Syndrome Goldenhar: incazelo, izimbangela, izimpawu, nendlela aphatha ngayo ifa

Goldenhar syndrome - nesifo esiwufuzo okuloaurikulovertebralnoy esifundeni, ie kokunqotshwa izakhiwo lovela lokuqala nelesibili gill nemengamo. Yayibizwa echazwe 1952 udokotela American Goldenhar. Lena isifo wesintu wonke wathola ifa lapho avame liphinyiselwe hypoplasia unilateral ebusweni, izindlebe normal, amajwabu amehlo, amazinyo, amathambo, nokunye. D.

Goldenhar syndrome

Goldenhar syndrome yaqale echazwe maphakathi nekhulu le-lamashumi amabili. Kodwa 'idla yodwa kamuva isifo ahlukene udokotela American ngegama zazo isusela igama layo. Kuvamisile-1: 3000-5000. Emhlabeni wonke, orekhodiwe ambalwa amacala lesi sifo.

syndrome ukubonakaliswa

Goldenhar syndrome kubonakala ngesimo anomalies zangaphandle. Lapho-ke okungukuthi waphawula ukusonteka auricles kokuba nobuntu neziqu. Zingaba flat noma aphumele, kuthinte indlebe eyodwa kuphela noma aphule wesimethri nhlangothi zombili. Ngezinye izikhathi exhunywe kwi ekhoneni lomlomo wakhe ngenxa umuntu ozuza ukubukeka wry. Izindlebe ngokuvamile kakhulu sikhubazekile, ngezinye izikhathi kukhona ukuvaleka endlebeni umsele noma khona fistulas.

Ngenxa nokungathuthuki ka umhlathi ongezansi ngokwenkolo avame ukubhekana nayo azilingani. Kunezindlela ezihlukahlukene anomalies emehlweni nezinkophe. Abakwazi okuthintekayo syndrome eyodwa emaceleni, kodwa ngaphezu kwakho konke - nhlangothi zombili. Emgogodleni kuthintwa kule isifo akusiyo njalo. Kodwa uma namanje anomalies namatha it, amathambo wesibeletho tho ne ngemuva ekhanda, noma nomunye.

Ngo emfantwini nolwanga kungenziwa, ombaxambili ulimi izinyo ukukhula kakhulu okhathazekile. Ngokuvamile in iziguli manifest retardation engqondo. degree wayo ungaba ezahlukene. Kodwa retardation engqondo kusekhona amaphesenti ayishumi kuphela eziguli.

Izimbangela isifo

Goldenhar syndrome uphenyo akwanele. Kodwa izazi eziningi ziyavumelana ukuthi isizathu nesenzakalo kwakhona ezakhini zofuzo. Ezimweni eziningi, lesi sifo oqabukelayo.

Kungani namanje Goldenhar syndrome? Izizathu ingase embozwe nabazali consanguineous. Kodwa i ababalulekile kule syndrome may play bangaphambili isisu nesineke unina wengane noma teratogenic izici, owenzeka ngo ekuseni sokukhulelwa. Ngaphezu kwalokho, imbangela ingane sifo kungase kube kuhle ukukhuluphala noma sikashukela unina.

ifa syndrome

Ifa Goldenhar syndrome idluliselwa i autosomal imfashini esikhubazayo noma evelele. Ingabe ihlelwe kabusha kwezinye chromosome. Ngesinye isikhathi, uma syndrome ezikhonjwe ukuguquka kwezakhi zofuzo isakhi sofuzo GSC. Uma kuguquka kuthinta futhi TCOF1 isakhi sofuzo, kuholela ukuthuthukiswa ezengeziwe syndrome Treacher-Collins.

sitholakele lesi sifo

Ukuxilonga Goldenhar syndrome osebenza izinqubo eziningi. Omunye wabo - ekutadisheni ukuzwa. Kuyasiza ukucacisa degree yokungezwa ohlangothini ethintekile. Kumelwe kwenziwe ngokushesha ngangokunokwenzeka ukuze ingane kamuva wawa ngemuva sokukhula kwengqondo futhi inkulumo.

Zisencane izingane isencane ihlolwe nekhono sokuzwa, ubuchopho impendulo kanye amathoni modulated. Hlola ingane ngesikhathi sokulala. Ukuze izingane ezindadlana for sitholakele ukuzwa inkulumo audiometry isetshenziswa ngesikhathi game. Nge yilesi sifo ngokuvamile kwembula lamadigri ukulahlekelwa kukuzwa obuhlukahlukene. Ukuxilongwa ukuzwa okufanele iqhutshwe kabili ngonyaka iminyaka eyisikhombisa.

Ngaphandle okungenhla esetshenziswa computed tomography yezindawo yesikhashana, esiqhutshwa izingane ngenhla neminyaka emithathu ubudala. Ingane kokuba Goldenhar syndrome, nencazelo engenhla pathologies athile uvezwa, kumelwe ibhekane unomphela luhlolo odokotela:

• wenhliziyo;
• kokwelashwa;
• zofuzo;
• Udokotela ohlinzayo;
• wamathambo;
• inkulumo pathologists;
• ophthalmologist;
• wezinzwa.

Ukwelashwa kwe-Goldenhar syndrome

Ngenxa ezihlukahlukene anomalies ebusweni kanye vertebral, lapho Kutholwe Goldenhar syndrome, ukwelashwa multi-izigwegwe ngokwemvelo. It is a multi-esiteji futhi kuncike inani pathologies. Uma kunjalo kulula, ingane ngaphansi kweso iminyaka emithathu kuphela, bese iqala ukwelashwa orthodontic nokuhlinzwa. Uma kunjalo iyisifo eziyinkimbinkimbi (emfantwini nolwanga isici emihlathini nokunye), isifunda ukwelashwa sidlula nezinye. Okokuqala, imisebenzi eyenziwa - ezingeqi kwezingu emibili ubudala. Futhi kuphela emva kwalokho lokufeza ukwelashwa eziyinkimbinkimbi.

Goldenhar syndrome (microsomia hemifacial) ngaso sonke isikhathi sidinga ukwelashwa kuhlinzwa. Kulokhu, ukuhlukanisa izigaba eziningana:

• kokucindezelwa-kokuthikamezeka osteogenesis;
• TMJ arthroplasty;
• osteotomy we ephansi kanye engenhla emihlathini;
• isigenco esiwela kwesokudla.

Ukuze izigaba orthodontic bathambekele ekuvimbeleni ukukhumuzeka asymmetries ezihlukahlukene emhlathini, nokuqedwa ka malocclusion, ukulungiswa emazinyweni imisipha yobuso ngoba imisebenzi.

syndrome Goldenhar: ukwelashwa orthodontic

ukwelashwa Orthodontic uhlukaniswe izigaba ezintathu bite:

1. Ubisi.
2. Replaceable.
3. Permanent.

Isigaba sokuqala iyona ebaluleke kakhulu. Ngesikhathi sakhe abazali kuqala okuphambene, layo kobukhulu kanye kungenzeka okwalandela izingozi kanye nokuthuthukiswa isifo. Khuluma edingekayo inhlanzeko yomlomo nokuthi ingane kufanele zilandelwe. Uma umntwana ayizange wathola ukwelashwa esifike ngesikhathi ngokushesha, bese ngeminyaka ke ungaboniswa nokungathuthuki amathambo ebusweni. Futhi intando intuthuko. ngokuhamba kwesikhathi, i bite normal iyoba ngokuya abavelele. Ukugwinya futhi betel ingane kuyoba nzima kakhulu. Futhi ngenxa yalezi abanokukhubazeka - uhlukunyezwe ngokwengqondo kwabazali nezingane.

Ekwelapheni esigabeni sokuqala asetshenziswa esikhiphekayo nokunwebeka emhlathini kanye plate amasethi. Lokhu okungukuthi isikhathi lapho main injongo odokotela - ukuzijwayelanisa ingane ekhethekile amadivayisi usizo kubo ukujwayela ukujwayela ukuzisebenzisa. Uma asymmetry awuzange ekuqaleni uzwakalise, ukuthi zinsizakuphila baxwayisa ubunjalo baso.

Okwesibili esiteji yokwelashwa

Umsebenzi main - ukuqeda malocclusion. Ukuze kulungiswe Umumo okungamukelekile osayizi nemengamo dentoalveolar. Umsebenzi uyaqhubeka ekuvikelweni kanye nokuqedwa okuphambene emihlathini kanye nokungathuthuki yayo. Ukuze ukwelashwa isicelo esikhiphekayo ipuleti nokunwebeka. Lezi amadivayisi ukwesekela nokusekela imihlathi. Ngaphezu kwalokho, ukusebenzisa uzimele-non-esikhiphekayo.

Isigaba sesithathu

In esigabeni sesithathu uyaqhubeka zonke ukwelashwa abalwe ngenhla. Kodwa ukuqala kakade ukusebenzisa besokunxele non-esikhiphekayo. Ekupheleni ukuphathwa osetshenziswayo nokugcinwa yinkathi maqembu ukuhlanganisa izuzwe Imiphumela yokuqala. Ezisetshenziswa non-esikhiphekayo ayephelezelwa wire kokungathembeki engenhla ulwanga namazinyo. Kwabanye zinsizakuphila ebonakalayo kakade.

Ngesikhathi esifanayo ingane ithola isiqeshana esikhiphekayo, esetshenziswa ngesikhathi sokulala kuphela. Ukugcinwa inkathi uphela kuphela lapho ingane iyeka ezikhulayo. Phakathi nalesi sikhathi olwenziwa survey gqwa. Ingane engaphansi ebhekiwe odokotela. Uma kudingeka, ukwenza ukuhlinzwa isikhumba. Ingane Ukuvuselelwa uphela iminyaka kuphela 18, okungukuthi, uma umzimba wakhe usevele bakheké ngokuphelele.