Epispadias me tōngāmimi exstrophy. Treatment, whakanohonoho

He aha te hari mo ngā mātua ka whanau ratou tamaiti te hauora. Kia aroha mai, e kore katoa te mea tenei. He pinepine o malformations congenital, nga meneti tuatahi o te toro tamaiti whanau te tumatetenga tona ora. Ko tētahi o te uaua tino - tōngāmimi exstrophy. E ai ki ngā tatauranga, ko reira tino onge, me te te rēhita i roto i pā ana ki 1 take mō te 30 mano whanau pēpi ora. Tenei uara toharite, no te mea i roto i te tahi mau wāhi kia whakatika i te maha o ngā tamariki ki te mate ki te 000 tuku iho 1:10 ki te 1:50 000. Ko ngā mātua ranei, fehangahangai ki te raruraru taua, e kore e tiaki e pā ana ki tatauranga, he mea nui ki te whakaora i te ora o te tamaiti, me te whakaara i te reira he te tangata oti. He aha te momo o te Pākatokato me he aha nga tūponotanga o ngā pēpi i whanau ki reira?

Nahea te Pūnaha mimi tangata

Hei mahino pai aha he tōngāmimi exstrophy, te āwhina i mohio te hanganga tika o te pūnaha mimi. Simplistically, rite tenei titiro reira: tukua tākihi mimi te i roto i te toto filtration, i te mea i rite ki te ureters ki te tōngāmimi. Ko te okana tuwhera e taea o totoro, e taea inu ki te whakaemi i roto i te reira ki te whānuitanga etahi, me te ka tukua ki te waho o te taiawa mimi. I roto i te wahi i reira tīmata te teihana, me te tōngāmimi ko runga, i reira te mea he uaua motuhake i huaina te sphincter. Ko tōna whakaaro - ki te ārai i leakage o mimi noa. Kei te tōngāmimi te i roto i te puku, i roto i te rohe tore. I tua atu i nga whekau katoa i te ao i waho, i hipokina e ia te kopa tinana, i roto i ngā te taiepa mua o te peritoneum. Tona taiepa ake tito o te maha paparanga - te mucosa, submucosa, uaua, me te tahi atu - ka î e te oko toto maha e whakatau tona tae. Kei te tōu e i roto i te tata ki te tōngāmimi, ko te taiepa, me te apa kiko ārai Mixing o mimi me feces.

āhuatanga waho o te māuiuitanga

Tōngāmimi exstrophy pāngia ki te kanohi tahanga i te taime matamua o te ora. I roto i tenei mate i reira he kore wahi o te peritoneum, i.e. te rohe tore he poka, te rahi o e taea rerekē i 3 ki te 12 cm. Ko te mutunga o taua tōngāmimi māuiuitanga ko waho o te tinana. Kei te ngaro ano tona taiepa mua, engari e kore e te mahi o nga ureters faaoti. Haere mai i ratou i te tākihi mimi he pata tukua kia rere ki te mea noho o te tōngāmimi, me ka i roto i te poka i roto i te peritoneum mai i roto i ranei. Ko te kupu i reira he kahore toto, no te mea te dissections mata taiepa puku, me te mirumiru i hanga, me te kore e pakaru pera toto. Heoi, ki te mucous tuwhera ngāwari tonu kino ara te pa o te kope. Ētahi atu hē kanohi kitea - tūnga iti rawa o te pito, ranei tona ngaro, me te teitei te tūranga o te nono. Ko tētahi hē ngali inconspicuous he hahiraa (tahuri) te wheua tore. Mai i te täkupu e, kia waiho tenei ahua tino rerekē (te rerekētanga o runga ki te 7 cm), e atu nui ketia kiwi e. Tūroro kore e nei i te wā i whakaritea ko te ine, neke, e whakangaueuetia i taha ki te taha (rite te parera). Ahakoa te matatini o te māuiuitanga, he tino taea, kaha te hou, me te ki te maimoatanga tika he tupono pai.

epispadias

Ka taea e tenei koha whanau kia motuhake, engari rawa maha ko reira he rere o hē atu i roto i te hunga ki te exstrophy tōngāmimi korerotia. Epispadias - he wāwāhi o te taiepa o te taiawa mimi. Ko reira oti (anake matamata wahia) ranei tapeke (te tino piri te taiawa mimi i te mata waho ki te tōngāmimi). Maimoatanga o kohakore hua hāparapara anake, a ki te epispadias he mate motuhake, hanga pokanga te tamaiti i muri i anake te tau o 1-3 tau, engari i roto i te wā i reira tahi te koha tōngāmimi exstrophy, tono wawaotanga tonu, ki te whakakore i taea te kawea mahi epispadias i roto i te atu mua.

take

Ko te whakahoki tangohia ki te pātai o te aha i reira he tōngāmimi exstrophy i roto i ngā tamariki ano. anake whakahaere Scientists ki te kitea e puta te māuiuitanga, ki te i roto i te hanganga o whēkau me te kopa (4-5 wiki i muri i haputanga) embryos ohorere kore ki te wehe me te uru pūtau. Ka rite ki te kore te hua o te membrane cloacal taea te kati, a ko waho o te tinana te tōngāmimi. He aha te raru te tukanga o te wehenga pūtau te? karanga noa ngā tohunga ki take malformations katoa:

• paowa, me te whakamahi waipiro i roto i te hapū;
• hyperthyroidism;
• mate huka;
• mate hopuhopu;
• disorders hormonal;
• hyperplasia adrenal;
• ngā pānga taha o taero;
• paihana matū me ētahi atu.

kitea tikanga taiwhanga e te ahua o te kararehe whakamātautau extrophy nui te awe e te pānga o te raau taero i te cortisone.

e kore e maha ngā tohunga hei rangatira i roto i te āhua iranga o te mate, na, te mau metua i te tōngāmimi exstrophy me i roto i te mau utuafare i reira whanaunga i tenei mate (kēkē, fetii, irāmutu), te tūponotanga o ia i te tamaiti ki te taua ine ko 1:70, a i roto i te mau utuafare te wahi i reira he kē he tamaiti mate, 1: 100. Otiia kotahi mahanga e rua he onge, me te tata kore tenei koha ki te he ōrite ratou.

Ahakoa hiahiatia tātaritanga te?

Ka puta i runga i tona mata i tetahi tākuta ki te hēkona tuatahi o whanau o te tamaiti tika pāngia tōngāmimi exstrophy. Whakaahua whakaatu pehea tenei koha e taea titiro, e kore e taea e huaina ahuareka, na e kore matou e te whakaputa ia ratou (nui image schematic). Te āhua ki te kia mārama, engari te tātaritanga i roto i te tikanga ki te tautuhi i te rerekētanga ka taea katoa e pā ana ki te whakahaere tika. Tuatahi, he mea e tika ana ki te titiro i te mimi mo te kimi i te reira inu peritoneal me exudate serous.

Ko reira tika hoki ki te hanga cystoscopy, ultrasound, atahanga me endoscopy. Ka rite ki te tikanga, ki te whakaaturanga me te rangahau ira whakaū hei rangatira i roto i Down syndrome, me Edwards ranei.

Malformations o te pūnaha mimi i roto i nga tane

tatauranga ta'etuku ta e tōngāmimi tamariki exstrophy, a ki reira epispadias kitea 2-5 wa atu pinepine atu i te mau tamahine. No te mea o ngā rerekētanga i roto i te hanganga o nga whekau taihemahema, me te pūnaha mimi disorders congenital i roto i ngā kōtiro me mau tamaroa rerekē ahua. mate te rere i roto i te tane i roto i tamarikitanga kia cryptorchidism, hypoplasia testicular, aplasia o te matepukupuku repeure, a muri mate o Peyronie (curvature o te mema). Kua i roto i to'na na'ina'iraa i roto i te mau tamaroa i te estrafiey tōngāmimi whakaiti mārika i nui i roto i rite ki te täkupu, te rahi o te ure, i roto i ētahi wā, kia kore e te reira. Ko e tika ana ki te meka i te wheua tore, tahuri rite whakakikī ake tenei te tinana cavernous, pokanoa ki ratou hononga. Ki te puta exstrophy roto i te taha ki te epispadias, kite tamariki tuakitanga o te ure panekire me te wāhi nono piri ki te Tunaroa.

Exstrophy o te tōngāmimi i roto i ngā kōtiro

I te takoto ataahua ko tino onge tenei mate. koha ◊oatu fakataha reira kia kia labia underdeveloped me / ranei tara, wāhinga clitoral, displacement o te nono ki te tata tara ki te 1.5 cm raro wāhi waha (i roto i ētahi wā, he reira mārama kitea). I roto i te whānui, tama a tane e rua i reira he he heke i roto i te kaha tōngāmimi, kore o sphincter, wāhi hē o te ureters, raruraru tākihi, ate, kōateate, a taea abnormalities ira.

tiaki hou ki te māuiuitanga o te pūnaha mimi

E 3 nga nekehanga o te tōngāmimi exstrophy:

1. He rua i roto i te peritoneum ki te 4 cm, he mea motu hē te rere.

2. Ko te rerekētanga i waenganui i te peritoneum me te wheua tore ake ki 5-6 cm, 1-2 he koha hāngai ana.

3. He maha ngā koha te rere kino (epispadias katoa, ecstrophy cloaca me ētahi atu), te hahiraa o te peritoneum me nga wheua tore o 8 cm.

I tetahi auau taumahatanga mimi o te tamaiti tonu ringihia mucous te mea ai ratou inflammations, tae noa ki peritonitis me pyelonephritis. mamae mucous i mo taipa, horoi, me ngā rauemi paninga atu, me te hauhauaitu, me te mate o te makuku mucous. Na reira tōngāmimi exstrophy i roto i whanau hou titau sterility mōrahi me te waihanga i tētahi taiao e tika ana. Ki te karo mate, noho i te kuao akoranga anō o te therapy paturopi me, ki te taea, hoatu ratou i roto i te incubator (pouaka iti).

Tonu ringihia te mimi kia arahi ki dermatitis, me te whakapataritari growths papilloma i hanga kē i 3-4 wiki o te ora pēpi.

Ko tētahi atu tohu kino: e kore e he tamaiti e tika ana ki te ritenga o te hūhi mucous me kino kore ki te kai, moe me te, rite te tupu, kei te ërä i muri i roto i te whanaketanga.

maimoatanga

Anake hāparapara i tenei wā o te whanaketanga o te rongoā tukinotia ki tōngāmimi exstrophy. epispadias Tapeke ki te tahi i te reira e te runga ake-ko Pākatokato, nui fakafaingata'a'ia'i te kua uaua, me te mōrearea mo te ora o te wawaotanga koati. Operations te tikanga noho te rota. Tuatahi, kia rite ki te nuinga o ngā tākuta i roto i te ao ki te mahi tata tonu i muri i whanau, tau o te tamaiti 1-10 ra. Ko tōna whakaaro - kiko e wātea ana ki te waihanga i tētahi pakitara mua o te tōngāmimi, me te kati i te reira ki te hanga i te sphincter ki te whakaiti i te wheua frontal, ki te ki te kore o te kiri, kati te āputa i roto i te peritoneum. Ki te mea nui rawa te poka, me te kopa ki te kati i te pēpi e kore he nui, kia whai i te whakamahi o te kiriata tāwhaiwhai e nekehia e muri. I muri i tenei mahi, te mōrea o te mate ngaro whekau mimi, engari i mau taikaha.

Ko te mahi tuarua ko whakamana i runga i te tamaiti ko te 1 tau, engari e kore e i muri atu i te 2 tau. Ko kikokiko kirihou. I roto i ngā kōtiro, ki te kahore he contraindications, e taea te kawea te reira i roto i, me te i roto i te wawaotanga tuatahi. Ētahi haumanu i reoperation ureters whakatokia ki sigmoid kopirua me te hanga i antireflyuksorny huarahi ki te whakaora i te tamaiti i te taikaha.

whakamana ana te toru o tafa e ina tae te tamaiti te tau o te 5 tau. I tenei wā, kua hanga e te kaki tōngāmimi, sphincter mahi atu mahi ki te whakahaere i te pupuri o te mimi (ki te kore i oti i mua i te reira), a te whanaketanga tika o te whekau urogenital.

E whakamana ana te ngā mahi e whai ake nei ka tohua. I roto i ngā, i te ngā tukanga mō te whakaora o Continence mimi i roto i ngā kōtiro i mahi i roto i te 16 tau tae noa, me nga tamariki - i 17 ki te 18 tau.

Exstrophy o te tōngāmimi i roto i te pakeke

Mai i roto i nuinga o te wā i te papaki te māuiuitanga e i roto i te tamarikitanga, he onge pakeke ki tōngāmimi exstrophy. Tenei tupu i roto i te wā i reira e kore e te tamaiti mō ngā take maha i hanga mahi i roto i te wa e tika ana, ranei kua kawea e ratou i roto i tika. People ki tenei koha i fifi urutaunga pāpori i roto i te kapa e tika ana ki te taikaha mimi. whakaaro ana tēnei raruraru te ki kia kotahi o te nui tino. Maoro, i reira e maha ngā tikanga o te mahi ki te whakatika i te reira, engari ōrau 40-80 anake tetahi o ratou o pai tukinotia pakeke tōngāmimi exstrophy. E whakaatu ana te whakaahua i raro nei te atamira faaineineraa i mua i te mahi.

te rua te āhuatanga nui o te raruraru - urutaunga fakasekisualé o ngā pakeke, nga tangata whanau rawa ki tōngāmimi exstrophy. Ahakoa nga tautooraa pai o kaiwhakaako, e kore e taea ki te whakatutuki i ora tonu o te cavernosa putunga o nga tangata ure. Tuatahi, no te mea kei te hauā ratou tata tonu, a tuarua, no te mea he noa e kore e nui to ratou rōrahi, ara ka Topenga ratou i te wheua tore. Na reira, ko te roa mōrahi o te ure, ko te tata kore neke atu i 6-7 cm, i te kore e te whakaarahanga pakaru. Tūroro mahi pokanga kirihou i runga i te urogenital whakaatu hoki hinengaro me te rongoā.

ārai

Mai whakaaro matou he unknown māuiuitanga take tangohia, e kore e taea e ngā tākuta i tenei ra tino whakahoki i te pātai o te aha i reira he tōngāmimi exstrophy. Prevention he hapu te nohopuku i kai paipa, taero, waipiro. Hoki, kia tino ki te whakahaere i te rangahau mo te aroaro o wheori herpes, kōpukupuku, pakewakewa, toxoplasmosis me ētahi atu mate embryotoxic. Ko te wāhi nui takaro e tohutohu ira, rawa, ki te he mate kua ki epispadias estrofem tōngāmimi ranei te utuafare. mārama E whakaatu ana tēnei i te ultrasound ine, e homai te tika o te wahine ki te whakatau ki te waiho ia tamaiti e mate ana e kore ranei.

People whanau ki tenei māuiuitanga, kia kite ora katoa i te roma mimi. O faufaa rahi ki a ratou, a ka he kai, e katia atu katoa te tote, raukikini, he maha, waipiro, me te whakamahi o buka nui o te wai.