Postmortem Characteristics of Lung Hypoplasia at Diaphragmatic Hernia: MRI – Pathomorphological Comparisons

Purpose: the study of postmortem MRI possibilities for the diagnosis of lung hypoplasia in congenital diaphragmatic hernia.Materials and methods. A comparison of the results of postmortem MRI study and data of pathoanatomical autopsy of 23 newborns was performed. In group I, the bodies of 10 deceased newborns with congenital diaphragmatic hernia without operative intervention were examined. In group II – the bodies of 7 newborns who died after surgery for congenital diaphragmatic hernia. Group III (control) included 6 bodies of newborns without diaphragmatic hernia and signs of lung hypoplasia. Before the autopsy, an MRI study was performed on a 3T Magnetom Verio device (Siemens, Germany) in standard T1 and T2 modes. The volumes of the lungs and chest cavity were calculated in the analysis of the tomograms data and their 3D reconstruction. The stage of the lung development and number of radial alveoli were identified at the microscopic study of histological preparations.Results.As a result of the postmortem MRI study, it was established that the observations of group I are characterized by minimal lung volumes. The mean lung volume on the side of the diaphragmatic hernia was 4.1 times less than the contralateral lung (p < 0.01), and the mean values of the volume of both lungs were 4.6 times less than the corresponding values of the control group (p < 0.01) . The average value of the specific volume of the lungs in newborns who died as a result of congenital diaphragmatic hernia (group I) was 8.8%, which is 4.2 times less than the control group (p < 0.01) and was accompanied by histological signs of hypoplasia. The operation in Group II observations led to an increase in lung size. However, the specific volume of the lungs in this group remained by 18.6% less than the control group, and on histological specimens there were signs of lung hypoplasia.Conclusion.The postmortem MRI of dead newborns allows for an objective quantification of lung volumes and verifies the presence of hypoplasia. This helps to clarify the pathogenesis and determine the immediate cause of death. Indices of specific lung volume relative to the chest cavity of less than 20% indicate lung hypoplasia as the immediate cause of death of the newborn.

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Possibility of application of prenatal ultrasound indexes in congenital diaphragmatic hernia in fetus to determine postnatal outcomes

10.21516/2413-1458-2021-20-1-17-24 ◽

2021 ◽

Author(s):

N.V. Mashinets

Keyword(s):

Congenital Diaphragmatic Hernia ◽

Diaphragmatic Hernia ◽

Pleural Cavity ◽

Prenatal Ultrasound ◽

Lung Hypoplasia ◽

Prenatal Period ◽

Compression Index ◽

Disease Group ◽

Group I ◽

Group Ii

Objectives. To assess the effectiveness of the use of prenatal ultrasound indexes in congenital diaphragmatic hernia of the fetus to determine the postnatal prognosis. Materials. The analysis of 95 observations of left-sided congenital diaphragmatic hernia of the fetus was carried out. In the prenatal period, the composition of organs displaced into the pleural cavity was determined, the heart compression index (HCI), O/E LHR according to Jani and DeKoninck, and QLI were calculated. Results. Survival rate of newborns was 57.9%, mortality rate was 42.1%. The newborns were divided into two groups depending on the outcome of the disease. Group I — surviving newborns (n = 55), group II — deceased patients (n = 40). In the analyzed groups, there were no statistical differences in the timing of delivery, birth weight of newborns, the severity of asphyxia after birth and the type of hernia. In group I, the intestinal loops and stomach were significantly more often identified in the pleural cavity in isolation, less often the liver. HCI corresponded to 1.3, Jani O/E LHR 45.7%, DeKoninck O/E LHR 38.7%, QLI 0.7. In group II, concomitant malformations, polyhydramnios and displacement of the liver into the pleural cavity were significantly more frequent. HCI was 1.5, Jani O/E LHR 38.6%, DeKoninck O/E LHR 32.0%, QLI 0.6. Conclusions. In predicting the outcome of the disease for a newborn, the most effective is a comprehensive assessment of the location of the liver, the heart compression index and the index of lung hypoplasia (O/E LHR according to Jani). The diagnostic accuracy of the method is 80%, the sensitivity is 74.4%, and the specificity is 83.3%.

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Motion corrected fetal body MRI provides reliable 3D lung volumes in normal and abnormal fetuses

10.22541/au.163251136.65436407/v1 ◽

2021 ◽

Author(s):

Joseph Davidson ◽

Alena Uus ◽

Alexia Egloff Collado ◽

Milou Van Poppel ◽

Jacqueline Matthew ◽

...

Keyword(s):

Congenital Diaphragmatic Hernia ◽

Diaphragmatic Hernia ◽

Lung Volume ◽

Fetal Mri ◽

Fetal Lung ◽

Lung Hypoplasia ◽

Healthy Controls ◽

Lung Volumes ◽

Total Lung Volume ◽

Volume Registration

Abstract Objective:Evaluate deformable slice-to-volume registration (DSVR) to calculate 3D-segmented total lung volume (TLV) in fetuses with congenital diaphragmatic hernia, congenital lung lesions and healthy controls, with comparison to 2D-manual segmentation. Design:Pilot study Setting:Regional fetal medicine referral centre Sample:Fetal MRIs performed for clinical indications (abnormal cases) or as research participants (healthy controls) Methods:Sixteen MRI datasets of fetuses (22-32 weeks GA). Diagnosis: CDH(n=5), CPAM(n=2), CDH with BPS(n=1) and healthy control(n=8). DSVR was used for reconstruction of 3D isotropic (0.85 mm) volumes of fetal body followed by semi-automated lung segmentation. The resulting 3D TLV were compared to the traditional 2D-based volumetry, and a normogram of DSVR-derived fetal lung volumes from 100 cases was produced. Main Outcome Measures:Concordance with 2D-volumetry assessed with Bland-Altman analysis, results of segmentations presented visually. Observed/Expected values were calculated for abnormal cases based upon the normogram. Results:DSVR-derived TLV values have high correlation with the 2D-based measurements but with a consistently lower volume; bias -1.44cm3 [95% limits: -2.6 to -0.3] with improved resolution able to exclude hilar structures even in severe motion corruption or in cases of lung hypoplasia. Conclusions:Application of DSVR for fetal MRI provides a solution for analysis of motion corrupted scans and does not suffer from the interpolation error inherent in 2D-segmentation as per current clinical practice. It increases information content of acquired data in terms of visualising organs in 3D space and quantification of volumes, which we believe will have important value for counselling and surgical planning. Keywords:Fetal MRI; congenital diaphragmatic hernia; CPAM; lung volume

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Defective mesothelium and limited physical space are drivers of dysregulated lung development in a genetic model of congenital diaphragmatic hernia

Development ◽

10.1242/dev.199460 ◽

2021 ◽

Vol 148 (10) ◽

Author(s):

Rachel M. Gilbert ◽

Laurel E. Schappell ◽

Jason P. Gleghorn

Keyword(s):

Mouse Model ◽

Congenital Diaphragmatic Hernia ◽

Diaphragmatic Hernia ◽

Genetic Model ◽

Ex Vivo ◽

Physical Space ◽

Lung Hypoplasia ◽

Lung Growth ◽

Knockout Mouse Model ◽

Chest Cavity

ABSTRACT Congenital diaphragmatic hernia (CDH) is a developmental disorder associated with diaphragm defects and lung hypoplasia. The etiology of CDH is complex and its clinical presentation is variable. We investigated the role of the pulmonary mesothelium in dysregulated lung growth noted in the Wt1 knockout mouse model of CDH. Loss of WT1 leads to intrafetal effusions, altered lung growth, and branching defects prior to normal closure of the diaphragm. We found significant differences in key genes; however, when Wt1 null lungs were cultured ex vivo, growth and branching were indistinguishable from wild-type littermates. Micro-CT imaging of embryos in situ within the uterus revealed a near absence of space in the dorsal chest cavity, but no difference in total chest cavity volume in Wt1 null embryos, indicating a redistribution of pleural space. The altered space and normal ex vivo growth suggest that physical constraints are contributing to the CDH lung phenotype observed in this mouse model. These studies emphasize the importance of examining the mesothelium and chest cavity as a whole, rather than focusing on single organs in isolation to understand early CDH etiology.

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Functional and structural evaluation in the lungs of children with repaired congenital diaphragmatic hernia

BMC Pediatrics ◽

10.1186/s12887-021-02586-3 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

June-Young Koh ◽

Euiseok Jung ◽

Hyun Woo Goo ◽

Seong-Chul Kim ◽

Dae Yeon Kim ◽

...

Keyword(s):

Pulmonary Function ◽

Congenital Diaphragmatic Hernia ◽

Diaphragmatic Hernia ◽

Lung Volume ◽

Main Bronchus ◽

Abdominal Circumference ◽

Control Group ◽

Lung Volumes ◽

Total Lung Volume ◽

Abnormal Pulmonary Function

Abstract Background To evaluate the long-term functional and structural pulmonary development in children with repaired congenital diaphragmatic hernia (CDH) and to identify the associated perinatal-neonatal risk factors. Methods Children with repaired CDH through corrective surgery who were born at gestational age ≥ 35 weeks were included in this analysis. Those who were followed for at least 5 years were subjected to spirometry and chest computed tomography for evaluation of their functional and structural growth. Main bronchus diameters and lung volumes (total, left/right) were measured. According to total lung volume (TLV) relative to body surface area, children were grouped into TLV ≥ 50 group and TLV < 50 group and the associations with perinatal-neonatal factors were analyzed. Results Of the 28 children (mean age, 6.2 ± 0.2 years) with left-sided CDH, 7 (25%) had abnormal pulmonary function, of whom 6 (87%) showed restrictive patterns. All pulmonary functions except FEF25–75% were worse than those in matched healthy control group. Worse pulmonary function was significantly associated with small head and abdominal circumferences at birth. The mean TLV was 1339.1 ± 363.9 mL and LLV/TLV was 47.9 ± 2.5 mL. Children with abnormal pulmonary function were more likely to have smaller lung volumes. In multivariate analysis, abdominal circumference at birth was significantly associated with abnormal lung volume. Conclusions A quarter of children with repaired CDH showed abnormal pulmonary function. Small abdominal circumference at birth was associated with abnormal pulmonary function and lower TLV.

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The co-occurrence of congenital diaphragmatic hernia, esophageal atresia/tracheoesophageal fistula, and lung hypoplasia

Birth Defects Research Part A Clinical and Molecular Teratology ◽

10.1002/bdra.20098 ◽

2005 ◽

Vol 73 (1) ◽

pp. 53-57 ◽

Cited By ~ 15

Author(s):

Marieke van Dooren ◽

Dick Tibboel ◽

Claudine Torfs

Keyword(s):

Congenital Diaphragmatic Hernia ◽

Esophageal Atresia ◽

Diaphragmatic Hernia ◽

Tracheoesophageal Fistula ◽

Lung Hypoplasia

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Computed tomography based measurements to evaluate lung density and lung growth after congenital diaphragmatic hernia

Scientific Reports ◽

10.1038/s41598-021-84623-w ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Timm Stoll-Dannenhauer ◽

Gregor Schwab ◽

Katrin Zahn ◽

Thomas Schaible ◽

Lucas Wessel ◽

...

Keyword(s):

Congenital Diaphragmatic Hernia ◽

Diaphragmatic Hernia ◽

Contralateral Side ◽

Chest Ct ◽

Control Group ◽

P Value ◽

Lung Density ◽

Ipsilateral Lung ◽

Contralateral Lung ◽

Pediatric Chest

AbstractEmphysema-like-change of lung is one aspect of lung morbidity in children after congenital diaphragmatic hernia (CDH). This study aims to evaluate if the extent of reduced lung density can be quantified through pediatric chest CT examinations, if side differences are present and if emphysema-like tissue is more prominent after CDH than in controls. Thirty-seven chest CT scans of CDH patients (mean age 4.5 ± 4.0 years) were analyzed semi-automatically and compared to an age-matched control group. Emphysema-like-change was defined as areas of lung density lower than − 950 HU in percentage (low attenuating volume, LAV). A p-value lower than 0.05 was regarded as statistically significant. Hypoattenuating lung tissue was more frequently present in the ipsilateral lung than the contralateral side (LAV 12.6% vs. 5.7%; p < 0.0001). While neither ipsilateral nor contralateral lung volume differed between CDH and control (p > 0.05), LAV in ipsilateral (p = 0.0002), but not in contralateral lung (p = 0.54), was higher in CDH than control. It is feasible to quantify emphysema-like-change in pediatric patients after CDH. In the ipsilateral lung, low-density areas are much more frequently present both in comparison to contralateral and to controls. Especially the ratio of LAV ipsilateral/contralateral seems promising as a quantitative parameter in the follow-up after CDH.

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Prenatal Diagnosis and Management of a Rare Central Tendon Defect Type of Congenital Diaphragmatic Hernia with a Massive Pericardial Effusion

Case Reports in Obstetrics and Gynecology ◽

10.1155/2020/6798253 ◽

2020 ◽

Vol 2020 ◽

pp. 1-5

Author(s):

Inas Babic ◽

Haifa Al-Jobair ◽

Osama Al Towaijri ◽

Huda Al-Shammary ◽

Merna Atiyah ◽

...

Keyword(s):

Pericardial Effusion ◽

Congenital Diaphragmatic Hernia ◽

Diaphragmatic Hernia ◽

Neonatal Morbidity ◽

Lung Hypoplasia ◽

Defect Type ◽

Tendon Defect ◽

Prenatal Diagnostic ◽

Large Pericardial Effusion ◽

Prenatal Intervention

The central tendon defect type of congenital diaphragmatic hernia (CDH) is extremely rare and usually associated with a significant pericardial effusion. Prenatal diagnostic ultrasound features of this quite rare entity remain often overlooked or misdiagnosed. There is a dearth of literature about the role of prenatal intervention, often through an elective pericardiocentesis, for the prevention of lung hypoplasia and to decrease the overall neonatal morbidity and mortality. To the best of our knowledge, till date, there is only one case that was subjected to a prenatal intervention. Here, we present a second case of a central tendon defect type of CDH with a large pericardial effusion that was subjected to a prenatal transthoracic pericardiocentesis. Although smooth intubation and ventilation were performed immediately after birth, the infant suffered for several months from respiratory instability. Laparoscopic central tendon hernia repair was performed, and neonate was discharged home at seven months of age. Although prenatal pericardiocentesis may facilitate smoother postnatal intubation and ventilation, its broader effect on respiratory function is uncertain and still remains elusive.

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Cellular Origin(s) of Congenital Diaphragmatic Hernia

Frontiers in Pediatrics ◽

10.3389/fped.2021.804496 ◽

2021 ◽

Vol 9 ◽

Author(s):

Gabriëla G. Edel ◽

Gerben Schaaf ◽

Rene M. H. Wijnen ◽

Dick Tibboel ◽

Gabrielle Kardon ◽

...

Keyword(s):

Congenital Diaphragmatic Hernia ◽

Diaphragmatic Hernia ◽

Birth Defect ◽

Congenital Disorder ◽

Diaphragmatic Defect ◽

Lung Hypoplasia ◽

Cellular Origin ◽

Recent Developments ◽

Mechanical Factors

Congenital diaphragmatic hernia (CDH) is a structural birth defect characterized by a diaphragmatic defect, lung hypoplasia and structural vascular defects. In spite of recent developments, the pathogenesis of CDH is still poorly understood. CDH is a complex congenital disorder with multifactorial etiology consisting of genetic, cellular and mechanical factors. This review explores the cellular origin of CDH pathogenesis in the diaphragm and lungs and describes recent developments in basic and translational CDH research.

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Tetralogy of Fallot with congenital diaphragmatic hernia and left lung hypoplasia: assessment of the adequacy of the peripheral pulmonary vascular growth after hernia repair

Cardiology in the Young ◽

10.1017/s104795111100134x ◽

2011 ◽

Vol 22 (2) ◽

pp. 235-238 ◽

Cited By ~ 3

Author(s):

Hong Ju Shin ◽

Won Kyoung Jhang ◽

Tae Jin Yun

Keyword(s):

Congenital Diaphragmatic Hernia ◽

Tetralogy Of Fallot ◽

Diaphragmatic Hernia ◽

Left Lung ◽

Lung Hypoplasia ◽

Vascular Growth ◽

Threatening Condition ◽

Central Pulmonary Artery ◽

Life Threatening ◽

Vascular Beds

AbstractCongenital diaphragmatic hernia is a life-threatening condition frequently associated with various congenital cardiac diseases. In congenital diaphragmatic hernia associated with tetralogy of Fallot, central pulmonary artery size of the affected side may not reflect the capacitance of peripheral pulmonary vascular beds. We report a case of congenital diaphragmatic hernia associated with tetralogy of Fallot, which was repaired after assessing the adequacy of the pulmonary vascular beds by intra-operative pulmonary blood flow study.

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