Ingaba umntwana wakho omncinci unengxaki yokuphefumla ngolu hlobo? Masithethe nge "Double Aortic Arch"!

Ngaba umntwana wakho uziva ngathi unengxaki yokuphefumla ngamanye amaxesha? Ngaba uyakrwitshwa xa esitya okanye encancisa? Okanye wenza iingxolo ezingaqhelekanga xa ephefumla? Nangona ezi zisenokuba zizifo eziqhelekileyo zabantwana, kunokubakho imeko embi kakhulu, kodwa enokunyangeka emva koku. Enye imeko engaqhelekanga kodwa ebalulekileyo ekufuneka uyiqaphele ibizwa ngokuba yi- "Double Aortic Arch." Makhe sithethe ngayo ngokweenkcukacha namhlanje.

Ngamafutshane, umthambo wegazi oyintloko othwala igazi ukusuka entliziyweni yomntwana wakho ukuya emzimbeni wakhe wonke yi -aorta . Ngokwesiqhelo ngumzimba omnye, omkhulu. Inxalenye egobileyo yalo mthambo ngaphezu kwentliziyo ibizwa ngokuba yi-aortic arch .

Nangona kunjalo, kumntwana one-"Double Aortic Arch", le "aortic arch" yenziwa kabini. Oko kukuthi, umthambo wegazi owongezelelweyo uqhagamshelwe kule "aortic arch" iphambili, yenza i-arch yesibini. Ezi arch zimbini ziyadibana, zenze indandatho ejikeleze umbhobho womoya womntwana (i-trachea) kunye nombhobho wokutya (i-esophagus) . Khawucinge, kwenzeka ntoni ukuba le ring ivalekile? Kulapho umntwana eqala ukuba neempawu ezinzima ezifana nobunzima bokuphefumla, ukwenza ingxolo ngelixa ephefumla, kunye nobunzima bokuginya ukutya.

Le "aortic arch" yenziwa kwisigaba sokuqala sokukhulelwa, phakathi kweeveki ezimbini ukuya kwezisixhenxe. Ngeli xesha, intliziyo kunye nemithambo yegazi zikhula ngendlela entsonkothileyo kakhulu. Ezinye iindawo zenziwa, kwaye emva kwexesha elithile azisafuneki kwaye ziyanyamalala. "I-double aortic arch" yenziwa ngenxa yento efana nale. Malunga neveki yesihlanu yokukhulelwa, umthambo wegazi obekufanele ukuba unyamalale, ngesizathu esingaziwayo, awunyamalali kwaye le "double aortic arch" yenziwa. Esi sisiphene sentliziyo esizalwa naso , oko kuthetha ukuba umntwana uzalwa naso.

Le meko, ebizwa ngokuba yi-"double aortic arch," ikwiqela leziphene zentliziyo ezizalwa nazo ezibizwa ngokuba yi- "vascular rings." Ezi zibangela ukuba umbhobho womoya kunye nomqala womntwana zicinezelwe.

Zithini ezi "rings zemithambo yegazi"?

Kulungile, "iiVascular Rings", njengoko benditshilo ngaphambili, ziimeko apho "i-aortic arch" ikhula ngendlela engaqhelekanga, nto leyo enokubangela ukuba umbhobho womoya womntwana (i-trachea), umbhobho wokutya (umphimbo), okanye zombini zicinezelwe. Ezi zizinto ezingaqhelekanga kakhulu ezibangela ukuba intliziyo ikhule. Cinga ngako, malunga nomntwana omnye kwabayi-100 uzalwa enesimo esithile sentliziyo esibangela ukuba intliziyo ikhule. Kodwa "i-vascular ring" ichaphazela umntwana omnye kwabayi-10,000 kuphela. Yinto engaqhelekanga kangako.

Ziziphi iintlobo eziphambili ze "Vascular Rings"?

Kukho iintlobo ezininzi eziphambili zezi "rings zemithambo yegazi". Makhe sibone ukuba zeziphi:

• I-Double Aortic Arch: Kule ndawo, "ii-arches ze-aortic" ezimbini (ekhohlo nasekunene) zijikeleze i-trachea kunye ne-esophagus. Le yingongoma ephambili esithetha ngayo.
• I-aortic arch yasekunene ene-left subclavian artery engaqhelekanga kunye ne-left ligamentum: Kukho i-aortic arch enye apha, kodwa ikwicala lasekunene le-trachea (ngesiqhelo ngasekhohlo). Kule meko, i-left subclavian artery, enika igazi kwiingalo zomntwana, iqala kwi-aorta ize idlule ngasemva kwe-trachea kunye ne-esophagus. Le ring yemithambo yegazi igqitywa ngokudibana ne -left ligamentum arteriosus, intsalela yomthambo wegazi owawukho ngexesha lobomi bomntwana ongekazalwa ize inyamalale.
• I-aortic arch yasekhohlo ene-subclavian artery engavumelekanga: Apha, i-subclavian artery yasekunene idlula ngasemva kwe-trachea kunye ne-esophagus, izicinezela.
• Ukucinezelwa kwemithambo yegazi engaphelelanga: Imithambo yegazi engaphelelanga (brachiocephalic) lisebe le-aorta. Kule meko, iqala kwicala lasekhohlo le-trachea, ize idlule phambi kwe-trachea iye ngasekunene. Oku kubeka uxinzelelo kwi-trachea.
• I-Pulmonary artery sling: I-pulmonary artery yasekhohlo iqala kwi-pulmonary artery yasekunene ize idlule phakathi kwe-trachea kunye ne-esophagus.

Okwenzekayo kuzo zonke ezi zinto kukuba indlela yomoya okanye umqala ichaphazeleka ngandlela thile.

Ngaba kukho iintlobo ze "Double Aortic Arch"?

Ewe, kukho iintlobo ezintathu eziphambili ze "double aortic arch." Ezi zahlulwe ngokuthi yeyiphi i-arch enkulu, nokuba loo arch inkulu ingasekunene okanye ngasekhohlo kwe-trachea.

• I-arch yasekunene elawulayo ene-arch encinci yasekhohlo: Olu lolona hlobo luqhelekileyo, luvela malunga nama-80 ekhulwini amatyala.
• I-arch yasekhohlo elawulayo ene-arch encinci yasekunene: Oku kubonakala malunga ne-10 ekhulwini yamatyala.
• Ii-arches ezilinganiselayo: Oku kubonakala nakwii-10 ekhulwini zamatyala.

Loluphi uhlobo "lwendandatho yemithambo yegazi" olubonakala kakhulu?

Oogqirha babecinga ukuba "i-double aortic arch" yayiyeyona ndlela ixhaphakileyo "ye-vascular ring." Kodwa uphando olutsha lufumanise ukuba "i-right aortic arch" ixhaphake kakhulu.

Kodwa kukho enye into. Iintsana ezine-"right aortic arch" zinamathuba amaninzi okuba nezinye iziphene zentliziyo ezizalwa nazo kuneentsana ezine-"double aortic arch." Nangona kunjalo, azinakwenzeka ukuba zifunyaniswe xa zizalwa. Oku kungenxa yokuba "right aortic arch" isenokungabangeli zimpawu kwizigaba zokuqala. Ngoko ke, utyando lwale ngxaki lunokufuneka phakathi kweenyanga ezi-6 nezili-18 ubudala.

Imeko ebizwa ngokuba yi- "Double Aortic Arch" yeyona ntlobo ixhaphakileyo yeringi yemithambo yegazi. Ngoko ke, ugqirha womntwana wakho unokuxilonga ingxaki kwangethuba. Xa kuthelekiswa neentsana ezinezinye iintlobo zeringi zemithambo yegazi, iintsana ezine-"Double Aortic Arch" zinokufuna utyando emva nje kokuzalwa.

Zithini iimpawu ze-"Double Aortic Arch"?

Iimpawu ze-"double aortic arch" zihlala zenzeka xa umphimbo womntwana, umqala, okanye zombini zicinezelwe. Olu cinezelo lunokuchaphazela ukuphefumla komntwana, ukutya, okanye ukusebenza kwentliziyo.

Amaxesha amaninzi, ezi mpawu zibonakala kwasebuntwaneni (ingakumbi ukuba imeko inzima), kodwa ngamanye amaxesha zinokubonakala kwasekuqaleni kweminyaka emithathu ubudala, okanye kwanakwiminyaka yokufikisa.

Abanye abantwana banokuchongwa ngendlela engafanelekanga njenge-asthma kuba iimpawu ziyafana. Le meko ichongwa kuphela xa unyango lwe-asthma lungaphumeleli kwaye kwenziwe uvavanyo olongezelelweyo.

Iimpawu zokuphefumla

Malunga neepesenti ezingama-91 zeentsana kunye nabantwana abane-"double aortic arch" bahlakulela ezi mpawu ezinxulumene nokuphefumla. Nazi:

• Isandi esingaqhelekanga xa uphefumla (isandi esivakala phezulu esibizwa ngokuba yi-`stridor` okanye isandi sokuphefumla esibizwa ngokuba yi-`wheezing`) .
• Ukukhwehlela okuvakala ngathi "kukukhonkotha kwetywina." Oku kufana nokukhwehlela okwenzeka xa kukho imeko ebizwa ngokuba yi "croup."
• Usulelo oluqhelekileyo lwendlela yokuphefumla esezantsi .

Iimpawu zesisu

Malunga nama-40 ekhulwini eentsana nabantwana abane-"double aortic arch" bafumana ezi mpawu zokugaya ukutya. Ziquka:

• Ukuminxeka xa uncancisa okanye uncancisa.
• Ubunzima bokuginya (dysphagia) . Oku kudla ngokubonakala emva kokuba umntwana eqale ukutya ukutya okuqinileyo.
• Ubunzima bokutyeba .
• Ukutya kubuyela phezulu emqaleni (`Ukubuyela umva/ukubuyela umva`) .

Iimpawu zentliziyo

Malunga nama-30 ekhulwini eentsana kunye nabantwana abane-"double aortic arch" bavelisa iimpawu ezinxulumene nentliziyo. Ezi ziquka:

• Iintlungu zesifuba .
• Isandi sentliziyo esingaqhelekanga ('Intliziyo ikhala') .
• Ulusu oluluhlaza okwesibhakabhaka (i-cyanosis) .

Yintoni ebangela "i-Double Aortic Arch"?

Isizathu esichanekileyo se-"double otic arch" asikafunyanwa . Nangona esi simo siqhele ukubonwa kwiintsana ezikhulelweyo "ngokuchumisa kwi-in-vitro" (IVF) , isizathu esichanekileyo asaziwa.

I-Double aortic arch inokunxulunyaniswa nezinye iimeko zonyango. Malunga ne-12 ekhulwini leentsana ezine-double aortic arch zinesinye isiphene sentliziyo esizalwa naso, njenge- ventricular septal defect (VSD) kunye ne-tetralogy of Fallot . Ukongeza, malunga ne-20 ekhulwini leentsana ezine-double aortic arch zineemeko zemfuza ezifana ne -chromosome 22q11 deletion kunye ne-trisomy 21 (Down syndrome) .

Indlela yokuqaphela "i-Double Aortic Arch"?

I-double aortic arch ifunyaniswa ngengozi okanye xa iimpawu ziqala. Ngamanye amaxesha, ifunyaniswa ngengozi ngexesha lokukhulelwa, emva kokuzalwa, okanye ngexesha lovavanyo lwemifanekiso olwenziwe ngesizathu esinye. Ukususela ngo-2004, inani labantu abafunyaniswa benesi simo ngokusebenzisa iiskeni ngexesha lokukhulelwa liye landa. Oku kuluncedo kakhulu, njengoko unyango lunokucwangciswa kwangoko emva kokuba umntwana ezelwe.

Okanye, ingafunyaniswa xa kuvela iimpawu emva kokuba umntwana ezelwe. Ugqirha womntwana wakho uza kuthetha nawe ngeempawu aze emva koko enze uvavanyo oluthile.

Uvavanyo lokufumanisa i-"Double Aortic Arch"

Iimvavanyo eziphambili ezisetyenziswayo ukufumanisa le meko zezi:

• I-X-ray yesifuba: Olu luvavanyo lokuqala oluyenziwa rhoqo .
• Iskeni ye-CT.
• Uvavanyo lwe-Echocardiogram (Echo): Oku kunceda kakhulu ekufumaniseni ukuba kukho ezinye iziphene zentliziyo.
• Uvavanyo lwe-MRI (MRI).

Ngamanye amaxesha i-bronchoscopy inokwenziwa ukuhlola umbhobho womoya womntwana. Oku kungenziwa ukuphendula ubunzima bokuphefumla ngaphambi kokuba kufunyaniswe ukuba kukho i-aortic arch ephindwe kabini. Okanye, kungenziwa ngaphambi kotyando ukunceda ugqirha otyandayo ukuba afumane umbono ongcono wento eyenzekayo.

Uvavanyo lwemfuza lunceda ekuchongeni iimeko zemfuza.

Indlela yokunyanga "i-Double Aortic Arch"?

Kufuneka utyando ukuze kulungiswe imeko "ye-double aortic arch." Kwanakwiintsana ezingenazimpawu, utyando lwenziwa ngcono phakathi kweenyanga ezi-6 ukuya kwezi-9 ubudala. Iintsana ezineempawu, ingakumbi ukuba iimpawu zinzima, zinokufuna utyando kwangethuba.

Khawucinge nje, imeko ethi "double aortic arch" yaqala ukuchongwa ngo-1737. Utyando lokuqala lwenziwa ngo-1947. Sihambe kangakanani ukusukela ngoko! Iintsana ezinesiphene sokuzalwa ziye zakwazi ukuphila ubomi obude nobunempilo ngenxa yonyango lotyando.

Utyando lwe-"Double Aortic Arch"

Utyando lwe-"Double Aortic Arch" lukhuselekile kakhulu, kwaye iziphumo ziphumelele kakhulu . Umntwana wakho akuyi kufuneka aqhagamshelwe kwi-"cardiopulmonary bypass" (umatshini wentliziyo nemiphunga) .

Olu tyando lwenziwa kusetyenziswa indlela ebizwa ngokuba yi- "lateral thoracotomy." Oku kuthetha ukuba ugqirha wenza i-incision kwelinye icala lesifuba somntwana, phakathi kweembambo ezimbini. Oku kudla ngokwenziwa kwicala lasekhohlo, kuba kulapho uninzi lweentsana lune-"extra" i-rib cage encinci. Uvavanyo lwemifanekiso olwenziwe ngaphambi kotyando luvumela ugqirha ukuba ajonge kakuhle ingaphakathi lomntwana aze acwangcise utyando ngokufanelekileyo.

Ugqirha usebenzisa indlela "yokubopha nokwahlula" . Oku kuthetha ukuba i-arch encinci ibotshelelwe ukuze kumiswe ukopha kuyo. Emva koko iyasikwa ize ithungwe. Le nkqubo iphindaphindwa de i-arch yahlulwe ngokupheleleyo kwaye uxinzelelo kumbhobho womoya kunye nomqala lususwe.

Umntwana wakho uza kuhlala esibhedlele kangangeveki, aze aphile aze abuyele ekhaya.

Unyango lwe "Kommerell diverticulum" (`Kommerell diverticulum - KD`)

Amaxesha amaninzi, ezinye iintsana ezine-"double aortic arch" zinokuba nento ebizwa ngokuba yi- "Kommerell diverticulum (KD)." Oku kuqheleke kakhulu kwiintsana ezine-"right aortic arch," kodwa kunqabile kakhulu ukuba ne-"double aortic arch".

Le `KD` yi`aneurysm`, umthambo wegazi okhula kwi`aorta` eyehlayo kufutshane ne`aortic arch`. Le qhubu ingathatha indawo ize icinezele umbhobho womoya womntwana kunye nombhobho wokutya. Isenokungabangeli naziphi na iingxaki ezikhawulezileyo. Nangona kunjalo, oogqirha botyando bathanda ukuyisusa le nto xa besenza i`aortic arch` ephindwe kabini ukuze bathintele iingxaki kamva.

Unyango lwezinye iziphene zentliziyo

Ukuba umntwana wakho unezinye iziphene zentliziyo ezifuna utyando, unokunyangelwa ngaxeshanye ne-double aortic arch. Oku kunokufuna inkqubo ebizwa ngokuba yi-"median sternotomy" , equka ukwenza i-incision embindini wesifuba. Ugqirha womntwana wakho uza kuxoxa ngeyona ndlela ifanelekileyo kuwe, kuxhomekeke kwimiba ekufuneka inyangwe.

Ziziphi iingxaki ezinokubakho xa utyando?

Umngcipheko weengxaki ezivela kutyando lwe-double aortic arch uphantsi kakhulu . Amaxesha amaninzi, umntwana unokuba nobunzima bokutya okanye iingxaki zokuphefumla ezingapheliyo. Ukuba kukho naziphi na iingxaki zokuphefumla ezingapheliyo, zihlala ziphela kungekapheli nonyaka emva kotyando.

Iingxaki zotyando lokulungisa i-double aortic arch kunye nezinye iziphene zentliziyo zinokwahluka. Xoxa ngazo zonke ezi ngozi kunye neengxaki nogqirha womntwana wakho.

Ngaba i-"Double Aortic Arch" ingathintelwa?

Hayi, imeko ebizwa ngokuba yi-"double aortic arch" ayinakuthintelwa . Yinto ekhula kwangethuba ekukhulelweni kwaye ikhona ekuzalweni.

Liyintoni ikamva labantu abane "Double Aortic Arch"?

Iintsana nabantwana abaye benza utyando lwe-"Double Aortic Arch" banekamva eliqaqambileyo . Banokuphila baze benze imisebenzi eqhelekileyo ngaphandle kwemiqathango.

Emva kotyando, umntwana wakho usenokuqhubeka ephefumla ngomsindo ukuya kuthi ga kunyaka. Oku kungenxa yokuba umbhobho womoya womntwana awukakhuli ngokupheleleyo ngenxa "ye-double aortic arch." Ngoko ke, kuya kuthatha ixesha ukuba umbhobho womoya ube namandla.

Kunqabile kakhulu ukuba iintsana nabantwana basenokuba neempawu zokuphefumla emva konyaka emva kotyando.

Eyona nto ibalulekileyo kukuthatha umntwana wakho aye kuhlolwa rhoqo kwaye uxelele ugqirha wakho ukuba uneempawu eziqhubekayo. Unyango oluqhubekayo lubaluleke kakhulu kuwo wonke umntwana oye wenza utyando lwentliziyo.

Ndifanele ndimtsalele nini ugqirha womntwana wam?

Ukuba ubona naziphi na iimpawu ezintsha, okanye ukuba iimpawu ezikhoyo zibonakala zisiba mandundu, thetha nogqirha womntwana wakho ngoko nangoko. Iintsana nabantwana abancinci batshintsha kakhulu kangangokuba ngamanye amaxesha kunokuba nzima ukufumanisa ukuba yintoni eqhelekileyo nengaqhelekanga. Kodwa ukuba umntwana wakho uneso sikhohlela "esifana neseal" okanye isandi sokuphefumla , xelela ugqirha wakho ngoko nangoko. Isenokuba sisifo esiqhelekileyo sobuntwana. Kodwa kunqabile, ukuba luphawu lwengxaki yentliziyo echaphazela amandla omntwana wakho okuphefumla.

Ufanele utsalele nini umnxeba ku-911 (inombolo yongxamiseko)?

"I-double aortic arch" inokubeka uxinzelelo olusongela ubomi kumphimbo womntwana wakho. Ukuba ulusu lomntwana wakho lubonakala luluhlaza okwesibhakabhaka (i-cyanosis) , okanye ukuba umntwana wakho unengxaki yokuphefumla , fowunela u-911 ngoko nangoko.

Yeyiphi imibuzo endifanele ndiyibuze ugqirha womntwana wam?

Ukuba umntwana wakho ufunyaniswe ene-"double aortic arch," kuqhelekile ukuba ube nemibuzo emininzi. Kubalulekile ukwazi imeko yomntwana wakho, kuquka nokuba kufuneka utyando nini, kunye nokuba umntwana wakho unayo nayiphi na enye ingxaki yempilo. Ungathanda ukubuza imibuzo efana nale:

• Umntwana wam ufanele atyandwe nini?
• Utyando lwenziwa phi? Ngubani olwenzayo?
• Zeziphi iimpawu ekufuneka ndiziqaphele ekhaya?
• Ndingenza ntoni ukuze kube lula ukuba umntwana wam ahlale ekhaya?
• Ngaba umntwana wam unezinye iziphene zentliziyo?
• Ngaba kufuneka ndivavanywe ufuzo lomntwana wam okanye lonke usapho lwam?
• Liza kuba njani ikamva lomntwana wam?

Yintoni "i-Aortic Arch Syndrome"? Ngaba yahlukile kwi-"Double Aortic Arch"?

Ewe, musa ukubhidanisa ezi zinto zimbini. I-"Aortic Arch Syndrome" luhlobo olungaqhelekanga kakhulu lwe- "Vasculitis" . Ikwaziwa nangokuthi yi- "Takayasu's arteritis" .

"I-Vasculitis" kukudumba kweendonga zemithambo yegazi yakho. Inokwenzeka nakweyiphi na imithambo yegazi, nokuba yimithambo yegazi emincinci, ii-capillaries. "I-Takayasu's arteritis" kukudumba kwemithambo yegazi ephakathi nekhulu. Ixhaphake kakhulu kwi-"aortic arch" kunye namasebe ayo amakhulu. Yiyo loo nto ikwabizwa ngokuba yi-"aortic arch syndrome."

I-Takayasu's arteritis (ekwaziwa ngokuba yi-"aortic arch syndrome") yenzeka kubantu abadala abancinci nakubantu abadala abancinci . Le yingxaki eyahlukileyo ngokupheleleyo kune-"double aortic arch" yokuzalwa esiyixubushileyo ngaphambili, kwaye unyango olufunekayo lwahlukile.

Ukuzalwa komntwana kudla ngokuba lixesha lovuyo nokubhiyozela. Nangona kunjalo, xa ufumanisa ukuba umntwana wakho unesifo sentliziyo, nokuba ngexesha lokukhulelwa okanye emva kokuzalwa, olo vuyo luthathelwa indawo yixhala kunye nokungaqiniseki. Ukuba usandula ukufumanisa ukuba umntwana wakho "une-double aortic arch," mhlawumbi uyazibuza ukuba kuza kwenzeka ntoni emva koko.

Ungakhathazeki. Iqela lakho lezonyango liza kukunceda kulo lonke eli nyathelo ngenyathelo. Sele uthathe inyathelo lokuqala, elikukufunda ngakumbi ngemeko yomntwana wakho. Iindaba ezimnandi zezokuba utyando lwe-double aortic arch lukhuselekile kwaye luphumelele. Ukuba umntwana wakho unezinye iziphene zentliziyo okanye izifo zofuzo, unyango lunokuba nzima ngakumbi. Nangona kunjalo, ngenkqubela phambili ekunyamekelweni kosana olusandul' ukuzalwa, unokunceda umntwana wakho afumane unyango olufanelekileyo aludingayo, aphile, kwaye abe nobuntwana obonwabisayo.

Ngoko ke, zeziphi izinto ezibalulekileyo ekufuneka sizikhumbule kwizinto esithethe ngazo? (Umyalezo Wokubuyela Ekhaya)

Kulungile, makhe sishwankathele ezinye zezinto ekufuneka uzikhumbule kwinto esithethe ngayo malunga nale "Double Aortic Arch":

• "I-Double aortic arch" sisifo sentliziyo esizalwa naso . I-aorta yomntwana yahlulwe yaba zii-arches ezimbini, nto leyo ebangela ukucinezelwa kwe-trachea kunye ne-esophagus.
• Iimpawu eziphambili kubunzima bokuphefumla, ukuphefumla kancinci, ubunzima bokuginya, kunye nosulelo oluqhelekileyo lokuphefumla .
• Le meko ifunyaniswa ngovavanyo olufana ne-'CT scan', 'MRI', 'Echocardiogram' . Ngamanye amaxesha inokufunyanwa kwanangexesha lokukhulelwa.
• Unyango lutyando . Olu tyando lukhuselekile kakhulu kwaye luphumelele.
• Umntwana emva kotyandoUngaphila ubomi obuqhelekileyo nobusempilweni.
• Ukuba umntwana wakho uneempawu ezifana nobunzima bokuphefumla okanye ukukrwitshwa, funa ingcebiso kagqirha ngoko nangoko .
• Ungakhathazeki . Ngokuhambela phambili kwezonyango zanamhlanje, le meko inokunyangeka ngempumelelo. Iqela lakho lezonyango liza kukunika inkxaso oyifunayo.

I- Double Aortic Arch, Isifo sentliziyo esizalwa naso, Iiringi zemithambo yegazi, i-Asphyxia yosana, Impilo yosana, Utyando lwentliziyo