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Journal of Pediatric Surgery May 2024Our study examines if the disease severity profile of our Congenital Diaphragmatic Hernia (CDH) patient cohort adherent to long-term follow-up differs from patients lost...
OBJECTIVES
Our study examines if the disease severity profile of our Congenital Diaphragmatic Hernia (CDH) patient cohort adherent to long-term follow-up differs from patients lost to follow-up after discharge and examines factors associated with health care utilization.
METHODS
Retrospective review identified CDH survivors born 2005-2019 with index repair at our institution. Primary outcome was long-term follow-up status: "active" or "inactive" according to clinic guidelines. Markers of CDH disease severity including CDH defect classification, oxygen use, tube feeds at discharge, and sociodemographic factors were examined as exposures.
RESULTS
Of the 222 included patients, median age [IQR] was 10.2 years [6.7-14.3], 61% male, and 57 (26%) were insured by Medicaid. Sixty-three percent (139/222) of patients were adherent to follow-up. Seventy-six percent of patients discharged on tube feeds had active follow-up compared to 55% of patients who were not, with similar findings for oxygen at discharge (76% vs. 55%). Kaplan-Meier analysis showed patients with smaller defect size had earlier attrition compared to patients with larger defect size. Other race (Hispanic, Asian, Middle Eastern) patients had 2.87 higher odds of attrition compared to white patients (95% CI 1.18-7.0). Medicaid patients had 2.64 higher odds of attrition compared to private insurance (95% CI 1.23-5.66).
CONCLUSION
Loss to follow-up was associated with race and insurance type. Disease severity was similar between the active and inactive clinic cohorts. Long-term CDH clinic publications should examine attrition to ensure reported outcomes reflect the discharged population. This study identified important factors to inform targeted interventions for follow-up adherence.
LEVEL OF EVIDENCE
Level III.
PubMed: 38806317
DOI: 10.1016/j.jpedsurg.2024.04.023 -
Ultrasound in Obstetrics & Gynecology :... May 2024
PubMed: 38804564
DOI: 10.1002/uog.27711 -
Prenatal Diagnosis May 2024We aim to provide a template structured report of fetal Magnetic Resonance Imaging in congenital diaphragmatic hernia (CDH) that was locally validated by the CDH study...
OBJECTIVE
We aim to provide a template structured report of fetal Magnetic Resonance Imaging in congenital diaphragmatic hernia (CDH) that was locally validated by the CDH study group in Mannheim.
METHODS
A selection of 50 fetal MRIs of patients with an isolated diaphragmatic hernia and associated radiology reports from five different senior radiologists from a single center resulted in a primary structured report, which was put into practice by using dedicated software. A questionnaire survey of the interdisciplinary CDH study group Mannheim was used to adapt the report to the clinical requirements.
RESULTS
There was a huge variability in how deep the free text reports go into detail. The side of the hernia was named in 94% of cases. In 58%, both the lung volume and the total lung volume were reported. A comparison with the expected lung volume was reported in 66% of cases. Additional findings, such as herniated organs, were reported in 96% of cases. Overall satisfaction with the newly established structured report was high within the CDH study group with a mean of 4.7.
CONCLUSIONS
The use of the structured report of this study can optimize the interdisciplinary dialog, the standardization of report content, increase report completeness and improve quality.
PubMed: 38797992
DOI: 10.1002/pd.6593 -
Journal of Perinatology : Official... May 2024Fetal Centers use imaging studies to predict congenital diaphragmatic hernia (CDH) prognosis and the need for fetal therapy. Given improving CDH survival, we...
BACKGROUND
Fetal Centers use imaging studies to predict congenital diaphragmatic hernia (CDH) prognosis and the need for fetal therapy. Given improving CDH survival, we hypothesized that current fetal imaging severity predictions no longer reflect true outcomes and fail to justify the risks of fetal therapy.
METHODS
We analyzed our single-center contemporary data in a left-sided CDH cohort (n = 58) by prognostic criteria determined by MRI observed-to-expected total fetal lung volumes: severe <25%, moderate 25-35%, and mild >35%. We compared contemporary survival to prior studies and the TOTAL trials.
RESULTS
Contemporary survival was significantly higher than past studies for all prognostic classifications (mild 100% vs 80-94%, moderate 95% vs 59-75%, severe 79% vs 13-25%; P < 0.01), and to either control or fetal therapy arms of the TOTAL trials.
CONCLUSIONS
Current fetal imaging criteria are overly pessimistic and may lead to unwarranted fetal intervention. Fetal therapies remain experimental. Future studies will require updated prognostic criteria.
PubMed: 38796522
DOI: 10.1038/s41372-024-02008-z -
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 -
Postgraduate Medicine May 2024Hiatal hernia (HH) is a common disease in the general population. It is often asymptomatic, but if it does present clinical manifestations, these are usually... (Review)
Review
Hiatal hernia (HH) is a common disease in the general population. It is often asymptomatic, but if it does present clinical manifestations, these are usually gastrointestinal. Gastroesophageal reflux is the main symptom that accompanies it. Depending on the severity of the hernia, it is classified into several subtypes from I-IV. Especially, IV type (giant HH) can lead to various cardiopulmonary symptoms with several degrees of severity. It is necessary to keep this possibility in mind among the various differential diagnoses that may occur in this clinical setting. The current paper aims to review the literature on classic and novel information on the HH - cardiovascular system relationship. Epidemiological data, physiological aspects of the heart compressed by HH, cardiovascular symptoms, electrocardiographic changes, echocardiographic alterations and clinical implications are discussed.
Topics: Hernia, Hiatal; Humans; Gastroesophageal Reflux; Electrocardiography; Echocardiography; Cardiovascular Diseases; Severity of Illness Index
PubMed: 38795063
DOI: 10.1080/00325481.2024.2360886 -
Life (Basel, Switzerland) May 2024Fryns syndrome (FS) is a multiple congenital anomaly syndrome with different multisystemic malformations. These include congenital diaphragmatic hernia, pulmonary...
Fryns syndrome (FS) is a multiple congenital anomaly syndrome with different multisystemic malformations. These include congenital diaphragmatic hernia, pulmonary hypoplasia, and craniofacial dysmorphic features in combination with malformations of the central nervous system such as agenesis of the corpus callosum, cerebellar hypoplasia, and enlarged ventricles. We present a non-consanguineous northern European family with two recurrent cases of FS: a boy with multiple congenital malformations who died at the age of 2.5 months and a female fetus with a complex developmental disorder with similar features in a following pregnancy. Quad whole exome analysis revealed two likely splicing-affecting disease-causing mutations in the gene: a synonymous mutation c.2619G>A, p.(Leu873=) in the last nucleotide of exon 29 and a 30 bp-deletion c.996_1023+2del (NM_176787.5) protruding into intron 12, with both mutations in configuration in the affected patients. Exon skipping resulting from these two variants was confirmed via RNA sequencing. Our molecular and clinical findings identified compound heterozygosity for two novel splice-affecting variants as the underlying pathomechanism for the development of FS in two patients.
PubMed: 38792648
DOI: 10.3390/life14050628 -
Cureus Apr 2024Morgagni hernia is a rare condition characterized by a congenital retrosternal defect of the diaphragm, leading to the protrusion of abdominal organs into the thoracic...
Morgagni hernia is a rare condition characterized by a congenital retrosternal defect of the diaphragm, leading to the protrusion of abdominal organs into the thoracic cavity. Here, we report the case of a 72-year-old woman with a Morgagni hernia incidentally discovered during evaluation for persistent dyspnea following COVID-19 pneumonia. The diagnosis was made by imaging, including a chest X-ray and a thoracic CT scan, which showed an ascent of the transverse colon and omentum through an anterior retrosternal defect. Surgical exploration via right posterolateral thoracotomy revealed an anterior diaphragmatic hernia with a small defect containing the greater omentum and transverse colon, which was repaired by resecting the hernia sac and closing the diaphragmatic defect by fixing the anterior rim of the diaphragm to the retrosternal fascia with interrupted silk sutures. Postoperative recovery was uneventful, and follow-up examinations revealed no abnormalities on chest X-rays obtained at one, three, and six months. This case highlights the incidental discovery and successful surgical management of a Morgagni hernia in an elderly patient through a thoracic approach.
PubMed: 38784344
DOI: 10.7759/cureus.58799 -
Archives of Gynecology and Obstetrics May 2024To evaluate the impact of the timing of MRI on the prediction of survival and morbidity in patients with CDH, and whether serial measurements have a beneficial value.
PURPOSE
To evaluate the impact of the timing of MRI on the prediction of survival and morbidity in patients with CDH, and whether serial measurements have a beneficial value.
METHODS
This retrospective cohort study was conducted in two perinatal centers, in Germany and Italy. It included 354 patients with isolated CDH having at least one fetal MRI. The severity was assessed with the observed-to-expected total fetal lung volume (o/e TFLV) measured by two experienced double-blinded operators. The cohort was divided into three groups according to the gestational age (GA) at which the MRI was performed (< 27, 27-32, and > 32 weeks' gestation [WG]). The accuracy for the prediction of survival at discharge and morbidity was analyzed with receiver operating characteristic (ROC) curves. Multiple logistic regression analyses and propensity score matching examined the population for balance. The effect of repeated MRI was evaluated in ninety-seven cases.
RESULTS
There were no significant differences in the prediction of survival when the o/e TFLV was measured before 27, between 27 and 32, and after 32 WG (area under the curve [AUC]: 0.77, 0.79, and 0.77, respectively). After adjustment for confounding factors, it was seen, that GA at MRI was not associated with survival at discharge, but the risk of mortality was higher with an intrathoracic liver position (adjusted odds ratio [aOR]: 0.30, 95% confidence interval [95%CI] 0.12-0.78), lower GA at birth (aOR 1.48, 95%CI 1.24-1.78) and lower o/e TFLV (aOR 1.13, 95%CI 1.06-1.20). ROC curves showed comparable prediction accuracy for the different timepoints in pregnancy for pulmonary hypertension, the need of extracorporeal membrane oxygenation, and feeding aids. Serial measurements revealed no difference in change rate of the o/e TFLV according to survival.
CONCLUSION
The timing of MRI does not affect the prediction of survival rate or morbidity as the o/e TFLV does not change during pregnancy. Clinicians could choose any gestational age starting mid second trimester for the assessment of severity and counseling.
PubMed: 38782762
DOI: 10.1007/s00404-024-07545-8