Ðe nèle dzesi dem tɔtrɔ aɖe le viwòa ƒe mo alo to mea? Ðe wòate ŋu anye Goldenhar Dɔlélea?

Ne míelé ŋku ɖe vidzĩ si wodzi yeyee ŋu la, míesena le mía ɖokui me be dzi dzɔa mí ŋutɔ eye wolɔ̃ mí, alo? Gake bu eŋu kpɔ, ɣeaɖewoɣi, togbɔ be ƒã ŋutɔ hã la, vidzĩ aɖewo vaa xexe sia me kple vovototo suesuesue siwo nɔa anyi tso wo dziɣi. Aleae, vevietɔ le mo, to, ŋku, alo akɔta me la, woyɔa nɔnɔme aɖe si ŋu míaƒo nu tsoe egbea be Goldenhar Syndrome. Mègavɔ̃ ne èse ŋkɔ sia o, míaƒo nu tso nusianu ŋu le mɔ bɔbɔe aɖe si gɔme nàte ŋu ase nu.

Nukae nye Goldenhar Dɔléle?

Kpuie ko la, Goldenhar Dɔléle nye dɔléle si mebɔ o, si wodzina . "Vidzidzi" fia be nɔnɔmea nɔa anyi le vidzidzi me. Wodae ɖe hatsotso si nye tagbɔ kple mo ƒe nɔnɔme me. Esia fia be enaa viwòa ƒe mo alo ta ƒe nɔnɔme alo eƒe tsitsi mesɔna o. Vevietɔ Goldenhar Dɔléle ate ŋu agblẽ nu le viwòa ƒe akɔta, to, kple ŋkuwo ŋu.

Zi geɖe la, ƒu kple lãmeka siwo le mo ƒe akpa ɖeka (si woyɔna hã be hemifacial microsomia) metsi nyuie o. Ɣeaɖewoɣi la, ate ŋu agblẽ nu le akpa evea siaa ŋu. Ðevi aɖewo hã ƒe nuyi me ʋuʋu alo aɖu me ʋuʋu.

Wotsɔ ŋkɔ nɛ be Goldenhar tsɔ de bubu numekula si ke ɖe nɔnɔmea ŋu zi gbãtɔ le ƒe 1952 me, si nye Maurice Goldenhar, ŋu. Atikewɔwɔ me ŋkɔ bubu si wotsɔ nɛ enye "oculo-auriculo-vertebral dysplasia." Ŋkɔa ɖi didi vie, alo? Mina míagbãe, alo?

• Oculo gɔmee nye nusi do ƒome kple ŋkuwo.
• Auriculo gɔmee nye nusi do ƒome kple to.
• Vertebral fia akɔta.
• Dysplasia nye ŋutilã ƒe akpa aɖe ƒe tsitsi si mesɔ o.

Fifia èse gɔmesese si le ŋkɔ sia ŋu gɔme, alo?

Aleke gbegbee nɔnɔme sia bɔe?

Le nyateƒe me la , Goldenhar Syndrome nye dɔléle si mebɔ kura o . Eŋutinunyalawo gblɔ be edzea vidzĩ yeye siwo ade ɖeka le 3,500 va ɖo 25,000 ɖesiaɖe me dzi. Wogblɔ hã be ebɔ vie le ŋutsuviwo me wu nyɔnuviwo.

Nukawoe hea Goldenhar Dɔlélea vɛ?

Nyabiase si le ame geɖe si enye "Nukatae esia dzɔna?" Ne míagblɔe tututu la, eŋutinunyalawo mekpɔ de dzesii haɖe o .Womenya nusi tututu gbɔ Goldenhar Dɔlélea tso o. Woxɔe se be domenyiŋusẽfianu ƒe tɔtrɔ gbɔe wòtso, gake menye ɣesiaɣie nusi gbɔ wòtso la me kɔna o. Le alafa memamã sue aɖe ŋutɔ me, abe 1% - 2% ene la, woate ŋu anyi nɔnɔmea ƒe dome tso dzila ɖeka alo evea siaa gbɔ.

Numekuku aɖewo ɖee fia be afɔku si le vidzĩa me be wòaxɔ Goldenhar Syndrome ate ŋu adzi ɖe edzi vie ne dɔléle aɖewo le vidadaa ŋu alo zã atike aɖewo le fufɔɣi. Le kpɔɖeŋu me:

• Suklidɔ le fufɔfɔ me.
• Atike aɖewo siwo wozãna na aklãdɔ, vevietɔ esiwo me retinoic acid le, abe isotretinoin (Accutane®) ene.

Eyata ne èfɔ fu la, ele vevie ŋutɔ be nàwɔ ɖe wò ɖɔkta ƒe mɔfiamewo dzi pɛpɛpɛ.

Nukae nye esia ƒe dzesiwo?

Goldenhar Dɔléle ƒe dzesiwo ate ŋu ato vovo tso ame ɖeka gbɔ yi bubu gbɔ. Gake dzesi siwo bɔ wu dometɔ ɖekae nye be mo ƒe akpa ɖeka metsi nyuie o . Wogayɔa esia be `hemifacial microsomia`, abe alesi míegblɔe va yi ene. Esia ate ŋu ana aɖu, ŋgonu, kple ŋku siwo le mo ƒe akpa ɖeka nanɔ sue eye womatsi wu akpa kemɛ o. Le kpɔɖeŋu me, ŋgonu ɖeka ate ŋu anɔ gbadzaa wu evelia, alo asike ate ŋu anɔ sue le akpa ɖeka.

Nu bubu siwo woate ŋu akpɔe nye:

• To ƒe dɔmawɔmawɔ nyuie: Ame aɖewo ƒe to ɖeka ate ŋu anɔ sue le mɔ si mesɔ o nu (si woyɔna be `microtia`). Ame bubuwo ateŋu anye be to ɖeka bu keŋkeŋ (si woyɔna be `anotia`). Esia ate ŋu ana ame ƒe to nasesẽ.
• Egblẽa nu le mo ƒe akpa eveawo ŋu: Ðevi siwo ŋu Goldenhar Syndrome le dometɔ siwo ade akpa etɔ̃lia kpɔa tsitsi sia si mewɔa dɔ nyuie o le mo ƒe akpa eveawo.
• Kuxi siwo le ŋutinu bubuwo ŋu: Kuxiwo ate ŋu ado mo ɖa le ayiku, dzi, lãkusi, alo ƒu siwo le akɔta (vertebrae) hã ŋu.
• Susu ƒe nuwɔametɔnyenye: Ame siwo ade 15% ate ŋu anye susu ƒe nuwɔametɔnyenye ƒe seƒe aɖe. Esia fia be nusɔsrɔ̃ ƒe kuxiwo, nugɔmesese ƒe ŋutete dzi ɖeɖe kpɔtɔ, kple bubuawo.

Tsɔ kpe ɖe esia ŋu la, woate ŋu akpɔ dzesi bubuwo:

• Nuyi si me ʋuʋu alo aɖu si me ʋuʋu le ƒe amesinɔnɔ.
• Dzi ƒe gbɔdzɔgbɔdzɔ siwo wodzina.
• Ɣeaɖewoɣi la, ƒu suesuesuewo (dermoid cysts) ate ŋu adzɔ ɖe ŋkua dzi.
• Tosese ƒe dɔmawɔmawɔ nyuie.
• Tsi ƒe ƒuƒoƒo ɖe taɖua me (Hydrocephalus).
• Alɔ̃dɔdɔ ƒe gbɔgbɔtsixetsixe si xea mɔ na ame.
• Ŋku ƒe akpa alo lãkusi bubu siwo le ŋkua me ƒe bu (coloboma) kple ŋku ƒe nukpɔkpɔ ƒe bu si dona tso eme.
• Scoliosis ƒe dɔléle.
• Nuƒoƒo ƒe kuxiwo.
• Ŋku siwo wotso (`Strabismus`).

Ðo ŋku edzi be menye amesiamee akpɔ dzesi siawo katã o. Ðewohĩ wo dometɔ ʋɛ aɖewo koe anɔ ame aɖewo si, eye woƒe sesẽme ate ŋu ato vovo.

Aleke nàwɔ anya ne Goldenhar Syndrome le ŋuwò?

Zi geɖe la, ɖɔktawo ate ŋu anɔ te ɖe gotagome dzesiwo abe mo kple to ƒe tɔtrɔ ene dzi anya Goldenhar Dɔlélea le vidzĩa dzidzi vɔ megbe.

Gake be ɖɔktaa naɖo kpe edzi geɖe wu eye wòakpɔe ɖa be ememe kuxi bubuwo hã li hã la, ate ŋu awɔ dodokpɔ ʋɛ aɖewo kpee. Esiawo dometɔ aɖewoe nye:

• CT-dɔdamɔnu: Esiawo ate ŋu ade dzesi tɔtrɔ siwo va le toa me ƒe wɔwɔme siwo ate ŋu ana ame ƒe to megale to ɖom o.
• Echocardiogram (echo test) alo electrocardiogram (EKG): Dodokpɔ siawo ate ŋu akpɔ alesi dzia le dɔ wɔm. Abe alesi míedzro eme va yi ene la, dzidɔ ate ŋu adzɔ ɣeaɖewoɣi.
• Ŋkudodokpɔ: Wowɔa dodokpɔ siawo be woatsɔ akpɔe ɖa be nusiwo mesɔ o le ŋkua me hã.
• Ultrasound kple X-ray: Esiawo ate ŋu akpɔe ɖa be tɔtrɔ aɖewo le tagbɔ, akɔta, lãkusi, alo ayiku me hã.
• Domenyiŋusẽfianu dodokpɔ: Dodokpɔ siawo kpena ɖe ame ŋu be woaɖe domenyiŋusẽfianu bubu siwo ate ŋu ahe dzesi mawo tɔgbe vɛ la ɖa.
• Alɔ̃dɔdɔ ŋuti numekukuwo: Wowɔa esiawo tsɔ léa ŋku ɖe alɔ̃dɔdɔ ƒe gbɔgbɔtsixetsixe si xea mɔ na ame ŋu hã.

Ðe woate ŋu ade dzesi esia hafi woadzi vidzĩa?

Esia mebɔ kura o. Gake ɣeaɖewoɣi le ultrasound scan wɔwɔ do ŋgɔ na vidzidzi me la, ɖɔktawo ate ŋu ade dzesi tɔtrɔ le fugboea ƒe aɖu, to, alo nu me. Ne edzɔ alea la, woate ŋu alé ŋku ɖe eŋu geɖe wu.

Nukawoe nye atike siwo wozãna na esia?

Goldenhar Dɔlélea dada toa vovo le dzesiawo nu . Menye ɖe wowɔa nu ɖe ​​amesiame ŋu ɖekae o. Ðewohĩ mahiã be woawɔ atike tɔxɛ aɖeke na ɖevi aɖewo ne woƒe dzesiwo nu mesẽ kura o. Gake wowɔa ɖoɖo ɖe atikewɔwɔ ŋu to eŋutinunyala vovovowo ƒe kpekpeɖeŋu me, ne ehiã.

Atikewɔwɔ ƒe mɔnu aɖewoe nye esi:

• Kpekpeɖeŋu nana vidzĩwo: Ate ŋu asesẽ na vidzĩ aɖewo be woano no. Le nɔnɔme mawo me la, ate ŋu ahiã be woatsɔ aŋetu tɔxɛwo alo nasogastric (NG) nuɖuɖu nana wo.
• Nukpɔkpɔ ƒe nyonyo: Àte ŋu azã ŋkumeɖobolo alo awɔ dɔ na wò be wò nukpɔkpɔ nanyo ɖe edzi.
• Tomemɔ̃wo: Woate ŋu ana tosese nanyo ɖe edzi ne wozã tomemɔ̃ alo tomemɔ̃ siwo wotsɔ ƒu bla ɖe tome.
• Nuƒoƒo ƒe atikewɔwɔ:Esia le vevie ŋutɔ na gbegbɔgblɔ kple dzeɖoɖo ƒe ŋutetewo tutuɖo.
• Amekoko: Woate ŋu awɔ dɔ na ame be woatsɔ aɖɔ dzidɔ, nuyi alo aɖu ƒe gbagbã, alɔ̃dɔdɔ ƒe gbɔgbɔtsixetsixe, to suewo (microtia), alo aklã ƒe dɔmawɔmawɔ nyuie ɖo.

Wowɔa esiawo katã be woatsɔ ana ɖevia ƒe agbenɔnɔ nanyo ɖe edzi alesi woate ŋui. Eya ta ele vevie ŋutɔ be míawɔ ɖe aɖaŋuɖoɖo siwo ɖɔkta la ɖo dzi.

Ðe woate ŋu aɖɔ mo ƒe tɔtrɔwo ɖoa?

Ẽ, esia nye kuxi na dzila geɖe. Woate ŋu awɔ atsyɔ̃ɖoɖo ƒe amekoko be mo ƒe dzedzeme nasɔ ɖe wo nɔewo nu wu. Wowɔa dɔ na vidzĩ geɖe siwo ŋu Goldenhar Syndrome le be woatrɔ woƒe aɖu, ŋgonu, to, alo ŋgonu ƒe dzedzeme. Zi geɖe la, wowɔa esia ne ɖevia tsi vie, le ƒe si sɔ me.

Ðe woate ŋu axe mɔ ɖe Goldenhar Dɔlélea nua?

Abe alesi míedzro eme va yi ene la, mɔ si ŋu kakaɖedzi le meli si dzi woato axe mɔ ɖe nɔnɔme sia nu o elabena womenya nusi tututu gbɔ wòtso haɖe o . Gake ele vevie be nàwɔ ɖe wò ɖɔkta ƒe aɖaŋuɖoɖowo katã dzi le fufɔɣi. Vevietɔ mègazã atike siwo me retinoic acid le (abe aklãdɔtike aɖewo ene) wò ɖɔkta ƒe aɖaŋuɖoɖo manɔmee o. Ele vevie hã be nàɖu nuɖuɖu si naa lãmesẽ.

Ne Goldenhar Syndrome le wò ƒomea me la, anyo be nàxɔ domenyiŋusẽfianu ŋuti aɖaŋuɖoɖo. Emegbe ne èle ɖoɖo wɔm be yeadzi vi la, àte ŋu ase afɔku si le ɖevi ma me be wòaxɔ nɔnɔme sia gɔme.

Aleke agbea anɔ le nɔnɔme sia me?

Esia ate ŋu aɖi vɔvɔ̃ na wò vie. Gake togbɔ be amesiwo ŋu Goldenhar Dɔlélea le ƒe etsɔme le vovovo hã la, nu nyui aɖe dona tso eme na ame akpa gãtɔ . Ne wowɔ atike nyuitɔ na ɖevi siwo ŋu dɔléle sia le la, zi geɖe la, woate ŋu anɔ agbe si sɔ. Wote ŋu srɔ̃a nu, ƒoa fefe, eye wokpɔa gome le hadomegbenɔnɔ me.

Nu kawoe nèdi be yeabia ɖɔktaa?

Ne èva nya be dɔléle sia le viwòa ŋu la, nyabiase geɖe ate ŋu anɔ asiwò. Ɣemaɣi la, mègavɔ̃ be yeabia nya siawo wò ɖɔkta o:

• "Ðɔkta/Aƒenɔ, nukawoe nye Goldenhar Syndrome ƒe dzesi veviwo?"
• "Dodokpɔ kawoe wòle be mawɔ be makpɔ egbɔ be esia le vinyea ŋu?"
• "Nukae nye atikewɔmɔnu siwo woate ŋu azã na esia? Nukae nyo na vinyea?"
• "Teƒe kawoe nye esiwo ŋu kakaɖedzi le be nàsrɔ̃ nu geɖe tso nɔnɔme sia ŋu?"
• "Ðe habɔbɔ alo dzilanyenye ƒe ƒuƒoƒo aɖewo li siwo kpena ɖe ɖeviwo kple ƒomewo ŋu le nɔnɔme sia ƒomevi mea?"

Nya siawo biabia akpe ɖe ŋuwò nàse nɔnɔmea gɔme nyuie wu eye wòana nu nyuitɔ kekeake nasu viwòa si.

Ðe Goldenhar Dɔlélea nye nuwɔametɔa?

Ẽ, ɣeaɖewoɣi la, esiaWoate ŋu abu nuwɔametɔ be enye nuwɔametɔ. Le kpɔɖeŋu me, ne nusese alo nukpɔkpɔ ƒe kuxi aɖe li la, ate ŋu anye nuwɔametɔ. Nenema ke ne susu ƒe nuwɔametɔ aɖe li la, ate ŋu ahiã be woakpe ɖe ame ma ŋu geɖe wu be wòate ŋu awɔ gbesiagbedɔwo ahasrɔ̃ nu. Eyata enye míaƒe agbanɔamedzi abe habɔbɔ ene be míana dɔwɔƒe kple kpekpeɖeŋu siwo hiã wo.

Nɔnɔme bubu kawoe ƒe dzesi mawo tɔgbe dzena le wo ŋu?

Nɔnɔme bubu geɖewo li siwo ƒe dzesiwo sɔ kple Goldenhar Dɔlélea tɔ. Eyata ele vevie ŋutɔ be woakpɔ dɔlélea nyuie. Nɔnɔme siawo dometɔ aɖewoe nye:

• Branchio-oto-ayiku ƒe dɔléle `(Branchioootorenal (BOR) ƒe dɔléle)`
• CHARGE Habɔbɔ
• Townes-Brocks ƒe dɔlélea
• Ameflutɔ Collins ƒe dɔlélea
• VACTERL Habɔbɔ

Togbɔ be esia aɖi nya sesẽ vie hã la, ɖɔktawo bua nusiawo katã ŋu ne wole dɔlélewo dim.

Nusi dzi wòle be míaɖo ŋkui tso nusiwo ŋu míeƒo nu tsoe me!

Goldenhar Dɔléle nye dɔléle si mebɔ o si nɔa anyi le vidzidzi me. Vidzĩ ƒe mo, ta, kple ɣeaɖewoɣi ŋutinu ememetɔwo ƒe nɔnɔme koŋue wògblẽa nu le. Ðɔktawo ate ŋu aɖɔ mo alo aklã ƒe gbɔdzɔgbɔdzɔ aɖewo ɖo le vidzĩme to amekoko me.

Nu vevitɔe nye be ɖewohĩ mahiã be woawɔ atike tɔxɛ na ɖevi aɖewo ne woƒe dzesiwo mesẽ o. Eye zi geɖe la, amesiwo ŋu Goldenhar Dɔlélea le la nɔa agbe le dzidzɔ me, eye woƒe agbenɔnɔ naa dzidzeme kple atikewɔwɔ kple kpekpeɖeŋu si hiã.

Ne èsusu be dzesi siawo dometɔ aɖe le viwòa ŋu la, mègatsi dzodzodzoe o, ke yi ɖɔkta gbɔ enumake hena aɖaŋuɖoɖo. Ne èkpɔ dɔlélea kaba eye nèdae nyuie la, ate ŋu ana etsɔme nyui nanɔ viwòa ŋu.

` Goldenhar ƒe dɔléle, nɔnɔme si wodzina, vidzidzi ƒe gbɔdzɔgbɔdzɔ, tagbɔ kple mo, mo ƒe akpa eve ƒe microsomia, oculo-auriculo-vertebral dysplasia, ɖeviwo ƒe lãmesẽ