Venolymphatic thoracic wall malformation

Case contributed by Dr Maxime St-Amant


Thoracic wall deformity. Haemangioma?

Patient Data

Age: 3
Gender: Male

Longitudinal image of the ultrasound at the site of deformity.

It shows a partly hyperechoic lesion containing many tortuous vessels (much less dans 6 vessels/cm2 as expected in hemangioma). There was a very slow venous flow in these vessels on Doppler imaging (not shown here).

This is highly suggestive of a venolymphatic malformation, but MRI is suggested to further evalute this malformation.

MRI confirms the presence of a voluminous lesion involving the anterior right thoracic wall. It creates an imprint on the liver without invading it, and a very small part of the lesion invades the pleura.

The lesion is hypervascular and contains many small & tortuous vessels, without flow voids, confirming the slow flow detected on the Doppler evaluation. It also has many small enhancing septations. It is mainly iso- to hypo-intense on T1, hyperintense on T2 & T2 FAT-SAT. However, there are no fluid-fluid levels.

A simple hemangioma would enhance more diffusely. It would also contain flow-voids and it is classicaly polylobulated. The presence of septations & tortuous slow-flow veins makes venolymphatic malformation the most plausible hypothesis.

Case Discussion

This case was later confirmed to be a venolymphatic malformation. The patient was treated with percutaneous sclerotherapy.

The main differential diagnosis includes : sarcoma, plexiform neurofibroma & teratoma.

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Case information

rID: 19082
Published: 5th Aug 2012
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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