Klippel - Feil syndrome: chithunzi, chithandizo, zotsatira zake zikhoza kukhala? Sprengel syndrome - yosalepera - Klippel: kokoka dziwe losambira

Awiri French katswiriyu Andre Feil ndi Moris Klippel mu 1912, anafotokoza mwatsatanetsatane deformities kobadwa nako mu khomo lachiberekero-thoracic msana ana. Onse anabadwa mu makolo a wachibale wina. Mu Middle Ages mkazi malamulo akhoza kukhala achibale. Choncho, kuchuluka kwa anomaly zimenezi si zodabwitsa. kudwala pambuyo unatchedwa sayansi. Klippel - Feil syndrome si matenda mokwanira. Ndi matenda obadwa nawo, nthawi zambiri kumayambitsa pathologies ena msana.

Features matendawa

Pansi syndrome Klippel - Feil amatanthauza matenda obadwa nawo a msana khomo lachiberekero, wopangidwa mwa synostosis ndi kuchepetsa chiwerengero cha vertebrae. Iwo amakhala ndi pafupipafupi mkulu wa mawonetseredwe - chitsanzo chimodzi pa kubadwa 120 000. Mbali ankaikira matenda akuti ndi kuchepetsa Amowabu m'litali mwa khosi. Nthawi zambiri, limodzi ndi kuvutika ena a dongosolo minofu ndi mafupa ndi kuvutika kobadwa nako ziwalo mkati. Mu matenda a nthendayo anapezeka akatswiri angapo :. ubongo, zamafupa zinthuzo, etc. chithandizo ndiwofatsa zikuphatikizapo mankhwala thupi, physiotherapy ndi kutikita. Nthawi kwambiri makamaka opaleshoni - opaleshoni tservikalizatsii.

Zimayambitsa waukulu malformations

Klippel - Feil syndrome amatanthauza matenda osiyana zotsimikizika. kusintha Pathological mu thupilo adzayamba kulankhula mu masabata choyamba pambuyo pathupi. Pakati pa zifukwa madokotala syndrome anati matenda kakulidwe a msana ndi kugawanika, makamaka mlingo verhnesheykovom. Anapanga synostosis thoracic ndi vertebrae khomo lachiberekero, kuchepetsa awo, matupi nonunion ndi arches kulola ife kudziwa chithunzi matenda a matenda. Gulu chiopsezo zikuphatikizapo ana ndi chibadwa akumane, kumene:

1. Chirema kwa maselo a DNA lapansi. Mu mwana wodwala ndi kuphwanya mapangidwe a zosiyanasiyana kukula koyenera kuti akule bwino za mafupa a. Izi amatipeza zikupezeka mu chitukuko cha m'madipatimenti thoracic ndi khomo lachiberekero vertebrae.
2. Autosomal chitsanzo lalikulu la cholowa. Ngati kholo limodzi ali matenda, Mwina kubereka mwana ndi kuvutika ndi 50-100%.
3. Autosomal recessive cholowa. Pankhaniyi, Mwina ndi mwana ndi kuvutika kwa 0-50%.

Kupewa akumane makolo cholowa ayenera kuonana ndi zachibadwa pa siteji ya kulera mwanayo.

Zizindikiro Chitsanzo cha matendawa

matenda wofanana ndi wa ku tingachipeze powerenga zizindikiro: kwambiri lalifupi khosi, kusintha hairline, yochepa kuyenda pa mutu. matendawo nthawi pamodzi pathologies ena. Mu 30% odwala anaulula kupindika msana, torticollis mawonekedwe okhwima, masamba mkulu ubwenzi - matenda Sprengel. Nthawi zina za kuvutika anati kwa miyendo chapamwamba, mapazi mapindikidwe, mano, nkhope asymmetry, hyperopia. 25% odwala matenda ndi ugonthi kobadwa nako.

Klippel syndrome - yosalepera - Sprengel - sikuti anasonyeza zodzikongoletsera chilema. Mwina mulinso zizindikiro ubongo. Kusintha CNS kumawonetsera maganizo retardation, hydrocephalus, khunyu. Kuyambira ali mwana wodwalayo minofu mavuto malekezero, komanso synkinesis. Pa zaka achikulire, wonse matenda chithunzithunzi wodzilemekeza ndi kusintha yachiwiri ya msana.

Kupenda Medical

Yotsimikiza matenda akhoza zochokera ananena wa ku matendawa deta thupi kuona ndi mayeso malangizo. A udindo wapadera mu matenda anapatsidwa kuphunzira mbiri banja la wodwalayo. Ikani Klippel - syndrome Feil ndi kufotokoza mwatsatanetsatane wotsagana anomalies mwina chifukwa cha ntchito yolongosoka akatswiri. Monga ulamuliro, izo Madokotala aubongo, geneticists, orthopedists, cardiologists ndi pulmonologists.

Kuwunika chikhalidwe cha kusintha anatumizidwa vertebral radiography. Ankachitira phunzirolo awiri ziyerekezo, amene nkhani ambiri amangoona ndi lateral. Chifukwa cha udindo uncharacteristic cha mthunzi wa chigaza cha mutu limagwirizana ndi chithunzi cha ndime msana, zikhale zovuta kudziwa zinthu. Komanso tikulimbikitsidwa kuti kujambula zithunzi pa pazipita flexion / kutambasuka kwa khosi. Zimenezi zimathandiza kudziwa Kusakhazikika zotheka ununited vertebrae ndi kutsimikizira syndrome Klippel - Feil. X-ray wa khomo lachiberekero-thoracic msana Zimathandizanso kudziwa:

• opunduka vertebrae;
• ndichepe mu chiwerengero chawo;
• maphatikizidwe wa matupi vertebral;
• nthenda masamba ubwenzi;
• kupindika msana.

Pambuyo pa kutsimikizira matenda a odwala Komanso yoikidwiratu ultrasound a ziwalo kudziwa anomalies mu dongosolo lawo. Pamaso pa kuvutika ubongo kungafune ziwiya Doppler ultrasound, angiography, akugwirira ndi Kujambula a msana khomo lachiberekero. Ndi mokakamizidwa kukaonana ndi dokotala-zachibadwa. Malinga ndi zotsatira zake katswiri adzakhala kudziwa akafuna cholowa, ndi kuwunika kuopsa kwa mibadwo ya m'tsogolo.

Mawonekedwe zoyamba za matendawa

Klippel syndrome - Feil ndi matenda osowa kwambiri. Malinga ndi kafukufuku, zizindikiro lililonse amapezeka mu mwana mmodzi pa 120 zikwi. The anomaly limapezeka mu mtima mwa munthu wa mitundu itatu:

• Kuchepetsa chiwerengero cha zigawo mu msana khomo lachiberekero, amene pang'onopang'ono angakondwere nazo zosakaniza ndi zowoneka kufupikitsa khosi. Izi mawonekedwe a kudwala amachititsa mavuto pamene kusuntha mutu.
• Synostosis khomo lachiberekero msana maphatikizidwe chapansipansi ndi fupa occipital. Wodwala mutu wake wopanda ululu. Khomo lachiberekero vertebrae ndi khosi ndi chimodzi.
• Mawonekedwe lachitatu amasonyeza matenda monga woyamba awiri.

A zosinthika makamaka za matendawa zingadziŵike dokotala kokha pamaziko a zotsatira kafukufuku.

zoyamba za chithandizo

Mwatsoka, mankhwala amakono sangathe kupereka njira chokwanira cha mmene wokonzeka kuthana ndi vuto la Klippel - Feil. Chithandizo kumaphatikizapo kuteteza chitukuko cha deformities sekondale. Unatenga maziko njira ndiwofatsa, monga mankhwala thupi ndi kutikita. mankhwala wapatsidwa kukachitika kuti ndondomeko matenda limodzi ndi ululu komanso nkhawa psinjika a mizu mitsempha. Monga ulamuliro, ntchito analgesics, odana ndi yotupa ndi mankhwala si steroidal. Yaitali psinjika a mizu mitsempha opaleshoni. Cholinga cha opaleshoni - kuthetsa ululu pang'ono vuto kunja.

mankhwala thupi ndi ntchito mu dziwe

chithandizo ndiwofatsa a matendawa ndi monga thanzi mankhwala Inde, achire kutikita a msana khomo lachiberekero ndi otchedwa khosi m'deralo. Zochita amasankhidwa payekha, zochokera chithunzi matenda a wodwalayo. Nthawi yoyamba, iwo ayenera idzachitika kuyang'aniridwa ndi katswiri, mukhoza pite pa zinthu m'banja. Odwala ndi matenda a "Sprengel syndrome - yosalepera - Klippel" dziwe Zochita kuthandiza kupewa zovuta njira ya matenda. Monga ulamuliro, achinyamata amene akudwala, madokotala amalangiza backstroke. Zochita More zovuta komanso amasankhidwa payekha. ayamikira Universal zokhudza olimbitsa kusambira kulibe.

chithandizo opaleshoni

ululu lekeza chifukwa cha psinjika mitsempha muzu ndi umboni chachikulu opaleshoni. Odwala ndi matenda a "syndrome Klippel - Feil" madokotala ananena zawo worsens ndi zaka. Choncho, ntchito ulipo yomweyo pambuyo chitsimikiziro cha kudwala. Kuonjezera kuyenda kwa khosi ntchito tservikalizatsiya Bonola. Pa ndondomeko, dokotala amachotsa pamwamba nthiti zinayi komanso periosteum kuti kuchepetsa kuthamanga ziwalo mkati. opaleshoni imagwiridwa mu magawo angapo. Poyamba kuchotsedwa nthiti mbali imodzi, pambuyo ayambe thupi - mzake.

Konzanso nthawi kumafuna nthawi ndi khama. Kwa miyezi ingapo, wodwala ayenera kukhala pa mpumulo. Komabe, kusiya ntchito Sikuti. Panopa ndi Njira kusintha zopindika zakunja pakutchulidwa amene akapsa vuto Klippel - Feil. Kodi zotsatira zake thanzi, ngati ife kusiya mankhwala, pansipa.

Zovuta ndi madokotala ananena zawo wodwalayo

Pambuyo opaleshoni konzanso nthawi wodwala kukhala moyo wathunthu. khosi ndi pang'ono pa izi kuwonjezeka nthawi m'litali, amene ku mfundo zokongoletsa maganizo, ankaona mbali yabwino. Ngati wodwala n'kunyalanyaza malangizo a dokotala kuti apeze, thupi limayamba kuti "kuyankha" zosokonezeka. Kwenikweni anayamba kudwala kwa ziwalo, pali ululu amphamvu khosi. M'mbuyomu chifukwa siudzalandilidwa ngathi lamulo zonse mizu mitsempha ndi kungachititse kuti amalize obezdvizhevaniyu miyendo. chiopsezo choipidwa njira sizingasinthe pathological, ndipo mwina chifukwa cha imfa oyambirira.

zodzitetezera

Kodi n'zotheka kuteteza matenda a Klippel - Feil? Photo odwala ndi matenda izi amakakamizidwa ndikuganiza za funsolo. Specific kupewa matenda kulibe, chifukwa cholowa. M'mabanja amene milandu ndi anomalies ofanana analemba, m'pofunika uphungu chibadwa. Kwenikweni ake inali kafukufuku banjali kukonzekera udindo wa makolo, kuti aone ngozi ya matenda.

kuti adzimvera

Klippel - Feil syndrome ndi anomaly kobadwa nako a msana chapamwamba. Iwo amakhala ndi wa ku zizindikiro kunja ndi zingapo zochititsa radiological. Matendawa ali ndi madokotala ananena zawo zabwino kwambiri, koma kuchiza kwathunthu zosatheka. Panthawi yake alowererepo opaleshoni kukonza yekha amalola zolakwika kunja zilipo. Ngati inu M'mbuyomo zikutsimikiziridwa ndi wachibale matenda zotero, m'pofunika kuti mwanjira majini mosamala zonse pamaso pathupi mwana. Khalani wathanzi!