Ukulandelana kukaPierre Robin

Ukulandelana kukaPierre Robin (noma i-syndrome) yisimo lapho usana lunomhlathi omncane ophansi kunokujwayelekile, ulimi olubuyela emuva emphinjeni, nobunzima bokuphefumula. Sikhona ngesikhathi sokuzalwa.

Izimbangela ngqo zokulandelana kukaPierre Robin azaziwa. Kungaba yingxenye yama-syndromes amaningi wezakhi zofuzo.

Umhlathi ongezansi ukhula kancane ngaphambi kokuzalwa, kodwa ungakhula ngokushesha eminyakeni embalwa yokuqala yokuphila.

Izimpawu zalesi simo zifaka:

• Ulwanga oluhlanzekile
• Ulwanga oluphakeme kakhulu
• Umhlathi lowo mncane kakhulu ngesilevu esincane
• Umhlathi osekude emuva emqaleni
• Ukutheleleka okuphindaphindiwe kwezindlebe
• Ukuvuleka okuncane ophahleni lomlomo, okungadala ukuthi ukuminyana noma uketshezi kuphume ngekhala
• Amazinyo avela lapho ingane izalwa
• Ulimi olukhulu uma luqhathaniswa nomhlathi

Umhlinzeki wezokunakekelwa kwempilo uvame ukuxilonga lesi simo ngesikhathi sokuhlolwa komzimba. Ukubonisana nodokotela wezofuzo kungakhipha ezinye izinkinga ezixhumene nalesi sifo.

Khuluma nodokotela wengane yakho ngezindawo zokulala eziphephile. Ezinye izinsana ezilandelana ngokulandelana kukaPierre-Robin zidinga ukulala ngezisu zazo esikhundleni somhlane wazo ukuvimbela ulimi lwazo ukuthi lungaphinde lubuyele emoyeni wazo.

Ezimweni ezilingene, ingane izodinga ukuthi kufakwe ishubhu emakhaleni nasendleleni yokuvikela umoya ukuvimba ukuvalwa komoya. Ezimweni ezimbi kakhulu, kudingeka ukuhlinzwa ukuze kuvinjelwe ukuvimba komgwaqo ongaphezulu. Ezinye izingane zidinga ukuhlinzwa ukuze zenze imbobo emoyeni wazo noma ukuhambisa umhlathi wazo phambili.

Ukondla kufanele kwenziwe ngokucophelela okukhulu ukugwema ukuminyaniswa nokuphefumula iziphuzo ezindleleni zomoya. Ingane ingadinga ukondliwa ngepayipi ukuvimbela ukuminyana.

Izinsizakusebenza ezilandelayo zinganikeza imininingwane eminingi ngokulandelana kukaPierre Robin:

• Ucwaningo Lwezinkinga Zokuzalwa Lwezingane - www.birthdefects.org/pierre-robin-syndrome
• ICleft Palate Foundation - www.cleftline.org
• Inhlangano Kazwelonke Yezinkinga Ezingajwayelekile --rarediseases.org/rare-diseases/pierre-robin-sequence

Izinkinga zokuminyaniswa nokondla zingazihambela zodwa eminyakeni embalwa yokuqala njengoba umhlathi ongezansi ukhula ube usayizi ojwayelekile. Kukhona ingozi enkulu yezinkinga uma imigwaqo yomoya yengane ingagcinwa ukuthi ivinjwe.

Lezi zinkinga zingenzeka:

• Ubunzima bokuphefumula, ikakhulukazi lapho ingane ilele
• Iziqephu zokuchofoza
• Ukwehluleka kwenhliziyo yokubopha
• Ukufa
• Ukondla ubunzima
• I-oxygen ephansi yegazi nokonakala kobuchopho (ngenxa yokuphefumula kanzima)
• Uhlobo lomfutho wegazi ophakeme obizwa nge-pulmonary hypertension

Izingane ezizalwa zinalesi sifo zivame ukutholakala lapho zizalwa.

Shayela umhlinzeki wakho uma ingane yakho ineziqephu zokuklinya noma izinkinga zokuphefumula. Ukuvaleka kwemigwaqo yomoya kungadala umsindo ophakeme lapho ingane iphefumulela ngaphakathi. Kungaphinde kuholele ekubeni mhlophe kwesikhumba (i-cyanosis).

Fonela futhi uma ingane yakho inezinye izinkinga zokuphefumula.

Akukho ukuvimbela okwaziwayo. Ukwelashwa kunganciphisa izinkinga zokuphefumula nokuklinya.

Isifo sikaPierre Robin; UPierre Robin complex; UPierre Robin anomaly

• Izinsana eziqinile nezithambile

UDhar V. Syndromes ngokubonakaliswa komlomo. Ku: Kliegman RM, Stanton BF, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 337.

UPurnell CA, uGosain AK. Ukulandelana kukaPierre Robin. Ku: Rodriguez ED, Losee JE, Neligan PC, eds. Ukuhlinzwa Kweplastiki: Umqulu Wesithathu: Ukuhlinzwa Kwe-Craniofacial, Head and Neck kanye nePediatric Plastic Surgery. Umhla wesi-4. IPhiladelphia, PA: Elsevier; 2018: isahluko 36.