Klippel - Feil chirwere: photo, kurapwa, chii zvaitika angava? Sprengel chirwere - Isingatadzikari - Klippel: muchiitwa ari pool

Two wetsinga French Andre Feil uye Moris Klippel muna 1912, akarondedzera zvakadzama vachiberekwa deformities ari remuromo-thoracal nomuzongoza vana. Vose vakaberekerwa vabereki rimwe ropa ukama. KuMiddle Ages, nomutemo madzimai anogona kuva kunyange voukama. Naizvozvo, kuwanda anomaly ichi hazvishamisi. Papera zvokurwara ainzi pashure masayendisiti. Klippel - Feil chirwere haisi chirwere zvakazara. Ihosha vachiberekwa, izvo kazhinji kunotungamirira kune dzimwe musana pathologies.

Zvakakurukurwa chirwere

Under chirwere Klippel - Feil rinoreva vachiberekwa nepfungwa yacho remuromo nomuzongoza, wakaumbwa synostosis uye kuderedza vertebrae. It kunoratidzwa refu kakawanda kuratidzwa - nyaya imwe 120 000 kuzvarwa. The kupfuura chairwo chepfungwa chirwere anoonekwa kuti anyatsoona zvinoderedza Kureba mutsipa. Kazhinji, aine vamwe abnormalities ari zvetsandanyama nemapfupa emuviri uye vachiberekwa abnormalities pakati zvomukati. In nechirwere chirwere iri kupinda akawanda vanachiremba :. Neurology, orthopedics, genetics, etc. weConservative kurapwa kunosanganisira mumuviri kurapa, physiotherapy uye masaja. In yakanyanya kuoma zviitiko zvinoda kuvhiya - kuvhiya tservikalizatsii.

The kukonzera malformations

Klippel - Feil chirwere rinoreva majini kutsunga zvirwere. Kumwe kuchinja kunotanga mumuviri kutanga kuitika muvhiki dzokutanga pashure pamuviri. Pakati huru zvikonzero vanachiremba chirwere wakadaro Makuriro chirwere pamusoro musana uye segmentation, kunyanya mu verhnesheykovom pamwero. Kuvakwa synostosis thoracal uye remuromo vertebrae, nokuderedza nhamba yavo, nonunion miviri uye namaberere kubvumira tinosarudza kunotoda mufananidzo chirwere. Boka pangozi zvinosanganisira vana vane vanoshorwa nhodzera, apo pane:

1. The majini urema ari okutodza. In mwana anorwara kutyora paumbwe Kusiyanisa kukura zvakakodzera kuti kusimudzira zvakakwana marangwanda. Izvi ndezvechokwadi zvinoratidzwa kukura thoracal uye remuromo vertebrae madhipatimendi.
2. Autosomal chikuru mufananidzo nhaka. Kana mumwe mubereki ane chirwere, mukana yokuva mwana pamwe abnormalities ndiyo 50-100%.
3. Autosomal recessive nhaka. Panyaya iyi, mukana yokuva mwana abnormalities ose 0-50%.

Kuti vadzivise vanoshorwa nhaka vabereki vanofanira kutanga geneticist panguva padariro kwekuronga mwana.

Typical zviratidzo zvechirwere urwere

chirwere ichi kunoratidzwa ne chaiwo vanamwari vatatu vatatu zviratidzo: kunyanya pfupi mutsipa, kuchinja hairline, zvava kundiomera kufambisa muviri musoro. The chirwere Kazhinji kubatanidzwa nemamwe pathologies. Muna 30% wevarwere yakaratidza scoliosis, torticollis vanachandagwinyira chimiro, mukuru akamira mashizha - Sprengel chirwere. Mune zvimwe zviitiko vaichengeta abnormalities okumusoro maoko nemakumbo, tsoka deformation, mazino, chiso asymmetry, hyperopia. Muna 25% wevarwere nechirwere vachiberekwa kusanzwa.

Klippel chirwere - Isingatadzikari - Sprengel - haasi chete akaratidza urema zvizorwa. Angasanganisira chetsinga zviratidzo. Zvakachinja CNS tichiratidza mupfungwa retardation, hydrocephalus, netsviyo. Kubvira vachiri vaduku murwere ane tsandanyama kusimba wamativi, uyewo synkinesis. Pana mazera vakura, zvinonakisa chiremba mufananidzo vaisanganisa nokuda yechipiri kuchinja kubva nomuzongoza.

Medical ongororo

Verification chirwere chinogona kwakavakirwa akaona vanamwari vatatu vatatu zviratidzo, panyama kuongororwa Data uye richishanda bvunzo. A rinokosha muchinangwa nechirwere yakapiwa kudzidza nhoroondo yemhuri murwere. Set Klippel - Feil chirwere nematsananguriro yakatodzama kuenda anomalies zvichida zvichikonzerwa kwakarongeka basa unyanzvi. Kazhinji, izvozvo neurologists, nhaka kwezvinhu, orthopedists, cardiologists uye pulmonologists.

Kuongorora hunhu kuchinja basa vertebral radiography. Kudzidza chaiitirwa zviviri fungidziro, iyo zvikuru mashoko yemanyorero ndiyo lateral. Nokuda uncharacteristic chinzvimbo mumvuri dehenya musoro dzichienderana mufananidzo musana mbiru, zvichiita kuti zviome kuziva zvinhu. Uyezve, Zvinokurudzirwa kutora mifananidzo pa kunonyanyisa flexion / kuwedzera mumutsipa. Izvi zvinobvumira kuona zvinoita kusagadzikana ununited vertebrae uye ongorora chirwere Klippel - Feil. Maekisirei muromo wechibereko urege-thoracal nomuzongoza rinobatsira kuziva:

• akaremara vertebrae;
• dzikira nokuwanda;
• Fusion of vertebral miviri;
• zvemamiriro yakamira mashizha;
• curvature of nomuzongoza.

Pashure pokusimbisa chirwere varwere anowedzera yakatarwa Ultrasound kuti nhengo dzomukati kuziva anomalies mu maumbirwo yavo. In pamberi chetsinga abnormalities kungada midziyo Doppler Ultrasound, angiography, eeg uye MRI of remuromo nomuzongoza. Zviri rokumanikidzirwa kunoona chiremba-geneticist. Maererano ahwo nyanzvi achakwanisa kuona chimiro nhaka, uye kuongorora zvinokanganisa zvizvarwa zvomunguva yemberi.

The chikuru chimiro chirwere

Klippel chirwere - Feil haiwanzowaniki chaizvo chirwere. Maererano nenhamba, yokuita zviratidzo vari panoonekwa mune mwana mumwe kubva 120 zviuru. The anomaly anooneka murwere mumwe matatu siyana:

• Kuderedza uwandu zvikamu zviri remuromo nomuzongoza, izvo zvishoma kubatanidza uye nemaziso kupfupisa mutsipa. chimiro ichi zvokurwara kunokonzera matambudziko kana uchifamba musoro.
• Synostosis remuromo nomuzongoza Fusion necheseri pamwe occipital pfupa. Murwere haagoni kutendeuka musoro wake pasina kurwadziwa. Remuromo vertebrae uye mutsipa tiri mumwe.
• Chechitatu chimiro chirwere kuratidzwa zvinosanganisira maviri okutanga.

A Kunyanya musiyano wezvinyorwa pamusoro chirwere chinogona tsunga chiremba chete pachishandiswa kuongorora zvabuda.

Basic misimboti kurapwa

Zvinosuruvarisa mushonga ano haagoni kupa zvakakwana nzira sei kubvisa zvachose chirwere pamusoro Klippel - Feil. Treatment kunosanganisira kutadzisa kukura yechipiri deformities. Sokugamuchira hwaro kuchinja nzira, iyo zvinosanganisira kurapa uye masaja. Drug kurapwa hunopiwa chiitiko kuti chirwere muitiro iri pamwe marwadzo uye kusagadzikana compression tsinga midzi. Kazhinji, vakashandisa analgesics, Anti-anogumbura uye non-steroidal zvinodhaka. Refu compression of etsinga midzi inoda kuvhiyiwa. Chinangwa chikuru kuvhiyiwa - kubvisa marwadzo ubatanidzwewo dzakarurama kwokunze kuremara.

kurapa mumuviri uye kurovedza mudziva

Kuchinja mabatiro of chirwere zvinosanganisira kurapa Chokwadi, kuchirapa masaja pamusoro remuromo musana uye vanonzi nzvimbo mutsipa. Exercises vanosarudzwa nomumwe, kwakavakirwa kunotoda mufananidzo murwere. nguva yokutanga, vanofanira kuitwa pasi pokudzora nyanzvi, unogona zvino anozodzidza epamba zvinhu. Varwere chirwere kuti "Sprengel chirwere - Isingatadzikari - Klippel" dziva kurovedza kudzivisa munhu yakaoma weboka chirwere. Kazhinji, varwere vaduku, vanachiremba vanoti backstroke. zviitwa More kunzwisisa uyewo vakasarudzwa nomumwe. mazano Universal panyaya swimming jimunasitikisi haako.

kuvhiya kurapwa

Persistent marwadzo anokonzerwa tsinga mudzi compression ndiyo chiratidzo chikuru kuvhiyiwa. Varwere chirwere kuti "chirwere Klippel - Feil" fungidziro Zvinoipisisa nezera. Naizvozvo, kuvhiyiwa iri basa pakarepo mushure Kusimbiswa zvokurwara. Kuwedzera mufambo kwemitsipa rinoshandiswa kuti tservikalizatsiya Bonola. Munguva nzira, chiremba anobvisa pamusoro mana mbabvu uye periosteum kuti kudzibatsira pamusoro zvomukati. The oparesheni rinoitwa mumatanho dzakawanda. Pakutanga akabvisa mbabvu rumwe rutivi uye, pashure kuzoita muviri - kune mumwe.

Rehabilitation nguva kunoda nguva uye kushanda nesimba. Kwemwedzi yakati kuti, murwere inofanira kuva pa zororo. Zvisinei, kurega kushanda hakusi zvakakodzera. Pari ndiyo chete inoshanda nzira kuchinja kwokunze kuremara, panowanikwa iyo kunokonzera kuti chirwere Klippel - Feil. Migumisiro utano, kana isu kusiya kurapwa, anorondedzerwa pazasi.

Matambudziko uye fungidziro murwere

Vavhiyiwa uye vapore nguva murwere anogona kurarama upenyu huzere. Mutsipa iri zvishoma panguva uku kuwedzera urefu, izvo, kubva aesthetic nemaonero, runoonekwa zvakanaka chinotaurwa. Kana murwere kuteerera zano chiremba kuti apore, muviri anotanga "kupindura" matambudziko. Zvikurukuru dzakabudirira zvokurwara kuti nhengo dzomukati, pane kurwadziwa vakasimba mumutsipa. Past nokuda kuramba kukanganisa pamusoro etsinga nemidzi uye kunogona kupedza obezdvizhevaniyu maoko nemakumbo. Visceral mukana kusingarapiki pathological urongwa, uye angava kukonzera mangwanani rufu.

matanho

Zvinoita kudzivisa chirwere pamusoro Klippel - Feil? Photo varwere nechirwere ichi kumanikidzwa kufunga mubvunzo uyu. Dzakananga kudzivirira chirwere ichi hakuwaniki, sezvo nhaka. Mumhuri umo nyaya dzine anomalies akafanana kunge zvakanyorwa, zvakakodzera kuti magene mazano. Chaizvoizvo Its mamota pasi kundorondedzera vaviri kugadzirira basa vabereki, kuongorora mukana chirwere.

To muchidimbu

Klippel - Feil chirwere chinhu vachiberekwa anomaly rokumusoro nomuzongoza. Zvinonzi dzinoratidzwa vanamwari vatatu vatatu nechitarisiko zviratidzo zvinoverengeka radiological zvinhu. The chirwere ane yakanaka zvinokosha fungidziro, asi kuti kurapa zvachose hazviiti. Zvinoenderana Nenguva kuvhiya kupindira kugadzirisa chete achibvumira zvimwewo zvikanganiso zviripo. Kana zvakanga zvamboshandiswa kupupurirwa yepedyo chirwere chakadai, zvinokurudzirwa narwo zvizere nemagene kuongorora asati yokubereka mwana. Stay utano!