Left sided heterotaxy syndrome (polysplenia)

Case contributed by Austin Donnelly


Collapse and upper GI bleed. Examination demonstrated generalized abdominal tenderness.

Patient Data

Age: 95 years
Gender: Female

Features of left sided heterotaxy syndrome:

1. Multiple aberrant nodules of splenic tissue.

2. Azygous continuation of the IVC.

3. Midgut malrotation.

Other findings: Hepatic steatosis. Hiatus hernia. Duodenal diverticulum. Calcified lung nodule (longstanding). Diverticular disease.

Saggital MIP images of CTPA study show:

Case Discussion

This patient presented with collapse and upper GI bleed. Examination revealed diffuse upper abdominal tenderness.

CT of the abdomen and pelvis did not identify a cause for the patients symptoms.

An incidental note was made of multiple features of left sided heterotaxy syndrome - multiple aberrant nodules of splenic tissue, an azygous continuation of the IVC, midgut malrotation.

This prompted review of previous imaging to identify further features of left sided heterotaxy syndrome.

A CTPA from 10 years earlier (above) was reviewed and demonstrated bilateral bilobed lungs.

Other features (not demonstrated in this case) of left sided heterotaxy include TAPVR/PAPVR; Truncated pancreas; biliary abnormalities; midline liver; renal abnormalities.

Life expectancy for left sided heterotaxy is better than for its right sided counterpart due to association with less severe cardiac abnormalities.

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