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P. Daniel McNeely and William J. Howes

Object. Periconceptual folic acid supplementation is effective in myelomeningocele prevention. The relationship between folic acid and lipomyelomeningocele (LMM) and the overall incidence of this occult form of spina bifida has never been studied. The objectives of this study were to determine the impact of dietary folic acid supplementation on the incidence of LMM and to measure its overall incidence.

Methods. In a retrospective population-based study the authors calculated the incidence of LMM in Nova Scotia between 1985 and 2001. Because of changes in public policy during this period, there are three intervals defined in relation to the treatment of the food supply with folic acid: 1) prior to folic acid fortification (1985–1994); 2) postsupplementation but prefortification (1995–1998); and 3) postfortification.

The overall incidence of LMM in Nova Scotia between 1985 and 2001 was 16 per 100,000 live births or one case per 6121 live births. Its incidence between 1985 and 1994 was 15 per 100,000 live births, and between 1995 and 1998 it was 12 per 100,000 live births (relative risk [RR] 0.82, 95% confidence interval [CI] 0.31–2.22; p = 0.7). Between 1999 and 2001, the incidence of LMM was 29 per 100,000 live births, which was not significantly different from that between 1995 and 1998 (RR 2.41, 95% CI 0.79–7.36; p = 0.11) or between 1985 and 1994 (RR 1.98, 95% CI 0.86–4.56; p = 0.1).

Conclusions. The overall incidence of LMM between 1985 and 2001 in Nova Scotia was 16 per 100,000 live births and has not been reduced by dietary folic acid supplementation. This finding provides epidemiological evidence that the embryogenesis of LMM is fundamentally different from that of myelomeningocele.

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Julia A. E. Radic, Michael Vincer and P. Daniel McNeely

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.

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Julia A. E. Radic, Michael Vincer and P. Daniel McNeely

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.

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Victoria Kuta, P. Daniel McNeely, Simon Walling and Michael Bezuhly

OBJECTIVE

Sagittal craniosynostosis results in a characteristic scaphocephalic head shape that is typically corrected surgically during a child’s 1st year of life. The authors’ objective was to determine the potential impact of being born with sagittal craniosynostosis by using validated health state utility assessment measures.

METHODS

An online utility assessment was designed to generate health utility scores for scaphocephaly, monocular blindness, and binocular blindness using standardized utility assessment tools, namely the visual analog scale (VAS) and the standard gamble (SG) and time trade-off (TTO) tests. Utility scores were compared between health states using the Wilcoxon and Kruskal-Wallis tests. Univariate regression was performed using age, sex, income, and education as independent predictors of utility scores.

RESULTS

Over a 2-month enrollment period, 122 participants completed the online survey. One hundred eighteen participants were eligible for analysis. Participants rated scaphocephaly due to sagittal craniosynostosis with significantly higher (p < 0.001) median utility scores (VAS 0.85, IQR 0.76–0.95; SG 0.92, IQR 0.84–0.98; TTO 0.91, IQR 0.84–0.95) than both monocular blindness (VAS 0.60, IQR 0.50–0.70; SG 0.84, IQR 0.68–0.94; TTO 0.84, IQR 0.67–0.91) and binocular blindness (VAS 0.25, IQR 0.20–0.40; SG 0.51, IQR 0.18–0.79; TTO 0.55, IQR 0.36–0.76). No differences were noted in utility scores based on participant age, sex, income, or education.

CONCLUSIONS

Using objective health state utility scores, authors of the current study demonstrated that the preoperatively perceived burden of scaphocephaly in a child’s 1st year of life is less than that of monocular blindness. These relatively high utility scores for scaphocephaly suggest that the burden of disease as perceived by the general population is low and should inform surgeons’ discussions when offering morbid corrective surgery, particularly when driven by aesthetic concerns.

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Gregory H. Jenkins, Nicola R. Smith and P. Daniel McNeely

A variety of surgical strategies are used to correct sagittal craniosynostosis. There is increasing experience with the use of endoscope-assisted techniques, although there is no consensus regarding the optimal technique. Pancraniosynostosis occurring after the surgical repair of single-suture craniosynostosis is an unusual complication. The authors describe the case of a nonsyndromic patient who underwent an endoscope-assisted strip craniectomy with the subsequent use of a helmet orthosis for correction of an isolated sagittal suture craniosynostosis. The patient's early postoperative course was uneventful. Pancraniosynostosis subsequently developed, requiring much more extensive surgical correction. Awareness of this potential complication is necessary to ensure its recognition and appropriate management.

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Abhaya V. Kulkarni, Iffat Shams, D. Douglas Cochrane and P. Daniel McNeely

Object

Quality of life (QOL) studies comparing treatment with endoscopic third ventriculostomy (ETV) and CSF shunting are very limited. The authors compared QOL outcomes following these 2 treatments in a large cohort of children with hydrocephalus by using multivariable statistical techniques to adjust for possible confounder variables.

Methods

The families of children between 5 and 18 years of age with previously treated hydrocephalus at 3 Canadian pediatric neurosurgery centers completed measures of QOL: the Hydrocephalus Outcome Questionnaire (HOQ) and the Health Utilities Index Mark 3 (HUI3). Medical records and recent brain imaging studies were reviewed. A linear regression analysis was performed with the QOL measures as the dependent variable. In multivariable analyses, the authors assessed the independent effect of initial hydrocephalus treatment (ETV vs shunting) while adjusting for the treatment center, current patient age, age at initial treatment, etiology of hydrocephalus, total number of days spent in the hospital for initial treatment, total number of days spent in the hospital for subsequent hydrocephalus complications, functioning ETV at follow-up assessment, frequency of seizures, and current ventricle size.

Results

Data from 603 patients were available for analysis. Fifty-eight patients had undergone ETV as their primary treatment and 545 had undergone CSF shunting. Endoscopic third ventriculostomy patients were slightly younger at the follow-up assessment, were older at the first surgery, and spent fewer days in the hospital for hydrocephalus complications. Without adjustment for any confounders, treatment with ETV was associated with significantly higher HOQ physical scores and HUI3 scores. After multivariable adjustment, however, there was no significant difference in any outcome measure. A functioning ETV at the time of the follow-up assessment was not significant in any model.

Conclusions

Treatment with either ETV or CSF shunting does not appear to be associated with any substantial difference in QOL outcome after adjusting for prognostic factors. Further study is needed to definitively determine the relative QOL benefit of either procedure, if any.

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Jennifer L. O. Merrimen, P. Daniel McNeely, Richard L. Bendor-Samuel, Matthias H. Schmidt and Robert B. Fraser

✓Amniotic band sequence is a disruption sequence having a broad spectrum of clinical manifestations ranging from partial amputations to major craniofacial and limb–body wall defects. Most reported cases of placental–cerebral adhesion pertain to patients with severe craniofacial defects who were either stillborn or who died a few hours after birth. The authors present a case of a male infant born with a placental–cerebral adhesion through a cranial defect. This adhesion was separated at birth, and duraplasty and primary scalp closure were performed. A detailed examination of the placenta revealed the presence of multiple amniotic bands. The case demonstrates that survival and normal early postnatal development are possible if the condition is treated promptly.

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John M. K. Mislow, Mark R. Proctor, P. Daniel Mcneely, Arin K. Greene and Gary F. Rogers

✓Calvarial osteolysis is a relatively rare finding in patients with neurofibromatosis. The authors describe two patients with neurofibromatosis Type 1 (NF1) and extensive cranial defects associated with underlying dural ectasia. Cranioplasties were performed in both patients with mixed results. One patient underwent cranioplasty using titanium mesh and methylmethacrylate. The other patient underwent an extensive cranioplasty with autogenous iliac crest grafting, and after initial healing has since had further bone resorption. In conclusion, the results of cranial reconstruction in patients with NF1 and dural ectasia are unpredictable because of the tendency for further bone resorption; techniques that protect the graft material from cerebrospinal fluid pulsations via a rigid mesh should be considered.