klippel - trenaunay syndrome (kts) when and what to do? · 1) spencer l, quarmby jw....
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Klippel - Trenaunay Syndrome(KTS)
When and What to do?
Dr. Ayhan ŞENOL
SBU.Gazi Yasargil ETH.
Diyarbakır /TURKEY
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Disclosure
Speaker name:
Ayhan ŞENOL
I have the following potential conflicts of interest to report:
Consulting
Employment in industry
Stockholder of a healthcare company
Owner of a healthcare company
Other(s)
x I do not have any potential conflict of interest
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Klippel - Trenaunay Syndrome(KTS)
In 1900, Klippel and Trenaunay first describedcapillary hemangiomas
in one or more extremities as a congenitalsyndrome
characterized by varicose veins, soft tissueand bone hypertrophy
[1]
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Klippel - Trenaunay Syndrome(KTS)
The incidence of this syndrome is equal in menand women.
Whether it is hereditary transition is unknown
BUT
Autosomal dominant inheritance was observed in some cases. [2]
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KTS
Most, if not all, cases of KTS are caused bysomatic mutations in the PIK3CA gene.
PIK3CA-related
overgrowth spectrum (PROS)
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KTS
Capillary malformations 98%
Varicose veins –venous malformation 72% 63%
Hypertrophy 67%
[3]
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.
symptoms
Capillary malformation + + + +
Varicose vein + + - +
Hypertrophy + + ± ±
Popliteal veinAgenesis-Anomaly
+ +Aneurysmal
dilatation
+Agenesis
-
Bone malformation + - - -
Port wine stain + + + +
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Capillary malformations
This is the most common
cutaneous manifestation of KTS.
Typically, capillary malformations
involve the enlarged limb.
It is seen 98% of patients.
Hypertrophy, hemorrhage, and
color changes in the extremities are
often the first conspicuous in patients
due to venous malformation and
varicosities at the skin.
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Varicose veins-Venousmalformations
Can occur in both the superficial and
deep venous systems.
Superficial venous abnormalities :
ectasia of small veins or large venous
malformations.
Deep venous abnormalities
aneurysmal dilatation, aplasia,
hypoplasia, duplications, and venous
incompetence.
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Hypertrophy
Enlargement of the extremity:
may be caused by bone abnormality,
circumferential soft-tissue
Hypertrophy or
lymphatic malformation.
Extremity growth usually stops at the end of the Adeloson period.
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Port - wine stain
port-wine stain
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KTS
,
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22 y.o female
Popliteal vein agenesis
Minimal hypertrophy
Port-wine stain
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55 y.o male
Port wine stain and
multiple varicose veins
on right leg.
Aneurysms were
detected in the popliteal
and femoral vein.
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KTS Treatment
How can we do for this patients
And
What is the purpose of treatment in KTS
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KTS Treatment
There is no cure for KTS
Treatment is symptomatic and supportive.
Reduce symptoms and risk of complications.
Conservative treatments most effective for undesired side effects.
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What can we do
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What to do?
-Elastic Socks
conservatively
• Protects the extremity from trauma
• Used to reduce the effects of venousinsufficiency.
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What to do?
-Surgery
-Ligation and Stripping
• a small incision to prevent pooling of blood.• Ligation and striping can be used for removal of the vein.
• should be known that deep veins are normal
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What to do?
Ambulatory phlebectomy
should be known that deep veins are
normal
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What to do?
Sclerotherapy
Sclerotherapy can eliminate the pain
and discomfort of varicose veins
can prevent complications such as
venous hemorrhage and ulceration
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What to do?
Sclerotherapy
Foaming agent pushes blood in the vein
so the sclerosing agent will have better contact with
the vein wall.
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What to do?-Sclerotherapy
Several sclerotherapytreatments usually arerequired to achieve the
desired results.
under ultrasoundguidance
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What can we do
And sometimes we can't do anything
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What to do?
Sometime
Nothing
no right leg
complaints
and happy
Loves his right leg more than his left.
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KTS
References1) Spencer L, Quarmby JW. Klippel-Trenaunay Syndrome and left iliac vein agenesis EJVES Extra 2007;13:50-1.
2) Whelan AJ, Watson MS, Porter FD, Steiner RD. Klippel- Trenaunay-Weber syndrome associated with a 5:11 balanced translocation. Am JMed Genet 1995;59:492-4.
3) Jacob AG, Driscoll DJ, Shaughnessy WJ, Stanson AW, Clay RP, Gloviczki P. Klippel-Trénaunay syndrome: spectrum and management.Mayo Clin Proc 1998;73:28-36.
4) Cherry KJ, Gloviczki P, Stanson AW. Persistent sciatic vein: Diagnosis and treatment of a rare condition. J Vasc Surg 1996;23:490-7.
5) Collins PS, Villavicencio JL, Abreu SH , et al. Abnormalities of lymphatic drainage in lower extremities: a lymphoscintigraphic study. J VascSurg 1989;9:145-52
6) Gloviczki P, Hollier LH, Telander RL, et al. Surgical implications of Klippel Trenaunay syndrome, Ann Surg 1983; 197: 353-62.)
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Thanks…
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,
Can it be seen in the upper extremity
Limited number of cases have been reported in the literature
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Klippel - Trenaunay Syndrome(KTS)
When and What to do?
Dr. Ayhan ŞENOL
SBU.Gazi Yasargil ETH.
Diyarbakır /TURKEY