Cerebromedullary malformation syndrome

Arnold-Chiari syndrome yeyona anomaly kakhulu naso yentloko ngobuchopho bomntu. kubume bayo siyifumana ukungalingani phakathi ubungakanani ngomva fossa ye lukakayi, kunye nabanye kule ndawo izakhiwo ebuchotsheni. Oku kukhokelela ngokungenzi kulandelwa tonsils copyright kwiCerebellum kunye isiqu ebuchotsheni. Le rhoqo kwezi mgceni na amatyala 4-8 zonke 95-100 amawaka. Man nganye.

Okwangoku, i-Pathogenesis kwesifo akunakuqinisekiswa. Wavele, kukho izinto ezintathu yayintsholongwane eyosulela igalelo lesi sifo:

1. bekhubazekile, peripheral amafa;
2. traumatic kukwenzakala nesinqumka-nesinqumka-Occipital kunye pfumerile inxalenye kwiziphambuka ngenxa sijamelane yokuzalwa;
3. ntwala CSF hydrodynamic eludongeni intambo emisele emqolo, ibekwe embindini.

Iintlobo ezine ze mgaqweni sele inikezelwe kwi-1891 Arnold Chiari, sebenzisa oluhlobo ukuya namhlanje.

Arnold-Chiari uhlobo syndrome ukuba kucinywe ngomva fossa ukakayi kunye nezakhiwo ezingqongileyo kwi emjelweni spinal angaphantsi kakhulu ngaphezu kwenqanaba magnum foramen. Xa uhlobo yesibini anomaly yi dislocation langoku caudal le Oblongata Medulla, kutshona kwezisezantsi iindawo ntshulube kunye ventricle IV, yaye ngokufuthi ukuphuhlisa hydrocephalus. Arnold-Chiari syndrome uhlobo yesithathu kunqabile kakhulu, kuba zibandakanya ontlakantlaka offset zonke izakhiwo ingqondo le fossa ngomva. Uhlobo lwesine imelwe hypoplasia cerebellar. Iindidi yesine kwaye sesithathu mgaqweni kwiimeko ezininzi, engahambelaniyo ubomi.

Ngowe-80% amatyala, Arnold-Chiari syndrome banesifo syringomyelia - ngezifo of umnqonqo luphawulwa uphuhliso cysts ze umnqonqo, leyo akhiwa kucinywe ngomva fossa ucinezelo ngayo, yaba lukakayi, kummandla wesibeleko ze umnqonqo. Ubukho kwezi cysts kukhokelela kuphuhliso myelopathy eqhubekayo.

Arnold-Chiari syndrome: Iimpawu

Lo mfanekiso kwezonyango sifo luphawulwa mpawu zilandelayo:

• intlungu entanyeni kunye neendawo Occipital, nto leyo eyandisa xa uthimla okanye ukhohlela;
• yandisa lobushushu nobuzaza iintlungu, ngokunjalo eliphezulu amandla omzimba kwezihlunu;
• kwemilenze UKUQINA;
• isiyezi, niphele, nedumbe, mandundu sakujika intloko yakho, sinciphise acuity ezibonwayo kunye nomgangatho mbono.

Kwiimeko kakhulu kakhulu iimpawu lungelelanisa ukuya kula elifutshane lwe lokuphefumla (umoya), ukuba buthathaka reflex pharyngeal ngokukhawuleza, iintshukumo ngokuzenzekelayo ye emehlweni.

Kwezinye iimeko, Arnold Chiari anomaly kusenokwenzeka ukuba naziphi na iimpawu kwaye kwafunyanwa ngokupheleleyo ngethuba ngexesha iinkqubo yoxilongo. Lokufunyaniswa sifo lwenziwa usebenzisa MR umfanekiso kunye Itomography ayifum ekwakheni-onamacala amathathu omqolo wesibeleko, kunye ithambo Occipital.

Arnold-Chiari syndrome: Treatment

unyango eyabelwe ixhomekeke kwisigulana kunye nobungozi besi sifo. Kwezi mpawu zaseprayimari intloko ebuhlungu kunye nezinye iintlobo iintlungu, ugqirha wakho iingcebiso amayeza eentlungu kunye ziye kuhlolwa njengesiqhelo. Ezinye izigulane ingxelo isiqabu xa uthatha amachiza-ukudumba ukuba unako ukuthintela okanye ukulibazisa umsebenzi.

Kakhulu, unyango, oogqirha bakhetha ukuchitha ukungaqheleki intsumpa. Olu tyando ukuyeka ukuqhubela phambili utshintsho yomqolo nobuchopho isakhiwo kunye nokuzinziswa iimpawu. Xa wenza vmeschatelstva ngempumelelo yotyando kunciphisa ngokubonakalayo uxinzelelo umnqonqo kunye kwiCerebellum, kwakunye nokuphola ukuphuma CSF. Kukho iindlela ezahlukahlukeneyo imisebenzi ezixhomekeke ukuba hydrocephalus. Ubude avareji zotyando yi enye enesiqingatha ukuya iiyure ezimbini, sokubuyisa ixesha ekuphulukenwe nalo kuthatha iintsuku ezintlanu ukuya esixhenxe.