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Background Surgical intestinal disorders, such as necrotizing enterocolitis ( NEC), focal intestinal perforation ( FIP), and meconium-related ileus ( MRI), are serious morbidities in very low-birthweight infants ( VLBWI). The aim of this study was to compare the composite outcomes of death or neurodevelopmental impairment ( NDI) in VLBWI with surgical intestinal disorders and assess independent risk factors for death and NDI at 18 months of corrected age. Methods A retrospective matched-cohort study was conducted at 11 institutes. We included VLBWI who had undergone laparotomy for NEC, FIP, and MRI. Two control subjects were chosen for every surgical patient and matched for gestational age and birthweight to form the comparison group. Death and neurodevelopmental outcome at 18 months of corrected age were evaluated. Results The number of infants in the NEC, FIP, MRI, and control groups was 44, 47, 42, and 261, respectively. In-hospital mortality was higher in infants with NEC and MRI relative to those in the control group ( P < 0.001). The incidence rate for NDI at 18 months of corrected age was higher in infants with MRI relative to those in the control group ( P = 0.021). On logistic regression analysis, low gestational age, male sex, small for gestational age, intraventricular hemorrhage, and MRI were associated with increased risk of death or NDI at 18 months of corrected age. Conclusions NEC and MRI were associated with in-hospital mortality, and MRI was associated with NDI or death at 18 months of corrected age. [ABSTRACT FROM AUTHOR]

Background Hypoxic-ischemic encephalopathy ( HIE) is one of the most critical pathologic conditions in neonatal medicine due to the potential for neurological deficits in later life. We investigated the incidence of term infants with moderate or severe HIE in Japan and identified prognostic risk factors for poor outcome in HIE. Methods Data on 227 infants diagnosed with moderate or severe HIE and born between January and December 2008 were collected via nationwide surveys from 263 responding hospitals. Using logistic regression, we examined the relationship between maternal, antepartum, intrapartum, and neonatal risk factors and clinical outcome at 18 months following birth. Results In Japan, the incidence of moderate or severe HIE was 0.37 per 1000 term live births. Outborn births, low Apgar score at 5 min, use of epinephrine, and low cord blood pH were intrapartum factors significantly associated with neurodevelopmental delay and death at 18 months. Serum lactate, lactate dehydrogenase, aspartate aminotransferase, alanine aminotransferase (all, P < 0.001) and creatine kinase ( P = 0.002) were significantly higher in infants with poor outcome compared to those with favorable outcomes. Abnormal brain magnetic resonance imaging ( MRI), an important prognostic factor, was significantly associated with poor outcome (odds ratio, 11.57; 95% confidence interval: 5.66-23.64; P < 0.001). Conclusions Risk factors predicting poor outcome in HIE include outborn birth, low Apgar score at 5 min, use of epinephrine, laboratory abnormalities, and abnormal MRI findings. [ABSTRACT FROM AUTHOR]

Introduction:  We aimed to establish and validate a risk score for fetuses with congenital diaphragmatic hernia (CDH) using only prenatal ultrasound findings.Material and Methods:  Derivation (2011-2016, n = 350) and validation (2006-2010, n = 270) cohorts were obtained from a Japanese CDH study group database. Using a logistic regression analysis, we created a prediction model and weighted scoring system from the derivation dataset and calculated the odds ratio of an unsatisfactory prognosis (death within 90 days of life or hospitalization duration exceeding 180 days). Five adverse prognostic factors obtained using prenatal ultrasound, including an observed/expected lung area-to-head circumference ratio (o/eLHR) <25%, liver herniation occupying more than one-third of the thoracic space, thoracic stomach, right-side CDH, and severe malformations, were used as predictors. The obtained model was validated using the validation cohort.Results:  The unsatisfactory prognosis prediction model was obtained based on the adjusted odds ratios. The C statistics of the model were 0.83 and 0.80 in the derivation and validation datasets, respectively. The five variables were weighted proportionally to their adjusted odds ratios for an unsatisfactory prognosis (o/eLHR <25%, 1 point; liver herniation occupying more than one-third of the thoracic space, 1 point; thoracic stomach, 1 point; right-side CDH, 2 points; and severe malformations, 3 points). Unsatisfactory prognosis rates for the low- (0-2 points), intermediate- (3-5 points), and high-risk (6-8 points) groups were 17, 46, and 100%, respectively (p < 0.001), in the validation cohort.Conclusion:  Our simple risk score effectively predicted the prognosis of fetuses with CDH. [ABSTRACT FROM AUTHOR]

Aim: Antenatal corticosteroids (ACS) are recommended for women at risk of preterm birth before 34 weeks' gestation. However, adverse effects of ACS on the fetal brain have also been reported. The time interval from ACS administration to delivery (ACS‐to‐delivery interval) might alter the effect of ACS on the fetal brain. This study aimed to evaluate the effect of ACS‐to‐delivery interval on cord blood S100 calcium‐binding protein B (S100B) levels as a biomarker of brain damage. Methods: Women who delivered between 2012 and 2020 at a tertiary medical center were divided into three groups according to ACS use and ACS‐to‐delivery interval, retrospectively: non‐ACS, ACS ≤7 days, and ACS >7 days. Patients who did not complete the ACS regimen were excluded. The primary outcome was cord blood S100B levels. Results: Cord blood S100B levels were significantly lower in the ACS ≤7 days group than in the non‐ACS and ACS >7 days groups. In the multiple regression analysis, birth ≤7 days after ACS showed a significant negative association with S100B level (p < 0.001). Conclusions: Reduced S100B levels were observed in infants born ≤7 days after ACS but not in infants born >7 days after ACS. These findings suggest the importance of ACS timing to optimize its effects on the fetal brain, although further studies are required to identify these mechanisms. [ABSTRACT FROM AUTHOR]

Background Very low-birthweight (VLBW) infants (VLBWI) are at increased risk for surgical intestinal disorders including necrotizing enterocolitis (NEC), focal intestinal perforation (FIP) and meconium-related ileus (MRI). The aim of this study was to identify disease-specific risk factors for surgical intestinal disorders in VLBWI. Methods A retrospective multicenter case-control study was conducted at 11 institutes. We reviewed VLBWI who underwent laparotomy for intestinal disorders including perforation and intractable bowel obstruction. The surgical disorders were classified into four categories (NEC, FIP, MRI, others) based on the macroscopic findings at operation. In order to identify risk factors, two matched controls for each subject were chosen based on gestational age and birthweight. OR and 95%CI were calculated using a conditional logistic regression model and a multivariate model. Results A total of 150 cases (NEC, n = 44; FIP, n = 47; MRI, n = 42; others, n = 17) and 293 controls were identified. The cases and controls were similar in terms of gestational age and birthweight (cases/controls, 26.7 ± 2.5/26.5 ± 2.6 weeks; 790 ± 256/795 ± 257 g). On multivariate modeling, disease-specific risk factors were as follows: female (OR, 0.23; 95%CI: 0.06-0.89), respiratory distress syndrome (OR, 35.7; 95%CI: 2.48-514) and patent ductus arteriosus (OR, 10.9; 95%CI: 1.51-79.3) for NEC; outborn delivery (OR, 5.47; 95%CI: 1.48-20.2) for FIP; and twin pregnancy (OR, 4.25; 95%CI: 1.06-17.1), PROM (OR, 6.85; 95%CI: 1.33-35.4) and maternal steroid (OR, 0.23; 95%CI: 0.07-0.79) for MRI. Conclusions Different risk factors were identified for NEC, FIP and MRI, suggesting that each disease has a different etiology, and that different strategies are required to prevent these diseases. [ABSTRACT FROM AUTHOR]

Purpose: Predicting lethal pulmonary hypoplasia in infants with congenital diaphragmatic hernia (CDH) before extracorporeal membrane oxygenation (ECMO) initiation is difficult. This study aimed to predict lethal pulmonary hypoplasia in patients with CDH prior to ECMO. Methods: This was a multicenter cohort study involving neonates prenatally diagnosed with isolated unilateral CDH (born 2006–2020). Patients who required ECMO due to respiratory insufficiency were included in this study. Patients who underwent ECMO due to transient disorders were excluded from analysis. Blood gas analysis data within 24 h of birth were compared between survivors and non-survivors. Predictive abilities were assessed for factors with significant differences. Results: Overall, 34 patients were included (18 survivors and 16 non-survivors). The best pre-ductal PaO2 was significantly lower in non-survivors than in survivors (50.4 [IQR 30.3–64.5] vs. 67.5 [IQR 52.4–103.2] mmHg, respectively; p = 0.047). A cutoff PaO2 of 42.9 mmHg had a sensitivity, specificity, and positive predictive value of 50.0%, 94.4%, and 88.9%, respectively, to predict mortality. Conclusion: The best PaO2 within 24 h after birth predicted mortality following ECMO initiation. This should be shared to families and caregivers to optimize the best interests of the infants with CDH. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to elucidate the clinical characteristics of neonates with congenital diaphragmatic hernia (CDH) associated with pneumothorax and evaluate the risk factors for the development of pneumothorax.Methods: A retrospective cohort study was conducted in the 15 institutions participating in the Japanese CDH Study Group. A total of 495 neonates with isolated CDH who were born between 2011 and 2018 were analyzed in this study.Results: Among the 495 neonates with isolated CDH, 52 (10.5%) developed pneumothorax. Eighteen (34.6%) patients developed pneumothorax before surgery, while 34 (65.4%) developed pneumothorax after surgery. The log-rank test showed that the cumulative survival rate was significantly lower in patients with pneumothorax than in those without pneumothorax. Univariate analysis revealed significant differences between patients with pneumothorax and those without pneumothorax with regard to the best oxygenation index within 24 h after birth, mean airway pressure (MAP) higher than 16 cmH2O, diaphragmatic defect size, and need for patch closure. Multiple logistic regression analysis indicated that only the MAP was associated with an increased risk of pneumothorax.Conclusions: The cumulative survival rate was significantly lower in isolated CDH patients with pneumothorax than in those without pneumothorax. A higher MAP was a risk factor for pneumothorax in CDH patients. [ABSTRACT FROM AUTHOR]

Background: Many small for gestational age (SGA) infants have catch‐up growth during the first 2 years of life, but approximately 10% have no catch‐up growth, and short stature continues into adulthood. Identification of risk factors for absence of catch‐up growth at an early age may be useful for earlier diagnosis and earlier treatment. Methods: This was a retrospective multicenter study. The subjects were SGA infants with very low‐birthweight (VLBW), who were followed up until the age of 3 years. The risk factors for absence of catch‐up growth were identified on statistical analysis. Results: Of the 217 SGA infants in this study, 181 were in the catch‐up group and 36 were in the no catch‐up group. The catch‐up rate was 83%. On multivariate analysis adjusted for gestational age, birthweight, birth height, and birth head circumference, multipara, Z and ΔZ scores of length at 12 months of corrected age, and the Z score of height at 24 months of corrected age were risk factors for lack of catch‐up at 3 years. Conclusions: The length Z and ΔZ scores at 12 months of corrected age may be useful for an earlier diagnosis and earlier initiation of growth hormone treatment in VLBW infants. [ABSTRACT FROM AUTHOR]

Background: The optimum enteral (EN) and parenteral nutrition (PN) regimens during acute management of congenital diaphragmatic hernia (CDH) remain unclear. We examined the effects of EN and PN on weight gain in CDH patients. Methods: A multicenter retrospective cohort study of neonates with CDH (born 2006–2010; n = 105) who survived to discharge was conducted. Patients were divided as receiving PN ≥ or <50 kcal/kg/day at 1 week of age, and EN ≥ or <60 kcal/kg/day at 2 weeks of age. Changes in bodyweight at 30, 60, and 90 days of age were compared. Results: The higher EN group (n = 39) had greater mean weight gain than the lower EN group (n = 66; 90 days: 2,501 g, 95% CI: 2,294–2,710 g vs 1,706 g, 95% CI: 1,553–1,861 g; P <0.001). When patients received lower EN, the higher PN group (n = 24) had greater mean weight gain than the lower PN group (n = 42; 90 days: 1,768 g, 95% CI: 1,574–1,961 g vs 1,411 g, 95% CI: 1,264–1,558 g; P = 0.004). Conclusion: The amount of EN in the acute phase of CDH management is essential for weight gain during infancy. When patients are intolerant to adequate EN, supportive PN is also essential. [ABSTRACT FROM AUTHOR]

Background Hydrops fetalis ( HF) has a low survival rate, particularly in the case of preterm birth. In addition, the severity index of HF has not been fully investigated yet. The aim of this study was to clarify the prognostic factors of HF with pleural effusion. Methods All live-born HF patients with pleural effusion, except for chromosomal abnormality or complex congenital heart disease, born from 2009 to 2013 in Aichi Prefecture in Japan were included. Prenatal, perinatal, and postnatal information was obtained from the medical records and was retrospectively analyzed. Results Forty-one HF patients with pleural effusion were included, and 28 patients (68%) survived. On multivariate logistic stepwise analysis, gestational birth week ( OR, 0.71; 95% CI: 0.52-0.96, P = 0.027) and standard deviation ( SD) score of the birthweight ( OR, 1.74; 95% CI: 1.01-2.99, P = 0.045) were significant factors for postnatal death. All patients with both ≥32 gestational weeks and <3.0 birthweight SD score survived. Conclusions Combined with the gestational weeks data, birthweight SD score may be useful to estimate the prognosis of HF with pleural effusion. [ABSTRACT FROM AUTHOR]

Introduction: Little information exists about the physical growth of patients with congenital diaphragmatic hernia (CDH). This study aimed to assess the growth of patients with CDH during long follow-up periods, and to identify growth retardation (GR) risk factors.Patients and Methods: A multicenter retrospective observational study was conducted in 2013. Of the 228 patients with CDH born between 2006 and 2010, 182 (79.8%) survived to discharge, and 174 cases were included in the study. Body weights and heights were measured at 1.5, 3, and 6 years of age. GR was defined as a Z-score relating to the weight or height of < - 2.0. Cases with GR at 1.5, 3, or 6 years of age comprised the GR group. The clinical variables of the GR and non-GR groups were compared using univariate analysis. Multiple logistic regression analyses were conducted successively on the factors that were significant at p < 0.01 in the univariate analysis and had low correlations with other factors (r < 0.7). The numerical data were divided into two groups based on a cutoff value that was calculated from a receiver operating characteristic curve.Results: The GR group comprised 35 cases (22.7%). The rates of GR at 1.5, 3, and 6 years of age were 19.5 (26/133), 14.4 (16/111), and 13.5% (5/37), respectively. The body weight Z-scores improved in cases with GR between the ages of 1.5 and 3 years (p = 0.036). As the patients aged, the wasting type of GR decreased in frequency (31, 0, and 0% at 1.5, 3, and 6 years of age, respectively) and the stunting type of GR increased in frequency (27, 31, and 100% at 1.5, 3, and 6 years of age, respectively). The univariate analysis showed that birth weight and height, liver-up, large defect size of the diaphragm, use of nitric oxide, patch repair, long hospital stay, home oxygen treatment (HOT), and vasodilator administration at discharge were significant risk factors of GR. The multivariate analysis determined that a birth weight of < 2,698 g (odds ratio [OR] = 5.5, 95% confidence interval [CI] = 2.1-16.8, p < 0.001) and HOT (OR = 5.8, 95%CI = 1.6-23.8, p = 0.007) were significant risk factors for GR.Conclusion: GR was observed in 22.7% of the CDH survivors. Body weight improved between 1.5 and 3 years of age in the GR cases, but some patients developed chronic malnutrition via acute malnutrition. Low birth weight and the need for HOT were GR risk factors. Aggressive management of acute malnutrition may improve the growth of patients with CDH. [ABSTRACT FROM AUTHOR]

Background The aim of this study was to clarify how extracorporeal membrane oxygenation ( ECMO) is used to treat congenital diaphragmatic hernia ( CDH) in Japan. Methods We completed a nationwide survey of CDH involving 614 infants. The subjects included 43 patients who underwent ECMO. We compared the clinical data of the patients who did and did not survive ≥90 days, and analyzed the 24 h blood gas data in isolated CDH cases in both groups. Results Of the 43 CDH patients, non-isolated CDH associated with other life-threatening or chromosomal anomalies was diagnosed in six patients. Only one of these six patients was able to discontinue ECMO and survived, and the other five died shortly after birth. The other 37 patients all had isolated CDH. The reason for initiating ECMO in 31 of these patients was persistent pulmonary hypertension of the newborn ( PPHN). In the 37 patients with isolated CDH, ECMO was initiated within 24 h after birth. Sixteen patients (37%) survived ≥90 days, and intact discharge was possible in eight cases. Among the isolated CDH patients, on ROC analysis of the lowest oxygenation index ( OI) to predict 90 day survival, the cut-off was 15. Conclusions ECMO is used to treat PPHN starting from an early period after birth, but the mortality and morbidity are not favorable. For lowest OI, the index used to predict survival following ECMO, the cut-off was 15. [ABSTRACT FROM AUTHOR]

Purpose: Gastroesophageal reflux disease (GERD) is one of the concomitant problems in infants with congenital diaphragmatic hernia (CDH). We assessed risk factors of GERD in CDH patients. Methods: The retrospective observational study for CDH infants was conducted. Cases of CDH who were born between January 2006 and December 2010, were operated in the 9 participating institutions, and survived to discharge were included. Completion of medical therapy for GERD and incidence of surgery were primary outcomes. Kaplan-Meier survival analysis and Cox proportional hazards regression were used. Results: In 182 cases of CDH, the medical therapies for GERD were performed in 23.8 % (40/168), and were completed in 60.0 % (24/40). Prenatal detection of CDH (HR 5.87, CI 1.6-18.8, p = 0.012) and tube feeding at discharge (HR 5.04, 95 % CI 1.3-33.1, p = 0.016) were significantly correlated with unsuccessful weaning from medical therapy. Surgery for GERD was performed in 10.7 % (18/169). Gestational age (HR 4.78, 95 % CI 1.5-21.1, p = 0.006) and diaphragmatic defect of more than 75 % (HR 4.3, 95 % CI 1.6-12.9, p = 0.005) were significantly correlated with need for antireflux surgery. Conclusion: Diaphragmatic defect of more than 75 % was risk factor of future need for antireflux surgery. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: To clarify the differences between infants with periventricular hemorrhagic infarction (PVHI) and those with periventricular leukomalacia (PVL). Methods: We retrospectively evaluated the clinical features, ultrasonography, and electroencephalogram (EEG) findings in 22 preterm infants with PVHI and 49 with PVL. EEG and cranial ultrasonography were serially performed in all participants starting immediately after birth. Acute and chronic stage EEG abnormalities were evaluated separately. Results: Gestational age and birth weight were significantly lower in infants with PVHI than those with PVL. EEGs were normal in the majority of infants with PVHI on days 1–2. However, EEG abnormalities appeared after ultrasonography abnormalities. The majority of infants with PVL showed acute-stage EEG abnormalities on days 1–2. The rate of infants with acute-stage EEG abnormalities decreased with age, whereas the rate of infants with chronic-stage EEG abnormalities increased with age. Normal EEG before ultrasonography abnormalities was more common in infants with PVHI than in those with PVL. However, deterioration of acute-stage EEG abnormalities was more frequent in infants with PVHI than in those with PVL. Conclusions: PVHI was presumed to cause mostly postnatal injury, whereas PVL was presumed to cause mostly pre-or perinatal injury. [Copyright &y& Elsevier]

Background The aim of this study was to clarify the outcome of patients with cardiovascular malformation ( CVM) among those with congenital diaphragmatic hernia ( CDH) who are indicated for the Fontan procedure. Methods The subjects included 76 CDH patients with CVM recruited from a national survey of 614 CDH patients. The outcomes were evaluated between two groups divided according to indication for the Fontan procedure. Patients with functional univentricular disease were considered to be candidates for the Fontan procedure. Results Sixteen (21.1%) of the 76 patients were candidates for the Fontan procedure, accounting for 2.6% of all 614 patients with CDH. None of these patients, however, underwent the Fontan procedure. Among the 16 patients, the absence of obstruction of the left ventricular outflow tract ( LVOTO) was significantly associated with better 90 day survival (71.4%, 5/7, for those without LVOTO vs 0.0%, 0/9, for those with LVOTO, P = 0.0007). After excluding 22 patients with chromosomal and/or genetic abnormalities or syndromes, the 90 day survival rate was significantly better in neonates without than with indication for the Fontan procedure (62.5%, 25/40 vs 28.6%, 4/14, P = 0.0271). Conclusions Patients with indications for the Fontan procedure are rare, and the outcome of patients with LVOTO among those with CDH is especially poor. [ABSTRACT FROM AUTHOR]

Background Congenital diaphragmatic hernia is associated with cardiovascular malformation. Many prognostic factors have been identified for isolated congenital diaphragmatic hernia; however, reports of concurrent congenital diaphragmatic hernia and cardiovascular malformation in infants are limited. This study evaluated congenital diaphragmatic hernia associated with cardiovascular malformation in infants. Factors associated with prognosis for patients were also identified. Methods This retrospective cohort study was based on a Japanese survey of congenital diaphragmatic hernia patients between 2006 and 2010. Frequency and outcome of cardiovascular malformation among infants with congenital diaphragmatic hernia were examined. Severity of congenital diaphragmatic hernia and cardiovascular malformation were compared as predictors of mortality and morbidity. Results Cardiovascular malformation was identified in 76 (12.3%) of 614 infants with congenital diaphragmatic hernia. Mild cardiovascular malformation was detected in 19 (33.9%) and severe cardiovascular malformation in 37 (66.1%). Their overall survival rate at discharge was 46.4%, and the survival rate without morbidity was 23.2%. Mortality and morbidity at discharge were more strongly associated with severity of cardiovascular malformation (adjusted OR 7.69, 95% CI 1.96-30.27; adjusted OR 7.93, 95% CI 1.76−35.79, respectively) than with severity of congenital diaphragmatic hernia. Conclusions The prognosis for infants with both congenital diaphragmatic hernia and cardiovascular malformation remains poor. Severity of cardiovascular malformation is a more important predictive factor for mortality and morbidity than severity of congenital diaphragmatic hernia. [ABSTRACT FROM AUTHOR]

Background: Infants born to mothers with hypertensive disorders of pregnancy (HDP) have adverse neurodevelopmental consequences in later life. Magnetic resonance spectroscopy (MRS) is used to predict subsequent neurodevelopment in the field of perinatology.Aim: We aimed to determine whether exposure to HDP in utero leads to alterations in brain metabolites in preterm infants using multi-voxel proton MRS at term-equivalent age.Study Design: Retrospective cohort study.Subjects: A total of 103 preterm infants born before 34 weeks of gestation at Nagoya University Hospital between 2010 and 2018 were eligible. Twenty-seven infants were born to mothers with HDP (HDP group), and 76 were born to mothers without HDP (non-HDP group).Outcome Measures: The peak area ratios of N-acetylaspartate (NAA)/choline (Cho), NAA/creatine (Cr), and Cho/Cr were evaluated at 10 designated regions of interest (bilateral frontal lobes, basal ganglia, thalami, temporal lobes, and occipital lobes).Results: The peak area ratios of NAA/Cho and NAA/Cr in the bilateral thalami were significantly higher in the HDP group than in the non-HDP group after adjustment for covariates (postmenstrual age at MRS assessment and infant sex). No significant differences were observed in other regions. Preeclampsia, abnormal umbilical artery blood flow, and fetal growth restrictions were significantly associated with increased NAA/Cho and NAA/Cr ratios in the thalami.Conclusions: Based on the evidence that NAA/Cho and NAA/Cr ratios constantly increase with postmenstrual age in normal brain development, exposure to maternal HDP in utero may accelerate brain maturation and increase neuronal activity in preterm infants. [ABSTRACT FROM AUTHOR]

Aim: To clarify auditory brainstem response (ABR) in preterm infants with bilirubin encephalopathy and the relationships between ABR and clinical variables.Method: We retrospectively reviewed the ABR waveforms of 56 preterm infants with BE and graded them as "no response", "abnormal interwave separation", or "normal". Patient backgrounds, the peak total bilirubin level, the bilirubin/albumin ratio, verbal communication ability, and newborn hearing screening test results from an automated ABR evaluation had been collected during an earlier nationwide survey.Results: The frequency of abnormal ABR findings decreased with age. Verbal communication tended to be poorer in patients with more severe ABR abnormalities. ABR findings improved in 7 of 29 infants with available serial ABR data. Both gestational age and the peak total bilirubin level were relatively lower in patients with than in those without improved ABR findings. Newborn hearing screening using automated ABR evaluation yielded data consistent with manual ABR findings in 16 of 20 patients who underwent both examinations.Conclusions: ABR abnormalities in preterm infants with bilirubin encephalopathy may improve over time, especially in those with a lower gestational age and peak total bilirubin level. Newborn hearing screening using automated ABR may fail to detect abnormalities in some infants. [ABSTRACT FROM AUTHOR]

Background The aim of this study was to investigate the natural history and associated predictors of musculoskeletal deformities in congenital diaphragmatic hernia (CDH) survivors. Methods A multicenter retrospective survey was conducted among CDH patients between January 2006 and December 2010 in Japan, and a follow-up survey was performed between September 2013 and October 2013. One hundred and eighty-two (79.8%) of the 228 patients were alive. An orthopedic survey of 159 survivors without severe coexisting congenital anomalies was subsequently carried out, and the rates of pectus excavatum (PE), scoliosis and chest asymmetry were evaluated. Results Scoliosis, PE and chest asymmetry were found in 20 (12.6%), 19 (11.9%) and 12 (7.5%) patients, respectively. In total, 44 patients (27.7%) developed orthopedic abnormalities. Reduction in the oxygenation index within 24 h after birth ( P = 0.044), large diaphragmatic defects ( P = 0.047) and patch repair ( P = 0.014) were predictive for scoliosis. In addition, Apgar score at 5 min was significantly lower in the patients who developed PE ( P = 0.034); and stomach herniation ( P = 0.004) and liver herniation ( P = 0.013) at prenatal diagnosis and large diaphragmatic defects ( P = 0.036) were predictive of chest asymmetry. Conclusions Approximately one-quarter of the survivors developed musculoskeletal abnormalities in the present survey of CDH patients. These data suggest that each musculoskeletal abnormality has its own specific predictors. [ABSTRACT FROM AUTHOR]