Cantrell kuti pentad: zvinokonzera, kurapwa, fungidziro

Pane zvakawanda malformations achiumbwa utero. Vanogona sei chero nhengo yomuviri hurongwa. Zvikuru ngozi mwoyo kuremara, bapu, zvomudumbu hurongwa, itsvo, uye uropi. Vamwe vavo haawirirani upenyu. Other amenable kuti kuvhiya kurapwa. Mumwe malformations kwazvo vaiona pentalogy Cantrell. chirwere ichi kunoratidzwa vanoverengeka kutyorwa anoverengeka nhengo. Mune zvimwe zviitiko, unogona kuwana imwe kukwana pentad Cantrell. Pasinei kwokuomerwa ndiko kuremara, dzimwe nguva vanachiremba vanogona kubata chirwere uye kudzorera anokosha muviri.

Cantrell kwakaita pentad - chii zvokurwara ichi?

zvokurwara uyu yakabatana vachiberekwa mwoyo chirwere, uye dzimwe nhengo. Sezvo zita pentad Cantrell inosanganisira 5 kuremara. Pakati pavo pane kutyora kumwoyo, iyo rinobatanidza kuchinja hemodynamics zvikuru ngozi. Uyewo kuremara ichi kunosanganisira rinotyisa urema zvizorwa. Ziva inogona pakarepo pakuzvarwa mwana, sezvo mumwoyo achangoberekwa "tumiti kunze". Cantrell kuti chirwere hapasi panofanira 5 kuremara. Kazhinji kuti 1 kana 2 zvinetso hatisi (tetrad vanamwari vatatu vatatu). Kuwedzera chemwoyo, chirwere kunosanganisira chimwe rinorema chiratidzo - omphalocele. Uyewo pane kuchinja Anterior abdominal uye muchipfuva rusvingo, diaphragm kuremara.

Cantrell kuti pentad: hunokonzera zvirwere

Zvakakosha kuziva kuti hosha achiumbwa munyika trimester akazvitakura wechipiri. Panguva iyi, kuisa nhengo dzose dzinokosha uye hurongwa. Sezvazvakanyorwa anozivikanwa, kwevhiki 14-18 akazvitakura kuumbwa mesoderm. Nokupesvedzera zvezvakatipoteredza zvinhu uye siyana mai kutapukirwa zvimwe kutyorwa embryogenesis kunogona kuitika panguva iyi. Rimwe abnormalities izvi zvinogona kuva pentalogy Cantrell. The chikonzero chaiwo hosha haina dzatangwa. Zvisinei, kuremara iri chokuita chromosomal abnormalities. Pakati pavo, 18 uye Trisomy 13, Turner urwere, X-zvakabatana chirwere. Zvinozivikanwa kuti kuremara uyu zvisinganyanyi kuitika. The kakawanda parinowanika ndiyo vacheche 3-5 miriyoni paminiti. Munyika asingasviki 100 zviitiko zvinorondedzerwa. The chirwere zvikuru nevakawanda vakomana.

Pasinei chokwadi kuti kuziva chinokonzera chirwere ichigere kutongovapo, unofanira kuziva pamusoro zvinokonzera njodzi kuti dzinotungamirira zvinetso zvakasiyana-siyana fetus. Pakati pavo:

1. Igaroziva inokuvadza uye zvine chepfu (munyu inorema nesimbi, zvinodhaka, uye mishonga).
2. Radiation ekisiposha.
3. Physical ekushandiswa (kukuvara).
4. Zvinokangaidza.
5. Kusvuta uye doro.

Mashandiro ose hemodynamic zvirwere

Nokuti chokuti pentad Cantrell aigona kuona zvakasiyana mwoyo kuremara, hemodynamic nenyonganiso saka kwakasiyana mumwe. Kakawanda chirwere ichi pamwe ventricular septal urema. Kukura hemodynamic zvirwere kunobva mutevedzeri. With zvinokosha zvikanganiso kuitika guru shunt duku denderedzwa, nokudaro zvichiita kuti pulmonary neBP. Kuruboshwe ventricle iri migweje uye compensatory hypertrophy. Somugumisiro, pane kuwedzera zvose RV uye? Lv.

Ishoma atrial septal kuremara. Hemodynamic vachivavhiringidza kana inobvawo pane kukura kuremara. With yakakura kutaurirana pakati atria chokubuda ropa mukati kurudyi aparadzana zviri kwakawedzera, zvichiguma hypertrophy uye dilatation of PP. The akasiya atrial zadzisa kunoitika.

The kuratidzwa chikuru Cantrell kuti pentad

Sezvo chikazozikamwa, ari dzenhoroondo Cantrell Syndrome inosanganisira 5 kuremara. Pakati pavo pane zvinotevera kutyorwa:

• Omphalocele mudumbu. Mune imwe nzira inonzi rokumusoro-achiine rukuvhute abdominal rusvingo kuremara. kukanganisa uku kunoratidzwa ndechokuti zvomukati anowanzoita kuwanikwa mudumbu, aunganidzwa mu heniya bhegi achiine rukuvhute uende panze.
• Chest rusvingo kuremara. It anowanzodanwa dzinoratidzwa asipo kana mahwanda xiphoid. Somugumisiro, mwoyo uyewo kuchienderera mberi.
• Pasina pericardium, ndiko kuva yakabatana diaphragm.
• Cardiac anomalies budiriro. Munenge munyika dzose zviitiko ectopia - kururama placement sangano. At mwoyo ichi chakaremara unogona kuva abdominal kana mutsipa. Mukuwedzera ectopia, uye pane kuremara siyana. Kuwedzera VSD uye ASD kunoitika Surgery pamusoro tricuspid vharuvhu, ari pulmonary tsinga. Mune zvisingawanzoitiki Muzviitiko, kwakakwana kusavapo mwoyo makamuri.
• Diaphragmatic heniya.

Kuwedzera Cantrell Syndrome angava kukwana, uye vakacherechedzawo pamwe wemishonga mamwe anomalies. Kakawanda, chirwere ichi CNS (uropi heniya, hydrocephalus), gastrointestinal (zvokurwara pamusoro nduru dundira uye chirwatata), kusavapo neminwe kana maoko nemakumbo.

The kunotoda Mufananidzo chirwere

Zviratidzo chirwere kuva pachena kubva pakuzvarwa. Wokutanga anoita huzivikanwe kucherechedza zvizorwa urema umo mumwoyo uye abdominal zvomukati "nokubudisa" kunze. Mukuwedzera, muna maawa youpenyu yokutanga uye kubatana zvikuru zvinotyisa nezviratidzo. Pamusana vachiberekwa mwoyo urema achangoberekwa anowana shoma okisijeni. Somugumisiro, akanga kupfupika mweya, cyanosis, indrawing pakati intercostal dzezvimwe ezasi chikamu sternum. Kakawanda, vacheche izvi zvinofanira chokuita imwe ventilator. Kuti chifungidziro chirwere, zvinokosha kuti tizive apo mwoyo kuzosimbisa mumwe mukuru zviri - ectopia muviri.

Chii chirwere kuti Cantrell kuti pentad?

Cantrell kuti chirwere chirwere kunobva zvikurukuru pamusoro kwokunze kuongorora achangozvarwa. Chokutanga, chiremba achitsvaka kuvapo omphalocele - zvakakanganisika of abdominal uye thoracal rusvingo, uye ipapo anosarudza apo mwoyo. Pamusoro nokusimbisa ectopic, kukanganisika kufema uye hemodynamic parameters, nechirwere iri anotofa. Kuti tizive kuvapo mwoyo urema, akaita auscultation uye echocardioscopy. Ultrassom of mudumbu mufambiro dzisingashandi nekuti pakutanga urema zvose zvinofanira kubviswa.

Kurapa pentad Cantrell

Pasinei nokuti pentad Cantrell - Izvi unobatanidzwa anotyisa mutevedzeri, mune zvimwe zviitiko zvinokwanisika kurarama nayo. Izvi zvinoshanda varwere hemodynamic abnormalities inowirirana upenyu. Huru kuremara kurapwa - a kuvhiya kupindira. The oparesheni ine matanho akawanda uye anonzi mumwe toughest. The incision rinoitwa munyika midline thoracal uye rusvingo abdominal. Panguva iyoyowo, vanachiremba vanovhiya vanofanira kuita rakasungwa mwoyo urema uye rutapudzo pamusoro zvomudumbu hurongwa. Ectopia mwoyo inowanzopiwa kusiya. Mune zvimwe zviitiko, kuvhiyiwa VSD duku uye ASD ndikaisa yakakodzera zera (3 years).

Prevention malformation

The nzira chikuru Kudzivirira kuti pamuviri kuongorora chirwere. Kazhinji chirwere pentalogy Cantrell ari mudumbu anogona kudzikinurwa kare panguva 20-24 vhiki okukura. Kakawanda, munhu mhedziso zvakadaro ndicho chikonzero kugumiswa pamuviri. Nokuti chikuru dziviriro dzinosanganisira:

• Kuti tidzivise zvinokonzerwa.
• Ramba Mararamiro utano.
• Dzivisa zvinokangaidza, kunyanya mune yokutanga yezana pamuviri.

The fungidziro chirwere ichi chinoreva zvakawanda, napo pamusoro mwero kuvadza mwoyo. Mune zvimwe zviitiko, vanachiremba vanogona kuponesa upenyu hwomwana. Kunyange zvakadaro, vana ava vanoda kugara uye pokudzora unyanzvi.