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.     

Liver position in fetal congenital diaphragmatic hernia retains a prognostic value in the era of lung-protective strategy

Background/Purpose

The aims of this study were to analyze the outcomes of fetuses with congenital diaphragmatic hernia (CDH) treated by a lung-protective strategy using high-frequency oscillatory ventilation (HFOV) in a single center with a perinatology service and extracorporeal membrane oxygenation (ECMO) capability and to define the natural history of CDH in the era of lung-protective ventilation.

Methods

A retrospective chart review of 30 neonates with CDH seen between April 2002 and October 2004 was conducted. All fetuses with a prenatal diagnosis were evaluated by fetal magnetic resonance imaging to define the liver position, and those with a significant volume of the liver in the chest were regarded as liver-up. Patients were managed by a lung-protective strategy using pressure-limited (maximum mean airway pressure [MAP], 18 cm H₂O) HFOV. The patients were initially placed on HFOV with a fraction of inspired oxygen (Fio₂) of 1.0 and a MAP of 12 cm H₂O. Hypercapnea and preductal saturation as low as 85% were accepted. Inhaled nitric oxide and ECMO were introduced when the baby could not be oxygenated with a MAP of 18 cm H₂O.

Results

Twenty-six neonates (22 inborns with prenatal diagnosis and 4 outborns) were treated with this protocol. Four cases were not treated or died in utero because of severe associated anomalies. Thirteen of the 14 liver-down cases survived without ECMO and were discharged home (93% survival). On the contrary, 4 of 12 liver-up cases survived (33% survival). ECMO was required for initial stabilization in 5 cases with 1 survivor.

Conclusions

Liver-down CDH babies have a good chance for survival without ECMO by a planned delivery and the lung-protective strategy using HFOV. Liver herniation demonstrated by prenatal magnetic resonance imaging retains a poor prognostic value even with this approach.     

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.     

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.     

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.     

Protocolized management of infants with congenital diaphragmatic hernia: effect on survival

Background/Purpose

In 2006, we introduced a new protocol for congenital diaphragmatic hernia (CDH) management featuring nitric oxide in the delivery room, gentle ventilation, lower criteria for extracorporeal membrane oxygenation (ECMO), and appropriately timed operative repair on ECMO. Our goals were to assess outcomes after institution of this protocol and to compare results with historical controls.

Methods

Charts were reviewed of all newborns admitted to a large metropolitan children's hospital from 2002 to 2009 with a diagnosis of CDH. Data were recorded regarding delivery, ECMO, operative repair, length of stay, comorbidities/anomalies, complications, and survival. Postprotocol outcomes were compared to those from the preprotocol era and to data from the international CDH Registry.

Results

Comparison of the protocolized group (n = 43) to the historical group (n = 51) revealed no significant differences in gestational age, birth weight, Apgar scores, or comorbidities. New treatment strategies substantially improved survival to discharge (67% preprotocol, 88% postprotocol; P = .015). Among ECMO patients, survival increased to 82% (20% preprotocol; P = .002).

Conclusions

Our new protocol significantly improved survival to discharge for newborns with CDH. Institution of such a protocol is valuable in improving outcomes for patients with CDH and merits consideration for widespread adoption.     

Surfactant replacement therapy on ECMO does not improve outcome in neonates with congenital diaphragmatic hernia

Background purpose

Respiratory failure in neonates with congenital diaphragmatic hernia (CDH) may in part be caused by a primary or secondary surfactant deficiency. Knowledge of the optimal approach to surfactant replacement in neonates with CDH and respiratory failure is limited. The aim of this study was to determine if surfactant replacement on extracorporeal membrane oxygenation (ECMO) results in improved outcomes in neonates ≥35 weeks’ gestation with unrepaired CDH.

Methods

Using the CDH Study Group Registry, the authors identified 448 neonates with CDH who were ≥35 weeks’ gestation, had no major anomalies, were treated with ECMO within the first 7 days of life, and underwent repair on or after ECMO therapy. Patients in 2 groups were compared: group 1 (− Surf, n = 334) consisted of patients who received no surfactant and group 2 (+ Surf, n = 114) consisted of patients who received at least 1 dose of surfactant while on ECMO. An analysis of all patients in both groups was performed. Additionally, subgroup analyses stratified by gestational age were performed for patients 351/7 to 366/7 weeks’ gestation and for patients ≥37 weeks’ gestation. Primary end-points for the study were survival and length of ECMO run. Secondary end-points were length of intubation, need for supplemental oxygen at 30 days of life, and at discharge to home. Demographic, clinical, and outcome variables were examined using Fisher’s Exact tests for categorical variables and using unpaired t tests for continuous variables. Odds ratios were calculated for categorical end-point variables.

Results

Demographic and clinical variables were similar between groups. Analyses of aggregate data showed no significant differences between groups in length of ECMO run, survival, number of days intubated, and percent of patients requiring supplemental oxygen at 30 days or discharge. Subgroup stratification by gestational age did not show significant differences between groups in any of the outcome variables.

Conclusions

The data from this study suggest that surfactant replacement on ECMO for neonates with congenital diaphragmatic hernia does not provide significant benefit in the infant’s clinical course with respect to survival, length of ECMO course, length of intubation, or subsequent need for supplemental oxygen.     

Ex utero intrapartum treatment with extracorporeal membrane oxygenation for severe congenital diaphragmatic hernia

Purpose

The purpose of this study was to determine whether ex utero intrapartum treatment with extracorporeal membrane oxygenation (EXIT to ECMO) is a reasonable approach for managing patients antenatally diagnosed with severe congenital diaphragmatic hernia (CDH).

Methods

A 6-year retrospective review was performed on fetuses with severe CDH (liver herniation and a lung/head ratio <1.4, percentage of predicted lung volume <15, and/or congenital heart disease). Fourteen of the patients underwent EXIT with a trial of ventilation. Fetuses with poor preductal oxygen saturations despite mechanical ventilation received ECMO before their delivery. Maternal-fetal outcomes were analyzed.

Results

There were no maternal-reported complications. Three babies passed the ventilation trial and survived, but 2 of them required ECMO within 48 hours. The remaining 11 fetuses received ECMO before their delivery. Overall survival after EXIT-to-ECMO was 64%. At 1-year follow-up, all survivors had weaned off supplemental oxygen, but 57% required diuretics and/or bronchodilators.

Conclusion

This is the largest reported experience using EXIT to ECMO in the management of severe CDH. The EXIT-to-ECMO procedure is associated with favorable survival rates and acceptable pulmonary morbidity in fetuses expected to have a poor prognosis under conventional management.     

Congenital diaphragmatic hernia: prognostic indices in the fetal endoluminal tracheal occlusion era

Introduction

Management of congenital diaphragmatic hernia (CDH) in the UK now includes the possibility of fetal endoluminal tracheal occlusion (FETO) for poor prognosis fetuses. The objective of this study was to investigate the value of variables previously thought prognostic in the FETO era.

Methods

A retrospective single-centre study was performed of all infants with CDH born between January 1994 and December 2007. Fetal endoluminal tracheal occlusion was available and had been used with parental consent for fetuses with lung-to-head ratio (LHR) of 1.0 or less and a liver-up position from 2002. Univariate analysis was used to predict survival (to leave hospital) using both prenatal (eg, polyhydramnios) and perinatal variables [eg, best oxygenation index on day 1, or BOI (d1)] and their dependence tested in a logistic regression model. Data were quoted as medians (range). P < .05 was regarded as significant.

Results

Eighty-six infants with CDH (1994-2002, n = 35 and 2002-2007, n = 51 “FETO era”) were studied. Successful FETO intervention was performed in 31 infants.Univariate analysis showed liver position, birth weight, LHR, and BOI (d1) were significant prognostic predictors (all P < .05); however, only BOI (d1) retained significance using logistic regression analysis (odds ratio, 21; 95% confidence interval, 6-74; P < .001). Best oxygenation index on day 1 was then used as a surrogate marker for outcome to test the relationship with LHR (available since 2002) and showed a significant inverse correlation (r_s = −0.5; P < .001). There was no difference in median BOI (d1) between the FETO group and all those treated expectantly (40 [34-1046] vs 59 (23-581); P = .3].

Conclusion

Best oxygenation index on day 1 is the best early postnatal predictor of survival. The more recently evaluated prenatal index, LHR, has an observable relationship with BOI (d1) when it is used as a surrogate marker of outcome.     

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.     

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.     

Factors associated with survival in infants with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: a report from the Congenital Diaphragmatic Hernia Study Group

Objective

To identify factors associated with survival in patients with congenital diaphragmatic hernia (CDH) treated with extracorporeal membrane oxygenation (ECMO).

Methods

We retrospectively analyzed the data on 3100 patients with CDH in the Congenital Diaphragmatic Hernia Study Group from 82 participating pediatric surgical centers (1995-2004). Covariates considered included prenatal and perinatal clinical information, specifics of surgical repair, and the duration of extracorporeal support.

Result

Nine hundred seven patients from the registry were identified as having been both managed with ECMO and undergone attempted surgical repair. The survival rate for the entire Congenital Diaphragmatic Hernia Study Group registry was 67% and 61% for those receiving ECMO in whom repair was attempted (P < .001). Among ECMO-treated children, survivors had a greater estimated gestational age (38 ± 2 vs 37 ± 2 weeks; P < .01), greater birth weights (3.2 ± 0.5 vs 2.9 ± 0.5 kg; P < .001), were less often prenatally diagnosed (53% vs 63%; P < .01), and were on ECMO for a shorter period of time (9 ± 5 vs 12 ± 5 days; P < .001). In logistic regression models, therapy-related variables, including the duration of ECMO, the nature of diaphragmatic repair, and the type of abdominal closure and certain comorbidities, particularly the presence of a concomitant severe cardiac abnormality, were independently associated with outcome.

Conclusion

Our model identifies a group of pre-surgical and postsurgical parameters that predict survival rate in patients with CDH on ECMO support. This model was derived from the retrospective data from a large database and will need to be prospectively tested.     

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.     

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.     

Influence of extracorporeal membrane oxygenation on subsequent surgeries after congenital diaphragmatic hernia repair

Purpose

Congenital diaphragmatic hernia (CDH) is a complex anomaly requiring intensive pulmonary and hemodynamic management. Survival has increased in this population placing them at risk for subsequent morbidities including surgery. The purpose of this study is to review the need for subsequent surgeries in the CDH population.

Methods

After receiving institutional review board approval, a retrospective chart review of all CDH patients between 1980 and 2007 was conducted noting subsequent surgeries and the impact of extracorporeal membrane oxygenation (ECMO) on the types of surgical procedures. Comparison of groups was done by Fisher's Exact test or nonparametric Wilcoxon rank-sum test where appropriate. A P value of less than .05 was considered significant.

Results

Data were analyzed for 227 of 294 patients during this period. Extracorporeal membrane oxygenation support was used in 45% of patients. Subsequent surgery was required in 117 patients. Seventy patients in the ECMO group (69%) required a subsequent operation. The most common operative procedures included inguinal hernia/orchiopexy, antireflux, and recurrent diaphragmatic hernias.

Conclusion

In this series, ECMO survivors are at a high risk for requiring subsequent surgeries compared to the total CDH group. This information can be used as an education tool for referring physicians and parents as they care for this group of children.     

Defining “liver-up”: does the volume of liver herniation predict outcome for fetuses with isolated left-sided congenital diaphragmatic hernia?

PurposeThe aim of the study was to evaluate the relationship between the degree of liver herniation and mortality or need for extracorporeal membrane oxygenation (ECMO) in isolated left-sided congenital diaphragmatic hernia (CDH).MethodsThis is a retrospective review of all fetuses with isolated left-sided Bochdalek-type CDH evaluated at our center with ultrafast fetal magnetic resonance imaging between January 2004 and December 2010. Percentage of liver herniation was defined as the ratio of herniated liver volume to total fetal liver volume. The liver/thoracic volume ratio was defined as the ratio of herniated liver volume to total thoracic volume. Data were analyzed using receiver operating characteristic curves and Fisher's Exact and Mann-Whitney U tests.ResultsFifty-three fetuses with isolated left-sided CDH were evaluated. Fetuses with “liver-up” (n = 32) and “liver-down” (n = 21) anatomy had similar rates of mortality (25% vs 14%, P = .49) and ECMO use (41% vs 29%, P = .40). The accuracy of liver-up (a dichotomous variable) to predict mortality or need for ECMO was 49% and 53%, respectively. Percentage of liver herniation greater than 21% was associated with mortality (P < .001) or need of ECMO (P < .001), with an accuracy of 87% and 79%, respectively. Liver/thoracic volume ratio of greater than 14% was also associated with mortality or ECMO use (P < .001 and P = .01, respectively), with an accuracy of 85% and 72%, respectively.ConclusionIncreased amounts of liver herniation in fetuses with isolated left-sided CDH are associated with higher rates of mortality or the need for ECMO support. Quantification of liver herniation (a continuous variable) is superior to overall liver herniation as a dichotomous variable (liver-up vs liver-down) in the prediction of perinatal morbidity and mortality.     

How low can you go? Effectiveness and safety of extracorporeal membrane oxygenation in low-birth-weight neonates

Purpose

Controversy exists regarding the criteria for placement of infants on extracorporeal membrane oxygenation (ECMO) at low birth weights. The authors hypothesized that ECMO is effective and safe in babies under 2 kg and sought to examine outcome and survival rate in these infants.

Methods

All patients less than 30 days old in the Extracorporeal Life Support Organization (ELSO) registry (n = 14,305) were divided into those less than 2 kg (n = 663) and more than 2 kg (n = 13,642). Multiple regression analysis determined factors that predicted survival rate and the lowest safe weight for ECMO.

Results

Overall survival rate was 76% and was lower in infants less than 2 kg (≥2 kg, 77% v <2 kg, 53%, P < .0001). Survival rate was significantly lower for patients with diaphragmatic hernia (CDH), bleeding, and intracranial hemorrhage (ICH) by regression. The incidence of ICH in babies less than 2.0 kg was 6% versus 4% in those more than 2.0 kg (P < .05). Regression analysis determined that the lowest weight at which a survival rate of 40% could be achieved was 1.6 kg.

Conclusions

Cannulation for ECMO may be safe and effective in babies under 2.0 kg and potentially as low as 1.6 kg. Judicious anticoagulation might limit bleeding, which occurred in a minority of these patients.     

Prosthetic patches for congenital diaphragmatic hernia repair: Surgisis vs Gore-Tex

Purpose

The sequelae of congenital diaphragmatic hernia (CDH) continue well beyond the perinatal period. Up to 50% of these patients have subsequent recurrent herniation or small bowel obstruction (SBO). A recent trend has been toward the use of bioactive prosthetic materials. We reviewed different patch closure techniques used for CDH repair at our institution and their association with these sequelae.

Methods

A retrospective review was performed of 152 records for patients with CDH. Newborns that underwent patch repair for CDH and survived for at least 30 days were included in the analysis. Primary outcomes evaluated were recurrent herniation and SBO. Two types of prostheses were examined, Gore-Tex, an artificial material, and Surgisis, a bioactive material.

Results

Twelve (44%) of 27 patients who had Surgisis repair had recurrent herniation. Seventeen (38%) of 45 patients who had a Gore-Tex repair had recurrent herniation. Two additional patients in each group presented with SBO. No significant difference in recurrent herniation rates was observed (P > .5). The time to recurrence was similar in both groups (log-rank, P = .75), with most recurrences (92% Surgisis, 76% Gore-Tex) occurring in the first year.

Conclusion

The rates of recurrent herniation and SBO after neonatal prosthetic patch repair of CDH were similar regardless of the prosthetic material used (Surgisis or Gore-Tex).     

Long-term functional impact of congenital diaphragmatic hernia repair on children

Background/Purpose

Congenital diaphragmatic hernia (CDH) is a malformation requiring neonatal surgical repair with in-hospital survival rates above 90%. We examined the long-term functional impact of CDH repair on a cross-sectional cohort of survivors.

Methods

A cohort of 53 CDH families participated in this study. Functional impact was evaluated with parent report of the Functional Status IIR and the Child Health Ratings Inventories General Health Module. Parents also provided a clinical severity score, the child's medical history, and family demographic information. The primary outcome was the effect of medical morbidity on the Functional Status IIR total score.

Results

Congenital diaphragmatic hernia survivors had a median age of 8 years; 50% were in third grade or above. Sixty-six percent had major medical issues at hospital discharge, whereas 48% had current clinical problems. Functional Status IIR total score was strongly correlated with child's clinical severity (r = −0.65; P < .0001) and was lower among children with ongoing medical morbidity, denoting worse functioning (P = .01). Child Health Ratings Inventories General Health Module scores followed a similar pattern.

Conclusions

A subset of long-term CDH survivors continues to have ongoing clinical problems a median of 8 years after surgery, translating to lower functional status. Affected children and their families may benefit from prospective identification and ongoing interventions.     

Impact of prenatal evaluation and protocol-based perinatal management on congenital diaphragmatic hernia outcomes

Background/Purpose

Although intuitive, the benefit of prenatal evaluation and multidisciplinary perinatal management for fetuses with congenital diaphragmatic hernia (CDH) is unproven. We compared the outcome of prenatally diagnosed patients with CDH whose perinatal management was by a predefined protocol with those who were diagnosed postnatally and managed by the same team. We hypothesized that patients with CDH undergoing prenatal evaluation with perinatal planning would demonstrate improved outcome.

Methods

Retrospective chart review of all patients with Bochdalek-type CDH at a single institution between 2004 and 2009 was performed. Patients were stratified by history of perinatal management, and data were analyzed by Fisher's Exact test and Student's t test.

Results

Of 116 patients, 71 fetuses presented in the prenatal period and delivered at our facility (PRE), whereas 45 infants were either outborn or postnatally diagnosed (POST). There were more high-risk patients in the PRE group compared with the POST group as indicated by higher rates of liver herniation (63% vs 36%, P = .03), need for patch repair (57% vs 27%, P = .004), and extracorporeal membrane oxygenation use (35% vs 18%, P = .05). Despite differences in risk, there was no difference in 6-month survival between groups (73% vs 73%).

Conclusions

Patients with CDH diagnosed prenatally are a higher risk group. Prenatal evaluation and multidisciplinary perinatal management allows for improved outcome in these patients.