Are there particular conditions that have more orthopedic complications?
There’s a wide variation in how severe the different vascular anomalies are. Some of them may just involve a certain body part (such asthe face or the neck or limb), but there are some syndromes where orthopedic problems are very common, such as the Klippel-Trenaunay Syndrome (or KTS), or CLOVES syndrome or some of the subgroups of lymphatic and venous malformations, especially what we call now the FAVAs, the fibroadipose vascular anomalies that can cause really painful contractures in the muscles they affect.
Samantha Spencer, MD
What causes leg length discrepancy in vascular anomalies?
It’s unclear exactly what the signal is but it’s felt that extra blood flow in general (whether it be from trauma, infection, fractures or a vascular anomaly) can make one limb grow more quickly than the other side. We believe that’s part of the reason.
Samantha Spencer, MD
What complications do orthopedic issues cause?
[There’s a] wide spectrum of complications. Pain can certainly be a big problem. If the vascular anomaly happens to be inside a joint, it can bleed and destroy the cartilage causing a very arthritic joint at a young age. It can cause pain in muscles and contracture in muscles so that you get stiff joints that don’t straighten out all the way which are very hard to use; it’s like trying to walk with your knee stuck bent 45 degrees. Then there can just be problems associated with the massive overgrowth; you may have a limb that’s gigantic and it’s very heavy and weighty. So there are a number of complications that can happen.
Samantha Spencer, MD
What different orthopedic surgical treatments do you provide?
A common thing that requires surgery is limb length differences. It’s very hard to walk around if one leg is much more than an inch longer than the other. In the younger years we usually give them a shoe lift, and then as they get towards puberty [we] usually do a surgery called an epiphysiodesis, or selective growth stoppage. Through small incisions we drill out the dividing growth plate, usually above and sometimes below the knee too. Sometimes we put screws across it and then the shorter leg can catch up in the last few years of growth. That’s probably the simplest common surgery. Then there are problems of masses on limbs that need to come off. You can debulk some of the vascular anomalies; you usually can’t cure them but you can take away the part that’s causing the problem. Then there are problems associated with contractures; if muscles are getting really tight and dysfunctional, sometimes you have to surgically lengthen them to release a contracture.
Samantha Spencer, MD
Bleeding into joints can be a real problem, so another common thing is looking inside joints with an arthroscopy. That just means looking in joints with little tools that have a camera and if we find vascular malformations inside the joint there are tools we can use to take them away. Sometimes you have to do that with an open incision but a lot of times you can do it through very small arthroscopic incisions. Then there are the salvage procedures; for example some people who have really severe limb problems, sometimes their leg is massive and it’s taking too much blood away from their heart, and sometimes amputation is the only solution. I do have partners also who help with spinal surgeries; some of the patients actually have these vascular malformations growing inside their spines. If it’s inside their spine affecting their nerves, sometimes our neurosurgeons will help. If it’s causing a curve in the back, one of my partners will fuse the spine with rods and screws. Then besides amputations, sometimes we have to fuse joints. Sometimes if the vascular malformation has taken away the cartilage at a young age, you make the joint permanently stiff so that the limb can still be used and it doesn’t hurt anymore.
Samantha Spencer, MD
How do you design treatments for patients with orthopedic complications?
The basics are as soon as you think the wound is healed enough you start range of motion and strengthening usually and it just depends as to how long that might take before they’re allowed to do something. You can somewhat map out what the three months post-op look like, but it’s different for each operation. There are some people that come and we’re just taking a little mass of vascular malformation off their foot. Then you say “Keep the wound out of a bathtub for two weeks and here’s crutches for a few days, and then go to therapy for a few weeks for range of motion and strengthening.” Then for people that have had an amputation say, it’s first getting the wound healed, wrapping it up to prevent swelling, and then fitting the prosthesis, then getting them used to walking again. It’s a wide range.
Samantha Spencer, MD