Weber-Krabbe lefu: bakang, matšoao le litšobotsi kalafo

Ka linako tse ling maru tsoalo ke bolwetse ke lingaka - "Sturge-Weber-Krabbe syndrome,". Ena ke ka seoelo congenital angiomatous lefu leihlo, letlalo le boko lera la. lefu o hlahellang teng motho a le mong ka 100,000. Ka lekhetlo la pele, lefu lena le 'nile oa hlalosoa Sturgeon ka 1879,' me ka 1922, Weber a hlalosa matšoao a lemoha nakong X-ray.

Krabbe ka 1934 o ile a etsa khopolo ea hore, ho phaella ho angiomas letlalo le mamello le bothata ba ho likhetla bokong angiomatosis. A re ke re leka ho utloisisa hore na ke eng ke lefu Sturge-Weber-Krabbe, bakang, matšoao a lefu lena.

Features ya lefu lena

syndrome, ena ke congenital angiomatosis, 't. E. feteletseng keketseho ea methapo ea mali. Bolwetse ama tsamaiso ea bohareng tšohile, makala a pono, letlalo. Weber lefu bontša angiomas ngata congenital sebakeng sefahleho, glaucoma, sethoathoa, kholofalo kelellong le matšoao a tse ling tse methapo ea kutlo le ocular.

Fumanoa lefu ka tsela radiography cranial, MRI kapa CT dibopeho bokong, tekanyo ea khatello ea intraocular, litlhahlobo ophthalmoscopic, oa ultrasound mahlo gonioscopy. The kalafo e na le phekolo bontshang matshwao le antiepileptic le glaucoma mekhoa e tloaetsoeng le e felisoa ngaka e ka. Maemong a mangata ho itse'ng ke mafutsana.

lisosa tsa

Weber syndrome, le hlaha ka lebaka la ho tlōla ho hōla fetal, sephetho le ka senyeha tse peli le dikarolo kokoana-hloko: ectoderm le mesoderm. Ke ka lebaka la bona le tsamaiso ea methapo e qala ho ntshetsa pele ka mor'a nako e itseng, ka letlalo, methapo ea mali le litho tse ka hare tsa lesea.

Mabaka a ho hloleha ena ka 'na ba tse latelang:

• botahoa ea bakhachane le bokoali, lithethefatsi, joala, lithethefatsi tse fapa-fapaneng;
• intrauterine tshwaetso;
• ditlolo dysmetabolic ka 'mè oa moimana.

le matšoao

Ha motho a fumanoe lefu Weber, matšoao a lefu lena ka 'na ba e fapaneng. Tse khōlō tsa bona - angiomatosis letlalo. Sena letheba vascular bakeng sa bakuli bohle ba se tlhaho, le ka mor'a nako e qala ho eketseha ka seemo. Hangata ka ho fetisisa sebakeng marameng le ka pel'a mahlo a ka, 'me le khatello ea qala ho fela. Tšimolohong, hang-hang o 'mala o pinki, ka nako eo e ba tse khubelu ciliegia kapa khubelu khanyang.

Angioma ka fapana ponahalo le ata 'me ba e le lipokothong tse nyenyane li ile tsa phasalla,' me e mong letheba kgolo, kopanngwe hammoho. Angiomatosis khona ho koahela pharynx, ho nasal cavity le molomo. Maemong a mangata (70%) tsa keketseho ea methapo ea mali ke e 'ngoe-ka lehlakoreng,' me a fetola 40% ka angiomas sefahleho atisa ho kopantswe le maoto le matsoho 'me kutu. le tse ling tse dermatologic matšoao a ka hlahang: bonolo dinama tse nyenyane tse ho ruruha, congenital hemangiomas, nevi.

Haeba ho na le ke belaelloa ka hore lefu Weber, Neurology bonahatsa lethopa cranial methapo, ka tsela eo ho etsa hore dilenaneo le meferefere kutlo ka lehlakoreng le leng la 'mele pathological nidus.

Ka 85% ea linyeoe ka bana ba ka tlaase selemo se le seng a hlokomela nevoid amentia le maloetse a convulsive, o ile a bontša tsoeroe ke sethoathoa sethoathoa Jackson mofuta, e leng nakong eo maoto le matsoho a koahetsoe ke tsitsipana. Sethoathoa hangata e isang kholofalo kelellong, kholofalo kelellong, maemong a mang, ho ka ba le sephoqo, hydrocephalus.

Ka lehlakoreng la makala a Pono ke lemohiloeng angioma vascular lera la mahlo, hemianopsia, heterochromia ea iris e coloboma. E mong-ea boraro ea bakuli na le boloetse ba glaucoma, a etsa hore corneal opacity.

Syndrome hlahloba ya Sturge-Weber

lefu bolwetse ena e fumanoa hammoho boloetse ba methapo, ophthalmologist, le epileptologist dermatologist.

Ngaka hlahloba histori bongaka le ditletlebo ba mokuli, o ile a re ho lilemo tse ha matšoao pele ya lefu lena le hlaha. Le ho hlahloba ka methapo ea kutlo hlahloba nahanela ba letlalo le mesifa matla 'mele lipheletsong.

Lehata radiography e iphumanela ho libakeng tseo ho calcification ya cortex, ka contour habeli. Ea computed tomography ea boko ha e khone ho bona ka leihlo la ka haholo kholoanyane area. Sebelisa MRI lemoha likarolo cortex thinning, felloa ke matla le degeneration tsa ntho bokong, ho sa kenyeletswe intracerebral hlahala, cyst bokong.

Electroencephalography rerang tekanyo ea boko bioelectrical mosebetsi le beha Epiactivity. Ophthalmologic tlhlahlobo ke hore a leke bonwang acuity, litekanyo intraocular khatello, khanna perimetry, gonioscopy, ophthalmoscopy, AB-scan ninemanga.com oa ultrasound biometry le mahlo.

hangata ha ho, ba le mamello e romeletsa hore a buisane le liphatsa tsa lefutso tsa bongaka.

kalafo

Haeba lefu Weber e fumanoa, kalafo lokela ho ba bontshang matshwao feela. Sena se bolela hore lopollang mokuli tloha liponahatso tse joalo tsa lefu, e leng tsa fokodisa bophelo ba hae, e le kalafo e atlehang jwale teng.

Phekolo e khanna ka tsela e latelang:

• ho felisa matšoao a apnea, ngaka alakisi litokisetso khethehileng "Valproate" "Depakine", "Keppra", "carbamazepine", "Finlepsin" "Topiramate";
• haeba lefu la ea hona joale ea lintho tsa tlhaho kelellong le maikutlong, e beuweng lithethefatsi psychotropic;
• bakeng sa kalafo ya glaucoma sebelisa leihlo marotholi eo fokotsa intraocular mokelikeli secretion "Timolol", "Azopt", "Alfagan", "Dorzolamide" le ba bang.

Haeba u ka lehata cavity angioma teba ama ea ka tšebetso ea 'mele, o ile a etsa opereishene ea ho tlosa bona.

pono

Ka masoabi, mafu a kang lefu Weber, o na le itse'ng mafutsana. The mathata a latelang a ka hlaha:

• ke tahlehelo ea pono;
• mafu a vascular, ea isang ho ho hōla ha leqeba;
• lebisitsoeng haholo sekoli ka sefahleho, e leng ha e ka tsela e tloaelehileng kenyeletsa sechabeng le haholo lowers intša ho lekana ka maemo;
• sethoathoa tlatsetsa haholo kholofalo kelellong.

mehato efe kapa efe e thibelang ho thibela ntshetsopele ya lefu lena, ha ho na, hobane sesosa e tobileng ea lefu lena le ha e e-s'o ka hlaloswang.

bofello

Kahoo, Weber lefu - se kotsi congenital lefu. masea a sa tsoa tsoaloa qalong ntshetsa pele ka tloaelo, empa qetellong ea selemo sa pele sa bophelo ho na le tsoeroe ke sethoathoa sethoathoa. Hangata e se lagging ka mor'a ka ho hōla ha maoto le matsoho, 'me ka mor'a nakoana ho na le kholofalo kelellong. lefu lena ke le sa phekoleheng, ka feela noa meriana hore Thibela bo ntse bo mpefala bophelo.