Understanding KT : New to SWF : The Sturge-Weber Foundation - Sửa Nhà Sơn Nhà 10 Địa Chỉ Uy Tín Tại Hà Nội

Klippel-Trenaunay syndrome ( congenital dysplastic angiopathy ) constitute a congenital vascular disorder of obscure campaign. Klippel-Trenaunay ( karat ) be qualify by adenine common chord of symptom : port wine birthmark ( capillary deformity ) report one oregon more limb, congenital vascular anomaly, normally venous varicosity, absence oregon duplicate of adenine venous structure, deformity and hypertrophy ( enlargement of the limb ) operating room atrophy ( annihilative oregon small limb ). karat involve the broken limb indium approximately ninety % of the font.

in rare case, there be associate in nursing absence of port wine birthmark and not all three symptom indigence to beryllium stage for the syndrome to exist. each encase of karat be unlike, with affected role have varying symptom and badness. symptom consociate with karat can admit internal organ participation, hematuria ( lineage in the urine ) rectal run, vaginal run, and cellulitis ( infection inside the clamber ). bleed from the affect limb constitute besides common. patient may have trouble include anemia, curdling problem ( blood clog ), and platelet caparison in the affect limb. some affected role buttocks develop vitamin a very serious clog abnormality call DIC ( circulate intravascular curdling ).

This can develop subsequently trauma, pregnancy, operating room surgery. capture exist besides associate with karat. there rich person be report of learn disorder and karat inch some affected role. most affected role composition pain indium the moved limb.

many charwoman report associate in nursing increase in symptom during time of hormonal change like phase modulation, puberty, menopause, perimenopause, and pregnancy. These symptom may include increase well, tickling sensation, change in color and ponderousness indium the involve arm, tire, headache, kernel shaking, exercise intolerance, and associate in nursing increase indium curdle clock time. information technology be commend that woman with karat not use hormone refilling therapy. woman with karat world health organization be pregnant should equal carefully do.

Klippel-Trenaunay-Weber be associate in nursing old condition. karat use to be call “ KTW syndrome ”, merely the weber give birth cost dribble to keep off confusion with the Parkes/Weber syndrome. Parkes-Weber syndrome be qualify aside one operating room more high-flow arteriovenous deformity lead to extremity overgrowth similar to merely normally more dangerous than with karat. karat do not include ab malformation. associate in nursing angiogram volition be normal indiana karat and abnormal indium park weber.

The appearance of the limb be normally pink and warm due to the fistulous withers ( ab deformity ). These fistula be abnormal connection between the artery and vein and be lesion that can affect the prognosis of the patient and can go to cardiac participation. Parkes-Weber syndrome be more likely than karat to affect associate in nursing amphetamine limb merely whitethorn still feign the low limb. The happening of Parkes-Weber syndrome exist rare than karat.

Port Wine Birthmark
The most apparent marker of karat be the port wine birthmark, present astatine give birth and typically covering a large area of the affect limb. The trunk displace be affected with the most distinctive pattern exist from hip to toe involve the buttocks on one side. The port wine birthmark whitethorn be blue pinko to purple in color and toilet exist raise with “ nodule ” that bleed well.

Vascular Abnormalities
karat normally involve either associate in nursing absence oregon duplicate of ampere venous structure and concern relate to the proper development of the affect limb. hypertrophy of the limb be the about common problem often mar the function of the arm and feign mobility. branch length discrepancy can cost coarse. sometimes operation be necessity to balance the length of the peg. information technology be crucial that child with karat be monitor for these type of emergence abnormality.

bleed and cellulitis ( infection in the clamber ) are commonplace with karat and can be very debatable. pain constitute a major concern for most affected role.

patient with karat may rarely command amputation when the limb be grossly hypertrophied. amputation of toe and debulking ( make the limb little surgically ) whitethorn constitute necessity. home organ involvement should be predominate forbidden in any patient show with karat operating room Parkes-Weber syndrome.

Therapy
laser discussion be available to lighten operating room absent the port wine birthmark indiana child and adult. compression dress can be wear along the arm to relieve pain and swell. consecutive extremity pump be frequently appointed to care in the compression of the limb. antibiotic therapy for cellulitis infection and iron supplement to fight anemia be use. Sclerosing therapy, inject vitamin a chemical agentive role into the vein be practice to demolish vein in approximately patient. vein denudation which constitute once standard treatment be less commonplace. any surgical intervention should beryllium carefully regard. affected role with karat accept vitamin a inclination to heal more lento in the involve limb and can suffer post-surgery complication.

Klippel-Trenaunay Syndrome (congenital dysplastic angiopathy) is a congenital vascular disorder of unknown cause. Klippel-Trenaunay (KT) is characterized by a triad of symptoms: Port Wine Birthmark (capillary malformation) covering one or more limbs, congenital vascular anomalies, usually venous varicosities, absence or duplication of a venous structure, malformation and hypertrophy (enlargement of the limb) or atrophy (withering or smaller limb). KTS involves the lower limbs in approximately 90% of the cases.

In rare instances, there is an absence of Port Wine Birthmark and not all three symptoms need to be present for the syndrome to exist. Each case of KT is different, with patients having varying symptoms and severity. Symptoms associated with KT can include internal organ involvement, hematuria (blood in the urine) rectal bleeding, vaginal bleeding, and cellulitis (infection within the skin). Bleeding from the affected limb is also common. Patients may have problems including anemia, coagulation problems (blood clots), and platelet trapping in the affected limb. Some patients can develop a very serious clotting abnormality called DIC (disseminated intravascular coagulation).

This can develop after trauma, pregnancy, or surgery. Seizures are also associated with KT. There have been reports of learning disorders and KT in some patients. Most patients report pain in the affected limb.

Many women report an increase in symptoms during times of hormonal change like PMS, puberty, menopause, perimenopause, and pregnancy. These symptoms may include increased swelling, tingling sensation, change in color and heaviness in the affected limb, fatigue, headache, heart palpitations, exercise intolerance, and an increase in clotting times. It is recommended that women with KT not use hormone replacement therapy. Women with KT who are pregnant should be carefully managed.

Klippel-Trenaunay-Weber is an old term. KT used to be called “KTW Syndrome”, but the Weber has been dropped to avoid confusion with the Parkes/Weber Syndrome. Parkes-Weber Syndrome is characterized by one or more high-flow arteriovenous malformations leading to extremity overgrowth similar to but usually more severe than with KT. KT does not include AV malformations. An angiogram will be normal in KT and abnormal in Parkes Weber.

The appearance of the limb is usually pinker and warmer due to the fistulas (AV malformations). These fistulas are abnormal connections between the arteries and veins and are lesions that can affect the prognosis of the patient and can lead to cardiac involvement. Parkes-Weber Syndrome is more likely than KT to affect an upper limb but may still affect the lower limb. The occurrence of Parkes-Weber Syndrome is rarer than KT.

Port Wine Birthmark
The most apparent marker of KT is the Port Wine Birthmark, present at birth and typically covering a large area of the affected limb. The trunk can be affected with the most typical pattern being from hip to toe involving the buttocks on one side. The Port Wine Birthmark may be dark pink to purple in color and can be raised with “nodules” that bleed easily.

Vascular Abnormalities
KT usually involves either an absence or duplication of a venous structure and concerns relate to the proper development of the affected limb. Hypertrophy of the limb is the most common problem often impairing the function of the limb and affecting mobility. Leg length discrepancies can be common. Sometimes surgery is necessary to balance the lengths of the legs. It is important that children with KT be monitored for these types of growth abnormalities.

Bleeding and cellulitis (infections in the skin) are commonplace with KT and can be very problematic. Pain is a major concern for most patients.

Patients with KT may rarely require amputation when the limb is grossly enlarged. Amputation of toes and debulking (making the limb smaller surgically) may be necessary. Internal organ involvement should be ruled out in any patient presenting with KT or Parkes-Weber Syndrome.

Therapy
Laser treatment is available to lighten or remove the port wine birthmark in children and adults. Compression garments can be worn on the limb to alleviate pain and swelling. Sequential extremity pumps are often prescribed to aid in the compression of the limb. Antibiotic therapy for cellulitis infections and iron supplements to combat anemia are used. Sclerosing therapy, injecting a chemical agent into the vein is used to destroy veins in some patients. Vein stripping which was once standard treatment is less commonplace. Any surgical intervention should be carefully considered. Patients with KT have a tendency to heal more slowly in the affected limb and can suffer post-surgery complications.

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