Uyini syndrome ka Klippel-Trenaunay-Weber?

Njengoba wazi, kwasekuzalweni zize zidlulele ngofuzo kubazali noma bavele ukwephulwa of embryogenesis. Anomalies kungenziwa elakhiwe kusuka kunoma iyiphi izicubu noma sitho. Ngokuvamile ziseZwini ngokushesha emva kokuzalwa, kodwa ngezinye izikhathi ezithinta kamuva kakhulu. Isibonelo azalwa naso anomaly syndrome, Klippel-Trenaunay-Weber. Lesi sifo kubhekisela esenzakalweni esiyisimangaliso zokugula we isimiso semithambo yegazi, ithinta ikakhulukazi aphansi esitho emthanjeni.

Ngokungafani nezinye anomalies eziningi azalwa naso, Klippel-Trenaunay-Weber syndrome zingase abe Izibikezelo esihle ekuphileni. Yena ibangelwa ukwelashwa esifike ngesikhathi kuhlinzwa lesi sifo.

Pathology: syndrome, Klippel-Trenaunay-Weber - kuyini?

Lesi sifo yaziwa kusukela ekuqaleni kwekhulu lama-20, ngesikhathi kuqala echazwe ososayensi ababili - futhi Klippel Trenaunay. Kamuva, Dr. Weber ungeze wayevele ulwazi mayelana nalesi sifo. Kuyaziwa ukuthi lesi sifo has elinye igama - osteogipertrofichesky nevus emithambo yegazi.

Syndrome Klippel-Trenaunay-Weber zitholakala ngokuyinhloko phakathi kwabantu besilisa. Okwamanje, ulwazi mayelana imbangela sifo futhi lwezifo yayo akwanele. Izimpawu ezisemqoka we zokugula kukhona olunzulu "amavukuzi" (nevi) on the imilenze, ezahlukene ubukhulu aphansi esitho varicosity. Naphezu ukuthuthuka komqondo zezibonakaliso emitholampilo nobukhali izilonda, kwezinye izimo, lesi sifo asilapheki. Lokhu inzuzo kufinyelelwa sibonga ukuvela entsha emkhakheni amasu ukuhlinzwa semithambo.

Izimbangela inkinga

Njengoba isifo wayechazwa esanda kutholakala (iminyaka engu-100 edlule), okwamanje ayikho ukwaziswa okunokwethenjelwa mayelana omsakazo yayo. Noma kunjalo, kukhona eyeza nezazi zezinkanyezi ezikhuluma eziningana, owawuthi ekuthuthukiseni Klippel-Trenaunay-Weber syndrome. Izimbangela isifo elihlobene ne umlando womndeni. Kuyaqondakala ukuthi main isici etiological iyona kuguquka zofuzo. Ngokusho omunye umbono, imbangela isifo itholakala ukwephulwa of embryogenesis ekukhulelweni ekuseni. Ngaphansi kwethonya yizici zemvelo (chemical elinobuthi, ukucindezeleka psycho-ngokomzwelo, ukuchayeka emisebeni) kwenzeka aplasia uhlelo emithambo yegazi. nokungathuthuki wayo buhlobene futhi izinto ezithwala izifo. Ngokwalo mbono, imbangela sifo amagciwane noma amagciwane sibhajwe umzimba kamama ngesikhathi sokukhulelwa.

Lwasemtholampilo isithombe lesi sifo

I ukubonakaliswa yokuqala yalesi sifo Klippel-Trenaunay-Weber lubonakale kakade esikhathini ezisanda kubelethwa. Ezimweni ezingavamile, zivela kamuva - ebuntwaneni. izimpawu Classic zesifo izimpawu ezilandelayo:

1. Angioma - spider izindawo. Ngokuvamile, ukuze zitholakale ngomlenze owodwa futhi indawo enkulu. Angiomas umbala zingahluka kusuka ukukhanya onsomi amnyama. Ngaphansi kwethonya izici ezingokomzimba (friction, umthelela) kwesikhumba "birthmarks" ka zilinyazwa kalula, kukhona ukopha.
2. varicose emithanjeni ka imilenze ebusweni. ukubonakaliswa Clinical lo muntu okukhulunywa ngaye - tortuosity, ukuqina, abagulela ukufa kukhuphuke nezitsha esitho engezansi.
3. Hypertrophy we umlenze ethintekile. Ngenxa yokulimala emithanjeni ezijulile futhi ayanda yabo esitho inyuka ububanzi. Kwezinye izimo, le nqubo amathambo futhi uhlelo ivuliwe. Khona-ke umpristi umlenze ethintekile kungenzeka isikhathi eside kunaleso esitho enempilo.

Ngezinye izikhathi omunye kuyizimpawu lesi sifo hhayi wabonakaliswa noma ngaphandle (isib varicose emithanjeni olukha phezulu). Kwezinye izimo, lo zokugula iqhubeka ngokushesha, futhi ukunqotshwa ukuqhutshwa bevela emikhawulweni sesihlahla nomzimba engenhla.

ukuhlola

Umsolwa Klippel-Trenaunay-Weber syndrome kungenzeka ngu Angioma ebanzi. Lokhu uphawu livela kuqala. Kamuva lahlangana emithanjeni varicose kanye hypertrophy esitho. Isici Isici yalesi sifo - ukunqotshwa eyodwa emaceleni. Uma usola isifo eyenziwa laboratory kanye ukuhlola wezinsimbi. Okokuqala wenziwa ultrasound futhi Doppler ultrasound nasemilenzeni aphansi. Isici yalesi sifo ukuntuleka ukuhlanganisa emkhatsini ingcindezi pooling eyeqile umsebenzi inhliziyo. Lesi sici lokhu kuvezwa umugqa oqondile phakathi venography.

Trenaunay Syndrome Klippel-Weber: ukwelapha izifo

Ukuqedwa isifo kuncike degree nolokubhujiswa vein ezijulile. Nge ukuvaleka enkulu dlula wenziwa. Uma Vienna main wamshaya endaweni enkulu, isitsha esenziwe kwezitho zokwenziwa impahla. Njengoba ukwelashwa eyengeziwe isicelo sclerotherapy (izidakamizwa "Trombovar", "Wayne Fibro"), egqoke kokucindezelwa zangaphansi. Futhi sidinga lokumamukela njalo anticoagulants (imithi "Warfarin"). Kwezinye izimo, ukuhlinzwa semithambo engenziwa nge laser.