Left sided heterotaxy syndrome (polysplenia)

Case contributed by Dr Austin Donnelly

Presentation

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.

Findings:

Bilateral bi lobed lungs.

Calcified lung nodule (longstanding).

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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