Early diagnosis and management of Congenital diaphragmatic hernia

This case report describes the case of a full term girl baby with left congenital diaphragmatic hernia(CDH)born to a 36-year-old mother at 38 weeks of gestation.The baby at birth had an APGAR 8 and birth weight 2.930 kgs.Pre operatively,the baby was ventilated and was kept Nil per oral and continued with Total parenteral nutrition.Nasogastric tube continuous suction was done.The parents were counselled for the surgical repair of CDH.On the third day of life,exploratory laparotomy was performed.5 cm diaphragmatic defect was found.The stomach,pancreas,small bowel,large bowel,and spleen were successfully restored from the left thoracic cavity and the diaphragmatic defect was closed.Post operatively,the baby was breastfed well,thermo regulated,maintained saturation,passed urine and stool and was discharged.

Ultrasound diagnosis of congenital Morgagni hernias: Ten years of experience at two Chinese centers

BACKGROUND Morgagni hernias are rare anomalies that are easily misdiagnosed or missed.AIM To summarize the ultrasound(US)imaging characteristics of Morgagni hernias through a comparison of imaging and surgical results.METHODS The records of children with Morgagni hernias who were hospitalized at two hospitals between January 2013 and November 2023 were retrospectively re-viewed in terms of clinical findings,US features,and operative details.RESULTS Between 2013 and 2023,we observed nine(five male and four female)children with Morgagni hernias.Upper abdominal scanning revealed a widening of the prehepatic space,with an abnormal channel extending from the xiphoid process to the right or left side of the thoracic cavity.The channel had intestinal duct and intestinal gas echoes.Hernia contents were found in the transverse colon(n=6),the colon and small intestine(n=2),and the colon and stomach(n=1).Among the patients,seven had a right-sided lesion,two had a left-sided lesion,and all of them had hernial sacs.CONCLUSION US imaging can accurately determine the location,extent,and content of Morgagni hernias.For suspected Mor-gagni hernias,we recommend performing sonographic screening first.

Antenatal use of bosentan and/or sildenafil attenuates pulmonary features in rats with congenital diaphragmatic hernia

Background:Lung hypoplasia,pulmonary persistent hypertension of the newborn and its morphological changes are the main features in congenital diaphragmatic hernia(CDH).This study was undertaken to investigate if antenatal use of sildenafil and/or bosentan attenuates vascular remodeling,promotes branching,and improves alveolarization in experimental nitrofeninduced CDH.Methods:Nitrofen(100 mg)was gavage-fed to pregnant rats at post conception day(PCD)9 to induce CDH.The rats were randomized to 5 groups:1)control;2)nitrofen;3)nitrofen+sildenafil 100 mg/kg per day at PCD 16-20;4)nitrofen+bosentan 30 mg/kg per day,at PCD 16-20,and 5)nitrofen+bosentan+sildenafil,same doses and administration days.After cesarean delivery,the offsprings were sacrifi ced.The diaphragmatic defect and pulmonary hypoplasia were identified,and the lungs were dissected.Arterial wall thickness,bronchiolar density and alveolarization were assessed.Results:The offsprings with CDH were characterized by severe pulmonary hypoplasia(lung weight-to-body weight ratio:0.0263[95%confidence interval(CI)0.0242-0.0278)]in the nitrofen group versus 0.0385(95%CI 0.0355-0.0424)in the control group(P=0.0001).Pulmonary arterial wall thickness was decreased to 3.0(95%CI 2.8-3.7)μm in the nitrofen+sildenafil group versus 5.0(95%CI 4.1-4.9)μm in the nitrofen group(P=0.02).Terminal bronchioles increased to 13.7(95%CI 10.7-15.2)μm in the nitrofen+bosentan group in contrast to 8.7(95%CI 7.2-9.4)μm in the nitrofen group(P=0.002).More significant differences(P=0.0001)were seen in terminal bronchioles in the nitrofen+sildenafil+bosentan group than in the nitrofen group[14.0(95%CI 12.5-15.4)μm versus 8.5(95%CI 7.1-9.3)μm].Pulmonary arterial wall thickness was also decreased in the former group.Conclusions:In this rat model,antenatal treatment with sildenafil attenuates vascular remodeling.Bosentan promotes the development of terminal bronchioles in nitrofen-induced CDH.

Adult congenital diaphragmatic hernia of the liver: a rare case report

Congenital diaphragmatic hernia (CDH), which mainly occurs in the newborn or in childhood with severe respiratory distress and high mortality, is rarely found in adult, especially for those uncommon right CDH [1–4]. Whereas, liver as the main

Combination of congenital cleft lip and palate with congenital diaphragmatic hernia: a severe disease course

Congenital cleft lip and palate （CLP） is the most ＇common birth defect now in China. The incidence is 1.62%0 according to the data （1988-- 1992） provided by the National Center for Birth Defects Monitoring. It is also one of the congenital anomalies that have excellent prognosis. But severe complications may occur in the cases accompanied some other deformities. Here we report a case of death caused by left-sided posterolateral congenital diaphragmatic hernia （CDH）, type Bochdalek, after the cleft operation.

Predicting Perinatal Outcomes in Fetuses with Congenital Diaphragmatic Hernia Using Ultrasound and Magnetic Resonance Imaging

Congenital diaphragmatic hernia is a congenital fetal disease,which mainly causes pulmonary hypoplasia and pulmonary hypertension.Effective early prenatal diagnosis can detect and predict the prognosis of congenital diaphragmatic hernia in infants,thus provide a reference for prenatal counseling,early intervention,and potential choices for the child’s family.Ultrasound and magnetic resonance imaging are the most commonly used methods for non-invasive examination of the fetus.This paper discusses evaluation parameters based on these two imaging modalities.

Epidemiology of congenital diaphragmatic hernia among 24 million Chinese births:a hospital-based surveillance study

Background The prevalence of congenital diaphragmatic hernia(CDH)varies across countries,with limited information available on its epidemiology in China.Our study aimed to investigate the prevalence,time trends,and perinatal outcomes of CDH in China,as well as its associated malformations and potential associations with maternal and infant characteristics.Methods This study included all birth and CDH cases from the Chinese Birth Defects Monitoring Network between 2007 and 2019,with CDH cases classified as either isolated or associated.We employed the joinpoint regression model to calculate the trends of prevalence and the annual percent change,with Poisson regression used for adjusted prevalence rate ratios.A P value≤0.05 was considered statistically significant.Results A total of 4397 CDH cases were identified among 24,158,029 births in the study period,yielding prevalence rates of 1.82,1.13 and 0.69 per 10,000 for overall,isolated,and associated CDH,respectively.The prevalence of each type of CDH increased over time.The prevalence of overall CDH varied significantly by infant sex(male vs.female,1.91/10,000 vs.1.63/10,000),maternal residence(urban vs.rural,2.13/10,000 vs.1.45/10,000),maternal age(<20 years,1.31/10,000;20-24 years,1.63/10,000;25-29 years,1.80/10,000;30-34 years,1.87/10,000;≥35 years,2.22/10,000),and geographic region(central,1.64/10,000;east,2.45/10,000;west,1.37/10,000).Cardiovascular anomalies were the most common malformations associated with CDH.Infants with associated CDH had a higher risk of premature birth and perinatal death than those with isolated CDH.Conclusion The increasing prevalence and high perinatal mortality rate of CDH highlight the need for further etiological,epidemiological,and clinical studies among the Chinese population.

A simple appendicitis?An anatomical pitfall:A case report

Rationale:We present a case of appendicitis with an uncommon course due to rare anatomical location of the appendix in the right retroperitoneal space below the diaphragm and above the liver.Patient’s concern:A 32-year-old,previously healthy male with a history of congenital diaphragmatic hernia repair in childhood,presented with 3 days of mild,colicky,central abdominal pain associated with fever,nausea and vomiting.At presentation,pain was localized to the right lower quadrant.Diagnosis:Even though diagnosis of appendicitis was clear,we decided to confirm it with computer tomography(CT).CT revealed elevation of the right dome of the diaphragm and perforated appendix located above the liver.Intervention:Appendectomy was performed via right subcostal approach instead of usual incision in the right lower quadrant.Outcome:Patient recovered well and was discharged on the 5th day after operation.Lessons:Previous congenital diaphragmatic hermia repair may change the location of the appendix.The appendix at rare locations could lead to an uncommon course of appendicitis.On this very note,surgeons should have a high index of suspicion,and CT may help avoid inadvertent complications.

Effect of prenatal tetrandrine therapy on pulmonary vascular structural remodeling in the nitrofen-induced CDH rat model

To examine the effects of prenatal tetrandrine (Tet) therapy on pulmonary arterial structural remodeling in nitrofen-induced congenital diaphragmatic hernia (CDH) Methods CDH was induced in fetal rats by maternal administration of 100*!mg nitrofen by gavage on day 9 5 of gestation (term, day 22) Control animals received olive oil (OO) Tet (24*!mg/kg per day) or normal saline (NS) was given by gavage every day from 16 to 20 days of gestation, and fetuses were delivered by caesarean section on day 21 5 Lung sections from 3 fetuses in each group were studied The number of vessels were calculated, the external diameter (ED), medial wall thickness (MT), percent of medial wall thickness, and wall structure were evaluated by image analysis software Results In the pre-acinar arteries, CDH-NS pups had a significantly increased %MT compared with the OO-NS group ( P <0 05), while CDH-Tet animals had a reduced %MT compared with the CDH-NS rats ( P <0 05) Similar results were seen in the intra-acinar level Significant differences were observed between CDH-NS animals and OO-NS controls in the percentage of muscularized intra-acinar blood vessels ( P <0 001) Tet-treated CDH pups had a reduced percentage of muscularized intra-acinar arteries compared with CDH-NS animals Conclusions Medial hypertrophy is present in both the pre-acinar and intra-acinar arteries in the nitrofen-induced CDH rat model Tet treatment inhibits medial hypertrophy and reduces the percentage of muscularized intra-acinar vessels Prenatal Tet therapy may be efficacious in reducing the risk of PH in human newborns with CDH