A population-based study of the outcome after small bowel atresia/stenosis in New South Wales and the Australian Capital Territory, Australia, 1992-2003

Purpose

The purpose of the study was to describe the incidence, epidemiology, and survival of infants with small bowel atresia/stenosis in New South Wales (NSW) and the Australian Capital Territory (ACT), Australia.

Methods

A population-based cohort study was conducted of infants diagnosed with small bowel atresia/stenosis in NSW and the ACT from 1992 to 2003. Data were obtained from the prospectively collated NSW and ACT Neonatal Intensive Care Units' data collection. Individual risk factors for mortality were assessed using the χ² test.

Results

The incidence of small bowel atresia/stenosis in NSW and the ACT was 2.9 per 10,000 births. Of 299 infants identified with small bowel atresia, 13 were stillborn. Of the 286 live born infants, most (52%) were delivered preterm (<37 weeks' gestation) with an 87% survival, whereas 48% were term with a 98% survival. More than half the infants (54%) had an associated birth defect. The overall mortality was 8%. Prematurity and low birth weight were identified as independent risk factors for mortality (P < .001).

Conclusions

This study of small bowel atresia/stenosis provides population-based outcomes for clinicians and families. It is important to investigate infants with small bowel atresia for associated birth defects. Although the mortality rate has decreased over the last 50 years, it remains substantial at 8% and is higher in premature and low birth weight infants.     

Surfactant does not improve survival rate in preterm infants with congenital diaphragmatic hernia

Background

Use of exogenous surfactant in congenital diaphragmatic hernia (CDH) patients is routine in many centers. The authors sought to determine the impact of surfactant use in the premature infant with CDH.

Methods

Data on liveborn infants with CDH from participating institutions were collected prospectively. Surfactant use and timing and outcome data were analyzed retrospectively. The authors evaluated the prenatal diagnosis patients as well. The outcome variable was survival to discharge. Odds ratios with confidence intervals were calculated.

Results

Five hundred ten infants less than 37 weeks’ gestation were entered in the CDH registry. Infants with severe anomalies (n = 80) were excluded. Information on surfactant use was available for 424 patients. Infants receiving surfactant (n = 209) had a greater odds of death than infants not receiving surfactant (n = 215, odds ratio, 2.17, 95% CI: 1.5 to 3.2; P < .01). In prenatally diagnosed infants with immediate distress, there was a trend toward worse survival rates among those receiving surfactant at 1 hour (52 patients) versus those that did not (93 patients; odds ratio, 1.93, 95% CI: 0.96 to 3.9; P < .07).

Conclusions

Surfactant, as currently used, is associated with a lower survival rate in preterm infants with CDH. The use of surfactant replacement in premature infants with CDH can be recommended only within the context of a randomized clinical trial.     

Preliminary observations of the use of high-frequency jet ventilation as rescue therapy in infants with congenital diaphragmatic hernia

Background/Purpose

Congenital diaphragmatic hernia (CDH) is associated with mortality of 10% to 50%. Several investigators have reported outcomes from centers using high-frequency oscillatory ventilation in their management of CDH, but there are no recent reports on use of high-frequency jet ventilation.

Methods

During the study period from January 2001 until August 2007, infants with CDH who were cared for at Duke University Medical Center received high-frequency jet ventilation as a rescue mode of high-frequency ventilation. We compared actual survival with predicted survival for infants treated only with conventional ventilation vs those rescued with high-frequency jet ventilation after failing conventional ventilation.

Results

Survival for the 16 infants that received high-frequency jet ventilation was predicted to be 63%; actual survival was 75%. Survival for the 15 infants that received only conventional ventilation was predicted to be 83%; actual survival was 87%. We observed no significant survival benefit for high-frequency jet ventilation, 8.0% (95 confidence interval, −22.0% to 38.1%; P = .59).

Conclusions

Although our sample size was small, we conclude with consideration of the absolute results, the degree of illness of the infants, and the biologic plausibility for the intervention that high-frequency jet ventilation is an acceptable rescue ventilation mode for infants with CDH.     

Corticosteroids for fetuses with congenital diaphragmatic hernia: can we show benefit?

Background and Purpose

Prenatal corticosteroids have been used in fetuses with congenital diaphragmatic hernia (CDH). We tested the utility of steroids by 2 methods.

Methods

Mothers carrying fetuses with CDH were randomized to 3 weekly doses of betamethasone or placebo starting at 34 weeks. Patients were followed until death or discharge. In a separate cohort study, the CDH Registry was used to compare infants who received prenatal steroids to those who had not.

Results

Thirty-four patients were enrolled at 7 centers, with 32 completing the trial. There were 15 placebo and 17 steroid patients. There was no difference in survival, length of stay, duration of ventilation, or oxygen use at 30 days. For the cohort study, we looked at infants older than 34 weeks who were born after October 2000 when data on prenatal steroids were collected. There were 1093 patients; 390 were evaluable, with 56 receiving steroids. There was no difference in survival, length of stay, ventilator days, or oxygen use at 30 days.

Conclusion

Neither the trial nor the CDH Registry suggest that late prenatal corticosteroids benefit fetuses with CDH. More than 1700 mothers and fetuses would need to be enrolled in a trial to show a 10% improvement in survival. It is unlikely that late steroids offer benefit to most fetuses with CDH.     

The relationship of pulmonary artery pressure and survival in congenital diaphragmatic hernia

Background/purpose

Pulmonary hypertension is an integral part of the pathophysiology of the respiratory failure associated with congenital diaphragmatic hernia. Hypothesizing that the evolution of the pulmonary hypertension would determine clinical outcome, the authors examined pulmonary artery pressures in relation to survival in their CDH patients.

Methods

The authors performed a retrospective chart review of all neonates with the CDH from 1991 to 2002 at their institution eliminating infants with complex congenital heart disease, prematurity, or limited treatment. Cardiac ECHO data were used to estimate pulmonary artery pressures as a ratio to systemic pressure. Statistical analyses of estimated pulmonary pressure ratios stratified by survival status and time were performed using χ² and Fisher’s Exact Test methods.

Results

Forty-seven full-term CDH infants with 428 cardiac ECHO evaluations were studied. Long-term survival rate was 74%. Forty-nine percent of patients had normal pulmonary artery pressure estimates within the first 3 weeks of life. All patients survived. Seventeen percent had persistent systemic or suprasystemic pressure estimates unrelieved by treatment interventions resulting in 100% mortality rate. Thirty-four percent had intermediate reductions in pressure estimates over time with 75% survival rate. Systemic pulmonary artery pressures were associated with decreased survival at all time-points when compared with normal-pressure survivors: week 1, 60% (P < .003); week 3, 38% (P < .007); week 6, 0% (P < .02).

Conclusions

The evolution of pulmonary hypertension is a critical determinant of survival in CDH patients with current treatment strategies. Three groups can be modeled with markedly different clinical performance patterns. Using serial cardiac ECHO examinations, pulmonary artery pressure estimations can be used to predict clinical outcome.     

Is contralateral exploration necessary in preterm infants with unilateral inguinal hernia?

Purpose

Contralateral inguinal exploration has been the preferred approach to preterm infants with unilateral inguinal hernia. There is no literature evidence to justify this. The aim of this prospective study is to analyze the incidence and to determine any risk factors for the development of contralateral metachronous hernia (MH) in the preterm when compared with term infants.

Methods

Preterm and term infants with unilateral inguinal hernia younger than 6 months who underwent surgery were included. Those infants who subsequently developed MH were analyzed.

Results

Two hundred sixty-six patients met the inclusion criteria. One hundred five were preterm and 161 were term. Ten preterm (10.31%) and 9 term infants (5.92%) presented subsequently with MH (P =.18). All but 1 were male. There was no relation between the side of hernia, presentation with incarceration, age at surgery, sex of the patient, and the development of MH. However, a trend toward a higher incidence of MH in very low birth weight and early gestational age preterms was noted.

Conclusion

There is no significant difference between the occurrence of MH in preterm when compared with term infants. Routine contralateral exploration in preterm infants with its associated risks is therefore not justified in unilateral hernia.     

Protocolized approach to the management of congenital diaphragmatic hernia: benefits of reducing variability in care

Purpose

Variable approaches to the care of infants with congenital diaphragmatic hernia (CDH) by multiple providers may contribute to inconsistent care. Our institution developed a comprehensive evidence-based protocol to standardize the management of CDH infants. This report reviews patient outcomes before and after the implementation of the protocol.

Methods

Retrospective chart review of CDH infants managed with individualized care (preprotocol group, January 1997-December 2001, n = 22) or on the protocol (Protocol group, January 2002-July 2009, n = 47). Survival and other categorical variables were compared by χ² analysis, and continuous variables were compared using 1-sided analysis of variance analysis, with significance defined as P < .05.

Results

Survival to discharge was significantly greater in the Protocol group (40/47; 85%) than the preprotocol group (12/22; 52%; P = .006), although mean gestational age, mean birth weight, and expected survival were not statistically different between the 2 groups. The use of supportive therapies, including high-frequency jet ventilation, inhaled nitric oxide, and extracorporeal life support, was similar between groups as well.

Conclusions

Since the implementation of a management protocol for infants with CDH, survival has improved significantly compared with expected survival and preprotocol controls. Reduction in the variability of care through use of an evidence-based protocol may improve the survival of CDH infants.     

Neonatal tracheal changes following in utero fetoscopic balloon tracheal occlusion in severe congenital diaphragmatic hernia

Objective

To report postnatal tracheal changes after in utero fetoscopic balloon tracheal occlusion in severe congenital diaphragmatic hernia (CDH).

Design

Case series.

Setting

Tertiary care center, CDH National Reference Center.

Patients

Seven consecutive newborn infants with severe CDH who underwent fetoscopic balloon tracheal occlusion.

Interventions

Flexible laryngotracheoscopy and histological aspect observed at necropsy in 2 nonsurvivors.

Results

All infants displayed elongation and relaxation of the posterior tracheal wall, intermittently obstructing the lumen during tidal breathing. Whereas the cartilage displayed adequate rigidity, the pars membranacea appeared both flaccid and loose. Tracheal widening (tracheomegaly) was seen in all cases. Histology (n = 2) pointed to structural modifications throughout the pars membranacea, that is, loss of epithelial folding and of longitudinal elastic network and focal muscular disruption. The cartilage displayed no visible or histologic changes. The above tracheal changes were not symptomatic, except for a barking cough during increased respiratory efforts.

Conclusions

Tracheal widening and intermittent collapse of the posterior wall of the trachea during tidal breathing was found in 7 consecutive newborns who underwent fetoscopic balloon tracheal occlusion, causing mild clinical symptoms. Endoscopic tracheal assessment might provide useful information in children with CDH, in particular, when they underwent in utero fetoscopic balloon tracheal occlusion.     

Perioperative course of pulmonary hypertension in infants with congenital diaphragmatic hernia: impact on outcome following successful repair

Background

Pulmonary hypertension (PH) associated with congenital diaphragmatic hernia (CDH) remains a significant cause of morbidity and mortality. For improved outcomes, preoperative stabilization is commonly practiced to control the PH in infants with CDH. Some CDH infants who have been considered stabilized and ready for surgery have nevertheless developed significant PH after surgical repair. In fact, the markers and consequences of the preoperative stabilization are still unclear. Therefore, we examine the perioperative course of PH to evaluate the impact of preoperative PH severity on mortality and morbidity of infants who underwent surgical repair of CDH.

Methods

The medical charts of all newborns (n = 49) with CDH who were treated at our institution between January 2000 and December 2009 were reviewed. General management and perioperative data were evaluated for all infants. The ratio of estimated pulmonary artery pressure to systemic pressure (P/S ratio or PSR), based on echocardiographic data, was used to assess the PH severity during the perioperative period.

Results

The overall survival rate in our group of infants with CDH was 71.4%. Of the 49 infants with CDH, 9 (18.4%) died during the preoperative phase. Forty infants underwent CDH repair at a median age of 3.5 days (range, 1-46 days). Five of these infants (12.5%) subsequently deteriorated and died after surgery. Using receiver operating characteristic curve analysis, a PSR cutoff value before surgery of 0.9 predicted mortality in CDH infants with a sensitivity of 100% and specificity of 84% and with an area under the curve of 0.93 (P = .002). Accordingly, 2 groups of infants with distinct outcomes were identified, as follows: a low-PSR cohort (PSR ≤0.9) with a survival rate of 100% and a high-PSR cohort (PSR >0.9) with a survival rate of 50% (P = .001). The rate of pneumothorax and the frequency of use of several inotropic agents after surgery were significantly higher in the high-PSR group (P = .001 and .007, respectively). Compared with low-PSR infants, infants with high PSR were operated on later (P = .03) and were postoperatively ventilated longer (P = .01). During the entire perioperative period, significant differences in the PH severity were noted between the 2 PSR groups. During the first week of life, infants in the high-PSR group had significantly higher PSRs than those in the low-PSR group (P = .001); and similar tendencies continued to be significant between the 2 groups after CDH repair (P = .04).

Conclusions

During the perioperative period, PH severity monitoring via the serial assessment of PSR is beneficial. Better outcomes were observed with a preoperative PSR less than or equal to 0.9, and this association needs to be confirmed by prospective study.     

Utility of PET, CT, and EUS to identify pathologic responders in esophageal cancer

Background

This study evaluates the utility of positron emission tomography (PET), endoscopic ultrasonography (EUS), and computed tomographic (CT) scans to predict pathologic response and survival following preoperative chemoradiation (CRT) in esophageal cancer.

Methods

One hundred three sequential patients with locoregionally advanced esophageal cancer, who were treated with CRT and esophageal resection between May 2001 and November 2003 at the University of Texas M.D. Anderson Cancer Center, were retrospectively reviewed. PET, EUS, and CT were performed before (pre) or after (post) CRT and before surgical resection. PET standardized uptake value (SUV) was defined as maximal uptake in primary tumor.

Results

Most patients were male (91 [88%]) with adenocarcinoma (90 [87%]). Pretreatment clinical stages were: IIA (42 [41%]), IIB (5 [5%]), III (50 [49%]), and IVA (6 [6%]). At the time of surgery, 58 patients (56%) had a pathologic response to CRT (≤10% viable cells). Post-CRT measurements that correlated with pathologic response were: CT esophageal wall thickness (13.3 vs 15.3 mm, p = 0.04), EUS mass size (0.7 vs 1.7 cm, p = 0.01) and PET SUV (3.1 vs 5.8, p = 0.01). Post-CRT PET SUV equal to or greater than 4 had the highest accuracy for pathologic response (76%). Univariate and multivariate Cox regression analysis demonstrated that a post-CRT PET SUV equal to or greater than 4 was an independent predictor of survival (HR, 3.5, p = 0.04).

Conclusions

The FDG-PET SUV is the most accurate noninvasive test to predict long-term survival after preoperative CRT and before surgical resection. Post-CRT FDG-PET cannot, however, rule out residual microscopic disease so esophagectomy should remain a therapeutic option even if the post-CRT imaging modalities are normal.     

Percent predicted lung volumes as measured on fetal magnetic resonance imaging: a useful biometric parameter for risk stratification in congenital diaphragmatic hernia

Purpose

This study was aimed at determining whether a new method of analyzing lung volumes on fetal magnetic resonance (MR) imaging could be used to predict the degree of pulmonary compromise in congenital diaphragmatic hernia (CDH).

Methods

Seventeen fetuses with CDH were prospectively evaluated by MR. Lung volumes were measured using an established technique and expressed as a percentage of the predicted lung volume (PPLV). Predicted lung volume was determined by subtracting measured mediastinal volume from total measured thoracic volume. The PPLV was correlated with postnatal outcomes. Statistical analyses were performed using the Mann-Whitney, Spearman correlation, or Fisher exact tests (P < .05).

Results

Of the 14 liveborn patients, the PPLV was 20.3±10.4 (gestational age at MR, 22.3 ± 5.7 weeks). The PPLV was significantly associated with extracorporeal membrane oxygenation (ECMO) use, hospital length of stay, and survival. All patients with a PPLV of less than 15 required prolonged ECMO support and had a 40% survival rate. In contrast, only 11% of patients with a PPLV of greater than 15 required ECMO, and survival was 100%.

Conclusion

The PPLV as measured by fetal MR imaging can accurately predict disease severity in CDH. A value of less than 15 is associated with a significantly higher risk for prolonged support and/or death, despite aggressive postnatal management.     

A risk-adjusted study of outcome and resource utilization for congenital diaphragmatic hernia

Purpose

Perinatal care of infants with congenital diaphragmatic hernia (CDH) is nonstandardized and costly. We examined a risk-adjusted cohort of patients with CDH and hypothesized that (1) among CDH survivors, the cost of the birth admission would be proportional to illness severity, and (2) this cost would be significantly higher compared with a matched non-CDH cohort.

Methods

A retrospective review of costs and outcomes for all patients with CDH admitted to British Columbia Children's Hospital between 1999 and 2003 was performed. Risk grouping of patients with CDH using a validated admission severity score (Score for Neonatal Acute Physiology-version II [SNAP-II]) was conducted, enabling comparison among infants surviving to discharge. Hospital costs were also compared with a contemporaneous, non-CDH cohort matched for birth weight and SNAP-II.

Results

Thirty-two infants with CDH were included, of who 5 required extracorporeal membrane oxygenation. Twenty-three (72%) infants survived to discharge, with an average length of stay of 46 days. Average cost per survivor to discharge was $54,102 (vs $13,722 for the non-CDH cohort; P < .05). After SNAP-II stratification of survivors into low-, moderate-, and high-risk groups, a significant cost difference was noted between the moderate- and low-risk and high- and low-risk groups, respectively.

Conclusions

Infants born with CDH require costly care and can be expected to consume disproportionate resources. Admission SNAP-II score correlates with total cost to discharge. Risk stratification and cost comparison of larger CDH populations may allow identification of cost-efficient treatment strategies.     

Survival of severe congenital diaphragmatic hernia has morbid consequences

Background/Purpose

Fetal tracheal occlusion (TO) was developed in an attempt to enhance prenatal lung growth and improve survival in fetuses with severe congenital diaphragmatic hernia (CDH). We conducted a randomized, controlled clinical trial in 24 fetuses with severe left CDH (liver herniated into the thorax and low lung-to-head ratio) to compare survival after endoscopic fetal TO vs standard perinatal care (control) and prospectively followed up the 16 survivors (9 control, 7 TO) to compare neurodevelopmental, respiratory, surgical, growth, and nutritional outcomes.

Methods

At 1 and 2 years old, subjects underwent evaluation consisting of medical and neurological history and physical, developmental testing, nutritional assessment, oxygen saturation and pulmonary function testing, chest radiograph, and echocardiogram. Growth and developmental measures were corrected for prematurity. Data were analyzed by Mann-Whitney rank sum test, Fisher's Exact test, and logistic and linear regression.

Results

Infants with TO were significantly more premature at birth (control vs TO, 37.4 ± 1.0 vs 31.1 ± 1.7 weeks; P < .01). Growth failure (z score for weight <2 SDs below mean) was severe in both groups at 1 year of age (control vs TO, 56% vs 86%; P = .31). There was considerable catch-up growth by age 2 years (growth failure: control vs TO, 22% vs 33%; P = .19). There were no differences in other growth parameters. There were also no differences in neurodevelopmental outcome at 1 and 2 years. Supplemental oxygen at hospital discharge was a significant predictor of worse neurodevelopmental outcome at 1 and 2 years old (P = .05 and P = .02, respectively). Hearing loss requiring amplification has been diagnosed in 44% of the group (control vs TO, 44% vs 43%; P = 1.0).

Conclusions

In this group of infants with severe CDH, there were no differences in outcome at 2 years old despite significant prematurity in the TO group. Oxygen supplementation at hospital discharge identified the most vulnerable group with respect to neurodevelopmental outcome, but all infants had significant growth failure, and hearing impairment is a substantial problem in this population. Severe CDH carries significant risk of chronic morbidity.     

Predicting the need for fundoplication at the time of congenital diaphragmatic hernia repair

Purpose

This study was conducted to examine the preoperative factors predictive of subsequent intervention for gastroesophageal reflux (GER) in children with congenital diaphragmatic hernia (CDH).

Methods

We conducted a retrospective cohort study on children who underwent repair of a CDH between January 1, 1995, and December 31, 2002 with follow-up continuing to September 1, 2005. Excluded in the study were children who died during their first admission, or who underwent fundoplication at the time of CDH repair. Univariate and multivariate logistic regressions were performed to examine preoperative factors predictive of subsequent intervention (fundoplication or gastrojejunal tube placement).

Results

Of 86 children, 13 underwent intervention (fundoplication, 10; gastrojejunal tube, 3) for GER. Univariate predictors included the following: right-sided CDH, use of nonconventional ventilation, liver within the chest, and patch closure of the CDH. However, only liver within the chest and patch closure of the CDH were significant predictors in a multiple variable analysis. The positive and negative predictive values of the multivariate model were 69.2% and 87.7%, respectively.

Conclusions

Infants with CDH who have liver within the chest or require patch closure of their hernia are at increased risk for subsequent intervention for GER. These children may represent a subpopulation that would benefit from fundoplication at the time of CDH repair.     

Right congenital diaphragmatic hernia: Prenatal assessment and outcome

Purpose

To understand the natural history of right congenital diaphragmatic hernia (CDH), the authors retrospectively reviewed 27 cases of right CDH that presented for prenatal evaluation or postnatal treatment.

Methods

Between 1995 and September 2002, a total of 194 cases of fetal CDH were evaluated and included 22 right-sided defects. The authors reviewed prenatal diagnostic studies (ultrasound scan, magnetic resonance imaging [MRI] echocardiography) and pre- and postnatal outcomes in these 22 cases of right CDH. Five additional cases of right CDH without a prenatal diagnosis were reviewed.

Results

The mean gestational age at evaluation was 26.1 weeks. The lung area to head circumference ratio (LHR) ranged from 0.32 to 2.5. In all cases, the fetal liver was herniated into the right chest. Associated anomalies were common. There were no karyotype abnormalities (17 of 22 tested). There were 4 terminations. Nine of the 18 (50%) continuing pregnancies had polyhydramnios, premature rupture of membranes, or preterm labor. The mean gestational age at birth was 36.8 weeks. One patient underwent tracheal occlusion at 27 weeks, and 2 patients died before postnatal repair. Overall survival rate (22 prenatal plus 5 postnatal diagnoses) was 19 of 27 (70%). Postnatal survival rate was 19 of 23 (83%). A Gore-tex (W. L. Gore and Associates, Flagstaff, AZ) patch was utilized in 14 of 21 neonates undergoing surgery. Twelve of 23 (52%) required extracorporeal membrane oxygenation (ECMO) with a 75% survival rate. Significant morbidity occurred in 10 of 19 survivors and included neurologic sequelae in 6 of 19 (32%).

Conclusions

MRI was helpful in the determination of liver position and confirmation of diagnosis. The high incidence of preterm complications, frequent need for ECMO, and high prevalence of comorbidities are indicative of the severity of this CDH population and warrant close prenatal surveillance and delivery at a tertiary care center with ECMO capability.     

Prenatal diagnosis of congenital diaphragmatic hernia: how should the babies be delivered?

Congenital diaphragmatic hernia (CDH) in many patients is diagnosed in utero. In these patients, the delivery can be planned as an elective cesarean, induced vaginal, or spontaneous vaginal delivery. The optimal method has yet to be determined.The aim of this study was to compare the outcome of patients with CDH delivered by different methods.

Methods

The Congenital Diaphragmatic Hernia Study Group was formed in 1995 to compile data on liveborn babies with CDH. Beginning in 2001, data concerning delivery were collected. By October 2005, delivery data were available on 1039 term and near-term infants without cardiac malformations. Five hundred forty-eight had a prenatal diagnosis and complete data on delivery (194 delivered by elective cesarean delivery, 121 by induced vaginal delivery, and 233 by spontaneous vaginal delivery). Patients delivered by a nonelective cesarean delivery were assigned to the delivery group for which they were originally planned.

Results

The overall survival among the 548 patients was 69%. It was highest in patients delivered by cesarean delivery (71%) followed by those delivered through induced vaginal delivery (70%) and spontaneous vaginal delivery (67%). The difference was not statistically significant.Fifty-three percent of all patients survived without extracorporeal membrane oxygenation (ECMO). This was significantly higher after cesarean delivery (60%) than after induced vaginal delivery (49%) or spontaneous vaginal delivery (49%) (P < .05).At 30 days of age, 45% of the patients delivered by cesarean delivery had survived and were on room air. This was slightly lower after induced vaginal delivery (37%) or after spontaneous vaginal delivery (37%), although not statistically significant.

Conclusion

Cesarean delivery was associated with a slightly better outcome in terms of a significantly higher survival without the use of extracorporeal membrane oxygenation, although there was no significant difference in total survival. Because this study was not randomized, it is not possible to determine if the elective cesarean delivery was the cause for the better outcome or if centers favoring elective cesarean delivery by protocol are more skillful in the management of patients with CDH. Mode of delivery for term and near-term infants with CDH deserves further prospective study.     

Survival rate in congenital diaphragmatic hernia: the experience of the Canadian Neonatal Network

Background/purpose

The Canadian Neonatal Network prospectively collects data from 17 pediatric hospitals accounting for 75% of all neonatal intensive care unit beds nationwide. The purpose of this study was to (1) analyze the database to compare actual survival rates of neonates with congenital diaphragmatic hernia (CDH) to predicted outcomes and (2) assess whether institutional CDH volume was associated with improved survival rate.

Methods

Actual survival rates for CDH patients born during a 22-month period were determined from the registry. Predicted survival rates were calculated using the CDH Study Group logistical regression equation. Actual survival rate was compared with predicted using χ² analysis. Survival rates were stratified by institutional CDH volume and compared using binomial analysis. P value of less than .05 was deemed statistically significant.

Results

Of approximately 20,500 neonatal admissions, 88 cases of CDH were recorded. Seventy-three of 88 (83%) neonates with CDH survived to discharge, whereas the predicted survival rate was 62% (P < .001). Three centers were considered “high-volume” centers with at least 12 CDH diagnoses, and 11 were considered “low-volume” centers. Actual CDH survival was significantly greater at high-versus low-volume centers (90% v 77%; P < .01).

Conclusions

From these prospective data, survival rates of Canadian neonates with CDH are significantly better than predicted by the CDH Study Group equation. Further, high-volume centers in Canada have a significantly higher CDH survival rate than low-volume centers.     

Patterns of cerebral injury in a series of infants with congenital diaphragmatic hernia utilizing magnetic resonance imaging

Background

Congenital diaphragmatic hernia (CDH) is a condition with significant mortality and respiratory morbidity. Long-term neurodevelopmental outcome is not yet well documented.

Methods

The authors examined all cases of CDH at their institution over a 12-month period and performed magnetic resonance imaging (MRI) of the brain postoperatively.

Results

Eight of 10 patients survived to have an MR brain scan. All 8 patients had cerebral injury detected on MRI, with a predominance of white matter and deep nuclear gray matter injury.

Conclusions

Cerebral injury detected with MRI is common in infants with congenital diaphragmatic hernia. The long-term significance of these MRI abnormalities is unknown, although the need for good long-term neurodevelopmental follow-up of infants with CDH is highlighted.     

Effect of hospital case volume on outcome in congenital diaphragmatic hernia: the experience of the Canadian Pediatric Surgery Network

Purpose

Despite advances in neonatal care of congenital diaphragmatic hernia (CDH), a significant variation exists in the mortality rates reported by individual centers. Center experience (reflected by case volume) may contribute to this variation in outcome. The aim of the study was to determine whether CDH mortality is affected by hospital case volume.

Methods

The CDH cases were abstracted from a disease-specific, 16-hospital, national network. Thirteen hospitals participated in this study. Anonymized hospitals were categorized as either high (>6 cases) or low-volume (≤6 cases) centers (HVC, n = 6; LVC, n = 7) according to the median case number per center. Risk-adjusted (Score for Neonatal Acute Physiology, version II [SNAP-II] score) mortality rates were compared between HVC and LVC.

Results

One hundred twenty-one CDH cases were identified. Overall in-hospital survival was 81%. No significant difference in SNAP-II score was observed between HVC and LVC. Of 97 (15%) infants treated in 6 HVC, 15 (15%) died compared to 8 (33%) of 24 in 7 LVC (P < .05).

Conclusion

Hospital case volume may be partially responsible for mortality rate variation in CDH. This result requires careful analysis, as case volume may merely be a surrogate for other predictive variables.