Cantrell a pentad: zimayambitsa, chithandizo, madokotala ananena zawo

Pali zambiri malformations aumbike mwa utero. Iwo akhoza bwanji dongosolo lililonse limba. The owopsa ndi zilema mtima, mapapo, dongosolo m'mimba, impso, ndi ubongo. Ena a iwo ali osayenerana ndi moyo. Other amenable mankhwala opaleshoni. Mmodzi wa malformations zikuluzikulu ankaona pentalogy Cantrell. matenda yodziwika ndi angapo kuyan'anila za ziwalo angapo. Nthawi zina, mungathe kupeza chosakwanira pentad Cantrell. Ngakhale kuopsa kwa chilema, nthawizina madokotala amatha kulimbana ndi matenda ndi kubwezeretsa ntchito yofunika kwambiri ya thupi.

Cantrell a pentad - kodi kudwala izi?

kudwala n'logwirizana matenda obadwa nawo a mtima ndi ziwalo zina. Monga dzina pentad Cantrell zikuphatikizapo zopindika 5. Pakati pawo ndi kuphwanya yoopsa kwambiri ya dongosolo mtima, amene kumatanthauza kusintha hemodynamics. Komanso chilema zophatikizidwamo zoopsa zodzikongoletsera chilema. Kuzindikira zingakhale pomwepo pa kubadwa kwa mwana, ngati mtima wa ana akhanda "amakhala kunja". syndrome Cantrell sikuti nthawi zonse zigwirizana ndi vuto 5. Nthawi zambiri kuti 1 kapena 2 matenda ndi kwina (tetrad wa ku). Kuwonjezera matenda a mtima, matenda kumaphatikizapo chizindikiro china lolemera - omphalocele. Komanso pali kusintha mu m'mimba zapambuyo ndi chifuwa khoma, chifundamtima chilema.

pentad Cantrell akuti: chimayambitsa matenda

Nkofunika kudziwa kuti matenda aumbike mwa trimester lachiwiri la mimba. Nthawi imeneyi, atagona ziwalo zonse zofunika ndi kachitidwe. Monga amadziwika, kwa masabata 14-18 mimba anapanga mesoderm. Mchikakamizo cha zinthu zachilengedwe ndipo zosiyanasiyana matenda amayi ena kuyan'anila za embryogenesis kumachitika nthawi imeneyi. Mmodzi wa kuvutika zimenezi zingakhale pentalogy Cantrell. Chifukwa chenicheni cha matenda osati akhazikitsidwa. Komabe, chilema izi amagwirizana ndi kuvutika chromosomal. Mwa iwo, 18 ndi trisomy 13, Turner syndrome, X-zogwirizana matenda. Amadziwika kuti chilema uyu ali osowa ndithu. Pafupipafupi pakuwonekera kwake ndi 3-5 makanda pa miliyoni. Mu dziko la milandu zosakwana 100 anafotokoza. Matendawa ambiri anyamata.

Ngakhale kuti kupeza muzu wa matenda silinakwaniritsidwe, muyenera kudziwa za zinthu chiopsezo zomwe zimayambitsa matenda yosiyanasiyana yoopsa. Pakati pawo:

1. Mmene zinthu zoipa ndi poizoni (salt zitsulo zolemera, mankhwala ndi mankhwala).
2. Cheza kukhudzana.
3. zotsatira thupi (choipa).
4. mavuto.
5. Kusuta ndi mowa.

Limagwirira matenda hemodynamic

Chifukwa chakuti pentad Cantrell adzionere zofooka zosiyana mtima, zipolowe hemodynamic Choncho kumasiyana Chrixitu. matenda nthawi zambiri limodzi ndi yamitsempha yamagazi septal chilema. Chitukuko cha matenda hemodynamic zimadalira wachiwiri wa. Ndi zilema kwambiri zimachitika lalikulu shunt yaing'ono bwalo, potero zikubweretsa oopsa m'mapapo mwanga. The ventricle kumanzere mwadzaza ndi compensatory hypertrophy. Chifukwa, pali kuwonjezeka onse RV ndi lv.

Zochepa wamba matenda. Hemodynamic chisokonezo pamene chimadaliranso kukula kwa chilema. Ndi kulankhulana lalikulu pakati pa atria kumaliseche kwa magazi lamanja olekanitsidwa ndi kwambiri kuchuluka, chifukwa mu hypertrophy ndi dilatation wa PP. Anachoka matenda zimamuchulukira amapezeka.

Mawonetseredwe waukulu pentad Cantrell a

Monga amadziwika, ndi chakale Cantrell Syndrome zikuphatikizapo zopindika 5. Ena mwa iwo ndi kuyan'anila zotsatirazi:

• Omphalocele mwana wosabadwayo. Mu njira ina imatchedwa chapamwamba-umbilical m'mimba khoma chilema. kuphwanya Izi amakhala ndi chakuti ziwalo zambiri zopezeka mu chifu, ndi pamodzi umbilical thumba chophukacho ndi kupita kunja.
• Pachifuwa khoma chilema. Iwo zambiri yodziwika ndi kusapezeka kapena kugawanika xiphoid. Chifukwa cha zimenezi, mtima amadziwikanso kupitabe patsogolo.
• Pakalibe pericardium, ndilo kukhala pafupi chifundamtima.
• Mtima anomalies chitukuko. Pafupifupi onse milandu ectopia - pachithunzichi Kusinthaku bungwe. Pa mtima chilema akhoza kukhala m'mimba kapena khosi. Komanso ectopia, ndipo pali zofooka zosiyanasiyana. Kuwonjezera pa VSD ndi ASD amapezeka atresia wa valavu tricuspid, womwe ndi mtsinje m'mapapo mwanga. Nthawi zina, ndi anasiya zipinda za mtima.
• Diaphragmatic chophukacho.

Kuwonjezera Cantrell Syndrome zikhoza kukhala zosatha, ndipo anatinso ndi mitundu anomalies ena. Nthawi zambiri, matenda a CNS (ubongo chophukacho, hydrocephalus), m'mimba (kudwala ya ndulu ndi ndulu), pakalibe zala kapena miyendo.

The matenda chithunzi cha matenda

Zizindikiro za matenda kumaonekera kwa tsiku la kubadwa. Woyamba zimapangitsa pakokha kudziwika zodzikongoletsera kwambiri chilema imene mtima ndi ziwalo m'mimba "tulutsani" kunja. Komanso, mu maora choyamba cha moyo ndipo agwirizane zizindikiro kwambiri. Chifukwa cha olumala mtima atabadwa afika mpweya pang'ono. Chifukwa, iye anali kupuma movutikira, cyanosis, indrawing pa malo intercostal ndi mmunsi mwa sternum lapansi. Nthawi zambiri, makanda awa ayenera olumikizidwa kwa othandizira munthu. Kuganiza matenda, m'pofunika kuti apeze komwe mtima kutsimikiza imodzi mwa mbali yaikulu - ectopia thupi.

ndi matenda a pentad Cantrell n'chiyani?

Cantrell ndi matenda syndrome maziko makamaka kuona kunja akhanda. Choyamba, dokotala akuwoneka pamaso pa omphalocele - chilema pa khoma m'mimba ndi thoracic, kenako amasankha komwe mumtima. Pa chitsimikiziro cha ectopic, matenda a magawo kupuma ndi hemodynamic, matenda ndi pafupifupi ena. Kudziwa pamaso vuto mtima, anachita auscultation ndi echocardioscopy. Ultrasound khalidwe pamimba kosathandiza chifukwa choyamba vuto onse ayenera kuchotsedwa.

Chithandizo pentad Cantrell

Ngakhale kuti pentad Cantrell - Izi amaphatikizidwa ndi lowopsya wachiwiri, zina n'zotheka kuthana nazo. Zimenezi zikugwira ntchito kwa odwala ndi kuvutika hemodynamic yogwirizana ndi moyo. Waukulu chilema mankhwala - analoŵererapo opaleshoni. ntchito imakhala magawo angapo ndipo chimodzi cha kuzisiya. ang'ambe thupilo The imagwiridwa mu midline thoracic ndi mpanda m'mimba. Pa nthawi yomweyo, madokotala ayenera kugwira malangizo vuto mtima ndi kuchepetsa dongosolo m'mimba. Ectopia za mtima nthawi kumanzere. Nthawi zina, opaleshoni kuti VSD ang'ono ndi ASD kuyika zaka yabwino (zaka 3).

Kuteteza malformation

The njira zikuluzikulu za kupewa ndi matenda akalandire chithandizo. Kawirikawiri matenda pentalogy Cantrell mu mwana wosabadwayo angathe kuperekedwa kale pa sabata 20-24 chitukuko. Nthawi zambiri, pozindikira zimenezi ndi chifukwa cha kuchotsa mimbayo. Kupewa oti:

• Kupewa mavuto.
• Ndi moyo wathanzi.
• Pewani mavuto, makamaka zoyambirira za mimba.

Kuti madokotala ananena zawo za matenda ndi osokoneza, monga izo zimatengera mlingo wa kuwonongeka kwa mtima. Nthawi zina, madokotala kusamalira kupulumutsa moyo wa mwana. Komabe, ana awa amafunika chisamaliro nthawi zonse ndi kuyang'aniridwa ndi akatswiri.