Cerebromedullary malformation syndrome

Arnold-Chiari syndrome yisimo anomaly ezinzima azalwa naso lwekhanda ubuchopho bomuntu. essence yayo itholakala kungalingani phakathi nenkundla posterior fossa Yekhanda, nabanye kule ndawo izakhiwo ingqondo. Lokhu kuholela ukungenzi balandela sokwephulwa kwe amalaka cerebellum nobuchopho stem. Imvamisa lezi anomalies kuyinto 4-8 yilesi sifo njalo. Man ayizinkulungwane 95-100 ngayinye.

Okwamanje, pathogenesis lesi sifo akwaziwa. Kucatshangwa ukuthi kukhona izinto ezintathu pathogenic neqhaza lesi sifo:

1. azalwa naso, peripheral lezinceku;
2. Kwenhlekelele ukulimala nhlonhlo-nhlonhlo-occipital kanye hlupha ingxenye kummango ngenxa enhlekeleleni lokuzalwa;
3. CSF igalelo hydrodynamic emdulini we umgogodla intambo emseleni, etholakala maphakathi.

izinhlobo ezine anomalies yabelwe ku-1891, u-Arnold Chiari, sebenzisa lokhu ngezigaba kuze kube namuhla.

Arnold-Chiari syndrome uhlobo ukususwa posterior fossa kogebhezi izakhiwo ezizungezile e umsele umgogodla kuyinto ephansi kakhulu kunalokho ezingeni Magnum foramen. Ngo uhlobo anomaly yesibili samanje dislocation caudal we oblongata medulla, ezindaweni eziphansi worm futhi i-ventricle IV, futhi ngokuvamile ukuthuthukisa iphimbo. Arnold-Chiari syndrome uhlobo lwesithathu ivelakancane, ngoba it libhekene indawo offset zonke izakhiwo ingqondo we fossa posterior. Eyesine imelelwa hypoplasia cerebellar. Imihlobo lesine futhi wesithathu anomalies ezimweni eziningi, engahambisani ukuphila.

Ngo-80% amacala, u-Arnold-Chiari sifo sitholakale nge syringomyelia - zokugula we umgogodla libhekene nokuthuthukisa izinhlumba we umgogodla, okuyinto akhiwa ukususwa posterior fossa kokucindezelwa Yekhanda nesifunda wesibeletho ka emgogodleni. Ukuba khona kwalawa izinhlumba kuholela ukuthuthukiswa myelopathy ezithuthukayo.

Arnold-Chiari syndrome: Izimpawu

Isithombe emitholampilo ye isifo libhekene zimpawu ezilandelayo:

• ubuhlungu entanyeni nezindawo occipital, okwandisa lapho sneezing noma ukhwehlela;
• ukwehlisa izinga lokushisa nobuhlungu ukuzwela, kanye engenhla amandla nemilenze imisipha;
• esitho spasticity;
• isiyezi, yokufikelwa, nedumbe, ukugebenga esebenzisa isihluku ngokuvula ikhanda lakho, kwehle acuity ezibukwayo kanye nekhwalithi umbono.

Ezimweni ezimbi kakhulu kakhulu izimpawu qondanisa zale ukumisa yesikhashana ukuphefumula (apnea), buthakathaka reflex pharyngeal futhi ngokushesha, ukunyakaza okungalindelekile of amehlo.

Kwezinye izimo, u-Arnold Chiari anomaly kungenzeka singabi izimpawu futhi itholakala ngokuphelele ngengozi phakathi izindlela zokuxilonga. Sifo sifo eyenziwa usebenzisa MR imaging futhi computed tomography ekwakheni ezintathu-ntathu of amathambo wesibeletho nangethambo occipital.

Arnold-Chiari syndrome: Ukwelashwa

ukwelashwa Inikezwe incike nesineke nokugasela ngamandla kwalesi sifo. Kulezi izimpawu eziyinhloko njengoba ikhanda kanye nezinye izinhlobo zobuhlungu, udokotela wakho angase akukhuthaze umuthi wezinhlungu futhi njalo bayohlolwa. Ezinye iziguli kubika impumuzo lapho kunokudla izidakamizwa eqeda ukuvuvukala olungavimba noma ungase ulibale ukuhlinzwa.

Ngokuvamile, ukwelashwa, odokotela uthanda ukuchitha kwezinso surgically. Ukusebenza ukumisa ukuqhubeka izinguquko yomgogodla kanye nobuchopho isakhiwo futhi kuqiniswa izimpawu. Ngezinga elithile lapho wenza vmeschatelstva yimpumelelo kuhlinzwa kakhulu kunciphisa ingcindezi emgogodleni cerebellum, kanye uyalulama outflow CSF. Kukhona amasu ezihlukahlukene imisebenzi encike kokuba iphimbo. Isikhathi isilinganiso ukuhlinzwa ingenye nengxenye emahoreni amabili, isikhathi yokutakula ithatha izinsuku ezinhlanu kuya eziyisikhombisa.