Orthopaedics
Most children with EvC will need to have surgery on their legs at some point. EvC is a skeletal dysplasia which means that the way that the bone grows is abnormal. This can manifest in different ways and places according to the child.
One of the commonest problems is knock knees ("genu valgum" in medical terms). The growth plate at the upper end of the shin bone (tibia) grows faster on the inside than the outside edge so that the knees point inwards and the feet point outwards over time as your child grows. If left unchecked this causes knee pain as the knee cap can’t slide properly in its groove, and the ligaments on the inside of the leg become progressively stretched. The stretching of the ligaments can lead to instability of the knee and knee cap. The knee cap can eventually dislocate because the angle at which the thigh muscle pulls it is so abnormal that it pulls it to the outer side of the leg. The feet and ankles are also affected by this as the leg position forces the feet to go flatter and flatter and the ligaments on the inside edge of the ankle become stretched. This makes walking very awkward and uncomfortable.
Some children benefit from having orthotics (special insoles or splints) made for them by an orthotist. This can delay, but not prevent the need for surgery in some cases.
The most common operation to treat this problem is where the tibial growth plate is stapled on the inside edge to slow down growth there. The staples are removed once the growth down the outside has caught up and the leg straightened. Sometimes a tibial osteotomy is needed to realign the shin bone. For this procedure a small triangular wedge of bone is cut out below the growth plate to straighten up the leg. After this operation your child will have full length leg casts for at least 6 weeks as the bone heals (actual length of time will be determined by your orthopaedic surgeon). Children usually recover really well from this operation and can return to their normal activities including sports.
A more recent procedure to straighten knees in children is the use of an "8-Plate". This was made available on the NHS in the UK this year (2006) and I'm really quite excited about it! You can find out all about it here - eight-Plate. Used early enough, this should seriously reduce the number of osteotomies required in kids with EvC. It's a day procedure and your child can be full-weightbearing straight after the operation! I would like to say that it will abolish the need for osteotomies and more radical procedures, but each child has their own particular orthopaedic problems, so no-one can promise that. Would be great though, wouldn't it? A couple of children in the support group have had this procedure done. The first to get it done has had very good results. The second has only recently had it done, but I shall update the site with details in about a year's time. Good pain relief is essential after this operation to encourage early weight bearing. The first child to have it done took a week or so to get on her feet because she was so sore, but she took up dancing again once the swelling died down. The removal was less traumatic.
If the deformity has become very severe, then it has to be corrected slowly with an external fixator. If it were done quickly with an operation, then the nerve and blood vessel down the outside of the leg could be damaged by being overstretched. This could have very serious consequences for your child. External fixation is set up by placing pins through the skin, into the bone and a rod goes down the outside of the leg. There are adjustments which can be made as the bone grows, so the leg slowly straightens. How long this takes will vary from child to child, but think in terms of at least 2 months or more. Kids can often walk with an external fixator in place with the aid of crutches, but this will be at the discretion of your orthopaedic surgeon.
External fixators in place (Click on thumbnails to see larger picture)
Physiotherapy may be needed to help your child rehabilitate if they have problems regaining knee joint movement and a normal walking pattern.
Another procedure which may be needed is bone lengthening. I refer to this only as a therapeutic measure to even up differences in growth between left and right legs or between the tibia (shin bone) and fibula (fine bone to the side of tibia), in order to improve walking function and comfort. (Bone lengthening as an elective procedure to increase height is a contentious issue within the restricted growth community, and as such I will not discuss it.) The following personal account will give you better insight than I can:
"I had knee operations and leg operations. Both were needed! My knees dislocated out of socket and so that had to be fixed when I was 7 and 8 years old. On my left knee.....the operation did not work, so I live with it. My legs were done when I was 16 and I had to have it done or I would be in a wheelchair now. My tibia (I think) was not going into my ankle right and causing major pain. From what the doctors said my tissue was being "eaten" away because of my tibia not going into my ankle right. So at 16, once the doctors could tell I was done growing (I had a LOT of tests done) they put 9 pins in one leg and 10 in the other. I was like that for 2 and a half months and then they pulled the pins out. The pins went ALL the way through the bone and out the other end. All the pins were held in place by a halo and 2 times a day I would turn a thing that slowly broke my leg (sounds like fun, huh? lol). The break of course filled in with more bone and made my leg correct. I am fine today and am VERY glad I had the operations. As painful as it was... At least now I can still walk without pain.
Peace,
Heather"
Different operations may be needed for problems at the ankles and hips.
I know nothing about these, so if anyone out there does, please write
in.
Page updated on 22/3/05 - Information checked by:
William G. Mackenzie, M.D.
Acting Chairman, Department of Orthopaedics
Pediatric Orthopaedic Surgery
Nemours/Alfred I. duPont Hospital for
Children