Epispadias ndi chikhodzodzo exstrophy. Chithandizo, konzanso

Kodi chimwemwe kwa makolo pamene mwana wangobadwa wathanzi. Mwatsoka, ili si onse. Kuli malformations kobadwa nako, maminiti loyamba kuyendera abadwe pachiswe moyo wake. Chimodzi mwa zovuta kwambiri - chikhodzodzo exstrophy. Malinga ndi kafukufuku, n'kovuta kwambiri ndi m'kaundula mu 1 operekera ana 30 zikwi anabadwa moyo. Khalidwe limeneli ambiri, chifukwa m'madera ena chiwerengero cha ana ndi matenda kukwera kwa 000 kapena adatchithisira 1:10 kuti 1:50 000. Koma makolo, anakumana ndi vuto, sasamala ziwerengero n'kofunika kupulumutsa moyo wa mwana wamasiyeyo ndi izo wathunthu anthu wokhalapo. Kodi mtundu wa anomaly ndi zimene mwayi wa ana obadwa ndi izo?

Kodi anthu mkodzo System

Kumvetsetsa zimene chikhodzodzo exstrophy, kuthandiza kudziwa kapangidwe olondola a dongosolo mkodzo. Simplistically, izo zikuwoneka ngati izi: impso mkodzo adzamasulidwa pa kusefera magazi, amene ali monga mwa ureters mu chikhodzodzo. Ndi chiwalo dzenje angathe anatambasula, amene amalola madzimadzi kudziunjikira mu izo pamlingo winawake kenako akatulutsidwa kunja kwa mkodzo ndi. Mu malo njira akuyamba, ndi chikhodzodzo yatha, pali minofu wakupambulika dzinache sphincter. cholinga chake - kuteteza kutayikira la mkodzo zokha. chikhodzodzo lili pamimba, mu malo ena obisika. Komanso ziwalo zonse za mkati ku dziko lakunja, iye anabisa zimakhala thupi, makamaka khoma kutsogolo kwa peritoneum lapansi. mpanda wake wapangidwa ndi zigawo zingapo - ndi mucosa, submucosa, minofu ndi zina - ndi chinamulowerera ndi ziwiya angapo magazi kuti adziwe mtundu. rectum lili moyandikana ndi chikhodzodzo, koma khoma ndi minofu wosanjikiza chindiletsa kusanganikirana mkodzo ndi ndowe.

mbali kunja kwa kudwala

Chikhodzodzo exstrophy anapezeka ndi apoyera kwa mphindi zoyambirira za moyo. Mu matenda palibe gawo la peritoneum ndi, i.e. m'dera ena obisika ali dzenje, kukula kwa amene akhoza zimasiyana masentimita 3 12 Chifukwa kudwala chikhodzodzo ndi kunja kwa thupi.. mpanda wake kutsogolo nawonso akusowa, koma ntchito ya ureters ndi zisadodometsedwe. Akubwera pa iwo kuchokera impso mkodzo ndi anamasulidwa madontho kapena trickles mu zotsalira za chikhodzodzo, kenako kupyolera mwa dzenje peritoneum abwera kutuluka. Choncho palibe magazi, chifukwa m'mimba khoma m'mphepete dissections ndi kuwira zinapangidwa si kuonongeka mitsempha magazi. Komabe, mucous lotseguka kophweka kuvulaza ngakhale zowawa za thewera lapansi. Other kuonekera diso kupunduka - otsika kwambiri udindo wa m'michombo, kapena sanamwe, ndi udindo wapamwamba wa chotulukira lapansi. Mmodzi zopindika mwakuwoneka inconspicuous ndi divergence (Nayenso) ndi obisika fupa. Mwakale, chithunzi angakhale zimasiyana (a kusiyana wa ku 7 cm), zomwe zina kwambiri kumasintha gait. Odwala amene alibe nthawi yoikika ndi kupatuka ndi kuyenda swaying uku (ngati bakha). Ngakhale zovuta wa kudwala, ndi obadwa kumene ndi yotheka ndithu, udakali ndi chithandizo yoyenera ali ndi mwayi wabwino.

epispadias

Chilema kubadwa akhoza kukhala pawokha, koma nthawi zambiri ndi concomitant vuto zina anthu ndi kutchulidwa chikhodzodzo exstrophy. Epispadias - ndi akuwaza a linga la mkodzo wa. Ndi chosakwanira (yekha unagawanika nsonga) kapena okwana (a mkodzo kwathunthu lamamatira kuchokera m'mphepete lakunja kwa chikhodzodzo). Chithandizo vuto kubala opaleshoni yekha, ndipo ngati epispadias matenda palokha, opaleshoni zimapangitsa mwana pokhapokha zaka 1-3, koma pa nthawi imene chilema silipita chikhodzodzo exstrophy, napempha alowererepo mwamsanga, kuthetsa epispadias ntchito angathe kuchitidwa mu more kale.

zifukwa

Yankho lenileni la funso la chifukwa pali chikhodzodzo exstrophy ana panobe. Asayansi yokha anatha kupeza kuti kudwala kumachitika ngati pa mapangidwe ziwalo ndipo zimakhala (masabata 4-5 pambuyo lingaliro) mazira mwadzidzidzi sichitha kugawanitsa ndi kusakaniza maselo. Chifukwa cha nembanemba cloacal akhoza siotseka, ndipo chikhodzodzo ndi kunja kwa thupi. N'chifukwa chiyani ndondomeko ya kugawikana selo akusokoneza? Akatswiri kuitana wamba kuti mavuto onse malformations:

• fodya ndi mowa pa mimba;
• hyperthyroidism;
• shuga;
• nthenda;
• matenda m'thupi;
• adrenal hyperplasia;
• mphamvu ya mankhwala;
• mankhwala poyizoni ndi zina.

njira Laboratory anapeza kuti maonekedwe a nyama mayeso extrophy zinakhudza kwambiri ndi zotsatira za mankhwala ndi cortisone.

Akatswiri ambiri sunaletse chikhalidwe cha majini a matenda, kotero, makolo ndi chikhodzodzo exstrophy ndi m'mabanja achibale matenda (amalume, azakhali, nephews), Mwina ndi mwana ndi kupatuka yemweyo ndiye 1:70, ndi m'mabanja kumene kale ndi mwana wodwala, 1: 100. Koma kamodzi mapasa onse chilema uyu ali osowa ndi pafupifupi konse ngati zofanana.

Kaya matenda chofunika?

Zikuoneka pa nkhope yake dotolo aliyense ndi masekondi loyamba la kubadwa kwa mwana molondola matenda chikhodzodzo exstrophy. Photo zosonyeza mmene chilema izi angakhoze kuyang'ana, sitinganene kuti zosangalatsa, choncho sitiganiza kufalitsa iwo (zokwanira chojambula image). Zikuoneka bwino bwino, koma matenda ndi cholinga chofuna kudziwa yofanana zonse zotheka zokhudzana kuchita kutero. Choyamba, m'pofunika kufufuza mkodzo kupeza izo peritoneal madzimadzi ndi serous exudate.

M'pofunikanso kuti cystoscopy, ultrasound, kulingalira ndi endoscopy. Monga ulamuliro, kumaonekera ndi maphunziro majini kutsimikiza kapena akatsimikizire ozerezeka ndi Edwards.

Malformations a dongosolo mkodzo amuna

ziwerengero osatekeseka limanena kuti anyamata chikhodzodzo exstrophy, ndi izo epispadias anati nthawi 2-5 zambiri kuposa atsikana. Chifukwa chosiyana kapangidwe ka ziwalo zoberekera ndi dongosolo mkodzo matenda obadwa nawo atsikana ndi anyamata osiyana. matenda Concomitant amuna ali wamng'ono mwina cryptorchidism, testicular hypoplasia, aplasia kansa ya prostate, ndipo kenako matenda Peyronie a (kupindika membala wa). Kale mu Chiyambi chake mu anyamata estrafiey chikhodzodzo ndinasintha kwambiri kuchepetsa poyerekeza ndi mwakale, kukula kwa mbolo nthawi zina izo zikhoza kukhala. Ichi ndi chifukwa chakuti mafupa obisika, kutembenuzira monga kumangitsa thupi cavernous, n'kusokoneza chaubale. Ngati exstrophy zinachitika molumikizana ndi epispadias, anyamata anati kung'ambika kwa mbolo glans ndi chotulukira malo kuyandikira scrotum lapansi.

Exstrophy wa chikhodzodzo atsikana

Pa kugonana fairer matenda ali osowa kwambiri. Pansiyi chilema pangakhale underdeveloped labia ndi / kapena ukazi, clitoral pogawanikana pamawere, kusamutsidwa kwa chotulukira kwa pafupi kumaliseche kwa 1.5 masentimita malo m'munsi khomo lachiberekero (zina, ndi bwino kuonekera). Ambiri, ana a amuna ndi akazi pali kuchepa chikhodzodzo mphamvu, kupanda sphincter, zosayenera malo ureters ndi vuto la impso, chiwindi, ndulu, ndi zotheka majini kuvutika.

chisamaliro Newborn ndi kudwala a dongosolo mkodzo

Pali madigiri 3 chikhodzodzo exstrophy:

1. dzenje A mu peritoneum cm 4, kupunduka concomitant ali kwina.

2. zikusiyana pakati pa peritoneum ndi fupa obisika kwa 5-6 cm, 1-2 ndi ofanana chilema.

3. angapo aakulu concomitant chilema (okwana epispadias, ecstrophy cloaca ndi ena), ndi divergence wa peritoneum ndi mafupa obisika wa masentimita 8.

Iriyonse mkodzo ta mwanayo zonse amatsanulira mucous chimene chimayambitsa inflammations awo, mpaka peritonitis ndi pyelonephritis. Mucous amadwala wokhudza matewera, amafafaniza ndi zinthu zina ❖ kuyanika, ndi hypothermia, ndi imfa chinyezi mucous. Choncho chikhodzodzo exstrophy mu akhanda amafuna sterility pazipita ndi kulenga malo oyenera. Kupewa matenda, ana amakhala maphunziro owonjezera mankhwala mankhwala, ndipo ngati n'kotheka, anawaika chofungatira (yaing'ono mabokosi).

Zonse atsanulira mkodzo kungayambitse dermatitis, komanso imfayo papilloma zophuka anapanga kale pa masabata 3-4 moyo mwana.

Chizindikiro chinanso zoipa: mwana chifukwa mkwiyo wa mucous ndi aakulu kusapeza akukana kudya, kodi kugona ndi, monga Motero, ndi zomwe zikutsalirazi kumbuyo chitukuko.

chithandizo

Only opaleshoni pa siteji iyi ya chitukuko cha mankhwala mankhwala chikhodzodzo exstrophy. Total epispadias ngati limodzi ndi pamwamba dzina anomaly, kwambiri complicates kale zovuta ndi zoopsa kwa moyo wa alowererepo mwana. Ntchito zambiri kuwononga. Choyamba, malinga ambiri madokotala dziko kuchita pafupifupi nthawi yomweyo pambuyo pa kubadwa, m'badwo mwana masiku 1-10. cholinga chake - zilipo minofu kulenga khoma kutsogolo kwa chikhodzodzo ndi kutseka kuti akonze sphincter kuchepetsa fupa mazunzo, ngati kupanda khungu, kutseka kusiyana mu peritoneum lapansi. Ngati dzenje ndi waukulu kwambiri ndipo zimakhala ndi kutseka mwana sikokwanira, ntchito filimu yokumba mwina amene anachotsa kenako. Pambuyo opaleshoni, kusatetezeka Mwamsanga ziwalo mkodzo, koma anapitiriza incontinence.

Ntchito yachiwiri amachitidwa pa mwana ndi 1 chaka koma pasanafike 2 zaka. Ndi pulasitiki kumaliseche. Atsikana, ngati palibe contraindications, izo zikhoza kuchitidwa ndi mu phukusi choyamba. Ena zipatala pa reoperation ureters kuziika mu sigmoid m'matumbo ndi kulenga limagwirira antireflyuksorny kupulumutsa mwana incontinence.

Opaleshoni lachitatu anachita pamene mwana umalandira zaka 5. Pa nthawi imeneyi, khosi chikhodzodzo aumbike, sphincter kuchita zochita zina kuti magawowa posungira wa mkodzo (ngati anali kale chakwaniritsidwa), ndipo popanga ziwalo urogenital.

Ntchito zotsatirazi anachita pamene anasonyeza. Makamaka, ndondomeko ayambe continence mkodzo atsikana anachita mu zaka 16, ndipo anyamata - kuchokera zaka 17 mpaka 18.

Exstrophy a chikhodzodzo othyoka

Popeza nthawi zambiri ndi kudwala uphungu ana, akulu ndi chikhodzodzo exstrophy n'zochepa. Izi zimachitika pa nthawi imene mwanayo zifukwa zambiri sanapange ntchito m'nthawi yake, kapena anawatengeralo kunja molondola. Anthu ndi chilema awa anatengera mavuto ena mu timu chifukwa incontinence mkodzo. Vuto limeneli ndi imodzi yofunika kwambiri. Posachedwapa, pali njira zingapo za opaleshoni vutolo, koma aliyense wa iwo peresenti 40-80 yekha wa bwino ankachitira chikhodzodzo exstrophy wamkulu. chithunzi chili m'munsichi chikusonyeza siteji kukonzekera pamaso ntchito.

The chachiwiri zofunika mbali vuto - anatengera kugonana akulu, makamaka amuna anabadwa ndi chikhodzodzo exstrophy. Anayesetsa kwambiri madotolo, sikutheka kuti tikwaniritse kuchira bwino za corpora cavernosa amuna mbolo. Choyamba, chifukwa iwo ali pafupifupi nthawi zonse olumala, ndipo kachiwiri, chifukwa buku awo si zokwanira, ngakhale pamene zodula iwo ku fupa obisika. Choncho, kutalika pazipita mbolo ndi pafupifupi konse kuposa 6-7 cm, pamene erection si wosweka. Odwala kuchita opaleshoni pulasitiki pa urogenital akusonyezanso misala ndi mankhwala.

kupewa

Popeza takambirana kudwala zimayambitsa enieni sakudziwika, madokotala angathe kuyankha funso la chifukwa pali chikhodzodzo exstrophy. Kupewa mimba osadya fodya, mankhwala, mowa. Komanso, onetsetsani kuchita kafukufuku pamaso pa mavairasi nsungu, chikuku, chindoko, toxoplasmosis ndi matenda ena embryotoxic. Udindo ankaimba ndi uphungu chibadwa, makamaka ngati banja kale odwala ndi epispadias estrofem chikhodzodzo. Izi zikusonyeza bwino lomwe kupatuka ultrasound, amene amapereka ufulu wa akazi kusankha kusiya mwana wake wodwala kapena ayi.

Anthu amabadwa ndi kudwala zimenezi, onse uyenera kuchitika pa urologist lapansi. Wofunika kwambiri kwa iwo, ndipo ali zakudya, zimene amakonda ndi mchere, zokometsera, pamaphulikaphulika, mowa onse ndi ntchito mabuku lalikulu la madzimadzi.