Indiana University-Purdue University Indianapolis (IUPUI) / The question of growth attenuation of the maxillary complex arises in connection with primary osteoplasty procedure. The deficiencies associated with the development of the jaws in unilateral complete cleft lip and palate (UCLP) children is an ongoing problem in terms of growth inhibition and orthodontic treatment. Retardation of growth can be attributed to intrinsic cleft factors and/or associated with the treatment of the cleft condition. In this study, the extent of such deficiency, if any, was investigated by assessing arch length, arch width, arch symmetry and inter-arch harmony. Seventeen orthodontic casts of UCLP children (mean age 7 years 11 months) were obtained. All were treated with a primary osteoplasty at James Whitcomb Riley Hospital for Children at Indianapolis, Indiana. The sample was compared to an age and sex matched non-cleft control group (n=38). None of the subjects had undergone orthodontic treatment beyond infant maxillary orthopedics. Anatomic landmarks were identified and digitized by means of optical electronics. The data were automatically fed into a computer which executed preprogrammed data manipulation algorithms. Significant (p<0.05) inhibition of growth has taken place in the maxillary arches of the UCLP group in both anteroposterior and transverse directions. lntercanine width (ICW) was reduced on average by 7.6 mm (23.6 percent) while the intermolar width (IMW) was shortened by 3.9 mm (7.8 percent). Sagittal growth was retarded by 5.5 mm (17.4 percent). A gradient of "normalization" originating at a point of surgical insult was observed anteroposteriorly in the transverse maxillary dimension. In the mandible, ICW was retarded by 2.0 mm (7.8 percent) while IMW and A-P growth vectors were not significantly affected. This investigation also revealed significant (p = 0.0001) differences in the size of the maxillary anterior palatal area (cleft mean = 83.5 mm2, non-cleft mean = 133.9 mm2). These findings lead to the conclusion that primary osteoplasty may contribute to maxillary growth attenuation with concomitant mandibular compensatory growth patterns.

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The mammalian palate develops early in embryogenesis by way of a carefully orchestrated series of temporally and spatially regulated signaling events. Molecular signaling pathways that have been proven to be vital to the process of palatogenesis include TGF-βs, BMPs, FGFs, EGF, and Wnts. The absence of connective tissue growth factor (CTGF/CCN2) has been shown previously to cause failure of proper palatogenesis, i.e. cleft palate. However, the details about the phenotype of this model of cleft palate were scarce. Additionally, CCN2 is known to interact with TGF-βs, BMPs, FGFs, EGF, and Wnts, though information on how these pathways were impacted in the developing palate lacking CCN2 were also not available. In Chapters 2 and 3, through our use of gross specimen and histological examination combined with cell and organ culture, we produced the most detailed characterization of the CCN2 knockout (KO) model of cleft palate with identification of negatively affected signaling pathways that lead to the clefting phenotype. Collection and examination of gross and histological sections revealed at 100% penetrance of cleft palate in which development is impaired around the phase of palatal shelf elevation. Organ culture also revealed that when artificially apposed, the CCN2 KO model system also suffers from a fusion deficit. Finally, utilizing cells isolated from the developing palates, we found a reduction in proliferation, adhesion, and spreading with an enhanced migratory ability. Addition of recombinant CCN2 was able to rescue cell spreading but not proliferation. CCN2 as an immobilized substrate did not rescue adhesive ability. Decreased adhesion and spreading in the KO cells are attributed to the inability of the KO cells to activate Rac1 and RhoA. Examination of gene expression differences by mRNA-sequencing and qRT-PCR revealed numerous gene expression alterations between the wild type (WT) and the KO palates, most notably FGF4 and EGFR. Addition of FGF4 or EGF to cell culture was unable to promote increased proliferation in the KO cells while producing a response in the WT cells. Examination of downstream signaling revealed highly amplified and prolonged ERK1/2 signaling in the FGF4 treated palate cells indicating that FGF signaling is significantly altered in the absence of CCN2. Treatment of the cells with EGF produced a response proportional to EGFR expression differences indicating that EGFR signaling is not impacted beyond the receptor protein levels. The link between EGFR protein levels and FGF mediated ERK1/2 activation is a protein called Spry2. We found greatly reduced Spry2 mRNA levels in the KO palates and upon FGF4 stimulation at 24 hours of exposure indicating that in the absence of CCN2, proper inhibition of FGF signaling and EGFR degradation is negatively altered. Collectively, the data demonstrate that CCN2 is vital to palatogenesis by impacting proliferation, shelf elevation, and shelf fusion through increased FGF signaling and reduced EGFR signaling resulting partially from reduced Spry2 activity. / Biomedical Sciences

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Thesis (MSocialWork)--Stellenbosch University, 2002. / ENGLISH ABSTRACT: In this study an explorative and a descriptive design has been implemented with the aim of obtaining knowledge, insight, and understanding of the psycho-social implications of a cleft lip and/or cleft palate for the individual child as well as the family. The motivation for this study was a result of the lack of knowledge on the psycho-social adjustments of an individual/child with a cleft lip and/or cleft palate and his/her family. The researcher became conscious of this lack during a full preliminary inquiry and this lack has been confirmed by the Department of Social Work as well as the Department of Maxillo-Facial-and Oral Surgery at Tygerberg Hospital. The aim of this study is therefore to create guidelines concerning social welfare interference in families after the birth of a baby with a cleft lip and/or cleft palate, in order to improve psycho-social functioning. The literature study included occurrence and incidence, embryological development, etiology of cleft lip and/or cleft palate as well as the psycho-social implications for the individual/child and the family. The medical aspects, namely the etiological and embryological development of cleft lip and/or cleft palate only serves as a background for the medical social worker that forms part of the interdisciplinary team of a cleft palate/lip clinic. This research focuses primarily on the psycho-social implications of a cleft lip and/or cleft palate on the individual/child as well on the family. The literature study also included the theoretical perspectives which determine the social worker's task and the system and strength perspective has been focused on. These perspectives have been implemented according to the development-aimed approach. The researcher decided to involve ten pre-school children with congenital deformity, between the ages of one month to six years as well as their mothers, who are from the service area of the Tygerberg Hospital in the research. The study was determined by a combination of qualitative as well as quantitative research methods, namely structured interviews as well as structured questionnaires. The results of this research confirmed the results of the literature study to a great extent, namely that individuals/children with a congenital deformity, as well as their families, are a highrisk group for psycho-social problems. These psycho-social problems indicate relationship problems in the family, cognitive problems for the individual/child as well as social adjustment problems for the individual/child and the family. Therefore the results gave an indication of what the content of intervention programmes for this target group should be. The recommendations focused on three areas, namely general recommendations concerning prenatal clinics where the cleft lip and/or cleft palate should be identified, general recommendations concerning guidelines for the social worker before intervention in the target group starts and recommendations with the aim of creating guidelines for social welfare intervention, in order to limit the psycho-social implications on the individual/child. The recommendations include further research in order to address society's ignorance concerning cleft lip and/or cleft palate. This research should focus on the development of social welfare programmes which, by primary prevention, aim at improved informing the society of this deformity. / AFRIKAANSE OPSOMMING: 'n Verkennende sowel as 'n beskrywende ontwerp is in hierdie studie geïmplementeer ten einde kennis, insig en begrip oor die psigo-sosiale implikasies van 'n gesplete lip en/of verhemelte vir individu/kind sowel as die gesin te verkry. Die motivering vir hierdie studie het na vore gekom weens die leemte rakende kennis oor die psigo-sosiale aanpassings van 'n individu/kind met 'n gesplete lip en/of verhemelte en sy/haar gesin. Die navorser het van hierdie leemte bewus geword tydens 'n volledige voorondersoek en hierdie leemte is deur die Departement Maatskaplike Werk sowel as die Departement Kaak- en Mondchirurgie te Tygerberg Hospitaal bevestig. Die doel van hierdie studie is dus om riglyne daar te stel ten opsigte van maatskaplike intervensie met gesinne ná die geboorte van 'n baba met 'n gesplete lip en/of verhemelte, ten einde psigo-sosiale funksionering te verbeter. Die literatuurstudie het die voorkoms en insidensie, embriologiese ontwikkeling, etiologie van gesplete lip en/of verhemelte sowel as die psigo-sosiale implikasies vir die individu/kind en die gesin ingesluit. Die mediese aspekte, naamlik die etiologiese en embriologiese ontwikkeling van gesplete lip en/of verhemelte dien slegs as agtergrond vir die geneeskundige maatskaplike werker wat deel vorm van die interdissiplinêre span van 'n gesplete verhemelte/lip kliniek. Hierdie navorsing fokus primêr op die psigo-sosiale implikasies van gesplete lip en/of verhemelte op die individu/kind sowel as op die gesin. Die literatuurstudie het ook die teoretiese perspektiewe wat die maatskaplike werker se taak rig, ingesluit en daar is op die sisteem- en sterkteperspektief gefokus. Hierdie perspektiewe is aan die hand van die ontwikkelingsgerigte benadering geïmplementeer. Daar is besluit om tien voorskoolse kinders met kongenitale deformiteit, dus tussen die ouderdomme van een maand tot ses jaar sowel as hul moeders, wat vanuit die Tygerberg Hospitaal se diensarea afkomstig is, by die ondersoek te betrek. Die ondersoek is deur 'n kombinasie van kwalitatiewe sowel as kwantitatiewe navorsingsmetodes, naamlik gestruktureerde onderhoude sowel as gestruktureerde vraelyste, gerig. Die resultate van hierdie ondersoek het tot 'n groot mate die bevindinge van die literatuurstudie bevestig naamlik dat individue/kinders met 'n kongenitale deformiteit, sowel as hul gesinne, 'n hoë risikogroep vir psigo-sosiale probleme is. Hierdie psigo-sosiale probleme dui byvoorbeeld op verhoudings-probleme in die gesin, kognitiewe probleme vir die individu/kind sowel as sosiale aanpassingsprobleme vir die individu/kind en die gesin aan. Die resultate het dus 'n aanduiding gegee van wat die inhoud van intervensieprogramme vir hierdie teikengroep behoort te wees. Die aanbevelings het op drie areas gefokus naamlik algemene aanbevelings ten opsigte van voorgeboorteklinieke waar gesplete lip en/of verhemelte geïdentifiseer moet word, algemene aanbevelings ten opsigte van riglyne vir die maatskaplike werker voordat intervensie met die teikengroep begin en algemene aanbevelings ten einde riglyne daar te stel vir maatskaplike intervensie, sodat die psigo-sosiale implikasies op die individu/kind beperk word. Die aanbevelings sluit in verdere navorsing ten einde die onkunde van die samelewing ten opsigte van gesplete lip en/of verhemelte aan te spreek. Hierdie navorsing moet fokus op die ontwikkeling van maatskaplikewerk-programme wat deur primêre voorkoming die samelewing beter aangaande die deformiteit inlig.

published_or_final_version / Dentistry / Master / Master of Dental Surgery

published_or_final_version / Speech and Hearing Sciences / Master / Master of Science in Audiology

published_or_final_version / Dentistry / Master / Master of Dental Surgery