Waa maxay syndrome ee Klippel-Trenaunay-Weber?

Sida aad ogtahay, cilladaha lagu dhasho ayaa la iskugu gudbiyaa hiddo waalidka ama ka xadgudub ee embryogenesis kac. Cillado la ahbaa karaa unugyada kasta ama xubin. Inta badan waa la helay isla markiiba dhalashada ka dib, laakiin mararka qaarkood isu muujin badan ka dib. Tusaale ahaan waa cillad-dhasho syndrome, Klippel-Trenaunay-Weber. Cudurku wuxuu loola jeedaa cudurada ka mid ah habka vascular, waxay saamaysaa inta badan ee xididka addinka hoose.

Marka la barbardhigo cillado kale oo badan oo lagu dhasho, Klippel-Trenaunay-Weber syndrome ayaa laga yaabaa in saadaal wanaagsan u ah nolosha. Wuxuu waxaa lagu gaarayaa by daaweynta qalliinka waqtiga cudurka.

Pathology: syndrome, Klippel-Trenaunay-Weber - waa maxay?

Cudurku ayaa loo yaqaan tan iyo qarnigii 20aad, markii ugu horeysay waxaa la tilmaamay by labada saynisyahano - iyo Klippel Trenaunay. Later, Dr. Weber haatan wax war ah oo ku saabsan cudurka this. Waxaa la og yahay in cudurka uu leeyahay magac kale - nevus arooriyaha osteogipertrofichesky.

Syndrome Klippel-Trenaunay-Weber dadka lab helay inta badan. Waqtigan xaadirka ah, macluumaad ku saabsan sababta asalka u ah cudurka iyo in saf kuma filna. Calaamadaha ugu muhiimsan ee cudurada waa ballaaran "jiirarka" (nevi) on lugaha, dhexroor hoose varicosity kala duwan Addinka. Inkastoo horumarinta hore ee calaamadaha lagu daawayn iyo adkaanta lesions, xaaladaha qaarkood, syndrome waa la daaweyn karaa. Faa'iidada Taana waxaa lagu gaarayaa mahad ah ee cusub oo duurka ku ah farsamooyinka qaliin vascular.

Sababaha dhibaatada

Tan iyo markii cudurka lagu tilmaamay xad dhawaan (oo ku saabsan 100 sano ka hor), hada ma jirto macluumaad lagu kalsoonaan karo oo ku saabsan in ay etiology. Si kastaba ha ahaatee, waxaa jira dhowr aragtiyood, sida laga soo xigtay oo soo koraya Klippel-Trenaunay-Weber syndrome. Sababaha cudurka la xidhiidha taariikhda qoyska. Waxaa la fahamsan yahay in factor ugu weyn etiological waa isbedel hiddo. Sida ay aragti kale, sababta oo ah cudur ka been xadgudub ku ah ee embryogenesis hore ee uurka. Under saameynta ay deegaanka (sumowga kiimikada, stress psycho-niyadeed, gaadhista radiation) dhacdaa nidaam arooriyaha aplasia. Its horumar waxa sidoo kale la wakiilada faafa. Sida laga soo xigtay aragtida this, sababta oo ah cudur waa bakteeriya ama fayras xanniban ee jirka hooyada inta ay uurka leedahay.

Sawirka caafimaad ee cudurka

The daliilka ugu horeeya ee syndrome ee Klippel-Trenaunay-Weber hore u dareemi in muddada dhalaanka. Marar dhif ah, waxay ka dib u muuqdaan - in carruurnimada. calaamadaha Classic cudurka yihiin calaamadaha soo socda:

1. baro sida xuubcaaro - Angioma. Inta badan, waxay waxaa laga heli karaa hal lug oo ay ku daboolaan aag aad u ballaaran. midabka angiomas kala duwanaan karaan iftiin si mugdi guduud. Under saameynta ay arrimo jirka (khilaaf, saamaynta) maqaarka ee "birthmarks" ee si fudud u burburay, waxaa dhiig.
2. Xidido Dhiig oo ka mid ah lugaha dusha sare. daliilka Clinical of this qof - tortuosity, dhagid, cudurrada oo kordhay iyo weelashii addinka hoose.
3. Hypertrophy ee lugta ay dhibaatadu saameysey. Sababo la xiriira dhaawac ee xididdada u qoto dheer oo soo batay addinka korodhka ay dhexroor. Xaaladaha qaarkood, geedi socodka ka mid ah lafaha iyo nidaamka on yahay. Markaas lugta ay dhibaatadu saameysey waxaa laga yaabaa in muddo ka badan addinka caafimaad.

Mararka qaarkood mid ka mid ah calaamadaha cudurka aan la muujiyey ama dibadda (tus, xididdada maqaar saar qaradhku). Xaaladaha qaarkood, cudurada ka si degdeg ah uga sii darayo, iyo guuldaradii ee laga jirka u jirridda iyo jidhka sare.

Baadhista xanuunada

Tuhmane Klippel-Trenaunay-Weber syndrome waa suurto gal by Angioma ballaaran. Cudurkaan ugu horeysay u muuqataa. Later biiray by xididdada qaradhku iyo hypertrophy ee addinka. feature The sifo cudurka this - guuldarro hal dhinac. Haddii aad ka shakisan tahay cudur qaadeen sheybaar iyo Baadhista xarkaha. First sameeyaa ultrasound ultrasound iyo Doppler ah addimada hoose. feature A of this syndrome waa la'aanta ah ee xiriir u dhaxeeya cadaadiska arooriyaha iyo dhaqdhaqaaqa wadnaha. dabeecad Tan waxaa ka muuqataa by xariiq toosan inta lagu guda jiro venography.

Trenaunay Syndrome Klippel-Weber, daawaynta cudurada

Tirtiridda cudurka ku xiran tahay heerka uu burbur xididka qoto dheer. Iyada oo cidhiidhi weyn oo isku tallaalidda waxaa la sameeyaa. Haddii Vienna ugu weyn ee ku dhuftay aag aad u ballaaran, markabka sameeyey wax isku dardaro badalida. Sida daaweyn dheeraad ah ka dalbatay sclerotherapy (daawooyinka "Trombovar", "Wayne Fibro"), xidhashada nigis riixo. Sidoo kale waxay u baahan tahay soo dhaweynta joogta ah daawada xinjirowga (daawo "Warfarin"). Xaaladaha qaarkood, qaliin vascular lagu samayn karaa iyadoo laser ah.