Fetal lung-head ratio is not related to outcome for antenatal diagnosed congenital diaphragmatic hernia

We asked if fetal lung-to-head ratio (LHR) of 1.0 or lower or liver herniation had a statistical effect on survival or need for extracorporeal membrane oxygenation (ECMO), compared with LHR above 1.0 in patients with congenital diaphragmatic hernia (CDH).

Methods

Antenatal records of all patients diagnosed with CDH from January of 2002 to June of 2005 were examined. Inclusion criteria were isolated left-sided CDH and absence of significant cardiac or other anomalies/syndromes, treated solely at this institution. Lung-to-head ratio values were compared based on the value currently proposed for fetal intervention: LHR of 1.0 or lower vs LHR above 1.0. Outcome was assessed as survival (discharge to home) or need for ECMO.

Results

Twenty-eight patients met inclusion criteria. Overall survival was 86% (24/28). Postnatal survival in fetuses with LHR of 1.0 or lower (8/11) was not statistically different from LHR above 1.0 (16/17) (73% vs 94%, P = .114). The need for ECMO in the group with LHR of 1.0 or lower (3/11) was not significantly different from those with LHR above 1.0 (1/17) (27% vs 6%, P = .114). Herniation of the fetal liver into the chest did not affect survival or need for ECMO (P = .228).

Conclusion

Neither LHR of 1.0 or lower nor liver herniation identified a risk factor significant enough to warrant fetal intervention. Multicenter studies may be more appropriate to investigate this clinical problem.     

Current consequences of prenatal diagnosis of congenital diaphragmatic hernia

Background

Today, the diagnosis of congenital diaphragmatic hernia (CDH) can readily be made in the prenatal period during screening ultrasound examination. Patients ought to be referred to rule out associated anomalies, and in isolated cases, prognosis is poor when the liver is intrathoracic and the lung-to-head ratio (LHR) is less than 1. In these patients, prenatal intervention aiming to reverse pulmonary hypoplasia can be considered.

Methods

We present our current algorithm for counselling patients presenting with CDH. Patients with a poor prognosis are offered percutaneous fetal endoluminal tracheal occlusion (FETO) with a balloon, inserted at 26 to 28 weeks. We report on the evolution of technique and results in a consecutive homogeneous case series and compare outcome in cases with similar severity managed in the postnatal period.

Results

Within a period of 28 months, FETO was performed between 26 and 28 weeks in 24 fetuses with severe left-sided CDH. Under general (n = 5), epidural (n = 17) or local (n = 2) anesthesia, the balloon was successfully positioned at first surgery (23/24) with a mean operation time of 20 minutes (range, 3-60 minutes). There were no serious maternal complications or direct fetal adverse effects. In the first 2 weeks after FETO, LHR increased from 0.7 to 1.7. Premature prelabour rupture of the membrane (PPROM) occurred in 16.7% and 33.3% at 28 and 32 weeks or earlier, respectively. Gestational age at delivery was 33.5 weeks. Patency of airways was restored either in the prenatal (n = 12) or perinatal period (n = 12). Early (7 days) and late (28 days) survival, and survival at discharge were 75% (18/24), 58.3% (14/24) and 50% (12/24), respectively. Half of nonsurvivors (n = 6) died of pulmonary hypoplasia and hypertension, in combination with PPROM and preterm delivery (n = 4) and balloon dislodgement (n = 2), which coincided with a short tracheal occlusion (TO) period (12 days). In the other 6, TO period was comparable to that in the 12 survivors (47 vs 42 days, respectively). In that group of 6 babies, only 2 died of pulmonary problems. Late neonatal survival (28 days) was higher with prenatal vs perinatal balloon retrieval 83.3% vs 33.3% (P = .013). In a multicentre study validating the criteria, survival till discharge in 37 comparable cases was 9% (3/32) and 13% (5/37) of parents opted for termination.

Conclusion

Fetuses with isolated left-sided CDH, liver herniation, and LHR of less than 1 have a poor prognosis. Percutaneous FETO is minimally invasive and may improve the outcome in these highly selected cases. Airways can be restored before birth, allowing vaginal delivery and return to the referring tertiary unit and may improve survival rate. The procedure carries a risk for PPROM, although that may decrease with experience.     

Factors influencing survival in newborns with congenital diaphragmatic hernia: the relative role of timing of surgery

Purpose

Controversy persists regarding the factors influencing survival in patients with congenital diaphragmatic hernia (CDH), in particular, the role of timing of surgery. The authors therefore sought to determine such factors and to assess the relative role of timing of surgery on outcome.

Methods

All CDH newborns 1991 through 2002 (n = 111) were divided into those undergoing repair before (“early” n = 35), or after (“late” n = 76) 48 hours. A multivariate analysis was performed to determine the relative impact of various factors on survival rate.

Results

Overall survival rate was 64%. There was no effect on survival of heart rate, temperature, systolic blood pressure, age, extracorporeal membrane oxygenation use, mesh use, infections, or intracranial hemorrhage, and there was no difference between early (68%) or late (62%) repair (P = .2). Initial pco₂ greater than 50, po₂ less than 40, cardiac defects, or renal failure significantly decreased survival rate.

Conclusions

Significant factors influencing survival rate in patients with CDH include cardiac defects, renal failure, and the initial blood gases and not the timing of surgery. CDH repair should be based on the optimization of clinical parameters as opposed to a specific time period to improve outcome.     

Fetal tracheal occlusion for severe congenital diaphragmatic hernia in humans: a morphometric study of lung parenchyma and muscularization of pulmonary arterioles

Objective

The aim of this study is to examine structure of lung parenchyma (Pp) and muscularization of pulmonary arterioles (PAs) in human fetuses that underwent tracheal occlusion (TO) therapy for severe congenital diaphragmatic hernia (CDH).

Material

Fifteen fetuses underwent TO, with 5 survivors (Am J Obstet Gynecol. 2000:183;1059-1066). Paraffin-embedded lung specimens from 7 of 10 nonsurvivors (CDH-TO) and 6 age-matched fetuses (CDH) were available for morphometric analysis, which included measurements of point fraction of lung Pp and surface density. The PAs were categorized according to external diameter (<70 μm and 70 ≤ 140 μm). Percent medial wall thickness (%MWT) was calculated by dividing raw measurements of MWT by external diameter.

Results

Gestational age at TO was 27.6 ± 0.9 weeks with a mean duration of TO of 32.6 ± 6.8 days. Gestational age at delivery (CDH-TO 31.9 ± 0.9 vs CDH 35.4 ± 1.8 weeks; P = .18) and postnatal survival time (CDH-TO 20.5 ± 6.0 vs CDH 18.6 ± 7.8 days; P = .85) were not significantly different between groups. Tracheal occlusion significantly increased the lung-to-body weight ratio (CDH-TO 13.0 ± 2.2 vs CDH 6.6 ± 0.9; P = .02). Tracheal occlusion tended to decrease right-lung Pp (CDH-TO 54.6% ± 2.6% vs CDH 65.7% ± 5.9%; P = .05), whereas left-lung Pp was not different between groups (CDH-TO 63.0% ± 3.5% vs CDH 66.7% ± 4.1%; P = .51). Surface density of airspaces was not different between groups in either left (CDH-TO 171.3 ± 16.1 cm⁻¹ vs CDH 151.1 ± 8.1 cm⁻¹; P = .34) or right (CDH-TO 172.0 ± 10.6 cm⁻¹ vs CDH 160.8 ± 3.6 cm⁻¹; P = .33) lungs. The %MWT in small and large PA was similar between groups.

Conclusions

Open prenatal TO in human fetuses increased lung growth, as evidenced by an increase in lung weight, but did not improve parenchymal structure or muscularization of PAs.     

Long-term neurodevelopmental outcomes of congenital diaphragmatic hernia survivors not treated with extracorporeal membrane oxygenation

Background/Purpose

Although there has been a marked improvement in the survival of children with congenital diaphragmatic hernia (CDH) in the past 2 decades, there are few reports of long-term neurodevelopmental outcome in this population. The present study examined neurodevelopmental outcomes in 10- to 16-year-old CDH survivors not treated with extracorporeal membrane oxygenation (ECMO).

Methods

Parents of 27 CDH survivors completed questionnaires assessing medical problems, daily living skills, educational outcomes, behavioral problems, and executive functioning. Fifteen CDH survivors and matched full-term controls completed standardized intelligence, academic achievement, phonological processing, and working memory tests.

Results

Non-ECMO-treated CDH survivors demonstrated high rates of clinically significant difficulties on standardized academic achievement measures, and 14 of the 27 survivors had a formal diagnosis of specific learning disability, attention deficit hyperactivity disorder, or developmental disability. Specific problems with executive function, cognitive and attentional weaknesses, and social difficulties were more common in CDH patients than controls. Perioperative hypocapnia was linked to executive dysfunction, behavioral problems, lowered intelligence, and poor achievement in mathematics.

Conclusions

Non-ECMO-treated CDH survivors are at substantial risk for neurodevelopmental problems in late childhood and adolescence.     

Predicting perinatal outcome in isolated congenital diaphragmatic hernia using fetal pulmonary artery diameters

Objective

The aim of the study was to evaluate the potential of fetal pulmonary artery (PA) diameters to predict perinatal death and pulmonary arterial hypertension (PAH) in congenital diaphragmatic hernia (CDH).

Study Design

In this prospective observational study, observed PA (main, right, and left) diameters were measured at the level of the 3 vessels in 21 fetuses with isolated CDH and in 85 controls at 22 to 36 weeks. The observed/expected (o/e) diameters of the main, contralateral, and ipsilateral PAs were calculated by comparing these measurements with reference values obtained in our previous study and correlated with perinatal death and postnatal PAH.

Results

The o/e PA diameters were significantly reduced in fetuses with CDH compared to controls (P < .001) and in fetuses with CDH who died (P < .050). However, there was no significant association between PA diameters and PAH (P ≥ .050).

Conclusions

The PA diameters might be useful to predict perinatal death in isolated CDH but not postnatal PAH, suggesting that PA diameters are probably related to the severity of pulmonary hypoplasia.     

Modulating effect of a selective endothelin A receptor antagonist on pulmonary endothelin system protein expression in experimental diaphragmatic hernia

Background/Purpose

Previously, we reported that perinatal administration of atrasentan, a selective endothelin A receptor (ET_A) antagonist, provided a beneficial effect on the cardiopulmonary profile under short-term conditions in newborn lambs with surgically induced congenital diaphragmatic hernia (CDH). We hypothesized that changes in the hemodynamic profile that we observed at birth in treated animals could be influenced by pulmonary modulation of the endothelin (ET) system.

Methods

The effect of atrasentan on protein expression levels of ETs and ET receptors (ET_A and ET_B receptor) was investigated by immunohistochemistry in lung tissues of untreated control (n = 3), treated control (n = 6), untreated CDH (n = 6), and treated CDH newborn lambs (n = 8).

Results

Right lung tissue of treated control lambs showed significantly higher ET_A protein expression levels in both vascular adventitia and airway epithelia when compared with that of untreated control lambs (P < .05). In contrast, protein expression levels of ET_A and ET _B receptor were significantly lower in the vascular smooth muscle cells among other tissue subcompartments of the right lung of treated CDH newborn lambs vs CDH lambs (P < .02 and P = .005, respectively).

Conclusions

We speculate that rapid pulmonary modulation of ET system protein expression levels by atrasentan results from an indirect effect possibly dependent on ventilation and/or perfusion. In CDH groups, this could contribute to the beneficial effect of the treatment.     

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.     

Right-sided congenital diaphragmatic hernia: high utilization of extracorporeal membrane oxygenation and high survival

Purpose

The purpose of the study was to assess the treatment strategies and outcome of right-sided congenital diaphragmatic hernia (R-CDH), particularly extracorporeal membrane oxygenation (ECMO).

Methods

We reviewed the cases of 42 patients treated for R-CDH at our institution from 1991 to 2006. We gathered demographic information, documented ECMO use and the type of surgical repair, and compared outcomes with predicted survival as calculated by the CDH Study Group's equation.

Results

Of the 35 patients included in our statistical analysis (7 were excluded), 12 (34%) were born at our institution, all of whom were prenatally diagnosed with R-CDH. Nineteen patients (54%) required ECMO therapy. Extracorporeal membrane oxygenation was initiated after repair of the R-CDH in 2 patients (11%). Of those patients who went on ECMO before repair, 4 patients (21%) were repaired on ECMO, 9 patients (47%) underwent repair after ECMO, and 4 patients (21%) underwent ECMO but died before their R-CDH could be repaired. Primary repair of the diaphragm was possible in 15 cases (56%), and primary closure of the abdominal incision was possible in 15 of the 23 open repairs (65%). The mean predicted survival for all 35 patients was 63%, whereas 28 (80%) actually survived. Logistic regression showed a significant association between the presence of cardiac defects and mortality (odds ratio = 0.008, P = .014).

Conclusions

Our data suggest that patients with R-CDH have high ECMO utilization and may experience greater relative benefit from ECMO as evidenced by their higher-than-expected overall survival. Extracorporeal membrane oxygenation may be found to have a distinctive role in managing R-CDH. More high-powered series are needed to elucidate differences between R-CDH and left-sided CDH that may dictate alternate forms of management.     

Definitive surgical management of antenatally diagnosed exomphalos

Background/Purpose

The management of exomphalos is controversial with many centers in the United Kingdom and elsewhere advocating a conservative nonsurgical approach for the larger examples. Nevertheless, this approach is not without problems or complication. The aim of the study was to ascertain the outcome of all infants with an antenatally diagnosed exomphalos treated recently at our institution using a policy of aggressive abdominal wall closure.

Methods

This is a retrospective review of all infants with exomphalos treated from January 1995 to September 2002.

Results

There were 35 infants, all of whom underwent surgery. These were separated into 3 groups: group A (all exomphalos minor) underwent primary closure (n = 11), group B (exomphalos major) underwent primary closure (n = 13), and group C (exomphalos major) underwent staged closure involving a silo (n = 11). Infants in group C had a lower birth weight (P = .05) and were less mature (P = .06). They required longer periods of ventilation (P < .001), a longer hospital stay (P = .001), and a longer period to achieve full enteral feeds (P < .001). Overall survival was 34 (97%) of 35 infants. One premature infant who was born with a ruptured exomphalos sac (birth weight, 862 g) died of nonsurgical complications (sepsis and respiratory failure) early after the creation of a silo.

Conclusions

An aggressive surgical approach in infants with exomphalos is a safe option resulting in effective abdominal wall closure. This requires a skilled multidisciplinary approach and possibly greater resources than other options.     

Postoperative regional distribution of pulmonary ventilation and perfusion in infants with congenital diaphragmatic hernia

Background/Purpose

Advances in management of patients with congenital diaphragmatic hernia (CDH) have improved mortality rates but with a risk of increased pulmonary morbidity. The prognosis for CDH survivors remains difficult to predict owing to the lack of adequate methods. We used single photon emission computed tomography (SPECT) to measure the regional distribution of ventilation and perfusion in CDH infants to quantify the degree of lung function impairment and relate it to neonatal clinical disease severity.

Methods

Single photon emission computed tomography was performed in 12 CDH infants at the mean age of six months. Ventilation and perfusion were traced with 5 MBq Technegas and technetium-labelled albumin macro-aggregates, respectively. Neonatal clinical data collected during the patient's stay in the pediatric intensive care unit was correlated with the SPECT data.

Results

Single photon emission computed tomography revealed varying degrees of ventilation-perfusion abnormalities which correlated with the presence of pulmonary artery hypertension, days on ventilator and days on extracorporeal membrane oxygenation.

Conclusions

The grade of clinical disease severity in infants following CDH repair is closely related to the ventilation-perfusion abnormality as seen using SPECT. The persistence of pulmonary artery hypertension into the postoperative neonatal period appears to be an important pathophysiological factor related to ventilation-perfusion abnormalities. Single photon emission computed tomography provides valuable clinical information for patient follow-up.     

Congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: does timing of repair matter?

Purpose

Severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) is associated with high mortality. Timing of CDH repair relative to ECMO therapy remains controversial. Our hypothesis was that survival would significantly differ between those who underwent repair during ECMO and those who underwent repair after ECMO therapy.

Methods

We examined deidentified data from the CDH study group (CDHSG) registry from 1995 to 2005 on patients who underwent repair and ECMO therapy (n = 636). We used Cox regression analysis to assess differences in survival between those who underwent repair during and after ECMO.

Results

Five covariates were significantly associated with mortality as follows: timing of repair relative to ECMO (P = .03), defect side (P = .01), ECMO run length (P < .01), need for patch repair (P = .03), birth weight (P < .01), and Apgar score at 5 minutes (P = .03). Birth year, inborn vs transfer status, diaphragmatic agenesis, age at repair, and presence of cardiac or chromosomal abnormalities were not associated with survival. Repair after ECMO therapy was associated with increased survival relative to repair on ECMO (hazard ratio, 1.407; P = .03).

Conclusion

These data suggest that CDH repair after ECMO therapy is associated with improved survival compared to repair on ECMO, despite controlling for factors associated with the severity of CDH.     

Hearing impairment in congenital diaphragmatic hernia: the inaudible and noiseless foot of time

Aim of the Study

Infants with congenital diaphragmatic hernia (CDH) are at high risk of sensorineural hearing loss (SNHL). Extracorporeal membrane oxygenation is known to increase this risk, but little is known about other potential causes. We evaluated the impact of several risk factors on SNHL development in CDH survivors not treated with extracorporeal membrane oxygenation.

Methods

All high-risk CDH survivors consecutively treated between 1999 and 2005 were included. SNHL was diagnosed based on formal assessment with standard audiologic tests. Patients with and without SNHL were compared for patient-related and treatment-related risk factors. Subsequently, a logistic regression analysis was performed to identify independent risk factors associated with SNHL development.

Main Results

Out of 87 CDH survivors, 82 had a formal audiologic evaluation and 40 (49%) had SNHL. Patients with SNHL had significantly lower gestational age (P = .045); higher prevalence of sepsis (P < .001); older age at audiologic examination (P < .001); more episodes of hypocapnia (P = .045); higher prevalence of inhaled nitric oxide use (P = .005); longer mechanical ventilation (P = .009); and longer aminoglycosides (P = .006), furosemide (P = .004), and pancuronium bromide (P = .001) treatments. On logistic regression analysis, the only variable independently associated with the development of SNHL was patient's age at audiologic follow-up (P = .012).

Conclusions

Several risk factors were associated with SNHL development at univariate analysis. After logistic regression, only age at evaluation remained independently associated with SNHL. Routine audiologic follow-up is advocated in all CDH patients. Further studies are needed to define if other (genetic) factors may be involved in the pathogenesis of SNHL in patients with CDH.     

Surgical challenges associated with intensive treatment protocols for high-risk neuroblastoma

Background

High-risk neuroblastoma (NB; age, >1 year; INSS stage 4) is associated with a poor outcome. At our institution, the current dose-intensive high-risk Children's Oncology Group protocol for advanced NB appears to have a higher surgical complication rate as compared with previous protocols.

Methods

All stage 4 patients (n = 51) entered in high-risk protocols between 1995 and 2005 were analyzed. Patients in the current high-risk protocol, Children's Oncology Group A3973 (n = 22), were compared with those in the 2 previous protocols, CCG 3891 and POG 9341 (n = 29).

Results

Patients were comparable in their mean age and tumor markers, including Shimada histology, MYCN amplification, 1p deletion, tumor origin, and extent of metastasis. However, transfusion requirement (86% vs 45%; P = .0019), postoperative infection rate (32% vs 3%; P = .02), and other postoperative issues including nutritional support (45% vs 3%; P = .0001) were significantly higher with the current protocol. No perioperative mortality was noted in either group, and the extent of resectability and margins were similar. Importantly, with the current protocol, the survival rate was higher (P = .0022) and the recurrence rate was significantly lower (P = .0003).

Conclusions

Despite higher surgical morbidity associated with the current high-risk protocol (2.59 vs 0.86 complications/person; P < .01), the recurrence rate is lower and interim survival rate is improved for patients with high-risk NB. Therefore, the higher surgical complication rates associated with the current high-risk protocol are acceptable.     

Late gestation fetal magnetic resonance imaging-derived total lung volume predicts postnatal survival and need for extracorporeal membrane oxygenation support in isolated congenital diaphragmatic hernia

Purpose

Magnetic resonance imaging (MRI) has been used as an imaging modality to assess pulmonary hypoplasia in congenital diaphragmatic hernias (CDHs). The objective of this study was to determine if there is a correlation between late gestational fetal MRI-derived total lung volumes (TLVs) and CDH outcomes.

Methods

From 2006 to 2009, 44 patients met criteria of an isolated CDH with a late gestational MRI evaluation. The prenatal TLV (in milliliters) was obtained between 32 and 34 weeks gestation. The measured study outcomes included survival, need for extracorporeal membrane oxygenation (ECMO), and length of stay.

Results

There were 39 left and 5 right CDH patients. The average TLV was significantly lower for nonsurvivors (P = .01), and there was a significant association between lower TLV and the need for ECMO (P = .0001). When stratified by TLV, patients with a TLV of greater than 40 mL had a 90% survival vs 35% survival for a TLV of less than 20 mL. Furthermore, patients with a TLV greater than 40 mL had a lower rate of ECMO use (10%) than patients with a TLV of less than 20 mL (86%). Shorter length of stay was found to correlate with increasing TLV (P = .022).

Conclusion

Late gestation fetal MRI-derived TLV significantly correlates with postnatal survival and need for ECMO. Fetal MRI may be useful for the evaluation of patients who present late in gestation with a CDH.     

Abnormal neuroimaging and neurodevelopmental findings in a cohort of antenatally diagnosed congenital diaphragmatic hernia survivors

Purpose

Prior studies have shown that survivors of congenital diaphragmatic hernia (CDH) repair may have long-term cardiac, pulmonary, and nutritional issues, as well as neurodevelopmental sequelae within the first 3 years of life. In this study, we examined the relationship between neuroimaging abnormalities and neurodevelopmental outcomes in a cohort of antenatally diagnosed CDH survivors.

Methods

Retrospective chart reviews were performed for CDH survivors born from January 2000 to December 2007 who were evaluated antenatally in the Advanced Fetal Care Center at Children's Hospital Boston (Mass). Prenatal and postnatal neuroimaging findings, clinical data, and neurodevelopmental findings were collected for a cohort of 45 patients who were evaluated by a developmental pediatrician at ages 1 and/or 3.

Results

Prenatal neuroimaging studies detected brain anomalies in this cohort with a false-negative rate of 7%. Of the 45 study participants, 87% had left-sided CDH, 22% had cardiac anomalies, and 18% had congenital malformations or genetic syndromes. Nearly all required ventilator management (98%) with a median ventilator time of 17 days (range, 3-56 days). Moreover, 24% required extracorporeal membrane oxygenation. While 84% of patients had medical issues at discharge, 68% and 77% had medical issues at ages 1 and 3, respectively. Pulmonary problems were noted in 32% and 47% of the ages 1 and 3 cohorts, respectively. Motor problems were detected in 46% and 71% of the ages 1 and 3 cohorts, respectively. More patients with motor problems at age 1 had abnormal rather than normal postnatal neuroimaging studies (P = .01). Children with motor problems at age 1 were more apt to have an abnormal postnatal neuroimaging finding (odds ratio [OR], 6.3; 95% confidence interval [CI], 1.5-26.8; P = .01), pulmonary problems at age 1 (OR, 4.0; 95% CI, 0.99-16.6; P = .04), and a history of ventilatory management with a linear ventilator time (OR, 1.1; 95% CI, 1.01-1.12; P = .03).

Conclusions

Prenatal neuroimaging can accurately image the brain of fetuses with CDH. Abnormal postnatal neuroimaging findings, the presence of pulmonary problems at age 1, and the length of ventilator time were predictors of motor problems at age 1. Ongoing follow-up of CDH survivors should include neurodevelopmental evaluations.     

Costs of congenital diaphragmatic hernia repair in the United States-extracorporeal membrane oxygenation foots the bill

Background

Congenital diaphragmatic hernia (CDH) is the costliest noncardiac congenital defect. Extracorporeal membrane oxygenation (ECMO) is a treatment strategy offered to those babies with CDH who would not otherwise survive on conventional therapy. The primary objective of our study was to identify the leading source of expenditures in CDH care.

Methods

All patients surviving CDH repair were identified in the Kids' Inpatient Database (KID) from 1997 to 2006, with costs converted to 2006 US dollars. Patients were categorized into groups based on severity of disease for comparison including CDH repair only, prolonged ventilator dependence, and ECMO use. Factors associated with greater expenditures in CDH management were analyzed using a regression model.

Results

Eight hundred thirty-nine patients from 213 hospitals were studied. Extracorporeal membrane oxygenation use decreased from 18.2% in 1997 to 11.4% in 2006 (P = .002). Congenital diaphragmatic hernia survivors managed with ECMO cost more than 2.4 times as much as CDH survivors requiring only prolonged ventilation postrepair and 3.5 times as much as those with CDH repair only (both P < .001). Age, multiplicity of diagnoses, patient transfer, inhaled nitric oxide use, prolonged ventilation, and ECMO use were all associated with higher costs. Extracorporeal membrane oxygenation use was the single most important factor associated with higher costs, increasing expenditures 2.4-fold (95% confidence interval, 2.1-2.8). Though the CDH repair with ECMO group constituted 12.2% of patients, this group has the highest median costs ($156,499.90/patient) and constitutes 28.5% of national costs based on CDH survivors in the KID. Annual national cost for CDH survivors is $158 million based on the KID, and projected burden for all CDH patients exceeds $250 million/year.

Conclusions

Extracorporeal membrane oxygenation use is the largest contributing factor to the economic burden in CDH. With limited health care resources, judicious resource utilization in CDH care merits further study.     

Influence of location of delivery on outcome in neonates with congenital diaphragmatic hernia

Background

Although it is often recommended that infants with antenatally diagnosed congenital diaphragmatic hernia (CDH) be delivered in a perinatal center, this practice has not been scientifically validated.

Methods

Data were obtained from The Canadian Pediatric Surgery Network, covering 16 pediatric surgical centers over a 4-year period. Inborn was defined as birth in a hospital with a neonatal intensive care unit or connected to a neonatal intensive care unit by a bridge or tunnel. Outborn was defined as requiring transfer by ambulance or flight. Primary outcome variable was mortality.

Results

Of 140 infants with antenatally diagnosed CDH, 75 were inborn and 65 were outborn. Univariate analysis demonstrated no significant difference between groups with respect to gestational age, birth weight, days to surgery, primary repair, need for ventilation, use of pressors or extracorporeal membrane oxygenation, or incidence of comorbidities. Severity of illness, as reflected by the Score for Neonatal Acute Physiology II (SNAP II), was significantly higher among inborn infants (21 [interquartile range, 7-32] vs 5 [interquartile range, 9-12]; P = .0001). Logistic regression analysis, controlling for severity of illness, revealed that location of delivery was a significant independent predictor for mortality, with an odds ratio of dying when outborn of 2.8 (P = .04).

Conclusions

Outborn delivery is a significant predictor of mortality for infants with antenatally diagnosed CDH.     

Salvaging the severe congenital diaphragmatic hernia patient: is a silo the solution?

Background

Infants with severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) have a high morbidity and mortality. We hypothesized that placement of an abdominal wall silo and staged abdominal wall closure may reduce problems associated with decreased abdominal domain in CDH.

Methods

We performed a retrospective review and identified 7 CDH patients requiring ECMO who had a silastic abdominal wall silo between 2003 and 2006. Variables analyzed included survival, ECMO duration, duration of silo, time to discharge, and long-term outcome.

Results

Predicted mean survival for the entire cohort using the published CDH Study Group equation was 47% (range, 9%-86%). All 7 patients (100%) survived. Extracorporeal membrane oxygenation duration averaged 15 days (range, 5-19 days). Four of the patients (58%) were repaired with a silo on ECMO, and 3 (42%) had their repair after ECMO. The abdominal wall defect was closed at a mean of 21 days (range, 4-41 days). Hospital stay after silo placement averaged 54 days (range, 20-170 days) with no infections or wound complications.

Conclusions

Abdominal wall silo placement in infants with CDH requiring ECMO appears to be an effective strategy for decreased abdominal domain. Further studies are warranted to determine the efficacy of such a strategy for these high-risk CDH patients.