|
Betegség leírása |
Scleromalacia perforans
Betegség megnevezésének szinonímái:
- Anterior necrotizing scleritis without inflammation
- Scleritis anterior necrotisans gyulladás nélkül
- Necrotizing scleritis without inflammation
- Perforáló ínhártya lágyulás
- Átfúródó ínhártya lágyulás
- Scleromalacia with perforation
BNO:
Alapadatok:
- Férfi: Bármely életkorban előfordulhat
- Nő: Bármely életkorban előfordulhat
Betegség leírása:
-
A scleromalacia perforans az ínhártya elülső részének elhalása gyulladás nélkül.
Betegség lefolyása:
-
Anatómiat háttér:
A sclera (ínhártya) a corneával (szaruhártya) együtt alkotja a szemgolyó rostos burkát. Bár szöveti felépítése nagyban hasonlít a corneáéhoz, a sclera átlátszatlan, fehér színű kötőszövet, mint a neve is mutatja, az ínhoz hasonló. A rostos burok alatt foglal helyet az érhártya (choroidea), majd a belső burok, az ideghártya (retina) következik.
A betegség leírása:
A scleromalacia perforans az ínhártya elülső részének gyulladás nélküli elhalása.Tipikusan olyan idős nőbetegekben jelenik meg, akik régóta seropozitív rheumatoid arthritisben szenvednek. Tünetmentesen, nem gyulladásos szemen, fájdalom nélkül kezdődik egy sárga necrotikus (elhaló) folttal a normális ínhártyán. Később az érhártya nagy területe válik láthatóvá a sclera kötőszövetének elhalása, elvékonyodása következtében. E súlyos folyamatok ellenére a szemgolyó spontán megnyílása (perforatio) extrém ritka, kivételt képez az az eset, mikor a szemnyomás a betegséggel egyidejűleg megnő. Hatékony terápia nem áll rendelkezésre, az alapbetegség (többnyire rheumatoid arthritis) megfelelő terápiája megelőzheti vagy lassíthatja a szemtünetek kialakulását.
Megelőzési és más fontos tanácsok:
-
Az alapbetegség megfelelő terápiája megelőzheti a szemtünetek kialakulását.
Előzmények:
- Ismeretlen kórok
- Betegségek
-
Seropositiv rheumatoid arthritis
-
Egyéb rheumatoid arthritis
-
Szisztémás lupus erythematosus
-
Polyarteritis nodosa
-
Wegener granulomatosis
-
Visszatérő polychondritis
- Egyéb prediszponáló tényezők
Gyakoribb panaszok, tünetek:
- Szem és látás
-
Fénykerülés
-
Fáj az egyik szeme
-
Fájnak a szemei
-
Vörös az egyik szeme
-
Rosszul lát
Fizikális vizsgálatok:
- Szem
-
Iris rajzolata elmosódott
-
Praecipitatumok a cornea hátlapján
-
Sclera kékes színű
-
Sárgás-fehér nekrotizáló csomók a sclerán
-
Uvea előboltosul az elvékonyodott sclerán
-
Vérbőség a cornea körül
Speciális, műszeres vizsgálatok:
- Szemészeti vizsgálatok
-
Réslámpa vizsgálat
Gyakoribb szövődmények:
- Szem és függelékei
-
Szaruhártya-gyulladás
-
Iridocyclitis
-
Perforatio sclerae
-
Szövődményes szürkehályog
-
Szemgyulladás okozta másodlagos zöldhályog
-
Látóideg rendellenességek m.n.o.
-
Vakság egyik szemen
-
Csökkentlátás egyik szemen
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