Published Online: May 17, 2007 - 11:48:20 PM (CDT)
Series Editor: Herbert L. Fred, MD
Prepared by David S. Wolf, MD, Chief Medical Resident, The University of Texas Health Science Center, Starr Borden, MD, Family Medicine in private practice, and Herbert L. Fred, MD, Professor of Medicine, The University of Texas Health Science Center, Houston
This 42-year-old woman presented with life-long hypertrophy of the entire left half of her body. Particularly striking were the left upper torso (Figure 1), arm (Figure 2), and hand (Figure 3). She also had a large port wine hemangioma on her back (Figure 1), and small, superficial varicosities in her left leg. Her family history was unremarkable. This constellation of findings is diagnostic of Klippel-Trenaunay syndrome.
Points to remember: Klippel-Trenaunay syndrome is a rare, sporadic, congenital vascular disorder of unknown cause characterized by the triad of overgrowth and hypertrophy of soft tissues and bones, hemangiomas (usually of the port wine type), and varicose veins - all on the same side. All 3 elements, however, are not invariably present and are not necessarily proportionate in severity or extent. These abnormalities may be localized to one extremity or affect as much as an entire half of the body. Prognosis is good, and treatment is mainly symptomatic.
