Myelomeningocele

ʻO Myelomeningocele kahi kīnā hānau kahi i pani ʻole ai ka iwi ʻaoʻao a me ka iwi ʻōiwi ma mua o ka hānau ʻana.

ʻO ke ʻano he ʻano o spina bifida.

ʻO ka maʻamau, i ka mahina mua o ka hāpai ʻana, hui pū nā ʻaoʻao ʻelua o ka iwi kuamoʻo o ka pēpē (a i ʻole ka iwi ʻaoʻao) e uhi i ka iwi kuamoo, nā ʻalalā, a me nā meninges (nā mea i uhi ʻia i ka iwi kuamoʻo). ʻO ka lolo e ulu nei a me ka iwi kuamoʻo i kēia kiko i kapa ʻia ka neural tube. ʻO Spina bifida e pili ana i nā kīnā hānau kahi i pau ʻole ai ka ʻōpū neural i ka wahi o ka iwi kuamoo.

ʻO Myelomeningocele kahi kīnā neural tube kahi e pau ʻole ai nā iwi o ka iwi kuamoʻo. ʻO kēia ka hopena i kahi aʻa iwi kīnā ʻole i piha. Kūleʻa ka iwi kuamoʻo a me nā meninges mai ke kua o ke keiki.

Hiki ke hoʻololi i kēia ʻano ma mua o 1 o kēlā me kēia 4,000 pēpē.

ʻO ke koena o nā hihia spina bifida ka mea maʻamau.

• Spina bifida occulta, kahi ʻano i pili ʻole nā ​​iwi o ka iwi kuamoo. Noho ka iwi kuamoʻo a me nā meninges i ka wahi a uhi mau ka ʻili i ka hemahema.
• ʻO Meningoceles, kahi kūlana kahi e puka ai nā meninges mai ka maʻi ʻōiwi. Noho paʻa ka iwi kuamoʻo ma kahi.

ʻO nā maʻi kūlohelohe ʻē aʻe a i ʻole nā ​​kīnā hānau hānau paha i kahi keiki me ka myelomeningocele. ʻEwalu o nā keiki he ʻumi me kēia maʻi he hydrocephalus.

Hiki ke ʻike ʻia kekahi mau maʻi ʻē aʻe o ke kuamoʻo a i ʻole musculoskeletal system, e like me:

• Syringomyelia (kahi cyst piha piha i ka wai i loko o ka iwi kuamoo)
• Hoʻokiʻoki Hip

ʻAʻole ʻike ʻia ke kumu o ka myelomeningocele. Eia nō naʻe, nā pae haʻahaʻa o ka waikawa folic i loko o ke kino o ka wahine ma mua a i ka wā o ka hāpai mua e hōʻike i kahi ʻāpana o kēia ʻano hānau kīnā. He mea nui ka Folic acid (a i ʻole folate) no ka ulu ʻana o ka lolo a me ka iwi kuamoo.

Inā hānau ʻia kahi keiki me myelomeningocele, ʻoi aku ka nui o ka makaʻu o nā keiki o ka wā e hiki mai ana i kēlā ʻohana ma mua o ka lehulehu. Eia nō naʻe, i nā manawa he nui, ʻaʻohe pilina ʻohana. ʻO nā mea e like me ka maʻi diabetes, ka momona, a me ka hoʻohana ʻana i nā lāʻau anti-seizure i ka makuahine e hoʻonui i ka hopena o kēia kīnā.

ʻO kahi keiki hānau hou me kēia maʻi e loaʻa kahi ākea a i ʻole kahi ʻeke piha piha i ka wai ma ka waena a i lalo.

Pākuʻi ʻia paha nā ʻōuli:

• Lilo o ka ʻōpū a i ʻole ka mana ʻōpū
• Hapa a piha ʻole paha o ka naʻau
• Pālua a piha paha i ka lōlō o nā wāwae
• Ka nāwaliwali o nā hiʻupuʻu, nā wāwae, a me nā wāwae o kahi keiki hānau hou

ʻO nā hōʻailona ʻē aʻe a me / a i ʻole nā ​​hōʻailona paha e hoʻopili i

• Nā wāwae a i ʻole nā ​​wāwae kūpono ʻole, e like me ka wāwae wāwae
• Kūkulu o ka wai i loko o ka iwi poʻo (hydrocephalus)

Hiki i ke kānana Prenatal ke kōkua e ʻike i kēia ʻano. I ka lua o ka trimester, hiki i nā wahine hāpai ke hōʻoia i ke koko i kapa ʻia ʻo ka quadruple screen. Mālama kēia pale i ka myelomeningocele, Down Syndrome, a me nā maʻi hānau ʻē aʻe i loko o ka pēpē. ʻO ka hapa nui o nā wahine e hāpai ana i kahi pēpē me ka spina bifida e hoʻonui i ka nui o ka protein i kapa ʻia ʻo alpha fetoprotein (AFP).

Inā maikaʻi ka hōʻike quadruple pale, pono hou e hōʻoia e hōʻoia i ka ʻike.

ʻO ia mau hoʻāʻo e komo pū paha:

• ʻO ke ultrasound hāpai keiki
• ʻO Amniocentesis

Hiki ke ʻike ʻia ʻo Myelomeningocele ma hope o ka hānau ʻana o ke keiki. E hōʻike paha kahi hōʻike neurologic ua nalowale ke keiki i nā hana pili i ke aʻalolo ma lalo o ka hemahema. ʻO kahi laʻana, ke nānā ʻana i ka pane ʻana o ka pēpē i nā pinprick ma nā wahi like ʻole e haʻi ai i kahi e hiki ai i ka pēpē ke hahaʻi i nā manaʻo.

ʻO nā hoʻokolohua i hana ʻia i ka pēpē ma hope o ka hānau ʻana e komo pū paha me nā x-ray, ultrasound, CT, a i ʻole MRI o ka ʻāʻī.

E ʻōlelo paha ka mea mālama ola i ka ʻōlelo aʻoaʻo ʻaoʻao. ʻO ke kīʻaha Intrauterine e pani i ka hemahema (ma mua o ka hānau ʻia ʻana o ka pēpē) hiki ke hōʻemi i ka makaʻu o kekahi mau pilikia ma hope.

Ma hope o ka hānau ʻia o kāu pēpē, hōʻike pinepine ʻia ke ʻoki ʻana e hoʻoponopono i ka kīnā i loko o nā lā mua o ke ola. Ma mua o ke kahakaha ʻana, pono e mālama pono ʻia ke pēpē e hōʻemi i ka hōʻino ʻana i ka iwi kuamoo hōʻike. Hoʻopili paha kēia:

• Mālama kūikawā a me ka hoʻonohonoho ʻana
• Nā hāmeʻa pale
• Nā loli i ke ʻano o ka lawelawe ʻana, hānai ʻana, a me ka ʻauʻau ʻana

ʻO nā keiki i loaʻa pū kekahi i ka hydrocephalus pono paha i kahi ventriculoperitoneal shunt i hoʻonoho ʻia. E kōkua kēia i ka hoʻoheheʻe ʻana i ka wai keu mai nā ventricle (i ka lolo) i ka lua o ka peritoneal (i loko o ka ʻōpū).

Hiki ke hoʻohana ʻia nā lāʻau antibiotic e mālama a pale aku ai i nā maʻi e like me ka meningitis a i ʻole nā ​​maʻi maʻi urinary tract.

Pono ka hapa nui o nā keiki i ka mālama ola no nā pilikia i hopena ʻia mai ka hōʻino ʻana i ka iwi kuamoʻo a me nā aʻa iwi kuamoʻo.

Pākuʻi ʻia kēia:

• Nā pilikia o ka ʻōpū a me ka ʻōpū - Hiki ke kōkua i ke kaomi haʻahaʻa ma luna o ka pākeke i ka hoʻoheheʻe ʻana i ka ʻai. ʻO nā paipu hoʻowahāwahā, i kapa ʻia he catheters, pono pū kekahi. Hiki i nā ʻōkuhi hoʻomaʻamaʻa bowel a me kahi papaʻai fiber kiʻekiʻe ke hoʻomaikaʻi i ka hana ʻōpū
• Nā pilikia Muscle a me nā hui pū - Pono paha ka Orthopedic a i ʻole ke olakino kino e mālama ai i nā hōʻailona musculoskeletal. Pono paha nā pale kila. Hoʻohana nui ka poʻe me ka myelomeningocele i kahi noho huila.

Ke hoʻomau mau nei nā hoʻokolohua ukali ma ke ola o ke keiki. Hana ʻia kēia mau mea iā:

• E hōʻoia i ka holomua ulu
• E mālama i nā pilikia ʻike, neurological, a i ʻole ke kino

Hiki i nā kahu maʻi kipa, nā lawelawe kaiāulu, nā hui kākoʻo, a me nā keʻena kūloko ke hāʻawi i ke kākoʻo naʻau a kōkua me ka mālama ʻana o kahi keiki me kahi myelomeningocele i loaʻa nā pilikia nui a i ʻole nā ​​palena.

ʻO ke komo ʻana i loko o kahi hui kākoʻo spina bifida he mea kōkua paha.

Hiki ke hoʻoponopono pinepine ʻia kahi myelomeningocele, akā ʻaʻole paha e hana maʻamau nā ʻalolo i hoʻopili ʻia. ʻO ke kiʻekiʻe o kahi o nā kīnā ma ke kua o ka pēpē, ʻo ka nui o nā ʻalalā e hoʻopili ʻia.

Me ka mālama mua ʻana, ʻaʻole hoʻopili ʻia ka lōʻihi o ke ola. ʻO nā pilikia kīwī ma muli o ka maikaʻi ʻole o ke kahe ʻana o ka mimi ke kumu maʻamau o ka make.

ʻO ka hapanui o nā keiki e loaʻa ka ʻike maʻamau. Eia nō naʻe, no ka pilikia o ka hydrocephalus a me ka meningitis, e loaʻa ana i nā keiki he nui aʻe i nā pilikia aʻo a me nā maʻi hopu.

Hiki i nā pilikia hou i loko o ka iwi kuamoʻo ke ulu ma hope o ke ola, keu hoʻi ma hope o ka hoʻomaka wikiwiki ʻana o ke keiki i ka wā ʻōpio. Hiki i kēia ke alakaʻi i ka lilo hou o ka hana a me nā pilikia orthopedic e like me ka scoliosis, ka wāwae a me nā kuʻekuʻe wāwae paha, nā pūhaka i hemo, a me nā paʻa paʻa a me nā kuʻikahi.

Hoʻohana nui ka poʻe me myelomeningocele i kahi noho huila.

Komo paha nā pilikia o ka spina bifida:

• Hānau hōʻeha a me ka hānau paʻakikī o ka pēpē
• ʻO nā maʻi maʻi urinary pinepine
• Kūkulu ka wai ma ka lolo (hydrocephalus)
• Ka nalowale ʻana o ka ʻōpū a i ʻole ka mālama ʻana o ka ʻai
• Lolo maʻiʻaʻai (meningitis)
• Nāwaliwali paʻa a paralysis paha o nā wāwae

ʻAʻole paha piha kēia papa inoa.

Kāhea i kāu mea hāʻawi inā:

• ʻIke ʻia kahi ʻeke a wahi ākea paha ma ka iwikuamoʻo o kahi pēpē hou
• Ua lohi kāu keiki i ka hele wāwae a me ke kolo ʻana
• Hoʻolālā nā ʻōuli o ka hydrocephalus, e laʻa me ka puʻu o ka wahi palupalu, ka huhū, ka hiamoe nui, a me nā pilikia hānai
• Hoʻonui nā ʻōuli o ka meningitis, e laʻa me ke kuni, ka ʻāʻī ʻoʻoleʻa, ka huhū, a me ka uē kiʻekiʻe

Hiki i nā mea kōkua Folic acid ke kōkua i ka hoʻoliʻiliʻi i ka pilikia o nā kīnā neural tube e like me myelomeningocele. Paipai ʻia i kēlā me kēia wahine e noʻonoʻo e lilo i hāpai i 0.4 mg o ka folic acid i ka lā. ʻO nā wahine hāpai me ka pilikia nui e pono i nā ana ʻoi aku.

He mea nui e hoʻomanaʻo i nā hemahema o ka waikawa folic e pono e hoʻoponopono ma mua o ka hāpai ʻana, no ka mea, ulu mua nā hemahema.

Hiki ke hoʻopaʻa ʻia nā wahine e hoʻolālā e hāpai e hoʻoholo i ka nui o ka waikawa folic i loko o kā lākou koko.

Meningomyelocele; Spina bifida; ʻ spineiwi cleft; Hemahema Tube Neural (NTD); Hewa hānau - myelomeningocele

• Shunt Ventriculoperitoneal - hoʻokuʻu

• Spina bifida
• Spina bifida (kekelē o ke koʻikoʻi)

Komite no ka hoʻomaʻamaʻa Obstetric, ka hui no ka lāʻau makuahine-Fetal. Ke KulanuiʻAmelika o nā Obstetricians a me nā Gynecologists. Manaʻo ke Kōmike ACOG no. 720: ʻoki makua-fetal no ka myelomeningocele. ʻO Obstet Gynecol. 2017; 130 (3): e164-e167. PMID: 28832491 pubmed.ncbi.nlm.nih.gov/28832491/.

Kinsman SL, Johnston MV. ʻO nā anomalies kūlohelohe o ka ʻōnaehana makaʻu waena. I: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 609.

ʻO Licci M, Guzman R, ʻo Soleman J. Maternal a me nā pilikia o ka obstetric i ka ʻoki ʻana i ka fetal no ka hoʻomaikaʻi ʻana i ka myelomeningocele prenatal: kahi ʻōnaehana loiloi.Kālele Neurosurg. 2019; 47 (4): E11. PMID: 31574465 pubmed.ncbi.nlm.nih.gov/31574465/.

ʻO Wilson P, Stewart J. Meningomyelocele (spina bifida). I: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 732.