Collectively, this study assists prenatal counselling women with a fetus affected by a described CNS anomaly.

Nationwide survey of fetal myelomeningocele in Japan: Background for fetal surgery.

BACKGROUND: Fetal surgery for myelomeningocele (MMC) has yet not been performed in Japan, and the clinical background of fetal MMC in Japan remains poorly described. We examined the prenatal characteristics and perinatal outcomes of fetal MMC to prepare for the introduction of fetal surgery. METHODS: A nationwide questionnaire survey was conducted with regard to fetuses with MMC between January 2012 and December 2014 at perinatal centers in Japan. RESULTS: In 50 tertiary centers, 188 cases of MMC were identified, of which 126 (67%) were isolated cases. Only half of the cases involved referral to tertiary centers with a diagnosis of MMC. The median time point for a prenatal diagnosis was 26 weeks' gestation (range, 12-38 weeks); in 54% of cases the diagnosis occurred after 26 gestational weeks, which is over the limit for fetal surgery for MMC. Furthermore, in 22% of cases the diagnosis was made before 22 gestational weeks, and in three-quarters of these cases termination of pregnancy was selected. No fetal or neonatal deaths were observed in the isolated MMC group. MMC repair, ventriculoperitoneal shunt and clean intermittent catheterization were required after birth in 100%, 73% and 55% of isolated MMC cases, respectively. In total, 96% of the tertiary centers cared for <5 cases of fetal MMC per year. CONCLUSIONS: Gestational age at MMC diagnosis was late mid-gestation, therefore earlier detection is essential when considering fetal treatment of MMC in Japan. Although the survival rate was excellent, in three-quarters of isolated MMC cases ventriculoperitoneal shunt was required. Early detection and centralization of MMC cases at specialized centers should be considered.

MOMS Plus: Single-Institution Review of Outcomes for Extended BMI Criteria for Open Fetal Repair of Myelomeningocele.

BACKGROUND: In utero repair has become an accepted therapy to decrease the rate of ventriculoperitoneal shunting and improve neurologic function in select cases of myelomeningocele. The Management of Myelomeningocele Study (MOMS) trial excluded patients with a BMI >35 due to concerns for increased maternal complications and preterm delivery, limiting the population that may benefit from this intervention. OBJECTIVES: The aim of this study was to evaluate outcomes associated with extending the maternal BMI criteria to 40 in open fetal repair of myelomeningocele. METHOD: Retrospective review of fetal closure of myelomeningocele at a quaternary referral center between 2013 and 2016 with maternal BMI ranging from 35 to 40. RESULTS: Eleven patients with a BMI >35 were identified. The average BMI was 37. The average maternal age at the time of evaluation was 27 years. The average gestational age at fetal surgery was 24 weeks. Gestational age at birth was an average of 32 weeks. There was one perinatal death immediately following the fetal intervention. The shunt rate at 1 year was 45% (5/11 patients). CONCLUSIONS: In this single-institution review of expanded BMI criteria for fetal repair of myelomeningocele, we did not observe any adverse maternal outcomes associated with maternal obesity; however, the gestational age at delivery was 2 weeks earlier compared to the MOMS trial.

The Use of Indomethacin with Complete Amniotic Fluid Replacement and Classic Hysterotomy for the Reduction of Perinatal Complications of Intrauterine Myelomeningocele Repair.

OBJECTIVE: This study presented outcomes of classical hysterotomy with modified antiprostaglandin therapy for intrauterine repair of foetal myelomeningocele (fMMC) performed in a single perinatal centre. STUDY DESIGN: Forty-nine pregnant women diagnosed with fMMC underwent classic hysterotomy with anti-prostaglandin management, complete amniotic fluid replacement and high dose indomethacin application. RESULTS: The average gestational age (GA) at delivery was 34.4 ± 3.4 weeks, with no births before 30 weeks GA. There were 2 foetal deaths. Complete reversal of hindbrain herniation (HH), assessed in magnetic resonance imaging at 30-31 weeks GA was found in 72% of foetuses (mostly with HH grade I prior to fMMC repair). Our protocol resulted in rare use of magnesium sulphate (6%), low incidence of chorioamniotic membrane separation - chorioamniotic membrane separation (6%), preterm premature rupture of membranes - preterm premature rupture of membranes (pPROM; 15%) and preterm labour - preterm labour (PTL; 17%). The postoperative wound continuity of the uterus was usually stable (in 72% of patients), with low frequency of scar thinning (23%). CONCLUSION: Our protocol results in rare use of tocolytics, and the low occurrences of CMS, pPROM and PTL in relation to other study cohorts: Management of Myelomeningocele Study, Children's Hospital of Philadelphia, and Vanderbilt University Medical Centre.

A randomized trial of prenatal versus postnatal repair of myelomeningocele.

BACKGROUND: Prenatal repair of myelomeningocele, the most common form of spina bifida, may result in better neurologic function than repair deferred until after delivery. We compared outcomes of in utero repair with standard postnatal repair. METHODS: We randomly assigned eligible women to undergo either prenatal surgery before 26 weeks of gestation or standard postnatal repair. One primary outcome was a composite of fetal or neonatal death or the need for placement of a cerebrospinal fluid shunt by the age of 12 months. Another primary outcome at 30 months was a composite of mental development and motor function. RESULTS: The trial was stopped for efficacy of prenatal surgery after the recruitment of 183 of a planned 200 patients. This report is based on results in 158 patients whose children were evaluated at 12 months. The first primary outcome occurred in 68% of the infants in the prenatal-surgery group and in 98% of those in the postnatal-surgery group (relative risk, 0.70; 97.7% confidence interval [CI], 0.58 to 0.84; P<0.001). Actual rates of shunt placement were 40% in the prenatal-surgery group and 82% in the postnatal-surgery group (relative risk, 0.48; 97.7% CI, 0.36 to 0.64; P<0.001). Prenatal surgery also resulted in improvement in the composite score for mental development and motor function at 30 months (P=0.007) and in improvement in several secondary outcomes, including hindbrain herniation by 12 months and ambulation by 30 months. However, prenatal surgery was associated with an increased risk of preterm delivery and uterine dehiscence at delivery. CONCLUSIONS: Prenatal surgery for myelomeningocele reduced the need for shunting and improved motor outcomes at 30 months but was associated with maternal and fetal risks. (Funded by the National Institutes of Health; ClinicalTrials.gov number, NCT00060606.).

Fetal endoscopic myelomeningocele closure preserves segmental neurological function.

AIM: Our aim was to compare the effect of prenatal endoscopic with postnatal myelomeningocele closure (fetally operated spina bifida aperta [fSBA]) versus neonatally operated spina bifida aperta [nSBA]) on segmental neurological leg condition. METHOD: Between 2003 and 2009, the fetal surgical team (Department of Obstetrics, University of Bonn, Germany) performed 19 fetal endoscopic procedures. Three procedures resulted in fetal death, three procedures were interrupted by iatrogenic hemorrhages and 13 procedures were successful. We matched each successfully treated fSBA infant with another nSBA infant of the same age and level of lesion, resulting in 13 matched pairs (mean age 14 mo; SD 16 mo; f/m=1.6; female-16, male-10). Matched fSBA and nSBA pairs were compared in terms of segmental neurological function and leg muscle ultrasound density (MUD). We also determined intraindividual difference in MUD (dMUD) between myotomes caudal and cranial to the myelomeningocele (reflecting neuromuscular damage by the myelomeningocele) and compared dMUD between fSBA and nSBA infants. Finally, we correlated dMUD with segmental neurological function. RESULTS: We found that, on average, the fSBA group were born at a lower gestational age than the nSBA group (median 32 wks [range 25-34 wks] vs 39 wks [34-41 wks]; p=0.001) and experienced more complications (chorioamnionitis, premature rupture of the amniotic membranes, oligohydramnios, and infant respiratory distress syndrome necessitating intermittent positive-pressure ventilation). Neurological function was better preserved after fSBA than after nSBA (median motor and sensory gain of two segments; better preserved knee-jerk [p=0.006] and anal [p=0.032] reflexes). The dMUD was smaller in fSBA than in nSBA infants (mean difference 24, 95% confidence interval [CI] 15-33; p<0.05), which was associated with better preserved segmental muscle function. INTERPRETATION: Fetal endoscopic surgery is associated with spinal segmental neuroprotection, but it results in more complications. Before considering clinical implementation of fetal endoscopic myelomeningocele closure as standard care, the frequency of complications should be appropriately reduced and results assessed in larger groups over a longer period of time.

Stridor at birth predicts poor outcome in neonates with myelomeningocele.

BACKGROUND: Stridor, associated with vocal cord paralysis, in neonates with myelomeningocele (MMC) is a recognized symptom related to Chiari II malformation (CM). In most children, stridor appears after birth. Control of hydrocephalus, if present, and urgent decompression of the CM are recommended for treatment of these patients. Such management typically improves symptoms. Occasionally, stridor is present at birth and may be secondary, in part, to maldevelopment or prenatal ischemia of the brain stem, rather than treatable compression. There is minimal literature describing the outcome after Chiari decompression in this population. The purpose of this study was to review the outcomes of neonates with MMC and stridor at birth and compare it to MMC patients who develop stridor later. We hypothesized that unlike stridor which develops after birth, stridor at birth predicts a dismal outcome, despite aggressive surgical treatment. METHODS: Retrospective review of newborns with MMC and CM was performed in our institution from 1975 to 2010. Patients with stridor at birth and those who developed stridor later in infancy were identified. Outcomes were analyzed. Autopsy findings were reviewed when available. RESULTS: Six patients with MMC who presented with stridor at birth were identified. Five of these patients had decompression of CM and treatment of hydrocephalus, if present, within the first 2 weeks of life. All patients died: three within 1 month and the oldest at 62 months. In the three patients with autopsies, vernix caseosa meningitis was present. Eight patients presented with stridor later in infancy. CM decompression was performed in seven of them. One patient out of the seven with late onset of stridor died at 13 months after CM surgery. The mortality rate after CM decompression was worse in patients with stridor at birth than those presenting later with stridor (chi-square p = 0.015). CONCLUSIONS: In newborns with MMC, stridor at birth may predict dismal outcome despite CM decompression. Unlike the situation in neonates who develop stridor after birth, the outcome in those presenting with stridor at birth does not seem to be impacted by decompression of the CM. Nonoperative management may be an option to offer in this population. Additionally, vernix caseosa meningitis may contribute to the severe irreversible brain stem dysfunction in these newborns.

Adult consequences of spina bifida: a cohort study.

BACKGROUND: Patients with spina bifida potentially experience social isolation with limited employment opportunities and restriction of independent living, neurologic and intellectual impairment, and orthopaedic and urologic consequences of their condition. However, the degree of disability as these individuals age into adulthood has not been completely delineated. QUESTIONS/PURPOSE: In adults with spina bifida we determined the cause of death for those who had died, IQ, level of education, work history, independent living status, physical disabilities, and urologic status; we then identified surgical procedures that led to better adult function. METHODS: We retrospectively reviewed 84 individuals with myelomeningocele, examining social, cognitive, and physical disabilities. The minimum age at followup was 20 years (mean, 31 years; range, 20-64 years). RESULTS: Forty-two percent had normal IQs. Seventy percent never needed a neurosurgical shunt and those without shunts had higher IQs. Forty-four percent had regular education and 8% achieved college degrees. Fifty-six percent were unemployed. Thirty percent lived independently. Twenty-three percent were either married or divorced with nine normal offspring. Eighty-five percent dressed themselves, 65% shopped independently, 54% drove. Thirty-one percent were at the thoracic neurologic level (all used wheelchairs), 12% were at L1-L3 (all used a wheelchair except one), 33% were at L4-L5 (78% used a wheelchair at least part-time), and 24% were at S1 and below (all walked). Fifty-four percent experienced decubiti and as a consequence, four required major extremity amputations. Spinal fusions protected sitting balance, but hip surgery did not produce congruent hips and occasionally resulted in debilitating stiffness. Pressure sores resulted in partial foot amputations despite plantigrade feet. CONCLUSIONS: Our observations confirm the consequences associated with the physical and cognitive disabilities of patients with spina bifida. Many of these consequences can likely be mitigated by modern multidisciplinary care and special education to increase independence.

Clinical characteristics of neonatal meningomyelocele cases and effect of operation time on mortality and morbidity.

OBJECTIVE: We aimed to evaluate the clinical features, short-term prognosis, and effect of operation time on mortality and morbidity in neonates with meningomyelocele. METHODS: This prospective study was conducted between January 2006 and December 2008. Clinical features, defective area, existence of additional anomalies, operation time, and morbidity and mortality data were recorded. The effect of operation time on mortality and morbidity was evaluated. RESULTS: Patients (n = 28) were assessed during the study period. Mean birth weight, height, and head circumference were found to be 3,160 ± 582 g, 46.2 ± 3.3 cm, and 36.7 ± 4.1 cm, respectively. None of the mothers received folate supplementation. Hydrocephaly (n = 13), pes equinovarus (n = 4), pelvicalyceal ectasia (n = 4), acetabular dysplasia (n = 2), Chiari II malformation (n = 2), and hydronephrosis (n = 2) accounted for additional anomalies. The mean postnatal age for surgical operation was 3.2 ± 2.8 days; mean length of hospital stay was 10.2 ± 9.7 days. The cases operated after 72 h were hospitalized longer, received antibiotherapies for a longer duration and had higher total complication rates (p = 0.04, 0.02, and 0.01, respectively). CONCLUSION: Surgical operation of patients with meningomyelocele within 72 h after delivery significantly reduces not only the duration of hospitalization and antibiotic usage, but also complication rates.

Clinical outcome of V-Y flap with latissimus dorsi and gluteal advancement for treatment of large thoracolumbar myelomeningocele defects: a comparative study.

OBJECTIVE: Surgical repair and closure of myelomeningocele (MMC) defects are important and vital, as the mortality rate is as high as 65%-70% in untreated patients. Closure of large MMC defects is challenging for pediatric neurosurgeons and plastic surgeons. The aim of the current study is to report the operative characteristics and outcome of a series of Iranian patients with large MMC defects utilizing the V-Y flap and with latissimus dorsi or gluteal muscle advancement. METHODS: This comparative study was conducted during a 4-year period from September 2013 to October 2017 in the pediatric neurosurgery department of Shiraz Namazi Hospital, Southern Iran. The authors included 24 patients with large MMC defects who underwent surgery utilizing the bilateral V-Y flap and latissimus dorsi and gluteal muscle advancement. They also retrospectively included 19 patients with similar age, sex, and defect size who underwent surgery using the primary or delayed closure techniques at their center. At least 2 years of follow-up was conducted. The frequency of leakage, necrosis, dehiscence, systemic infection (sepsis, pneumonia), need for ventriculoperitoneal shunt insertion, and mortality was compared between the 2 groups. RESULTS: The bilateral V-Y flap with muscle advancement was associated with a significantly longer operative duration (p < 0.001) than the primary closure group. Those undergoing bilateral V-Y flaps with muscle advancement had significantly lower rates of surgical site infection (p = 0.038), wound dehiscence (p = 0.013), and postoperative CSF leakage (p = 0.030) than those undergoing primary repair. The bilateral V-Y flap with muscle advancement was also associated with a lower mortality rate (p = 0.038; OR 5.09 [95% CI 1.12-23.1]) than primary closure. In patients undergoing bilateral V-Y flap and muscle advancement, a longer operative duration was significantly associated with mortality (p = 0.008). In addition, surgical site infection (p = 0.032), wound dehiscence (p = 0.011), and postoperative leakage (p = 0.011) were predictors of mortality. Neonatal sepsis (p = 0.002) and postoperative NEC (p = 0.011) were among other predictors of mortality in this group. CONCLUSIONS: The bilateral V-Y flap with latissimus dorsi or gluteal advancement is a safe and effective surgical approach for covering large MMC defects and is associated with lower rates of surgical site infection, dehiscence, CSF leakage, and mortality. Further studies are required to elucidate the long-term outcomes.

Twenty years' experience with myelomeningocele management at a single institution: lessons learned.

OBJECTIVE: The authors reviewed 20 years' experience with the surgical management of open myelomeningocele in a well-defined retrospective cohort from a single large academic medical center. Their goal was to define the characteristics of a modern cohort of children with myelomeningocele to allow for evidence-based decision-making for the treatment of these patients. METHODS: After IRB approval was obtained, the authors queried an operative database maintained by the Department of Neurological Surgery at Children's Hospital of Pittsburgh for patients who underwent closure of a myelomeningocele between 1995 and 2015. They identified 153 infants, and a retrospective chart review was performed. RESULTS: Eighty-eight percent of the patients required placement of a ventriculoperitoneal shunt, and 15% of these patients acquired shunt-related infections. Eighteen percent of patients underwent Chiari malformation type II (CM-II) decompression. Sixteen percent of patients underwent a tethered cord release. Three percent of patients died within the 1st year of life. Predictors of an early demise included poor Apgar scores, large head circumference, and need for early CM-II decompression. Functional motor outcome was slightly better than predicted by anatomical level of defect. CONCLUSIONS: Myelomeningoceles represent a severe birth defect with life-threatening complications. The authors provide long-term follow-up data and insight into factors that contribute to early death.

Predictors of mortality in children with myelomeningocele and symptomatic Chiari type II malformation.

OBJECTIVE Chiari malformation type II (CM-II) in myelomeningocele is associated with a significant rate of mortality and poor outcome. Death is frequently heralded by the onset or progression of neurological symptoms. The authors sought to identify predictors of poor outcome and mortality within the myelomeningocele population at Children's Hospital of Pittsburgh. METHODS A retrospective chart and radiology review was performed on all infants who underwent primary closure of a myelomeningocele defect at Children's Hospital of Pittsburgh between the years of 1995 and 2015. Preoperative symptoms and signs leading to CM-II decompression, as well as operative details and postoperative changes in these symptoms and signs, were investigated in detail and correlated to outcome. Poor outcome was defined as death, stridor, or ventilator dependence. Deceased patients were separately assessed within this subgroup. RESULTS Thirty-two (21%) of 153 patients were found to have symptomatic CM-II. Of the 32 patients meeting inclusion criteria, 12 (38%) had poor outcomes. Eight patients (25%) died since initial presentation; 5 of these patients (16% of the overall cohort) died within the 1st year of life and 3 (9%) died during adolescence. Seven (88%) of the 8 patients who died had central apnea on presentation (p = 0.001) and 7 (44%) of the 16 patients who developed symptoms in the first 3 months of life died, compared with 1 (6.3%) of 16 who developed symptoms later in childhood (p = 0.04). The median Apgar score at 1 minute was 4.5 for patients who died and 8 for surviving patients (p = 0.006). The median diameter of the myelomeningocele defect was 5.75 cm for patients who died and 5 for those who survived (p = 0.01). The anatomical level of defect trended toward higher levels in patients who died, with 4 patients in that group having an anatomical level at L-2 or higher compared with 5 of the surviving patients (p = 0.001). The median initial head circumference for the 5 patients dying in the 1st year of life was 41.5 cm, versus 34 cm for all other patients (p = 0.01). CONCLUSIONS CM-II in spina bifida is associated with a significant mortality rate even when surgical intervention is performed. Death is more frequent in symptomatic patients presenting prior to 1 year of age. Late deaths are associated with symptom progression despite aggressive surgical and medical intervention. In this patient cohort, death was more likely in patients with symptomatic presentation during the first 3 months of life, low Apgar scores, large myelomeningocele defects, early central apnea, and large head circumference at birth.

Myelomeningocele: long-term neurosurgical treatment and follow-up in 202 patients.

OBJECT: In this paper the authors focus on the long-term management of myelomeningocele (MMC) and its associated conditions. METHODS: During a 25-year period, 220 consecutive patients with MMC underwent surgical repair. There were 203 cases (92%) of enlarged ventricles, but only 171 cases (78%) of hydrocephalus. Seven infants (3%) presented with early brainstem dysfunction; two improved after ventricular drainage, whereas five required craniocervical decompression. RESULTS: Overall, five neonates (2%) died; 215 patients (98%) were eventually discharged from the hospital and of these, 202 (94%) remained in the authors' outpatient program. During the follow-up (range 1-25 years, mean 9.3 years), 96 patients required shunt revision (63% of shunts); 16 patients (8%) experienced late brainstem dysfunction that was treated by shunt placement in eight and by craniocervical decompression in the other eight; 137 patients (68%) harbored hydrosyringomyelia, but only six required surgical treatment; 40 patients (20%) presented symptoms of tethered cord, but surgical detethering was indicated in just 22 patients (11%). There were five deaths (2%), and severe adjunctive neurological morbidity was reported in 18 patients (9%) (owing to various causes). "Social" results have been evaluated by extrapolating 38 patients older than 15 years of age whose initial lesions were below L-2: 37 (97%) of these patients were fully independent, had "social urinary continence," and attended normal schools. CONCLUSIONS: Currently, many patients with MMC reach adulthood and social continence; self-care may be expected in a large percentage of cases. Nevertheless, there are many associated neurological conditions that have to be faced, and a coordinated network of care remains necessary throughout the patient's life. Moreover, these patients often present with so many peculiarities that indications for treatment, choice of proper techniques, and results are not always clear and evident.

The transition from child to adult in neurosurgery.

The transition from child to adult is a growing concern in neurosurgery. Data documenting long-term follow-up are necessary to define this population's healthcare needs. In order to evaluate the problems posed by the child-to-adult transition in neurosurgery, we have studied the neurological, functional and social outcome of patients treated in our department for tumor of the central nervous system, hydrocephalus or myelomeningocele, and followed beyond the age of eighteen years. A large number of patients suffered from chronic ailments, either sequelae of their initial disease, or delayed complications of their initial treatment, with significant morbidity. The mortality during adulthood was 4.6% in the tumor group, 1.1% in the hydrocephalus group, and zero in the spina bifida group. The proportion of patients employed in normal jobs was 35.6, 18.7 and 11.5% for tumors, hydrocephalus and myelomeningocele respectively. IQ score and performance at school generally overestimated the capacity for social integration. Based on these data and on the available literature, we tried to identify the problems and devise solutions for the management of the transition from child-to-adulthood transition. Many problems present during childhood persist to adulthood, some of which are made more acute because of a more competitive environment, the lack of structures and inadequate medical follow-up. The transition from child to adult must be managed jointly by pediatric and adult neurosurgeons. More clinical research is required in order to precisely evaluate the problems posed by adult patients treated during childhood for the different neurosurgical diseases. Based on these data, a concerted trans-disciplinary approach is necessary, tailored to the specific needs of patients suffering from different diseases.

The mortality rate after hospital discharge in patients with myelomeningocele decreased after implementation of mandatory flour fortification with folic acid.

OBJECTIVE:: To evaluate the mandatory folic acid fortification of flour on mortality rates after the hospital discharge of children born with myelomeningocele, the most affected age group and the most frequent cause of death. METHODS:: A retrospective study of 383 children born with myelomeningocele from January 1990 to December 2013 in a high-fetal-risk reference hospital. RESULTS:: A total of 39 patients died (10.1%),of which 23 (6%) died after discharge. Most children who died were younger than 12 months of age. The most frequent cause of death was infection of the central nervous system, followed by urinary tract sepsis and infections of the respiratory system. Symptomatic Chiari II malformation was the most frequent comorbidity factor. CONCLUSION:: Although there was no significant difference in infant mortality before and after folic acid fortification, there was a significant reduction in deaths after hospital discharge in babies born after implementation of mandatory folic acid fortification.

Correlation of mortality with lesion level in patients with myelomeningocele: a population-based study.

OBJECTIVE The aim of this study was to assess myelomeningocele mortality, correlate these findings to lesion level, and investigate mortality evolution. METHODS From the population-based western Denmark myelomeningocele database, the authors extracted the records of 187 patients born between January 1, 1970, and July 1, 2015. Patients were categorized according to their most rostral lesion level into cervical, thoracic, lumbar, or sacral groups. Furthermore, patients were categorized based on their birth dates (1970-1979, 1980-1989, and 1990-2015). Mortality data was extrapolated from the university hospital's electronic charts, which are based on the Danish Civil Registration System, and compared according to mortality, lesion level, and date of birth. Data were also extracted from nationwide Danish registers. Additionally, the authors divided the patients according to date of birth before or after the advent of prenatal detection (2004), and compared mortality rates of these two groups. RESULTS A thoracic lesion level was associated with a significantly higher mortality rate (p = 0.01). Two patients had a cervical lesion and were alive at the end of follow-up. The mortality rate decreased over time, although not significantly for the subsequent time periods. Prenatal detection did not affect mortality. CONCLUSIONS The presented data suggest increased mortality with ascending lesion level in patients with myelomeningocele, except for patients with cervical lesions. The mortality rate improved over time, suggesting that modern treatment modalities improve survival in patients with myelomeningocele.

Ten-year survival of Ugandan infants after myelomeningocele closure.

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.