Epispadias uye dundira exstrophy. Treatment, vapore

Chokwadi mufaro vabereki kana mwana wavo aberekwa vatano. Zvinosuruvarisa, izvi hazvizizvo zvese. Kune dzakawanda vachiberekwa malformations, maminitsi kushanyira mwana vachiberekwa pangozi upenyu hwake pakutanga. Chimwe chezvinhu zvakaoma - dundira exstrophy. Maererano nenhamba, ndizvo apo neapo uye rinonyoreswa munenge 1 nyaya kuti zviuru 30 akaberekwa vapenyu vacheche. Ichi avhareji kukosha, nokuti dzimwe nzvimbo nhamba yevana vane chirwere anogona kumuka 000 kana kutura 1:10 kusvika 1:50 000. Asi vabereki, atarisana nedambudziko rakadaro, regai hanya nhamba, zvakakosha kuti aponese upenyu mwana uye uchaimutsa yakakwana munhu. Rudzii anomaly uye chii mikana vakaberekwa nayo?

Sei Urinary womunhu System

Kuti tinzwisise zviri nani zviri dundira exstrophy, batsira kuziva yakarurama nemamiriro Urinary hurongwa. Simplistically, zvinoita sokunge ichi: itsvo weti asunungurwa panguva filtration ropa, riri maererano ureters kupinda dundira. Zviri negomba nhengo vanokwanisa ndichisveerera runovabvumira Urume kuunganidza mairi kusvika pamwero uye ipapo pauri kusvikira kunze urethra. Mune imwe nzvimbo inoshandiswa kwatanga, uye dundira yapera, pane mhasuru chaiyo inonzi sphincter. Its chinangwa - kudzivirira leakage kuti weti nomuzvarirwo. The dundira iri mudumbu, munzvimbo pubic munharaunda. Uyewo zvose zvomukati kubva kunze kwenyika, rakafukidza nyama dzomuviri, zvikurukuru rusvingo pamberi peritoneum. rusvingo yake riine anoverengeka akaturikidzana - the mucosa, submucosa, tsandanyama uye vamwe - uye zvapararira ne vakawanda midziyo ropa kuti kusarudza ruvara rwayo. The mugaranhoko riri mu napedyo kuti dundira, asi rusvingo uye zvinyama rukoko kunodzivisa vaiitawo kuti weti uye mutsvina yadzo.

Exterior zviri zvokurwara

Dundira exstrophy nechirwere akashama ziso kubva nguva upenyu chokutanga. In chirwere ichi hapana chikamu peritoneum, i.e. ari pubic nharaunda ine gomba, kukura zvinogona dzinosiyana 3 kusvika 12 cm. Somugumisiro zvokurwara dundira akadaro kunze kwemuviri. rusvingo Its pamberi ari kushaikwa, asi basa ureters hadzina maturo. Kuuya kwavari kubva itsvo weti iri akasunungura madonhwe kana trickles kupinda chii vasara dundira, uye ipapo kuburikidza buri peritoneum chinobuda. Saka hapana kubuda ropa, nekuti mudumbu rusvingo pamucheto dissections uye sefuro akaumba uye haana kukanganisa ropa arteries. Zvisinei, noruvazhe mucous nyore nguva dzose kuti kukuvadza kunyange Kumubata mutambo. Other zvinooneka ziso zvikanganiso - wo yakaderera kweguvhu, kana ayo kusavapo, uye yakakwirira nzvimbo horo. Mumwe inoita asazivikanwa zvikanganiso ndiyo siyana (chijana) ari pubic pfupa. Kubva ndozvakajairwa, nhamba iyi ingava yakasiyana (a musiyano anosvika 7 cm), izvo alters mberi zvikuru gait. Varwere vasina yakamisirwa nguva ndiyo kutsaudzira, kutamira, Kuzungunuka kumativi (somunhu Dhadha). Pasinei kunzwisisa kwakaita zvokurwara, achangoberekwa rakanyatsojeka chine musoro, vanoshingaira uye vane kodzero kurapwa ane mukana wakanaka.

epispadias

kusununguka ichi chakaremara zvinogona yakazvimirira, asi kazhinji kacho chiri concomitant yakawedzerwa urema vari vaya raidudzwa dundira exstrophy. Epispadias - a kukamurwa parusvingo yacho urethra. Hazvina kukwana (chete akatsemura muromo) kana zvachose (the urethra iri dzainamatira zvachose rokunze kumucheto kusvikira dundira). Treatment urema zvibereko chete surgically, uye kana epispadias yakazvimirira chirwere, kuvhiya inoita mwana chete pashure pemakore 1-3, asi zviitiko apo mhosva kuita dundira exstrophy, rinoda nokukurumidza kupindira, kubvisa epispadias kushanda anogona kuitwa nenzira dzakawanda yapfuura.

zvikonzero

The mhinduro chaiyo yomubvunzo wokuti nei paine dundira exstrophy muvana asi. Masayendisiti chete vakakwanisa kuwana kuti zvokurwara kunoitika kana panguva paumbwe zvomukati uye munyama (vhiki 4-5 mushure pamuviri) anenge pakarepo anokundikana kugovera uye kusanganisa masero. Somugumisiro pakati cloacal Kamudzira hazvigoni kuvharwa, uye dundira riri kunze kwemuviri. Nei urongwa foni weboka iri kwakakanganiswa? Nyanzvi vanodana zvakajairika kuti zvinokonzera zvose malformations:

• kusvuta uye doro kushandiswa panguva pamuviri;
• hyperthyroidism;
• chirwere cheshuga;
• zvirwere;
• hormone zvirwere;
• adrenal hyperplasia isiri yekenza;
• divi anokonzerwa zvinodhaka;
• makemikari nomuchetura nezvimwe.

Laboratory nzira vakawana kuti pakuonekwa bvunzo mhuka extrophy zvikuru nokuda kwechirwere zvinodhaka cortisone.

Nyanzvi dzakawanda regai ushe kubva majini yezvakaita chirwere, saka, vabereki vane dundira exstrophy uye mumhuri umo hama dzine chirwere ichi (Vanasekuru, vanatete, vazukuru), mukana yokuva mwana pamwe chete kutsauka ndiye 1:70, uye mumhuri apo pane kare mwana anorwara, 1: 100. Asi kamwe mapatya vose kuremara ichi hariwanikwi uye akapotsa kumbobvira kana vari zvakafanana.

Kana chirwere chinodiwa?

Ikaonekwa ayo chiso chero chiremba pamwe masekonzi kuberekwa ari mwana wokutanga chirwere zvakarurama dundira exstrophy. Photo kuratidza kuti urema ichi vanogona kutarira, haagoni kunzi inofadza, saka hatina muzviparidze (zvakakwana schematic mufananidzo). Zvinoita sokuti kuva akajeka, asi chirwere kuti tizive zvose zvinogoneka zvakasiyana nechokuita kuitisa zvakakodzera. Chekutanga, zvinokosha kuongorora weti kuti vanouwana peritoneal mvura uye serous exudate.

Zvinokoshawo kuti cystoscopy, Ultrasound, kuri uye endoscopy. Kazhinji, anoratidza uye masero zvidzidzo kusimbisa kana ushe kubva syndrome uye Edwards.

Malformations nezvezvimwe zvinetso zvedundira mu vanhurume

Shingirira nhamba anoti vakomana dundira exstrophy, uye pamwe pacho epispadias kuchengetwa 2-5 nguva kakawanda kupfuura vasikana. Nokuda kusiyana nemamiriro nhengo dzokubereka nadzo uye Urinary gadziriro vachiberekwa zvirwere zviri vasikana nevakomana anosiyana zvishoma. Concomitant nezvirwere vanhurume muuduku angava cryptorchidism, testicular hypoplasia, aplasia kenza yeprostate, uye gare gare Peyronie nehosha (curvature kwenhengo). Nechekare kuchangotanga mune vakomana vane estrafiey dundira chaizvo rinokosha zvinoderedza tichienzanisa ndozvakajairwa, kukura yemukomana, mune zvimwe zviitiko zvingasava. Izvi imhaka yokuti mafupa pubic, akatendeuka sezvo sungisa kumusoro ari cavernous muviri, kuchivhiringidza kubatana kwavo. Kana exstrophy kwakaitika pachiimbwa epispadias, vakomana vakachengeta kwetsinga pamusoro glans mboro uye horo nzvimbo pedyo scrotum.

Exstrophy of dundira pavasikana

Panguva naka pabonde chirwere ichi apo neapo. Apa urema pangava underdeveloped labia uye / kana kusangana nekutsi kwemukadzi, clitoral cleavage, misha yacho horo kuti nyinyo pedyo 1.5 masendimita yakaderera nzvimbo remuromo (dzimwe nguva, zviri pachena zvinoonekwa). Kazhinji, vana aroorane pane kuderera dundira chinzvimbo, kushaya sphincter, kukodzera nzvimbo yacho ureters, itsvo matambudziko, chiropa, chirwatata, uye pazvinobvira nemagene abnormalities.

Achangoberekwa hanya pamwe zvokurwara nezvezvimwe zvinetso zvedundira

Pane 3 inosiyana dundira exstrophy:

1. buri peritoneum kusvika 4 cm concomitant zvikanganiso hatisi.

2. kusawirirana pakati peritoneum uye pubic mapfupa kusvikira 5-6 cm 1-2 ndiyo kufanana kuremara.

3. anoverengeka zvakakomba concomitant urema (zvachose epispadias, ecstrophy cloaca nevamwe), kuti kusiyana uku kunoita pamusoro peritoneum uye mapfupa pubic 8 cm.

Chero zvazvingava hwomwana weti utachiona nguva dzose rinodurura mucous chii chinokonzera inflammations dzavo, kusvikira peritonitis uye pyelonephritis. Mucous achitambura anobata manapukeni, unopisika, uye nezvimwe unhani zvokuvakisa, uye hypothermia, uye kurasikirwa mucous unyoro. Naizvozvo dundira exstrophy vachangoberekwa kunoda kunonyanyisa kusabereka nokusika munhu akakodzera ezvinhu. Kuti tidzivise chirwere, vana kupedza imwe mapoka asingaurayiwi kurapa uye, kana zvichiita, akazviisa mazai chenguva duku (mabhokisi diki).

Kuramba anodira kuti weti anogona kukonzera dermatitis, uyewo vanotsamwisa papilloma growths vakaumba kare panguva kwevhiki 3-4 upenyu mwana.

Chimwe chiratidzo yakaipa: mwana nokuda kutsamwa ari mucous uye zvikuru kusanzwa anoramba kudya, hazvirevi kurara uye, sezvo zvakaguma iri lagging shure muna kukura.

mabatiro

surgically chete panguva iyi kukura mushonga kubatwa dundira exstrophy. Total epispadias kana pamwe pamusoro-ainzi anomaly, zvinoomesa zvikuru kare zvakaoma uye ngozi upenyu nembudzana kupindira. Operations kazhinji kupedza. Chokutanga, maererano vakawanda vanachiremba munyika kuita zvinenge pakarepo pashure pokuberekwa, hwomwana zera mazuva 1-10. Its chinangwa - iripo zvinyama kuumba rusvingo pamberi dundira uye kuvhara kuti pave sphincter kuderedza frontal pfupa, kana kushaya ganda, kuvhara gwanza riri peritoneum. Kana gomba iri guru uye nyama kuvhara mwana hazvina kukwana, kushandiswa chakagadzirwa firimu angava iyo chinobviswa vakazochinja. Avhiyiwa ichi, mukana wokutapukirwa nokupera Urinary zvomukati, asi wakasara incontinence.

The oparesheni wechipiri rinoitwa pasi mwana 1 gore asi kwete papera pane 2 years. Hazvina epurasitiki sikarudzi. In vasikana, kana kusina contraindications, rinogona kuitwa uye wokutanga kupindira. Mamwe makiriniki panguva reoperation ureters wakasimwa kupinda sigmoid muura nokusika antireflyuksorny mashandiro kuponesa mwana kubva incontinence.

Wechitatu Kuvhiya rinoitwa apo mwana anotanga makore 5 years. Panguva iyi, dundira mutsipa aumbwa, sphincter kuita zvimwe zviito kuti zviite chengetedzo kuti weti (kana harina kare zvaitwa), uye zvakakodzera kukura urogenital zvomukati.

Zvinotevera akavhiyiwa anoteererwa kana akaratidza. Kunyanya, nzira kuti kuzoita Urinary continence pavasikana anoitwa kunyange makore 16, uye vakomana - kubva 17 kusvika 18 makore.

Exstrophy of dundira iri mukuru

Sezvo kakawanda kuti zvokurwara zano pauduku, vakuru vane dundira exstrophy hadziwanzowaniki. Izvi zvinoitika muna zviitiko apo mwana zvakawanda hazvina kuitwa oparesheni munguva yakafanira, kana ivo rakaitwa nomazvo. Vanhu vane urema ichi nevamwe kwadzakabva matambudziko ari chikwata nokuda Urinary incontinence. dambudziko iri kuonekwa chimwe chezvinhu zvinokosha. Munguva ichangopfuura, kune nzira dzakawanda kushanda kuchigadzira, asi chimwe chazvo 40-80 chete muzana vakabudirira kurapwa dundira exstrophy mukuru. The Photo pazasi inoratidza regadziriro Danho pamberi oparesheni.

The chinokosha wechipiri dambudziko - pabonde kwadzakabva vakuru, kunyanya varume vakaberekwa vaine dundira exstrophy. Pasinei nokuedza vanachiremba, hazvibviri kuita apore zvizere corpora cavernosa pakati yemukomana varume. Chekutanga, nokuti dzinenge nguva dzose akaremara, uye chepiri nekuti inzwi ravo hadzisi kukwana, kunyange apo clipping kubva pubic pfupa. Naizvozvo, kufadza kureba yemukomana ranyura kumbobvira kupfuura 6-7 cm nepo erection haisi kuputswa. Varwere kuita epurasitiki kuvhiyiwa pamusoro urogenital kunoratidzawo psychotherapy nemishonga.

dziviriro

Sezvo isu vaiona zvokurwara chaiwo kukonzera vasingazivikanwi, vanachiremba nhasi havagoni kunyatsonzwisisa kupindura mubvunzo wokuti nei paine dundira exstrophy. Kudzivirira pamuviri pakuzvinyima kusvuta, kushandisa zvinodhaka, doro. Uyewo, iva nechokwadi kuti kuitisa kuongorora kuvapo herpes nemavhairasi, rubella, njovhera, toxoplasmosis nezvimwe zvirwere embryotoxic. The rinokosha reDare Genetic Counseling, kunyanya kana mhuri iri kare vachirwara estrofem epispadias kana dundira. Izvi zvinoratidza zvakajeka kutsaudzira Ultrasound, aunopiwa kodzero vakadzi vanosarudza kusiya mwana wake anorwara kana kwete.

Vanhu vaine zvokurwara ichi, zvose upenyu zvinofanira kuchengetwa ari chiremba wezvedundira. Of zvikuru kwavari, uye ane zvokudya, izvo inosiya vose vava, Spicy, peppered, doro uye kushandisa guru zvemaBhaibheri Urume.