I-Goldenhar syndrome yafumana igama layo ngugqirha waseMerika owaqala ukuyichaza phakathi kwekhulu lokugqibela. Ukususela ngelo xesha ulwazi malunga nale nzala luye lwafakwa kancinci ngenxa yobunzima balo kunye nobunzima bokufunda, kodwa ngenxa yobuchwepheshe bwanamhlanje ayifumaneki kuphela kwi-utero, kodwa iphinde iphathwe ngempumelelo.
I-Goldenhar syndrome - yintoni na?
I-syndrome iyakucingwa, apho kwimithombo yezokwelapha iphinde ibhekiswe ngokuthi "i-oculo-auriculo-vertebral dysplasia", "isifo se-microsomy syndrome", isifo esizalwe ngumzimba kunye nenani elikhulu leempawu ezihambelanayo. I-Pathology inxulumene nomonakalo ngexesha lokuphuhliswa kwamabrikhi amacandwe-i-gill-formations, apho i-jaw ephansi, i-temporomandibular kunye kunye nesakhiwo senkxaso yokuphulaphulwa.
Ukufunda ngokubanzi iinkcukacha ze-Goldenhar syndrome, luhlobo luni lokugula, yintoni isizathu sokubonakala kwayo, izazinzulu zifumene ukuba abafazi abanako ukuhlaselwa kwimeko yokukhubazeka, kunye nokuxhaphaka kwendawo yesithathu phakathi kweempembelelo zophuhliso lwendawo ye-cranio-maxillofacial emva kokuphambuka okunjengokuthi "i-hare lip " Futhi" umlomo wempuphu ". Ukuchonga kwesi sifo emntwaneni kuyakwenzeka kwiveki ye-20-24 yesigxina ngokuxilongwa kwe-ultrasound nge-scanning in three dimensions.
I-Goldenhar syndrome - ibangela
Ayinakunyaniswa ngokucacileyo oko i-Goldenhar syndrome inesizathu, kodwa ezininzi izazinzulu ziba neengcamango malunga nesimo sengqondo sesifo. Iziqhelo zesifo ziyimvelo engaqhelekanga, kodwa ngokuphindaphindiweyo emva kwemibuzo yezihlobo zezigulane i-heredity factor is traced. Ezinye iingcamango ziqwalasela ubudlelwane bokuphuhliswa kwezilwanyana kunye neempembelelo kwiimigangatho zokuqala zokukhulelwa kwamachiza athile, i-viral pathogens.
Ukongezelela, ezi nkcukacha ezilandelayo kwi-anamnesis yowesifazane okhulelweyo zibhekwa njengengozi yokuphuhliswa kwesifo:
- umtshato wegazi;
- izisu zangaphambili;
- bunzima bomzimba;
- isifo sikashukela.
I-Goldenhar syndrome - iimpawu
Isifo se-Goldengen sifumaneka kwiintsana eziza kuzalwa ngexesha lokuvavanywa kokubonakalayo, ngokuqhelekileyo kubonakala ngolu hlobo olunzima:
- ukuphuhliswa kwengca emfutshane;
- ukwakheka okungalunganga kweso (ukuphulwa kwesimo se-orbit kunye nobukhulu bayo);
- ukuchithwa kwe-auricle;
- ukuphuhliswa kwesiphene kwi-vertebrae yomlomo wesibeleko.
Kwiimeko ezininzi, iimpawu zibonwa kwelinye icala lobuso kunye nesigxina, izilonda zomhlaba ziqhelekileyo. Iqondo lobunzima kunye nokudibanisa kwemibonakalo ngabanye. Ukongeza kwezi zinto, kukho iziganeko eziqhelekileyo zesifo se-Goldenhar:
1. Iimpawu zesakhiwo sobuso kunye nomlomo:
- asymmetry ebusweni;
- ukuphuhliswa kweendawo zygomatic;
- ebunzini;
- umlomo omkhulu;
- iibhanti ezongezelelweyo;
- izibizo zeelwimi;
- i-palate okanye i-lip.
2. Iimpazamo zeenjongo zokuva nokubona:
- ukungabi khona kwe-auricle;
- ukuhlambalaza kwendlela yokucoca;
- isithulu;
- i-strabismus;
- isiphako se-oculomotor musculature;
- i-cyst eye;
- i-atrophy ye-iris;
- iliso elinamandla.
3. I-Pathology yezitho zangaphakathi kunye nesistim:
- isiphako sodonga lwentliziyo lwangaphakathi;
- ukunciphisa i-aorta;
- tetrad of Fallot ;
- izimo ezingekho phantsi kweenkqubo ze-bronchopulmonary;
- i-hydrocephalus;
- ukuchithwa kwe-renal pelvis;
- ukulungiswa kwezimbambo;
- clubfoot;
- ukuhlanganiswa kwe-vertebra yokuqala yesibeleko kunye nethambo le-occipital;
- i-scoliosis;
- ukungaphuhliswa kwengqondo.
I-Goldenhar syndrome - unyango
Ngokubhekiselele kwimbonakaliso ye-multicharacteristic, izigulane ezine-Goldenhar syndrome zixhomekeke kwiinkatho ezahlukileyo, ezenziwa rhoqo kwiinqanaba eziliqela, njengoko umntwana ekhula. Kwiimeko ezibuhlungu, kunokwenzeka ukugcina iingcali ezahlukeneyo kude kube ngumntwana ofikelela kwiminyaka emithathu, emva koko amanyathelo okuphulukisa aphuculwayo, okubandakanya ukusetyenziswa kweendlela zokugonywa, i-orthodontic therapy. Kwiimeko ezinzima, uninzi lwongenelelo lwenziwa kuze kube linye okanye kwiminyaka emibili ubudala.
Unyango lwe-orthodontics luqhutyelwa kwizigaba ezintathu eziphambili, ezihambelana nokuphuhliswa kwenkqubo ye-dentoalveolar (ixesha lamazinyo obisi, ixesha lokutshintsha, ixesha lokuluma ngokusisigxina). Izigulane zibonelelwa ngamadivayisi asusweyo angenakususwa ukulungiswa kweengcambu kunye neengxaki zokuluma, kunye neziphakamiso zinikezelwa malunga nemigaqo yokunonyelwa ngomlomo. Ngokuqhelekileyo uneminyaka eyi-16-18, onke amanyathelo enyango kunye nokuvuselela agqityiwe.
- Goldenhar syndrome
I-microsomia ye-gemifacial iphathwa ngokunyanzeliswa kwamanyathelo okungenelela, uhlobo, umthamo kunye nenani lalo lihluka ngoxhomekeke kwinqanaba lokulimala. Ngokuqhelekileyo, ezi ntlobo zemisebenzi zinikezelwa:
- i-endoprosthetics yokudibanisa i-temporomandibular, ihlunu (ngaphantsi, phezulu);
- ukunyanzeliswa kwe-osteosynthesis;
- i-osteotomy yempumlo;
- i-osteotomy yamasaya;
- genioplasty;
- rhinoplasty;
- iplastiki ye-auricle;
- umgca wecala lomhlathi osezantsi, njl.
Abantu abane-syndrome ye-Goldenhar
Izigulane ezifunyaniswa ukuba zibe ne-Goldenhar syndrome ngaphambi nangemva kokuhlinzwa zingakhangela ngendlela eyahlukileyo. Ukuba ixesha elifanelekileyo ngexesha lobuntwana liya kwenziwa imisebenzi, kuquka iiplastiki, ngoko iimpawu zangaphandle zesifo zingaba zingekho. Kukho imizekelo emininzi apho abantu abane-Goldenhar syndrome bafundela ngempumelelo, bafumane umsebenzi omhle, baholele iintsapho baze babele abantwana.
I-Goldenhar syndrome - isalathiso
Kwizigulane ezine-syndrome ye-Goldenhar, i-prognosis iyanceda kakhulu kwiimeko ezininzi, kwaye kuxhomekeka kakhulu kumlinganiselo wonakalo kwizitho zangaphakathi. Ngokufumanisa zonke izinto eziyinkimbinkimbi, ukusetyenziswa kwazo zonke iindlela ezifanelekileyo zokulungisa ukuphulwa, isimo sengqondo esondeleyo kwisigulane, inkxaso yengqondo, kukho ithuba lokufumana ngokupheleleyo.