Right-sided Congenital Diaphragmatic Hernia Research Articles

Thoracoscopic repair of right-sided congenital diaphragmatic hernia in the pediatric age: systematic review

Thoracoscopic repair of right-sided congenital diaphragmatic hernia in the pediatric age: systematic review

Prenatal MRI assessment of mediastinal shift angle as a feasible and effective risk stratification tool in isolated right-sided congenital diaphragmatic hernia.

To develop a mediastinal shift angle (MSA) measurement method applicable to right-sided congenital diaphragmatic hernia (RCDH) in fetal MRI and to validate the predictive value of MSA in RCDH. Twenty-seven fetuses with isolated RCDH and 53 controls were included in our study. MSA was measured on MRI axial image at the level of four-chamber view of the fetal heart. The angle between the sagittal midline landmark line and the left boundary landmark line touching tangentially the lateral wall of the left ventricle was used to quantify MSA for RCDH. Appropriate statistical analyses were performed to determine whether MSA can be regarded as a valid predictive tool for postnatal outcomes. Furthermore, predictive performance of MSA was compared with that of lung area to head circumference ratio (LHR), observed/expected LHR (O/E LHR), total fetal lung volume (TFLV), and observed/expected TFLV (O/E TFLV). MSA was significantly higher in the RCDH group than in the control group. MSA, LHR, O/E LHR, TFLV, and O/E TFLV were all correlated with postnatal survival, pulmonary hypertension (PH), and extracorporeal membrane oxygenation (ECMO) therapy (p < 0.05). Value of the AUC demonstrated good predictive performance of MSA for postnatal survival (0.901, 95%CI: (0.781-1.000)), PH (0.828, 95%CI: (0.661-0.994)), and ECMO therapy (0.813, 95%CI: (0.645-0.980)), which was similar to O/E TFLV but slightly better than TFLV, O/E LHR, and LHR. We developed a measurement method of MSA for RCDH for the first time and demonstrated that MSA could be used to predict postnatal survival, PH, and ECMO therapy in RCDH. Newly developed MRI assessment method of fetal MSA in RCDH offers a simple and effective risk stratification tool for patients with RCDH. • We developed a measurement method of mediastinal shift angle for right-sided congenital diaphragmatic hernia for the first time and demonstrated its feasibility and reproducibility. • Mediastinal shift angle can predict more prognostic information other than survival in right-sided congenital diaphragmatic hernia with good performance. • Mediastinal shift angle can be used as a simple and effective risk stratification tool in right-sided congenital diaphragmatic hernia to improve planning of postnatal management.

Surgical Risk Factors for Delayed Oral Feeding Autonomy in Patients with Left-Sided Congenital Diaphragmatic Hernia.

Congenital diaphragmatic hernia (CDH) is a rare disease associated with major nutritional and digestive morbidities. Oral feeding autonomy remains a major issue for the care and management of these patients. The aim of this study was to specify the perinatal risk factors of delayed oral feeding autonomy in patients treated for CDH. This monocentric cohort study included 138 patients with CDH. Eighty-four patients were analyzed after the exclusion of 54 patients (11 with delayed postnatal diagnosis, 5 with chromosomal anomaly, 9 with genetic syndrom, 13 with right-sided CDH, and 16 who died before discharge and before oral feeding autonomy was acquired). They were divided into two groups: oral feeding autonomy at initial hospital discharge (group 1, n = 51) and nutritional support at discharge (group 2, n = 33). Antenatal, postnatal, and perisurgical data were analyzed from birth until first hospital discharge. To remove biased or redundant factors related to CDH severity, statistical analysis was adjusted according to the need for a patch repair. After analysis and adjustment, delayed oral feeding autonomy was not related to observed/expected lung-to-head ratio (LHR o/e), intrathoracic liver and/or stomach position, or operative duration. After adjustment, prophylactic gastrostomy (OR adjusted: 16.3, IC 95%: 3.6-74.4) and surgical reoperation (OR adjusted: 5.1, IC 95% 1.1-23.7) remained significantly associated with delayed oral feeding autonomy. Delayed oral feeding autonomy occurred in more than one third of patients with CDH. Both prophylactic gastrostomy and surgical reoperation represent significant risk factors. Bowel obstruction might also impact oral feeding autonomy. Prophylactic gastrostomy seems to be a false "good idea" to prevent failure to thrive. This procedure should be indicated case per case. Bowel obstruction and all surgical reoperations represent decisive events that could impact oral feeding autonomy.

Congenital diaphragmatic (Bochdalek) hernia of the fetus

Congenital diaphragmatic hernia (CDH) is a developmental malformation characterized by a defect in the diaphragm that allows abdominal viscera to move into the chest cavity. A routine ultrasound screening performed during pregnancy detects over half of all cases. This paper describes two women who were prenatally diagnosed to have a fetus with isolated posterolateral CDH. Both mothers underwent medical abortion. In the first case, a postmortem examination of the fetus revealed a large right-sided CDH, in which a part of the liver and small intestine were propagated into the chest. In the second one, a huge left-sided CDH was identified, in which the entire stomach, a large proportion of the small intestine and part of the left lobe of the liver were pushed up into the thoracic cavity. CDH is a diagnostically challenging birth defect with a marked variation in severity and corresponding survival. In utero-diagnosed cases usually represent more severe and prognostically unfavorable conditions than those that were postnatally diagnosed. Early prenatal detection of CDH may help improve the clinical outcome as the mother can be referred to a specialist center before the onset of labor for optimal perinatal management. A legal medical abortion should be the last option after all the others have been exhausted.

Brain growth in fetuses with congenital diaphragmatic hernia.

To perform a volumetric evaluation of the brain in fetuses with right or left congenital diaphragmatic hernia (CDH), and to compare brain growth trajectories to normal fetuses. We identified fetal MRIs performed between 2015 and 2020 in fetuses with a diagnosis of CDH. Gestational age (GA) range was 19-40 weeks. Control subjects consisted of normally developing fetuses between 19 and 40weeks recruited for a separate prospective study. All images were acquired at 3 Tesla and were processed with retrospective motion correction and slice-to-volume reconstruction to generate super-resolution 3-dimensional volumes. These volumes were registered to a common atlas space and segmented in 29 anatomic parcellations. A total of 174 fetal MRIs in 149 fetuses were analyzed (99 controls [mean GA: 29.2±5.2weeks], 34 fetuses left-sided CDH [mean GA: 28.4±5.3weeks], and 16 fetuses right-sided CDH [mean GA: 27±5.4weeks]). In fetuses with left-sided CDH, brain parenchymal volume was -8.0% (95% confidence interval [CI] [-13.1, -2.5]; p=.005) lower than normal controls. Differences ranged from -11.4% (95% CI [-18, -4.3]; p<.001) in the corpus callosum to -4.6% (95% CI [-8.9, -0.1]; p=.044) in the hippocampus. In fetuses with right-sided CDH, brain parenchymal volume was -10.1% (95% CI [-16.8, -2.7]; p=.008) lower than controls. Differences ranged from -14.1% (95% CI [-21, -6.5]; p<.001) in the ventricular zone to -5.6% (95% CI [-9.3, -1.8]; p=.025) in the brainstem. Left and right CDH are associated with lower fetal brain volumes.

The results of treatment of newborns with right-sided and left-sided intrathoracic localization of the liver with diaphragmatic hernia

BACKGROUND: The right-sided congenital diaphragmatic hernia is the rare pathology. Results of diagnostics, prognosis and treatment usally published as a collection of cases.&#x0D; AIM: Comparition of treatment results of congenital diaphragmatic hernia with the liver as its content in dependancy of the side.&#x0D; MATERIALS AND METHODS: We present a retrospective analysis of 50 newborn patients with congenital diaphragmatic hernia. Patiens were divided in two groups, first with right-sided (19 patients), and second with left-sided hernia (31 patients). Groups were compared by gender and weigth. Comparition criteria was the results of prenatal and postnatal diagnostics, intraoperative data, postoperative period, complications and outcomes.&#x0D; RESULTS: We found predominance of prenatal diagnosis in the second group (48% vs 84%, p = 0,001). The lung-to-head circumference ratio were the same in both groups (0,52 in first, 0,46 in second, p = 0,058). Chance to use thoracoscopic approach in the second group was higer in 5,7 times (48% vs 84%). Postoperative period was easier in the group of right-sided congenital diaphragmatic hernia: artificial ventilation lasted on average 8 days (min 3; max 28) versus 11 (min 4; max 50) days in the first group (p = 0,036).&#x0D; Hospital stay was significantly lower in the second group 18 days (min 12; max 28), versus 50 days in the first group (min 13; max 64), p = 0,011. Recovery chance in patients with right-sided hernia was higher (45% vs 79%, confidence interval 0,0590,814).&#x0D; CONCLUSIONS: Every type of diaphragmatic hernia, including right-sided location, need to be the subgect of research of high compitantive center. Prognostic criteria for right-sided congenital diaphragmatic hernia require furter advanced study, wich is possible only in case of concentration of patients in the same center.

The myocardial capillary network is altered in congenital diaphragmatic hernia in the fetal rabbit model

Congenital diaphragmatic hernia (CDH) is associated with thoracic compression of the lungs and heart caused by the herniated abdominal content, leading to cardiac modifications including pressure and vascular changes. Our aim was to investigate the experimental immunoexpression of the capillary proliferation, activation, and density of Ki-67, VEGFR2, and lectin in the myocardium after surgical creation of a diaphragmatic defect. Pregnant New Zealand rabbits were operated on the 25th gestational day in order to create left-sided CDH (LCDH, n=9), right-sided CDH (RCDH, n=9), and Control (n=9), for a total of 27 fetuses in 19 pregnant rabbits. Five days after the procedure, animals were sacrificed, and histology and immunohistochemistry studies of the harvested hearts were performed. Total body weight and heart weight were not significantly different among groups (P=0.702 and 0.165, respectively). VEGFR2 expression was increased in both ventricles in the RCDH group (P<0.0001), and Ki-67 immunoexpression was increased in the left ventricle in the LCDH group compared to Control and RCDH groups (P<0.0001). In contrast, capillary density was reduced in the left ventricle in the LCDH compared to the Control and RCDH groups (P=0.002). Left and right ventricles responded differently to CDH in this model depending on the laterality of the diaphragmatic defect. This surgical model of diaphragmatic hernia was associated with different expression patterns of capillary proliferation, activation, and density in the myocardium of the ventricles of newborn rabbits.

Prenatal diagnosis of right-sided congenital diaphragmatic hernia

Prenatal diagnosis of right-sided congenital diaphragmatic hernia

Associated intraabdominal malformations of right-sided congenital diaphragmatic hernia: A rare anomaly and review

There is scarce literature on associated anomalies of right-sided congenital diaphragmatic hernias (CDH). The purpose of this study was to expand the presentation of a unique clinical experience by a literature review. Only six articles of a right-sided CDH linked to non-cardiac anomalies with complicated diagnostic and treatment scenarios were found related to ours. To the best of our knowledge, it is the first presented case of a right-sided CDH with multiple intestinal atresias and an intrathoracic right kidney. This case alerts physicians to take all examination details into account to avoid delaying CDH diagnosis. Most importantly, it gives valuable insight into possible associated anomalies of right-sided congenital diaphragmatic hernias and may benefit future embryological or genetic studies.

The interplay between prenatal liver growth and lung development in congenital diaphragmatic hernia.

ObjectiveLiver herniation is a known risk factor for increased severity in CDH and is associated with clinically significant pulmonary hypoplasia and pulmonary hypertension. Better studies are needed to understand the growth of the herniated liver compared to the liver that remains in the abdomen and how this liver growth then affects lung development. Serial hi-resolution fetal MRI enables characterization of liver growth throughout gestation and examination of macroscopic features that may regulate liver growth. Here, we hypothesized that the nature of liver herniation affects liver growth and, in turn, affects lung growth.MethodsClinical data were retrospectively collected from consecutive cases of prenatally diagnosed isolated left-sided or right-sided CDH from June 2006 to August 2021. Only those cases with MRI lung volumetry for both mid-gestation and late-gestation time points were recruited for analysis. Cases with fetal chromosomal abnormalities and other major structural abnormalities were excluded. Fractional liver volume and liver growth was indexed to estimated fetal weight and compared to lung growth.ResultsData was collected from 28 fetuses with a left liver-down CDH (LLD), 37 left liver-up CDH (LLU) and 9 right liver-up CDH (RLU). Overall, RLU fetuses had greater overall and fractional (intra-thoracic vs. intra-abdominal) liver growth when compared to LLD and LLU fetuses. Additionally, intra-thoracic liver growth was consistently slower than intra-abdominal liver growth for either right- or left-sided CDH. When the liver was not herniated, a positive correlation was seen between liver growth and lung growth. However, when the liver was herniated above the diaphragm, this positive correlation was lost.ConclusionRight-sided CDH fetuses exhibit greater liver growth compared to left-sided CDH. Liver herniation disrupts the normal positive correlation between liver and lung growth that is seen when the liver is entirely within the abdomen.

Image-based prenatal predictors of postnatal survival, extracorporeal life support, and defect size in right congenital diaphragmatic hernia.

To determine the association between prenatal ultrasound (US) and magnetic resonance imaging (MRI) characteristics in right congenital diaphragmatic hernia (RCDH) with postnatal outcome. CDH Study Group data were reviewed for all RCDH infants (n = 156) born between 2015 and 2019. Prenatal US and MRI lung size measurements were correlated with survival, extracorporeal life support (ECLS), and defect size. Overall survival was 64.1%. ECLS was required in 40.4%. US and MRI-based prenatal assessment of pulmonary hypoplasia does not predict survival. Prenatal measurement of lung size using either US or MRI correlates with ECLS use. Only MRI-based measures of lung size are associated with defect size. Image-based prenatal predictors of survival, ECLS, and defect size are of limited value in RCDH. Extrapolation of prenatal survival and morbidity indicators from left to right-sided CDH is not appropriate. There is an urgent need to develop RCDH prenatal prediction models.

Congenital diaphragmatic hernia in an 81-year-old female presenting as painless jaundice: A case report

Introduction and importanceAdult congenital diaphragmatic hernia (CDH) is rare and may present as an incidental finding in an asymptomatic individual.Case presentationWe report the case of an 81-year-old female who presented to the ED with painless jaundice and bilirubin of 19.9 mg/dL. She was diagnosed with biliary obstruction secondary to a right-sided CDH. The patient underwent surgery, which resulted in the correction of her CDH and a downward trend in her bilirubin.Clinical discussionCDH usually presents in the newborn period with respiratory distress and can have a high mortality rate. Although CDH is most often associated with newborns, it can rarely present in adulthood, manifesting insidiously with a variety of signs and symptoms ranging from a mild cough to biliary occlusion.ConclusionCDH may be considered in the differential diagnosis when other investigations for respiratory or gastrointestinal complaints have been unrevealing.

The Survivorship Bias in Congenital Diaphragmatic Hernia.

Current literature for congenital diaphragmatic hernia (CDH) focuses on the comparison of the overall mortality in CDH patients. Only a few studies concentrate on analyzing the unstable patients who could not achieve surgical repair, as well as those who could but did not survive after. Hence, this study aimed to analyze the effects of various parameters on the timing of death. A retrospective analysis was performed by using the data of all CDH patients from 2003 to 2016 at a single tertiary center. Patients who were diagnosed with left-sided CDH and expired were included in the study regardless of the cause. Of the 66 expired patients, 5 were excluded due to right-sided CDH. The study population constituted a total of 61 patients, of which 31 patients expired prior to CDH repair, and 30 patients expired at different times after CDH repair. Multinomial regression analysis identified that the ECMO need (B = 20.257, p = 0.000, OR: 62.756, 95% CI 10.600–371.384) and O/E LHR (B = 20.376, p = 0.000, OR: 70.663, 95% CI 48.716–102.415) values were the independent predictors that influenced mortality in this cohort. Prenatal pulmonary measurements are the major predictors determining the severity of the disease in patients with CDH.

Right-sided congenital diaphragmatic hernia masquerading as complicated pneumonia in a young child

Right-sided congenital diaphragmatic hernia masquerading as complicated pneumonia in a young child

Does creating a dome reduce recurrence in congenital diaphragmatic hernia following patch repair?

PurposeTo develop a quantifiable approach to identify a dome shape of the repaired diaphragm using post-operative chest radiograph and to determine if a dome-shaped prosthetic patch repair is associated with a decreased rate of CDH recurrence. MethodsWe conducted a retrospective review of all neonates evaluated at our institution from January 2004 to August 2017 with left- and right-sided CDH with at least 6 months of follow-up after CDH repair. Patch use, post-operative imaging and postnatal outcomes were analyzed. Neonates with patch repair were divided into two groups based on the presence of a dome. Using postoperative chest radiographs, the presence of a dome was classified as having a vertical-horizontal diaphragm ratio (VHDR) greater than 20%. Primary outcome was CDH recurrence after repair. ResultsWe identified 192 neonates who met our inclusion criteria. Cohort survival was 96%, recurrence rate was 15%, 78% had a left-sided CDH; 54% received a patch repair, of which 58% had a type C defect. Of the 104 infants with patch repairs, the CDH recurrence rate was 22% (n = 23) at a median age of 8.5 months (3.8, 20.1). Although neonates with a dome repair had more ECMO use and longer hospital stay, their recurrence rate was about half of those with a non-dome repair (14% vs 28%, p = 0.07). ConclusionsA dome-shaped repair may reduce recurrence following prosthetic patch repair of congenital diaphragmatic hernia. A larger, multi-institutional study is needed to statistically validate this clinically significant observation. Type of StudyRetrospective review. Level of EvidenceIII.

In-Hospital Morbidities for Neonates with Congenital Diaphragmatic Hernia: The Impact of Defect Size and Laterality

To determine in-hospital morbidities for neonates with right-sided congenital diaphragmatic hernia (R-CDH) compared with those with left-sided defects (L-CDH) and to examine the differential effect of laterality and defect size on morbidities. This retrospective, multicenter, cohort study from the international Congenital Diaphragmatic Hernia Study Group registry collected data from neonates with CDH surviving until hospital discharge from 90 neonatal intensive care units between January 1, 2007, and July 31, 2020. Major pulmonary, cardiac, neurologic, and gastrointestinal morbidities were compared between neonates with L-CDH and R-CDH, adjusted for prenatal and postnatal factors using logistic regression. Of 4123 survivors with CDH, those with R-CDH (n=598 [15%]) compared with those with L-CDH (n=3525 [85%]) had an increased odds of pulmonary (1.7; 95% CI, 1.4-2.2, P<.0001), cardiac (1.4; 95% CI, 1.1-1.8; P=.01), gastrointestinal (1.3; 95% CI, 1.1-1.6; P=.01), and multiple (1.6; 95% CI, 1.2-2.0; P<.001) in-hospital morbidities, with a greater likelihood of morbidity with increasing defect size. There was no difference in neurologic morbidities between the groups. Neonates with R-CDH and a larger defect size are at an increased risk for in-hospital morbidities. Counseling and clinical strategies should incorporate knowledge of these risks, and approach to neonatal R-CDH should be distinct from current practices targeted to L-CDH.

Right-sided congenital diaphragmatic hernia associated with hepatopulmonary fusion and congenital pulmonary malformation

Hepatopulmonary fusion is an uncommon malformation associated with right-sided congenital diaphragmatic hernia. We are presenting a case of a full-term male newborn, who was diagnosed with right sided congenital diaphragmatic hernia. During the surgical procedure, hepatopulmonary fusion was incidentally detected. We aimed to describe a patient clinical manifestation, intraoperative finding and the management dilemma.

Clinical characteristics and outcomes of the right congenital diaphragmatic hernia compared to the left: a 10-year single-center experience.

The features of right-sided congenital diaphragmatic hernias (RCDHs) are quite different from those of left-sided CDHs (LCDHs). We have summarized the features of RCDHs experienced in our institution. This retrospective study analyzed the cases of patients with CDH registered at our institution between 2011 and 2020. Defects on each side were compared based on prenatal diagnosis, medical treatment, type of surgery, and outcomes. A total of 101 patients underwent surgery at our institution during the neonatal period, and 11 had RCDHs. RCDHs and LCDHs were significantly different in terms of extracorporeal membrane oxygenation (36% vs. 6%, p = 0.002), patch repair (81% vs. 28%, p < 0.001), recurrence rate (36% vs. 11%, p = 0.022), and length of hospital stay (117days vs. 51days, p = 0.012). The severity of the fetal diagnosis did not reflect postnatal severity. All patients with RCDH survived to discharge, and there was no significant difference in survival rate between the right and left sides. Neonates with RCDH required more intensive treatments; however, the survival rate was comparable between RCDH and LCDH. RCDH was significantly different from LCDH and an optimal treatment strategy for RCDHs should be established.

Fusión hepatopulmonar asociada a hernia diafragmática congénita derecha.

Resumen&#x0D; La hernia diafragmática derecha representa el 20% de los defectos congénitos diafragmáticos. Una de sus complicaciones es la fusión hepatopulmonar, sobre la cual se han reportado 23 casos a nivel mundial; la cual puede ser fibrovascular o parenquimatosa. &#x0D; Presentación de caso: neonato femenino de 34 semanas, con dificultad respiratoria severa, quien fallece 6 días después de su nacimiento. En la necropsia se identifica un defecto central en hemidiafragma derecho con herniación de una porción del lóbulo hepático derecho al tórax, que presenta fusión con el pulmón ipsilateral, el cual se encuentra hipoplásico.

Sonographic Evaluation of a Fetal Right-Sided Diaphragmatic Hernia: A Case Study

Congenital diaphragmatic hernia (CDH) is a condition that occurs during fetal development where the abdominal viscera herniates through a defect in the diaphragm. CDH manifests on either the right or left side of the diaphragm, but more often on the left side. With advancement in sonographic imaging, this condition can be diagnosed as early as the first trimester. Knowledge of the sonographic appearance and any differential diagnosis of CDH will allow sonographers to document this condition. This permits for diagnosis and early intervention for the fetus. A case report is reviewed of a right-sided CDH with the gallbladder, liver, and bowel present in the thoracic cavity.