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An 18mth old child was recently brought into the clinic with Klippel Trenaunay Weber Syndrome.
The internet provided a wealth of information, but limited resources for podiatry.
This child has a LLD of 1cm, and a shoe size varience secondary to the hypertrophy. She has started tiptoeing on the shorter leg, and excessively pronates and abducts on the long leg.
The paediatrician has indicated to the parents that no treatment is required until puberty when the epiphyseal plates can be interupted.
How much intervention can we provide in the interim with regards to her gait? She has demonstrated pain on occasion when mobilising as disclosed by her parents.
I am intrigued by this syndrome. Has anyone else come across it before?
Cheers
Tree.
Hi guys, just an addit to the above.
Whilst a LLD is within my ability to treat without concern, I am wondering if the leg discrepancy will fluctuate with this syndrome. Will it always maintain a consistant discrepancy, or will the assymetry at times be minimal? The literature I am accessing doesn't address this clearly.
It does not mention whether there can be a fluctuation in LLD, but as she is still very young, I would expect that as she grows it is likely for growth rate of the limbs to be different so there is a chance of it changing.
My response would be to address it as 1cm now, and reassess 6 monthly.
As I said, I havent experienced a patient with KTMS before,this is just the line of approach I would take.
Good luck, keep us posted
Sally
Last edited by MelbPod : 25th May 2009 at 03:55 PM.
As in many infrequent syndromes, there are a lot of unknown factors in reference to the disorder evolution, mainly when affects to child.
I've only visited one case of KTWS, and I think that we must take in account a couple of things. One of them is the exquisite relationship that the parents need and deserve, due to the syndrome may produce familiar anxiety. I recommend avoid initially take pictures and other forms to report case, until confidence among parents-practitioner be great.
The 8 year old boy that I seen, had all characteristics of the syndrome, but in comparison with other rare syndromes, he has his capacities (cognitive-physical-relation) undamaged, and pain free. Obviously, LLD was the main evidence to be treated. In our case he uses an orthotic with a whole plantar compensation, to avoid an equinus.
As one lower limb in bigger than other, he needs to use a larger shoe size. They can purchase two pair of footwear, or we can improve the shoe fitting introducing a simple plantar plate in normal size foot.
I would like to tell you many things more, but the therapeutic strategies depends on the evolution (soft tissues), growth, etc.
The best way to take care for these child is by a good follow-up and using a common sense.
Nothing can be compared to a smiling child.
best regards
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I haven't, however am treating a 2 year old with Syndactyly at present. She has one foot, 2 sizes smaller, 4 toes and 3 mets. That leg is also already 1 cm shorter. They are already planning lengthening procedures to start at 6. I think it depends a lot on the syndrome and experience of those doing the procedure as to what they advise. In the meantime we are just making sure appropriate footwear and heel raises/lifts are used.
__________________
Cheers,
Cylie.... in a permanent state of confusion
I have a patient with KTWS who I have been treating since the age of 13 years. He is now a 19 year old university student. He was originally referred from his family doctor for biomechanical exam and foot orthoses to assist in treatment of patellofemoral pain syndrome, which settled long ago. He had, by the age of 13, had 3 epiphysiodeses to left leg in an attempt to retard limb growth on that side.
Vascularly he displays extensive varicosities and hemangioma in the affected leg. There is extensive soft tissue and osseous hypertrophy on the left side which has created significant circumferential differences in size and shape between lower limbs.
Biomechanical features include hypermobility in the joints of both feet and hands (not sure if this is idiopathic, familial or a feature of KTWS). A flexible planovalgus foot deformity is demonstrated in both feet in static stance and gait. There is a limb length deficit on the non-affected right side of approximately 10 mm. However, this is not at this stage clinically significant. This young man is 6'4" tall and as a percentage of limb length 10 mm is not producing the compensations you would expect to see in the 18 month old child previously described in this thread.
I have provided foot orthoses with a full length raise tapering from 10mm in the heel to 4 mm in the forefoot. These have been replaced over the past 6 years as required through growth and are worn at all times. This young man leads a full and active life participating in basketball at a competitive level without current injuries.
Hope this provides some useful insight into how your somewhat younger patient may develop!
Barry Hawes
Dip, BSc, MSc (Pod)
The Following User Says Thank You to barry hawes For This Useful Post:
An 18mth old child was recently brought into the clinic with Klippel Trenaunay Weber Syndrome.
The internet provided a wealth of information, but limited resources for podiatry.
This child has a LLD of 1cm, and a shoe size varience secondary to the hypertrophy. She has started tiptoeing on the shorter leg, and excessively pronates and abducts on the long leg.
The paediatrician has indicated to the parents that no treatment is required until puberty when the epiphyseal plates can be interupted.
How much intervention can we provide in the interim with regards to her gait? She has demonstrated pain on occasion when mobilising as disclosed by her parents.
I am intrigued by this syndrome. Has anyone else come across it before?
Cheers
Tree.
One thing that really annoys me about doctors is that they are so quick to ensure parents that no treatment is needed when it comes to limb length discrepancies. Obviously this is a rare and complex case but surely the fact that the child is already quite obviously compensating for the limb length difference would lead you to do something about it? As Barry pointed out, a limb length difference of a centimeter for someone of her size is proportionately huge.
Klippel-trenaunay syndrome: a case report.
[Article in English, French]
Ciss AG, Aïdara C, Beye SA, Diarra O, Dieng PA, Ba P, N'diaye A, N'diaye M.
Mali Med. 2009;24(2):65-67.
Quote:
Summary Objectives The purpose of this study is to illustrate a rare vascular malformation and to make a literature review.
Patient Klippel-Trenaunay's syndrome is a congenital disorder which characterized by capillary malformation, varicosities and bony or soft tissue hypertrophy. This disease is subject to significant morbidity like bleeding, deep vein thrombosis, embolic complications and in some cases enlargement of limb that may require amputation. Our case study was a young woman of twenty years suffering since birth, from a painful, heaviness and enlargered left lower limb that reached 2, 5 cm. The distal hypertrophy at the big toe which the site of a botriomycoma (telengectasic granuloma). The inner side of the thigh was also the site of small port-wine stains and varicose of the great saphenous vein. Vascular Doppler ultrasound, skeletal computed tomography and angio-MRI of the limb indicated a klippel-Trenaunay disease. A surgical abstention was decided. The patient underwent only to resection of the toe's botriomycoma followed by an elastic contention associated with ôa heel pad compensationö and an anti platelet therapy to prevent deep vein thrombosis and embolic complications.
Conclusion The syndrome of Klippel-Trenaunay is a rare vascular malformation. The basic pathology can not be corrected. The treatment's aims are to control varicose veins, to prevent complications and to preserve aesthetic and functional prognosis of the leg.
Klippel Tenaunay Weber Syndrome
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