
bonat-datume-blanck syndrome

bonat-datume-blanck syndrome, jise viburn-messon syndrome key roop mein bhee jaanaa jaataa haye, ack durlabh janmajaat vikaar haye joe mastishk, retina yaa chehare key nevi kii dhamani vikritiyon kii visheshta haye. syndrome mein kaee sambhaavit lakshan hotey hain aur shayed hii kabhi, twacha, haddiyon, gurde, maanspeshiyon aur gastroentestinal tract koo prabhaavit curr sakate hain. jaba syndrome mastishk koo prabhaavit kartaa haye, tou loga dhamani-sambandhi shunt key kaaran honey vaale teevra yaa puraane ischemia key kaaran gambhir sirdard, daure, teevra aghaat, meningism aur pragatisheel tantrika sambandhi kamee kaa anubhav curr sakate hain.
retina mein, syndrome retinocephalic samvahani vikritiyon kaa kaaran banata haye joe intracranial raktasraav key saath maujood hotey hain aur isase drishya teekshnata, proptosis, pupillary dosh, optic atrophy, bulber conjunctiva kii bheed aur drishya kshetra dosh mein kamee aatee haye. drishtipatal key ghaav ektarafa aur jatil hoe sakate hain, aur lakshan jeevan key dusare aur teesare dashakon mein dikhaai dene lagate hain.
syndrome twacha key ghaavon key saath, yaa twacha key saath alag banaavat, motai aur rung key saath, aamtaur parr chehare parr upasthit hoe sakata haye. syndrome key kaaran chehare kii visheshtaen twacha key halke rung badalane seey laker vyaapak nevi aur angioma taka bhinn hoti hain. kuchh mamalon mein, frontal aur maxillary sinus bhee prabhaavit hoe sakate hain.
2012 taka bonnet-decam-blanck syndrome key rogiyon key kewal 52 mamle saamane aae hain. bachchon mein lakshan shayed hii kabhi dekhe jaate hain aur syndrome kaa nidan aksar bachpan key anta yaa praarambhik vayaskata mein kiya jaataa haye jaba drishti haani dekhi jaati haye. fluoresin angiography kaa upayog aamtaur parr syndrome kaa nidan karane key liye kiya jaataa haye.
bonat-decham-blanck syndrome key rogiyon key ilaaj mein kaee tareeke hain. haalaanki, ishtatam upchaar anishchit haye. intracranial ghaavon vaale rogiyon kaa shalya chikitsa hastakshep seey ilaaj kiya gayaa haye aur kuchh mamalon mein, yeh prakriya safal rahee haye. anya upchaaron mein embolisation, vikiran chikitsa aur nirantar avalokan shamil hain.
bonnet-decam-blanck syndrome parr seemit shodh key saath, shodhkartaon ney iss durlabh aur gair-viraasat syndrome koo prabandhit karane key bajaay naidaanik aur radiological nishkarshon parr dhyaan kendrit kiya haye.
sanket aur lakshan.
aam taur parr bachpan key anta yaa baad mein nidan naheen kiya gayaa, bonat-decam-blanck syndrome aamtaur parr kendriya tantrika tantra kii visheshtaon (madhya mastishk), netra visheshtaon (retina) aur chehare kii visheshtaon key sanyojan key saath khud koo prastut kartaa haye. syndrome key ghatakon kii abhivyakti kii degree naidaanik aur sanrachanaatmak donon roop seey bhinn hoti haye. samanya lakshan joe nidan kii or leey jaate hain way hain sirdard, poorv-kakshiya dard aur hemianopia.
bonat-decam-blanck syndrome kii netra sambandhi visheshtaen retina arteriovenous vikriti (aeye. vee. aim. aiss.) key roop mein hoti hain. aeye. vee. aim. kii teen shreniyaan unaki gambhirta key aadhar parr hain. pahali shreni mein rogi key chhote ghaav hotey hain joe aamtaur parr lakshanheen hotey hain. doosari shreni, pahali kii tulanaa mein adhik gambhir, taba hoti haye jaba rogi kii vikriti ack dhamani aur ack nus key beach ack jodane vaali keshika kii kamee hoti haye; isake binaa, shoth, raktasraav aur drishti haani hoe sakati haye. shreni teen itani gambhir vikritiyon koo sandarbhit karati haye kii unaki faili hui vaahikaaen abb dhamani aur nus key beach antar naheen karati hain, aur rogi koo drishti haani kaa kaafee adhik khatra hotaa haye. choonki retina key ghaavon koo vargeekrit kiya gayaa haye, way badey samvahani vikritiyon seey laker joe retina key adhikansh hisse koo prabhaavit karate hain, vikritiyon taka joe mushkil seey dikhaai dete hain, ghaavon seey kaee prakaar key lakshan hoe sakate hain, jinamen drishya teekshnata mein kamee, proptosis, pupillary dosh, optic tantrika apakshay aur drishya kshetra dosh shamil hain. aeye. vee. aim. aiss. key kaaran sabse aam prakaar kii drishti kshetra haani samnamit hemianopia haye, joe aamtaur parr ektarafa hoti haye.
bonat-decam-blanck syndrome key kendriya tantrika tantra (see. aine. aiss.) key lakshan mastishk aeye. vee. aim. key sthaanon aur aakar parr atyadhik nirbhar karate hain. sabse aam see. aine. aiss. visheshta madhya mastishk mein ack intracranial hemangioma haye. mastishk vikriti key parinamswarup gambhir sirdard, mastishk raktasraav, ulti, meningism, daure, teevra stroke aur dhamnijanit shunt key kaaran honey vaale teevra yaa puraane ischemia key kaaran pragatisheel tantrika sambandhi kamee hoe sakati haye.
bonat-decam-blanck syndrome kii chehare kii visheshtaen mamle seey mamle mein bhinn hoti hain. syndrome key sanket dikhaane vaalaa vyakti twacha kaa peela rung, nevi yaa angioma pradarshit curr sakata haye. iss vikaar key kuchh rogi maxillofacial yaa mandibular (jabade) kshetron mein uchch pravaah vaale aeye. vee. aim. key saath bhee maujood hotey hain. iss bimari kaa ack anya chehare kaa sanketak saamane yaa maxillary sinus koo prabhaavit karane vaali vikritiyaan hain.
kaaran.
bonat-datume-blanck syndrome dhamani vikritiyon key parinamswarup hotaa haye. iss vikaar kaa sateek kaaran agyaat haye, aur koee vishisht aanuvanshik asamanyata kii pehchaan naheen kii gaee haye. syndrome ack janmajaat vikaar haye joe garbhavastha key saatven saptah key aspas vikasit honah shuru hoe jaataa haye jaba retina messenkimal koshikaon kii paripakvata thheek seey naheen badhati haye. samvahani ootak key asamanya vikas seey dhamani vikritiyaan hoti hain, joe drishya aur mastishk sanrachana donon koo prabhaavit karati hain.
nidan.
nidan aamtaur parr bachpan mein baad mein hotaa haye aur aksar sanyog seey binaa lakshan vaale rogiyon mein yaa drishti haani key kaaran hotaa haye. pahale lakshan aamtaur parr niyamit drishti jaanch key dauran paae jaate hain.
syndrome kii seema aur isaki gambhirta koo nirdhaarit karane key liye kaee pareekshaon kaa upayog kiya jaa sakata haye. fluoresin angiography roga kii retina visheshtaon key nidan mein kaafee upayogi haye, aur ultrasonography aur optical coherance tomography (auct) kaa upayog roga kii pushti karane mein sahayak haye. tantrika-netra parikshanon seey pupillary doshon kaa pataa chalata haye (udaa.jee. marcus gunn chhaatr). funduscopic jaanch, aankh key fundus kii jaanch, dhamani vikritiyon kaa pataa lagaane kii anumati detee haye. tantrika sambandhi jaanch hemiperasis aur peresthesia jaise tantrika sambandhi kamiyon koo nirdhaarit curr sakati haye. aim. aar. aaee. scan kaa upayog mastishk kii imaging mein kiya jaataa haye aur yeh optic tantrika aur kissy bhee sambhaavit shosh kii kalpana kii anumati dey sakata haye. aim. aar. aaee., ct. aur cerebral angiography sabhi kaa upayog mastishk koo prabhaavit karane vaale kissy bhee samvahani ghaav kii seema aur sthaan kii jaanch karane key liye kiya jaa sakata haye.
upchaar.
bonat-decam-blanck syndrome kaa upchaar dhamani vikritiyon key ilaaj key liye vibhinn chikitsiya prakriyaon parr aam sahamati kii kamee key kaaran vivadaspad haye. pahala safal upchaar morgan aur anya logon dwara kiya gayaa thaa., jinhone intracranial vichhedan, netra dhamani kaa bandhan, aur baahari caroted dhamani key chayanaatmak dhamani bandhan koo sanyukt kiya. vishesh roop seey, rogi koo retina samvahani vikriti naheen thee.
aakar mein parivartan key liye ghaavon parr bariki seey nazar rakhee jaati haye. roga kaa purvanuman sabse achchha taba hotaa haye jaba ghaavon kii lambai 3 semi seey cumm hoti haye. adhikansh jatilataaen taba hoti hain jaba ghaav 6 semi seey adhik aakar key hotey hain. intracranial ghaavon key liye shalya chikitsa safaltapurvak kii gaee haye. gair-shalya chikitsa upchaaron mein embolisation, vikiran chikitsa aur nirantar avalokan shamil hain.
upchaar karate samay, vikritiyon key aakar, unake sthaanon aur tantrika sambandhi bhagidari parr vichaar karna mahatvapurna haye. kyonki yeh ack janmajaat vikaar haye, lakshnon kii nigrani key liye doctor key saath niyamit anuvarti kaarravaai seey alag karane key liye koee nivaarak kadam naheen hain taaki bhavishya kii jatilataon seey bachaa jaa sakey.
mahamari vigyaan.
iss syndrome kaa pahali baar 1943 mein varnan kiya gayaa thaa aur maanaa jaataa haye kii yeh retina key resmos haemangiomatosis aur mastishk kii dhamani vikritiyon seey judaa huaa haye. yeh gair-vanshaanugat haye aur ise phacomatosis maanaa jaataa haye lekin isamen shayed hii kabhi twacha shamil hoti haye.

