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.

Neurodevelopmental outcome in congenital diaphragmatic hernia: Evaluation, predictors and outcome

To review the reported neurodevelopmental outcome of congenital diaphragmatic hernia(CDH) survivors, identify important predictors of developmental disabilities, and describe the pathophysiological mechanisms contributing to adverse outcome. A Medline search was performed for English-language articles cross-referencing CDH with pertinent search terms. Retrospective, prospective, and longitudinal follow-up studies were examined. The reference lists of identified articles were also searched. Neurodevelopmental dysfunction has been recognized as one of most common and potentially most disabling outcome of CDH. Intelligence appears to be in the low normal to mildly delayed range. Neuromotor dysfunction is common during early childhood. Behavioral problems, hearing impairment, and quality of life related issues are frequently encountered in older children and adolescence. Disease severity correlates with the degree of neurological dysfunction. Neurodevelopmental follow-up in CDH children should become standard of care to identify those who would benefit from early intervention services and improve neurological outcomes.

Chylothorax after Repair of Congenital Diaphragmatic Hernia in a Neonate: Usefulness of Conservative Management

Aim: Chylothorax is a recognized complication after surgery for congenital diaphragmatic hernia (CDH) in a neonate. Management strategies for chylothorax include cessation of enteral feedings, repeated aspiration, chest drainage, and total parenteral nutrition. It is important to determine which is the better plan for treatment of chylothorax after repair of CDH. The authors report successful management by use of the MCT diet for a neonate with chylothorax after repair of CDH. Case: A male infant weighing 3.0 kg was delivered by cesarean section at 38 weeks of gestation and intubated immediately after birth. Prenatal ultrasonography had disclosed left-sided posterolateral diaphragmatic hernia. After stabilization, surgery was performed via a left-sided transverse supra-abdominal incision. The unfixed colon, small bowel, stomach, and spleen were reduced from the chest with little difficulty. A hernia sac was not present and the left-sided posterolateral diaphragm showed a defect 3.5 cm × 2.0 cm in width. The child was fed via a nasogastric tube starting on postoperative day 4 and dyspnea disappeared. Plain chest X-ray on postoperative day 7 showed left pleural radioopacity. A specimen of the chest drainage examined on postoperative day 10 was typical of lymph, with a triglyceride level of 328 mg/dl. The chest drainage was dark yellowish, and a medium-chain triglyceride formula was used until postoperative day 30, by which time the effusion has disappeared. Discussion: Chylothorax after repair of CDH may be a transient disorder that will resolve after a period of diminished flow through the thoracic lymphatics.

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.

Clinical analysis of congenital diaphragmatic hernia in newborn infants:report of 33 cases

Objective To review the clinical experience of diagnosis and treatment of the congenital diaphragmatic hernia in newborn infants. Methods Thirty-three neonates were diagnosed having congenital diaphragmatic hernia in our hospitalfrom Jan. 1,2004 to Sept. 30,2009. The clinical data was retrospectively reviewed. Results 21 cases were treated

Intestinal gangrene secondary to congenital transmesenteric hernia in a child misdiagnosed with gastrointestinal bleeding: A case report

BACKGROUND Congenital transmesenteric hernia in children is a rare and potentially fatal form of internal abdominal hernia,and no specific clinical symptoms can be observed preoperatively.Therefore,this condition is not widely known among clinicians,and it is easily misdiagnosed,resulting in disastrous effects.CASE SUMMARY This report presents the case of a 13-year-old boy with a chief complaint of abdominal pain and vomiting and a history of duodenal ulcer.The patient was misdiagnosed with gastrointestinal bleeding and treated conservatively at first.Then,the patient’s symptoms were aggravated and he presented in a shock-like state.Computed tomography revealed a suspected internal hernia,extensive small intestinal obstruction,and massive effusion in the abdominal and pelvic cavity.Intraoperative exploration found a small mesenteric defect approximately 3.5 cm in diameter near the ileocecal valve,and there was about 1.8 m of herniated small intestine that was treated by resection and anastomosis.The patient recovered well and was followed for more than 5 years without developing short bowel syndrome.CONCLUSION In this report,we review the pathogenesis,presentation,diagnosis,and treatment of congenital transmesenteric hernia in children.

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.

Long-term follow-up of patients with congenital diaphragmatic hernia

Neonates with congenital diaphragmatic hernia encounter a number of surgical and medical morbidities that persist into adulthood.As mortality improves for this population,these survivors warrant specialized follow-up for their unique disease-specific morbidities.Multidisciplinary congenital diaphragmatic hernia clinics are best positioned to address these complex long-term morbidities,provide long-term research outcomes,and help inform standardization of best practices in this cohort of patients.This review outlines long-term morbidities experienced by congenital diaphragmatic hernia survivors that can be addressed in a comprehensive follow-up clinic.

Pulmonary artery diameter ratio as a prognostic indicator of congenital diaphragmatic hernia

Background Following on from an earlier study published in 2008 about left pulmonary artery(LPA)flow measured on serial echocardiography being strongly prognostic in left-sided congenital diaphragmatic hernia(CDH)and the ratio of LPA to right pulmonary artery(RPA)diameters being a simple and reliable indicator for commencing nitric oxide(NO)therapy,the ratio of LPA:RPA diameters(PA ratio orPAR)was hypothesized to possibly reflect cardiopulmonary stresses accompanying CDH better.Methods Subjects with isolated left-sided CDH treated between 2007 and 2020 at a single pediatric surgical center were recruited and classified according to survival.Data obtained retrospectively for subject demographics,clinical course,LPA/RPA diameters,and PAR were compared between survivors and non-survivors.The value of PAR for optimizing the prognostic value of PA diameter data in CDH were analyzed with receiver operating characteristic(ROC)curve analysis.Results Of 65 subjects,there were 54 survivors(82.3%)and 11 non-survivors(17.7%);7 of 11 non-survivors died before surgical repair could be performed.Mean PAR for survivors(0.851±0.152)was significantly higher than for non-survivors(0.672±0.108)(p=0.0003).Mean PAR for non-survivors was not affected by surgical repair.Characteristics of survivors were:LPA≥2 mm(n=52 of 54;mean PAR=0.866±0.146)and RPA≥3mm(n=46 of 54;mean PAR=0.857±0.152).Non-survivors with similar LPA and RPA diameters to survivors had significantly lower mean PAR.ROC curve cut-off for PAR was 0.762.Subjects with high PAR(≥0.762)required high-frequency oscillatory ventilation/NO less than subjects with low PAR(<0.762)(p=0.0244 and p=0.0485,respectively)and subjects with high PAR stabilized significantly earlier than subjects with low PAR(1.71±0.68 days vs 3.20±0.87 days)(p<0.0001).Conclusions PAR would appear to be strongly correlated with clinical outcome in CDH and be useful for planning management of cardiopulmonary instability in CDH.

Lung-protective ventilation in the management of congenital diaphragmatic hernia

Prioritizing lung-protective ventilation has produced a clear mortality benefit in neonates with congenital diaphragmatic hernia(CDH).While there is a paucity of CDH-specific evidence to support any particular approach to lung-protective ventilation,a growing body of data in adults is beginning to clarify the mechanismsbehind ventilator-induced lung injury and inform safer management of mechanical ventilation in general.This review summarizes the adult data and attempts to relate the findings,conceptually,to the CDH population.Critical lessons from the adult studies are that much of the damage done during conventional mechanical ventilation affects normal lung tissue and that most of this damage occurs at the low-volume and high-volume extremes of the respiratory cycle.Consequently,it is important to prevent atelectasis by using sufficient positive end-expiratory pressure while also avoiding overdistention by scaling tidal volume to the amount of functional lung tissue rather than body weight.Paralysis early in acute respiratory distress syndrome improves outcomes,possibly because consistent respiratory mechanics facilitate avoidance of both atelectasis and overdistention—a mechanism that may also apply to the CDH population.Volume-targeted conventional modes may be advantageous in CDH,but determining optimal tidal volume is challenging.Both high-frequency oscillatory ventilation and high-frequency jet ventilation have been used successfully as'rescue modes'to avoid extracorporeal membrane oxygenation,and a prospective trial comparing the two high-frequency modalities as the primary ventilation strategy for CDH is underway.

Congenital Hernia of the Diaphragmatic Dome with Neonatal Revelation, Experience from the Neonatology and Neonatal Intensive Care Unit at the University Hospital Center of Oujda

Congenital hernia of the diaphragmatic dome (CHDD) is an embryonic malformation in which all or part of the diaphragmatic dome fails to develop properly. In the majority of cases (80% to 90%), this malformation affects the left posterolateral part of the diaphragm, while in 10% to 15% of cases it affects the right. Bilateral cases are extremely rare, accounting for less than 1% of cases. This malformation is estimated to occur at a frequency of around 1 in 3500 births, with a male predominance. The diaphragmatic defect causes the abdominal organs to rise into the thoracic cavity during critical phases of lung development. These anomalies result in bilateral pulmonary hypoplasia, a reduced number of pulmonary vessels, and pulmonary arterial hypertension (PAH). The combination of these anatomical and functional anomalies, in varying degrees, explains the wide variability of symptoms at birth. Diagnosis is usually made prenatally by ultrasound, which enables severe forms of the disease to be detected and appropriate management initiated. The prognosis remains generally grave, with a neonatal mortality rate of between 30% and 60% depending on the study, and around half of all children will have long-term sequelae.

Gastroesophageal reflux and congenital gastrointestinal malformations

Although the outcome of newborns with surgical congenital diseases(e.g.,diaphragmatic hernia;esophageal atresia;omphalocele;gastroschisis) has improved rapidly with recent advances in perinatal intensive care and surgery,infant survivors often require intensive treatment after birth,have prolonged hospitalizations,and,after discharge,may have longterm sequelae including gastro-intestinal comorbidities,above all,gastroesophageal reflux(GER).This condition involves the involuntary retrograde passage of gastric contents into the esophagus,with or without regurgitation or vomiting.It is a well-recognized condition,typical of infants,with an incidence of 85%,which usually resolves after physiological maturation of the lower esophageal sphincter and lengthening of the intra-abdominal esophagus,in the first few months after birth.Although the exact cause of abnormal esophageal function in congenital defects is not clearly understood,it has been hypothesized that common(increased intra-abdominal pressure after closure of the abdominal defect) and/or specific(e.g.,motility disturbance of the upper gastrointestinal tract,damage of esophageal peristaltic pump) pathological mechanisms may play a role in the etiology of GER in patients with birth defects.Improvement of knowledge could positively impact the long-term prognosis of patients with surgical congenital diseases.The present manuscript provides a literature review focused on pathological and clinical characteristics of GER in patients who have undergone surgical treatment for congenital abdominal malformations.

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

Traumatic Diaphragmatic Hernia in Children: A Case Report

Introduction: Traumatic diaphragmatic hernia is a rare condition in children complicating closed or penetrating trauma to the abdomen and thorax. We report the case of an 11-year-old girl with a traumatic diaphragmatic hernia. Case Presentation: An 11-year-old girl was seen in the paediatric surgery department for a thoracolumbar spine deformity and intermittent chest pain. These symptoms occurred after a domestic accident involving a fall from a low wall onto the thoracolumbar spine 5 months previously. The diagnosis was suggested by the presence of a left hemithoracic hydroaera and confirmed by a thoraco-abdominal CT scan. Surgical exploration revealed a linear rupture of the entire left hemi-diaphragm with herniation of the stomach, small intestine, cecum, transverse colon and omentum. We performed a double-layer suture of the diaphragmatic rupture with a non-absorbable suture without edge rejuvenation after the reduction of the hernia. The outcome was favourable with normal postoperative radiographs at one year follow-up. Conclusion: Traumatic diaphragmatic hernia, although uncommon and difficult to diagnose, is a condition that is relatively easy to manage surgically, even if it is discovered late. In all cases of trauma to the thoracolumbar spine, regular follow-up and repeat X-rays are necessary if pain persists.

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.

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.

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.

A report on the congenital hepatic diaphragmatic hernia

To the Editor:The author found a suspected congenital hepatic diaphragmatic hernia in a middle-aged male specimen about 170 cm in height.The specific report is as follows.The specimen was laid flat and the thoracic cavity and abdominal cavity were opened with conventional anatomical method.Then it was surprisingly found that most of the right lobe of the liver passed through the aortic hiatus in the diaphragm and passed through the oblique fissure hernia of the right lung into the thoracic cavity,causing the lower lobe of the right lung severely squeezed and deformed,followed by dysplasia.Gallbladder and dark green bile was also visible around it,and the left lobe of the liver was still in the abdominal cavity[Figure 1].The project followed the principle of respecting life and voluntariness,and it had passed the review of the Ethics Committee(Approval No.JZFY201620).

Congenital Diaphragmatic Hernia Revealed by an Inaugural Diabetic Ketoacidosis in a 9-Year-Old Child

Introduction: Congenital diaphragmatic hernia is often detected during the prenatal or neonatal period by severe respiratory symptoms. Late-presenting congenital diaphragmatic hernia is uncommon entity resulting in frequent misdiagnosis and inappropriate treatment. Case Report: We report the case of a left congenital diaphragmatic hernia revealed by an inaugural diabetic ketoacidosis in a 9-year-old girl. She has presented progressive weight loss without loss of appetite associated with polyuro-polydipsia, then epigastric pain with vomiting. Blood glucose was 3.2 g/L, ketonuria and 2+ glycosuria. Despite a well-conducted treatment, there was persistence of dyspnea. Chest X-ray and chest CT-scan confirmed the presence of a left diaphragmatic hernia. Evolution was marked by the death of the child on day 2 post-operative from a multivisceral failure. Conclusion: Clinical and radiological signs of congenital diaphragmatic hernia after the neonatal period may be difficult to interpret and may result in delayed diagnosis, erroneous treatment and potentially fatal outcome.

Ex utero intrapartum therapy in infants with congenital diaphragmatic hernia:a propensity score matching analysis

Objective Previous studies have shown that ex utero intrapartum therapy(EXIT)is safe and feasible for newborns with congenital diaphragmatic hernia(CDH).This study reports our experience with EXIT in fetuses with CDH in an attempt to explore the efficacy of EXIT on the survival rate of this population.Methods A retrospective analysis of the clinical data of 116 children with CDH was conducted.The children were assigned to EXIT and non-EXIT groups.Propensity score matching(PSM)toward clinical data was performed,and the clinical characteristics and outcomes were compared.Taking survival at discharge as the main outcome,logistic regression analysis was carried out to explore the efficacy of EXIT on survival.Results During the study period,30 of 116 children received EXIT.After PSM,the survival rates of the EXIT group and the non-EXIT group were 82.76%(24/29)and 48.28%(14/29),respectively(p=0.006).EXIT(OR=0.083,95%CI=0.013to 0.525,p=0.008),liver herniation(OR=16.955,95%CI=2.342 to 122.767,p=0.005),and gestational age at diagnosis(OR=0.662,95%CI=0.497 to 0.881,p=0.005)were independent mortality-related risk factors of all children with CDH.Ninety-nine of 116 children underwent surgery.After PSM,the postoperative survival rates of the EXIT group and non-EXIT group were 84.6%(22/26)and 76.9%(20/26),respectively(p=0.754).Liver herniation(OR=10.451,95%CI=1.641 to 66.544,p=0.013)and gestational age at diagnosis(OR=0.736,95%CI=0.577 to 0.938,p=0.013)were independent mortality-related risk factors of children after surgery.Conclusion EXIT can be performed safely for selected prenatally diagnosed CDH neonates with potentially better survival and does not cause more maternal complications compared with traditional cesarean section.