ʻO ka Omphalocele a me ka laparoschisis nā anomalies congenital i hōʻike ʻia e kahi kīnā i ka pani ʻana i ka paia o ka ʻōpū o ka pēpē, pili me ka exteriorization (herniation) o kahi ʻāpana o kona viscera ʻōpū. Pono kēia mau malformations i ka mālama kūikawā i ka hānau ʻana a me ke ʻoki ʻana e hoʻohui hou i ka viscera i loko o ka ʻōpū. He maikaʻi ka prognosis i ka hapa nui o nā hihia.

Definition

ʻO ka Omphalocele a me ka laparoschisis nā anomalies congenital i hōʻike ʻia e ka hiki ʻole ke pani i ka paia o ka ʻōpū o ka pēpē.

Hōʻike ʻia ka omphalocele e ka puka ākea ʻoi aʻe a liʻiliʻi paha ma ka paia o ka ʻōpū, e kau ʻia ma luna o ke kaula umbilical, ma ia wahi o ka ʻōpū a i kekahi manawa e puka mai ke ake mai ka ʻōpū o ka ʻōpū, e hana ana i ka mea i kapa ʻia he hernia. Ke koʻikoʻi ka hemahema o ka pani ʻana i ka pā, hiki i kēia hernia ke komo i ka ʻāpana digestive a me ke ake.

Mālama ʻia ka viscera i waho e kahi "ʻeke" i loaʻa kahi ʻāpana o ka membrane amniotic a me kahi papa o ka membrane peritoneal.

Hoʻohui pinepine ʻia ka omphalocele me nā hemahema hānau ʻē aʻe:

• ʻo ka hapa pinepine o ka naʻau,
• nā maʻi maʻi genitourinary a i ʻole cerebral,
• gastrointestinal atresia (ʻo ia hoʻi ka ʻāpana a i ʻole ke keakea holoʻokoʻa)…

I nā keiki hānai me ka laparoschisis, aia ka hemahema o ka paia o ka ʻōpū ma ka ʻākau o ka piko. Hoʻopili ʻia ia me ka hernia o ka ʻōpū liʻiliʻi a i kekahi mau hihia o nā viscera ʻē aʻe (colon, ʻōpū, ʻoi aku ka maʻamau o ka ʻōpū a me nā ovaries).

ʻO ka ʻōpū, ʻaʻole i uhi ʻia me ka membrane pale, lana pololei i loko o ka wai amniotic, ʻo nā ʻāpana urinary i loko o kēia wai ke kuleana no nā ʻeha ʻeha. Hiki ke loaʻa nā ʻano ʻino like ʻole: nā hoʻololi a me ka mānoanoa o ka pā o ka ʻōpū, atresias, etc.

ʻO ka mea maʻamau, ʻaʻohe hewa e pili ana.

Nā kumu

ʻAʻole i hōʻike ʻia ke kumu kikoʻī no ka pani kīnā ʻole o ka paia o ka ʻōpū ke ʻike ʻia ka omphalocele a i ʻole ka laparoschisis ma kahi kaʻawale.

Eia nō naʻe, ma kahi o ka hapakolu a i ka hapalua o nā hihia, ʻo ka omphalocele he ʻāpana o ka maʻi polymalformative, pili pinepine me ka trisomy 18 (hoʻokahi chromosome 18), akā me nā mea ʻino ʻē aʻe e like me trisomy 13 a i ʻole 21, monosomy X (a. chromosome X hoʻokahi ma kahi o nā chromosomes sex) a i ʻole triploidy (ke loaʻa ʻana o kahi pūʻulu o nā chromosomes). Ma kahi o hoʻokahi manawa ma 10 ka hopena o ka maʻi ma muli o kahi kīnā gene localized (ʻoi aku ka omphalocele pili me Wiedemann-Beckwith syndrome). 

PalekanaEND_LINK

Hiki ke hōʻike ʻia kēia mau hemahema ʻelua ma ka ultrasound mai ka trimester mua o ka hāpai ʻana, maʻamau e ʻae i ka maʻi prenatal.

Nā kānaka e pili ana

ʻOkoʻa ka ʻikepili epidemiological ma waena o nā haʻawina.

Wahi a Public Health France, ma nā papa inoa Farani ʻeono o nā anomalies congenital, i ka manawa 2011 - 2015, omphalocele i hoʻopilikia ʻia ma waena o 3,8 a me 6,1 hānau mai 10 a me ka laparoschisis ma waena o 000 a me 1,7 hānau ma 3,6.

Nā mea pilikino

Ma hope o ka hāpai ʻana (ma hope o 35 mau makahiki) a i ʻole ma ka in vitro fertilization e hoʻonui i ka pilikia o ka omphalocele.

Hiki ke komo i ka laparoschisis nā kumu pilikia e like me ka paka makuahine a i ʻole ka cocaine.

ʻO ka manaʻo lapaʻau Antenatal

No ka pale ʻana i nā ʻeha nui o ka ʻōpū i nā fetuses me ka laparoschisis, hiki ke hana i ka amnio-infusions (ka hoʻokele o ka serum physiological i loko o ka lua amniotic) i ke kolu o ka trimester o ka hāpai ʻana.

No kēia mau kūlana ʻelua, pono e hoʻonohonoho pono ʻia ka mālama kūikawā e kahi hui multidisciplinary e pili ana i nā loea i ka ʻoki pediatric a me ka hoʻōla hou ʻana o nā neonatal mai ka hānau ʻana i mea e pale aku ai i nā pilikia infectious a me ka ʻeha ʻōpū, me ka hopena e make.

Hoʻonohonoho pinepine ʻia kahi hoʻouna i hoʻokomo ʻia e maʻalahi i ka hoʻokele. No ka omphalocele, ʻoi aku ka maikaʻi o ka lawe ʻana i ka vaginal. Hoʻohana pinepine ʻia ka ʻāpana Cesarean no ka laparoschisis. 

ʻoki kino

ʻO ka mālamaʻana i nā pēpē me ka omphalocele a iʻole ka laparoschisis ka manaʻo e hoʻohui hou i nā'ōpū i loko o ka'ōpū o ka'ōpū a pani i ka puka o ka paia. Hoʻomaka koke ma hope o ka hānau ʻana. Hoʻohana ʻia nā ʻano hana like ʻole e kaupalena i ka pilikia o ka maʻi.

ʻAʻole lawa ka nui o ka ʻōpū o ka ʻōpū i ka wā hāpai ʻana no ka mālama ʻana i nā ʻōpū herniated a paʻakikī paha ke pani ʻana, ʻoi aku ka nui o ka omphalocele nui o kahi pēpē liʻiliʻi. A laila pono e hoʻomau me ka hoʻohui hou ʻana i hoʻolaha ʻia i kekahi mau lā, a i ʻole kekahi mau pule. Hoʻohana ʻia nā hoʻonā manawa no ka pale ʻana i ka viscera.

Evolution a me ka wānana

ʻAʻole hiki ke pale ʻia nā pilikia maʻi maʻi a me nā ʻokiʻoki i nā manawa a pau a noho mau i kahi hopohopo, ʻoi aku ka nui o ka noho lōʻihi o ka haukapila.

ʻO Omphalocèle

ʻO ka hoʻohui hou ʻana i loko o ka ʻōpū ʻōpū liʻiliʻi o kahi omphalocele nui hiki ke hoʻopilikia i ka hanu o ka pēpē. 

No ke koena, ʻoi aku ka maikaʻi o ka prognosis o ka omphalocele kaʻawale, me ka hoʻomaka hou ʻana o ka hānai waha a me ke ola ʻana i hoʻokahi makahiki o ka hapa nui o nā pēpē, e ulu maʻamau. I ka hopena o nā malformations pili, ʻoi aku ka maikaʻi o ka prognosis me ka nui o ka make ʻana, hiki i ka 100% i kekahi mau syndromes.

Laparochisis

I ka nele o nā pilikia, pili pono ka prognosis o ka laparoschisis i ka maikaʻi o ka hana o ka ʻōpū. He mau pule paha ia no ka hoʻōla hou ʻana o ka mākau kaʻa a me ka absorption o ka ʻōpū. No laila pono e hoʻokō ʻia ka meaʻai makua (ma ka infusion). 

ʻEiwa o nā pēpē he ʻumi e ola ana ma hope o hoʻokahi makahiki a no ka hapa nui, ʻaʻohe hopena i kēlā me kēia lā.