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Seminars in Fetal & Neonatal Medicine Oct 2023The ex-utero intrapartum treatment (EXIT) procedure was originally developed to reverse tracheal occlusion in fetuses with severe congenital diaphragmatic hernia that... (Review)
Review
The ex-utero intrapartum treatment (EXIT) procedure was originally developed to reverse tracheal occlusion in fetuses with severe congenital diaphragmatic hernia that underwent fetal tracheal occlusion. The EXIT procedure has since been applied to a wide range of indications, but the primary indication remains securing a patent airway and providing respiratory support in fetuses with anticipated difficult airways. The authors review perinatal management of the anticipated difficult airway and their single-institution's experience with the EXIT procedure.
Topics: Pregnancy; Female; Humans; Cesarean Section; Tracheostomy; Hernias, Diaphragmatic, Congenital; Fetus; Airway Obstruction
PubMed: 38030438
DOI: 10.1016/j.siny.2023.101485 -
The Journal of Pediatrics Sep 2023
Topics: Humans; Hernias, Diaphragmatic, Congenital; Hypertension, Pulmonary; Echocardiography; Ventricular Function
PubMed: 37315779
DOI: 10.1016/j.jpeds.2023.113550 -
The Journal of Pediatrics Aug 2023To compare disease severity and mortality differences between female and male patients with congenital diaphragmatic hernia (CDH).
OBJECTIVE
To compare disease severity and mortality differences between female and male patients with congenital diaphragmatic hernia (CDH).
STUDY DESIGN
We queried the CDH Study Group (CDHSG) database for CDH neonates managed between 2007 and 2018. Female and males were compared in statistical analyses using t tests, χ² tests, and Cox regression, as appropriate (P ≤ .05).
RESULTS
There were 7288 CDH patients, of which 3048 (41.8%) were female. Females weighed less on average at birth than males (2.84 kg vs 2.97 kg, P < .001) despite comparable gestational age. Females had similar rates of extracorporeal life support (ECLS) utilization (27.8% vs 27.3%, P = .65). Although both cohorts had equivalent defect size and rates of patch repair, female patients had increased rates of intrathoracic liver herniation (49.2% vs 45.9%, P = .01) and pulmonary hypertension (PH) (86.6% vs 81.1%, P < .001). Females had lower survival rates at 30-days (77.3% vs 80.1%, P = .003) and overall lower survival to discharge (70.2% vs 74.2%, P < .001). Subgroup analysis revealed that increased mortality was significant among those who underwent repair but were never supported on ECLS (P = .005). On Cox regression analysis, female sex was independently associated with mortality (adjusted hazard ratio 1.32, P = .02).
CONCLUSION
After controlling for the established prenatal and postnatal predictors of mortality, female sex remains independently associated with a higher risk of mortality in CDH. Further study into the underlying causes for sex-specific disparities in CDH outcomes is warranted.
Topics: Infant, Newborn; Pregnancy; Humans; Male; Female; Hernias, Diaphragmatic, Congenital; Treatment Outcome; Proportional Hazards Models; Survival Rate; Hypertension, Pulmonary; Retrospective Studies
PubMed: 37196780
DOI: 10.1016/j.jpeds.2023.113481 -
American Journal of Respiratory Cell... Nov 2023
Topics: Humans; Hernias, Diaphragmatic, Congenital; NF-kappa B; Organogenesis; Signal Transduction; Lung
PubMed: 37566564
DOI: 10.1165/rcmb.2023-0258ED -
Acute Medicine & Surgery 2023Traumatic intrapericardial diaphragmatic hernias are very rare; they occur when the central tendon of the diaphragm ruptures, allowing abdominal viscera to herniate into...
Traumatic intrapericardial diaphragmatic hernias are very rare; they occur when the central tendon of the diaphragm ruptures, allowing abdominal viscera to herniate into the pericardial sac, causing life-threatening cardiac tamponade. Computed tomography imaging with multiplanar reconstruction is useful in evaluating such cases.
PubMed: 37814652
DOI: 10.1002/ams2.895 -
Clinics in Perinatology Mar 2024Long-term outcomes of persistent pulmonary hypertension of newborn (PPHN) depend on disease severity, duration of ventilation, and associated anomalies. Congenital... (Review)
Review
Long-term outcomes of persistent pulmonary hypertension of newborn (PPHN) depend on disease severity, duration of ventilation, and associated anomalies. Congenital diaphragmatic hernia survivors may have respiratory morbidities and developmental delay. The presence of PPHN is associated with increased mortality in hypoxic-ischemic encephalopathy, though the effects on neurodevelopment are less clear. Preterm infants can develop pulmonary hypertension (PH) early in the postnatal course or later in the setting of bronchopulmonary dysplasia (BPD). BPD-PH is associated with higher mortality, particularly within the first year. Evidence suggests that both early and late PH in preterm infants are associated with neurodevelopmental impairment.
Topics: Infant; Infant, Newborn; Humans; Nitric Oxide; Infant, Premature; Hypertension, Pulmonary; Bronchopulmonary Dysplasia; Hernias, Diaphragmatic, Congenital
PubMed: 38325946
DOI: 10.1016/j.clp.2023.10.002 -
Thoracic Surgery Clinics May 2024A variety of diaphragmatic and non-diaphragmatic pathologies may require resection, reconstruction, or repair of the diaphragm. Adequate reconstruction is crucial in...
A variety of diaphragmatic and non-diaphragmatic pathologies may require resection, reconstruction, or repair of the diaphragm. Adequate reconstruction is crucial in cases of diaphragmatic resection to prevent the herniation of abdominal organs into the chest and to maintain optimal respiratory function. This article aims to provide a detailed overview of the techniques used for surgical diaphragm reconstruction, taking into account factors such as the size and location of the defect, available options for reconstructive materials, potential challenges and pitfalls, and considerations related to the recurrence or failure of the repair.
Topics: Humans; Diaphragm; Hernia, Diaphragmatic; Plastic Surgery Procedures; Surgical Flaps
PubMed: 38705660
DOI: 10.1016/j.thorsurg.2024.01.001 -
Hernia : the Journal of Hernias and... May 2024To determine the relationship between abdominal hernia and obesity. Although obesity is frequently cited as a risk factor for abdominal hernia, few studies have...
PURPOSE
To determine the relationship between abdominal hernia and obesity. Although obesity is frequently cited as a risk factor for abdominal hernia, few studies have confirmed this association (Menzo et al. Surg Obes Relat Dis 14:1221-1232. 10.1016/j.soard.2018.07.005, 2018).
METHODS
A cross-sectional study of primary care ambulatory patients aged older than 16 years treated at UCLA Health from 01/01/2018 to 06/06/2023. Abdominal hernia was identified by clinic encounter ICD-10 codes (K40-K46).
RESULTS
There were 41,703 hernias identified among 1,362,440 patients (306.1 per10,000) with a mean age of 62.5 ± 16.1 years, and 57.6% were men. Nearly half (44.7%) of all abdominal hernias were diaphragmatic. There was an approximately equal distribution of the ventral (28.7%) and inguinal (24.3%) hernia. Each hernia type had a different relationship with obesity: The odds of having a ventral hernia increased with BMI in both sexes: BMI 25-29.9 kg/m odds ratio (OR) = 1.65, (CI 1.56-1.74); BMI 30-39.9 kg/m OR = 2.42 (CI 2.29-2.56), BMI 40-49.9 kg/m OR = 2.28 (CI 2.05-2.54) and BMI > = 50 kg/m OR = 2.54 (CI 2.03-3.17) all relative to normal BMI. In contrast, the odds of having an inguinal hernia decreased with obesity relative to normal weight [obesity (BMI 30-39.9 kg/m): OR = 0.60 (CI 0.56-0.65)], morbid obesity (BMI 40-49.9 kg/m): OR = 0.29 (CI 0.23-0.37). The OR for diaphragmatic hernia peaks with obesity in women and overweight status in men but was found to decrease with morbid obesity [OR = 1.18 (CI 1.07-1.30)]. There was no significant difference between men and women in the prevalence of femoral hernia (men: 0.7/per10,000, women: 0.9/per10,000, p = 0.19).
CONCLUSIONS
The relationship between hernia and obesity is complex with some hernias decreasing in prevalence as obesity increases. Further research is needed to better understand this paradoxical relationship.
PubMed: 38795218
DOI: 10.1007/s10029-024-03034-8 -
The Journal of Pediatrics Sep 2023To describe the types of congenital heart disease (CHD) in a congenital diaphragmatic hernia (CDH) cohort in a large volume center and evaluate surgical decision making...
OBJECTIVE
To describe the types of congenital heart disease (CHD) in a congenital diaphragmatic hernia (CDH) cohort in a large volume center and evaluate surgical decision making and outcomes based on complexity of CHD and associated conditions.
STUDY DESIGN
A retrospective review of patients with CHD and CDH diagnosed by echocardiogram between 01/01/2005 and 07/31/2021. The cohort was divided into 2 groups based on survival at discharge.
RESULTS
Clinically important CHD was diagnosed in 19% (62/326) of CDH patients. There was 90% (18/20) survival in children undergoing surgery for both CHD and CDH as neonates, and 87.5 (22/24) in those undergoing repair initially for CDH alone. A genetic anomaly identified on clinical testing was noted in 16% with no significant association with survival. A higher frequency of other organ system anomalies was noted in nonsurvivors compared with survivors. Nonsurvivors were more likely to have unrepaired CDH (69% vs 0%, P < .001) and unrepaired CHD (88% vs 54%, P < .05), reflecting a decision not to offer surgery.
CONCLUSIONS
Survival was excellent in patients who underwent repair of both CHD and CDH. Patients with univentricular physiology have poor survival and this finding should be incorporated into pre and postnatal counseling about eligibility for surgery. In contrast, patients with other complex lesions including transposition of the great arteries have excellent outcomes and survival at 5 years follow-up at a large pediatric and cardiothoracic surgical center.
Topics: Infant, Newborn; Humans; Child; Hernias, Diaphragmatic, Congenital; Transposition of Great Vessels; Survival Rate; Heart Defects, Congenital; Retrospective Studies; Decision Making
PubMed: 37268035
DOI: 10.1016/j.jpeds.2023.113530