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American Pediatric Surgical Association Prenatal Counseling Series Congenital Diaphragmatic Hernia from the Fetal Diagnosis and Treatment Committee of the American Pediatric Surgical Association Editor-in-Chief: Ahmed I. Marwan, MD Special thanks to Loren Berman, MD, TM and Erin Perrone, MD ©2018, American Pediatric Surgical Association American Pediatric Surgical Association Prenatal Counseling Series Congenital Diaphragmatic Hernia Congenital Diaphragmatic Hernia Congenital Diaphragmatic Hernia (CDH) refers to congenital absence of at least one diaphragm, which results in abnormal displacement of typically abdominal structures into the fetal chest. Morbidity and mortality of infants born with this congenital anomaly is dependent on many factors including possible coexistence of congenital heart disease, other congenital or genetic anomalies, degree of herniation of structures into the fetal chest, in particular the liver, degree of pulmonary hypoplasia and pulmonary hypertension. Sac-type congenital diaphragmatic hernia (CDH) is a variant in which there is some residual diaphragm membrane with improved prognosis. Source: Colorado Fetal Care Center CongenitalCongenitalCongenitalCongenital Diaphragmatic Diaphragmatic DiaphragmaticDiaphragmatic Hernia Hernia HerniaHernia American Pediatric Surgical Association Prenatal Counseling Series CongenitalCongenitalCongenitalCongenitalCongenital DiaphragmaticDiaphragmatic Diaphragmatic Diaphragmatic Diaphragmatic HerniaHernia Hernia Hernia (CDH)(CDH) (CDH) (CDH) refersrefers refers refers toHerniato to to congenitalcongenital congenital congenital absenceabsence absence absence ofof of of atat at at leastleast least least oneone one one diaphragmdiaphragm diaphragm diaphragm,, ,whichwhich ,which which resultsresults results results inininin abnormalabnormal abnormal abnormal displacementdisplacement displacement displacement ofof of of typicallytypically typically typically abdominalabdominal abdominal abdominal ss tructuress tructuresstructurestructures intointo into into thethe the the fetalfetal fetal fetal chest.chest. chest. chest. MorbidityMorbidity Morbidity Morbidity andand and and mortalitymortality mortality mortality ofof of of infantsinfantsinfantsinfants bornborn born born withwith with with thisthis this this congenitalcongenital congenital congenital anomalanomal anomal anomalyyy isyis is is dependendependen dependen dependentt t onont on on manymany many many factorsfactors factors factors includingincluding including including possiblepossible possible possible coexistencecoexistence coexistence coexistence ofof of of congenitalcongenitalcongenitalcongenital heartheart heart heart disease,disease, disease, disease, otherother other other congenitalcongenital congenital congenital oror or or geneticgenetic genetic genetic anomalies,anomalies, anomalies, anomalies, degreedegree degree degree ofof of of herniationherniation herniation herniation ofof of of structuresstructures structures structures intointo into into thethe the the Differentialfetalfetalfetalfetal chest,chest, chest, chest, inin in in particularparticular particular particular Diagnosis thethe the the liver,liver, liver, liver, degreedegree degree degree ofof of of pulmonarypulmonary pulmonary pulmonary hypoplasiahypoplasia hypoplasia hypoplasia andand and and pulmonarypulmonary pulmonary pulmonary hypertensionhypertension hypertension hypertension.. .SacSac .Sac Sac--type-type-typetype congenitalcongenitalcongenitalcongenital diaphragmaticdiaphragmatic diaphragmatic diaphragmatic herniahernia hernia hernia (CDH)(CDH) (CDH) (CDH) isis is is aa a variantavariant variant variant inin in in whichwhich which which therethere there there isis is is somesome some some residualresidual residual residual diaphragmdiaphragm diaphragm diaphragm memmem mem membrabrabrabranenenene withwith with with Differential diagnosis of congenital diaphragmatic hernia includes: Morgagni improvedimprovedimprovedimproved prognosis.prognosis. prognosis. prognosis. hernia, congenital hiatal hernia, pulmonary agenesis, pulmonary aplasia, pulmonary hypoplasia, pericardial teratoma, congenital pulmonary airway malformation (CPAM),DifferentialDifferentialDifferentialDifferential bronchopulmonary Diagnosis Diagnosis DiagnosisDiagnosis sequestration (BPS), mediastinal lymphatic DifferentialDifferentialDifferentialDifferential diagnosisdiagnosis diagnosis diagnosis ofof of of congenitalcongenital congenital congenital diaphragmaticdiaphragmatic diaphragmatic diaphragmatic herniahernia hernia hernia includes:includes: includes: includes: MorgagniMorgagni Morgagni Morgagni hernia,hernia, hernia, hernia, congenitalcongenital congenital congenital hiatalhiatal hiatal hiatal hernia,hernia, hernia, hernia, malformation, pericardial teratoma, bronchogenic cyst, neurenteric cyst and pulmonarypulmonarypulmonarypulmonary agenesis,agenesis, agenesis, agenesis, pulmonarypulmonary pulmonary pulmonary aplasia,aplasia, aplasia, aplasia, pulmonarypulmonary pulmonary pulmonary hypoplasia,hypoplasia, hypoplasia, hypoplasia, pericardialpericardial pericardial pericardial teratoma,teratoma, teratoma, teratoma, congenitalcongenital congenital congenital pulmonarypulmonary pulmonary pulmonary pleuropulmonaryairwayairwayairwayairway malformationmalformation malformation malformation blastoma. (CPAM),(CPAM), (CPAM), (CPAM), bronchopulmonarybronchopulmonary bronchopulmonary bronchopulmonary sequestrationsequestration sequestration sequestration (BPS),(BPS), (BPS), (BPS), mediastinalmediastinal mediastinal mediastinal lymphaticlymphatic lymphatic lymphatic malformation,malformation, malformation, malformation, pericarpericarpericarpericardialdialdialdial teratoma,teratoma, teratoma, teratoma, bronchogenicbronchogenic bronchogenic bronchogenic cyst,cyst, cyst, cyst, neurentericneurenteric neurenteric neurenteric cystcyst cyst cyst andand and and pleuropulmonarypleuropulmonary pleuropulmonary pleuropulmonary blastoma.blastoma. blastoma. blastoma. Representative MRI images of some of the differential diagnosis Bochdalek hernia Sac-type hernia Morgagni hernia Hiatal hernia BochdalekBochdalekBochdalekBochdalek herniahernia hernia hernia Sac Sac Sac Sac--typetype-type-type herniahernia hernia hernia Morgagni Morgagni Morgagni Morgagni herniahernia hernia hernia Hiatal Hiatal Hiatal Hiatal herniahernia hernia hernia Figure 1 Figure 2 Fig.1: Fetal MRI with Single Shot Fast Spin Echo Fig. 2: Fetal MRI with SSFSE parasagittal image coronal image demonstrating fluid filled stomach demonstrating bowel loops and left lobe of the and bowel loops in the left chest. Both the left and liver (arrow) in the left thorax. right lung are displaced superior and to the right Images courtesy of Kimberly Dannull, MD, and are small in size (arrows). Colorado Fetal Care Center American Pediatric Surgical Association Prenatal Counseling Series Congenital Diaphragmatic Hernia Initial Evaluation Obstetrical Ultrasound Fetal echocardiography Fetal magnetic resonance imaging Prenatal Imaging The goal of prenatal imaging is to exclude alternative diagnoses, evaluate for additional congenital anomalies, identify location and contents of the hernia, predict outcome and guide perinatal and postnatal management. Obstetrical Ultrasound • Evaluate fetal growth • Evaluate the amount of amniotic fluid. The herniated stomach can result in polyhydramnios, which can predispose to preterm labor and delivery • Evaluate anatomy for the presence of other potential congenital anomalies • Evaluate for hydrops: pleural fluid, ascites, pericardial fluid, skin edema, placentomegaly • Measure the Lung-to-Head Ratio (LHR) (Length x width of the lung opposite the side of the hernia, divided by the head circumference with formula L x W/HC) o Mild pulmonary hypoplasia: LHR >1.4 o Moderate pulmonary hypoplasia: LHR 1-1.4 o Severe pulmonary hypoplasia: LHR<1 • Observed-to-expected LHR should be calculated o Mild CDH: O/E LHR >35% o Moderate: O/E LHR 25-35% o Severe: O/E LHR <25% • Right- vs left-sided CDH: Most experts feel that right-sided diaphragmatic hernia has a same or worse prognosis compared to left-sided defect with similaro parameters, Mild pulmonary with the hypoplasia: exception LHR of >1.4 liver positiono (theModerate liver is pulmonary always up hypoplasia:with right-sided LHR 1 defect-1.4 as it is the only organ immediatelyo Severe beneath pulmonary the righthypoplasia: diaphragm.) LHR<1 • Observed-to-expected LHR should be calculated (Zaretsky to add detail of this method) o Mild CDH: O/E LHR >35% o Moderate: O/E LHR 25-35% o Severe: O/E LHR <25% • Modified McGoon Index (MMI) is obtained to evaluate risk for pulmonary hypertension: (diameter of the left pulmonary artery plus the right pulmonary artery divided by the aorta at the level of the expected diaphragm (LPA + RPA/Aorta). A MMI <1 indicates high risk for pulmonary hypertension. • Right- vs left-sided CDH: Most experts feel that right-sided diaphragmatic hernia has a same or worse prognosis compared to left-sided defect with similar parameters, with the exception of liver position (the liver is always up with right-sided defect as it is the only organ immediately beneath the right diaphragm.) Fetal Echocardiogram • Evaluate cardiac position, structure and function (the combination of congenital heart disease and CDH drastically increases mortality, particularly if univentricular anatomy) • Modified McGoon Index (MMI) is obtained to evaluate risk for pulmonary hypertension: (diameter of the left pulmonary artery plus the right pulmonary artery divided
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