Hopohopo e pili ana i ka ulu ʻana o ka lolo o kāu pēpē? E kamaʻilio kākou e pili ana i ka Dandy-Walker Syndrome

Ke ʻano nui iki nei ke poʻo o kāu keiki liʻiliʻi? A i ʻole ke manaʻo nei ʻoe ua lohi loa ʻoe e hana i nā mea no ko lākou mau makahiki? I kekahi manawa he mea maʻamau ka makaʻu iki ke ʻike ʻoe i nā mea e like me kēia. I kēia lā e kamaʻilio mākou e pili ana i kahi kūlana i hiki ke lilo i mea ʻeha poʻo no nā mākua, akā hiki ke mālama ʻia inā ʻike ʻoe. ʻO ia ka Dandy-Walker Syndrome . Mai hopohopo, e kamaʻilio mākou e pili ana i ia mea me nā kikoʻī a me ka maʻalahi.

He aha ka Dandy-Walker Syndrome?

I ka ʻōlelo maʻalahi, ʻo Dandy-Walker Syndrome kahi maʻi e kū mai ana i ka wā e hānau ʻia ai kahi pēpē me kahi loli i ke ʻano o ka ulu ʻana o ko lākou lolo . I kekahi manawa ua kapa ʻia kēia ʻo Dandy-Walker Malformation. Hana ʻia kēia i ka wā e noho ana ke pēpē i loko o ka ʻōpū, i ka wā e ulu ana ka lolo. ʻO ke ʻano kēia he maʻi hānau ʻia .

Hoʻopilikia nui kēia i ka cerebellum, ka lolo liʻiliʻi ma ke kua o ko kākou lolo, ma ke kumu lolo , a me ka hakahaka a puni. Ua ʻike anei ʻoe ʻo kēia ʻāpana i kapa ʻia ʻo ka cerebellum ka ʻāpana koʻikoʻi loa o kā kākou ʻōnaehana nerve waena . ʻO ia ka mea e hoʻonohonoho i nā neʻe o ko kākou kino. ʻAʻole wale kēlā, akā kōkua pū kekahi i nā mea ʻē aʻe he nui. E nānā:

• Hoʻokele ia i ke kaulike , ka hoʻonohonoho ʻana, a me ke kūlana o ko mākou kino.
• Kōkua nō hoʻi ia i nā mea e pili ana i ka ʻike .
• Pili pū ko kākou hiki ke noʻonoʻo (cognition) i nā mea e like me ka hoʻomaopopo ʻana a me ka hoʻomanaʻo ʻana i kahi mea.
• ʻO nā mākau motor nā mea e like me ka hoʻopunipuni ʻana i nā lālā.
• He mea kōkua pū kekahi ia i ka kaohi ʻana i ka hana .

Ua kapa ʻia kēia ʻo Dandy-Walker no ka hoʻohanohano ʻana i nā neurosurgeons ʻelua, ʻo Walter Dandy, MD, a me Arthur Walker, MD, nāna i wehewehe i ke ʻano i ka makahiki 1900.

He aha ka mea e hiki mai ana i ka lolo o kekahi me Dandy-Walker?

ʻAe, he aha ka mea e hana ʻia i ka lolo o kekahi me Dandy-Walker? Eia kekahi mau mea e hiki ke hana:

• ʻOi aku ka nui o ka ventricle ʻehā o ka lolo. ʻO kēia ka hakahaka a puni ka cerebellum. ʻO kēia ka mea e kōkua ai i ke kahe ʻana o ka wai cerebrospinal (CSF) ma waena o nā ʻāpana o luna a me lalo o ka lolo a me ke kaula spinal .
• ʻO nā hemispheres ʻelua o ka cerebellum, ʻo ia hoʻi, ka ʻāpana waena e hoʻohui i nā ʻaoʻao ʻelua, ua kapa ʻia ʻo cerebellar vermis , a hiki ke nalowale loa a hapa paha.
• Hoʻokumu ʻia kahi cyst , e like me ka pehu wai, kokoke i ka ventricle ʻehā.
• ʻO ke kumu o ke poʻo, ʻo ia hoʻi, ka fossa hope, e lilo i mea nui aʻe.
• I kekahi manawa, hiki i ka wai cerebrospinal (CSF) ke hōʻiliʻili, e hoʻonui ana i ke kaomi i loko o ke poʻo a e pehu ai. ʻO kēia ka mea a mākou e kapa nei he hydrocephalus .

Pehea ka maʻamau o ka Dandy-Walker Syndrome?

Wahi a ka ʻikepili mai ʻAmelika Hui Pū ʻIa, hoʻopilikia ʻo Dandy-Walker Malformation ma kahi o hoʻokahi i loko o 25,000 a 35,000 mau pēpē . ʻOi aku ka maʻamau i nā kaikamahine ma mua o nā keikikāne.

He aha ke kumu o ka Dandy-Walker Syndrome?

Hiki mai kēia kūlana ke loaʻa kahi pilikia me ka ulu ʻana o ka cerebellum o ka pēpē i loko o ka ʻōpū . I kekahi mau hihia, hiki ke hana ʻia kēia kūlana e kahi mutation genetic .

Loaʻa paha i kekahi poʻe me Dandy-Walker nā kūlana chromosomal . ʻO ke ʻano kēia ua nalowale a keu paha nā ʻāpana o nā chromosomes. Ua ʻike ʻoe, ua like nā chromosomes me nā pūʻolo e lawe ana i kā mākou mau genes. Hiki i ka Dandy-Walker Syndrome ke lilo i ʻāpana o kahi maʻi genetic e kū mai ana me kekahi mau kīnā hānau ʻē aʻe.

Aia kekahi mau kumu ʻē aʻe e hiki ai:

• Hiki ke hoʻoili ʻia kekahi ʻano maʻi mai ka makuahine a i ka pēpē i ka wā hāpai.
• Ka ʻike ʻia ʻana i kekahi mau mea ʻawahia a lāʻau lapaʻau paha i ka wā hāpai.
• Loaʻa i koʻu makuahine ka maʻi diabetes .

I ka manawa hea e hoʻomaka ai nā hōʻailona Dandy-Walker?

I kekahi manawa, ʻike koke ʻia nā hōʻailona a maopopo . I kekahi manawa, loaʻa paha i nā mākua nā hōʻailona me ka ʻike ʻole he hewa kekahi mea.

ʻO ka hapa pinepine, ʻike ʻia nā hōʻailona i loko o nā mahina mua o ke ola o kahi pēpē , akā ʻaʻole paha e ʻike ʻia kekahi mau keiki a hiki i ko lākou mau makahiki 3 a 4 paha .

He aha nā hōʻailona o ka Dandy-Walker Syndrome?

ʻO nā hōʻailona e hiki ke ʻike ʻia i loko o nā pēpē:

• ʻO nā lohi o ka ulu ʻana he lohi ia i ka hoʻokō ʻana i nā pae holomua kūpono i ka makahiki . Eia kekahi laʻana, nā lohi i ka noho ʻana i luna, ke kolo ʻana, a me ka hele wāwae.
• ʻOi aku ka nui o ke poʻo ma mua o ka maʻamau.
• Hypotonia , ʻo ia hoʻi, ua palupalu wale ke kino.
• ʻO ke ʻano o ka spasticity e lilo nā ʻiʻo o ke kino i paʻakikī ke kūlou.

Nā hōʻailona i ʻike ʻia i nā keiki makua:

• Ka luaʻi ʻana, ka ʻūlū ʻana, a me ka huhū - he mau hōʻailona kēia o ka hoʻonui ʻia o ke kaomi i loko o ka lolo.
• ʻO ka nalowale ʻana o ka hoʻonohonoho ʻana, ka hele wāwae ʻana, a me nā neʻe ʻana maʻamau e like me ka ʻūlū ʻana o nā maka - he mau hōʻailona kēia o nā pilikia me ka cerebellum.

Aia paha kekahi mau hōʻailona ʻē aʻe:

• He pehu a puʻupuʻu paha ma ke kua o ke poʻo.
• Nā pilikia me nā aʻalolo e kaohi ana i ka ʻāʻī, ka maka, a me nā maka.
• Nā ʻano ʻē i nā ʻano hanu .
• Nā ʻūlū .
• Nā Kīnā Noʻonoʻo .
• Nā hōʻailona o ka Hydrocephalus .

He aha ka ʻokoʻa ma waena o ka Dandy-Walker Complex a me ka Dandy-Walker Syndrome?

He mea paʻakikī paha kēia, akā e maʻalahi wau. ʻO ka Dandy-Walker Complex kahi hui o nā kūlana i loaʻa nā hōʻailona like. ʻO Dandy-Walker Syndrome kekahi o kēlā mau kūlana.

Aia kekahi mau kūlana ʻē aʻe no ka Dandy-Walker Complex:

• ʻO ka Isolated Cerebellar Vermis Hypoplasia a i ʻole Dandy-Walker Variant : Ma kēia kūlana, ua liʻiliʻi ka cerebellar vermis a ʻaʻole paha i ulu piha. Eia nō naʻe, ʻaʻole ʻike ʻia nā loli ʻē aʻe o ke ʻano i loko o ka lolo o nā keiki me Dandy-Walker Syndrome. ʻO ka hapa nui o nā keiki i ʻike ʻia me kēia kūlana e ulu maʻamau.
• Mega Cisterna Magna : Ma kēia kūlana, ua hoʻonui ʻia ka fossa hope ma ke kua o ke poʻo, akā he mea maʻamau ka cerebellum. ʻAʻole pinepine ka Mega Cisterna Magna i nā pilikia olakino.
• ʻO ka Posterior Fossa Arachnoid Cyst : He cyst kēia e ulu ana ma ka posterior fossa. He mea kākaʻikahi loa i nā keiki ke loaʻa nā hōʻailona ʻoiai inā loaʻa iā lākou kēia ʻano cyst.

He aha nā kūlana ʻē aʻe e pili ana me ka Dandy-Walker Syndrome?

Hiki i nā keiki me Dandy-Walker ke loaʻa nā pilikia me nā ʻāpana ʻē aʻe o ka ʻōnaehana nerve waena. Eia kekahi laʻana:

• ʻO ka nele o ka Corpus Callosum (ACC) he maʻi hānau keiki kakaikahi ia.
• Nā pilikia me ke ʻano o ka hana ʻia ʻana o nā mea e like me nā lālā, ka maka, ka puʻuwai, nā manamana lima.

E loaʻa anei i kaʻu keiki ke kīnā noʻonoʻo?

ʻOi aku ka liʻiliʻi o ka hapalua o nā keiki me Dandy-Walker i loaʻa ke kīnā noʻonoʻo . ʻO ke kīnā noʻonoʻo ka mea maʻamau i waena o nā keiki me Dandy-Walker nona nā kūlana penei:

• ʻO ka hydrocephalus koʻikoʻi.
• Nā Kūlana Chromosomal .
• Nā kūlana hānau ʻē aʻe.

E hoʻomanaʻo, he ʻokoʻa kēlā me kēia keiki, no laila, ʻaʻole like ka ʻike o kekahi keiki me ko kekahi.

Pehea e ʻike ʻia ai ka maʻi Dandy-Walker?

I kekahi manawa, ʻike paha nā kauka a i ʻole nā ​​mākua ua nui loa ke poʻo o ke keiki . A i ʻole e ʻike paha lākou ʻaʻole i hoʻokō ke keiki i nā pae hoʻomohala . Hiki i nā kauka ke kauoha i nā hoʻokolohua kiʻi lolo e ʻike iā Dandy-Walker. Hiki i kēia mau hoʻokolohua ke komo pū me:

• Hoʻokolokolo ʻana o ka ultrasound .
• Kiʻi CT .
• Nānā MRI .

I kekahi manawa, hiki ke ʻike ʻia kēia ʻano i ka wā o ka ultrasound prenatal a i ʻole ka MRI fetal ma mua o ka hānau ʻana o ke pēpē.

Inā loaʻa iā Dandy-Walker kaʻu keiki, pono anei i ka ʻohana ke hana i nā hoʻāʻo genetic?

Inā loaʻa i kāu keiki ʻo Dandy-Walker Malformation, he mea nui e kamaʻilio me kāu kauka e pili ana i ke aʻoaʻo genetic . Hiki i kahi hapa liʻiliʻi o ka poʻe me kēia maʻi ke loaʻa i kahi lālā o ka ʻohana me ke ʻano. No ka mea aia paha kahi ʻāpana genetic i loko, nui nā kauka e paipai i ka hoʻāʻo genetic .

He aha nā lāʻau lapaʻau no ka Dandy-Walker Syndrome?

Aia ka lāʻau lapaʻau i nā hōʻailona o ka Dandy-Walker Syndrome. He mea nui e loaʻa kahi hoʻokolokolo piha e ke kauka ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau. No ka laʻana, hiki i nā kauka ke ʻōlelo i kēia mau mea:

• ʻO Ventriculoperitoneal (VP) Shunt : Hoʻokomo nā kauka lapaʻau i kahi mea liʻiliʻi i kapa ʻia ʻo VP shunt e wehe i ke kahe keu mai ka lolo. Hiki i kēia shunt ke hōʻemi i ke kaomi ma ka lolo a hoʻomaikaʻi i nā hōʻailona.
• Nā lāʻau lapaʻau : Hiki i ke kauka o kāu keiki ke kuhikuhi i nā lāʻau lapaʻau e kāohi i nā hopu ʻana.
• Lapaʻau : Kōkua ka hoʻōla kino a me ka hoʻōla hana i nā keiki e mālama i ka ikaika o nā ʻiʻo. Hiki i nā mea hoʻōla ke aʻo i nā keiki i nā ala hou e hana ai i nā hana o kēlā me kēia lā. Kōkua ka hoʻōla ʻōlelo me ka hoʻomohala ʻōlelo a me ka ʻōlelo.
• Hoʻonaʻauao Kūikawā : Kōkua kahi ʻano aʻo i hoʻopilikino ʻia i nā keiki e hoʻokō i kā lākou mau pahuhopu hoʻonaʻauao a me nā pahuhopu pili kanaka.

He aha ka wā e hiki mai ana o nā keiki me ka Dandy-Walker Syndrome?

Hoʻoholo ʻia ka wā e hiki mai ana a me ke ola o kāu keiki e kona kūlana.Aia nō ia i ke koʻikoʻi o kona ʻano , a inā he mau maʻi hānau ʻē aʻe paha i loaʻa i ke keiki.

Hiki i nā kānaka me kēia maʻi ke loaʻa nā ʻano ʻike like ʻole. Loaʻa i kekahi nā hōʻailona iki . Loaʻa i kekahi ke kīnā koʻikoʻi . Hiki i kekahi mau keiki ke hoʻokō i nā hiki noʻonoʻo maʻamau me kahi hoʻolālā lapaʻau kūpono. No kekahi poʻe, ʻoiai inā ʻike koke kā lākou hui lapaʻau a mālama i ke ʻano, ʻaʻole paha lākou e hiki i kēlā pae.

Hiki ke pale ʻia ka Dandy-Walker Syndrome?

ʻAʻohe ala i ʻike ʻia e pale aku ai i ka Dandy-Walker Syndrome. Eia nō naʻe, ʻo ka mālama mau ʻana i ka wā hāpai e hāʻawi iā ʻoe i ka manawa kūpono loa e loaʻa ai kahi hāpai olakino. E hahai i nā ʻōlelo aʻoaʻo a kāu kauka e noho olakino i kou wā hāpai.

Pehea e mālama ai i kaʻu keiki me kahi Dandy-Walker?

Hiki i ke komo mua ʻana ke hāʻawi i kāu keiki i ka manawa kūpono loa no ka mālama pono ʻana. Inā ʻike ʻoe ʻaʻole i hoʻokō kāu keiki i nā pae hoʻomohala e like me ka noho ʻana, ka hele wāwae ʻana, a i ʻole ke kamaʻilio ʻana i ka manawa kūpono, e ʻike i kāu kauka. A laila hiki iā lākou ke hana i kahi hōʻailona pololei a hāʻawi i ka hoʻolālā mālama kūpono loa no kāu keiki.

Hiki i ka hui mālama Dandy-Walker o kāu keiki ke komo pū me:

• Kauka keiki .
• Nā Loea Hoʻomohala .
• Nā Kauka ʻŌlelo, Hana, a me nā Kauka Hoʻōla Kino .
• Nā ʻOihana Hoʻonaʻauao Kūikawā .

He aha kaʻu e nīnau aku ai i kaʻu kauka e pili ana i ka Dandy-Walker Syndrome?

Inā ʻike ʻia kāu keiki me Dandy-Walker, e nīnau i kāu kauka e pili ana i kēia mau mea:

• E pono anei kaʻu keiki i kahi shunt ?
• He aha nā maʻi ʻē aʻe i loaʻa i kaʻu keiki?
• Pehea lā ke ʻano o ka wā e hiki mai ana (nānā ʻana) o kaʻu keiki?
• Pehea e hiki ai iā mākou ke hoʻomaikaʻi i nā hōʻailona o kaʻu keiki?
• Pono anei mākou e hana i nā hoʻokolohua genetic ?

ʻO ka mea hope loa, e hoʻomanaʻo i kēia.

ʻO Dandy-Walker Syndrome, i ʻike ʻia hoʻi ʻo Dandy-Walker Malformation, he maʻi lolo ia e ulu ana ma mua o ka hānau ʻana o kahi pēpē. Hoʻopaneʻe pinepine nā pēpē me Dandy-Walker i nā pae hoʻomohala. Pono kekahi mau keiki i kahi shunt e wehe i ka wai keu mai ka lolo. Hiki i nā lāʻau lapaʻau ke kōkua i nā keiki e hoʻokele i nā hana o kēlā me kēia lā, holomua i ke kula, a ola i nā ola piha. Inā ʻike ʻoe ua nui loa ke poʻo o kāu keiki a ʻaʻole paha lākou e noho ana, e hele ana, a e kamaʻilio ana paha e like me ka mea i manaʻo ʻia, e kamaʻilio me kāu kauka. He mea nui ka ʻike mua ʻana a me ka mālama kūpono. Mai hopohopo, hiki ke mālama ʻia kēia maʻi me ka ʻōlelo aʻo lapaʻau.

ʻO ka maʻi Dandy-Walker, nā kīnā o ka lolo, nā kīnā hānau, cerebellum, hydrocephalus, nā lohi o ka ulu ʻana, olakino o ke keiki