A case of intrathoracic ectopic kidney presenting with congenital diaphragmatic hernia

Morgagni Hernia in Children: Nine Cases and a Review of the Literature

Congenital Diaphragmatic Hernia: Not Quite There Yet

Congenital diaphragmatic eventration (CDE) and congenital diaphragmatic hernia (CDH) with or without hernia sac are three different types of congenital diaphragmatic malformations, which this study evaluates. All surgically treated patients with CDE or Bochdalek type CDH between 2000 and 2016 were included in this retrospective analysis. Demographics, CDH-characteristics, treatment, and clinical outcome were evaluated. In total, 200 patients were included. Patients with an eventration or hernia sac had no significant differences and were compared as patients without a true defect to patients with a true defect. The 1-year survival of patients with a true defect was significantly lower than patients with no true defect (76% versus 97%, p = 0.001). CDH with no true defect had significantly better short-term outcomes than CDH with true defect requiring patch repair. However, at 30 days, they more often required oxygen supplementation (46% versus 26%, p = 0.03) and had a higher recurrence rate (8% versus 0%, p = 0.006) (three eventration and two hernia sac patients). Conclusion: Patients without a true defect seem to have a more similar clinical outcome than CDH patients with a true defect, with a better survival. However, the recurrence rate and duration of oxygen supplementation at 30 days are higher than CDH patients with a true defect.What is Known:• Congenital diaphragmatic hernia with or without hernia sac and congenital diaphragmatic eventration (incomplete muscularization) are often treated similarly.• Patients with hernia sac and eventration are thought to have a relatively good outcome, but exact numbers are not described.What is New:• Congenital diaphragmatic eventration and patients with hernia sac seem to have a more similar clinical outcome than Bochdalek type CDH with a true defect.• Patients without a true defect (eventration or hernia sac) have a high recurrence rate.

Short-Term Outcome of Neonates With Congenital Heart Disease and Diaphragmatic Hernia Treated With Extracorporeal Membrane Oxygenation

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a serious condition associated with abnormal development of the diaphragm. Its occurrence among newborns ranges from 1 per 2000 to 1 per 4000. Depending on location, CDH can be divided into four types: diaphragmatic-pleural, parasternal, diaphragmatic-pericardial, and hernias at the esophageal opening of the diaphragm. Hernias can be classified as true or false depending on the presence or absence of the hernia sac. In case of diaphragmatic-pleural hernias, when the intestine, spleen, and stomach almost completely protrude into the pleural cavity, symptoms manifest early in the form of severe cardiorespiratory and gastrointestinal disorders. CLINICAL CASE DESCRIPTION: The article discusses a case of a three-year-old child with a congenital diaphragmatic pleural hernia. From birth, the child experienced difficulty breathing at feeding, coughing; he also had recurrent pneumonia. X-ray examination and computed tomography of the chest revealed a left-sided hernia. Surgery was done via a left-sided thoracotomy, no complications. In four months after the surgery, X-ray examination showed straightened lungs and normal mediastinum; left diaphragm cupula was also normal. The child is considered to be completely healthy due to the absence of symptoms and normal chest X-ray findings. The authors' main goal was to raise awareness among doctors about rare congenital diseases such as CDH. Also, they would like to draw attention to the fact that congenital malformations of the lungs, intestines, and other organs can be unexpectedly manifested at any age. Children with frequent respiratory problems or cardiorespiratory conditions have to be X-rayed as soon as possible to detect congenital malformations of the diaphragm or other chest organs for timely surgical intervention. CONCLUSION: As the authors have noted, in recent years X-ray examination of children often reveals undiagnosed at an early age "findings" related to diaphragm pathology, such as false and true diaphragmatic hernias and dome relaxation. CDH is rare in children older than 3 years and has no specific symptoms. It often leads to delayed diagnostics, often visits to pediatricians because of respiratory infections, retardation in physical development and, as a result, to late surgical treatment.

Objective To analyze the concomitant malforrnations,chromosomal abnormalities and outcomes in prenatally diagnosed congenital diaphragmatic hernia (CDH) cases. Methods Cases of fetal CDH,prenatally identified in the First Affiliated Hospital of Sun Yat-sen University from January 2002 to November 2008,were recruited.The concomitant realformations,chromosomal abnormalities and outcomes of fetal CDlH were analyzed.Fisher's exact test was applied. Results During the study period,71 CDH cases were identified including 62(87.3%) left-sided CDH and 9 (12.7%) right-sided ones.Among the 71 CDH fetuses,38(53.5%)were isolated CDH.33 (46.5%)were complicated with other realformations(complex CDH),including 18(54.5 0A) cardiovascular defects,10 (30.3%)central nervous system abnormalities,9(27.2 0A)genitourinary abnormalities and others.Fetal karyotying was performed in 19 out of the 71 CDH fetuses.among which 12 were isolated CDH cases with normal karyotype,and 4 of the rest 7(4/7)complex CDH cases with chromosomal abnormalities showing a significant differenee compared to the isolated CDH (P.0.009).Sixty-five pregnancies were terminated including all complex CDH(n=33)and 32 isolated CDH.The rest 6 isolated CDH fetuses were term delivered and 5 of them survived after repair of diaphragmatic hernia and one died after birth. Conclusions Left-sided CDH are more common than right-sided ones. Approximately half of the CDH cases are complicated with other malformations,especially cardiovascular abnormalities.The risk of chromosomal abnormalities increases in complex CDH and is relatively low in isolated CDH.The influence of surgical procedure on the prognosis of CDH has not yet determined. Key words: Hernia,diaphragmatic; Ultrasonography,prenatal; Chromosome aberrations

Prenatal Diagnosis and Management for Congenital Intrapericardial Diaphragmatic Hernia with Massive Cardiac Effusion: A Case Report and Literature Review

Effect of prenatal tetrandrine administration on transforming growth factor- β1 level in the lung of nitrofen-induced congenital diaphragmatic hernia rat model

Asymptomatic Congenital Intrapericardial Diaphragmatic Hernia and Epigastric Hernia in the Adult

Objective To investigate the effects of prenatal tetrandrine (TET) treatment on the expression of surfactant protein B (SP-B) and proliferation cell nuclear antigen (PCNA) in lung tis-sues of fetal rats with nitrofen-induced congenital diaphragmatic hernia (CDH). Methods Ten preg-nant female Sprague-Dawley rats were randomly divided into 3 groups: Control group (n = 2),CDH group (n = 4) and TET group (n = 4). The rats of CDH group and TET group were administered with 125 mg nitrofen dissolved in 3.0 ml olive oil by gastric gavage. The same volume of olive oil was given to the rats of Control group. From gestation day 18.5,30 mg TET was administered by gastric gavage to the TET group rats once a day for three days. Same dose of natural saline was given to the rats of Control group and CDH group. On day 21 of gestation,all the fetuses were delivered by cesarean sec-tion. The rat fetuses were sacrificed and examined for diaphragmatic hernia. Lung histological studies were made on H&E staining slides. Immunohistochemical staining was performed to detect SP-B and PCNA in lung tissues. Results Of the 74 fetuses harvested from the rats of CDH and TET groups,CDH was found on 48 fetuses of (64.9%). The incidence of CDH between CDH group and TET group was similar (P>0.05). Hypoplastic lungs were noted on the fetal rats of CDH group. Im-proved lung development was found on TET group fetal rats. On the rats of control group,strong ex-pression of SP-B was found in the alveolar and distal bronchial epithelium,but no SP-B positive cells were found in the lung tissues of CDH group fetal rats. After prenatal TET treatment,moderate ex-pression of SP-B was found in the lung tissues of TET group,and it was significantly lower than that of Control group (P 0.05). Compared with the other 2 groups,PCNA posi-tive cells in TET group were significantlydecreased (P < 0.01). Conclusions Prenatal tetrandrine (TET) treatment can accelerate fetal lung differentiation and maturation,and increase the expres-sion of SP-B in the hypoplastic lungs of the fetal rats with nitrofen-induced CDH. PCNA positive cells were decreased in the lung tissue of TET group rats,which indicates TET may not enhance lung mat-uration in CDH rat fetuses by increasing proliferation. Key words: Diaphragmatic hernia,Congenital; Pulmonary hypoplasia; Bronchopulmonary dys-plasia

A rare case of hepatopulmonary fusion associated with a right congenital diaphragmatic hernia: Case report and review of the literature

Objective To explore the effects of prenatal administration of vitamin A(VitA) on late fetal lung development and expression of Hoxa5 and elucidate the possible mechanism of pulmonary maturation in a rat model of congenital diaphragmatic hernia(CDH). Methods A total of 12 pregnant rats were randomly divided into CDH, CDH+ VitA and control groups. CDH was induced in pregnant rats after administration of 100 mg nitrofen at E9.5(day 9.5 of gestation, term in 22 days). Nitrofen-induced CDH+ VitA group received VitA(15 000 IU dissolved in 1 ml oil) i. g. at E10.5. Pregnant rats without nitrofen were selected as control group. Fetal lungs were collected at E21.5. Histological pulmonary development was analyzed by hematoxylin & eosin staining. The expressions of Hoxa5 mRNA and protein were detected by real-time quantitative polymerase chain reaction(qRT-PCR) and Western blot respectively. Results The rate of CDH decreased markedly after prenatal VitA(CDH group 50.0% vs CDH+ VitA group 28.3%). The areas of alveolar space of CDH, control and CDH+ VitA groups were(332.83±72.19),(1 443.37±285.94)and(907.07±54.78) μm2; thickness of alveolar septum(9.72±2.18),(6.17±1.54) and(7.26±1.52)μm; number of pulmonary artery 3.68±1.03, 7.20±0.91 and 6.24±0.88; luminal area to total transectional area(15.76±4.87)%,(38.74±4.94)% and(30.18±7.03)% respectively. Histological finding showed that antenatal administration of VitA promoted lung maturity in CDH+ VitA group. The expression of Hoxa5 significantly decreased in CDH+ VitA group versus CDH group. Conclusions Prenatal administration of VitA promotes pulmonary maturation in nitrofen-induced CDH and down-regulates the expression of Hoxa5. And the improvement of pulmonary hypoplasia may be regulated by the expression of Hoxa5. Key words: Hernia, diaphragmatic; Lung; Vitamin A

Objective To explore the effects of antenatal administration of dexamethasone (Dex) on fetal lung development and the expression of Wnt7b in rat model of nitrofen-induced congenital diaphragmatic hernia (CDH) and elucidate the possible mechanism of prenatal dexamethasone in improving pulmonary hypoplasia associated with CDH.Methods A total of 12 pregnant rats were randomly divided into CDH + Dex,CDH and control groups.CDH was induced in pregnant rats after a dose of 100 mg nitrofen at Day 9.5 of gestation (term,22 days).Dex (0.4 mg/kg) was given by an intraperitoneal injection at Days 18.5 and 19.5 of gestation.Cesarean section was performed at Day 21 of gestation.The fetuses were divided into 3 group of A,control; B,nitrofeninduced CDH; C,nitrofen-induced CDH with antenatal Dex treatment.The histological structures of fetal rat lungs were observed microscopically.The parameters of area of alveolar space,thickness of alveolar septum,number of pulmonary artery and diameter of pulmonary artery were compared to examine the lung development.The expression of Wnt7b mRNA in fetal lungs was detected by realtime quantitative polymerase chain reaction (PCR).And the expression of Wnt7b protein was detected by immunohistochemistry.Results Histological detection showed that prenatal Dex dosing improved lung development in CDH rats.The alveolar space of control,CDH and CDH + Dex groups was (1 443.37 ± 285.94),(332.83 ± 72.19) and (929.36 ± 110.31) μm2 respectively,the thickness of alveolar septum (6.17 ± 1.54),(9.72 ± 2.18)and (7.01 ± 1.32)μm,the number of pulmonary artery (7.20 ± 0.91),(3.68 ± 1.03) and (6.84 ± 0.78) and diameter of pulmonary artery (38.74 ± 4.94) ％,(15.76±4.87)％ and (37.12 ± 7.03)％.At Day 21,Wnt7b relative mRNA expression in CDH and CDH + Dex groups was 1.58 ± 0.19 and 0.72 ± 0.15 compared to control group.The Wnt7b expression in CDH group was significantly higher than that in control group.And it decreased significantly after prenatal Dex.Immunohistochemistry showed that Wnt7b protein was expressed predominantly in airway epithelium.Conclusions Prenatal Dex administration improves pulmonary hypoplasia associated with CDH and down-regulates the expression of Wnt7b rnRNA in fetal lung of CDH rats.It is hypothesized that prenatal Dex may improve pulmonary hypoplasia in CDH by regulating the expression of Wnt7b. Key words: Hernia, diaphragmatic ; Bronchopulmonary dysplasia ; Dexamethasone

despite progress in neonatal care and despite changing concepts in treatment in the recent years, the mortality rate of infants with congenital diaphragmatic hernia (CDH) remains high ([20][1]). Pulmonary hypoplasia and vascular alterations associated with CDH have tremendous postnatal functional

Congenital diaphragmatic hernia (CDH) is a lethal human birth defect occuring in 1 : 2 500 births. An infant with CDH is born every 24–36 hours in the UK. Hypoplastic lung development is the leading contributor to the 40–50% mortality of CDH (1). Efforts to improve survival have focused on fetal intervention, advances in intensive care, in utero diagnosis and delivery at specialist centres (1–3). High frequency oscillatory ventilation, permissive hypercapnia, inhaled nitric oxide and ECMO have been employed with variable outcome (4–6). Preoperative stabilisation and delayed surgical repair is currently performed at most centres (7,8). The impact of abnormal lung development on affected infants has stimulated research on the developmental biology of CDH. Traditionally lung hypoplasia has been viewed as a consequence of in utero compression of the fetal lungs by herniated viscera prolapsing through a defect in the diaphragm. Based on this ‘compression theory’ fetal surgery was pioneered to repair CDH and rescue lung growth in ‘high risk’ cases. It has shown no survival benefits. New insights into mechanisms regulating fetal lung growth and maturation are providing alternative less invasive antenatal strategies. Prompted by the observation that humans with congenital larnygeal atresia have enlarged hyperplastic lungs fetoscopic tracheal occlusion (FETENDO-PLUG) is being evaluated in human trials for the ‘high risk’ CDH fetus With liver herniation (3). Pharmacological strategies to ameliorate pulmonary hypoplasia in CDH are under investigation. Human studies have shown that the lungs of babies with severe CDH resemble the immature lungs of premature newborns with respiratory distress syndrome (RDS). Prenatal hormonal therapy regimens adapted from RDS protocols have demonstrated striking improvements in lung maturation in experimental CDH models. An international multicentre trial of corticosteroid therapy is now underway in prenatally diagnosed CDH patients (9). Closure of the human diaphragm at 8 weeks gestation limits detailed embryological investigation of CDH to teratogenic models. The ‘compression theory’ of pulmonary hypoplasia has been challenged by studies of primordial lung development prior to diaphragmatic hernia (10). Disruption of stereotyped airway branching correlates with and precedes CDH indicating an intrinsic defect of lung development in CDH. Organotypic culture sytems are providing invaluable research tools to manipulate lung hypoplasia and investigate the role of growth factors and cell signalling pathways. Genetic studies in murine models and the fruitfly Drosophila melanogaster have elucidated the molecular control of respiratory organogenesis. Testing fibroblast growth factors (FGFs), epidermal growth factor (EGF) and heparin on normal and hypoplastic lung primordia has demonstrated profound differences in embryonic growth and pulmonary morphogenesis that indicate intrinsic defects in mitagenic regulatory pathways fundamental to CDH (11–13). What are the future therapeutic implications for clinicians treating CDH? Selection of patients for fetal surgery will require more accurate prognostic indicators of poor perinatal outcome. Fetal surgery at present may be ‘too little too late’ to correct an established embryopathy in pulmonary development. Antenatal corticosteroid therapy is the subject of a multicentre trial. Fetoscopic delivery of targeted growth factors to enhance airway development in CDH will require careful appraisal. The concept of a pharmacological agent or medical ‘PLUG’ to reverse pulmonary hypoplasia is a goal for the future. Postnatal therapies to reduce barotrauma will salvage those newborns with the less severe degrees of pulmonary hypoplasia. Finally prevention of the birth defect by preconceptual prophylaxis (as in the case of neural tube defects) may represent the ultimate solution for this highly lethal human anomaly (14).