Weber-Krabbe chirwere: zvinokonzera, zviratidzo uye kurapwa mamiriro

Dzimwe nguva yokuberekwa makore chirwere navanachiremba - "Sturge-Weber-Krabbe urwere". Izvi haiwanzowaniki vachiberekwa angiomatous chirwere ziso, ganda uye pfungwa membranes. chirwere ichi rinowanikwa mune munhu imwe 100,000. Kokutanga, chirwere ichi akarondedzerwa Sturgeon muna 1879, uye muna 1922, Weber akarondedzera zviratidzo dzakaonekwa panguva X-ray.

Krabbe muna 1934 akaita kubvuma kuti kuwedzera ganda angiomas uye murwere anotambura chepfungwa angiomatosis anoshongwa. Ngatiedzei kufunga chii chirwere Sturge-Weber-Krabbe, hunokonzera, zviratidzo chirwere ichi.

Zvakakurukurwa chirwere

chirwere ichi ane vachiberekwa angiomatosis, t. E. Kunwisa yokuwedzera midziyo ropa. Zvokurwara kunokanganisa nechepakati tsinga, nhengo nechaanoona, ganda. Weber chirwere kunoratidza vachiberekwa angiomas dzakawanda pachiso nharaunda, yemaziso, pfari, retardation mupfungwa, uye nezvimwe chetsinga uye ocular zviratidzo.

Chirwere chirwere med cranial radiography, MRI kana CT chepfungwa zvivako, nokuyerwa intraocular kumanikidzwa, ophthalmoscopic bvunzo, Ultrasound meso gonioscopy. Kurapwa ine symptomatic kurapa uye antiepileptic uye yemaziso kuchinja uye panobviswa surgically. Kazhinji mashoko fungidziro ari murombo.

zvinokonzera

Weber chirwere kunoitika nokuda kutyorwa fetus, zvichiguma kukuvaradzwa maviri utachiona akaturikidzana: ectoderm uye mesoderm. Imhaka kwavari tsinga anotanga kuva pashure imwe nguva, ganda, tsinga uye nhengo dzomukati kwemwana.

Zvikonzero kukundikana uku kungava zvinotevera:

• kudhaka vakadzi nepamuviri nechikakananda, zvinodhaka, doro, mishonga siyana;
• intrauterine chirwere;
• dysmetabolic yokutyora ari amai vanepamuviri.

zviratidzo

Kana munhu akanzi Weber urwere, zviratidzo chirwere ichi chingava zvakasiyana. The chikuru kwavari - angiomatosis ganda. Izvi vascular aizonyatsovachenesa varwere zvose zviri chokuziva, uye pamusoro nguva inotanga dzichiwedzera kukura. Kakawanda iri pamatama uye pasi maziso, uye kushushikana inotanga kupera. Pakutanga, panzvimbo ine blue ruvara, ipapo anova tsvuku Cherry kana piriviri.

Angioma anogona kuchinja chitarisiko uye kuwanda uye vakaita dzakapfirirwa Homwe diki, uye imwe huru gwapa, kubatanidzwa nomumwe pamwe chete. Angiomatosis anogona kufukidza pharynx, iyo nasal mhango uye muromo. Muzviitiko zvizhinji (70%) kubva yokuwedzera midziyo ropa riri rimwe madziro, uye 40% shanduko chiso angiomas kazhinji pamwe maoko nemakumbo uye hunde. uye nezvimwe dermatologic zvingaratidza kuitika: nyoro zvinyama kuzvimba, vachiberekwa hemangiomas, nevi.

Kana paine fungidziro pakati Weber urwere, Neurology akaratidza lesion cranial tsinga, nokudaro vachikonzera injini uye kutsinga dzinouya nenyonganiso pakatarisana muviri pathological nidus.

Muna 85% nyaya muvana pasi rimwe gore vakachengeta nevoid amentia pamwe convulsive zvinetso, akaratidza zvipusha pfari Jackson mhando, umo maoko nemakumbo vari kufukidzwa nokugwinha. Pfari kazhinji kunotungamirira hwepfungwa retardation, retardation mupfungwa, mune zvimwe zviitiko, pangava chinhu fuza, hydrocephalus.

Pamusoro chikamu nhengo wezvakaonekwa zvinochengetwa angioma vascular membranes kwemaziso, hemianopsia, heterochromia yacho Iris coloboma. Mumwe pavatatu varwere nechirwere yemaziso, zvichikonzera corneal opacity.

Syndrome chirwere pamusoro Sturge-Weber

urwere zvokurwara iri panoonekwa pamwe wetsinga, ophthalmologist, uye epileptologist dermatologist.

Doctor kuongorora dzokurapa nhoroondo uye murwere zvichemo, vati zera kana zviratidzo chirwere wokutanga akazviratidza. With chetsinga kuongororwa rokuongorora aikanganiswa ganda uye tsandanyama simba muviri migumo.

radiography dehenya anoona nzvimbo calcification of cortex, vakavirikidza contours. Computed tomography uropi anogona kufungidzira imwe nzvimbo yakakura. Uchishandisa MRI padzakacherechedza migove cortex rive dete, atrophy uye degeneration pamusoro chepfungwa fuma, dzakakudzingai intracerebral bundu, chepfungwa chikofu.

Electroencephalography anosarudza ukuru uropi bioelectrical basa uye anogadza Epiactivity. Ophthalmologic ongororo iri kuedza kuona acuity, intraocular kumanikidzwa kuyerwa, kuitisa perimetry, gonioscopy, ophthalmoscopy, AB-Scan Ultrasound biometry uye maziso.

Kazhinji, murwere akatuma kubvunzana kuti vanachiremba genetics.

mabatiro

Kana Weber chirwere kunokwanisa kuonekwa, kurapwa kunofanira chete symptomatic. Izvi zvinoreva kuti anobatsira murwere kubva kuratidzwa akadaro chirwere, iyo kanganisa hwake, sezvo anobudirira mishonga iripo parizvino.

ARV kuitwa sezvinotevera:

• kubvisa kufema zviratidzo, chiremba prescribes chinokosha kugadzirira "Valproate" "Depakine", "Keppra", "carbamazepine", "Finlepsin" "Topiramate";
• kana aripo kusagadzikana mupfungwa hunhu, ushandiswe mishonga psychotropic;
• nokuti dambudziko yemaziso kushandisa ziso anodonhedza izvo kuderedza intraocular mvura secretion "Timolol", "Azopt", "Alfagan", "Dorzolamide" uye vamwe.

Kana muri dehenya mhango angioma kukanganisa kushanda kwemuviri, akaita oparesheni kubvisa navo.

mataridzikiro

Zvinosuruvarisa zvirwere zvakadai Weber urwere, ane murombo fungidziro. Zvinotevera yematambudziko anogona kuitika:

• kurasikirwa wokuratidzwa;
• vascular zvinetso, zvichiita kuti pave sitiroko;
• raidudzwa chaizvo kuremara kumeso chisingazivi anowanzoita nokusangana kupinda vanhu uye yekudyidzana lowers nokuzviremekedza;
• pfari kubatsira zvikuru mupfungwa retardation.

Chero matanho kudzivirira kukura chirwere ichi, kwete, nokuti chikonzero chaiwo hosha haina zvazivikanwa.

mhedziso

Saka, Weber chirwere - nengozi vachiberekwa chirwere. Vana vacheche Pakutanga kuva anowanzoita, asi pakupera kwegore upenyu chokutanga pane zvipusha pfari. Kazhinji iri lagging shure kukura maoko nemakumbo, uye pashure penguva yakati pane pfungwa retardation. chirwere ichi hapangapori, anogona chete kutora mishonga kuti kudzivirira husina utano.