Klippel-Trénaunay syndrome

Case contributed by Dr Chinmaya Deepak

Presentation

Patient presented with lymphangioma circumscriptum in right upper limb extending into chest wall. Additional features include hypertrophy of phalanges.

Patient Data

Age: 10 years
Gender: Male
X-ray

Overgrowth of 1st-3rd metacarpals and phalanges in right hand and soft tissue overgrowth in the left forearm and hand.

Ultrasound

Multiple hyperechoic lesions throughout the spleen, probably hemangiomas.

MRI

There are multiple hyperintense masses throughout the spleen, perhaps hemangiomas. Heterogeneous, predominately hyperintense lesions along the right chest wall, consistent with a vascular malformation.

Case Discussion

The combination of skeletal, soft tissue and vascular findings is consistent with a congenital overgrowth syndrome associated with vascular anomalies, probably Klippel-Trénaunay syndrome.

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Case information

rID: 30380
Published: 3rd Jun 2015
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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