Goldenhar syndrome: kufotokoza, zimayambitsa, zizindikiro, ndi chithandizo cha cholowa

Goldenhar syndrome - otengera kwa makolo matenda okuloaurikulovertebralnoy dera, mwachitsanzo, kugonjetsedwa kwa nyumba ya wochokera yoyamba ndi yachiwiri arches gill. Limafotokonzera 1952 ndi dokotala American Goldenhar. Izi ndi zimene tinatengera matenda anthu amene nthawi zambiri kutchulidwa unilateral hypoplasia ya nkhope, makutu nthenda, zikope, mano, vertebrae, ndi zina zotero. D.

syndrome Goldenhar

Goldenhar syndrome anafotokozedwa koyamba mu m'ma makumi awiri. Koma olekanitsidwa ku matenda osiyana kenako dokotala American dzina limene zichokela dzina lake. Kuchuluka 1: 3000-5000. Padziko lonse, lolembedwa zina za matenda.

syndrome mawonetseredwe

syndrome Goldenhar akuwonetseredwa mu mawonekedwe a anomalies kunja. Pamene anaona mapindikidwe auricles ndi zophuka khalidwe. Iwo akhoza kukhala lathyathyathya kapena yotundumukira kunja kwa mpanda, bwanji khutu mmodzi kapena kuswa chosokonekera mbali zonse. Nthawi zina olumikizidwa kwa ngodya m'kamwa mwake chifukwa cha munthu amapeza ndi wry tione. makutu amakhala chikuthandiza kwambiri olumala, nthawizina uko ndi kuchepetsa a ngalande khutu kapena pamaso pa fistulas.

Chifukwa underdevelopment la nsagwada m'munsi amakumana ndi asymmetrical. Pali zosiyanasiyana anomalies mu maso ndi zikope. Zikhoza kugwida ndi matenda a munthu amaganiza, koma koposa zonse - mbali zonse. msana amakhudzidwa matenda si nthawi zonse. Koma ngati inu anomalies mbakuya, ndi vertebrae khomo lachiberekero ali zinaphatikizidwa ndi kumbuyo kwa mutu wake, kapena ndi mzake.

Mu vuto angathe forked manenedwe ndi kukula dzino kwambiri kusokonezedwa. Nthawi zambiri odwala ndi kuwonetseredwa retardation maganizo. digiri yake kukhala osiyana. Koma retardation maganizo komabe pali yokwana khumi limodzi chabe odwala.

Zimayambitsa matenda

Goldenhar syndrome ankafufuza sikokwanira. Koma akatswiri ambiri amavomereza kuti chifukwa pakuwonekera kwake ndi chibadwa. Nthawi zambiri chikhalidwe cha matenda ayi.

Chifukwa adakali angayambe syndrome Goldenhar? zifukwa yokutidwa ndi makolo consanguineous. Koma yofunika kwambiri mu matendawa akhoza kuimba kale mimba mtima mayi wa mwanayo kapena zinthu teratogenic, komwe kunachitika mu mimba msanga. Komanso, chifukwa cha mwana amene ali ndi matenda mwina kungakhale kunenepa kapena shuga mu mayi.

syndrome cholowa

Madalitso syndrome Goldenhar imafalikira mu autosomal recessive kapena lalikulu mafashoni. Kodi kuwayalanso ena DNA. Nthawi zina, pamene syndrome ndi kuzindikiridwa masinthidwe GSC mbewu. Ngati kusintha amakhudza komanso TCOF1 jini, tidzapeza chitukuko cha matenda zina Treacher-Collins.

matenda a matendawa

Matenda syndrome Goldenhar likukhudza njira zambiri. Mmodzi wa iwo - kuphunzira kumva. Zimathandiza kudziwa mlingo wa ugonthi kumbali akhudzidwa. Ziyenera kuchitika mwamsanga kuti mwana kenako anagwa kumbuyo chitukuko maganizo ndi malankhulidwe.

Pa ana oyambirira zaka ndi kuyesedwa Zoyenereza Makutu, ubongo ayankhe ndi malankhulidwe ndi modulated. Pendani mwana akagona. Ana akulu matenda a kumva kulankhula audiometry ntchito pa masewera. Ndi matenda kawirikawiri limasonyeza amawasonyeza mosiyanasiyana kumva imfa. Matendawa kumva iyenera kuchitikira kawiri pachaka kwa zaka zisanu ndi ziwiri.

Kuwonjezera pa pamwamba ntchito tomography yopangidwa mwa madera wosakhalitsa, imene ana pamwamba zaka zitatu. mwana wokhala Goldenhar syndrome, kulongosola pamwamba pathologies angapo amene timalandira ayenera kukumana ndi madokotala okhazikika Kupenda:

• cardiologist;
• lolankhula;
• chibadwa;
• dokotala;
• mafupa;
• kulankhula pathologists;
• maso;
• katswiriyu.

Chithandizo cha matenda Goldenhar

Chifukwa cha zosiyanasiyana anomalies nkhope ndi vertebral, pamene wapezeka Goldenhar matenda, mankhwala Mipikisano chilango m'chilengedwe. Ndi Mipikisano gawo ndi zimadalira kuchuluka kwa pathologies. Ngati choncho n'zosavuta, mwanayo kuyang'aniridwa ndi zaka zitatu zokha, kenako amayamba chithandizo orthodontic opaleshoni. Ngati choncho ndi matenda zovuta (abadwe chilema nsagwada ndi zina zotero), mankhwala dera umadutsa ena. Choyamba, ntchito zimagwiridwa - munthu pazipita zaka ziwiri. Ndipo pambuyo kuti agwire chithandizo zovuta.

Goldenhar syndrome (hemifacial microsomia) nthawi zonse amafuna chithandizo opaleshoni. Pankhaniyi, kusiyanitsa magawo angapo:

• psinjika-chododometsa osteogenesis;
• TMJ arthroplasty;
• osteotomy la nsagwada m'munsi ndi chapamwamba;
• opaleshoni pulasitiki.

Pakuti magawo orthodontic amakonda popewa chitukuko cha asymmetries zosiyanasiyana pachibwano kuwonongedwa kwa malocclusion, yokonza mano ndi minofu nkhope kwa ntchito.

Goldenhar syndrome: orthodontic chithandizo

chithandizo Orthodontic lagawidwa magawo atatu a kuluma:

1. Mkaka.
2. Replaceable.
3. Okhazikika.

Gawo loyamba ndi zofunika kwambiri. Pa nthawi ya makolo ake choyamba lipoti wachiwiri, ukulu wake ndi zotheka mavuto wotsatira ndi chitukuko cha matenda. Kulankhulana chofunika m'kamwa ukhondo ndi kuti mwana ayenera Pamilunguyo. Ngati Mwanayo sanalandire chithandizo yake yomweyo, ndiye ndi zaka zikhoza wasonyeza underdevelopment a mafupa a nkhope. Ndipo patsogolo. pa nthawi, ndi kuluma nthenda adzakhala kwambiri otchuka. Kumeza ndi kutafuna mwana kudzakhala kovuta kwambiri. Ndipo chifukwa cha impairments awa - mwakayakaya maganizo a makolo ndi ana.

Mankhwalawa gawo loyamba ntchito zochotseka zotanuka nsagwada ndi mbale waika. Izi ndi nthawi pamene cholinga chachikulu cha madokotala - kuti anaziphunzitsa kwa mwana ndi zipangizo wapadera kuwathandiza kuti azolowere azolowere ntchito. Ngati asymmetry sanali poyamba umaonetseredwa, kuti zipangizo amenewa kuchenjeza khalidwe lake.

Gawo lachiwiri la mankhwala

Ntchito yaikulu - kuthetsa malocclusion. Pofuna kukonza akalumikidzidwa osasamba ndi makulidwe arches dentoalveolar. Kugwira ntchito pa kupewa ndi kuwonongedwa kwa wachiwiri nsagwada ndi underdevelopment ake. Mankhwala ntchito zochotseka zotanuka mbale. zipangizozi kutumikira monga thandizo ndi kuthandiza m'kamwa mwa. Komanso, ntchito chimango sanali zochotseka.

Mbali yachitatu

Mu gawo lachitatu akupitiriza njira zonse tatchulazi. Koma kale kuyamba ntchito braces sanali zochotseka. Kumapeto kwa mankhwala ntchito nthawi posungira nthawi wakupambana kuphatikiza yapindula choyamba. Kodi ntchito retainers sanali zochotseka wa waya kukonza chapamwamba m'kamwa ndi mano. Kwa ena zipangizo kale kuonekera.

Pa nthawi yomweyo mwanayo amalandira kopanira zochotseka, amene ntchito kokha akagona. Posungira nthawi umatha pamene mwana wasiya kukula. Panthawi imeneyi imachitika Kafukufuku nthawi. Mwanayo kuyang'aniridwa zonse madokotala. Ngati ndi kotheka, kuchita opaleshoni pulasitiki. Mwana Kukonza umatha zaka 18, ndiye pamene thupi lake kale athanzi.