The Cleft Palate Craniofacial Journal

In developing countries cleft lip and palate (CLP) patients arrive late, and there is a risk of drop out for functionally important palatoplasty after lip repair. Patients may be underweight, anemic, and prone to recurrent infections.

To repair cleft palate at an appropriate time and secondly to avoid the drop out after the first surgery.

A new surgical protocol has been designed for patients with CLP in whom the cleft palate is first repaired at 6 to 9 months of age or whenever the patient presents at the clinic. The cleft lip is repaired 3 to 6 months after the first surgery.

When cleft lip repair was performed before palate repair (conventional protocol group) in 89 patients with CLP, the median interval between first and second surgery was 290 days. However, when the modified protocol was followed in 330 patients over 13 years, the median interval was 193 days (p < .0002). The reduction in the interval is statistically significant. In eight patients cleft palate fistula encountered after palatoplasty was repaired during cleft lip repair thus avoiding a third surgery.

The compliance for two surgeries in CLP has improved. Apart from achieving the main aim of the new protocol, a number of advantages were noticed. The palate repair was easier in the presence of an unrepaired cleft lip. The anterior palate repair was more dependable with reduced incidence of anterior palatal or alveolar fistula.

Authors encounter a large percentage of wide cleft palates while operating in a Third World situation. They define the terms wide clefts and unrepairable clefts in terms of measurement. They describe their technique, which they developed to deal with wide clefts. They describe some previously unreported modifications. They also report the velar lengthening obtained.

A total of 77 cases of primary cleft palate repaired with this technique by a single author, during the period May 2006 to February 2009, were selected for the study. These were divided into two groups on the basis of measurements. Group B consisted of all clefts deemed wide or unrepairable. Group A consisted of all other cleft palates. Difference in fistula rate was studied. Velar lengthening was measured in all patients.

Two fistulae occurred in Group B. The overall fistula rate for the series was 2.6%. The series consisted of 44% wide clefts, which included one case of unrepairable cleft. Lengthening in the velum ranged from 20% to 155%. Statistically significant correlations were found between narrow clefts and age group 0 to 1 year (p = .0094) and Veau Group 4 with wide clefts (p = .0194).

The Furlow technique as originally described has shortcomings. The authors describe their technique of incorporating the Furlow repair, which enables them to use it as a primary palatoplasty, in a scenario consisting of a large percentage of wide clefts in an older age group, thereby minimizing the fistula rate while increasing palatal length.

To evaluate nasal morphology, symmetry, and nostril shape in patients with unilateral cleft lip and palate following mixed-dentition alveolar bone grafting.

Prospective stereophotogrammetric study.

Hospital-based.

Thirty-nine patients with a history of repaired unilateral cleft lip and palate who received an iliac crest alveolar bone graft were recruited prospectively to participate in the study. Each patient served as his/her own control.

Partial facial impressions of all patients were acquired before and a minimum of 6 months after the alveolar bone grafting procedure. Image acquisition and analysis of the casts constructed from these models were carried out using three-dimensional stereophotogrammetry.

Surface-based registrations and linear measurements were performed to assess nasal morphology and nostril shape. A modified Procrustes technique was used to determine the change in nasal symmetry. A two-tailed, paired t test and an analysis of covariance were used to assess statistical significance.

Significant side-to-side asymmetry exists in the nasal region of patients with unilateral cleft lip and palate, both before and after alveolar bone grafting. No significant changes were observed between pre–alveolar bone graft and post–alveolar bone graft images based on linear measurements, asymmetry scores, and registrations. Gender and surgeon were not significant factors.

Under the conditions of this study, mixed-dentition alveolar bone grafting appears to have no significant long-term effect on nasal morphology, symmetry, or nostril shape.

The purposes of this study were (1) to develop imaging methods and objective numeric parameters to describe nose morphology, and (2) to correlate those parameters with nasal esthetics for patients with clefts.

A total of 28 patients with repaired complete unilateral cleft lip and palate (CUCLP) and 20 age- and gender-matched individuals without clefts were identified. A panel of orthodontists rated and ranked nasal esthetics from nose casts for the cleft group. Best and worst esthetic cleft groups were established from the cast assessments. Three-dimensional surface coordinates of the casts were digitally mapped with an electromagnetic tracking device. Digitized nasal images were oriented, voxelated, sliced, and mathematically curve-fitted. Maximum difference, percent area difference, and maximum and minimum derivative differences between cleft and noncleft and between right and left nose sides were calculated. Differences in parameters between groups were assessed with the use of analysis of variance (ANOVA) and t tests, and correlations with esthetics were assessed with the Spearman rank correlation test.

Differences were seen between cleft and noncleft and best and worst esthetic groups for all four parameters (p < .05). The best esthetic cleft group had (1) lower percent area difference (p < .0001), (2) lower maximum difference (p < .001), and (3) smaller differences in slope of the nose in the coronal plane (p < .0001) than the worst esthetic cleft group.

Maximum difference and maximum derivative difference and, to a lesser degree, percent area difference can be used to identify differences between cleft and noncleft nasal morphology and to assess levels of nasal esthetics for patients with CUCLP.

To present a new orthopedic method for treatment of infants with Pierre Robin sequence (PRS) and upper airway obstruction (UAO) as an alternative to other established nonsurgical and surgical techniques such as positioning, nasopharyngeal or endotracheal intubation, tongue-lip adhesion, extension, distraction, or tracheostomy.

Review of the literature and presentation of novel orthopedic appliances.

Department of Orthodontics, Dental Clinic, Medical Faculty of the University of Wuerzburg, Germany, Department and Clinic of Pediatrics, Medical Faculty of the University of Wuerzburg, Germany, 2005 to 2008.

Seven patients with significant respiratory and feeding difficulties between 0 and 6 months of age. Both patients with nonsyndromic PRS and patients with syndromic PRS were included.

The type of respiratory tract obstruction was defined by nasopharyngoscopy. Patients with type 1 obstruction received a plate with an epiglottic spur; whereas, patients with obstruction type 2, 3, or 4 received a plate with a pharyngeal tube.

All patients were successfully treated with orthopedic appliances alone. Under plate therapy they showed good oxygen saturation and could consequently be better nourished orally.

The presented novel method is a noninvasive technique in treatment of infants with UAO.

The purpose of this study was (1) to determine benefit of the Baha Softband coupled to the Softband for infants and children with bilateral conductive hearing loss; and (2) to verify audibility of the speech spectrum for octave frequencies 500 through 4000 Hz.

The research design for this retrospective chart study is pretest-posttest repeated measures.

The study was conducted in the Department of Audiology and Speech Pathology, Arkansas Children's Hospital.

Twenty-five children aged 6 months to 18 years with craniofacial disorders and bilateral conductive hearing loss participated in the study. Participants were consistent, full-time unilateral Baha users with the Baha Compact bone-conduction amplifier coupled to the head via the Softband.

The intervention was the Baha device coupled to the head via the Softband as a prerequisite to surgical implantation.

The primary study outcome measures used aided and unaided soundfield audiometric thresholds to calculate functional gain. Audibility of the speech spectrum was verified by comparison with target aided thresholds.

Results revealed an improvement in soundfield thresholds with Baha amplification for the four octave frequencies. Means, standard deviations, and confidence intervals for aided and unaided thresholds are reported. Percentages of thresholds meeting target levels were significant at all frequencies, exceeding the 80% criterion.

Benefit of the Baha in providing audibility of the speech spectrum for infants and children with bilateral congenital conductive hearing loss has been demonstrated, offering important and timely data supporting third-party reimbursement.

To explore the factors that predict psychosocial adjustment in young people with a cleft and their parents.

The study used a cross-sectional postal questionnaire design involving young people aged between 11 and 16 and their parents from two cleft services. Data are presented for 91 adolescents and their mothers.

Participants completed measures of psychological adjustment, coping, social experiences, satisfaction with appearance, stressful life events, cleft-related factors, and demographic information.

Psychosocial adjustment in adolescents was predicted by their social experiences and maternal well-being. Satisfaction with appearance, perceived speech problems, and the use of avoidant coping strategies were also important factors relating to their adjustment. For mothers, adjustment was predicted by use of coping strategies such as self-blame, venting, and acceptance, in addition to perceived problems with their child's hearing and the number of stressful life events experienced.

The findings are discussed in relation to the concepts of adjustment, coping, satisfaction with appearance, and maternal mental health. Directions for future research are outlined, and a number of opportunities and challenges for cleft services regarding the provision of timely interventions for this age group and their families are discussed.

To determine whether raters' first impressions of youth with craniofacial differences are modifiable.

Observational study of the association between first impressions and social skills as related to youth aged 11 to 18 years with craniofacial differences.

University research offices and clinics.

Youth aged 11 to 18 years with (n = 29) and without (n = 31) craniofacial differences; adults (n = 40), dental/medical students (n = 46), and education students (n = 29), all without craniofacial differences. Participants were recruited from medical clinics and through community advertising at all three study sites.

The First Impressions Rating Scale.

After viewing 1-minute portrayals of positive social skills by actors with craniofacial differences, raters' perceptions moved significantly in the positive direction for all 26 attributes on the First Impressions Rating Scale; whereas, after viewing negative social skills, ratings moved significantly in the negative direction for 25 of 26 First Impressions Rating Scale attributes.

It appears that first impressions others have of youth with craniofacial differences are significantly affected by how these youth present themselves in social situations, suggesting that positive social skills may help reduce the amount of stigma that youth with craniofacial differences encounter.

The aim of this work was to investigate in more detail the dental clinical features that could serve to define subphenotypes of oral clefts.

Dental records of oral cleft subjects from a group of 164 cases were examined, and 157 were included in this study. In addition, 65 families with two or more siblings born with clefts and 30 control families were evaluated to determine whether dental phenotypes were sporadic. Type of oral cleft and dental phenotypes (tooth agenesis, supernumerary teeth, taurodontism, dental transposition, and microdontia) outside the cleft area were investigated. Association of dental anomalies with preferential subtypes of cleft (subphenotype) was assessed.

A total of 74 subjects presented at least one developmental dental anomaly. Tooth agenesis was the most common dental anomaly (28.6%), followed by taurodontism (15.2%). Supernumerary teeth were associated with cleft palate only (p = .05). The absence of maxillary left lateral incisors was significantly associated with unilateral right cleft lip (p = .02). Bilateral clefts were strongly associated with bilateral dental anomalies (p < 0.001). In the cleft lip and palate group, tooth agenesis was associated with dental transposition (p = .03) and with supernumerary teeth (p = .009). Subjects with oral clefts have a higher risk of tooth agenesis (odds ratio = 3.33; 95% confidence interval, 1.18 to 10.13) and taurodontism (odds ratio = 3.95; 95% confidence interval, 2.28 to 6.82). Tooth agenesis, microdontic upper lateral incisors, and supernumerary teeth were most commonly found in unaffected siblings and parents of children born with clefts in comparison with families with no family history of clefts (p = .01).

The preferential associations between specific cleft types with dental phenotypes suggest dental anomalies can be used as clinical markers to define the subphenotype isolated cleft lip and palate.

The aim of this study is to identify gene targets of TFII-I transcription factors involved in craniofacial development.

Recent findings in individuals with Williams-Beuren syndrome who show facial dysmorphism and cognitive defects have pointed to TFII-I genes (GTF2I and GTF2IRD1) as the prime candidates responsible for these clinical features. However, TFII-I proteins are multifunctional transcriptional factors regulating a number of genes during development, and how their haploinsufficiency leads to the Williams-Beuren syndrome phenotype is currently unknown.

Here we report the identification of three genes with a well-established relevance to craniofacial development as direct TFII-I targets. These genes, craniofacial development protein 1 (Cfdp1), Sec23 homolog A (Sec23a), and nuclear receptor binding SET domain protein 1 (Nsd1), contain consensus TFII-I binding sites in their proximal promoters; the chromatin immunoprecipitation analysis showed that TFII-I transcription factors are recruited to these sites in vivo.

The results suggest that transcriptional regulation of these genes by TFII-I proteins could provide a possible genotype-phenotype link in Williams-Beuren syndrome.