I-Goldenhar syndrome yaqamba igama layo ngudokotela waseMelika owaqala ukuyichaza phakathi nekhulu leminyaka elidlule. Kusukela ngaleso sikhathi ulwazi mayelana nalesi sifo luye lwafakwa kancane ngenxa yobunzima bayo nokuyinkimbinkimbi yokutadisha, kodwa ngenxa yobuchwepheshe bamuva akukhona nje kuphela okungatholakala ku-utero, kodwa futhi nokuphathwa ngokuphumelelayo.
I-Goldenhar syndrome - iyini?
I-syndrome iyacatshangelwa, okuyinto emithonjeni yezokwelapha ebizwa nangokuthi "i-oculo-auriculo-vertebral dysplasia", "i-microsomy syndrome", isifo sesifo sofuba esinenombolo enkulu ye-anomalies ehambisanayo. I-pathology ihlotshaniswa nomonakalo phakathi nokuthuthukiswa kwe-embryonic yamagill e-gill - ama-cartilaginous formations formations, okuvela kuwo umhlathi osemncane, i-temporomandibular kanye nesakhiwo sosizo lokuzwa.
Ukufunda ngokuningiliziwe nge-Goldenhar syndrome, yikuphi ukugula okuyiyo, yisiphi isizathu sokubonakala kwayo, ososayensi bathola ukuthi abesifazane abatholakali kangako kulezi zifo, futhi ngokusabalalisa ukuthi kunesilinganiso sesithathu phakathi kweziphambeko zokuthuthukiswa kwendawo ye-cranio-maxillofacial ngemuva kokuphambuka okunjenge "hare lip " Futhi" umlomo wempisi ". Ukuthola lesi sifo emfubeni kungenzeka ngesonto lama-20-24 lokubeletha ngokuxilongwa kwe-ultrasound nge-scanning ngezilinganiso ezintathu.
I-Goldenhar syndrome - ibangela
Akusungulwe kahle ukuthi i-Goldenhar syndrome inesizathu, kodwa ososayensi abaningi banombono wokuthi isifo siyini. Iziqephu zalesi sifo zingokwemvelo, kodwa ngokuvamile ngemva kokubuza izihlobo zeziguli ukuthi i-heredity factor is followed. Ezinye izinkolelo zicabangela ubuhlobo bokuthuthukiswa kwezifo kanye nomthelela ezinkathini zokuqala zokukhulelwa kwamakhemikhali athile, ama-viral pathogens.
Ngaphezu kwalokho, amaqiniso alandelayo avela ku-anamnesis wesifazane okhulelwe abhekwa njengengozi yokuthuthukiswa kwesifo:
- umshado wegazi;
- ukukhipha izisu zangaphambilini;
- isisindo somzimba ngokweqile;
- isifo sikashukela.
I-Goldenhar syndrome - izimpawu
Isifo se-Goldengen sitholakala ezinsana ngesikhathi sokuhlolwa kokubukwa, ngokuvamile kuvame ukubonakala okunzima kwezibonakaliso ezinjalo:
- ukuthuthukiswa komhlathi omncane;
- ukwakheka okungalungile kweso (ukwephulwa kwesimo se-orbit nobukhulu bayo);
- ukuchithwa kwe-auricle;
- ukuthuthukiswa kokukhubazeka kwe-vertebrae yomlomo wesibeletho.
Ezimweni eziningi, izimbonakaliso ziyabonakala ngakunye kolunye ubuso nesiqu, izilonda zomhlaba azifani kakhulu. Izinga lobukhulu kanye nezinhlanganisela zokubonakaliswa ngabanye. Ngaphezu kwalokhu, kunezibonakaliso ezilandelayo ezingavamile ze-Goldenhar syndrome:
1. Izici zesakhiwo sobuso nomlomo:
- i-asymmetry ebusweni;
- ukuthuthukiswa kwendawo ye-zygomatic;
- ehamba phambili ebusweni;
- ukuhlulwa komlomo;
- amathanga angeziwe;
- ukuhlukunyezwa kolimi;
- i-cleft palate noma i-lip.
2. Izinkinga zezinzwa zokuzwa nokubona:
- ukungabi khona kwe-auricle;
- ukuphasiswa komsele wokuhlola;
- isithulu;
- i-strabismus;
- isici se-oculomotor musculature;
- i-cyst iso;
- i-atrophy ye-iris;
- iso elishayo.
3. I-pathology yezitho zangaphakathi kanye nesistimu yamathambo:
- isici sodonga lwenhliziyo lwangaphakathi;
- i-aorta encane;
- i-tetrad ye-Fallot ;
- izimo eziphuthumayo zesistimu ye-bronchopulmonary;
- i-hydrocephalus;
- ukuchithwa kwe-renal pelvis;
- ukuhlukunyezwa kwezimbambo;
- i-clubfoot;
- ukuxuba kwe-vertebra yokuqala yomlomo wesibeletho ngethambo le-occipital;
- i-scoliosis;
- ukuthuthukiswa kwengqondo.
I-Goldenhar syndrome - ukwelashwa
Ngokuphathelene nokubonakaliswa kwezinto eziningi, iziguli ezine-Goldenhar syndrome zingaphathwa ukwelashwa okuhlukile, okuvame ukwenziwa ngezigaba eziningana, njengoba ingane ikhula. Ezimweni ezinzima, kungenzeka ukuba uqaphele ochwepheshe abahlukene kuze kube yilapho ingane ifika eneminyaka emithathu, emva kwalokho izinyathelo zokwelapha zinconywa, kuhlanganise nokuphathwa okuhlinzekwayo, ukwelapha i-orthodontic. Ezimweni ezimbi, iningi lalezi zinyathelo liyenziwa kuze kube yilapho eyodwa noma emibili ubudala.
Ukwelashwa kwe-orthodontics kwenziwa ngezinyathelo ezintathu eziyinhloko, okuhambisana nokuthuthukiswa kwesistimu ye-dentoalveolar (isikhathi samazinyo ebisi, isikhathi sokushintsha, isikhathi sokulunywa unomphela). Iziguli zinikwe amadivaysi akhiphekayo angenakususwa ekulungiseni izimpungushe zamasongo kanye nokuluma, futhi kunikezwa izincomo mayelana nemithetho yokunakekelwa ngomlomo. Ngokuvame ukuthi uneminyaka engu-16-18, zonke izinyathelo zokwelashwa nezokuvuselela ziyagcwaliswa.
I-Goldenhar syndrome - ukusebenza
I-microsomia ye-gemifacial iphathwa ngokuziphatha okugunyazayo kokungenelela kokuhlinzwa, uhlobo, ivolumu kanye nenombolo yalo ehlukahluka kuye ngokuthi izinga lokulimala. Ngokuvamile, lezi zinhlobo zokusebenza zinikezwa:
- i-endoprosthetics yokuhlanganiswa kwe-temporomandibular, imifino (ephansi, engenhla);
- i-compression-distraction osteosynthesis;
- i-osteotomy yekhala;
- i-osteotomy yamazinyo;
- i-genioplasty;
- i-rhinoplasty;
- ipulasitiki ye-auricle;
- umdwebo ogqamile womhlathi omncane, njll.
Abantu abane-Goldenhar syndrome
Iziguli ezitholwe ukuthi zinesifo se-Goldenhar syndrome ngaphambi nangemva kokuhlinzwa zingabheka ngendlela ehlukile. Uma isikhathi esifanele ebuntwaneni kuzokwenziwa imisebenzi, kufaka phakathi ipulasitiki, khona-ke izibonakaliso zangaphandle zesifo kungenzeka zingabi khona. Kunezibonelo eziningi lapho abantu abane-Goldenhar syndrome befunda ngempumelelo, bathola umsebenzi omuhle, bahola imindeni futhi babelethe izingane.
I-Goldenhar syndrome - ukubikezela
Kulezi ziguli ezinesifo se-Goldenhar syndrome, ukuphawula kwesimo kuhle kakhulu ezimweni eziningi, futhi kuncike ekutheni umonakalo wezitho zangaphakathi. Njengoba kutholakala zonke izinto eziyinkimbinkimbi, ukusetshenziswa kwazo zonke izindlela zokulungisa ukuphulwa, isimo sengqondo esiseduze nesiguli, ukusekelwa kwengqondo, kunethuba lokuthola ngokugcwele.