Kodi mukuona kusintha kulikonse pankhope kapena m'makutu a mwana wanu? Kodi kungakhale Goldenhar Syndrome?

Tikamaona mwana wakhanda, timamva kusangalala komanso kukondedwa, sichoncho? Koma taganizirani izi, nthawi zina, ngakhale kuti nthawi zambiri, makanda ena amabwera m'dziko lino ndi kusiyana pang'ono komwe kulipo kuyambira kubadwa. Umu ndi momwe, makamaka pankhope, m'makutu, m'maso, kapena msana, vuto lomwe tikambirane lero limatchedwa Goldenhar Syndrome. Musachite mantha mukamva dzina ili, tidzakambirana chilichonse m'njira yosavuta yomwe mungamvetse.

Kodi Goldenhar Syndrome ndi chiyani?

Mwachidule, Goldenhar Syndrome ndi vuto losowa kwambiri, lobadwa nalo . "Congenital" amatanthauza kuti vutoli limapezeka pobadwa. Limagawidwa ngati vuto la craniofacial. Izi zikutanthauza kuti limayambitsa zolakwika mu mawonekedwe kapena kukula kwa nkhope kapena mutu wa mwana wanu. Makamaka, Goldenhar Syndrome ingakhudze msana, makutu, ndi maso a mwana wanu.

Kawirikawiri, anthu omwe ali ndi Goldenhar Syndrome amakhala ndi mafupa ndi minofu yosakwanira mbali imodzi ya nkhope (yomwe imatchedwanso hemifacial microsomia). Nthawi zina mbali zonse ziwiri zimatha kukhudzidwa. Ana ena alinso ndi milomo yosweka kapena pakamwa posweka.

Dzina lake ndi Goldenhar polemekeza wofufuza yemwe adapeza matendawa koyamba mu 1952, Maurice Goldenhar. Dzina lina lachipatala ndi "oculo-auriculo-vertebral dysplasia." Dzinalo limamveka lalitali pang'ono, sichoncho? Tiyeni tifotokoze mwachidule, sichoncho?

• Oculo amatanthauza kugwirizana ndi maso.
• Auriculo amatanthauza kugwirizana ndi khutu.
• Vertebral imatanthauza msana.
• Dysplasia ndi chitukuko chosazolowereka cha gawo lina la thupi.

Tsopano mwamvetsa tanthauzo la dzina ili, eti?

Kodi vutoli ndi lofala bwanji?

Matenda a Goldenhar ndi vuto losowa kwambiri . Akatswiri amati limakhudza pafupifupi mwana m'modzi mwa ana obadwa kumene 3,500 mpaka 25,000. Akutinso ndi lofala pang'ono mwa anyamata kuposa atsikana.

Kodi ndi zifukwa ziti zomwe zimayambitsa Goldenhar Syndrome?

Funso lomwe anthu ambiri ali nalo ndi lakuti "Chifukwa chiyani izi zimachitika?" Kunena zoona, akatswiri sanamvetsebe .Chifukwa chenicheni cha Goldenhar Syndrome sichikudziwika. Amakhulupirira kuti imayambitsidwa ndi kusintha kwa chromosome, koma chifukwa chake sichimveka bwino nthawi zonse. Pa chiwerengero chochepa kwambiri, pafupifupi 1% - 2% ya milandu, vutoli likhoza kutengera kwa kholo limodzi kapena onse awiri.

Kafukufuku wina wasonyeza kuti chiopsezo cha mwana kudwala Goldenhar Syndrome chingachuluke pang'ono ngati mayi ali ndi matenda enaake kapena akugwiritsa ntchito mankhwala enaake panthawi ya mimba. Mwachitsanzo:

• Matenda a shuga a mimba.
• Mankhwala ena omwe amagwiritsidwa ntchito pa ziphuphu, makamaka omwe ali ndi retinoic acid, monga isotretinoin (Accutane®).

Choncho, ngati muli ndi pakati, ndikofunikira kwambiri kutsatira malangizo a dokotala wanu molondola.

Kodi zizindikiro za izi ndi ziti?

Zizindikiro za Goldenhar Syndrome zimatha kusiyana malinga ndi munthu. Komabe, chimodzi mwa zizindikiro zodziwika bwino ndichakuti mbali imodzi ya nkhope sikukula bwino . Izi zimatchedwanso `hemifacial microsomia`, monga tanenera kale. Izi zingayambitse nsagwada, masaya, ndi maso kumbali imodzi ya nkhope kukhala ang'onoang'ono komanso osakula bwino kuposa mbali inayo. Mwachitsanzo, tsaya limodzi lingakhale losalala kuposa lina, kapena chibwano chingakhale chaching'ono mbali imodzi.

Zina zomwe zimawoneka ndi izi:

• Matenda a m'makutu: Anthu ena akhoza kukhala ndi khutu limodzi laling'ono kwambiri (lotchedwa `microtia`). Ena angakhale ndi khutu limodzi losowa konse (lotchedwa `anotia`). Izi zingayambitse kutayika kwa kumva.
• Zimakhudza mbali zonse ziwiri za nkhope: Pafupifupi gawo limodzi mwa magawo atatu a ana omwe ali ndi Goldenhar Syndrome amakumana ndi kukula kolakwika mbali zonse ziwiri za nkhope.
• Mavuto ndi ziwalo zina: Mavuto amathanso kuchitika ndi impso, mtima, mapapo, kapena mafupa a msana (vertebrae).
• Kulephera kwa nzeru: Pafupifupi 15% ya anthu akhoza kukhala ndi vuto la nzeru. Izi zikutanthauza mavuto ophunzirira, kuchepa kwa luso lomvetsetsa, ndi zina zotero.

Kuwonjezera pa izi, zizindikiro zina zingawonekere:

• Kukhala ndi milomo yosweka kapena yosweka.
• Zilema za mtima zobadwa nazo.
• Nthawi zina ziphuphu zazing'ono (dermoid cysts) zimatha kupangika pa diso.
• Kutaya kumva.
• Kusonkhana kwa madzi mkati mwa chigaza (Hydrocephalus).
• Kulephera kugona mokwanira.
• Kutayika kwa chikope kapena minofu ina m'diso (coloboma) ndipo zotsatira zake ndi kutayika kwa masomphenya.
• Scoliosis.
• Mavuto olankhula.
• Maso opindika (`Strabismus`).

Kumbukirani kuti si aliyense amene angakumane ndi zizindikiro zonsezi. Anthu ena angakhale ndi zizindikiro zochepa chabe, ndipo mphamvu zake zimasiyana.

Kodi mungadziwe bwanji ngati muli ndi Goldenhar Syndrome?

Kawirikawiri, madokotala amatha kuzindikira Goldenhar Syndrome mwana akangobadwa kutengera zizindikiro zakunja, monga kusintha kwa nkhope ndi makutu.

Komabe, kuti atsimikizire zambiri ndikuwona ngati pali mavuto ena amkati, dokotala angachite mayeso ena angapo. Ena mwa awa ndi awa:

• Kujambula kwa CT: Izi zimatha kuzindikira kusintha kwa kapangidwe ka mkati mwa khutu komwe kungayambitse kutayika kwa kumva.
• Echocardiogram (mayeso a echo) kapena electrocardiogram (EKG): Mayeso awa amatha kuwona momwe mtima ukugwirira ntchito. Monga tafotokozera kale, nthawi zina matenda a mtima amatha kuchitika.
• Kuyezetsa maso: Mayesowa amachitika kuti aone ngati pali vuto mkati mwa diso.
• Ultrasound ndi X-ray: Izi zimatha kuwona kusintha kwa chigaza, msana, mapapo, kapena impso.
• Kuyesa majini: Kuyesa kumeneku kumathandiza kuchotsa matenda ena a majini omwe angayambitse zizindikiro zofanana.
• Maphunziro a kugona: Izi zimachitika pofuna kuwona ngati pali vuto la kupuma movutikira.

Kodi izi zitha kuzindikirika mwana asanabadwe?

Izi zimachitika kawirikawiri. Komabe, nthawi zina, panthawi yowunikira mwana wosabadwayo, madokotala amatha kuwona kusintha kwa nsagwada, makutu, kapena pakamwa pa mwana wosabadwayo. Ngati izi zitachitika, angayang'anire kwambiri.

Kodi mankhwala ochizira izi ndi otani?

Chithandizo cha Goldenhar Syndrome chimasiyana malinga ndi zizindikiro . Si aliyense amene amachizidwa mofanana. Ana ena sangafunike chithandizo chapadera ngati zizindikiro zawo zili zochepa kwambiri. Komabe, chithandizo chimakonzedwa mothandizidwa ndi akatswiri osiyanasiyana, ngati pakufunika.

Nazi njira zina zochiritsira:

• Chithandizo cha kuyamwitsa: Ana ena angavutike kuyamwa. Pazochitika zotere, mabotolo apadera kapena kuyamwitsa kwa nasogastric (NG) kungafunike.
• Kukonza masomphenya: Mutha kugwiritsa ntchito magalasi kapena kuchitidwa opaleshoni kuti muwone bwino masomphenya anu.
• Zipangizo zothandizira kumva: Kumva kumatha kukonzedwa pogwiritsa ntchito zothandizira kumva kapena zomangira zomvera zomwe zimamangiriridwa ndi mafupa.
• Chithandizo cha kulankhula:Izi ndizofunikira kwambiri pakukulitsa luso la chilankhulo ndi kulankhulana.
• Opaleshoni: Opaleshoni ingachitike kuti ikonze matenda a mtima, milomo yosweka kapena mkamwa, kupuma movutikira, makutu ang'onoang'ono (microtia), kapena kufooka kwa msana.

Zonsezi zimachitika kuti moyo wa mwana ukhale wabwino momwe angathere. Chifukwa chake, ndikofunikira kwambiri kutsatira malangizo omwe dokotala wapereka.

Kodi kusintha kwa nkhope kungakonzedwe?

Inde, ili ndi vuto kwa makolo ambiri. Opaleshoni yokongoletsa nkhope imatha kuchitika kuti nkhope ikhale yofanana. Ana ambiri omwe ali ndi Goldenhar Syndrome amachitidwa opaleshoni yosintha mawonekedwe a nsagwada, masaya, makutu, kapena mphumi. Izi nthawi zambiri zimachitika mwanayo akakula pang'ono, ali ndi zaka zoyenera.

Kodi Goldenhar Syndrome ingapewedwe?

Monga tafotokozera kale, palibe njira yotsimikizika yopewera vutoli chifukwa chomwe chimayambitsa sichikudziwika . Komabe, ndikofunikira kutsatira malangizo onse a dokotala wanu panthawi ya mimba. Makamaka, musagwiritse ntchito mankhwala okhala ndi retinoic acid (monga mankhwala ena a ziphuphu) popanda upangiri wa dokotala wanu. Ndikofunikiranso kudya zakudya zabwino.

Ngati wina m'banja mwanu wakhala ndi Goldenhar Syndrome, ndi bwino kupeza upangiri wa majini. Kenako, ngati mukukonzekera kukhala ndi mwana, mutha kumvetsetsa chiopsezo cha mwana ameneyo kukhala ndi vutoli.

Kodi moyo udzakhala bwanji ndi vutoli?

Izi zingakuwonekere ngati zoopsa pang'ono. Komabe, ngakhale tsogolo la anthu omwe ali ndi Goldenhar Syndrome limasiyana, anthu ambiri amakhala ndi zotsatira zabwino . Ndi chithandizo choyenera, ana omwe ali ndi vutoli nthawi zambiri amakhala ndi moyo wabwinobwino. Amatha kuphunzira, kusewera, komanso kutenga nawo mbali pagulu.

Ndi zinthu ziti zomwe mukufuna kufunsa dokotala?

Mukazindikira kuti mwana wanu ali ndi vutoli, mungakhale ndi mafunso ambiri. Panthawiyo, musaope kufunsa dokotala wanu zinthu izi:

• "Dokotala/Abiti, kodi zizindikiro zazikulu za Goldenhar Syndrome ndi ziti?"
• "Ndi mayeso otani omwe ndiyenera kuchita kuti nditsimikizire kuti mwana wanga ali ndi izi?"
• "Kodi njira zochiritsira izi ndi ziti? Ndi chiyani chomwe chili chabwino kwa mwana wanga?"
• "Ndi malo ati odalirika oti mudziwe zambiri za vutoli?"
• "Kodi pali mabungwe kapena magulu olera ana omwe amathandiza ana ndi mabanja pa vuto lamtunduwu?"

Kufunsa mafunso awa kudzakuthandizani kumvetsetsa bwino zomwe zikuchitika komanso kupatsa mwana wanu zabwino kwambiri.

Kodi Goldenhar Syndrome ndi chilema?

Inde, nthawi zina iziChilema chingaonedwe ngati chilema. Mwachitsanzo, ngati pali vuto la kumva kapena kuona, chingakhale chilema. Mofananamo, ngati pali vuto la nzeru, munthu ameneyo angafunike thandizo lochulukirapo kuti agwire ntchito ya tsiku ndi tsiku ndikuphunzira. Chifukwa chake, ndi udindo wathu monga gulu kuwapatsa zinthu zofunikira komanso chithandizo.

Ndi matenda ena ati omwe ali ndi zizindikiro zofanana?

Palinso matenda ena angapo omwe ali ndi zizindikiro zofanana ndi za Goldenhar Syndrome. Chifukwa chake, ndikofunikira kwambiri kupeza matenda olondola. Ena mwa matenda awa ndi awa:

• Matenda a Branchio-oto-renal `(Branchioootorenal (BOR) syndrome)`
• Bungwe la CHARGE
• Matenda a Townes-Brocks
• Matenda a Treacher Collins
• Mgwirizano wa VACTERL

Ngakhale kuti izi zingamveke zovuta pang'ono, madokotala amaganizira zonsezi akamafufuza matenda.

Zimene tiyenera kukumbukira kuchokera pa zomwe takambirana!

Matenda a Goldenhar ndi matenda osowa omwe amapezeka pobadwa. Amakhudza kwambiri mawonekedwe a nkhope ya mwana, mutu wake, komanso nthawi zina ziwalo zake zamkati. Madokotala amatha kukonza zolakwika zina pankhope kapena msana ali mwana pogwiritsa ntchito opaleshoni.

Chofunika kwambiri ndichakuti ana ena sangafunike chithandizo chapadera ngati zizindikiro zawo zili zochepa. Ndipo, nthawi zambiri, anthu omwe ali ndi Goldenhar Syndrome amakhala ndi moyo wosangalala komanso wokhutiritsa ndi chithandizo chofunikira.

Ngati mukuganiza kuti mwana wanu ali ndi zizindikiro izi, musachite mantha, koma pitani kwa dokotala nthawi yomweyo kuti akupatseni upangiri. Kuzindikira matendawa msanga komanso kulandira chithandizo choyenera kungapatse mwana wanu tsogolo labwino.

Matenda a Goldenhar , vuto lobadwa nalo, vuto lobadwa nalo, vuto la khungu, microsomia ya nkhope, dysplasia ya oculo-auriculo-vertebral, thanzi la mwana