The Cleft Palate Craniofacial Journal

The aim of the present study was to compare the morphometrics of the craniofacial and alveolar arch structures of anencephalic fetuses with those of normal human fetuses without anencephaly or any other anomaly.

Original article.

Standard lateral and posteroanterior cephalometric radiographs of 23 anencephalic and 33 normal human fetuses aged between 21 and 40 weeks of gestation were compared. These fetuses were divided into three periods based on gestational age, and vertical, sagittal, transverse, and angular measurements were taken in the cranial base and facial regions. Moreover, intraoral measurements from the maxillary and mandibular arches were also made to determine the width and depth.

N-S and S-Ba lengths and the N-S-Ba angle, measured on lateral cephalometric radiographs, were statistically smaller in anencephalic fetuses (p < .05). The S-N-ANS, S-N-Pg, and ANS-N-Pg angles and the Go-Me length were significantly greater in anencephalic fetuses (p < .05). Maxillary length, ramus length, and anterior facial heights were similar in both groups. On posteroanterior radiographs, all parameters except bigonial distance were found to be smaller in the anencephalic fetuses (p < .05). Maxillary alveolar width was small among anencephalic fetuses in all three periods; whereas, the mandibular alveolar width was narrow only in the third period.

Cranial and facial structures were affected morphometrically to various degrees in anencephalic cases. The most significant alterations in anencephalic cases were related to measurements in the transverse direction. This indicated that cephalic tissue influenced not only the cranial base but also all facial structures.

Prosthetic rehabilitation of edentulous patients presenting with severely scarred oronasal cleft defects. To document the outcome of telescopic obturator prostheses attached to zygomatic implants in edentulous cleft lip and palate patients.

Four edentulous patients suffering from the consequences of cleft lip and palate were selected from a cohort of 52 patients who had received zygomatic implants between 1998 and 2010. Oronasal communication had persisted and severe scars and chronic infection were noted in the cleft region. Bone grafting procedures were contraindicated due to high risk for dehiscence. For the functional support of telescopic prostheses, nine zygomatic implants and two standard dental implants had been placed.

The zygomatic implants and the telescopic prostheses survived an average of 62 months (37 to 99 months) and were successful. Patients' satisfaction and oral function improved (on visual analog scale and oral health impact profile).

Telescopic obturator dentures supported by zygomatic implants represent a feasible option for the prosthetic rehabilitation of cleft lip and palate patients with severe impairments of the edentulous ridges including atrophy, scar tissue, ridge defects, oronasal communication, and chronic infection in the cleft region.

To compare the accuracy of three-dimensional computed tomography (3D-CT) and panoramic radiography in the evaluation of mandibular hypoplasia in patients with hemifacial microsomia (HFM).

Retrospective study of imaging data.

Images selected from the archives of the University of Tokyo Hospital.

Twenty patients with unilateral HFM who had undergone both panoramic radiography and 3D-CT in the same period.

Mandibular deformities were classified according to the Pruzansky classification; eight patients had Grade I deformity and 12 patients had Grade II deformity. Ramus heights were measured on both panoramic radiographs and 3D-CT.

Magnification in panoramic radiographs and extent of mandibular asymmetry as estimated by the affected/unaffected side ratio based on two methods were examined. The Pearson product-moment correlation coefficient was used to estimate correlations between parameters.

The magnification of ramus heights on panoramic radiographs showed large variations in Grade II patients. The affected/unaffected side ratio estimated by the two methods showed a strong correlation in Grade I patients (correlation coefficient 0.99; p < .0001). Conversely, a weak correlation was seen in Grade II patients (correlation coefficient 0.77; p = .0036), and affected/unaffected side ratios from panoramic radiographs were both over- and underestimated.

The accuracy of evaluation using panoramic radiography was fairly reliable in Grade I patients. Conversely, accuracy was poor in Grade II patients, and evaluation using 3D-CT seems preferable. The combination of two methods with careful consideration is recommended for clinical applications.

Symptoms of stress velopharyngeal incompetence (SVPI) have been reported by many wind instrument players. The current study was designed to determine (1) if symptoms of SVPI were accompanied by aeromechanical signs of SVPI and (2) if signs of SVPI differed across musical tasks.

Participants were studied during a single recording session.

The study was conducted in a university laboratory.

Participants were 10 collegiate trombone players. They were separated into two groups: six who reported symptoms of SVPI and four who reported no symptoms.

Nasal pressure recorded during trombone playing was used to determine velopharyngeal status (open or closed).

None of the participants exhibited an open velopharynx during trombone playing; however, all participants had positive nasal pressure (indicating an open velopharynx) immediately prior to sound onset on at least some of their breath groups. Two participants had positive nasal pressure prior to the vast majority of their productions and were given biofeedback and instruction to change this behavior.

Symptoms of SVPI do not necessarily indicate the presence of a velopharyngeal-nasal leak during wind instrument playing but may reflect awareness of air leaks immediately prior to sound production. Pre-sound velopharyngeal-nasal air leaks may be amenable to behavioral modification by biofeedback and instruction. Nasal pressure measurement (using a nasal cannula) provides a simple, yet powerful, way to identify SVPI.

To evaluate the prevalence, types, location, and characteristics of enamel defects in anterior permanent teeth of patients with complete unilateral and bilateral cleft lip and palate, as well as the relation with the cleft.

Hospital for Rehabilitation of Craniofacial Anomalies, Bauru, São Paulo, Brazil.

Eighty patients of both genders, 12 years and older, with unilateral or bilateral cleft lip and palate.

A single examiner carried out clinical examination under artificial light with a dental probe and mirror after drying teeth according to the modified DDE index.

Seventy-four of 80 patients presented with at least one tooth affected by enamel defects: 165 of 325 evaluated teeth (50.8%) presented enamel defects, with hypoplasia being the most prevalent (50.7%), followed by diffuse opacity (23.1%) and demarcated opacity (18.4%). The most affected tooth was 21 (36.5%), followed by 11 (34%), located at the middle (40%) and incisal (33%) thirds. Most defects occur at the buccal surface (47.7%), followed by the distal (22.7%), the mesial (19%), and the palatal (10.6%) surfaces. A significant relationship was found between the cleft side and enamel defects.

Upper anterior teeth of patients with complete cleft lip and palate present a high prevalence of enamel defects; the highest percentage on the cleft side suggests that the cleft does influence the occurrence of enamel defects in permanent teeth.

Many studies have demonstrated a high frequency of dental anomalies in patients with cleft lip and/or palate. Because dental anomalies may complicate dental treatment, we investigated the prevalence of dental anomalies in a group of Brazilian patients with nonsyndromic cleft lip and/or palate.

Retrospective analysis was performed using clinical records of 296 patients aged between 12 and 30 years with repaired nonsyndromic cleft lip and/or palate without history of tooth extraction and orthodontic treatment. Associations between oral clefts and presence of dental anomalies outside the cleft area were investigated.

Dental anomalies were identified in 39.9% of the nonsyndromic cleft lip and/or palate patients, and tooth agenesis (47.5%), impacted tooth (13.1%), and microdontia (12.7%) were the most common anomalies. Cleft lip patients were less affected by dental anomalies compared with cleft palate or cleft lip and palate patients (p = .057). Specifically, patients with unilateral cleft lip and palate were significantly more affected by dental anomalies than those with bilateral cleft lip and palate (p = .00002), and individuals with unilateral complete cleft lip and palate (p = .002) and complete cleft palate (p = .01) were significantly more affected by tooth agenesis than other cleft types. Agenesis of the premolars (p = .043) and maxillary lateral incisors (p = .03) were significantly more frequent in patients with unilateral complete cleft lip and palate.

The present study revealed a high frequency of dental anomalies in nonsyndromic cleft lip and/or palate patients and further demonstrated that patients with unilateral cleft lip and palate were frequently more affected by dental anomalies than those with bilateral cleft lip and palate. Moreover, our results demonstrate that dental anomalies should be considered during dental treatment planning for individuals affected by nonsyndromic cleft lip and/or palate.

To evaluate the hypothesis that children with craniosynostosis and their parents have differences in psychosocial outcomes, as compared with an unaffected control group.

Two studies were conducted, both which followed children born with and without craniosynostosis. Study 1 ascertained affected children from clinics, and study 2 ascertained affected children from a population-based study of birth defects.

Study 1 included 22 children with single-suture craniosynostosis and 18 controls, ages 4 to 5 years. Study 2 included 24 children with nonsyndromic craniosynostosis and 124 unaffected controls, ages 5 to 9 years.

Outcome measures included the Child Behavior Checklist, Social Competence Scale, Pediatric Quality of Life Inventory, and Parenting Stress Index.

We observed lower scores on measures of health-related quality of life in cases versus controls, with adjusted effect sizes ranging from −0.72 to −0.44 (p < .05) on summary measures. Small but statistically nonsignificant increases in behavioral problems were observed in cases versus controls, with no apparent differences in social competence or parenting stress.

Results provide preliminary evidence suggesting that children with nonsyndromic craniosynostosis may have elevated risk for psychosocial difficulties, particularly health-related quality of life. Continued follow-up through preadolescence and adolescence is warranted.

Adults with craniofacial conditions experience more psychosocial problems than adults in the general population, but little is known about the factors that render a person more or less susceptible to these problems. Guided by research on adults with other conditions that affect appearance, this study examined predictors of psychosocial outcome in adults with craniofacial conditions.

Single-sample cross-sectional design.

The Australian Craniofacial Unit, Women's and Children's Hospital, Adelaide, one of the main craniofacial treatment centers in Australia.

Adults (N = 93; 36.9% of the potential sample) with congenital craniofacial conditions (excluding cleft lip and/or cleft palate) who were treated in the Australian Craniofacial Unit.

All participants completed measures assessing anxiety, depression, and quality of life (Hospital Anxiety and Depression Scale, Short-Form Health Survey) and variables predicted to affect these outcomes (SF-36 Health Survey - Multidimensional Scale of Perceived Social Support, Rosenberg Self-Esteem Scale, Cleft Satisfaction Profile, Brief Fear of Negative Evaluation Scale, Derriford Appearance Scale).

Multiple regression analyses revealed that anxiety was predicted by social support, self-esteem, and fear of negative evaluation, while depression was predicted by self-esteem and social support. Physical quality of life was not predicted by any of the measures. Satisfaction with appearance, gender, age, and education were not related to outcome.

Interventions designed to increase perceived social support and self-esteem and reduce fear of negative evaluation appear to be indicated and may assist in establishing a causal relationship between these variables.

To describe demographic and clinical-genetic characteristics of patients from a poor area of Brazil and to share experience on how the local genetic unit has addressed their major health needs.

Descriptive cohort.

A clinical-genetic unit, a cytogenetics unit, and a regional cleft team located in the northeast and southeast of Brazil.

A total of 133 individuals with orofacial clefts who attended the surgical call of a nongovernmental organization. From this group, 125, 77, and 13 patients completed phases 1, 2, and 3, respectively.

Phase 1 comprised a description of demographic characteristics recorded through interviews. Phase 2 included a clinical-genetic evaluation using a pretested form, as well as cytogenetic analyses of selected patients. Phase 3 comprised collaborative action to address major health needs of patients without primary surgery. The Fisher test was used for statistics with p value < .05.

A majority of patients were rural residents with isolated cleft lip with cleft palate. Ages ranged between 0 and 30 years. Fifty percent had never undergone surgery; whereas, 100% had never attended a genetic evaluation. Isolated cleft was diagnosed in 77.9%, syndromes in 14.3%, and multiple congenital abnormalities in 7.8%. Positive familial history of clefts occurred in 28%; whereas, parental consanguinity was present in 7.8% cases. A total of 23 individuals without cleft surgery were registered for multidisciplinary treatment.

Findings revealed high levels of unmet medical needs and provided an evidence base for health care planning. Collaborative action was crucial and might be applied to other regions in Brazil.

To identify factors related to quality of life (QoL) and satisfaction with nasal appearance among patients treated for unilateral cleft lip and palate (UCLP).

Cross-sectional population study with long-term follow-up.

All patients with UCLP born between 1960 and 1987, treated at Uppsala University Hospital, were invited (n = 109); 86 (79%) participated. Mean follow-up time was 35 years.

Quality of life was measured with Short Form–36 (SF-36) and analyzed using mental and physical cluster scores (MCS and PCS). Nasal appearance was self-assessed with the “Satisfaction With Appearance” questionnaire and by panel judgment. Multivariate regression analyses explored endogenous factors (age, gender, infancy cleft width, nasal function, nasolabial appearance) and exogenous factors (marital status, number of children, education level, operation method, number of rhinoplasties performed).

A larger cleft width in infancy was associated with less satisfaction with nasal appearance as adults. A lower mental health QoL was associated with less satisfaction with nasal appearance. Despite female gender being linked to less satisfaction with nasal appearance, it was associated with higher mental health QoL. Higher resistance during nasal breathing was associated with lower physical health QoL.

Gender and infant cleft width may affect QoL and satisfaction with nasal appearance among adults. They are potential predictive factors for satisfaction with nasal appearance and QoL during adulthood. The correlation of nasal function impairment and decreased physical health QoL underlines the importance of treatment of nasal symptoms in these patients.

To describe oral manifestations in Brazilian individuals with Kabuki syndrome, a multiple congenital anomaly/mental retardation syndrome.

A total of 16 Kabuki syndrome individuals, aged between 8 to 24 years and of both sexes, were referred by the Department of Clinical Genetics for oral treatment and follow-up to the Oral Care Center for Inherited Diseases, University Hospital of Brasília, Brasília, Brazil. Each individual underwent complete physical examination, as well as intraoral and radiographic examinations.

Craniofacial and dental alterations were observed in all Kabuki syndrome patients examined. In addition, atypical shape of the molars' crowns, occlusal convergence of the premolars' crowns, and root dilaceration were also observed. Enamel diffuse opacities were observed in permanent dentition (n = 10).

A great clinical heterogeneity was observed in Kabuki syndrome individuals in line with previous studies in the literature. Further clinical and molecular studies are necessary in order to better understand the presence of dental anomalies in this syndrome.

This study evaluated the caries experience in the primary teeth of children with and without cleft lip and palate (CLP).

Observational study.

Dental hospital clinic.

The sample consisted of 132 pairs of Chinese children with and without CLP matched by gender, age, ethnicity, and socioeconomic status. Their ages were between 2 and 7 years.

The children were dichotomized into 2- to 4-year-old (n = 71) and 5- to 7-year-old groups (n = 61). The oral examination was performed by one trained and calibrated dentist, and the caries experience was determined using the dmft (decay missing filled teeth) index.

For the 2- to 4-year-old group, the dmft values were 1.5 for the cleft group and 1.6 for the non-CLP group, but the difference was not statistically significant. For the 5- to 7-year-old group, the dmft indices were 5.2 for the cleft group compared with 2.9 for the control group; this difference was statistically significant (p = .0006). The caries rate for the various tooth types in the 5- to 7-year-old group was higher in the CLP group than in the non-CLP group; the most marked difference was for the lateral incisors, mandibular molars, and maxillary left first molars (p = .0001).

The 5- to 7-year-old children with CLP had a higher caries experience in the primary dentition than the children without CLP.

To study if Danish children with cleft palate display lexical selectivity in their early lexicon at 18 months of age.

A cross-sectional study.

Thirty-four children with unilateral cleft lip and palate and 35 children without cleft palate, matched for gender and age.

All participants were video recorded at 18 months of age during play interaction with a parent. The video recordings were transcribed according to the International Phonetic Alphabet and an individual consonant inventory was established for each participant. The video recordings were also analyzed with respect to word productions, establishing an observed productive vocabulary size for each participant.

At 18 months of age Danish children with cleft palate showed marked lexical selectivity in their early words. The distribution of consonant classes observed at 11 months of age in a previous study of the children with cleft palate was almost perfectly reflected in their early lexicon at 18 months. The early lexicon of children with cleft palate differed from the early lexicon of their noncleft peers.

Danish toddlers with cleft palate display lexical selectivity in the early lexicon as it has been described for English-speaking toddlers with and without cleft palate, even though some qualitative differences were found.

To assess the congenital and postnatal factors that affect degree of malocclusion in patients with unilateral cleft lip and palate using multivariate statistical analysis.

Retrospective study.

All information on 135 subjects with unilateral cleft lip and palate was obtained from an oral examination and radiograph at the initial examination at an orthodontic clinic and from surgical records. Plaster models were taken before orthodontic treatment. The ages of the subjects ranged from 5 to 8 years with a mean age of 6.9 years. All primary surgeries for the patients were performed at a university hospital.

The GOSLON Yardstick was used to assess the dental arch relationships (degree of malocclusion) in patients. Family history of Class III, degree of cleft, and congenitally missing upper lateral incisor on the cleft side were chosen as congenital factors inducing malocclusion. Presurgical orthopedic treatment, cheiloplasty, and palatoplasty were chosen as postnatal factors. Associations between various factors and dental arch relationships were assessed using logistic regression analysis.

According to adjusted odds ratios, family history of Class III is associated with a significantly worse dental arch relationship. Palatoplasty using push-back alone correlated to a dental arch relationship that was significantly worse than palatoplasty using push-back with a buccal flap.

Multivariate analysis shows evidence that a positive family history of Class III and palatoplasty using push-back alone are associated with worse malocclusion of unilateral cleft lip and palate patients.

The aim of this study was the initiation of systematic data collection so as to improve the capacity for outcome measurement after cleft repair. Also, a clinical audit was done for evaluation of the process and assessment of outcomes of cleft care.

A questionnaire-based survey and outcome assessment was carried out over a 1-year period from March 2008 to February 2009 at the combined outpatient cleft clinic of a tertiary care center in India.

Data collection (basic demographic and environmental information) was done twice a week throughout the year by students from the Department of Pediatric Dentistry, at the outpatient cleft clinic.

A total of 68 completed cleft lip and palate registry forms, from which all the information was available, were analyzed. There was a skewed sex ratio, with a higher preponderance of boys seeking treatment. Of affected males, 19.1% were between 2 and 5 years of age when they first reported to the cleft clinic. Surprisingly, no bilateral clefts of lip, unilateral cleft lip (right) and unilateral cleft lip and palate (right) were observed in girls. Oral health was poor in 74% of patients; among the dental referrals only 26% could be recruited for orthodontics with a reasonably good prognosis.

Poverty, illiteracy, and superstitions prevent an average patient from India from receiving multidisciplinary cleft care. This emphasizes the need to create systems that suit the needs of our target patients.

To study early changes in cleft size and maxillary arch dimensions and to evaluate these changes in relation to performed surgical procedures.

Retrospective longitudinal study.

The Cleft Lip and Palate Center, Uppsala University Hospital, Sweden.

Dental study models of 79 consecutive children (28 with unilateral cleft lip and palate, 39 with cleft palate, and 12 with Pierre Robin sequence) were analyzed.

Lip repair at 3 to 4 months, soft palate repair at 6 to 10 months, and hard palate repair at 25 to 26 months of age.

Cleft size was measured before each surgical intervention up to 2 years and arch dimensions were measured before each surgical intervention and at 5 years.

Cleft widths decreased from infancy up to 2 years, but the anteroposterior cleft length in cleft palate was unchanged. Arch widths between cuspid points (C-C1) and tuberosity points (T-T1) and also the change over time in C-C1 and T-T1 differed significantly between the groups from infancy up to 5 years.

Cleft widths decreased after lip closure and/or soft palate closure. The children with unilateral cleft lip and palate had wider maxillary arch dimensions than the children with cleft palate or Pierre Robin sequence during the first years of life, but after hard palate closure the transverse growth was reduced in the children with unilateral cleft lip and palate. At 5 years the children with unilateral cleft lip and palate had similar maxillary widths as the children with cleft palate and/or Pierre Robin sequence.

This study investigated both the ability of children to rate nasality and the relationship of those ratings to expert ratings and social acceptance judgments.

A total of 10 speech samples were judged for nasality by 44 children ranging in age from 8 to 11 and by an expert judge. Listeners rated nasality on a 3-point response scale. The peer listeners also made five social acceptance ratings about each speaker.

Kappas for interrater reliability were moderate to substantial. There was no difference between peer ratings and expert ratings. As ratings of nasality increased, social acceptance ratings became more negative.

Professionals who evaluate and treat children with cleft palate should consider the negative social consequences of even mild hypernasality.