BACKGROUND AND OBJECTIVE

Pediatric post-operative congenital diaphragmatic hernia (CDH) patients have been shown to encounter reduced pulmonary function tests (PFT) potentially leading to respiratory symptoms. Strategies involving exercise have been used to improve PFT. This meta-analysis aims to determine the effectiveness of exercise in improving lung function in pediatric post-operative CDH patients.

An electronic search was done on May 2023 in MEDLINE via Pubmed, Cochrane Library, Embase, ClinicalKey, Scopus, Google Scholar, and Herdin Plus, using the search terms "exercise" and "congenital diaphragmatic hernia" and "children" or "pediatric" and "pulmonary function" or "lung function". The study included pediatric patients in whom CDH has been surgically corrected, and excluded patients who were unable to perform the test maneuvers, have cardiopulmonary instability, and have serious associated anomalies. Randomized controlled trials (RCT) were identified and independently assessed by two review authors. Each RCT was independently assessed for bias by two review authors using the Cochrane Handbook for Systematic Reviews of Interventions. The RevMan 5.4 software was used for statistical analysis.

A total of 124 participants from three studies were included in the meta-analysis. The pooled mean difference showed a significantly higher mean functional vital capacity (FVC) (MD=6.12, 95%CI=3.91 to 8.33, p-value < 0.00001) and forced expiratory volume in 1 second (FEV1 ) (MD=6.25, 95%CI=3.39 to 9.10, p-value < 0.0001) in the study group compared to the control group.

Exercise may be effective in improving lung function in children with pediatric post-operative CDH. However, the study is limited by its small sample size, the lack of assessment of long-term outcomes, and the difference in exercise regimens used in each RCT. Further studies are recommended to determine the most optimal exercise regimen and to measure its effect on the other outcomes for this population.

Hernias, Diaphragmatic, Congenital/surgery* ; Humans ; Liver/diagnostic imaging*

Gastric volvulus (GV) is an uncommon pathology, with 10-20% of cases occurring in children, typically before one year of age. It often occurs in people with congenital diaphragmatic hernias, intestinal malrotation, eventration of the diaphragm, paraesophageal hernias, wandering spleens, asplenism, or intra-abdominal adhesions. We report a rare case of chronic GV after left hemihepatectomy for hepatoblastoma in a child. The patient was a 9-year-old boy who complained of upper abdominal pain and postprandial upper abdominal distension for one year. At the age of 4 months, he was diagnosed with hepatoblastoma and had undergone left hemihepatectomy. The upper gastrointestinal contrast study revealed chronic organoaxial gastric volvulus. After a surgical procedure involving adhesiolysis and an anterior wall gastropexy, the patient improved and the symptoms resolved. Although GV is a rare disease, it should be suspected in a patient with a previous abdominal surgical history who is complaining of abdominal distension and pain.
Abdominal Pain ; Child ; Diaphragm ; Gastropexy ; Hepatectomy ; Hepatoblastoma ; Hernia, Hiatal ; Hernias, Diaphragmatic, Congenital ; Humans ; Male ; Pathology ; Rare Diseases ; Stomach Volvulus ; Wandering Spleen

Adolescent ; Colon ; diagnostic imaging ; pathology ; Hernias, Diaphragmatic, Congenital ; diagnostic imaging ; pathology ; Humans ; Ileum ; diagnostic imaging ; pathology ; Male ; Thoracic Cavity ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed

To investigate risk factors of death in newborns with congenital diaphragmatic hernia (CDH). A total of 126 newborns with CDH from June 2012 to September 2018 were enrolled. Concomitant malformations were recorded by descriptive analysis. Newborns received surgical treatment (=120) for CDH were divided into survival group and fatal group. The risk factors of death were analyzed by univariate and multivariate logistic regression and the ROC curve with generated with relevant variables. There were 55 CDH newborns with concomitant malformations (43.7%), including 20 cases (15.9%) with multi-malformation. Logistic regression analysis showed that premature rupture of membranes (PROM), postoperative atelectasis, long duration of postoperative mechanical ventilation, postoperative high oxygenation index (OI) were related to death (all <0.05), and the delayed surgery was a protective factor (<0.05). In ROC analysis of postoperative OI in predicting death, the area under the curve (AUC) was 0.841, with the cutoff value of 5.74, the sensibility and specificity of OI was 81.0% and 75.0%, respectively(<0.01). Newborns with CDH have a high rate of malformations. The risk factors of death were PROM, postoperative atelectasis, postoperative long duration of mechanical ventilation and higher postoperative OI, and delayed surgery may reduce mortality.
Hernias, Diaphragmatic, Congenital ; diagnosis ; mortality ; surgery ; Humans ; Infant ; Infant Death ; Infant, Newborn ; Logistic Models ; ROC Curve ; Retrospective Studies ; Risk Factors ; Sensitivity and Specificity

Reported here is the case of a 6 year old male child with a left Congenital Diaphragmatic Hernia who underwent thoracoscopic repair. The use of continuous low pressure CO2 insufflation pneumothorax during the procedure not only made manipulation during the reduction of bowel contents back into the abdomen easier, but also facilitated repair of the defect as it maintained bowel reduction during suturing. Although prolonged CO2 has been hypothesized to be hazardous leading to hypercapnea and aggravating pulmonary hypertension in Congenital Diaphragmatic Hernia patients. The authors found it to be a safe modification of the technique as long as pre-operative planning, patient selection and intra-operative maneuvers were proprely performed.
Hernias, Diaphragmatic, Congenital

PURPOSE: Congenital diaphragmatic hernia (CDH) is rare but potentially fatal. The overall outcome is highly variable. This study aimed to identify a simple and dynamic parameter that helps predict the mortality of CDH patients in real time, without invasive tests. METHODS: We conducted a retrospective chart review of 59 CDH cases. Maternal and fetal information included the gestational age at diagnosis, site of defect, presence of liver herniation, and lung-to-head ratio (LHR) at 20 to 29 weeks of gestational age. Information regarding postnatal treatment, including the number of days until surgery, the need for inhaled nitric oxide (iNO), the need for extracorporeal membrane oxygenation (ECMO), and survival, was collected. The highest respiratory severity score (RSS) within 24 hours after birth was also calculated. RESULTS: Statistical analysis showed that a younger gestational age at the initial diagnosis (P < 0.001), a lower LHR (P=0.001), and the presence of liver herniation (P=0.003) were prenatal risk factors for CDH mortality. The RSS and use of iNO and ECMO were significant factors affecting survival. In the multivariate analysis, the only remaining significant risk factor was the highest preoperative RSS within 24 hours after birth (P=0.002). The area under the receiver operating characteristic curve was 0.9375, with a sensitivity of 91.67% and specificity of 83.87% at the RSS cut-off value of 5.2. The positive and negative predictive values were 82.14% and 92.86%, respectively. CONCLUSION: Using the RSS as a prognostic predictor with simple calculations will help clinicians plan CDH management.
Diagnosis ; Extracorporeal Membrane Oxygenation ; Gestational Age ; Hernias, Diaphragmatic, Congenital* ; Humans ; Infant, Newborn ; Liver ; Mortality* ; Multivariate Analysis ; Nitric Oxide ; Parturition ; Prognosis ; Retrospective Studies ; Risk Factors ; ROC Curve ; Sensitivity and Specificity

PURPOSE: We aimed to compare the clinical characteristics between neonates with persistent pulmonary hypertension of neonates (PPHN) with parenchymal lung disease (PLD) and those with idiopathic PPHN. METHODS: We reviewed the medical records of 67 neonates with gestational ages not lesser than 34⁺⁰ weeks who were born at Inje University Sanggye Paik Hospital between June 1, 2005 and December 31, 2016. We excluded 10 neonates who presented with congenital anomalies (n=3), dextrocardia (n=1), triple X syndrome (n=1), death before treatment (n=1), neonatal asphyxia (n=2), and congenital diaphragmatic hernia (n=2). Neonates were categorized into 2 groups—PPHN with PLD (PLD group, those diagnosed with PLD such as respiratory distress syndrome or meconium aspiration syndrome, n=36) and idiopathic PPHN (idiopathic group, n=21). We compared the clinical characteristics, treatment, and laboratory findings between the groups. RESULTS: The PLD group neonates showed a greater requirement for positive pressure ventilation in the delivery room, higher frequency of meconium staining of amniotic fluid, and greater need for surfactant application than those belonging to the idiopathic group. In contrast, epinephrine use was more common in the idiopathic PPHN group than in the PLD group. The 1-minute Apgar score and pH observed on initial capillary blood gas analysis were lower in the PLD than in the idiopathic group. Severity scores were higher in the idiopathic than in the PLD group 4–7 days after birth. CONCLUSION: In our study, an overall simplified severity score in the first week after birth was higher in the idiopathic than in the PLD group. These results were particularly statistically significant over postnatal days 4–7.
Amniotic Fluid ; Apgar Score ; Asphyxia ; Blood Gas Analysis ; Capillaries ; Delivery Rooms ; Dextrocardia ; Epinephrine ; Female ; Gestational Age ; Hernias, Diaphragmatic, Congenital ; Humans ; Hydrogen-Ion Concentration ; Hypertension, Pulmonary* ; Infant, Newborn* ; Lung Diseases* ; Lung* ; Meconium ; Meconium Aspiration Syndrome ; Medical Records ; Parturition ; Positive-Pressure Respiration

Malposition of the extracorporeal membrane oxygenation (ECMO) venous cannula in the azygos vein is not frequently reported. We hereby present such a case, which occurred in a neonate with right-sided congenital diaphragmatic hernia. Despite ECMO application, neither adequate flow nor sufficient oxygenation was achieved. On the cross-table lateral chest radiograph, the cannula tip was identified posterior to the heart silhouette, which implied malposition of the cannula in the azygos vein. After repositioning the cannula, the target flow and oxygenation were successfully achieved. When sufficient venous flow is not achieved, as in our case, clinicians should be alerted so they can identify the cannula tip location on lateral chest radiograph and confirm whether malposition in the azygos vein is the cause of the ineffective ECMO.
Azygos Vein* ; Catheters* ; Extracorporeal Membrane Oxygenation* ; Heart ; Hernias, Diaphragmatic, Congenital* ; Humans ; Infant, Newborn* ; Oxygen ; Radiography, Thoracic

Malposition of the extracorporeal membrane oxygenation (ECMO) venous cannula in the azygos vein is not frequently reported. We hereby present such a case, which occurred in a neonate with right-sided congenital diaphragmatic hernia. Despite ECMO application, neither adequate flow nor sufficient oxygenation was achieved. On the cross-table lateral chest radiograph, the cannula tip was identified posterior to the heart silhouette, which implied malposition of the cannula in the azygos vein. After repositioning the cannula, the target flow and oxygenation were successfully achieved. When sufficient venous flow is not achieved, as in our case, clinicians should be alerted so they can identify the cannula tip location on lateral chest radiograph and confirm whether malposition in the azygos vein is the cause of the ineffective ECMO.
Azygos Vein ; Catheters ; Extracorporeal Membrane Oxygenation ; Heart ; Hernias, Diaphragmatic, Congenital ; Humans ; Infant, Newborn ; Oxygen ; Radiography, Thoracic