Polysplenia syndrome - Bochdalek hernia

Case contributed by Dr Ammar Haouimi

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

 

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Case information

rID: 84864
Published: 13th Jan 2021
Last edited: 14th Jan 2021
Inclusion in quiz mode: Included
Institution: Aurès Medical Imaging Center - Batna Algeria

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