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.

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.

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.

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.

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

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.

Maternal diaphragmatic hernia in pregnancy:A systematic review with a treatment algorithm

BACKGROUND Diaphragmatic hernia(DH)is extremely rarely described during pregnancy.Due to the rarity,there is no diagnostic or treatment algorithm for DH in pregnancy.AIM To summarize and define the most appropriate diagnostic methods and therapeutic options for DH in pregnancy based on scarce literature.METHODS Literature search of English-,German-,Spanish-,and Italian-language articles were performed using PubMed(1946–2021),PubMed Central(1900–2021),and Google Scholar.The PRISMA protocol was followed.The search terms included:Maternal diaphragmatic hernia,congenital hernia,pregnancy,cardiovascular collapse,mediastinal shift,abdominal pain in pregnancy,hyperemesis,diaphragmatic rupture during labor,puerperium,hernie diaphragmatique maternelle,hernia diafragmática congenital.Additional studies were identified by reviewing reference lists of retrieved studies.Demographic,imaging,surgical,and obstetric data were obtained.RESULTS One hundred and fifty-eight cases were collected.The average maternal age increased across observed periods.The proportion of congenital hernias increased,while the other types appeared stationary.Most DHs were left-sided(83.8%).The median number of herniated organs declined across observed periods.A working diagnosis was correct in 50%.DH type did not correlate to maternal or neonatal outcomes.Laparoscopic access increased while thoracotomy varied across periods.Presentation of less than 3 days carried a significant risk of strangulation in pregnancy.CONCLUSION The clinical presentation of DH is easily confused with common chest conditions,delaying the diagnosis,and increasing maternal and fetal mortality.Symptomatic DH should be included in the differential diagnosis of pregnant women with abdominal pain associated with dyspnea and chest pain,especially when followed by collapse.Early diagnosis and immediate intervention lead to excellent maternal and fetal outcomes.A proposed algorithm helps manage pregnant women with maternal DH.Strangulated DH requires an emergent operation,while delivery should be based on obstetric indications.

Right Post-Traumatic Diaphragmatic Hernia with Liver Dislocation

Diaphragmatic hernia is a rare consequence of thoraco-abdominal trauma. It may be associated with high morbidity and mortality, particularly if surgical intervention is delayed. We report a case of a right diaphragmatic hernia in a 75-year-old woman. The patient was referred to our hospital with mild dyspnea. Chest radiograph showed an overtly elevated right hemi-diaphragm. Thoracic and abdominal computed tomographic scan was requested and showed a defect of the right diaphragmatic muscle wall with intrathoracic ascension of the liver. During the postoperative course, the patient was still on mechanical ventilation, hemodynamically unstable. She developped urinary peritonitis and an extensive bowel ischemia worsening. We report this case to show that the prognosis is related to associated injuries and possible complications. The possibility of a diaphragmatic rupture should be kept in mind and surgery is mandatory in order to avoid complications.

Congenital Diaphragmatic Hernia: A Benin Teaching Hospital Experience

Congenital diaphragmatic hernia is the outcome of the abdominal contents in the thorax by a breach caused by a failure to close the pleuroperitoneal canal. It is a rare and serious disease. Our teaching hospital had registered these seven last years five cases of congenital diaphragmatic hernia, operated, but never published. We report the most recent one, a case of a newborn baby seen at the 5th hour of life for respiratory distress. Initial examination revealed: an asymmetric thorax, respiratory distress with 88% oxygen saturation at ambient air, tachycardia and abolition of vesicular murmur in the left lung field. L-abdomen was flat and soft. Thoraco-abdominal radiography revealed a clear left intra-thoracic image with mass effect on the left lung parenchyma. He has benefited from a reduction of the herniated visceras and a successful closing of the breach. The operation had few after-effects after five months follow-up with a very good recovery of the cardio-pulmonary function.

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.

Congenital Diaphragmatic Hernia with Delayed Diagnosis: Report of Two Cases

Introduction: Advances in prenatal imaging studies have allowed early diagnosis of Congenital Diaphragmatic Hernia (CDH), although neonatal mortality remains at high levels (60% survival). Despite advances, this study found delayed diagnosis, demonstrating subdiagnosis, possibly caused by lack of resources and/or prenatal diagnostic failure. Case Report 1: Infant, 4 months and 22 days old, dyspneic, cyanotic, with fever and vomiting for 3 days, treated as pneumonia. After the second chest radiography, a right CDH was revealed. Surgical correction was performed and removal of Meckel’s diverticulum was surgical findings. The patient was discharged on the 10th days after admission. Case Report 2: Infant, 11 months and 3 days old, distended abdomen, with fever and dyspnea for 2 days, treated as pneumonia. After the second chest radiography, a left CDH was revealed. The patient was submitted to surgery, intestinal loop incarceration without necrosis was observed, and the correction of CDH was performed. The patient was discharged on the 7th day after admission. Discussion: Three cases, between July 2012 and July 2013, were diagnosed at Dom Malam Hospital;2 Cases of CDH, with delayed diagnosis, showed incidence of 66.66%. Delayed diagnosis of these patients with initial hypotheses of pneumonia demonstrated high risk, emphasizing the importance of diagnosis research through observation of the clinical course, the correct imaging interpretation, in order to establish early diagnosis and treatment.

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

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.

Laparoscopic reduction and repair of acute traumatic diaphragmatic hernia:A video review

Traumatic diaphragmatic hernia is one of the sequela of thoraco-abdominal injury,occurring in about 0.5%-5% of patients presenting post major trauma.Motor vehicle collision is the leading blunt trauma etiology.A high level of suspicion is essential to discover such injuries,as a majority of the patients can be asymptomatic.A case of a successful trans-abdominal laparoscopic reduction and repair of a large acute traumatic diaphragmatic hernia is presented as a video demonstration.We were able to evacuate all intrathoracic air,obliviating the need for a chest tube.Post operatively the patient did well with no complications.If expertise is available,laparoscopic intervention is a feasible option even for large traumatic diaphragmatic hernia preventing the need for a large midline laparotomy incision.