Asplenia syndrome: situs ambiguous, asplenia with bilateral right sidedness

Case contributed by Dr Essam G Ghonaim

Presentation

Archived case, no previous investigations were available.

Patient Data

Age: 12
Gender: Female

Dextro-position of the heart, absent spleen, transverse liver, bilateral tri-lobed lungs. 

Incidental finding is the presence of two renal arteries are seen on each side.

Case Discussion

Heterotaxy syndrome is the presence of certain organs forming on the opposite side of the body. It may affect the heart, liver, lungs, intestines, and spleen, yet the first identification in babies with heterotaxy syndrome is usually by problems with their hearts or livers. 

It has no sex predilection and the heart is involved about 80% of cases.

The aetiology is not yet known. Few cases were reported to have some familial linkage.

Findings may include (all imaging findings need not be present):

  • Thoracic: bilateral right sidedness,  dextro- meso- or levocardia, bilateral SVC, absent coronary sinus and multiple associated congenital heart diseases.
  • Abdominal: transverse liver, splenic agenesis, left sided stomach (with dextrocardia), right sided stomach (with levocardia) or midline stomach. 
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Case information

rID: 37551
Case created: 12th Jun 2015
Last edited: 5th Dec 2016
Inclusion in quiz mode: Included

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