Lippen-Kiefer-Gaumenspalten - Hintergründe und Betreuungskonzept

Lippen-Kiefer- und/oder Gaumenspalten (LKG-Spalten) gehören zu den häufigsten Fehlbildungen. Viele Betroffene fielen früher sofort auf durch stark beeinträchtigte Sprachqualität und ein sehr konkaves Gesichtsprofil; ihre soziale Integration war dadurch oft beeinträchtigt. Sie wurden für speziell kariesanfällig gehalten und brauchten häufig grosse zahnärztlich-prothetische Versorgungen wegen Fehlens von Zähnen im Bereich der Kieferspalte. Die ab ca. 1970 Geborenen haben im Falle von LKG-Spalten sehr viel bessere Aussichten unter entwicklungsbezogenen Betreuungskonzepten welche nicht nur den möglichst raschen Verschluss der Spalte bezwecken, sondern einen optimalen Einbau der notwendigen Eingriffe in den Ablauf der spontanen Entwicklung des Kiefer-Gesichtskomplexes wie auch der Sprache anstrebe

Behandlung von Lippen-Kiefer-Gaumenspalten - Ein Fallbericht

Deutsch: In einem Fallbericht für die Angle Society of Europe wird ein Patient mit einer unilateralen Lippen-Kiefer-Gaumenspalte vorgestellt. Die umfassend dokumentierte Behandlung beginnt in den ersten Lebenstagen und erstreckt sich bis ins frühe Erwachsenenalter. Die Bedeutung der multidisziplinären Zusammenarbeit für die Geradlinigkeit und Effizienz der Behandlung wird klar zum Ausdruck gebracht. English: A case report for the Angle Society of Europe is being presented and the multidisciplinary treatment approach for cleft lip and palate patients will be discussed

Upper airway changes in Pierre Robin sequence from childhood to adulthood

OBJECTIVES: To investigate pharyngeal airway changes in patients with Pierre Robin sequence (PRS) longitudinally from childhood to adulthood. SETTING AND SAMPLE POPULATION: Cleft Lip and Palate Unit, Clinic of Orthodontics, University of Zurich. Twenty-four patients born between 1970 and 1990 with non-syndromic PRS. MATERIALS AND METHODS: Lateral cephalograms at age 5 (T1), 10 (T2), 15 (T3) and 20 (T4) years were available. Variables describing pharyngeal airway dimensions, soft palate morphology, tongue and hyoid position, skeletal morphology and head posture were assessed. RESULTS: A significant increase in nasopharyngeal depth was found over the entire observation period (T1 10.7 to T4 19.1 mm, p  0.05). CONCLUSIONS: Upper airway dimensions in children with PRS improve with time, except for the oropharyngeal airway. Despite large interindividual variation, the mean remained in the lower reaches of normality described in other studies. Thus, further research should investigate the prevalence of obstructive sleep apnoea in adults with PRS

Clinical variability of Stickler syndrome with a COL2A1 haploinsufficiency mutation: implications for genetic counselling

Clinical variability in Stickler syndrome is well known,6 14 15 but correlations with specific mutations are scarce. We report a novel COL2A1 gene mutation found in a patient with flat face, cleft palate, myopia, and hearing loss (Stickler syndrome) and unexpectedly also in her father and her paternal grandmother who were considered to be healthy. The patient is the first child of healthy, non-consanguineous Swiss parents. The pregnancy was uneventful and she was delivered at term by caesarean section because of breech position. Birth weight was 3890 g (90th centile), birth length 50 cm (50th centile), and head circumference 37 cm (>97th centile). Macrocephaly and facial dysmorphism were noted in the neonatal period, including a flat midface, deep set ears, exophthalmos, palpebral oedema with telangiectasia, micrognathia, and median clefting of the soft and part of the hard palate (fig 1). No other abnormalities were recognised at that time. At the age of 5 years, midface hypoplasia and micrognathia were still evident. In addition, she presented with mild bilateral hypoacusis, bilateral myopia (4 dioptres), slight webbing of the neck, minimal pectus carinatum, and flat feet (fig 1). Her growth was on the 90th centile. The clinical signs and symptoms as well as radiological findings of mild spondyloepiphyseal dysplasia suggested the diagnosis of Stickler syndrome

Facial attractiveness of patients with unilateral cleft lip and palate and of controls assessed by laypersons and professionals

OBJECTIVES:The aim of the study was to identify differences in the aesthetic evaluation of profile and frontal photographs of (1) patients treated for complete left-sided cleft lip and palate and (2) control patients by laypeople and professionals.MATERIALS, SUBJECTS, AND METHODS:Left-side profile and frontal photographs of 20 adult patients treated for complete left-sided cleft lip and palate (10 men, 10 women, mean age: 20.5 years) and of 10 control patients with a class I occlusion (five men, five women, mean age: 22.1 years) were included in the study. The post-treatment photographs were evaluated by 15 adult laypeople, 14 orthodontists, and 10 maxillofacial surgeons. Each photograph was judged on a modified visual analogue scale (VAS, 0-10; 0 'very unattractive' to 10 'very attractive'). A four-level mixed model was fitted in which the VAS score was the dependent variable; cases, profession, view, and rater were independent variables.RESULTS:Compared with laypersons, orthodontists gave higher VAS scores (+0.69, 95% confidence interval (CI) [0.53, 0.84]; P < 0.001), followed by surgeons (+0.21, 95% CI [0.03, 0.38], P = 0.02). Controls were given significantly higher scores than patients with clefts for profile and frontal photographs (+1.97, 95% CI [1.60; 2.35], P < 0.001). No significant difference was found between the scores for the frontal and lateral views (P = 0.46).CONCLUSIONS:All the different rater panels were less satisfied with the facial aesthetics of patients with clefts compared with that of control patients. Further research should evaluate whether these findings correlate with patients' self-perception and to what extent it affects the patients' psychosocial well-being

Effect of infant orthopedics on facial appearance of toddlers with complete unilateral cleft lip and palate (Dutchcleft).

Contains fulltext : 70434.pdf (publisher's version ) (Closed access)OBJECTIVE: To evaluate the effect of infant orthopedics (IO) on facial appearance of 54 patients with unilateral cleft lip and palate (UCLP), aged 4 and 6 years. DESIGN: Prospective two-arm randomized controlled clinical trial in three Cleft Palate Centers in the Netherlands (Dutchcleft-trial). INTERVENTIONS: Patients were divided randomly into two groups. Half of the patients (IO+) had a plate until surgical closure of the soft palate at the age of +/- 52 weeks; the other half (IO-) received no intervention. MAIN OUTCOME MEASURES: Facial appearance at 4 and 6 years of age assessed on full face photographs and photographs showing only nose and mouth. Ratings were performed on a VAS-scale by professionals and laymen. RESULTS: At 4 years of age the full face pictures of IO+ children were scored to be more attractive than those of IO- children. However, this difference had disappeared at 6 years of age. At the age of 6, only professionals saw a significant difference on nasolabial photographs between IO+ and IO-. Regression analysis showed a minor effect of occlusion, lip revision, or type of nose reconstruction on the esthetic results. CONCLUSIONS: IO had a positive effect on full facial appearance of UCLP children at the age of 4 years, but at the age of 6, only professionals saw a positive effect of IO on the nasolabial photographs. This is irrelevant for UCLP patients since they deal with laymen in their daily life