Epispadias kunye lwesinye exstrophy. Treatment, ukuvuselelwa

Olunjani uvuyo abazali xa usana lwabo ezelwe esempilweni. Ngelishwa, oku konke. Kukho ezininzi malformations iziphene, imizuzu yokuqala lokutyelela umntwana ozelwe engozini ubomi bakhe. Enye yezinto ezinzima kakhulu - isinye exstrophy. Ngokutsho manani, inqabile kakhulu kwaye ebhaliswe malunga 1 kwimeko ye-30 amawaka abantwana bazalwa ziphila. Eli xabiso avareji, kuba kwezinye iindawo inani labantwana kunye sifo kuvuka ku 000 okanye kwehliswe 1:10 ukuya 1:50 000. Kodwa abazali, senze ingxaki enjalo, musa andikhathali ngezibalo, kubalulekile ukugcina ubomi bomntwana kunye kuyivusa ntu epheleleyo. Loluphi uhlobo anomaly noko amathuba abantwana bazalwa nayo?

Njani System yabantu womchamo

siqonde ukuba yintoni na isinyi exstrophy Ukuze ngcono, ukunceda ukwazi indlela efanelekileyo kwinkqubo womchamo. Simplistically, kubonakala ngathi oku: kidney umchamo usebenze ngexesha ukuhluza igazi, oko ku- ureters kungena lwesinye. Kuyinto ilungu umngxuma ekwaziyo ekusoluleni, nto leyo evumela ulwelo ukuba yande kuyo ukusa kumkhamo othile waza akhutshelwe ngaphandle kwe urethra. Kwindawo apho ijelo kuqala, kwaye esinyini liphelile, kukho umsipha okhethekileyo sphincter. injongo yawo - ukunqanda ukuvuza umchamo izenzekele. Isinye ise esiswini, kwindawo zikawonke wonke. Njengoko ngokunjalo onke amalungu angaphakathi kwihlabathi ngaphandle, wayifulela kwinyama umzimba, ingakumbi udonga ngaphambili peritoneum. udonga yakhe yabhalwa lamanqanaba ezininzi - le isifo, submucosa, izihlunu kunye nezinye - kunye luphokoke neempahla ezininzi yegazi ezibonakalisa umbala wayo. Isikali ise kufuphi esinyini, kodwa ke udonga kunye umaleko inyama luthintela ukuxuba umchamo ngendle.

iimpawu yangaphandle leengxaki

Lwesinye exstrophy kwafunyaniswa ngeliso lenyama ukusuka imizuzu yokuqala yobomi. Kule sifo akukho nxalenye peritoneum, ngamanye ummandla zikawonke inomngxuma, ubukhulu apho ziyahluka ukusuka cm-3 ukuya ku-12. Ngenxa yolo lwesinye ngezifo ngaphandle komzimba. udonga yalo elikhokelayo Okungekhoyo kwakhona, kodwa ke umsebenzi ureters kungabi sengozini. UKuza kubo ukususela kidney umchamo amathontsi kukhululwa okanye uyehla oko wahlala lwesinye, yaye ke ngalo umngxuma peritoneum uyaphuma. Ngenxa yoko akukho ukopha, kuba zomzimba kumngcipheko isisu udonga bubble kwakhiwa kwaye konakale imithambo yegazi. Nangona kunjalo, le yangaphakathi ezivulekileyo kusoloko kulula ukumenzakalisa nkqu touch le ilweyile. Ezinye iziphene eye ebonakalayo - phantsi kakhulu indawo ye inkaba, okanye ukungabikho kwayo, kwaye ephezulu indawo empundwini. Enye iziphene ezibonakala zokuqhubela yi divergence (turn) amathambo zikawonke wonke. Kulo mmiselo, eli nani kwahluka kakhulu (umahluko ukuya ku-7 cm), odlulisela ngakumbi kakhulu iqinelwa. Izigulane Andinaxesha esisigxina kude, ihamba, kwaneso ukusuka ngapha nangapha (njenge nedada). Nangona ukuntsonkotha ngezifo, usana kakhulu inokwenzeka, iyasebenza kwaye unyango ekunene indenzele ithuba elihle.

epispadias

Le ozalwe kunokuba uzimele, kodwa kakhulu iba ehamba iziphene ezongezelelweyo nabo iqapheleka isinye exstrophy. Epispadias - lwahlukane kodonga le urethra. Oku akuhambelani (incam kuphela zahlulwe) okanye iyonke (i urethra ke lwanamathela ngokupheleleyo ukususela ekupheleni olungaphandle isinye). Unyango lwezinto ezonakeleyo ukuvelisa intsumpa kuphela, yaye xa epispadias ngesifo ezimeleyo, utyando kwenza umntwana kuphela emva kweminyaka engama-1-3, kodwa kwiimeko apho isiphene ihamba isinyi exstrophy, efuna ukungenelela, ukuze kupheliswe umsebenzi epispadias enokwenziwa e ngakumbi ngaphambili.

izizathu

Impendulo ngqo umbuzo wokuba kutheni kukho isinye exstrophy ebantwaneni okwangoku. Izazinzulu kuphela wakwazi ukufumana ukuba ngezifo kwenzeka ukuba ngexesha ukuyilwa Amalungu nezihlunu (iiveki 4-5 emva kokukhulelwa) iimbumba ngesiquphe asilele zahlule uwahlanganise iiseli. Ngenxa yoko le inwebu cloacal ngazo ivaliwe, kwaye isinye zingaphandle komzimba. Kutheni inkqubo candelo yeseli iphazamisekile? Iingcali ikhwelo ziqhelekile zonke izinto ezibangela malformations:

• ukutshaya nokusetyenziswa utywala ngethuba lokukhulelwa;
• hyperthyroidism;
• diabetes;
• isifo esosulelayo;
• izifo hormone;
• hyperplasia adrenal;
• imiphumela emibi iziyobisi;
• chemical ityiwe nezinye.

iindlela Laboratory bafumanisa ukuba imbonakalo uvavanyo izilwanyana extrophy kuchatshazelwa ifuthe iziyobisi kunye cortisone.

Iingcali abaninzi anegunya ubunjani genetic zesi sifo, ngoko, abazali sesinye exstrophy kwaye kwiintsapho apho izalamane esi sifo (oomalume, oomalumekazi, abatshana), amathuba okufumana umntwana ukuphambuka elifanayo 1:70, kunye neentsapho apho sele kukho umntwana, 1: 100. Kodwa xa omabini amawele le isiphako enqabileyo phantse zange ukuba efanayo.

Nokuba isifo efunekayo?

Ivela ngobuso yayo nayiphi na ugqirha kunye imizuzwana yokuqala yokuzalwa komntwana kakuhle owona isinye exstrophy. Photo obonisa indlela le sifo khangela, asikwazi kubizwa ngokuba mnandi, ngoko musa ukuwashicilela (umfanekiso ngokwaneleyo udandalaziso) thina. Kubonakala ukuba kucace gca, kodwa uthole ukuze kuchongwe lonke utshintsho kunokwenzeka ezinxulumene ukuba baqhube kuyimfuneko. Okokuqala, kuyimfuneko ukuhlola umchamo ukuba abayifumanayo fluid peritoneal kunye exudate serous.

Kuyimfuneko ukwenza cystoscopy, ultrasound, umfanekiso kunye endoscopy. Njengomgaqo, imiboniso kunye nezifundo genetic ukuqinisekisa okanye yadalwa Down syndrome kunye Edwards.

Malformations yenkqubo wokuchama in amaduna

manani Ukuphikelela uthi amakhwenkwe zesinye exstrophy, kwaye nalo epispadias litola 2-5 kaninzi ngaphezu amantombazana. Ngenxa umahluko kubume amalungu esini kunye nenkqubo wokuchama ukuphazamiseka iziphene xa amantombazana namakhwenkwe kwahluka thile. izifo ebonakalayo kumadoda ngexesha ebuntwaneni inokuba cryptorchidism, hypoplasia wamatyhalarha, aplasia umhlaza yesinye, yaye kamva isifo Peyronie ngayo (ugobile yelungu). Sele isanda yayo namakhwenkwe kunye estrafiey isinyi ekunciphiseni ngonyuka ebalulekileyo kuthelekiswa nemigangatho,, kwezinye iimeko isenokungabi ubungakanani lobudoda kuyo. Oku kungenxa yokuba amathambo zikawonke wonke, ajike njengoko ziqinise umzimba cavernous, uphazamisana eziwafaka. Ukuba exstrophy kwenzeka libambisene kunye epispadias, amakhwenkwe waphawula asike le lobudoda inkanda kunye nendawo empundu kufutshane kwisingxobo samatyhalarha.

Exstrophy lwesinye emantombazaneni

Ngelo besini wamhle esi sifo kunqabile kakhulu. siphene ehamba kusenokubakho kwiLibia ngokwaneleyo kunye / okanye kwilungu lobufazi, cleavage clitoral, ukubana ngezantsi kokuvala ikuku ku 1.5 cm kwindawo wesibeleko elisezantsi (kwezinye iimeko, kucace ebonakalayo). Ngokubanzi, abantwana amadoda namabhinqa kukho ukuhla umthamo isinyi, ukungabikho sphincter, indawo ngokungafanelekanga le ureters, iingxaki zezintso, isibindi, udakada, kunye ukungaqheleki zemvelo ezinokubakho.

ukunakekela usana kunye ngezifo inkqubo womchamo

Kukho 3 degrees lulawulo lwesinyi exstrophy:

1. A umngxuma peritoneum ku-4 cm, iziphene ezihamba bengekho.

2. Le ukungangqinelani phakathi peritoneum kunye ithambo zikawonke wonke ukuya ku 5-6 cm, 1-2 yi siphene ezinxulumeneyo.

3. Zininzi kakhulu siphene ezihamba (epispadias zizonke, ecstrophy cloaca kunye nabanye), lo divergence le peritoneum kunye namathambo zikawonke 8 cm.

Kuyo nayiphi na imeko neentlobo umchamo lo mntwana rhoqo Wayiphalazela uxakaxa yintoni ebangela inflammations zabo, kude peritonitis kunye pyelonephritis. Uxakaxa ubunzima njengokubamba ukucupha, wosula, kunye nezinye izinto daka, kunye emzimbeni, kunye nokulahleka kokufuma yangaphakathi. Ngoko lwesinye exstrophy xa kuzalwa kufuna inzalo ubuninzi nokudala indawo efanelekileyo. Ukunqanda ukusuleleka, abantwana bachitha izifundo ezingaphezulu unyango zintsholongwane kwaye, ukuba kunokwenzeka, wazibeka incubator (iibhokisi ezincinane).

Njalo ethulula umchamo kunokukhokelela dermatitis, kwakunye bandiqumbise neziqu papilloma abunjwe sele kwiiveki 3-4 ubomi baby.

Olunye uphawu embi: umntwana ngenxa kwinwebu ukukhathazeka uxakaxa kwaye yababuhlungu akavumi ukutya, ngaba nokulala, ngenxa yoko, lisasalele emva kakhulu kuphuhliso.

unyango

Kuphela intsumpa kweli nqanaba lophuhliso yamayeza iphathwa sesinye exstrophy. Iyonke epispadias ukuba oko kukhatshwa kungasentla ebizwe anomaly, nzima kakhulu ukuba sele ezinzima neziyingozi ubomi ungenelelo kid. Imisebenzi ngokuqhelekileyo bachitha eninzi. Okokuqala, ngokutsho uninzi loogqirha ehlabathini ukwenza phantse ngoko nangoko emva kokuzalwa, ubudala bomntwana iintsuku 1-10. injongo yawo - izihlunu ekhoyo ukwenza udonga ngaphambili lwesinyi ivale ukwenza sphincter ukunciphisa ithambo ngokungqalileyo, ukuba ukungabikho eluswini, ukuvala umsantsa kwi peritoneum. Ukuba hole kuba inkulu kakhulu nezihlunu ukuvala usana akwanelanga, ukusetyenziswa kwezindlela umboniso abe leyo isuswe emva koko. Emva kwalo msebenzi, ingozi yosulelo liyalahleka amalungu wokuchama, kodwa zigcinwe ngumchamo.

yenzelwa phezu umntwana Umsebenzi wesibini ngunyaka-1, kodwa hayi emva kwe-2 iminyaka. Kuyinto zangasese zeplastiki. Xa amantombazana, ukuba akukho Izimo, oko kwenziwa kunye nongenelelo yokuqala. Ezinye iikliniki e reoperation ureters zilinywe kwi sigmoid colon nokudala antireflyuksorny indlela ukugcina umntwana ngumchamo.

Utyando wesithathu luyenziwa xa umntwana efikelela kwiminyaka engama-5. Kweli nqanaba, intamo isinyi ibunjiwe, sphincter ukwenza ezinye izenzo ukulungiselela ukugcinwa umchamo (ukuba akazange ngaphambili kufezwa), kunye nophuhliso olufanelekileyo amalungu enzala.

Imisebenzi elandelayo yenziwa xa kuboniswe. Ngokukodwa, neenkqubo ukufunyanwa continence wokuchama emantombazaneni engenziwa kwiminyaka eli-16, yaye amakhwenkwe - ukususela kwiminyaka eli-17 ukuya kwi-18.

Exstrophy lwesinye kwi omdala

Ekubeni kwiimeko ezininzi le leengxaki lilungiswa ebuntwaneni, abadala sesinye exstrophy zinqabile. Oku kwenzeka kwiimeko apho umntwana ngenxa yezizathu ezininzi zaye zenziwa ukusebenza ngexesha elililo, okanye baye yenziwa ngokuchanekileyo. Abantu kule isiphene babenengxaki lithathelwe zentlalo kwiqela ngenxa ngumchamo womchamo. Le ngxaki kucingelwa ukuba yenye yezinto ezibalulekileyo. Kutshanje, kukho iindlela ezininzi yokusebenza ukulungisa, kodwa nayiphi na kuzo ekhulwini 40-80 kuphela iphathwa ngempumelelo lwesinye exstrophy abadala. Ifoto ingezantsi ibonisa a yamalungiselelo isigaba phambi kokuba ukusebenza.

Inkalo yesibini obalulekileyo ingxaki - lithathelwe ngokwesini abadala, ingakumbi amadoda abazelwe sesinye exstrophy. Nangona kwemizamo emihle oogqirha, akunakwenzeka ukuba ukuphumeza ukuphucuka ngokupheleleyo corpora cavernosa lamadoda lobudoda. Okokuqala, ngenxa yokuba phantse lonke gqitha, yaye okwesibini, kuba umthamo yabo nje akwanelanga, kwanaxa clip kwabo ithambo zikawonke wonke. Ngoko ke, ubude iphezulu umphambili phantse zange ngaphezu 6-7 cm, lo gama i-ulwakhiwo akukho kwaphulwa. Izigulane ukwenza uqhaqho zeplastiki kwi enzala lubonisa nonyango lwengqondo kunye namayeza.

ukuvimbela

Ekubeni ingqalelo ngezifo oonobangela ngqo ayaziwa, oogqirha namhlanje akakwazi ukuphendula ngokupheleleyo umbuzo wokuba kutheni kukho isinye exstrophy. Ukuthintela abophe okhulelweyo ngenxa yokutshaya, iziyobisi, utywala. Kwakhona, qiniseka ukuba aqhube uphando ubukho iintsholongwane herpes, rubella, negcushuwa, toxoplasmosis kunye nezinye izifo embryotoxic. Indima ebalulekileyo edlalwa yi-genetic counselling, ingakumbi xa intsapho egula sele kunye epispadias estrofem okanye isinye. Oku kubonisa ngokucacileyo ultrasound kuphambuka, enika ilungelo abafazi isigqibo ukushiya umntwana wakhe ogulayo okanye hayi.

Abantu abazelwe nale ngezifo, bonke ubomi kufuneka owayejongwe womchamo. Ibaluleke kakhulu kubo, kwaye ukutya, loo magumbi onke ezinetyuwa, okuneziqholo, yayifafazeke, utywala kunye nokusetyenziswa imiqulu ezinkulu ulwelo.