Ðe viwòa ƒe ŋutigbalẽ biã eye wòvuvua? Mina míasrɔ̃ nu tso Klippel-Trenaunay Dɔléle (KTS) ŋu!

Ède dzesii kpɔ be wodzia vidzĩ aɖewo kple dzigbedzesi gã dzĩ aɖe le woƒe ŋutilã ƒe akpa ɖeka, vevietɔ le alɔ alo afɔ dzi, eye be alɔ/afɔ lolo vie wu akpa kemɛa? Alo ɣeaɖewoɣi la, àte ŋu akpɔ ʋukawo le akpa ma? Esɔ be nàvɔ̃ vie ne èkpɔ nane si le abe esia ene. Egbea míele nu ƒom tso nɔnɔme aɖe si ɖea dzesi mawo tɔgbe fiana, si mebɔ vie o, si fia be menye amesiamee wòxɔna o, eye wònye nusi wodzina. Woyɔa esia be Klippel-Trenaunay Dɔléle (KTS) . Mègatsi dzi o, míaƒo nu tso esia ŋu bɔbɔe, le mɔ si gɔme nàte ŋu ase nu.

Nukae nye Klippel-Trenaunay Dɔléle (KTS)? Kpuie ko la,...

Bu eŋu kpɔ, KTS nye nɔnɔme si mebɔ o si vaa mía vidzĩa gbɔ ne eva xexe sia me (si fia be, nɔnɔme si wodzina ). Esia hea tɔtrɔ sue aɖewo vaa alesi vidzĩa ƒe lãmeka falɛfalɛwo, ƒuwo, kple ʋukawo tsinae me. Esia ƒe dzesi veviwo dometɔ ɖekae nye dzigbedzesi aɖe si ƒe amadede nye aŋutiɖiɖi, si zi geɖe le abɔ alo afɔ ɖeka dzi, si woyɔna be ‘port-wine stain’ . Zi geɖe la, kuxi aɖewo hã doa go amesiwo ŋu KTS sia le le woƒe ʋukawo me . Lãmetsiŋusẽ sia nye ɖoɖo vevi aɖe si kpena ɖe míaƒe ŋutilã me tsiwo ŋu be woada asɔ.

Fifia la, atike aɖeke meli na KTS o. Ke hã, mègatsi dzodzodzoe o. Atikewɔwɔ nyui geɖe li woatsɔ akpɔ dzesiawo gbɔ . Ne wokpɔ dɔlélea eye wodae kaba ale si woate ŋui, abe le vidzĩa dzidzi vɔ megbe ene la, woate ŋu aɖe lãmesẽkuxi siwo ate ŋu ado mo ɖa le KTS ta dzi akpɔtɔ ŋutɔ.

Ðɔkta aɖewo hã yɔa KTS ƒe nɔnɔmea be CLVM . Esiae nye Eŋlisigbe me nya ene ƒe ŋɔŋlɔdzesi gbãtɔ.

• C nye na Capillaries - esiawo nye ʋuka sue siwo doa ka kple míaƒe ʋukawo kple ʋukawo.
• L tsi tre ɖi na Lymphatic system - Esia nye míaƒe dɔlélenutsiŋutete ƒe akpa aɖe. Etsɔa tsi si woyɔna be lymph yia ŋutilã bliboa me.
• V tsi tre ɖi na Veins - esiawo nye ʋuka siwo tsɔa ʋu yia míaƒe dzi me.
• Ŋɔŋlɔdzesi M tsi tre ɖi na Malformation . Efia be ŋutilã ƒe akpa aɖe metsina le mɔ si sɔ nu o, alo be nuwɔametɔ aɖe le eme.

Franseawo ƒe ɖɔkta eve siwo nye Maurice Klippel kple Paul Trenaunay, siwo ke ɖe dɔlélea ŋu le ƒe 1900. Eŋutinunyalawo bu akɔnta be ame 1 le 100,000 ɖesiaɖe me le xexeame katã ƒe ŋkɔe wotsɔ na KTS. Ate ŋu akpɔ ŋusẽ ɖe ame sia ame dzi, metsɔ le ameƒomevi alo ŋutsu alo nyɔnu nyenye me o.

Nukawoe nye KTS ƒe dzesiwo? Aleke nèwɔ dea dzesii?

Klippel-Trenaunay Syndrome (KTS) ƒe dzesiwo gblẽa nu le míaƒe ŋutilã ƒe ʋukawo, ʋuka siwo me ʋu sina tona, lãkusi falɛfalɛwo, ƒuwo, kple ʋuka siwo me ʋu sina tona ŋu koŋ. Na míakpɔ alesi:

1. Ʋukawo ƒe Dɔmawɔmawɔ nyuie (CM):

Afi siae ‘port-wine stain’ si míeyɔ va yi la dzɔna le. Esia tsoa ʋuka siwo le míaƒe ŋutigbalẽ te ƒe vuvu gbɔ. Dzidzimedzesi siawo ate ŋu anye dzesi gbãtɔ siwo fia be KTS le ŋuwò la dometɔ ɖeka . Teƒe siawo ate ŋu anɔ amadede vovovowo me, tso pink si me kɔ dzi va ɖo aɖabɛ dzĩ (wein-dzẽ) dzi. Ne èle tsitsim la, teƒe siawo ate ŋu anɔ abe ʋuʋudedi ene, woakɔ ɖe edzi, alo ado viviti.

2. Ʋukawo ƒe Dɔmawɔmawɔ nyuie (VM):

Amesiame kloe si ŋu KTS le la ƒe ʋukawo ƒe dɔmawɔmawɔ nyuie. Esiawo ate ŋu akpɔ ŋusẽ ɖe ʋuka siwo le ŋutigbalẽa tame vie dzi, si ana ʋuka siwo me ʋu sina tona le afɔwo me, vevietɔ le akɔta kple ata. Woate ŋu akpɔ ŋusẽ ɖe ʋuka goglo siwo sina yia ŋutilãa me goglo hã dzi. Esia ate ŋu ana ʋu naƒo ɖe ʋuka gogloawo me (Deep Vein Thrombosis - DVT) . DVT nye nɔnɔme si me afɔku le.

3. Lãmetsi falɛfalɛwo kple ƒu ƒe tsitsi fũ akpa:

Esia nye nɔnɔme si me, tso ɖevime ke la, zi geɖe la, alɔ alo afɔ ɖeka lolona wu evelia. Zi geɖe la, alɔ alo afɔ ɖeka koe esia gblẽa nu le, eye zi geɖe la, afɔ ɖeka koe wògblẽa nu le. Ɣeaɖewoɣi la, afɔ ɖeka ate ŋu adidi wu evelia. Esia ate ŋu ana màda asɔ o, azɔlizɔzɔ nasesẽ na wò, eye wòate ŋu aʋã wò hã.

4. Lãmetsiŋusẽ ƒe Dɔmawɔmawɔ nyuie (LM):

Ame aɖewo siwo ŋu KTS le ate ŋu akpɔ ʋuka bubuwo le woƒe ʋukawo me, alo ʋuka siwo li fifia ƒe nɔnɔme ate ŋu asɔ. Esi ʋuka siawo siwo kpe ɖe eŋu mewɔa dɔ nyuie o ta la, ʋuka me tsi ate ŋu ado , si ana afɔwo navuvu, vevietɔ afɔwo, alo kuxiwo nado mo ɖa le akɔta, tsinyegoe, alo dɔgbo ƒe akpa si le ete.

Nukae hea KTS vɛ? Nukatae esia dzɔna ɖo?

Zi geɖe la, domenyiŋusẽfianu si woyɔna be PIK3CA ƒe tɔtrɔ gbɔe KTS tsona. Domenyiŋusẽfianu ƒe tɔtrɔ sia dzɔna ɣeaɖewoɣi, si fia be womenya nusi gbɔ wòtso o. Menye dzilawo gbɔe wònyi o . Esia fia be ne dzila eveawo dometɔ aɖeke mexɔ KTS o hã la, ɖevi ate ŋu axɔ KTS le domenyiŋusẽfianu ƒe tɔtrɔ sia ta.

KTS le ame aɖewo ŋu siwo me PIK3CA domenyiŋusẽfianu ƒe tɔtrɔ sia mele o. Eyata numekulawo xɔe se be ɖewohĩ domenyiŋusẽfianu bubu geɖe ƒe tɔtrɔ gbɔe KTS tso. Wogale numekuku wɔm tso esia ŋu kokoko.

Nukawoe nye kuxi siwo ate ŋu ado tso KTS me?

KTS ate ŋu ahe kuxi vovovowo vɛ. Esia tae atikewɔwɔ kaba le vevie ɖo. Mina míalé ŋku ɖe nusiwo nye kuxi mawo ŋu:

• Ʋu ƒe sisi: Ate ŋu adzɔ, vevietɔ le ʋuka goglowo (DVT) me.
• Cellulitis: Esia nye dɔlékuiwo ƒe dɔléle si dzɔna ɖe ŋutigbalẽa te.
• Lãmetsi ƒe ƒoƒo ƒu kple vuvu (Lymphedema): Etsoa ʋukawo ƒe dɔwɔwɔ ƒe gbegblẽ gbɔ.
• Ʋu si dona le ememe: Ʋu ate ŋu ado le dɔgboa (GI) me, tsinyegoe, alo nyɔnuwo ƒe vidzidzi.
• Aklã me ʋu ƒe sisi: Esia nye nɔnɔme si dea ame ƒe agbe afɔku me si me ʋu si ƒona ɖe ʋuka gogloawo me la gbãna eye wòxea ʋuka aɖe si le lãkusi me.

Ƒã ŋutɔ hafi amesiwo ŋu KTS le ate ŋu akpɔ vidzidzi ƒe gbɔdzɔgbɔdzɔ le woƒe asiwo alo afɔwo me. Le kpɔɖeŋu me:

• Asibidɛ bubuwo ƒe amesinɔnɔ (Polydactyly) .
• Asibidɛwo lé ɖe wo nɔewo ŋu (Syndactyly) .

Ðe KTS hea vevesese vɛa?

Ẽ, KTS ate ŋu anye vevesese.

• Varicose veins ate ŋu ana ame naʋuʋu alo ave ame.
• Ʋu ƒe sisi ƒe kuxiwo ate ŋu ana ame navuvu eye wòase veve, vevietɔ le afɔ siwo le ete.
• Ŋutinu aɖe, abe afɔ ene, ƒe tsitsi fũ akpa ate ŋu ana afɔ ma nase le eɖokui me be yeƒe kpekpeme le kpekpem eye wòase veve.

Aleke ɖɔktawo wɔa dea dzesi KTS? (Dɔléle ƒe dzesi) .

Ðɔktawo nɔa te ɖe ŋutilã me dzesiwo dzi dea dzesi KTS gbã. Wosusua KTS ne kuxiwo le nu vevi etɔ̃ siwo ŋu míeƒo nu tsoe va yi la dometɔ eve ya teti me — ʋuka siwo me ʋu sina tona, ʋukawo, alo abɔ kple afɔ ƒe tsitsi. Esi wònye be KTS ƒe dzesi geɖe dzena le vidzidzi me ta la, ate ŋu adzɔ be nànya KTS hafi viwòa nadzo le kɔdzi.

Dodokpɔ kawoe wozãna tsɔ dea dzesi KTS?

Le dzesiawo nu la, ɖɔkta ate ŋu awɔ dodokpɔwo abe esiawo ene atsɔ aɖo kpe dɔlélea dzi geɖe wu eye wòanya alesi gbegbe kuxia keke tae:

• CT scan alo MRI scan: Kpɔ alesi lãmeka falɛfalɛwo kple ƒuwo le ɖa.
• Magnetic Resonance Angiogram (MR angiogram): Esia nye MRI dodokpɔ tɔxɛ aɖe si te ŋu kpɔa ʋukawo kple ʋukawo ƒe nɔnɔme nyuie.
• Doppler ultrasound: Esia kpena ɖe ame ŋu wòkpɔa ʋu si le sisim to ʋukawo kple ʋukawo me.

Nukawoe nye atike siwo wozãna na KTS?

Klippel-Trenaunay Syndrome (KTS) ƒe atikewɔwɔ toa vovo le dzesi siwo le ame ɖesiaɖe ŋu nu.. Menye amesiamee wowɔa nu ɖe ​​ame ŋu ɖekae o. Taɖodzinu vevitɔ si le atikewɔwɔ ŋue nye be woaɖu dzesiawo dzi eye woaɖe kuxi siwo ate ŋu ado mo ɖa dzi akpɔtɔ. Mina míakpɔe ɖa be atikewɔmɔnu vevi siwo li:

• Atike siwo nana ʋu mekɔna o / Atike siwo tsia ʋu ƒe sisi nu : Le kpɔɖeŋu me, atikewo abe Heparin ene . Esiawo ɖea ʋu ƒe sisi le afɔwo me (DVT) kple aklã me ʋu ƒe sisi ƒe afɔkua dzi kpɔtɔna.
• Sirolimus: Zi geɖe la, wozãa atike sia tsɔ xea mɔ na ŋutilãa be wòagagbe ŋutinu si wodo na ame o. Gake wokpɔe hã be enaa ʋukawo ƒe dɔmawɔmawɔ nyuie megagblẽna ɖe edzi o.
• Compression stockings: Esiawo nye sox ƒomevi tɔxɛ aɖewo siwo kpena ɖe ʋu ŋu wòtrɔna tso afɔwo me yia dzia me. Esia ate ŋu akpe ɖe ame ŋu be wòaɖe abi, vevesese, kple ʋu ƒe sisi ƒe afɔkua dzi akpɔtɔ.
• Endovenous thermal ablation: Esia bia be woazã ŋusẽ ƒe keklẽ siwo ŋu wotsia dzi ɖo atsɔ axe ʋuka siwo me kuxi le la tso ememe. Wowɔa esia esime ʋukaawo gakpɔtɔ le afima. Esia wɔnɛ be ɣeyiɣi si woatsɔ ahaya la dzi ɖena kpɔtɔna eye vevesese hã dzi ɖena kpɔtɔna.
• Laser-dɔdamɔnu: Woate ŋu azã ŋusẽ ƒe keklẽ sesẽ siwo ŋu woƒe susu le atsɔ agblẽ lãmenugbagbevi siwo womedi o la dome alo aɖe wo ɖa. Wozãa laser-dɔdamɔnu sia tsɔ naa ‘port-wine stain’ dzigbedzesi ƒe amadede nakɔ.
• Sclerotherapy: Le esia me la, wodoa atike tɔxɛ aɖe ɖe ʋuka, ʋuka siwo me ʋu sina tona, alo ʋuka siwo me ʋu sina tona me tẽ. Emegbe nugoawo nu tsona. Esia nye atike si wɔa dɔ ŋutɔ na ʋuka siwo me ʋu sina tona.
• Afɔkpa kɔkɔwo: Ne wò afɔwo ƒe didime mesɔ o la, àte ŋu atsɔ afɔ kɔkɔ aɖe aɖo afɔkpa ɖeka dzi atsɔ aɖɔ esia ɖo. Esia ate ŋu akpe ɖe ame ŋu hã be woaxe mɔ ɖe ƒunukpeƒetetedɔ nu .
• Amekoko: Woate ŋu awɔ amekoko be woatsɔ aɖɔ kuxi siwo le ʋukawo ŋu ɖo eye woana afɔawo ƒe didime nasɔ. Alo, woate ŋu awɔ dɔ nɛ atsɔ aɖe ami alo lãkusi si sɔ gbɔ ɖe eme ɖa be woaɖe alɔ alo afɔ si tsi akpa ƒe lolome dzi akpɔtɔ. Ƒã hafi ne afɔbidɛ si lolo le mɔ si mesɔ o nu na wòsesẽna be woado afɔkpa alo azɔ la, woate ŋu awɔ dɔ na afɔbidɛa.

Ðe woate ŋu axe mɔ ɖe KTS nua?

Nublanuitɔe la, esi wònye be KTS dzɔna le vome ta la, mɔ aɖeke meli si dzi woato axe mɔ nɛ be wòagadzɔ o . Gake abe alesi míegblɔe va yi ene la, atikewɔwɔ nyuie ate ŋu akpe ɖe amesiwo ŋu KTS le ŋu be woanɔ agbe si de ŋgɔ wu.

Etsɔme ka ƒomevie amesi ŋu KTS le ate ŋu akpɔ mɔ na?

Togbɔ be atike aɖeke meli na KTS o hã la, woate ŋu akpɔ dzesiawo gbɔ. Zi geɖe la, amesiwo ŋu dɔlélea le nɔa agbenɔƒe si bɔ .

Gake KTS ƒe mɔkpɔkpɔ ate ŋu ato vovo tso ame ɖeka gbɔ yi bubu gbɔ. Enɔ te ɖe alesi wò ʋukawo ƒe dɔmawɔmawɔ nyuie nu sẽe dzi. Esiawo ate ŋu agblẽ ɖe edzi le ɣeyiɣi aɖe megbe. Eyata ne ʋu si le dodom le GI me, ʋuka goglowo me ʋu si le dodom le ŋuwò, alo aklã me ʋu ƒe sisi , ele be nàyi atikewɔƒe enumake . Ʋu ƒe sisi alo ʋu si le dodom fũ ate ŋu awu ame.

Atikewɔlawo ƒe aɖaŋuɖoɖo kple atikewɔwɔ edziedzi ate ŋu aɖe kuxiwo ƒe afɔkua dzi akpɔtɔ ŋutɔ.

Ne KTS le ŋunye la, aleke mawɔ akpɔ ɖokuinye dzii? / Aleke mawɔ akpɔ vinye dzi?

Ne KTS le ŋuwò alo viwòa ŋu la, nya nu tso nusiawo ŋu:

• Lé be na wò ŋutigbalẽ nyuie: Ele vevie be nàxe mɔ ɖe dɔlékuiwo xɔxɔ nu.
• Zi geɖe la, womeɖoa aɖaŋu be woazã lãmetsiŋusẽ ƒe mɔxexeɖefufɔfɔnu ƒe mɔnu siwo me estrogen le o: Woate ŋu ana ʋu ƒe sisi ƒe afɔkua nadzi ɖe edzi. Ðo dze kple wò ɖɔkta tso esia ŋu.
• No atike siwo axe mɔ na ʋu ƒe sisi le fufɔɣi: Wò ɖɔkta aɖo aɖaŋu na wò le esia ŋu.
• No atike siwo nana ʋu mekɔna o hafi nàwɔ dɔ nɛ: Ðe ʋu ƒe sisi ƒe afɔkua dzi kpɔtɔ.

Ɣekaɣie wòle be makpɔ ɖɔkta?

Kpɔ wò ɖɔkta ɖa edziedzi le wò agbemeŋkekewo katã me be woado wò kpɔ . Esia ana wò ɖɔkta nalé ŋku ɖe wò nɔnɔmea ŋu ahada kuxi yeye ɖesiaɖe si ate ŋu ado mo ɖa. Dodokpɔ edziedzi akpe ɖe wò ɖɔkta ŋu wòakpɔ alesi wò atikewɔwɔa le dɔ wɔm nyuie eye wòawɔ tɔtrɔ le wò atikewɔwɔ ƒe ɖoɖoa ŋu ne ehiã.

Ɣekaɣie wòle be mayi Atikewɔwɔ Kpata ƒe Dɔwɔƒe (ETU) ?

Ne èsusu be ʋu le dodom le ye ŋu (e.g., DVT, Pulmonary Embolism ƒe dzesiwo) alo ne ʋu le dodom le ye ŋu vevie (ʋu le dodom vevie) la, ele be nàyi xɔ si me wowɔa dɔ kpata le enumake.

Nya vevi kawoe wòle be míabia ɖɔktaa?

Ne KTS le ŋuwò alo viwòa ŋu la, àte ŋu abia nya aɖewo abe:

• Atikewɔwɔ kawoe nyo na nye/vinye?
• Zi nenie wòle be mava do wo kpɔ?
• Ne míebu nɔnɔme si me mele fifia ŋu la, nya kae nàte ŋu agblɔ tso nye etsɔme ŋu (nyagblɔɖi/nukpɔsusu)?

Ðe KTS ate ŋu ade ame ƒe agbe afɔku mea?

KTS ŋutɔ mekpɔa ŋusẽ ɖe agbenɔƒe dzi tẽ o. Gake kuxi aɖewo siwo ate ŋu ado mo ɖa le KTS ta, abe ʋu si le dodom le ememe alo aklã me ʋu ƒe sisi ene, ate ŋu ade ame ƒe agbe afɔku me . Atikewɔwɔ na afɔku siawo edziedzi ate ŋu aɖe kuxi siwo ate ŋu ado mo ɖa dzi akpɔtɔ.

Ðe KTS nye nuwɔametɔa?

Ate ŋu adzɔ. Ne mète ŋu le dɔ wɔm o le KTS ƒe kuxiwo, abe deep vein thrombosis (DVT) alo aklã me ʋu ƒe sisi ene ta la, àte ŋu adze na nuwɔametɔnyenye ƒe kpekpeɖeŋu. Àte ŋu adze na viɖewo abe nuwɔametɔwo ƒe ʋutɔɖoƒe ƒe dzesi ene hã na kuxiwo abe azɔlizɔzɔ ƒe kuxiwo le abɔ kple afɔ siwo lolo ta ene.

Ate ŋu asesẽ be woase KTS ƒe dzesiwo kple kuxiwo katã gɔme zi ɖeka. Ke hã, wò ɖɔkta ate ŋu aɖe wo katã me na wò eye wòakpe ɖe ŋuwò nàda wò kuxiwo. Mègavɔ̃ be yeana yeƒe ɖɔkta nanya alesi nèle sesem le ɖokuiwò me kple dzesi yeye ɖesiaɖe si nèle dzedzem o . Nuƒoƒo faa alea ana wòanɔ bɔbɔe be nàbia kpekpeɖeŋu ne kuxi aɖewo do mo ɖa.

Nusi míedi be míatsɔ ayi aƒemee tso ŋutinya sia me (Take-Home Message) .

Enyo, eyata míeƒo nu geɖe tso KTS ŋu, alo? Nu ʋɛ aɖewo siwo wòle be wòanɔ susu me na wòe nye esi:

• KTS nye dɔléle si mebɔ o, si wodzina . Eyata mègatsi dzi o, menye wò ɖeka koe o.
• Dzesi vevitɔwoe nye dzigbedzesi si nye ‘port-wine stain’, alɔ/afɔ si lolo, kple ʋuka me kuxiwo .
• Togbɔ be womekpɔ atike blibo aɖeke na esia o hã la, atike nyui ŋutɔ aɖewo li siwo dzi woato aɖu dzesiawo dzi .
• Ele vevie ŋutɔ be woakpɔ dɔlélea ahadze edada gɔme kaba ale si woate ŋui .
• Ele vevie be woaxɔ atikewɔlawo ƒe dzikpɔkpɔ ɣesiaɣi ahawɔ atike si hiã le agbemeŋkekewo katã me.
• Lé kadodo nyui me ɖe asi kple wò ɖɔkta. Bia nya ɖesiaɖe si le asiwò, eye nàgblɔ alesi nèsena le ɖokuiwò me na wo.

Mele mɔ kpɔm be nyatakaka sia kpe ɖe ŋuwò nèse KTS gɔme nyuie wu. Ne dzesi siawo le wò alo ame aɖe si nènya ŋu la, anyo wu be nàdi atikewɔlawo ƒe aɖaŋuɖoɖo.

` Klippel-Trenaunay Syndrome, KTS, port-wine stain, dzigbedzesi, ʋukawo ƒe dɔmawɔmawɔ nyuie, ʋukawo ƒe dɔwɔwɔ, abɔ kple afɔ ƒe tsitsi fũ, vidzidzi ƒe dɔléle, dzigbedzesi dzĩ, ʋukawo ƒe dɔmawɔmawɔ nyuie, ʋukawo ƒe dɔwɔwɔ, abɔ kple afɔ ƒe tsitsi fũ, domenyiŋusẽfianu ƒe dɔléle, CLVM