IMPORTANT: We currently have a number of bugs related to image cropping and are actively trying to resolve them. In the meantime, we have disabled cropping. Apologies for any inconvenience. Stay informed: radiopaedia.org/chat

Polysplenia syndrome - Bochdalek hernia

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Abnormal chest radiograph done for ? chest infection. Chest CT was requested.

Patient Data

Age: 5 months
Gender: Female

The CT scan demonstrates:

  • right posterior diaphragmatic defect with intrathoracic herniation of:
    • the stomach which is located on the right
    • and numerous well-defined nodules of various size and homogeneous density (multiple splenules)
  •  no parent spleen is seen in the left hypochondrium
  • ​intrahepatic IVC interruption with azygos/hemiazygos continuation
  • midline/transverse liver
  • bilateral bilobed lungs on lung window
  • bilateral hyparterial bronchi
  • preduodenal portal vein 
  • the body and tail of the pancreas are difficult to identify from the bowel loops that have the same density
  • no evidence of midgut malrotation

Case Discussion

CT features of a polysplenia syndrome with associated Bochdalek hernia. This association is considered uncommon and extremely rare 1.

The patient was referred to a cardiopaediatrician to rule-out an associated congenital heart disease.

 

Additional contributor: ZE. Boudiaf, MD, CHU, Constantine, Algeria

 

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.