Hernia Diaphragmatica Congenital (Cdh)

ʻO ka hernia diaphragmatic congenital (CDH) kahi kūlana maʻamau ʻole e loaʻa i ka pēpē ma mua o ka hānau ʻana. E kū mai ia i ka wā hāpai mua ke hāʻule ka diaphragm o ka pēpē—ʻo ka ʻiʻo e hoʻokaʻawale i ka umauma mai ka ʻōpū—mai pani e like me ke ʻano. E waiho kēia i kahi puka ma ka diaphragm. Ua kapa ʻia ka puka he hernia.

Hoʻokumu kēia hernia ma ka ʻiʻo o ka diaphragm i kahi wehe ʻana ma waena o ka ʻōpū a me ka umauma. Hiki i nā ʻōpū, ka ʻōpū, ke ate a me nā ʻōpū ʻē aʻe ke neʻe ma o ka puka i loko o ka umauma o ka pēpē. Inā aia nā ʻōpū i loko o ka umauma, ʻaʻole lākou e hoʻomohala i nā pilina maʻamau e hoʻopaʻa iā lākou ma kahi kūpono i loko o ka ʻōpū (malrotation). Hiki iā lākou ke huli ma luna o lākou iho, e ʻoki ana i kā lākou lako koko (volvulus).

ʻO ka mālama ʻana i ka CDH e hilinaʻi ana i ka wā i loaʻa ai ke kūlana, pehea ka nui o kona koʻikoʻi a inā paha he mau pilikia me ka naʻau.

ʻO ka hernia diaphragmatic i hānau ʻia mai ka nui o ka nui. Hiki ke māmā a loaʻa paha he mau hopena iki a ʻaʻohe paha i ke pēpē, a i ʻole hiki ke koʻikoʻi a hoʻopilikia i ka hiki ke lawe mai i ka oxygen i ke koena o ke kino.

Hiki i nā pēpē i hānau ʻia me ka CDH ke loaʻa:

• Ka pilikia nui o ka hanu ma muli o nā māmā liʻiliʻi ʻaʻole hana maikaʻi (pulmonary hypoplasia).
• Nā pilikia me ka hoʻomohala ʻana o ka naʻau.
• Ka pōʻino i nā ʻōpū, ka ʻōpū, ke ate a me nā ʻōpū ʻē aʻe o ka ʻōpū inā neʻe lākou ma o ka hernia i loko o ka umauma.

Hiki ke loaʻa ka CDH i ka wā o ka ultrasound fetal maʻamau. Hiki i kāu mea lawelawe ola ke kūkākūkā i nā koho lapaʻau me ʻoe.

I ka hapanui o nā hihia, ʻaʻole maopopo ke kumu o ka hernia diaphragmatic congenital. I kekahi mau hihia, hiki ke pili ka CDH i kahi maʻi kino genetic a i ʻole nā ​​hoʻololi gene kūpono ʻole i kapa ʻia he mutations. I kēia mau hihia, hiki i ke pēpē ke loaʻa nā pilikia hou aku i ka wā hānau, e like me nā pilikia me ka naʻau, nā maka, nā lima a me nā wāwae, a i ʻole ka ʻōpū a me nā ʻōpū.

Hōʻino e hiki mai ana me ka CDH:

• Nā pilikia o ka māmā.
• Nā pilikia o ka ʻōpū, nā ʻōpū a me ke akepaʻa.
• Maʻi naʻau.
• Nā maʻi hoʻi hou.
• Liliu lohe.
• Hoʻololi i ke ʻano o ka umauma a me ke kūlou o ke kua.
• Hoʻi hou ʻana o ka wai ʻōpū - ka wai ʻōpū e kahe hou ana i loko o ka paipa i kapa ʻia ʻo ka esophagus, ka mea e hoʻohui ana i ka waha a me ka ʻōpū.
• Nā pilikia me ka ulu ʻana a me ka loaʻa kaumaha.
• Nā hoʻopaneʻe ulu a me nā pilikia aʻo.
• Nā pilikia ʻē aʻe i loaʻa mai ka hānau ʻana.

Hoʻomaʻamaʻa pinepine ʻia ka hernia diaphragmatic congenital i ka wā o kahi hoʻokolokolo ultrasound fetal maʻamau i hana ʻia ma mua o ka hānau ʻana o kāu pēpē. Hoʻohana kahi hoʻokolokolo ultrasound prenatal i nā nalu leo ​​e hana i nā kiʻi o kou uterus a me kāu pēpē.

I kekahi manawa, ʻaʻole paha e hana ʻia ka ʻike ma hope o ka hānau ʻana. I ka manawa pinepine, ʻaʻole paha e ʻike ʻia ka CDH a hiki i ka wā kamaliʻi a ma hope paha. Hiki paha kēia no ka mea ʻaʻohe hōʻailona a i ʻole hōʻailona a i ʻole no ka mea he māmā nā hōʻailona a me nā hōʻailona e like me nā pilikia hanu a me nā ʻōpū.

Hoʻohana kāu mea lawelawe olakino i ka ultrasound prenatal a me nā hoʻokolokolo ʻē aʻe e hahai i ka ulu a me ka hana o nā māmā, na puʻuwai a me nā ʻōnaehana ʻē aʻe o kāu pēpē i ka wā hāpai.

ʻO ka maʻamau, loaʻa iā ʻoe kāu ultrasound fetal mua i loko o nā mahina mua (trimester mua) o kāu hāpai ʻana. Hoʻomaopopo ia ua hāpai ʻoe a hōʻike i ka nui a me ka nui o kāu pēpē a i ʻole nā ​​pēpē.

ʻO ka manawa pinepine, loaʻa iā ʻoe kahi ultrasound ʻē aʻe i nā mahina ʻehā a ʻeono (trimester ʻelua) o kāu hāpai ʻana. Nānā kāu mea lawelawe olakino i ka ulu a me ka hoʻomohala ʻana o kāu pēpē. Nānā kāu mea hoʻolako i ka nui a me ka wahi o nā māmā, na puʻuwai a me nā ʻōnaehana ʻē aʻe o kāu pēpē.

Inā hōʻike kāu pēpē i nā hōʻailona o ka CDH, hiki i kāu mea hoʻolako ke loaʻa iā ʻoe nā hoʻokolokolo ultrasound pinepine. Hiki i kēia ke hōʻike i ka nui o ka CDH a inā paha e ʻoi aku ka maikaʻi.

Hiki ke hana ʻia nā hoʻokolokolo hou aku e loiloi i ka hana o nā ʻōnaehana o kāu pēpē. Hiki i kēia ke komo:

• Fetal magnetic resonance imaging (MRI). He ʻenehana kiʻi lapaʻau kēia e hoʻohana ana i kahi kahua magnetic a me nā nalu radio i hana ʻia e kamepiula e hana i nā kiʻi kikoʻī o nā ʻōnaehana a me nā pūnaewele i loko o ke kino o ka pēpē.
• Fetal echocardiogram. Hoʻohana kahi echocardiogram i nā nalu leo ​​e hana i nā kiʻi o ka puʻuwai o ka pēpē e kuʻi ana a e hoʻopuka ana i ke koko. Hiki i nā kiʻi mai kahi echocardiogram ke ʻike i nā pilikia me ka puʻuwai e ulu ana.
• Nā hoʻokolokolo genetika. Hiki i ka hoʻokolokolo genetika ke ʻike i nā syndromes genetika a i ʻole nā ​​hoʻololi gene ʻē aʻe i pili pinepine ʻia me ka CDH. Hiki i ka ʻōlelo aʻo genetika ke kōkua iā ʻoe e hoʻomaopopo i kēia mau hopena hoʻokolokolo a hāʻawi iā ʻoe i ka ʻike hou aku e pili ana i ke kūlana o kāu pēpē.

ʻO ke ʻano o ka mālama ʻana i ka hernia diaphragmatic congenital e hilinaʻi ana i ka wā i loaʻa ai ke kūlana a me kona nui. E kōkua kāu hui mālama ola iā ʻoe e hoʻoholo i ka mea maikaʻi loa no ʻoe a me kāu pēpē.

E nānā pono kāu hui mālama ola iā ʻoe ma mua o ka hānau ʻana o kāu pēpē. E loaʻa pinepine iā ʻoe nā ultrasound a me nā hoʻokolohua ʻē aʻe e nānā i ke olakino a me ka ulu ʻana o kāu pēpē.

ʻO kahi mālama hou no ka CDH koʻikoʻi e aʻo ʻia nei i kapa ʻia ʻo ka fetoscopic endoluminal tracheal occlusion (FETO). Hana ʻia kēia ʻoki ma kāu pēpē ʻoiai ʻoe e hapai ana. ʻO ka pahuhopu, ʻo ia ke kōkua i ka ulu ʻana o nā māmā o ke pēpē i ka nui loa ma mua o ka hānau ʻana.

FETŌ hana ʻia i ʻelua kaʻina hana:

• Kaʻina hana mua. Hana ʻia ka hana mua i ka hoʻomaka ʻana o nā mahina hope loa (third trimester) o kāu hapai ʻana. E hana kāu kauka i kahi ʻoki liʻiliʻi ma kou ʻōpū a me kou uterus. E hoʻokomo ka kauka i kahi paipu kūikawā me kahi kāmela ma ka hopena, i kapa ʻia ʻo ka fetal endoscope, ma o ka waha o kāu pēpē a komo i loko o ka windpipe (trachea). E waiho ʻia kahi pōpō liʻiliʻi i loko o ka trachea o kāu pēpē a hoʻonui ʻia.

  E kahe ana ka wai uterine maoli i ka wā hapai, i kapa ʻia ʻo ka wai amniotic, i loko a i waho o nā māmā o kāu pēpē ma o ka waha. ʻO ka hoʻonui ʻana i ka pōpō e mālama i ka wai amniotic i loko o nā māmā o kāu pēpē. E hoʻonui ka wai i nā māmā e kōkua i ko lākou ulu ʻana.

• Kaʻina hana ʻelua. Ma hope o kahi 4 a 6 mau wiki, e loaʻa iā ʻoe kahi kaʻina hana ʻelua. E wehe ʻia ka pōpō i mea e mākaukau ai kāu pēpē e lawe i ka ea i loko o nā māmā ma hope o ka hānau ʻana.

  Hiki ke hoʻohana ʻia kahi ʻano hānau kūikawā inā hoʻomaka ka hana ma mua o ka wehe ʻana o ka pōpō a ʻaʻole hiki ke wehe ʻia ka pōpō me ka endoscope. Kīaʻia kēia ʻano he kaʻina hana ex utero intrapartum treatment (EXIT). Hana ʻia ka hānau ʻana ma ke ʻano C-section me ke kākoʻo placental. ʻO ia hoʻi, e hoʻomau ana kāu pēpē e loaʻa ka oxygen ma o ka placenta ma mua o ke ʻoki ʻana o ka umbilical cord. E hoʻomau ana ke kākoʻo placental a hiki i ka pau ʻana o ka pōpō a waiho ʻia kahi paipu hanu, e ʻae ana i ka mīkini e lawe i ka hanu.

Kaʻina hana mua. Hana ʻia ka hana mua i ka hoʻomaka ʻana o nā mahina hope loa (third trimester) o kāu hapai ʻana. E hana kāu kauka i kahi ʻoki liʻiliʻi ma kou ʻōpū a me kou uterus. E hoʻokomo ka kauka i kahi paipu kūikawā me kahi kāmela ma ka hopena, i kapa ʻia ʻo ka fetal endoscope, ma o ka waha o kāu pēpē a komo i loko o ka windpipe (trachea). E waiho ʻia kahi pōpō liʻiliʻi i loko o ka trachea o kāu pēpē a hoʻonui ʻia.

E kahe ana ka wai uterine maoli i ka wā hapai, i kapa ʻia ʻo ka wai amniotic, i loko a i waho o nā māmā o kāu pēpē ma o ka waha. ʻO ka hoʻonui ʻana i ka pōpō e mālama i ka wai amniotic i loko o nā māmā o kāu pēpē. E hoʻonui ka wai i nā māmā e kōkua i ko lākou ulu ʻana.

Kaʻina hana ʻelua. Ma hope o kahi 4 a 6 mau wiki, e loaʻa iā ʻoe kahi kaʻina hana ʻelua. E wehe ʻia ka pōpō i mea e mākaukau ai kāu pēpē e lawe i ka ea i loko o nā māmā ma hope o ka hānau ʻana.

Hiki ke hoʻohana ʻia kahi ʻano hānau kūikawā inā hoʻomaka ka hana ma mua o ka wehe ʻana o ka pōpō a ʻaʻole hiki ke wehe ʻia ka pōpō me ka endoscope. Kīaʻia kēia ʻano he kaʻina hana ex utero intrapartum treatment (EXIT). Hana ʻia ka hānau ʻana ma ke ʻano C-section me ke kākoʻo placental. ʻO ia hoʻi, e hoʻomau ana kāu pēpē e loaʻa ka oxygen ma o ka placenta ma mua o ke ʻoki ʻana o ka umbilical cord. E hoʻomau ana ke kākoʻo placental a hiki i ka pau ʻana o ka pōpō a waiho ʻia kahi paipu hanu, e ʻae ana i ka mīkini e lawe i ka hanu.

ʻAʻole paha ʻo FETO ka koho kūpono no nā kānaka a pau. A ʻaʻohe hōʻoia e pili ana i nā hopena o ka ʻoki ʻana. E loiloi kāu hui mālama ola iā ʻoe a me kāu pēpē e ʻike inā he mau moho ʻoe no kēia ʻoki. E kamaʻilio me kāu hui e pili ana i nā pono a me nā pilikia hiki ke loaʻa iā ʻoe a me kāu pēpē.

ʻO ka maʻamau, hiki iā ʻoe ke hānau i kāu pēpē ma ke ʻano vaginal a i ʻole ma ke ʻano C-section. ʻO ʻoe a me kāu mea hoʻolako mālama ola e hoʻoholo i ka ʻano maikaʻi loa no ʻoe.

Ma hope o ka hānau ʻana, e kōkua kāu hui mālama ola iā ʻoe e hoʻolālā i ka mālama e hoʻokō i nā pono o kāu pēpē. E mālama ʻia paha kāu pēpē i loko o ka newborn intensive care unit (NICU).

Pono paha kāu pēpē e loaʻa kahi paipu hanu. Hoʻopili ʻia ka paipu i kahi mīkini e kōkua i kāu pēpē e hanu. Hāʻawi kēia i ka manawa i nā māmā a me ka naʻau e ulu a hoʻomohala.

Pono paha nā pēpē me nā pilikia māmā weliweli i kahi mālama i kapa ʻia ʻo ka extracorporeal membrane oxygenation (ECMO). ʻIke ʻia kēia ʻo ka extracorporal life support (ECLS). Hana ka mīkini ECMO i ka hana o ka naʻau a me nā māmā o kāu pēpē, e ʻae ana i kēia mau ʻōnaehana e hoʻomaha a hoʻōla.

Pehea ka lōʻihi o ka pono o kāu pēpē i ke kākoʻo e hanu e hilinaʻi ana i ka pane i ka mālama ʻana a me nā mea ʻē aʻe.

ʻO ka hapa nui o nā pēpē me ka CDH e loaʻa i ka ʻoki ʻana e pani i ka puka ma ka diaphragm. ʻO ka wā e hana ʻia ai kēia ʻoki e hilinaʻi ana i ke olakino o kāu pēpē a me nā mea ʻē aʻe. ʻO ka mālama hoʻomau e hōʻoia i ka noho ʻana o ka hoʻoponopono ʻana e komo pinepine i nā X-rays umauma.

Ma hope o ka haʻalele ʻana i ka haukapila, pono paha kāu pēpē i ke kākoʻo hou aku. Hiki ke komo pū kēia i ka oxygen hoʻohui. Hāʻawi ʻia ka oxygen ma o ka paipu plastik lahilahi me nā prongs e kūpono i nā ihu a i ʻole ka paipu lahilahi i hoʻopili ʻia i kahi mask i kau ʻia ma luna o ka ihu a me ka waha. Pono paha ke kākoʻo hānai e kōkua i ka ulu ʻana a me ka hoʻomohala ʻana. Hiki ke hāʻawi ʻia ka lāʻau lapaʻau no nā kūlana e pili ana i ka CDH, e like me ka acid reflux a i ʻole ka pulmonary hypertension.

Hiki i nā hālāwai mālama hoʻomau maʻamau me ka mea hoʻolako mālama ola o kāu keiki ke hoʻoponopono i nā pilikia i ka wā mua.

ʻO ke aʻo ʻana ua loaʻa i kāu pēpē ka hernia diaphragmatic congenital hiki ke lawe mai i kahi ākea o nā manaʻo. Loaʻa paha iā ʻoe nā nīnau he nui e pili ana i ka hoʻolālā mālama no ʻoe a me kāu pēpē.

ʻAʻole pono ʻoe e kū i kēia wale nō. Loaʻa nā kumuwaiwai he nui e kākoʻo iā ʻoe a me kāu pēpē. Inā loaʻa iā ʻoe nā nīnau e pili ana i ke kūlana a me ka mālama ʻana o kāu pēpē, e kamaʻilio me kāu hui mālama ola.