Yintoni na syndrome ka Klippel-Trenaunay-Weber?

Njengoko usazi, kwasekuzalweni zidluliselwa zemfuza esuka kubazali okanye ukusuka nokuhluthwa embryogenesis kuvuka. Mgaqweni enokuveliswa naliphi na inyama okanye umbutho. Amaninzi zifumaneka ngoko nangoko emva kokuzalwa, kodwa ngamanye amaxesha azibonakalisa kakhulu kamva. Umzekelo i anomaly naso syndrome, Klippel-Trenaunay-Weber. Isifo libhekisela ngezifo kwenkqubo semithambo, kuchaphazela ikakhulu lomzimba vein asezantsi.

Ngokungafaniyo nezinye mgaqweni ezininzi enaso, Klippel-Trenaunay-Weber syndrome unokuba ALS kakuhle ubomi. Yena siyifumana unyango ngexesha zoqhaqho esi sifo.

Pathology: syndrome, Klippel-Trenaunay-Weber - yintoni na?

Esi sifo iye kwaziwa ukususela kwinkulungwane yama-20, xa echazwe ekuqaleni zizazinzulu ezimbini - kunye Klippel Trenaunay. Kamva, uGqr Weber wongezelela kakade ulwazi malunga nesi sifo. Yinto eyaziwayo ukuba izifo inomnye igama - osteogipertrofichesky nevus lwemithambo.

Syndrome Klippel-Trenaunay-Weber wafumana kakhulu phakathi kwabantu abangamadoda. Okwangoku, ulwazi malunga ingcambu unobangela izifo kunye lwezifo yayo akwanelanga. Iimpawu eziphambili zale ngezifo ezinkulu "abamenzelayo" (nevi) apha emilenzeni, ezahlukeneyo ubukhulu asezantsi lomzimba varicosity. Nangona uphuhliso yokuqala imiqondiso zonyango nobukhali emathunjini, kwezinye iimeko, syndrome ngcono. Le iadvanteji iya kuphunyezwa enkosi ukuvela omtsha kummandla ubuchule uqhaqho yegazi.

Ezibangela ingxaki

Ekubeni esisifo echazwe ngokwentelekiso kutshanje (malunga neminyaka eli-100 eyadlulayo), akukho okwangoku akukho inkcazelo enokuthenjwa umyalezo wayo. Noko ke, kukho iingcamango ezininzi, ngokoko apho asakhasayo Klippel-Trenaunay-Weber syndrome. Izinto ezibangela izifo ezinxulumene ngembali yosapho. Kuyaqondakala ukuba into ephambili etiological ke wezakhi sofuzo. Ngokutsho kwenye inkolelo, unobangela esi sifo ilele ekugxojweni embryogenesis ekukhulelweni ekuqaleni. Phantsi ngempembelelo yokusingqongileyo (imichiza Ityhefu, uxinezeleko yengqondo-ngokweemvakalelo, sesichengeni sosasazeko lwemitha) yenzeka inkqubo aplasia lwemithambo. baswele walo kwakhona ezinxulumene izifo. Ngokwalo mbono, unobangela wezifo iintsholongwane okanye iintsholongwane labanjwa emzimbeni kamama ngethuba lokukhulelwa.

Lo mfanekiso kwezonyango sifo

Wabonakala lokuqala syndrome Klippel-Trenaunay-Weber sele olubonakalayo kwixesha lweentsana. Kwiimeko ezinqabe kakhulu, zivela kamva - ebuntwaneni. Iimpawu Classic kwesi sifo ezi mpawu zilandelayo:

1. amabala spider - Angioma. Amaninzi, ukuba ziyafumaneka kwi ngomlenze omnye, wagubungela indawo enkulu. Angiomas umbala ingahluka ukukhanya ukuya emfusa ngebala. Phantsi yempembelelo yezinto eziphathekayo (ukukhuhlana, impembelelo) ulusu kwi 'azalwa' ka lula, owophayo apho.
2. ngemithambo Varicose ye imilenze komhlaba. obubonakala kwezonyango sinalento - tortuosity, ukuqina, ukwanda nokudodobala kunye neempahla mlenze asezantsi.
3. Hypertrophy yomlenze achaphazelekayo. Ngenxa konzakale imithambo nzulu kunye nomlenze proliferation ukunyuka zabo ubukhulu. Kwezinye iimeko, inkqubo amathambo kunye nenkqubo ivuliwe. Emva koko ke umlenze abachaphazelekayo ukuze ibe ngaphezu kwesebe esempilweni.

Maxa wambi omnye linophawu esi sifo kodwa wabonakalaliswa okanye ngaphandle (umz, imithambo varicose ozenzisayo). Kwezinye iimeko, le ngezifo iqhubeka ngokukhawuleza, kwaye ngokoyiswa imali kwiziphelo ukuya komboko nomzimba ongasentla.

ekuxilongweni

Usola Klippel-Trenaunay-Weber syndrome kunokwenzeka yi Angioma olubanzi. Le uphawu elivela kuqala. Kamva wangena efaka varicose kunye hypertrophy alo mlenze. Olona phawu uphawu kwesi sifo - ukoyiswa elinye macala. Ukuba urhanela isifo wenziwe elebhu kunye uxilongo zomculo. Okokuqala wenza ultrasound kunye Doppler ultrasound imilenze asezantsi. A phawu yale syndrome kukunqongophala unxulumano olukhoyo phakathi kwamandla egesi lwemithambo kunye nomsebenzi intliziyo. Olu phawu lubonakala ngelayini ngqo ngexesha venography.

Trenaunay Syndrome Klippel-Weber: unyango lwezifo

Ukuphelisa esi sifo kuxhomekeke iqondo ukutshatyalaliswa ndlela enzonzobila. Ekubeni ukuvala ebalulekileyo ukudlula luyenziwa. Ukuba Vienna eziphambili wambetha indawo enkulu, isitya wenza zokwenziwa izinto abohlukeneyo. Njengoko unyango ezongezelelweyo isicelo sclerotherapy (iziyobisi "Trombovar", "UWayne Fibro"), enxibe ucinezelo sangaphantsi. Kwakhona kufuna wondamkela rhoqo anticoagulants (amayeza "Warfarin"). Kwezinye iimeko, utyando nemithambo inokwenziwa nge laser.