Pierre Robin maʻi pale: Hawaii photo, kumu, lapaʻau, ka hopena. Pepeiaohao nui poʻe me ka maʻi pale o Pierre Robin

Pierre Robin maʻi pale - pathology o ka fetus i loko o ka opu. Ke keiki diagnosed mandibular hypoplasia, i ua pu ma ke alo aku o ka mauae kīleo arcuate a me ka airway iaʻi. Ke haiahū kūmau mālama i loko o kekahi keiki mawaena o ka 30,000 na kamalii uuku.

He aha ka i kēia maʻi?

I ka maʻi i ole nā hui pili koko a me ka nohona mau kaʻao. Ia e ae aku e like me ka poe hune, ka poe mai ole i pono ola mālama, a me ka waiwai kaulana kanaka. Pierre Robin maʻi pale hou pili i kekahi mau hiʻona. Mua o nā mea a pau no na keiki e ae aku, mai o lakou, wehewehe aku ma ka mea uuku loa Tcea. Inā kona nui mea kokoke i ka hae, a laila ka mea i e loa ectopic hoʻi: ka huina o ka puniu wale waihona ipu mea nui loa, a ua ole ke hoʻohana maʻamau. Pela kīleo i oi convex kinona i loko o ke kino o ka piʻo, a me ka alelo ua aia kokoke i ka luna hoʻi o ka puu.

Ka maʻi pale i ka inoa i loko o ka hanohano o ka nui kaulana kahua niho surgeon Pierre Robin. Medic Ua hānauʻia i ka 1867, make aku la - i ka 1950. No ka aneane hoʻokahi haneri makahiki o kona ola anaʻo ia i hana i ka luna hoʻoponopono o ka Magazine "kahua niho Review", e kākau i ia hoʻi ma luna o ka ulu ana o na niho a me ka kūlana e kamaiʻilio ana i loko o ke kaʻina hana, lilo i ka mea kākau o ka hanohano akeakamai hana ma hoʻopololei niho. Ua i ka mea nana i wehe i ka maʻi pale, i ke kumu nui hiʻona o i ka poe hana pela anatomical hiʻona: uuku loa ke poo i ke kao keiki, uuku emi ana chin, a kūpono 'ole o ka' ōlelo. Ke keiki, ua hiki ole i ka hanu, nau, a ke ale wale iho. Inā ka lolo ua poino, ka mea hiki ke malama noʻonoʻo retardation. Nō hoʻi, i kēia mau keiki i ole pono ulu kāohi.

Mele kamepiula kumumea

Pierre Robin maʻi pale Ua kaʻawale. Ma nā hihia, ia mea hapa o ka aaiaoe abnormalities. Actuating une lōhai o ka likeʻole ma mua o ka maʻi lawelawe 'ana mīkini'opihia iwi i loko o ka embryo. Kona ulu ka hoʻonāukiukiʻana mau hāpai keiki a me na mea uuku kūloko sila i loko o ka wahine uterus like me ka me ka makaʻu no, hikoko, cysts, fibrosis. Eia hou, i ka mandibular ulu retardation i loko o ka fetus hoʻonāukiukiʻana mai o kekahi mea hoʻomaʻi maʻiʻaʻai i loko o ka makuahine, i ka trimester mua o ka hāpai keiki, nele no ka mea, o ka wikamina B9 a neurotrophic kāna hana.

Me ka pili ana i genetically paa Pierre Robin maʻi pale, ia mea autosomal Hoʻohaku. Pinepine keia pathology ua pu ma'ē aʻe kāna hana. No ka laʻana, hoʻokahi-kolu o na hihia a pau e kau mai maluna Stickler maʻi pale, kahi i ulu deflection, e like me ia i ho'ākāka 'ia e Pierre Robin, a nānā i ka hai arthritis a me ka myopia. Inā na kupuna mua pēpē i hanau me ka noho kaʻawaleʻana ano o pathology, ka pahiki o ka hanau ana o ke keiki lua o ka hoʻomanawanui mea 1-5%. I ka wā o ka aaiaoe ano o ke kamepiula maoli he kūpono kou.

ho okumu ana o pathology

E maopopo i ka pā o ka haiahū kūmau, ia mea pono e ike pehea a me ke kumu o ka fetus e hoʻomōhala ana i Pierre Robin maʻi pale. Ke kumu, e olelo kauka, i ka pololei a me ka koke loulou me ka etymological ololi i loko o ka embryonicʻilikai. Ua ua ike ia ma ka hebedoma 5 o ke keiki i loko o ka opu, e hoʻomaka i ke kaʻina hana o ka ho okumu i paakiki kīleo ku i ke kuʻi nukeliu o ka medial nā wānana waiwai i ka poʻe hana i ka mea lehelehe luna, a me nā puka ihu. Inā ma kēia kahua, he mea iʻaihue, ke kīleo a me ka lehelehe bifurcated. Kela manawa, hoomaka ae la 5 pule, e lilo haumāna kīleo o kaʻaoʻao o ka hua o ka lani. Ia laua 'ōlelo palena manawa o ke kanaka, a maoli he ka makahiki no ka hooulu ana i ku una hale. 'Ōlelo i lalo i wahi, a me ka lani, ua paʻa keʻano o ka hanaʻana me ka uila buckle.

Pierre Robin maʻi pale i ka wa a ka lalo iwi ā mea uuku loa. No ka mea, o keia 'ōlelo i ole wahi e lawa, a me ka mea i hoʻololi i ka hope paia. Aia ma luna o ka mea maʻamau kaʻilikai, e ālai 'ia ka pilina lani keʻano o ka hanaʻana. Kēia, ma ka huli,ʻo ia ka mea i ka pahee kīleo pani ole loa, ho okumu i ka pio mai shape.

pathogenesis

I mea e loaa i ka piha informative hōʻike e pili ana i ka maʻi, e makemake eʻike i nā kiʻi o nā keiki a me Pierre Robin maʻi pale. Photos o kēia mau keiki i okoa mai ka mau kiʻi o nā keiki. Haiahū kūmau pinepine waiwai pio i ke ano o ke keiki:ʻo ia ua loa ka waha, underdeveloped haʻahaʻa maka a me ka retrognatiya - hope kulana o na luna a lalo iwi i ka iwipoo. Language pela ikaika 'oni iki: ilaila glossoptoz.

Ke keiki ua mau malama airway iaʻi - ka ai-kapa iaʻi. Baby haʻelemakule. Kona kulana ua hoʻomaikaʻi wale iloko o ke kino paha, e hea ana ma hope o kā lākou mau iaʻi ia lŘlŘ hou. Ke Kula Kaiapuni 'naehana kāna hana complicate hanai ana i hāʻaweʻia, no laila ka mea, ua pinepine ole hou i ke reflexively hea, a hookuu i ka ÷ naehana hanu'āpana. E like me ka hopena, i ka bebe i ho onui ana ke kaupaonaʻana,ʻo ia, ua nawaliwali ia a me ka sluggish. Inā i hana e ia, i ka hemahema kalapona ke alakai koke i ka depletion o ke kino i ka mea uuku, he piha cessation o ka functioning o kekahi mau 'oihana, a hiki i ka make ana o ke keiki.

loa o ka maʻi

Pierre Robin maʻi pale loaʻa paha kekahi mau pepa i oko ma ke degere o ka maʻi oolea:

• Easy palapala. A keikiʻike piʻi nīnūnē pilikia a me nā hana a he leo i ka ai ana. Kona hanu 'utrudneno. Ma keia hihia, ke kauka prescribes makeʻe kulana Inc, i mea ka positional a me ka mālama 'ia i loko o ka outpatient okoʻa no ka manawa.
• Ka awelika degere o ka oolea. Iloko o ka mōhai i ke kao keiki Kahalaopuna lōpū pilikia me ka māna a me ka moni 'ia. He loaa ka mea paakiki i ka hanu, akā, i ke kulana mea,ʻaʻole pilikia. Nō kāu, ua hoʻokomo i ka haukapila, kahi positional i hoakaka ia lapaʻau ia i kekahi keiki, a pana aku au i glossopeksiyu - 'ōlelo puakai, a fixation i ka lalo lehelehe.
• Loa palapala. Tot ka loa paʻakikī i hiki 'ola, e ai i ka ai, a me ka hanu. Ma keia kulana home 'ole pono kaʻenaʻena hana -ʻoki kino. Kekahi kanaka paipai aku hanai ana, e komo i ka naʻimi, a lawe mai i ka tracheostomy - he dissection o ka trachea, a me ka hoike i loko o ka mea, o ka Tube.

Me kekahi degere o ka oolea o ka maʻi Pono, e kukakuka lakou i ka hoao ana i ka hoʻomalu. Pakiko-ka lapaʻauʻia ke pilikia i ka pēpē, i kaumaha irreversible hopena.

complications

Diagnose i ka hoʻomanawanui bebe mea,ʻaʻole paʻakikī. Akā, i ka hua'ōlelo e nīnau i ka hoao ana i ka orthodontist a me aaiaoe?. Together a me nā 'inikua e waiho aku i kekahi mau hana i mea e pale aku ka hopena nui. Ma kēia hihia, na complications hiki e loa okoa: suffocation i ka hiamoe, i ka ulu ana o ka laryngeal kina, i ka helehelena o ka walaʻau, raucous hanu. Ka hoouka kaua a me ka Holomua o ka naau maʻi i hoʻonāukiukiʻana mai o Pierre Robin maʻi pale: hopena o keia ano e malama i ka 14% o nā keiki.

A keiki e loaa ana mai o keia maʻi, e ka loa ino i ka ai. Ka hopena maikaʻi puʻe pono i breastfeeding, palatal mauae e leie aku oe, e ho okumu ana mai. Eia hou, keia kamaiki mea uuku loa haʻahaʻa iwia, a aole e haawi ia ia i ka hoopai oki hoʻopoepoe nāʻiʻo o ka waha a me ka paa Haiʻina mai umauma i ka makuwahine. I ka hanai ana i ka hoʻi i hāʻawe hiki ke kinai iho e like me palatal mauae mea hoʻokaʻawale i ke nasal a me ka moʻolelo wā, mahuahua i ka pilikia o ka suffocation. Mawaena o na complications, kauka i kapa deformation o nā niho, malocclusion, maʻi pepeiao nā mea palahēhē, a hiki e alakai i ka pepeiao kuli, a me ka hiki stunted kino a me ka noʻonoʻo 'ulu.

makeʻe kulana lapaʻau

Manawa, e uku no ka palapala hōʻoia no mālama ', na makua i ka manawa pono e hoʻopau i ka Pierre Robin maʻi pale. Iapaau ana hiki pela ke conserved. Mua, ke kamaiki mea ma lalo o ia a eia i loko o hoʻomakākiu aaiaoe?, Otolaryngologist, ophthalmologist, he orthodontist, he dentist, a me ka olelo kino. ʻO ka lua, i ke keiki hooponopono i ke kaʻina hana o ka ai ana a me ka ho'āʻo e hoʻopau i ka iaʻi o ke keena airway. Inā e hoopili aku i nā mea a pau i ke koi, alaila, i ka 6 makahiki, loa na keiki i ka nui a me ke kinona o ka lalo iwi ā i nā normalized.

A nui loa ka hoʻokō hana o ka makeʻe kulana lapaʻau mea positional Inc. Ua komo ai kau i ke kamaiki ma kona'ōpū i na manawa a pau e ia i ka mea ulu o ka lalo iwi ā. I ka wā kamaliʻi, he kamaiki e mau ia i loko o kēia kūlana. A hiki i ke keiki ulu mai, nā mākua Pono e ao aku ia ia e hiamoe wale nō i loko o ka hookahi kulou. Inā ka i hāʻaweʻia e moe ana ma luna o kona opu, oia hana i ka puʻe i loko o ke poo ma ka umekaumaha hoʻololi ai a me ka'ōlelo i ka wahi. Kēia Inc kōkua i loko o 80% o ka hihia.

ʻoki lāʻau lapaʻau 'uao

He pono no ka kaumaha a me ka meakino ano o ka maʻi. Pierre Robin maʻi pale i ke hapa 'ia' eliminated hoʻohana 'ana i ka hana ana' ōlelo puakai, a hoʻopunipuni ia i loko o ka lalo lehelehe wahi. Ke kaʻina hana, i kapa glossopeksiya, noonoo ole a me ka ole pilikia loa. Nō hoʻi, keiki hiki hou i ka trachea a me ka Tube hookomoia i loko o ka lua: no ka mea, o keia ka mea, e hiki e ke hanu hoopuni, a me ka pilikia. Pani i kēia puka i loko o kahi o 3 makahiki. Ka papa hana ua kākaʻikahi hoʻohana ', he nele ma kahi o 1% o ke keiki me ka loa mai ka manaʻo pohihihi.

Kauka e hāʻawi aku mīkini'opihia-ka hihia osteosynthesis. Ke kaʻina hana kŰia breaching clippings i loko o ka iwi o ka iwi ā lalo. Ka mea, e hoʻokomo i loko o ka makia e paʻa ai i ke poo, a me ka kūikawāʻano papa hana no ka hoʻonui a me ka mahuahua o ka iwi ā. Kela a me keia lā, 'ō ka hope o ka hoʻololiʻana, ke kahuna lapaau hoʻokō' ia he kali hoʻi koʻu pono akā, hōʻai'ē o ka malalo o ke alo. Oia ka palena manawa ua lawe mai no i ka mahina, a laila, e ka mokuāhana ma no ka 8 pule. He pono no ka maʻamau iwi hoola a me kona ike loea fixation i loko o ka hou ho okumu.

kōkua maikaʻi kėia manawa

Children me Pierre Robin maʻi pale kauoha i kekahi loli ma o ka ai ana kaʻina. Mua, ka mea, pono e hoonui i ka caloric ka maʻiʻo o ka waiu. A hiki i kēia hopena umauma decanted a hui me kaʻimi hoʻopunipuni kekahi. Ka lua, i, e ai ana pinepine pono e lawe, mai hemo mai, e hāʻawi aku i kāu keiki i ka manawa e hopu i kou hanu, a me ke kōkua o ke kahea ana a me ka luaʻi ae hoomaemae loa oia i ka airway. Eia hou, ina o ka makuahine e stroking kona chin i hāʻaweʻia, ka mea nui e kokua ia e ka hoʻokemuʻana i ka waiū.

Apana, i kēia mau keiki pono, e kūʻai i ka kūikawā hue, papahana e akamai no ka hanai ana keiki me ka maʻi pale. Hole i loko o ia mea nui lehulehu ma mua i loko o kaʻike mau nipple, ma ka hou, he mea he kūikawā kiwikā e leie aku ka makua i ka mana i ke kahe ana o ka waiu. No na kamalii uuku, a me kona poe papahana pacifiers. Fourthly, lapaau e paipai aku, e hookupaa ai ia i loko o ka moʻolelo lua tot obturator e pani i ka naele. Mākou e mai poina i kekahi o ka mea ma luna o kāu kiʻina hana nei i ke ala o ka lapaau. He mea pono i ka manaʻo, e hōʻeleu i, a kōkua i ka laulā o ka Inc ke ano o kou hoʻi i hāʻawe.