Evaluating mortality and disease severity in congenital diaphragmatic hernia using the McGoon and pulmonary artery indices

Purpose

Lung hypoplasia is associated with mortality in congenital diaphragmatic hernia (CDH). However, the association between lung hypoplasia and disease severity is unclear. Early prediction of disease severity would provide parents with more precise information about the anticipated course of treatment, minimize treatment disruption, and maximize the efficient management of patients with CDH. We aimed at identifying the relationship between McGoon index (MGI) and pulmonary artery index (PAI) scores and disease severity among infants with CDH.

Methods

We retrospectively reviewed the medical records of 19 high-risk patients with CDH born between January 2006 and December 2007. McGoon index and PAI scores were determined on admission. We evaluated statistically the relationship between these scores and variables representing severity as follows: number of vasodilators, use of inhaled nitric oxide (iNO), closed method of diaphragm, duration of intubation, duration of hospitalization, and use of home oxygen therapy. Statistical significance was P < .05.

Results

Overall median MGI and PAI scores were 1.40 and 108, respectively; scores for nonsurvivors were significantly (P < .05 and P < .01, respectively) lower than those for survivors. Among survivors, PAI scores were significantly (P < .05) lower in infants requiring iNO than in infants not requiring iNO and patch repair. The PAI scores were significantly correlated with the number of vasodilators (r = −0.789; P < .01) and duration of intubation (r = −0.610; P < .05).

Conclusions

McGoon index (cutoff value, 1.31) and PAI (cutoff value, 90) are reliable indices for predicting mortality in CDH. Pulmonary artery index appears to be more useful than MGI for predicting disease severity among survivors.     

Iatrogenic pneumothorax and mortality in congenital diaphragmatic hernia

In a review of 22 infants who underwent surgical repair of congenital diaphragmatic hernias, it was noted that one of the most common factors correlating with death was perioperative tension pneumothorax. Each of the seven infants who died developed an iatrogenic pneumothorax with air leak due to face-mask or endotracheal ventilation with pressures in excess of 34 cm H2O, or to thoracentesis. Only two infants with tension pneumothorax survived. Infants who developed tension pneumothorax experienced severe respiratory distress at an earlier age and required more vigorous resuscitative measures than those infants without an air leak.     

The presence of a hernia sac in isolated congenital diaphragmatic hernia is associated with less disease severity: A retrospective cohort study

IntroductionWe aimed to determine if the presence of a hernia sac in neonates with isolated congenital diaphragmatic hernia (CDH) was associated with better clinical outcomes.MethodsWe performed a retrospective cohort study of infants with isolated CDH from 1991 to 2015. Primary outcome measures were oxygen-dependence and mortality at 28 days. Secondary measures were: inhaled nitric oxide use, vasoactive medication use, ventilator support, and recurrence rates.ResultsSeventy-one patients met the inclusion criteria: 14 patients (19.7%) had a hernia sac, and 57 patients (80.3%) did not. Mortality did not differ between the 2 groups [0 of 14 versus 3 of 57 (5.3%) (p = 1.000)]. Hernia sac patients had similar oxygen-dependence after 28 days [1 of 14 (7.1%) versus 14 of 57 (24.6%) (p = 0.273)]. Hernia sac children required less iNO (0.64 ± 2.41 vs. 6.35 ± 12.2 days, p = 0.002), vasoactive medications (2.79 ± 3.07 vs. 5.36 ± 5.52, p = 0.027), and time on ventilation (7.62 ± 6.12 vs. 15.9 ± 19.2, p = 0.010). Hernia sac children had similar recurrence rates within 2 years [0 of 14 versus 7 of 57 (12.3%) (p = 0.331)].ConclusionThe presence of a hernia sac was not associated with lower rates of oxygen dependency or death at 28 days but was associated with decreased inhaled nitric oxide, vasoactive medication, and ventilator use.Level of evidenceIII     

Right congenital diaphragmatic hernia associated with anorectal malformation

We describe a neonate in whom a right congenital diaphragmatic hernia and an anorectal malformation coexisted. Their coexistence in the same patient is rare.     

Reversal of mortality for congenital diaphragmatic hernia with ECMO.

Extracorporeal Membrane Oxygenation (ECMO) has been available to neonates with respiratory failure at the University of Michigan School of Medicine since June 1981. In order to evaluate the impact of this type of pulmonary support, a retrospective analysis of 50 neonates with posterolateral congenital diaphragmatic hernia (CDH) who were symptomatic during the first hour of life and were treated between June 1974 and December 1987 was carried out. The patients were divided into two groups, those treated before June 1981 (16 patients) and those treated after June 1981 (34 patients). Overall survival improved from 50% (eight of 16 patients) during the pre-ECMO era to 76% (26 of 34 patients) during the post-ECMO period (p = 0.06). During the period after June 1981, 21 neonates were unresponsive to conventional therapy and were therefore considered for ECMO. Failure of conventional therapy was defined as acute clinical deterioration with an expected mortality of greater than 80% based on an objective formula previously reported. Six patients were excluded on the basis of specific contraindications to ECMO. Thirteen of 15 infants (87%) supported with ECMO survived. Three patients treated before 1981 met criteria for ECMO; all three died while receiving treatment using conventional therapy. These survival differences are significant (p less than 0.01). In addition, the survival of 87% for the infants treated with ECMO versus the expected mortality of greater than 80% for these same patients when treated with conventional therapy is highly significant (p less than 0.005). Based on this data, ECMO appears to be a successful, reliable, and safe method of respiratory support for selected, critically ill infants with CDH.     

The congenital diaphragmatic hernia composite prognostic index correlates with survival in left-sided congenital diaphragmatic hernia

PurposeWe developed the congenital diaphragmatic hernia congenital prognostic index (CDH-CPI) to incorporate all known prognostic variables into a single composite index to improve prognostic accuracy. The purpose of this study is to examine the ability of the CDH-CPI to predict survival in patients with left-sided congenital diaphragmatic hernia and to determine if the index has a stronger correlation with survival than each of the individual components.MethodsA retrospective review of patients with left-sided congenital diaphragmatic hernia between 2004 and 2010 was conducted. Ten prenatal parameters of the CDH-CPI were collected, total score was tabulated, and patients stratified according to total score and survival.ResultsSixty-four patients with a prenatal diagnosis of left-sided congenital diaphragmatic hernia were identified. Patients with a CDH-CPI score of 8 or higher had a significantly higher survival than patients with a CDH-CPI score of lower than 8. The CDH-CPI has the strongest correlation with survival compared with the individual parameters measured. The CDH-CPI correlates with extracorporeal membrane oxygenation use, and 75% of patients with a score of 5 or lower were placed on extracorporeal membrane oxygenation.ConclusionsThe CDH-CPI accurately stratifies survival in left-sided congenital diaphragmatic hernia. The amalgamation of 10 prenatal parameters of the CDH-CPI may be a better prenatal predictor than any single prognostic variable currently used.     

Birth weight and McGoon Index predict mortality in newborn infants with congenital diaphragmatic hernia

Background

Despite improvements in clinical management, mortality of congenital diaphragmatic hernia (CDH) remains high. Early prediction of mortality risk helps in comparing strategies and/or performances of different centers. Birth weight (BW), Apgar Score at 5 minutes, and modified McGoon Index (MGI) calculated by the ratio between the diameters of pulmonary arteries and the descending aorta have been used to determine mortality of CDH.

Aim

The purpose of this study is to evaluate the relationship between early detectable variables and survival in newborns with CDH intubated at birth, managed with “gentle” ventilation and delayed surgery.

Methods

All medical records of patients affected by high-risk CDH and treated with a standardized protocol at Bambino Gesù Children's Hospital, Rome, Italy, between January 2002 and September 2004 were reviewed. Prenatal diagnosis, gestational age, BW, sex, side of hernia, and MGI were recorded on admission. The relationship with mortality of each variable was evaluated by univariate analysis. Subsequently, a predictive model of mortality was developed using a logistic regression: the explanatory variables, BW, and MGI were dichotomized in high (HBW and HMGI) and low (LBW and LMGI) according to the best cutoff found with receiver-operating characteristic curves.

Results

Thirty-four newborns with CDH, treated with a standardized protocol, were studied. The main characteristics of the 34 patients were BW, 2886 g (1500-3620 g); gestational age, 37.7 weeks (32-42 weeks); male/female, 22/12; right/left, 8/26; prenatal diagnosis, 29; MGI, 1.31 (0.9-1.85). Only BW and MGI were significantly (P < .05) associated with mortality at the univariate analysis. The best cutoff values were 2755 g for BW (sensitivity, 70%; specificity, 74%) and 1.25 for MGI (sensitivity, 73%; specificity, 78%). Using these limits, BW and MGI resulted independently associated with mortality in the multivariate analysis. Using the 4 possible combinations, the LBW associated with the LMGI presented the highest prediction of mortality (80%).

Conclusions

Birth weight and MGI, variously combined, were predictive of mortality. Because they are not influenced by subsequent modalities of care, they can be considered as valid early severity scores in CDH and used for comparing strategies and/or performances of different centers.     

Identifying neonatal and pediatric cardiac and congenital diaphragmatic hernia extracorporeal membrane oxygenation patients at increased mortality risk

A previous review from our institution established clinically measured cut-points that defined the late implementation of extracorporeal membrane oxygenation (ECMO) correlating to increased mortality in neonatal and pediatric respiratory patients. Using the same methods, this review evaluates pediatric and neonatal cardiac and congenital diaphragmatic hernia (CDH) patients to determine if the same cut-points exist in this higher risk patient population. Neonatal and pediatric cardiac and CDH patients placed on ECMO between November 1989 and December 2008 were retrospectively reviewed to determine the first adjusted anion gap (AGc), the first venoarterial carbon dioxide (CO2) gradient (p[v-a]CO2), and the first Viability Index (AGc + p[v-a]CO2 = INDEX) on ECMO. These markers were then analyzed to identify the presence of specific cut-points that marked an increased risk of mortality. The timing of surgery was also reviewed to assess the surgical morbidity on survival. The review of neonatal and pediatric cardiac and CDH patients (n = 205) with an overall survival of 46% showed that all three markers were elevated to varying degrees in the expired patients (n = 110). Histograms identified the following specific cut-points for increased mortality: the AGc > or = 23 mEq/L, the p[v-a]CO2 _ 16 mmHg, and the INDEX > or = 28. An elevated AGc and INDEX correlated with a significantly higher risk for mortality (p < .05), survival to discharge being 20% or less. Patients under the cut-points had survival rates of 51% or higher. The timing of surgery (before or after ECMO initiation) did not significantly impact survival in the combined cardiac and CDH group. An INDEX > or = 28 correlates with non-survival. We speculate that the late implementation of ECMO may lead to reperfusion injury, which causes reduced survival, and that ECMO intervention prior to reaching the cut-points may improve survival in neonatal and pediatric cardiac and CDH patients. Keywords: cardiac, congenital, diaphragmatic, extracorporeal membrane oxygenation, neonate, pediatric.     

Left-sided congenital diaphragmatic hernia associated with intrathoracic ectopic liver lobule.

A case of intrathoracic ectopia of the left lobe lateral segment of the liver in a patient with left-sided diaphragmatic hernia is described. The ectopic liver part was found in the left hemithorax during excision of the diaphragmatic hernia sac, but it was not the content of the sac. The ectopic liver part has not been described previously in the left hemithorax and is actually the primary abnormality. The diaphragmatic hernia is merely a secondary development through this potential gap on the diaphragm where the pedicle of the ectopic liver lobule passes.     

Skeletal malformations associated with congenital diaphragmatic hernia: experimental and human studies

BACKGROUND/PURPOSE: Skeletal malformations are seen occasionally in infants with congenital diaphragmatic hernia (CDH). This study examines whether nitrofen, able to produce CDH in fetal rats, also induces skeletal anomalies and, if so, whether these are similar to those seen in CDH patients. METHODS: Pregnant rats received either nitrofen (100 mg, n = 7) or no treatment (n = 2) on gestational day 9.5. Skeletal anatomy was studied in fetuses recovered on day 21 after alcian blue-alizarin red staining. The charts and postmortem records of 117 stillborns or newborns who died of CDH were investigated retrospectively for skeletal defects. The proportions of anomalies found in the different groups were compared. RESULTS: The 15 control rat fetuses were normal, whereas 57 of 90 nitrofen-exposed animals (63%) had CDH accompanied by other malformations. Skeletal defects limited to vertebral segmentation or identity anomalies (split vertebra or absent, hypoplastic, or fused ribs) were seen at low thoracic and high lumbar levels in 68% of animals with CDH and in 57% of those without. Delayed ossification of limbs was seen in treated animals. There were skeletal malformations in 31.6% of the 117 human patients with CDH. Costovertebral defects (malformed, extra or defective vertebral bodies or ribs and spina bifida) were comparably frequent in infants with syndromes and in those without them (31.2% v 17.8%, not significant), whereas limb defects were significantly more frequent in those with syndromes (56.2% v 13.9%, P<.05). CONCLUSION: The nature and location of costovertebral malformations found in both CDH patients and nitrofen-exposed rats suggest that the diaphragmatic defect and the associated organ malformations might be caused by the same early embryonal disturbance involving axial and para-axial mesoderm.     

Right congenital diaphragmatic hernia associated with fusion of the liver and the lung

We present the case of a male infant at a gestational age of 41 weeks who presented with a right congenital diaphragmatic hernia. During surgical exploration, right hepatopulmonary fusion was encountered. We review the existing literature on this problem, describe a novel surgical approach, and review the embryology of this complex lesion.     

Fryns syndrome in children with congenital diaphragmatic hernia

Purpose: Fryns syndrome is characterized by multiple congenital anomalies including Congenital Diaphragmatic Hernia (CDH), and has a reported poor prognosis with a survival rate during the neonatal period of approximately 15%. This report details the management and outcome of patients with Fryns syndrome and CDH. Methods: Records of all liveborn patients with CDH between 1995 and 2001 in 83 hospitals were entered into the CDH database. Those with Fryns syndrome were reviewed retrospectively. Results: A total of 1,833 patients were entered in the database, 23 of these had Fryns (1.3%). All patients experienced early distress requiring intubation. Ten patients (43%) were found to have other major anomalies. Seven patients underwent surgical repair at an average age of 7.5 days (range, 6 hours to 14 days). Mortality rate was 83% compared with 33% of patients with unilateral CDH (P = .01). Ten patients died within the first 24 hours. The parents of 6 patients withdrew support. Of the 4 survivors, 3 have marked developmental delay, whereas the fourth has not yet undergone formal assessment. Conclusions: The prognosis of infants with Fryns syndrome and congenital diaphragmatic hernia remains grim. Early genetic counseling and recognition of lethal anomalies may assist in determining which patients may survive.     

Airway anomalies in patients with congenital diaphragmatic hernia

PURPOSE: Congenital diaphragmatic hernias (CDH) sometimes are associated with airway anomalies such as congenital stenosis, abnormal branching of the bronchi, and pulmonary hypoplasia. The incidence of these associated airway anomalies has not been reported previously. METHODS: Bronchoscopy was performed in all neonates with CDH from 1987 to 1999. In addition to anatomic anomalies, bronchial hypoplasia was defined as narrowing and shortening of the bronchi at bronchoscopy. RESULTS: Anatomic anomalies were identified in 7 of 39 patients with CDH: 1 had congenital tracheal stenosis with pulmonary artery sling, 1 had a defect of the right upper lobe bronchus, 2 had a tracheal bronchus, and 3 had a trifurcated trachea. Bronchial hypoplasia on the affected side was identified in 15 patients and was seen in all patients with anatomic anomalies of the tracheobronchial tree except the 2 with tracheal bronchus. After excluding 5 patients with severe associated anomalies, 6 of 14 patients with an abnormal tracheobronchial tree died, whereas 1 of 20 patients without airway abnormalities died. CONCLUSIONS: Anatomic anomalies of the tracheobronchial tree and bronchial hypoplasia on the affected side were identified in 17.9% and 38.4% of patients with CDH, respectively. CDH patients who exhibited these abnormalities showed a poor outcome.     

Congenital heart disease associated with congenital diaphragmatic hernia: A systematic review on incidence,prenatal diagnosis,management, and outcome

PurposeThe purpose of this study was to evaluate the impact of congenital heart disease (CHD) on infants with congenital diaphragmatic hernia (CDH).MethodsUsing a defined search strategy (PubMed, Cochrane, Embase, Web of Science MeSH headings), we searched studies reporting the incidence, management, and outcome of CDH infants born with associated CHD.ResultsOf 6410 abstracts, 117 met criteria. Overall, out of 28,974 babies with CDH, 4427 (15%) had CHD, of which 42% were critical. CDH repair was performed in a lower proportion of infants with CHD (72%) than in those without (85%; p < 0.0001). Compared to CDH babies without CHD, those born with a cardiac lesion were more likely to have a patch repair (45% vs. 30%; p < 0.01) and less likely to undergo minimally invasive surgery (5% vs. 17%; p < 0.0001). CDH babies with CHD had a lower survival rate than those without CHD (52 vs. 73%; p < 0.001). Survival was even lower (32%) in babies with critical CHD.ConclusionCHD has a strong impact on the management and outcome of infants with CDH. The combination of CDH and CHD results in lower survival than those without CHD or an isolated cardiac defect. Further studies are needed to address some specific aspects of the management of this fragile CDH cohort.Type of studySystematic review and meta-analysis.Level of evidenceLevel III.     

One-year outcomes of congenital diaphragmatic hernia repair: Factors associated with recurrence and complications

PurposeCongenital diaphragmatic hernia (CDH) is a congenital anomaly associated with lifelong multisystem morbidity. This study sought to identify factors contributing to hospital readmission after CDH repair.MethodsThe Nationwide Readmissions Database from 2010 to 2014 was used to identify patients with CDH who underwent surgical repair. Primary outcomes included all cause readmission at 30-days and 1 year and readmission for hernia recurrence. Patient and hospital factors were compared using chi-squared analysis.ResultsFive hundred eleven patients were identified with neonatal CDH. All repairs were performed at teaching hospitals via laparotomy in 59% (n = 303), thoracotomy in 36% (n = 183), and minimally invasive (MIS) repair in 5% (n = 25). The readmission rate within 30-days was 32% (n = 163), and 97% (n = 495) within 1 year. The most common conditions surrounding readmission were for gastroesophageal reflux (20%), CDH recurrence (17%), and surgery for gastrostomy tube and/or fundoplication (16%). Recurrence was significantly higher after MIS repair (48%) compared to those with open repair via either approach (16%), p < 0.001.ConclusionsThis is the first study to evaluate nationwide readmissions in newborns with CDH. Readmission is commonly due to CDH recurrence and reflux-associated complications. The recurrence rate is higher than previously reported and is more common after MIS and repair via thoracotomy.Level of evidenceLevel III treatment study.