THE PHYSICIAN AND SPORTSMEDICINE - VOL 26 - NO. 4 - APRIL 98
Casey Martin's case: the medical story
Professional golfer Casey Martin's court fight to use a golf cart in PGA tournaments has focused considerable attention on Klippel-Trénaunay (KT) syndrome, a rare congenital disorder involving complex vascular anomalies and general hypertrophy in an extremity. Ironically, Martin may not actually have the syndrome. His symptoms do, however, threaten his leg.
In February, Martin, 25, used the Americans with Disabilities Act to argue before a federal magistrate that he should be allowed to ride a cart in competition because of the pain caused by his condition. On February 11, Martin won his case; the PGA said it would appeal.
It has been widely reported that Martin has KT syndrome, a combined vascular disorder that is characterized by cutaneous capillary malformations (port-wine stain), bony and soft-tissue hypertrophy, and venous malformations (1). But of these three manifestations, Martin has only venous malformations, according to John B. Mulliken, MD, a pediatric plastic surgeon at Children's Hospital in Boston who has reviewed Martin's case. "It's acceptable to say he has a KT-like vascular anomaly, but he doesn't have KT syndrome," says Mulliken.
In a recently published Mayo Clinic survey of 252 patients who had KT syndrome (2), 98% had capillary malformations, 72% had venous malformations, and 67% had limb hypertrophy. Patients are considered to have KT syndrome if they have two of the three features, the authors wrote.
Patients who have true KT syndrome typically have overgrowth in limb length and girth, but Martin's affected leg is smaller than his other limb, says Mulliken. The venous malformation is within the muscles and skin of the right calf and knee region. "I like to make the analogy to a stagnant, sluggish, swampy sponge containing venous blood," Mulliken says. Varices are also seen superficially. "The veins in his leg swell up and cause pain and localized clotting. It's slowly getting worse," he says. "His doctors worry that the weakened tibial bone could fracture and not heal well."
A Range of Disabilities
Amid the publicity about Martin's case, patients who have true KT syndrome are welcoming public interest in the rare condition and are eager to show that sports participation is possible for some patients who have it, says Judy Vessey, of Edina, Minnesota, coordinator of a national KT syndrome support group. (See "Klippel-Trénaunay Not a Hindrance for Some Athletes," on page 16.)
David J. Driscoll, MD, a pediatric cardiologist at the Mayo Clinic in Rochester, Minnesota, who specializes in treating KT syndrome patients, says patients vary greatly in their ability to be active. "KT syndrome has protean manifestations. On one end of the spectrum are patients who have mild KT syndrome in which one lower extremity is a little bigger than the other; they might have a few varicose veins and have no symptoms and can play professional football," Driscoll says. "On the other end of the spectrum are patients who have a huge malformed leg that they can't walk on."
Pain can discourage sports participation in many patients; the Mayo clinic survey (2) showed that 35% of patients had pain. Martin says that walking is painful and that he ices the leg after activity and takes ibuprofen, according to a newspaper report (3) of court testimony during the PGA trial. "Pain occurs for a variety of reasons: venous congestion, joint involvement, or the abnormal mechanics that result from muscle or bone mass discrepancies," Driscoll says. "When massive blood vessels are in the synovial membrane of the joint, patients can have significant arthritis."
Contact sports pose a bleeding risk for patients who have port-wine stains or superficial varicosities, says Driscoll, and those patients are advised to wear protective covering over the area if they participate.
Currently there is no cure for KT syndrome; treatment goals are to manage symptoms and restore function to and maintain affected limbs. Treatment depends on the patient's symptoms. Martin and other patients who have lower-extremity swelling wear an elastic stocking to relieve the venous congestion and protect the limb from trauma. Vessey says some patients relieve swelling and discomfort by using an external intermittent pressure pump.
Surgery is indicated for patients who have major functional limitations (2). Options for patients who have limb hypertrophy are epiphysiodesis, ray resection, or soft-tissue debulking. For patients who have port-wine stains, laser treatment is available; however, treatment isn't always successful and multiple treatments are sometimes needed. For those who have venous anomalies, malformations or varicosities can be removed only if the deep venous system is patent. Sclerotherapy can reduce the size of venous malformations, but the procedure can be painful and carries a risk of skin breakdown.
Mulliken says that orthopedic surgeons are considering placement of a rod in Martin's tibia to help support the weakened bone. "But it's not going to help the underlying problem," he says.
Klippel-Trénaunay not a hindrance for some athletes
While Casey Martin's experience with a condition resembling Klippel-Trénaunay (KT) syndrome is grabbing the headlines, there are unheralded athletes with true KT syndrome who are competing at high levels.
Bodybuilder Jeff Dotson, 29, of Wichita, Kansas, says he first experienced KT symptoms at age 11, when swelling developed in his right leg and ankle. His affected leg, which has extra veins, is a little larger than his other leg. He played baseball, basketball, and football from elementary school through high school, wearing a compression stocking to control the swelling. Now he's a competitive bodybuilder. Dotson says activity isn't painful, except during phlebitis episodes, which are controlled with antibiotics. "My local doctors said to do what I felt like I could do in sports," Dotson says. "I think exercise helps my symptoms."
Matthew Nole of Columbus, Ohio, says he was diagnosed as having KT syndrome when he was in high school. He says his vascular surgeon encouraged him to continue participating in his sports, which then included wrestling, soccer, golf, football, volleyball, shot put, and discus. "I was having too much fun in sports, so I just kept going," says Nole, who is currently a varsity wrestler and football player at Baldwin-Wallace College in Berea, Ohio. Venous congestion and mildly increased girth affect his left leg, as does port-wine stain. He says his biggest problem is reduced endurance of his left calf muscle. He wears a compression stocking to protect the skin of his leg and reduce swelling in his ankle.
Nole says he welcomes the attention that Casey Martin has brought to KT syndrome. "This is great for other people who have the disorder. Now they can say, 'I can do that, too.'"
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