Cerebromedullary malformation chirwere

Arnold-Chiari chirwere chinhu kwazvo vachiberekwa anomaly romusoro hwomunhu. Chaizvoizvo Its uvete disparity pakati kukura posterior fossa yeDehenya, uye nevamwe munharaunda iyi uropi zvivako. Izvi zvinotungamirira Kunomutsa Mubvunzo vakatevera kukanganisa tonsils cerebellum uye pfungwa hunde. The kakawanda anomalies izvi ndizvo 4-8 nyaya muzana 95-100 zviuru. Man ose.

Iye zvino, pathogenesis chirwere hakunyatsozivikanwi. Anofungidzirwa, pane vatatu pathogenic zvinokonzera kubatsira nyonganyonga ichi:

1. vachiberekwa, nhaka neuropathy;
2. dzechimwe Kukuvara achikanganisa-achikanganisa-occipital uye bvunzurudza mugove ndokudzikunda somugumisiro imwe yokuberekwa mupfungwa;
3. CSF hydrodynamic kurwadzisa pamusoro rusvingo musana canal, riri pakati.

nezvemarudzi mana anomalies dzave dzinounzwa muna 1891 Arnold Chiari, shandisa kupatsanura iyi nhasi.

Arnold-Chiari chirwere mhando ndiyo deletion posterior fossa dehenya uye akapoteredza ezvirevo ari musana Canal dererei zvikuru pane ufanane foramen magnum. In chechipiri mhando anomaly aripo caudal dislocation pamusoro medulla oblongata, dzapasi honye uye IV ventricle, uye kazhinji kutanga hydrocephalus. Arnold-Chiari chirwere ari wechitatu mhando hariwanikwi chaizvo, nokuti kunoratidzwa zvinonyadzisa zvaizopedza zvose uropi zvivako zviri posterior fossa. Mhando chechina chinomiririrwa cerebellar hypoplasia. The Mhando anomalies wechina uye wechitatu kakawanda, haawirirani upenyu.

Muna 80% nyaya, Arnold-Chiari chirwere iri nechirwere syringomyelia - zvokurwara kuti musana hwokuratidza kukura cysts ari musana, ayo inoumbwa deletion posterior fossa compression yeDehenya uye remuromo munharaunda musana. Kuvapo cysts izvi inotungamirira kukura zvishoma nezvishoma myelopathy.

Arnold-Chiari urwere: Zviratidzo

The kunotoda Mufananidzo chirwere kunoratidzwa anotevera zviratidzo:

• marwadzo ari pamutsipa uye occipital nzvimbo, iyo anowedzera apo sneezing kana vangakosora;
• achideredza tembiricha uye marwadzo wanda, uyewo okumusoro nemakumbo tsandanyama simba;
• richiripo spasticity;
• dzungu, kufenda, unsteadiness, kuwedzerwa wekushanduka musoro wako, ungaderedza kuona acuity uye unhu wezvakaonekwa.

In kwazvo Muzviitiko zviratidzo swatanudza kune izvi kwenguva pfupi kumira kufema (kufema), iro simba pamusoro pharyngeal dzepanguva uye kukurumidza, involuntary fambisa kwemaziso.

Mune zvimwe zviitiko, Arnold Chiari anomaly anogona vasina zviratidzo uye rinowanikwa zvachose netsaona munguva chirwere dzokuvhiya. Chirwere chirwere rinoitwa kushandisa MR kuri uye computed tomography pamwe-dimensional matatu vavakezve remuromo vertebrae, uye occipital mapfupa.

Arnold-Chiari urwere: Treatment

Basa marapirwo anoenderana murwere uye kukomba kwechirwere chacho. In izvi chikuru zviratidzo sezvo nomusoro uye dzimwe mhando marwadzo, chiremba wako ringavakurudzira marwadzo mishonga uye kugara kunoonekwa nachiremba. Vamwe varwere yakashuma kuzorora kana kushandisa zvinodhaka Anti-anogumbura anogona kudzivirira kana kunyange kunonoka kuvhiyiwa.

Kakawanda, kurapwa, vanachiremba vanosarudza kupedzera abnormalities surgically. The oparesheni ndiko kumisa kufambira mberi kuchinja ari musana uye pfungwa marongerwo uye Kudzikamiswa zviratidzo. Kana akatakura anobudirira kuvhiya vmeschatelstva yekudyidzana zvinoderedza dzvinyiriro pamusoro musana uye cerebellum, uyewo kuwanazve CSF water drainage. Pane nzira dzakasiyana-siyana remapazi kuti kuvimba kuva hydrocephalus. Avhareji nguva wokuvhiya ndomumwe nehafu kusvika maawa maviri, ari kupora nguva zvinoda mashanu mazuva manomwe.