Congenital diaphragmatic hernia - right-sided

Case contributed by Fabien Ho , 6 Jul 2018
Diagnosis certain
Changed by Daniel J Bell, 7 Jul 2018

Updates to Case Attributes

Age changed from 26WG to 26 weeks gestation.
Body was changed:

Most diaphragmatic hernias (90%) are left-sided, whilethus right-sided are encountered inaccount for only 10% of the cases. Diagnosis of left-sided is usually easier thanks to the stomach acting as an acoustic window. US contrast resolution is often confusing to depictresults in difficulties in distinguishing lung from liver in standard screening, hence small right-sided diaphragmatic hernias may be missed.MR imaging depicts a clearer image of the anatomy, thanks to its multiplanar analysis and improved contrast. LikeIn fetal US the lung to head ratio is employed, whilst with Lung To Head Ratio, MR provides additional prognosisprognostic factors with Estimated Fetal Lung Volume assessementestimated fetal lung volume assessment, which is compared to normal fetal lung volume expected for gestationnalgestational age.Right-sided hernia may be part of a syndromic condition such as wider pathology, e.g. Fryns syndrome (Omim #229850), which includes additional facefacial and limbslimb anomalies. This; this is not the case here.

  • -<p>Most diaphragmatic hernias (90%) are left-sided, while right-sided are encountered in only 10% of the cases. Diagnosis of left-sided is usually easier thanks to the stomach. US contrast resolution is often confusing to depict lung from liver in standard screening, hence small right-sided diaphragmatic hernias may be missed.<br>MR imaging depicts a clearer image of the anatomy, thanks to its multiplanar analysis and improved contrast. <br>Like US with Lung To Head Ratio, MR provides additional prognosis factors with Estimated Fetal Lung Volume assessement, which is compared to normal fetal lung volume expected for gestationnal age.<br><br>Right-sided hernia may be part of a syndromic condition such as Fryns syndrome (Omim #229850) which includes additional face and limbs anomalies. This is not the case here.</p>
  • +<p>Most diaphragmatic hernias (90%) are left-sided, thus right-sided account for only 10% of the cases. Diagnosis of left-sided is usually easier thanks to the stomach acting as an acoustic window. US contrast resolution often results in difficulties in distinguishing lung from liver in standard screening, hence small right-sided diaphragmatic hernias may be missed.<br>MR imaging depicts a clearer image of the anatomy, thanks to its multiplanar analysis and improved contrast. <br>In fetal US the lung to head ratio is employed, whilst with MR provides additional prognostic factors with estimated fetal lung volume assessment, which is compared to normal fetal lung volume expected for gestational age.<br><br>Right-sided hernia may be part of a wider pathology, e.g. <a title="Fryns syndrome" href="/articles/fryns-syndrome-1">Fryns syndrome</a>, which includes additional facial and limb anomalies; this is not the case here.</p>

References changed:

  • 1. Mehollin-Ray AR, Cassady CI, Cass DL, Olutoye OO. Fetal MR imaging of congenital diaphragmatic hernia. (2012) Radiographics : a review publication of the Radiological Society of North America, Inc. 32 (4): 1067-84. <a href="https://doi.org/10.1148/rg.324115155">doi:10.1148/rg.324115155</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/22786994">Pubmed</a> <span class="ref_v4"></span>
  • 2. Rypens F, Metens T, Rocourt N, Sonigo P, Brunelle F, Quere MP, Guibaud L, Maugey-Laulom B, Durand C, Avni FE, Eurin D. Fetal lung volume: estimation at MR imaging-initial results. (2001) Radiology. 219 (1): 236-41. <a href="https://doi.org/10.1148/radiology.219.1.r01ap18236">doi:10.1148/radiology.219.1.r01ap18236</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/11274563">Pubmed</a> <span class="ref_v4"></span>
  • 3. G. Gorincour, J. Bouvenot, M.G. Mourot, P. Sonigo, K. Chaumoitre, C. Garel, L. Guibaud, F. Rypens, F. Avni, M. Cassart, B. Maugey‐Laulom, B. Bourlière‐Najean, F. Brunelle, C. Durand, D. Eurin. Prenatal prognosis of congenital diaphragmatic hernia using magnetic resonance imaging measurement of fetal lung volume. (2005) Ultrasound in Obstetrics and Gynecology. 26 (7): 738. <a href="https://doi.org/10.1002/uog.2618">doi:10.1002/uog.2618</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/16273597">Pubmed</a> <span class="ref_v4"></span>
  • Mehollin-Ray AR, Cassady CI, Cass DL, Olutoye OO. Fetal MR imaging of congenital diaphragmatic hernia. (2012) Radiographics : a review publication of the Radiological Society of North America, Inc. 32 (4): 1067-84. <a href="https://doi.org/10.1148/rg.324115155">doi:10.1148/rg.324115155</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/22786994">Pubmed</a> <span class="ref_v4"></span>
  • Rypens F, Metens T, Rocourt N, Sonigo P, Brunelle F, Quere MP, Guibaud L, Maugey-Laulom B, Durand C, Avni FE, Eurin D. Fetal lung volume: estimation at MR imaging-initial results. (2001) Radiology. 219 (1): 236-41. <a href="https://doi.org/10.1148/radiology.219.1.r01ap18236">doi:10.1148/radiology.219.1.r01ap18236</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/11274563">Pubmed</a> <span class="ref_v4"></span>
  • G. Gorincour, J. Bouvenot, M.G. Mourot, P. Sonigo, K. Chaumoitre, C. Garel, L. Guibaud, F. Rypens, F. Avni, M. Cassart, B. Maugey‐Laulom, B. Bourlière‐Najean, F. Brunelle, C. Durand, D. Eurin. Prenatal prognosis of congenital diaphragmatic hernia using magnetic resonance imaging measurement of fetal lung volume. (2005) Ultrasound in Obstetrics and Gynecology. 26 (7): 738. <a href="https://doi.org/10.1002/uog.2618">doi:10.1002/uog.2618</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/16273597">Pubmed</a> <span class="ref_v4"></span>

Systems changed:

  • Chest

Updates to Study Attributes

Findings was changed:

Thoracic and abdominal situs concords, with both heart and stomach on the left side.US assessment of cardiac anatomy is difficult due to poor acoustic window within this patient.Cardiac axis looks normal, but heart position is shifted to the left: this situation suggests absence of a left-sided structure or a compressive right-sided structure.Color dopplerDoppler shows on the right side a vessel which was wellcould be clearly followed from the umbilical cord, compatible with a portal vein sinus. Suspicion of liver ascension within a normal sized-thorax: right diaphragmatic hernia?

Updates to Study Attributes

Findings was changed:

MR allows better visualization of lung and liver: lung's signal is hyperT2whyper T2W while liver's signal is hypoT2whypo T2W.MR confirms the right sided-sided diaphragmatic defect with an intrathoracic liver within thorax. Portal vein sinus is seen in the coronal and axial planeplanes as a tubular structure without signal due to flow-voids.Right lung is not seen.Left lung is shrunkatrophic. Pulmonary volumetry 7mL7 mL, while expected volumetry for gestationnalgestational age is around 40mL~40 mL.

T1 sequence allows meconium identification: hence the colon is found here in the right abdominal upper quadrant. Note that liver is slightly hyperT1hyper T1W compared to lung as well.

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