Epispadias kuma mafitsara exstrophy. Jiyya, fi

Abin da wani farin ciki ga iyaye a lokacin da jariri da aka haife lafiya. Abin takaici, wannan ba duk. Akwai da dama daga nakasar malformations, na farko minti na ziyartar baby haife guje ransa. Daya daga cikin mafi wuya - mafitsara exstrophy. Bisa kididdigar da, shi ne musamman rare da aka rajista, a game da 1 hali for 30 dubu haife rai jariran. Wannan talakawan darajar, saboda a wasu yankunan da yawan yara da cutar na iya tashi zuwa 000 ko saukar 1:10 zuwa 1:50 000. Amma iyaye, da fuskantar irin wannan matsala, ba su damu da statistics, yana da muhimmanci domin ya ceci rai na yaro, kuma tãyar da shi cikakken mutum. Abin da irin anomaly da abin da chances na jariran haife tare da shi?

Ta yaya mutum Urinary System

Don fi gane abin da yake mafitsara exstrophy, taimaka sanin daidai tsarin da urinary tsarin. Simplistically, shi kama da wannan: koda fitsari da aka saki a lokacin jini tacewa, wanda shi ne bisa ga ureters cikin mafitsara. Yana da wani m sashin jiki iya mikewa, wanda damar ruwa zuwa tara a cikin shi da wani har sa'an nan sallami zuwa waje na mafitsara. A wani wuri inda tashar farawa, da kuma mafitsara ne a kan, akwai wani musamman tsoka kira da sphincter. Its manufa - su hana yayyo na fitsari spontaneously. A mafitsara aka located a ciki, a cikin pubic yankin. Kazalika da dukan kayan ciki daga waje duniya, ya rufe jikin kyallen takarda, musamman da gaban bango na peritoneum. Nasa bango hada da dama yadudduka - da mucosa, submucosa, tsoka da sauran - da kuma Ribar da mahara jini da cewa sanin da launi. The dubura aka located in kusanci zuwa ga mafitsara, amma bangon da nama Layer hana hadawa da fitsari da kuma tumbi.

Waje fasali na Pathology

Mafitsara exstrophy kamu da ido tsirara daga farko lokacin rayuwa. A wannan cuta babu wani rabo daga peritoneum, Ina nufin pubic yankin yana da wani rami, girman wanda za a iya bambanta daga 3 zuwa 12 cm. A sakamakon irin wannan Pathology mafitsara ne a wajen jiki. Its gaban bango an kuma m, amma aikin na ureters ba damuwa. Akuma a gare su daga koda fitsari ne saki saukad ko trickles cikin abin da ya kasance daga cikin mafitsara, sa'an nan ta cikin rami a peritoneum fito. Kamar wancan akwai wani jini, saboda ciki bango baki dissections da kumfa kafa da ba su lalace jini jijiyoyi. Duk da haka, da bude mucous ko da yaushe sauki cuta ko shãfar kyallen. Sauran bayyane ido lahani - ma low matsayi na cibiya, ko ta bayãnka, kuma mafi girma da matsayin da dubura. Daya alama inconspicuous lahani ne bambanta rarrabuwar (nuna) da pubic kashi. Daga cikin na kullum, wannan adadi na iya zama da muhimmanci daban-daban (da wani bambanci na har zuwa 7 cm), wanda kara ƙwarai musanya masa Gait. Marasa lafiya wanda ba su da ajali ambatacce ne sabawa, motsi, swaying daga gefe zuwa gefe (kamar duck). Duk da wuya daga cikin Pathology, da jariri ne quite mai yiwuwa, aiki da kuma da dama magani yana da kyau dama.

epispadias

Wannan haihuwa lahani iya zama m, amma sau da yawa sosai shi ne concomitant na ƙarin lahani a waɗanda suke tãre da pronounced mafitsara exstrophy. Epispadias - a tsagawa da bango na mafitsara. Shi ne bai cika (kawai raba tip) ko total (mafitsara ne gaba daya cleaved daga cikin matsanancin baki zuwa mafitsara). Jiyya na lahani samar da kawai surgically, kuma idan epispadias mai zaman kanta cuta, tiyata sa da yaro kawai bayan da shekaru 1-3 shekaru, amma a lokuta inda aibi accompanies mafitsara exstrophy, bukata nan da nan baki, don kawar da epispadias aiki za a iya za'ayi a cikin wani more a baya.

dalilai

A daidai amsar tambayar dalilin da ya sa akwai mafitsara exstrophy a yara ba tukuna. Masana kimiyya ne kawai gudanar ya gano cewa Pathology auku idan lokacin da samuwar gabobin da kyallen takarda (4-5 makonni bayan ganewa) tãyuna ba zato ba tsammani ya kasa raba da Mix Kwayoyin. A sakamakon da cloacal membrane ba za a iya rufe, da kuma mafitsara ne a wajen jiki. Me ya sa aka aiwatar da cell division zai lalace? Masana sun kira ga kowa da kowa Sanadin malformations:

• shan taba da kuma shan barasa amfani a lokacin daukar ciki.
• hyperthyroidism.
• ciwon sukari.
• cutar.
• hormonal cuta.
• adrenal hyperplasia.
• illa da kwayoyi.
• sinadaran da guba da kuma sauran.

Laboratory da hanyoyin gano cewa bayyanar gwajin dabbobi extrophy ƙwarai rinjayi da tasiri na kwayoyi da cortisone.

Kwararru da yawa ba sarauta daga kwayoyin halitta da cutar, saboda haka, iyaye da mafitsara exstrophy kuma a cikin iyalai inda dangi da wannan cuta (gwaggwanninku, goggonninka da ', dan'uwa), yiwuwar samun ciwon yaro da wannan sabawa ne 1:70, kuma a cikin iyalai inda akwai riga mai rashin lafiya yaro, 1: 100. Amma da zarar biyu tagwaye wannan aibi ne m, kuma kusan ba idan sun kasance m.

Ko ganewar asali ne da ake bukata?

Bayyana a kan ta fuskar wani likita da farko seconds na haihuwa na yaro daidai kamu mafitsara exstrophy. Photo nuna yadda wannan aibi iya duba, ba za a iya kira m, don haka ba mu buga su (isasshen Schematic image). Da alama ya zama bayyananne, amma ganewar asali domin gano duk yiwu bambancin da alaka da za su gudanar zama dole. Da fari dai, shi wajibi ne, don su bincika fitsari ga gano shi peritoneal ruwa da kuma serous exudate.

Haka ma wajibi ne don yin cystoscopy, duban dan tayi, Dabarar kuma endoscopy. Kamar yadda mai mulkin, nuna da kuma kayyade nazarin tabbatar ko sarauta daga Down ciwo da kuma Edwards.

Malformations na urinary tsarin a cikin maza

M statistics ce boys mafitsara exstrophy, kuma tare da shi epispadias lura 2-5 sau mafi sau da yawa fiye da 'yan mata. Saboda bambance-bambance a cikin tsarin da al'aura gabobin da kuma urinary tsarin nakasar cuta a cikin 'yan mata da yara maza ta bambanta da ɗan. Concomitant cututtuka a cikin maza a lokacin suna yara na iya zama cryptorchidism, testicular hypoplasia, aplasia na prostate ciwon daji, kuma daga baya Peyronie ta cuta (curvature daga cikin memba). Tuni a cikin ta jariri a boys da estrafiey mafitsara markedly gagarumin raguwa a kwatanta da na kullum, da girman da azzakari, a wasu lokuta yana iya zama. Wannan shi ne saboda cewa da pubic kasũsuwa, ashe, juya shi ya ƙara ja har da cavernous jiki, tsoma baki da su dangane. Idan exstrophy faru a tare da epispadias, boys lura dissection na glans azzakari da kuma dubura wuri kusa da Kwalatai (Scrotum).

Exstrophy na mafitsara a 'yan mata

A mafi kyau jima'i da wannan cuta ne musamman rare. Rakiya aibi akwai iya zama underdeveloped labia da / ko farji, clitoral tsakiyan nonon, hijirar na dubura da farji kusa da 1.5 cm ƙananan mahaifa wuri (a wasu lokuta, shi ne a fili a bayyane). A general, yara na biyu mata da maza akwai wani karu a mafitsara iya aiki, rashin sphincter, m wuri na ureters, koda matsaloli, hanta, saifa, da kuma zai yiwu a kayyade munanan.

Jariri kula da Pathology na urinary tsarin

Akwai 3 digiri na mafitsara exstrophy:

1. A rami a peritoneum zuwa 4 cm, concomitant lahani ne mãsu fakowa ba.

2. A hilafa tsakanin peritoneum da pubic kashi up to 5-6 cm, 1-2 ne related lahani.

3. dama mai tsanani concomitant aibi (total epispadias, ecstrophy cloaca da sauransu), da bambanta rarrabuwar na peritoneum kuma da pubic ƙasũsuwa na 8 cm.

A wani kudi da yaron ya fitsari damuwa kullum pours mucous abin sa su inflammations, har peritonitis da pyelonephritis. Mucous sha daga taba diapers, Yana shãfe, da kuma sauran shafi kayan, da kuma hypothermia, kuma asarar mucous danshi. Saboda haka mafitsara exstrophy a jarirai na bukatar iyakar sterility da kuma haifar da wani m yanayi. Don kauce wa kamuwa da cuta, da yara ciyar karin darussa daga kwayoyin far kuma, idan zai yiwu, ya sa su a cikin incubator (kananan kwalaye).

Kullum pours da fitsari na iya haifar da dermatitis, kazalika da tsokane papilloma growths kafa riga a 3-4 makonni na rayuwa baby.

Wani mummunan ãyã: yaro saboda hangula na mucous kuma mai tsanani da rashin jin daɗi ki ci, ba ya barci kuma, kamar yadda wani sakamako, an lagging a baya a ci gaba.

magani

Kawai surgically a wannan mataki na ci gaba da magani bi da tare da mafitsara exstrophy. Total epispadias idan shi yana tare da sama-mai suna anomaly, ƙwarai dagula riga wuya da kuma kawo hadari ga rayuwa da yaro baki. Ayyuka yawanci ciyar mai yawa. Da farko, a cewar yawancin likitoci a duniya yi kusan nan da nan bayan haihuwa, da yaro ta shekaru 1-10 kwana. Its manufa - samuwa nama don ƙirƙirar gaban bango na mafitsara da kuma rufe shi don ƙirƙirar wani sphincter don rage frontal kashi, idan rashin fata, don rufe rata a cikin peritoneum. Idan rami ne ma manyan da kyallen takarda don rufe baby bai isa ba, da yin amfani da wucin gadi fim na iya samun wanda aka cire daga baya. Bayan wannan aiki, da hadarin kamuwa da cuta vuya urinary gabobin, amma ya bar incontinence.

Na biyu aiki aka yi a kan yaro ne shekara 1 amma ba daga baya fiye da shekaru 2. Shi ne roba al'aurar. A 'yan mata, idan babu contraindications, shi za a iya za'ayi da kuma a cikin na farko baki. Wasu kananan dakunan shan magani a reoperation ureters transplanted cikin sigmoid ciwon da kuma haifar da antireflyuksorny inji ajiye yaro daga incontinence.

The uku tiyata da aka yi a lokacin da yaro ya kai shekara 5 shekaru. A wannan mataki, mafitsara wuyansa aka kafa, sphincter yi wasu ayyuka don sauƙaƙe riƙe fitsari (idan an ba a baya cika), da kuma dace ci gaban da urogenital gabobin.

Wadannan ayyukan ake yi a lokacin da ya nuna. A musamman, da hanyoyin dawo da urinary continence a girls yi ko da a cikin shekaru 16, da kuma yara maza - daga 17 zuwa shekaru 18.

Exstrophy na mafitsara a fara tasawa

Tun da a mafi yawan lokuta da Pathology da aka gyara a cikin shimfiɗar jariri, manya da mafitsara exstrophy ne rare. Wannan ya faru a lokuta inda yaro domin dalilai da yawa da aka ba yi aiki a saboda lokaci, ko su yi aka za'ayi daidai. Mutane tare da wannan aibi da zamantakewa karbuwa matsaloli a cikin tawagar ba saboda urinary incontinence. Wannan matsala aka dauke su daya daga cikin mafi muhimmanci. Kwanan nan, akwai hanyoyin da dama na aiki a gyara shi, amma wani daga gare su ne kawai 40-80 bisa dari na nasarar bi mafitsara exstrophy adult. A hoto a kasa ya nuna wani shiri mataki kafin aiki.

Na biyu muhimmanci al'amari na matsalar - jima'i karbuwa daga manya, musamman maza haife tare da mafitsara exstrophy. Duk da wannan yunkurin na likitoci, ba shi yiwuwa a cimma cikakken maida daga cikin corpora cavernosa na azzakari maza. Da fari dai, saboda ana kusan ko da yaushe maras kyau, kuma abu na biyu, saboda su girma ne kawai bai isa ba, ko da clipping su daga pubic kashi. Saboda haka, matsakaicin tsawon na azzakari ne kusan ba fiye 6-7 cm, yayin da erection ba karya. Marasa lafiya yi roba tiyata a kan urogenital ma ya nuna psychotherapy da kuma magani.

rigakafin

Tun da muka gani Pathology daidai haddasawa ne ba a sani ba, likitoci a yau ba zai iya cikakken amsa tambaya dalilin da ya sa akwai mafitsara exstrophy. Rigakafin ne ciki abstinence daga shan taba, kwayoyi, barasa. Har ila yau, a tabbatar da su gudanar da wani binciken for gaban herpes ƙwayoyin cuta, rubella, syphilis, toxoplasmosis da kuma sauran cututtuka embryotoxic. A muhimmanci irin rawa da kwayoyin shawara, musamman idan iyali shi ne riga da lafiya tare da estrofem epispadias ko mafitsara. Wannan a fili ya nuna da sabawa duban dan tayi, wanda ya bada dama na mata yanke shawara a bar ta da lafiya yaro ko ba.

Mutane haife tare da wannan Pathology, duk rayuwa ya kamata a lura a urologist. Na bayar da muhimmanci gare su, kuma yana rage cin abinci, wanda ta kunshi dukkan m, yaji, ikonsa ya, barasa da kuma yin amfani da babban kundin na ruwa.