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Articles » Syndromes » Klippel Trenaunay-Weber syndrome
2009-02-28-09 Klippel-Trenaunay-Weber syndrome © Volkov
Klippel-Trenaunay-Weber syndrome


Andrey Volkov, MD, PhD*; A. Matsionis, MD, PhD**, V. Voloshin, MD, PhD***, N. Mikhanoshina, MD**.

*    Department of Obstetric and Gynecology ¹ 1. Rostov Medical University, Rostov on Don, Russia;
**   Rostov regional Institute of Pathology department experimental pathology, Rostov on Don, Russia;
***  Department of  Pathological anatomy. Rostov Medical University, Rostov on Don, Russia.


Case report

A 30-year-old woman (G1P0), with negative family history for congenital anomalies was referred to our unit at 22 weeks of gestation due to fetal edematous lesions of the skin. Our sonographic examination discovered several subcutaneous lesions of the fetus. The lesions were asymmetric, irregular, and multilocular. The findings were suggestive of Klippel-Trenaunay-Weber syndrome, and the diagnosis was confirmed postnatally.

Images 1, 2: 22 weeks of gestation; the images show transverse (image 1) and parasagittal (image 2) scans of the fetal thorax with bilateral multilocular sonolucent subcutaneous lesions located in axillary and perithoracic regions of the fetal trunk. The right side is predominantly affected.


Images 3, 4: 22 weeks of gestation; the images show right upper limb of the fetus with multilocular sonolucent subcutaneous masses.


Images 5, 6: 22 weeks of gestation; the images show the right axilla (image 5) and right upper limb of the fetus (image 6). The multilocular sonolucent subcutaneous lesions can be seen.


Images 7, 8: 22 weeks of gestation; the images show power Doppler (image 7) and color Doppler (image 8) scans of the multilocular sonolucent subcutaneous fetal lesions. Areas of blood flow can be detected in some parts of the lesions, indicating the mixed, lymphatic and vascular, character of the lesions.


Images 9, 10, and 11: Pathological specimens showing the affected fetus. Asymmetric tumorous lesions predominantly affecting the right upper limb and axilla can be seen.



Images 12, 13, 14, 15, and 16: Histological images of the subcutaneous lesions confirming the diagnosis of the Klippel-Trenaunay-Weber syndrome. Dilated lymphatic, capillary and venous vessels can be seen.






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