Surgical Repair of Congenital Diaphragmatic Hernia After Extracorporeal Membrane Oxygenation Cannulation: Early Repair Improves Survival.

OBJECTIVE: To determine the optimal timing of congenital diaphragmatic hernia (CDH) repair after extracorporeal membrane oxygenation (ECMO) cannulation. SUMMARY BACKGROUND DATA: The timing of CDH repair after ECMO cannulation remains a controversial topic due to studies with low power or strong selection bias. METHODS: This is a 2-aim retrospective cohort study based on the CDH Study Group registry for the period of 2007-2017. Aim 1-Compare On versus After ECMO repair. Aim 2-Compare Early versus Late repair on ECMO. In order to minimize selection bias and account for non-repairs, subjects in each aim were stratified into study groups based on their treatment center's characteristics. In each aim, the study groups were matched based on propensity score (PS). The main outcomes included mortality rate and incidence of non-repair. RESULTS: In aim 1, 136 patients remained in each group after PS matching. Compared to the After ECMO group, patients in the On ECMO group demonstrated a lower mortality rate, hazard ratio (HR) 0.54 (0.38, 0.77) (P < 0.001), and lower incidence of non-repair, 5.9% versus 33.8% (P < 0.001). In aim 2, 77 patients remained in each group after PS matching. Compared to the Late group, Early repair of CDH on ECMO was associated with a lower mortality rate, HR 0.51 (0.33, 0.77) (P = 0.002), and lower incidence of non-repair, 9.1% versus 44.2% (P < 0.001). CONCLUSIONS: The approach of early repair after ECMO cannulation is associated with improved survival compared to delayed surgical correction.

Non-operative management of extralobar pulmonary sequestration: a safe alternative to resection?

PURPOSE: This retrospective cohort study compares the natural history of patients with extralobar sequestrations (ELS) who do not undergo intervention with those who undergo resection to assess the safety of non-operative management. METHODS: 126 patients with pulmonary sequestrations or congenital pulmonary airway malformations born between 1999 and 2016 were identified. 49 patients had ELS on postnatal imaging, but two were excluded for associated congenital diaphragmatic hernia. Demographic and clinical data were retrospectively reviewed, with phone follow-up for non-operative patients with no records for > 1 year. Statistical analysis was by Fisher's exact test or Wilcoxon signed-rank test (two-tailed p < 0.05). RESULTS: 40% (19/47) were managed non-operatively and 60% (28/47) underwent resection. Non-operative patients were less likely to have an intrathoracic ELS: 47% (9/19) vs. 75% (21/28), p = 0.07. No symptoms were attributable directly to the ELS. Non-operative patients had median follow-up 3.2 years, during which time 88% (15/17) of ELS decreased in size on serial imaging. For patients who underwent resection, there was 100% concordance between imaging and intraoperative findings. There was no evidence of inflammation, infection or malignancy on final pathology, though 57% (16/28) of resected lesions had foci of non-aerated cysts. CONCLUSIONS: Although further longitudinal study is required, this study supports the safety of non-operative ELS management.

Aggressive Surgical Management of Congenital Diaphragmatic Hernia: Worth the Effort?: A Multicenter, Prospective, Cohort Study.

OBJECTIVE: The objectives of this study were (i) to evaluate infants with congenital diaphragmatic hernia (CDH) that do not undergo repair, (ii) to identify nonrepair rate by institution, and (iii) to compare institutional outcomes based on nonrepair rate. BACKGROUND: Approximately 20% of infants with CDH go unrepaired and the threshold to offer surgical repair is variable. METHODS: Data were abstracted from a multicenter, prospectively collected database. Standard clinical variables, including repair (or nonrepair), and outcome were analyzed. Institutions were grouped based on volume and rate of nonrepair. Preoperative mortality predictors were identified using logistic regression, expected mortality for each center was calculated, and observed /expected (O/E) ratios were computed for center groups and compared by Kruskal-Wallis ANOVA. RESULTS: A total of 3965 infants with CDH were identified and 691 infants (17.5%) were not repaired. Nonrepaired patients had lower Apgar scores (P < 0.05) and increased incidence of anomalies (P < 0.0001). Low-volume centers ("Lo", n=44 total, < 10 CDH pts/yr) and high-volume centers ("Hi", n = 21) had median nonrepair rates of 19.8% (range 0%-66.7%) and 16.7% (5.1%-38.5%), respectively. High-volume centers were further dichotomized by rate of nonrepair (HiLo = 5.1-16.7% and HiHi = 17.6-38.5%), leaving 3 groups: HiLo, HiHi, and Lo. Predictors of mortality were lower birth weight, lower Apgar scores, prenatal diagnosis, and presence of congenital anomalies. O/E ratios for mortality in the HiLo, HiHi, and Lo groups were 0.81, 0.94, and 1.21, respectively (P < 0.0001). For every 100 CDH patients, HiLo centers have 2.73 (2.4-3.1, 95% confidence interval) survivors beyond expectation. CONCLUSIONS: There are significant differences between repaired and nonrepaired CDH infants and significant center variation in rate of nonrepair exists. Aggressive surgical management, leading to a low rate of nonrepair, is associated with improved risk-adjusted mortality.

Percent predicted lung volume changes on fetal magnetic resonance imaging throughout gestation in congenital diaphragmatic hernia.

PURPOSE: Percent predicted lung volume (PPLV)<15% on fetal MRI predicts high-risk CDH. Potential changes in PPLV throughout gestation and impact on risk stratification are unknown. We reviewed CDH patients with serial fetal MRIs to follow PPLV and determine correlation with postnatal outcomes. METHODS: CDH patients with serial fetal MRIs from 2005 to 2015 were included. We recorded prenatal MRI gestational age (GA) and PPLV, postnatal ECMO use, and survival. Data were analyzed by logistic regression and Fisher's exact test. RESULTS: 57 patients had 127 fetal MRI studies. PPLV decreased from mean 25.4% to 19.6% between GA 22.1 and 32.6weeks. A steeper decline in PPLV, regardless of final PPLV, was independently predictive of higher ECMO use (p=0.046) and death (p=0.045). All patients with first PPLV<15% remained high-risk with poor outcomes. Of those with first PPLV>15%, 31% dropped below 15%, having similar ECMO use as the high-risk cohort, but trending toward greater survival (p=0.09). Those with first and final PPLV>15% had significantly less ECMO use (p=0.015) and greater survival (p<0.001) than the high-risk cohort. CONCLUSIONS: On average, PPLV decreases throughout gestation in fetuses with CDH. Serial MRI is recommended for those with initial PPLV>15%, as clinical outcomes tend to mirror the lowest PPLV. TYPE OF STUDY: Treatment study LEVEL OF EVIDENCE: III.

Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change morbidity outcomes for high-risk congenital diaphragmatic hernia survivors?

PURPOSE: In high-risk congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before extracorporeal membrane oxygenation (ECMO) can be initiated. We previously examined ex utero intrapartum treatment (EXIT)-to-ECMO in our most severe CDH patients, but demonstrated no survival advantage. We now report morbidity outcomes in survivors of this high-risk cohort to determine whether EXIT-to-ECMO conferred any benefit. METHODS: All CDH survivors with <15% predicted lung volume (PPLV) from September 1999 to December 2010 were included. We recorded prenatal imaging, defect size, and pulmonary, nutritional, cardiac, and neurodevelopmental outcomes. RESULTS: Seventeen survivors (8 EXIT-to-ECMO, 9 non-EXIT) had an average PPLV of 11.7%. Eight of 9 non-EXIT received ECMO within 2days. There were no significant defect size differences between groups, mostly left-sided (13/17) and type D (12/17). Average follow-up was 6.7years (0-13years). There were no statistically significant differences in outcomes, including supplemental oxygen, diuretics, gastrostomy, weight-for-age Z scores, fundoplication, pulmonary hypertension, stroke or intracranial hemorrhage rate, CDH recurrence, and reoperation. No survivor in our cohort was neurologically devastated. All had mild motor and/or speech delay, which improved in most. CONCLUSIONS: In this pilot series of severe CDH survivors, EXIT-to-ECMO confers neither significant survival nor long-term morbidity benefit. LEVEL OF EVIDENCE: Level III treatment study.

Pulmonary support on day of life 30 is a strong predictor of increased 1 and 5-year morbidity in survivors of congenital diaphragmatic hernia.

PURPOSE: Pulmonary support (PS) on day-of-life-30 (DOL-30) has been shown to be the strongest predictor of subsequent morbidity and in-patient mortality in congenital diaphragmatic hernia (CDH). We hypothesized that PS on DOL-30 can also predict long-term outcomes in CDH survivors. METHODS: We analyzed records of 201 CDH survivors followed by a single multidisciplinary clinic (1995-2010). Follow-up was 83 and 70% at 1 and 5years respectively. PS was defined as: (1) invasive support (n=44), (2) noninvasive support (n=54), or (3) room air (n=103). Logistic regression was used to estimate the adjusted association of PS on DOL-30 with outcomes at 1 and 5-years. RESULTS: Use of PS on DOL-30 was significantly associated with pulmonary and developmental morbidities at 1 and 5-years. Even after adjusting for defect-size and presence of ventilation/perfusion mismatch, greater PS on DOL-30 was associated with a significantly increased odds of requiring supplemental oxygen and developmental referral at 1-year, and asthma and developmental referral at 5-years. CONCLUSION: CDH survivors continue to have significant long-term pulmonary and developmental morbidities. PS on DOL-30 is a strong independent predictor of morbidity at 1 and 5-years and may be used as a simple prognostic tool to identify high-risk infants.

Nutritional outcomes in survivors of congenital diaphragmatic hernia (CDH)-factors associated with growth at one year.

BACKGROUND: Malnutrition is prevalent among congenital diaphragmatic hernia (CDH) survivors. We aimed to describe the nutritional status and factors that impact growth over the 12-months following discharge from the pediatric intensive care unit (PICU) in this cohort. METHODS: CDH survivors, who were discharged from the PICU from 2000 to 2010 with follow-up of at least 12months, were included. Nutritional intake, anthropometric, and clinical variables were recorded. Multivariable linear regression was used to determine factors associated with weight-for-age Z-scores (WAZ) at 12months. RESULTS: Data from 110 infants, 67% male, 50% patch repair, were analyzed. Median (IQR) WAZ for the cohort was -1.4 (-2.4 to -0.3) at PICU discharge and -0.4 (-1.3 to 0.2) at 12-months. The percentage of infants with significant malnutrition (WAZ<-2) decreased from 26% to 8.5% (p<0.001). Patch repair (p=0.009), protein intake<2.3g/kg/day (p=0.014), and birth weight (BW)<2.5kg (p<0.001) were associated with lower WAZ at 12-months. CONCLUSIONS: CDH survivors had a significantly improved nutritional status in the 12-months after PICU discharge. Patch repair, lower BW, and inadequate protein intake were significant predictors of lower WAZ at 12-months. A minimum protein intake in the PICU of 2.3g/kg/day was essential to ensure optimal growth in this cohort.

Sensorineural hearing loss in congenital diaphragmatic hernia survivors is associated with postnatal management and not defect size.

BACKGROUND/PURPOSE: We determined the incidence of sensorineural hearing loss (SNHL; >20dB at any frequency) in a contemporary cohort of congenital diaphragmatic hernia (CDH) survivors at a single tertiary care center and identified potential risk factors for SNHL. METHODS: From 2000 through 2011, clinical and audiologic data were collected on 122 surgically-repaired Bochdalek CDH patients. CDH defect size, duration of ventilation, and cumulative aminoglycoside treatment were used for multivariate logistic regression. RESULTS: Incidence of SNHL was 7.4% (9/122). We identified one significant independent predictor of SNHL: cumulative length of aminoglycoside treatment (P<.001; OR 1.44, 95% CI: 1.13-1.83). CONCLUSIONS: Over the last decade, the incidence of SNHL in our CDH patients is low compared to previous reports in the literature (7.4%) and is not associated with CDH defect size or the need for extracorporeal membrane oxygenation. Prolonged duration of aminoglycoside treatment increases the risk of SNHL independent of defect size and duration of ventilation.

Standardized reporting for congenital diaphragmatic hernia--an international consensus.

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) remains a significant cause of neonatal death. A wide spectrum of disease severity and treatment strategies makes comparisons challenging. The objective of this study was to create a standardized reporting system for CDH. METHODS: Data were prospectively collected on all live born infants with CDH from 51 centers in 9 countries. Patients who underwent surgical correction had the diaphragmatic defect size graded (A-D) using a standardized system. Other data known to affect outcome were combined to create a usable staging system. The primary outcome was death or hospital discharge. RESULTS: A total of 1,975 infants were evaluated. A total of 326 infants were not repaired, and all died. Of the remaining 1,649, the defect was scored in 1,638 patients. A small defect (A) had a high survival, while a large defect was much worse. Cardiac defects significantly worsened outcome. We grouped patients into 6 categories based on defect size with an isolated A defect as stage I. A major cardiac anomaly (+) placed the patient in the next higher stage. Applying this, patient survival is 99% for stage I, 96% stage II, 78% stage III, 58% stage IV, 39% stage V, and 0% for non-repair. CONCLUSIONS: The size of the diaphragmatic defect and a severe cardiac anomaly are strongly associated with outcome. Standardizing reporting is imperative in determining optimal outcomes and effective therapies for CDH and could serve as a benchmark for prospective trials.

Pulmonary support on day 30 as a predictor of morbidity and mortality in congenital diaphragmatic hernia.

PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with significant in-hospital mortality, morbidity and length-of-stay (LOS). We hypothesized that the degree of pulmonary support on hospital day-30 may predict in-hospital mortality, LOS, and discharge oxygen needs and could be useful for risk prediction and counseling. METHODS: 862 patients in the CDH Study Group registry with a LOS ≥ 30 days were analyzed (2007-2010). Pulmonary support was defined as (1) room-air (n=320) (2) noninvasive supplementation (n=244) (3) mechanical ventilation (n=279) and (4) extracorporeal membrane oxygenation (ECMO, n=19). Cox Proportional hazards and logistic regression models were used to determine the case-mix adjusted association of oxygen requirements on day-30 with mortality and oxygen requirements at discharge. RESULTS: On multivariate analysis, use of ventilator (HR 5.1, p=.003) or ECMO (HR 19.6, p<.001) was a significant predictor of in-patient mortality. Need for non-invasive supplementation or ventilator on day-30 was associated with a respective 22-fold (p<.001) and 43-fold (p<.001) increased odds of oxygen use at discharge compared to those on room-air. CONCLUSIONS: Pulmonary support on Day-30 is a strong predictor of length of stay, oxygen requirements at discharge and in-patient mortality and may be used as a simple prognostic indicator for family counseling, discharge planning, and identification of high-risk infants.

Obstruction of the superior vena cava after neonatal extracorporeal membrane oxygenation: association with chylothorax and outcome of transcatheter treatment.

BACKGROUND: Obstruction of the superior vena cava is one of the potential complications of neonatal extracorporeal membrane oxygenation. Chylothorax is a known complication of surgery involving the thoracic cavity in children, and of extracorporeal membrane oxygenation. The aim of this study was to evaluate the association between chylothorax and superior vena cava obstruction after neonatal extracorporeal membrane oxygenation. METHODS AND RESULTS: Twenty-two patients diagnosed with superior vena cava obstruction at ≤ 6 months of age (median 1.8 months) after neonatal extracorporeal membrane oxygenation were compared with a randomly selected cohort of 44 neonatal extracorporeal membrane oxygenation patients without superior vena cava obstruction. Among patients with superior vena cava obstruction, 18 underwent extracorporeal membrane oxygenation for respiratory disease and four for cardiac insufficiency. Chylothorax was more prevalent among patients with superior vena cava obstruction than controls (odds ratio 9.4 [2.2-40], p = .01) and was associated with extension of obstruction into the left innominate vein. Patients with superior vena cava obstruction were supported by extracorporeal membrane oxygenation for a longer duration than controls. Nineteen patients with superior vena cava obstruction (86%) underwent transcatheter balloon angioplasty and/or stent implantation (median 7 days after diagnosis), which decreased the superior vena cava pressure and superior vena cava-to-right atrium pressure gradient and increased the superior vena cava diameter (all p < 0.001). There were no serious procedural adverse events. Six study patients died within 30 days of the diagnosis of superior vena cava obstruction (including three of nine with chylothorax), which did not differ from controls. During a median follow-up of 2.7 yrs, two additional patients died and nine underwent 14 superior vena cava reinterventions. CONCLUSIONS: Among neonates treated with extracorporeal membrane oxygenation, superior vena cava obstruction is associated with an increased risk of chylothorax. In neonates with chylothorax after extracorporeal membrane oxygenation, evaluation for superior vena cava obstruction may be warranted. Although mortality is high in this population, transcatheter treatment can relieve superior vena cava obstruction and facilitate symptomatic improvement.

Management of giant congenital pulmonary airway malformations requiring pneumonectomy.

BACKGROUND: Congenital pulmonary airway malformation (CPAM) is a rare congenital anomaly that occurs sporadically throughout fetal development. CPAM is usually a lobar process, but involvement of an entire lung is exceedingly rare. We present a small series of patients with multilobar CPAM who, to the best of our knowledge, are the first reported cases of giant CPAMs treated with pneumonectomy at birth. METHODS: After institutional review board approval, 93 medical records were reviewed for infants 0 to 12 months with congenital cystic adenomatoid malformation (CCAM) from 1990 to 2011. Three patients with entire lung involvement were included. RESULTS: Two patients were identified prenatally by ultrasonography and 1 was diagnosed at birth. Prenatally, the 2 patients exhibited mediastinal shift and steroids were administered. A pericardial effusion and decreased movement developed in 1 patient, requiring an emergent cesarean section at 31 weeks' gestation. All 3 patients underwent immediate newborn resection. One infant went into cardiac arrest on delivery and was resuscitated and taken for emergent resection. The 2 patients delivered vaginally at term tolerated spontaneous ventilation without positive pressure. All 3 patients underwent an emergent pneumonectomy on the first day of life. The premature infant died intraoperatively after CPAM removal from severe pulmonary hypertension (HTN) and contralateral lung hypoplasia. The hospital course for these patients was characterized by prolonged stays in the intensive care unit (ICU), pneumonia, ventilator dependence, and pulmonary HTN. Bronchoscopy revealed tracheomalacia in 1 patient that was relieved by aortopexy; the other patient had bronchomalacia requiring tracheotomy. CONCLUSIONS: Emergent total pneumonectomy can be lifesaving, although significant pulmonary HTN can be expected. Management options may include fetal surgical intervention, ex utero intrapartum treatment (EXIT), and expectant management. Extracorporeal membrane oxygenation (ECMO), a prolonged ICU course, and symptomatic tracheomalacia/bronchomalacia may be anticipated. Families can be counseled that survival is achievable with adequate contralateral lung development.

Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change outcomes for high-risk patients with congenital diaphragmatic hernia?

PURPOSE: In the most severe cases of congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before advanced therapies such as extracorporeal membrane oxygenation (ECMO) can be initiated. We have previously examined the use of the ex utero intrapartum treatment (EXIT) to ECMO procedure (EXIT with placement on ECMO) in high-risk infants and reported a survival advantage. We report our experience with EXIT to ECMO in a more recent cohort of our patients with most severe CDH. METHODS: Every patient with less than 15% predicted lung volume during January 2005 to December 2010 was included. We obtained data on prenatal imaging, size and location of the defect, and survival. RESULTS: Seventeen high-risk infants were identified. All 17 (100%) received ECMO and required a patch. Six children were delivered by EXIT to ECMO, and only 2 (33%) survived. An additional patient was delivered by EXIT to intubation with ECMO on standby and died. Of the 10 children who did not receive EXIT, 5 (50%) survived. CONCLUSIONS: No clear survival benefit with the use of the EXIT to ECMO procedure was demonstrated in this updated report of our high-risk CDH population. The general application of EXIT to ECMO for CDH is not supported by our results.

Congenital intra-abdominal bilateral juvenile granulosa cell tumors of the testis associated with constitutional loss of material from chromosome 4.

Juvenile granulosa cell tumor (JGCT) is an uncommon gonadal stromal tumor that occurs rarely in the testis. We report a newborn boy with bilateral intra-abdominal JGCT presenting with abdominal distention and respiratory distress at birth. He was taken to the operating room emergently, and 2 large masses connected by gubernacula to the inguinal canals were resected. Associated abnormalities included a constitutional chromosome 4 abnormality, polymicrogyria, and renal cysts. This report describes a rare presentation of JGCT with abdominal compression and expands the literature to include bilateral testicular involvement. Additionally, it is the 1st report of JGCT associated with a chromosome 4 abnormality, highlighting a genetic region that may be important in JGCT development.

Neurodevelopmental outcomes of congenital diaphragmatic hernia survivors followed in a multidisciplinary clinic at ages 1 and 3.

PURPOSE: Infants who survive congenital diaphragmatic hernia (CDH) repair may have ongoing medical and neurodevelopmental morbidity after hospital discharge. We evaluated the relationship between medical and neurodevelopmental outcomes of CDH survivors seen in a multidisciplinary clinic at ages 1 and/or 3. METHODS: From January 1997 to December 2004, 69 (61%) of 112 CDH survivors were followed in our CDH clinic at ages 1 and/or 3. Medical issues (cardiac, pulmonary, gastrointestinal) were tabulated at hospital discharge and at follow-up. Neurodevelopmental data were obtained from clinic assessments by a neurodevelopmental pediatrician. Descriptive results were summarized for each cohort. Multivariate analyses were performed to identify predictors of motor problems at age 1. RESULTS: Of the 69 study participants, 64% were male, 75% had left-sided CDH, 17% had cardiac anomalies, and 25% had other congenital malformations. Nearly all required ventilator management (99%) with a median ventilator time of 14 days (range, 1-54 days); 30% required extracorporeal membrane oxygenation. While 87% of patients had medical issues at hospital discharge, 61% and 67% had medical issues at ages 1 and 3, respectively. Pulmonary problems were noted in 34% and 33% of the ages 1 and 3 cohorts, respectively. Motor and language problems were detected in 60% and 18% of the age 1 cohort and 73% and 60% of the age 3 cohort, respectively. Multivariate analysis found ventilator time as the only independent predictor of motor problems at age 1 (odds ratio, 1.12 per day; 95% confidence interval, 1.05-1.20; P < .01). CONCLUSIONS: Young CDH survivors continue to have ongoing medical problems and a high incidence of motor and language problems. Duration of neonatal ventilatory support was a significant predictor of motor problems at age 1. Prospective studies are needed to confirm these findings.

Impact on family of survivors of congenital diaphragmatic hernia repair: a pilot study.

BACKGROUND/PURPOSE: We hypothesized that long-term impact on the family of survivors of congenital diaphragmatic hernia (CDH) would vary based on the child's current clinical problems and clinical severity, family income, insurance, and out-of-pocket expenses. METHODS: Parents of a retrospective cohort of 53 CDH survivors who underwent repair at Children's Hospital Boston in Massachusetts from 1991 through 1999 completed the Impact on Family Scale (IFS), the Child Health Ratings Inventories General Health Module Parent Report, a rating of their child's clinical severity, an inventory of current clinical problems, and family demographics. Statistical tests of the primary outcome, the IFS total score, were performed. RESULTS: Survivors of CDH had a median age of 8 years. Most families had private insurance (78%) and annual income levels higher than the federal poverty level (96%). The IFS total score was correlated with child's clinical severity (r = 0.71, P < .001) and was worse among families of children with current clinical problems (P = .01), families with public insurance (P = .01), and those with out-of-pocket expenses of at least $500 (P = .002). Regression analysis identified the independent effect of clinical severity on family impact (P < .001). CONCLUSION: Family impact is profound and long-standing at a median of 8 years after surgery for a subset of CDH survivors with more severe conditions and current clinical problems.

Approaches to neurodevelopmental assessment in congenital diaphragmatic hernia survivors.

BACKGROUND: Infants with congenital diaphragmatic hernia require complex surgical care and may have neurodevelopmental morbidity. We examined the performance of reports of motor functioning in 25 congenital diaphragmatic hernia survivors using the parent-completed Developmental Profile-II and a clinical evaluation by a neurodevelopmental pediatrician (MD) measured against the Bayley motor scale. METHODS: Bayley motor scores were dichotomized as normal or abnormal. Sensitivity and specificity were calculated for each test. RESULTS: The median age at assessment was 25 months. Bayley motor scores were abnormal in 77% of infants tested (10/13). The MD examinations detected motor problems in 92% (12/13). Sensitivity and specificity of the MD examination were 1.0 and 0.33, respectively. Developmental Profile-II physical scores were abnormal in 15% (2/13); sensitivity and specificity were 0.2 and 1.0, respectively. CONCLUSIONS: The high rate of abnormal motor findings in this study supports the need for ongoing screening and evaluation. The sensitivity of MD examinations was excellent, but hypotonia findings were not universally corroborated by the Bayley. Although specificity of parent-reported motor findings was high, parents underreported abnormal motor findings. Parental reports of neurodevelopmental problems should be heeded, and physicians should perform screening motor examinations. Bayley assessments may be warranted to determine the functional implications of observed abnormalities.

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.

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.