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Journal of Clinical Medicine Mar 2024Pleural effusion and chylothorax are common complications in the treatment of congenital diaphragmatic hernia (CDH). We set out to identify risk factors for chylothorax...
Pleural effusion and chylothorax are common complications in the treatment of congenital diaphragmatic hernia (CDH). We set out to identify risk factors for chylothorax development in patients with CDH and to investigate the association of pleural effusion and chylothorax with neonatal morbidity and mortality. In this retrospective cohort study, we included 396 neonates with CDH treated at our institution between January 2013 and June 2019. Preoperative and postoperative chest radiographs and clinical data were evaluated and correlated with morbidity, complications and mortality. Laboratory-confirmed chylothorax occurred in 58 (18.6%) of all CDH cases. Pleural effusion was frequently observed as a postoperative complication but also occurred as a pre-existing condition. Neonates with large defects of size C and D, patch repair, the need for presurgical and/or postsurgical ECMO support, pulmonary hypertension, liver-up phenomenon and lower relative fetal lung volume were associated with higher occurrences of chylothorax. After stepwise logistic regression, larger CDH defects ( < 0.0001) and the need for postsurgical ECMO ( = 0.0158) remained significant risk factors for CTX to occur (AUC 0.71). The same potential risk factors were used to assess their association with both presurgical and postsurgical pleural effusion. After stepwise logistic regression, only the need for presurgical ECMO remained significantly associated with presurgical PE ( < 0.01, AUC 0.65) and patch repair as the therapeutic intervention remained significantly associated with the occurrence of postsurgical PE ( < 0.0001, AUC 0.80). Patients with CTX had longer durations of both MV ( < 0.0001) and subsequent ventilatory assistance with spontaneous breathing ( = 0.0004), increased total lengths of hospitalization ( < 0.0001), increased durations of ECMO ( < 0.01) and increased incidences of CLD ( < 0.0001) compared to patients without CTX. No significant difference could be found for survival in both groups ( = 0.12). Our data suggest that the incidence of chylothorax is associated with large diaphragmatic defects, the need for postsurgical ECMO and the development of chronic lung disease, but not with survival.
PubMed: 38541987
DOI: 10.3390/jcm13061764 -
Diagnostics (Basel, Switzerland) Mar 2024This is a retrospective study investigating biometric measurements using magnetic resonance imaging (MRI) examinations in congenital diaphragmatic hernia (CDH). CDH is...
This is a retrospective study investigating biometric measurements using magnetic resonance imaging (MRI) examinations in congenital diaphragmatic hernia (CDH). CDH is one of the more common causes of pulmonary hypoplasia, with grave consequences for the fetus. Inclusion criteria were patients diagnosed with CDH as the only observed anomaly, who underwent MRI examination after the second-trimester morphology ultrasound. The patients came from three university hospitals in Bucharest, Romania. In total, 19 patients were included in the study after applying exclusion criteria. Comparing the observed values of the thoracic transverse diameter, the thoracic anterior-posterior diameter, the thoracic circumference, the thoracic area, and the thoracic volume with values from the literature, we observed a predictive alteration of these parameters, with most showing Gaussian distribution. We observed statistical significance for most of our correlations, except between the observed and expected thoracic anterior-posterior diameters and the observed and expected thoracic volume values. This is very helpful when complex studies that can calculate the pulmonary volume cannot be obtained, as in the case of movement artifacts, and allows the clinicians to better assess the severity of the disease. MRI follow-up in CDH cases is a necessity, as it offers the most accurate thoracic biometry.
PubMed: 38535061
DOI: 10.3390/diagnostics14060641 -
Cureus Mar 2024Congenital diaphragmatic hernias are primarily found in infants and have a high mortality rate due to neonatal respiratory distress. The most common type of congenital...
Congenital diaphragmatic hernias are primarily found in infants and have a high mortality rate due to neonatal respiratory distress. The most common type of congenital diaphragmatic hernia is Bochdalek hernia, which occurs in the posterolateral diaphragm, with the left side being the most commonly affected. However, congenital diaphragmatic hernias are extremely rare in adults and are often misdiagnosed due to their subtle symptoms. Therefore, we suggest that a contrast-enhanced CT scan should be used for early screening and diagnosis in all patients with sudden severe pain or recurrent ambiguous symptoms in the chest and abdomen. This case report presents a rare occurrence of Bochdalek hernia in an adult male. The patient experienced nonspecific abdominal symptoms after eating. The hernia resulted in the displacement of the left kidney, the transverse colon of the splenic flexure, and most of the stomach into the thoracic cavity. This displacement led to atelectasis of the left lung, which reached three-fifths of its capacity. The patient underwent successful treatment using a combination of laparoscopy and open surgery. Follow-up CT scans conducted two weeks, three months, and one year later revealed a stable condition with no complications.
PubMed: 38533320
DOI: 10.7759/cureus.56950 -
Cureus Feb 2024Morgagni's hernia (MH) occurs when the abdominal viscera herniates into the thoracic cavity through a congenital anatomical defect in the diaphragm, termed the foramen...
Morgagni's hernia (MH) occurs when the abdominal viscera herniates into the thoracic cavity through a congenital anatomical defect in the diaphragm, termed the foramen of Morgagni. Although it is more frequently detected in childhood, its delayed presentation in adults and the elderly could be easily overlooked due to the non-specificity of its symptoms. Here, we report the case of an elderly female who presented purely with dyspnea and desaturation, necessitating admission to the intensive care unit. Her computed tomography (CT) scan revealed the presence of MH with complete lobar collapse. Laparoscopy was successful in reducing the hernia, and the patient improved with a good prognosis. Surgical treatment for MH is advised for all cases in order to prevent the occurrence of serious complications.
PubMed: 38533138
DOI: 10.7759/cureus.54876 -
World Journal of Pediatric Surgery 2024Standardization of care seeks to improve patient outcomes and healthcare delivery by reducing unwanted variations in care as well as promoting the efficient and... (Review)
Review
Standardization of care seeks to improve patient outcomes and healthcare delivery by reducing unwanted variations in care as well as promoting the efficient and effective use of healthcare resources. There are many types of standardization, with clinical practice guidelines (CPGs), based on a stringent assessment of evidence and expert consensus, being the hallmark of high-quality care. This article outlines the history of CPGs, their benefits and shortcomings, with a specific focus on standardization efforts as it relates to congenital diaphragmatic hernia management.
PubMed: 38532942
DOI: 10.1136/wjps-2024-000783 -
Pediatric Surgery International Mar 2024Sandifer syndrome (SS), which combines gastroesophageal reflux (GER) and a neurological or psychiatric disorder, is an uncommon condition that often takes a long time to... (Meta-Analysis)
Meta-Analysis
PURPOSE
Sandifer syndrome (SS), which combines gastroesophageal reflux (GER) and a neurological or psychiatric disorder, is an uncommon condition that often takes a long time to diagnosis. We aimed to systematically review available papers regarding SS.
METHODS
After presenting our two cases of SS, we systematically reviewed articles published in MEDILINE/PubMed, Cochrane Library, and Web of Science.
RESULTS
The meta-analysis included 54 reported cases and 2 of our own cases. Our results showed that all cases achieved symptom improvement with appropriate treatment for GER. Notably, 19 of the 56 cases exhibited anatomical anomalies, such as hiatal hernia and malrotation. Significantly more patients with than without anatomical anomalies required surgery (p < 0.001). However, 23 of the 29 patients without anatomical anomalies (79%) achieved symptom improvement without surgery. Patients who did not undergo surgery had a median (interquartile range) duration to symptom resolution of 1 (1-1) month.
CONCLUSION
The primary care providers should keep SS in the differential diagnosis of patients presenting with abnormal posturing and no apparent neuromuscular disorders. Fundoplication may be effective especially for patients with anatomical anomalies or those whose symptoms do not improve after more than 1 month with nonsurgical treatment.
Topics: Child; Humans; Gastroesophageal Reflux; Torticollis; Fundoplication; Hernia, Hiatal; Treatment Outcome; Laparoscopy
PubMed: 38526644
DOI: 10.1007/s00383-024-05683-3 -
Medicine Mar 2024Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly with abnormal diaphragm development, typically diagnosed prenatally or soon after birth.... (Review)
Review
BACKGROUND
Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly with abnormal diaphragm development, typically diagnosed prenatally or soon after birth. Late-presenting CDH presents diagnostic challenges due to nonspecific symptoms that can lead to misdiagnoses.
METHODS
This report discusses a 35-month-old female initially presenting with predominant gastrointestinal symptoms and minimal respiratory distress. Initial radiographic findings suggested a left tension pneumothorax, prompting further investigation.
RESULTS
Subsequent diagnostic efforts revealed a Bochdalek-type left CDH, with several abdominal organs herniated into the thoracic cavity. The case was managed through laparotomy, where herniated contents were successfully repositioned into the abdominal cavity. This intervention underscores the need for high clinical suspicion and the importance of distinguishing between similar presentations, such as tension pneumothorax and tension gastrothorax, which require different management strategies.
CONCLUSION
The case illustrates the importance of considering CDH in differential diagnoses for older pediatric patients with atypical symptoms. Early recognition and appropriate management are key to improving patient outcomes.
Topics: Child, Preschool; Female; Humans; Diagnosis, Differential; Dyspnea; Hernias, Diaphragmatic, Congenital; Laparotomy; Pneumothorax
PubMed: 38518042
DOI: 10.1097/MD.0000000000037450 -
Journal of Veterinary Internal Medicine 2024Sliding hiatal herniation (SHH) and gastroesophageal reflux (GER) commonly occur in French bulldogs. Wireless pH monitoring can quantitatively assess acidic GER in dogs...
BACKGROUND
Sliding hiatal herniation (SHH) and gastroesophageal reflux (GER) commonly occur in French bulldogs. Wireless pH monitoring can quantitatively assess acidic GER in dogs affected by SHH.
HYPOTHESIS/OBJECTIVES
Measure acidic GER in French bulldogs with SHH, pre- and post-brachycephalic obstructive airway syndrome (BOAS) surgery, utilizing a wireless pH capsule (Bravo Calibration-free, Medtronic, Minnesota), and correlate with owners' observations of regurgitation.
ANIMALS
Eleven French bulldogs diagnosed with SHH via swallowing fluoroscopy.
METHODS
Prospective cohort study. A pH capsule was endoscopically placed in the esophagus. Up to 96 hours of data were acquired as the owner logged clinical signs. Spearman's correlation and Wilcoxon rank-sum tests evaluated factors correlated with acid exposure time (AET), defined by the % time pH < 4. In 4/11 dogs, Bravo monitoring was repeated 2-4 months after BOAS surgery.
RESULTS
Medians (Q1-Q3) for age and weight were 21 months (17-35.5) and 10.0 kg (8.9-11.5). BOAS severity was mild (3), moderate (4), or severe (4). Medians (Q1-Q3) for AET and reflux events were 3.3% (2.6-6.4) and 70 (34-173). Clinical score (P = .82) and BOAS severity (P = .60) were not correlated with AET, but age was negatively correlated (rho = -.66, P = .03). Median probability (Q1-Q3) that regurgitation was associated with a reflux event was 72.5% (0-99). Percent AET numerically improved in all 4 dogs that underwent BOAS surgery although not statistically assessed.
CONCLUSIONS AND CLINICAL IMPORTANCE
Wireless pH monitoring documented acidic GER in French bulldogs with SHH, captured subclinical events, and showed improvements after BOAS surgery.
Topics: Animals; Dogs; Dog Diseases; Gastroesophageal Reflux; Male; Prospective Studies; Hernia, Hiatal; Female; Esophageal pH Monitoring; Cohort Studies; Hydrogen-Ion Concentration
PubMed: 38514174
DOI: 10.1111/jvim.17048 -
Trials Mar 2024Infants born with congenital diaphragmatic hernia (CDH) are at high risk of respiratory insufficiency and pulmonary hypertension. Routine practice includes immediate... (Randomized Controlled Trial)
Randomized Controlled Trial
Multicentre, randomised controlled trial of physiological-based cord clamping versus immediate cord clamping in infants with a congenital diaphragmatic hernia (PinC): statistical analysis plan.
BACKGROUND
Infants born with congenital diaphragmatic hernia (CDH) are at high risk of respiratory insufficiency and pulmonary hypertension. Routine practice includes immediate clamping of the umbilical cord and endotracheal intubation. Experimental animal studies suggest that clamping the umbilical cord guided by physiological changes and after the lungs have been aerated, named physiological-based cord clamping (PBCC), could enhance the fetal-to-neonatal transition in CDH. We describe the statistical analysis plan for the clinical trial evaluating the effects of PBCC versus immediate cord clamping on pulmonary hypertension in infants with CDH (PinC trial).
DESIGN
The PinC trial is a multicentre, randomised controlled trial in infants with isolated left-sided CDH, born ≥ 35.0 weeks of gestation. The primary outcome is the incidence of pulmonary hypertension in the first 24 h after birth. Maternal outcomes include estimated maternal blood loss. Neonatal secondary outcomes include mortality before discharge, extracorporeal membrane oxygenation therapy, and number of days of mechanical ventilation. Infants are 1:1 randomised to either PBCC or immediate cord clamping using variable random permutated block sizes (4-8), stratified by treatment centre and estimated severity of pulmonary hypoplasia (i.e. mild/moderate/severe). At least 140 infants are needed to detect a relative reduction in pulmonary hypertension by one third, with 80% power and 0.05 significance level. A chi-square test will be used to evaluate the hypothesis that PBCC decreases the occurrence of pulmonary hypertension. This plan is written and submitted without knowledge of the collected data. The trial has been ethically approved.
TRIAL REGISTRATION
ClinicalTrials.gov NCT04373902 (registered April 2020).
Topics: Infant, Newborn; Pregnancy; Animals; Female; Humans; Hernias, Diaphragmatic, Congenital; Umbilical Cord Clamping; Hypertension, Pulmonary; Constriction; Respiration, Artificial; Umbilical Cord
PubMed: 38509614
DOI: 10.1186/s13063-024-08027-7 -
Cureus Feb 2024Background Congenital diaphragmatic hernia (CDH) is a condition where abdominal contents protrude into the chest due to defects in the diaphragm muscle. It is considered...
Background Congenital diaphragmatic hernia (CDH) is a condition where abdominal contents protrude into the chest due to defects in the diaphragm muscle. It is considered an emergency that needs urgent intervention to prevent further complications or death. Our study aimed to estimate survival and evaluate predictors of mortality in newborns with CDH using available prediction tools in the literature. Methods This retrospective cohort study included neonates with CDH in King Abdulaziz Medical City (KAMC), Jeddah, from 2000 to 2021. Prevalence, demographics, and clinical characteristics were compared between surviving and deceased infants. C-statistics were used to measure the area under the curve for the prenatal and postnatal predictor tools, and a p-value of <0.05 was considered significant. Results Between 2000 and 2021, 45 neonates with CDH were included (six per 10,000 inborn live births). The mortality rate was 51.1%. The differences in demographics were not significant among surviving and deceased patients. One prenatal predictor tool, the lung-to-head ratio, was found to be significant; in addition, three postnatal predictor tools of mortality, SNAP-II, CDHSG-probability survival, and Brindle Score, had the highest concordance (C) statistics of 0.8, 0.79, and 0.8, respectively. Conclusion Although the incidence of CDH was found to be higher in our study compared to global statistics, our mortality rates correspond with international figures. The most significant differences between predictors and prediction models of mortality were lung-to-head ratio prenatally, SNAP-II, CDHSG-probability survival, and Brindle Score postnatally. Further multicentered studies are recommended with a larger sample size.
PubMed: 38500943
DOI: 10.7759/cureus.54364