Congenital Diaphragmatic hernia - imaging appearance in Fetal MRI. Poster No.:

C-0405

Congress:

ECR 2016

Type:

Educational Exhibit

Authors:

E. Olmane; Riga/LV

Keywords:

Hernia, Congenital, Diagnostic procedure, MR, Foetal imaging

DOI:

10.1594/ecr2016/C-0405

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Learning objectives Congenital diaphragmatic hernia (CDH) occurs in about 1/2500-1/5000 of newborns and accounts of approximately 8% of all major congenital anomalies. Most commonly, CDH are left-sided, in 13% right-sided, in 2 % bilateral. The diagnosis of CDH can be easily made on ultrasound by demonstration of stomach, intestines or liver in the thorax and the associated mediastinal shift to the opposite side. Left sided diaphragmatic hernia are easy to demonstrate with ultrasound because of echo-free fluid-filled stomach and small bowel contrast with more echogenic fetal lung. Right-sided hernia is more difficult identify because the echogenity of the feral liver is similar to lung. Fetal MRI has many benefits - excellent tissue contrast, large field of view, both the ispilateral and contralateral lungs can be visualised. The purpose of this poster is to demonstrate how easy is to made correct diagnosis of CDH , and to detect effect of adjacent lung tissue with MR imaging in cases if its not possible with ultrasound.

Background Congenital diaphragmatic hernia (CDH) occurs in about 1/2500-1/5000 of newborns, depending on whether stillbirths are included or not and accounts of approximately 8% of all major congenital anomalies. It is a surgically correctable, anatomical defect with unknown aetiology. Majority of cases are sporadic, less than 2% are familial . The defect is believed to aries in embryological period and lung development during further gestation is impaired. CDH is classified according to the location to the defect in the diaphragm Most commonly, CDH are left-sided, in 13% right-sided, in 2 % bilateral. The diagnosis of CDH can be easily made on ultrasound by demonstration of stomach, intestines or liver in the thorax and the associated mediastinal shift to the opposite side. Left sided diaphragmatic hernia are easy to demonstrate with ultrasound because of echo-free fluid-filled stomach and small bowel contrast with more echogenic fetal lung. Right-sided hernia is more difficult identify because the echogenity of the feral liver is similar to lung. Fetal magnetic resonance imaging plays a role in the evaluation and management of congenital diaphragmatic hernia. Fetal MR imaging has proved valuable for anatomic assessment, determination of the specific type of CDH on the basis of which organs are involved and the effect of the hernia contents on adjacent structures, evaluation and exclusion of hernia-related complications and associated malformations. All of these parameters may aid the fetal care team in terms of prenatal counselling and perinatal planning.

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Findings and procedure details Although CDH has traditionally been classified as posteriolateral (Bochdalek hernias) and anterior (Morgagni hernias), for the purposes of fetal imaging and prenatal counseling it is more practical to classify CDH as intrapleural and mediastinal. Intrapleural hernias occur through defects in the muscular diaphragm. Intrapleural hernia contents create significant mass effect within the thorax, causing pulmonary hypoplasia and contralateral mediastinal shift. Hernias that have traditionally been classified as Bochdalek hernias fall into this category. Mediastinal hernias can be divided into retrosternal and central types. Retrosternal hernias are often under the term Morgagni hernias. The true Morgagni hernias is a subtype of retrosternal mediastinal hernias, with larger ventral hernias being defects in the central tendon. Distinguishing ventral mediastinal hernias from intrapleural hernias is important because the mediastinal hernias tend not to cause pulmonary hypoplasia, the most significant cause of morbidity in neonates with CDH. Historically, ultrasonography (US) has been the mainstay of prenatal imaging for CDH, with ultrasound CDH is easily to diagnosed prenatally. The type of hernia can be more easily identified at fetal MR imaging on the basis of its location, the contents of the hernia, and the effect of these contents on adjacent structures. With ultrasound imaging prenatal prognostication remains a challenge. In view of the current availability of fetal therapy this becomes even more important. To date, one of the most studied markers is the lung area to head circumference ratio, witch is predictive of postnatal outcome - survival and morbidity. The advantage of fetal MRI is that both lungs can be measured accurately, also the fetal body volume and intrathoracic position of the liver can be quantified.

MR Imaging Technique Assessment of whole fetal body is important in complex malformations. All of the patients are imaged on a 1.5-T system (Achieva; Philips Medical Systems) using a torso coil.The mother is positioned supine or on her left side, and no sedative or intravascular contrast agent is ad- ministered. Sequences performed - T2-weighted imaging of the fetal body in the axial, coronal and sagittal planes, T1 -weighted sequence

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(witch are performed in a single breath-hold) of the fetal body in the sagital, coronal plane, and DW -weighted imaging of the fetal body in the axial plane. The determination whether the herniated abdominal viscera in the fetal thorax include the liver and the measurement of contralateral lung and head ratio and was done. Nearly 90% of diaphragmatic hernias are intrapleural, and most of these on the left side. Typically, these hernias contain stomach, small bowel, and spleen, and they may contain part of the liver.These hernias cause cardiomediastinal shift to the contralateral side and result in hypoplasia of both lungs to at least some degree. Liver position has been shown to be an independent predictor of perinatal outcome, with fetuses with left-sided intrapleural hernias containing liver having a worse prognosis than those without liver herniation. Right sided intrapleural hernias are less common, always contain liver, and may contain variable amounts of bowel and stomach. Rarely, intrapleural hernias may be bilateral; these tend to be associated with severe pulmonary hypoplasia.

Images for this section:

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Fig. 1: Fig.1 MRI T2 WI sagital, coronal view of fetus at 26 weeks of gestation - left sided intrapleural CDH , hernia contains stomach, bowel, spleen, and partly liver left lobe, small amount of left pleural cavity fluid, horseshoe kidney . LHR (lung-head ratio) was < 1.0. The fetus has both of two most widely studied bad prognostic factors. The fetus has both of two most widely studied bad prognostic factors.(Exitus letalis antenataly) © - Riga/LV

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Fig. 2: Fig.2 MRI T2 WI coronal, sagital view of fetus in 22 weeks of gestation - right sided intrapleural CDH, hernia contains small bowels, partly right liver lobe, there is mediastinal shift to the left, right sided intrapleural fluid, right lung hypoplasia, compression of the left lung. LHR (lung -head ratio) < 1.0. The fetus has both of two most widely studied bad prognostic factors. (Exitus letalis 3 days after birth). © - Riga/LV

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Fig. 3: Table 1 Malformations Associated with CDH © Amy R. Mehollin-Ray, MD, FAAP • Christopher I. Cassady, MD, FRANZR Darrell L. Cass, MD • Oluyinka O. Olutoye, MB, ChB, PhD, , Fetal MR Imaging of Congenital Diaphrag- matic Hernia

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Conclusion The Ultrasound is first and routinely used screening examination modality for early detection of congenital diaphragmatic hernia, lung hypoplasia, but in some cases after ultrasound examination there is need for another examination, to define pathology and make decision for the therapy. MRI is relatively new, excellent imaging method, to accurately diagnose CDH, location of hernia - intrapleural or mediastinal, content of hernia, the mass effect of mediastinum, fetal lungs. Using MR it is possible precisely define two most widely used prognostic factors - the position of the liver and LHR (lunghead ratio) with pivotal points 1.0. MR method is very important step to develop intrauterin CHD therapy.

Personal information

This work comes from the Radiology Department of Private Medical Centre "Diamed", R#ga, Latvia

e-mail adress of the author: [email protected]

References 1. D.Preyer, A.L.Baert, M.F.Reiser, H.Hricak, M.Knauth, "Fetal MRI" 2. A. Kristina Kilian1 Thomas Schaible2 Valeska Hofmann1 Joachim Brade3 K. Wolfgang Neff1 Karen A. Büsing. Congenital Diaphragmatic Hernia: Predictive Value of MRI Relative Lung-to-Head Ratio Compared with MRI Fetal Lung Volume and Sonographic Lung-to-Head Ratio

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3. Amy R. Mehollin-Ray, MD, FAAP • Christopher I. Cassady, MD, FRANZR Darrell L. Cass, MD • Oluyinka O. Olutoye, MB, ChB, PhD, "Fetal MR Imaging of Congenital Diaphrag- matic Hernia"

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