Ukulandelana kukaPierre Robin (noma i-syndrome) yisimo sokubeletha esibangela ukuhlanganiswa kwezici ezilandelayo ezikhona ngesikhathi sokuzalwa:
- umhlathi omncane ongaphansi kokuthuthukiswa (lokhu kuthiwa i-micrognathia)
- i-cleft palate ngokungabikho okucacile komlomo we-cleft, ngokuvamile u-horsehoe noma u-U
- i-palate ephakeme kakhulu
- ulimi olubekwe kude kakhulu emphinjeni futhi lungavimbela ukuhamba emoyeni okwenza kube nzima ukuphefumula (glossoptosis)
- cishe ngamaphesenti angu-10-15 amacala ama-macroglossia (ulimi olungavamile), noma i- ankyloglossia (ulimi tie) kungenzeka
- amazinyo akhona ngesikhathi sokuzalwa kanye nezinkinga zezinyosi
- izifo ezitholakala njalo
- ama-anomalies angaphilile (amaphesenti angu-75 wamacala)
- ukulahlekelwa ukuzwa (amaphesenti angu-60 amacala - ngokuvamile aqhuba)
- i-atresia yangaphandle ye-canal (okungajwayelekile)
- ezinye iziphutha zendlebe ezibandakanya ukungavamile emathanjeni endlebe, ngaphakathi kwamanzi amachiza , nasemanzini ase-vestibular
- ukukhubazeka komzimba (okungavamile)
Lokhu okungajwayelekile ngesikhathi sokuzalwa ngokuvamile kuholela ezinkingeni zokukhuluma ezinganeni ezihambisana nohlelo lukaPeter Robin. Ezingamaphesenti angu-10-85 amacala nezinye ukubonakaliswa kwesistimu kungenzeka kuhlanganise:
- Izinkinga zamehlo (i-hypermetropia, i-myopia, i-astigmatism, i-corneal sclerosis, i-duct stenosis engavamile)
- Izinkinga zenhliziyo ziye zabhalwa ngamacala angu-5-58 amacala (ukukhononda kwenhliziyo, i-patent ductus arteriosis, i-patent foramen ovale, i-atrial septal defect, ne-blood pressure)
- Izinkinga ze-musculoskeletal ziqaphelwa njalo (amacala angu-70-80 amacala) futhi zingabandakanya syndactyly, polydactyly, clinodactyly, futhi oligodactyly, clubfeet, ama-hypress, amalysis, kneliosis, kyphosis, lordosis, nezinye izinto ezingavamile
- Ukungahleleki esimisweni sezinzwa zomphakathi kubonakala ngamacala angama-50% futhi kungafaka: ukulibaziseka kokuthuthukiswa, ukulibaziseka kwenkulumo, i-hypotonia, ne-hydrocephalus.
- Iziphambeko ze-Genitourinary zingavamile kodwa zingabandakanya izivivinyo ezingenasidingo, i-hydronephrosis noma i-hydrocele.
Isimo sePeter Robin ukulandelana cishe ku-1 ku-8500 ukubeletha, okuthinta abesilisa nabesifazane ngokulinganayo. Ukulandelana kukaPierre Robin kungahle kwenzeke futhi kuqobo kodwa kuhlotshaniswa nezinye izimo zofuzo ezibandakanya: I-Stickler syndrome, i-CHARGE syndrome, i-Shprintzen syndrome, i-Mobius syndrome, i-trisomy 18 syndrome, i-trisomy 11q syndrome, isuse i-4q syndrome, nabanye.
Kunemibono emithathu mayelana nokuthi yini ebangela ukulandelana kukaPeter Robin. Okokuqala ukuthi i-mandibular hypoplasia kwenzeka ngesikhathi seviki le-7-11 lokukhulelwa. Lokhu kubangela ukuthi ulimi luhlale lukhuni emlonyeni womlomo oluvimbela ukuvalwa kwamashalofu ase-palatal futhi kubangele i-U-cleped palate. Inani elincane le-amniotic fluid lingase libe yisici.
Ingqikithi yesibili yukuthi kukhona ukubambezeleka ekuthuthukiseni kwezinzwa zezinhlanzi zezilimi, izinsika ze-pharyngeal, ne-palate ehambisana nokulibaziseka ekuqhubeni kwe-hypoglossal nerve conduction. Le ncazelo ichaza ukuthi kungani izimpawu eziningi zixazulula cishe iminyaka engu-6 ubudala.
Inkolelo yesithathu yukuthi inkinga enkulu kwenzeka ngesikhathi sokuthuthukiswa okuholela ekudyseniurulation of the rhombencephalus (i-hindbrain - ingxenye yengqondo equkethe brainstem ne-cerebellum).
Ayikho ikhambi lokulandelana kukaPeter Robin. Ukuphathwa kwalesi simo kuhilela ukuphatha izimpawu zomuntu ngamunye. Ezikhathini eziningi umhlathi ophansi ukhula ngokushesha phakathi nonyaka wokuqala wokuphila futhi ngokuvamile uvela evamile nge-enkulisa. Ukukhula ngokwemvelo kuvame ukuphulukisa izinkinga zokuphefumula (ezindiza emoyeni) ezingase zibe khona. Ngesinye isikhathi i-airway yokufakelwa (okufana ne-nasophargyngeal noma ngomlomo ohamba emoyeni) idinga ukusetshenziselwa isikhathi esithile. I-flaft palate kufanele ilungiswe ngokuhlinzwa njengoba ingabangela izinkinga ngokudla noma ukuphefumula. Izingane eziningi ezinokulandelana kukaPaul Robin zizodinga ukwelashwa kokukhuluma.
Ubukhulu bokulandelana kukaPaul Robin buhluka kakhulu phakathi kwabantu njengoba abanye abantu bengase babe nezimpawu ezimbalwa ezihambisana nalesi simo kanti abanye bangase babe nezimpawu eziningi.
Izimpawu ze-cardiovascular noma central central zingase zibe nzima nakakhulu ukuphatha kunezinye izimo ezingavamile ezihlobene nohlelo lukaPeter Robin. Ucwaningo luye lwabonisa ukuthi ukulandelana kukaPierre Robin okuhlukile, (lapho isimo senziwa ngaphandle kwesinye isifo esihambisanayo), ngokuvamile asikhulise ingozi yokufa, ikakhulukazi lapho izinkinga zesistimu zenhliziyo noma ezisemkhatsini zingekho khona.
> Imithombo:
> I-Cleft Palate Foundation. Ukulandelana kukaPeter Robin. http://www.cleftline.org/parents-individuals/publications/pierre-robin-sequence/
> Medline Plus. Ukulandelana kukaPierre Robin. https://www.nlm.nih.gov/medlineplus/article/001607.htm
> I-Medscape. Pierre Robin Syndrome. http://emedicine.medscape.com/article/844143-overview#a4