The Cleft Palate Craniofacial Journal

To determine the presence of specific patterns of dental defects in a group of individuals born with clefts.

Case-control.

Nossa Senhora do Loreto Municipal Hospital and Federal University of Rio de Janeiro Pediatric Dentistry Clinics, Rio de Janeiro, Brazil.

642 radiographic and clinical records were evaluated for 321 cases and 321 controls.

Individuals born with clefts presented considerably more dental anomalies in comparison with controls (p = .0001). The most frequent was tooth agenesis (n = 53; p = .001), followed by supernumerary teeth (n = 14; p = .11) and tooth malposition (n = 11; p = .33). Regarding tooth agenesis, the upper lateral incisors were more frequently absent in the cleft group (31/86), and control individuals presented more agenesis of the mandibular second premolars (19/45).

The higher prevalence of dental anomalies, especially number anomalies, opposite the cleft area, in individuals born with cleft lip and/or palate is the most common associated dental anomaly outside the cleft area.

Comparison of nasopharyngeal airway dimensions among UCLP patients and the control group.

Computed tomography imaging data and cephalometric films of 20 patients with UCLP and 20 controls were acquired prior to treatment. Three-dimensional (3D) reconstructions of nasal, superior, middle, and inferior pharyngeal airways were carried out from dicom data, and volumes of these compartments were calculated. Nasal width and superior, middle, and inferior nasopharyngeal areas were calculated from cephalometric films.

The only statistically significant difference among the cleft and noncleft patients was detected in nasal volume (p < .01). Upper and middle pharyngeal airway areas were found to display significant larger areas in the noncleft group on cephalometric appraisal. Nonsignificantly larger nasal widths were measured in the cleft group.

Evaluation of the nasopharyngeal airways of cleft and noncleft patients was done, resulting in only the nasal region showing statistically significant difference. Our results showed significantly lower nasal volume measurements of cleft patients when compared with the healthy individuals (p < .01). This inadequacy can be interpreted as an outcome of the deficiency. The controversy among results of cephalometric appraisal and 3D imaging can be associated with superimpositions, density, and contrast inadequacies and obscured landmarks. When 2D data are compared with 3D data, the former can be insufficient and therefore deceiving. Consequently, although the radiation dose of 3D imaging systems is greater, they have the advantage of superior diagnostic outputs.

To determine the incidence and most frequent anatomical locations of residual oronasal fistulas in children with different types of clefts who were treated at the University Department of Maxillofacial and Oral Surgery in Ljubljana.

Retrospective analysis of 857 consecutive patients with cleft born between 1984 and 2003.

After primary surgical repair, 33 out of 857 (3.9%) children had residual oronasal fistulas. The incidence of clinically significant fistulas that required surgical repair was 17 of 857 (2.0%). In children with unilateral cleft lip and palate (UCLP), bilateral cleft lip and palate (BCLP), or isolated cleft palate (CP), the incidence of palatal fistulas was 23 of 644 (3.6%). Of these palatal fistulas, 12 (1.9%) were clinically significant. Oronasal fistulas were most frequently found in children with BCLP (9 of 88, 10.2%), followed by UCLP (12 of 215, 5.6%) and CP (12 of 341, 3.5%). No fistulas were found in children with unilateral cleft lip and alveolar ridge. In 10 cases (30.3%), the oronasal fistula remained in the alveolar ridge, in 11 cases (33.3%) in the anterior part of the hard palate, and in 11 cases (33.3%) at the junction of the hard and soft palates. In one case (3%), the records were lost.

The low incidence of oronasal fistulas is the result of a surgical technique and tensionless suturing, followed by a two-layer closure, two-stage palate repair in BCLP and UCLP patients, and preoperative orthopedics in UCLP cases.

To study long-term maxillofacial development in a sample of patients born with unilateral cleft lip and palate. The patients’ palatal repair had been performed according to a two-stage protocol with early velar closure and delayed hard palate surgery.

Retrospective, longitudinal cohort study.

The sample consisted of 50 consecutive patients with unilateral cleft lip and palate born from 1980 to 1989. All of them had been operated on at the Sahlgrenska University Hospital in Gothenburg, Sweden.

Certain maxillary casts as well as lateral roentgencephalograms, obtained at 5, 10, 16, and 19 years of age, were analyzed.

Our patients’ maxillofacial growth was very good even up to the final examination in early adulthood. We ascribe the advantageous midfacial morphology of our patients particularly to limited growth restriction from palatal scars due to the surgical protocol used in our cleft center.

The two-stage protocol we advocate for repair of the palatal cleft resulted in very satisfactory growth outcome. A palatal scar, which might impair maxillary development, was created only by the velar surgery. If this scar is located close to the posterior border of the hard palate, it might result in less maxillary growth restriction than if it is positioned further anteriorly.

To investigate long-term, longitudinal speech outcome in patients born with unilateral cleft lip and palate treated according to a two-stage primary palatal protocol with early veloplasty and delayed hard palate closure.

Retrospective, longitudinal cohort study.

A university hospital in western Sweden.

A consecutive series of 55 patients from the total cohort of 65 were included. All patients had surgical procedures at Sahlgrenska University Hospital, Gothenburg, Sweden.

Standardized audio recordings were blindly analyzed at 5, 7, 16, and 19 years of age and after at a clinical visit at 10 years of age. Typical cleft speech variables were rated independently on ordinal scales. Intelligibility and perceived velopharyngeal function were assessed also. Prevalences of speech characteristics were determined, and interrater and intrarater agreement were calculated.

Prominent hypernasality, nasal air leakage, and retracted oral articulation at 5 years were markedly reduced throughout the years with low prevalences at ages 16 and 19 years. Perceived velopharyngeal competence was noted in 82% at age 16 and 87% at age 19 years along with normal intelligibility. Pharyngeal flap surgery was performed in 6 of the 55 patients (11%).

Long-term speech outcome in patients with two-stage palatoplasty with early soft palate repair was considered good and improved even before hard palate repair. The typical retracted oral articulation was quite frequent during the early ages; whereas, nonoral misarticulations were almost nonexistent, implying good velopharyngeal competence.

To compare palatal dimensions in 6-year-old children with unilateral cleft lip and palate (UCLP) treated by different protocols with those of noncleft children.

Retrospective intercenter outcome study.

Upper dental casts from 129 children with repaired UCLP and 30 controls were analyzed by the trigonometric method.

Six European cleft centers.

Sagittal, transverse, and vertical dimensions of the palate were observed.

Palate variables were analyzed with descriptive methods and nonparametric tests. Regarding several various characteristics measured on a relatively small number of subjects, hierarchical, k-means clustering, and principal component analyses were used.

Mean values of the observed dimensions for five cleft groups differed significantly from the control (p < .05). The group with one-stage closure of the cleft differed significantly from all other cleft groups in most variables (p < .05). Principal component analysis of all 159 cases identified three clusters with specific morphologic characteristics of the palate. A similar number of treated children were classified into each cluster, while all children without clefts were classified in the same cluster. The percentage of treated children from a particular group that fit this cluster ranged from 0% to 70% and increased with age at palatal closure and number of primary surgical procedures.

At 6 years of age, children with stepwise repair and hard palate closure after the age of two more frequently result in palatal dimensions of noncleft control than children with earlier palatal closure and one-stage cleft repair.

To evaluate the skeletal maturation of male children with orofacial clefts using the cervical vertebral maturation (CVM) method.

Case-control study.

The School of Stomatology, Peking University.

Subjects were 443 boys with cleft lip and/or cleft palate (CL/P) aged 8 to 16 years who were compared with 1772 boys without clefts in an age-matched control group.

The 8- to 10-year-old boys of both groups were in CS1. Most of the 11- to 14-year-old boys without CL/P were in CS3 and CS4, though it was not so obvious in boys with CL/P. Most 15- to 16-year-old boys without CL/P were in CS5 and CS6, while there were a lot of boys with CL/P who were still in cervical stages before CS5. Clefts had a significant effect on growth, and boys without CL/P were 1.737 times more likely to have achieved higher cervical stages. From Mann-Whitney test of every age phase, the difference existed in each year of the age range of 11 to 16 years, except from 12 to 13 years (p = .100). In these age phases, the cervical stages of boys with CL/P were lower than those for boys without CL/P. By the age of 14, children with CL/P had a 4.679 times higher risk of delayed CS3, and the 95% confidence interval for the odds ratio was 2.129 to 10.286.

Boys with CL/P are at a higher risk of delayed growth period and retarded pubertal growth peak.

Nasal reconstruction for patients with unilateral cleft lip and palate (UCLP) is a challenge for the reconstructive surgeon. Presurgical nasoalveolar molding (PNAM) was introduced to reshape the cleft nasal structures prior to lip repair. This study analyzed two-dimensional nasal changes before and after PNAM in patients with complete UCLP.

Thirty UCLP patients (19 males; 11 females) who received PNAM before lip repair were included in this study. PNAM was applied for 100 days. Nasal casts were obtained before and after PNAM. Frontal and 45° standardized digital photographs were taken from all casts, and a photogrammetric analysis (16 linear, six angular, and two area measurements) was performed. Paired Student's t tests were used to search for differences by time, and time versus side (cleft versus noncleft).

Significant reduction of cleft columella deviation with an increase in columella length, nostril height, and axial inclination on the cleft side were recorded. This resulted in an increase in the projection of the nasal tip. The noncleft measurements remained without significant changes. The cleft nostril area increased significantly more than the noncleft side by 90% with PNAM treatment. Significant normal growth changes were observed in nasal width and nasal height.

A favorable reshaping of the nose after PNAM was achieved, resulting in an improvement in form before lip surgery. These changes lead to improved nasal symmetry before primary lip and nasal reconstruction in UCLP patients.

To explore a new method of repair of alveolar cleft by trans-sutural distraction osteogenesis.

Nine 8-week-old mongrel dogs were assigned randomly to two groups with three in the control group and six in the experimental group.

First, an alveolar cleft model was created surgically in all animals. After 2 weeks, a U-shaped distractor, made of nickel-titanium (NiTi) shape memory alloy wire with 200 g tensile force, was inserted into the premaxilla of the experimental dogs to distract the mid-premaxillary suture for 3 weeks. Periosteoplasty of the alveolar cleft was performed when the premaxilla at the side of cleft approached the maxilla at the same side. The distractor was removed 2 weeks post periosteoplasty.

The results were evaluated clinically, radiographically, and morphologically.

The cleft model was stable and similar to the human alveolar cleft. No spontaneous bone union occurred in the control. In experimental dogs, the premaxilla was moved slowly toward the maxilla, and the cleft became gradually narrower and closed in the third week. Radiographically, the distracted mid-premaxillary suture showed a gradually widened triangle, with the tip of the triangle pointed posteriorly. The density of the distracted triangle suture was increased gradually. The alveolar cleft was completely bony 3 months post periosteoplasty. The morphology of the mid-premaxillary suture was also restored.

The alveolar cleft could be repaired by the technique of mid-premaxillary suture distraction using the elastic device of NiTi shape memory alloy.

This study was performed to investigate whether nasal and oropharyngeal microbiological swabs taken prior to cleft lip and palate surgery correlated with the oronasal flora at the time of surgery and whether specific culture results affected surgical outcome.

Prospective audit set in two designated U.K. cleft centers each with a single surgeon. Nasal and oropharyngeal microbiological swabs were taken within 2 weeks prior to surgery and again on the operating table. Adverse outcome measures included postoperative pyrexia, wound dehiscence, or fistula formation.

One hundred forty-four cases were recruited over 12 months. Nasal swabs cultured organisms significantly more often than oropharyngeal swabs (p < .0001). No significant difference was detected in the number of cases with a positive microbiology culture preoperatively compared with perioperative sampling (48% and 50%). The specific organisms cultured from preoperative swabs were the same as those cultured at surgery in only half of cases. Preoperative microbiology swabs were poorly predictive of the oronasal flora at surgery. Antibiotic treatment of patients with positive preoperative microbiology did not significantly reduce the incidence of bacterial colonization or significantly alter clinical outcome.

Preoperative microbiological investigation is not helpful in predicting the nasal and oropharyngeal flora at the time of surgery. Further, culture results did not correlate with postoperative outcome, regardless of whether pre- or perioperative antibiotic therapy was instigated. This evidence suggests that microbiology screening swabs are an unnecessary investigation.

Recent genome-wide association studies identified susceptibility loci for nonsyndromic cleft lip with or without cleft palate (NSCL±P) on 8q24.21, 10q25.3, 13q31.1, 15q13.3, 17q22, and 18q22 in populations of European origin. The purpose of this study was to determine, using DNA samples, whether 8q24.21 was a susceptibility locus for the development of NSCL±P in Japanese patients.

We used DNA from 167 Japanese NSCL±P patients (45 cleft lip without cleft palate and 122 cleft lip with cleft palate patients) and 190 Japanese unaffected control individuals. We performed an association study using 13 single nucleotide polymorphisms (SNPs) selected on the 8q24.21 locus. Genotyping of each SNP was carried out by direct sequencing of genomic DNA. Additionally, a haplotype block was constructed using the selected SNPs.

The 13 selected SNPs were successfully genotyped in 357 individuals. The p values obtained were not low enough to indicate a significant association between the haplotypes and the development of NSCL±P in this population.

Our results suggest that the 8q24.21 locus is not associated with susceptibility to NSCL±P in Japanese patients and provide further evidence that ethnicity is a strong factor in determining susceptibility loci, albeit using a limited number of samples. Further studies are needed to identify regions involved in the development of NSCL±P in the Japanese population.

To investigate the impact of parental diagnosis of cleft lip and/or palate on factors associated with parental adaptation to having a child with a cleft.

A mixed-methodological, quasi-experimental, causal-comparative research design.

A total of 27 parents born with a cleft lip and/or palate and 27 parents born without a cleft lip and/or palate completed the study measures.

The Ways of Coping Questionnaire, the Connor-Davidson Resilience Scale, the Interpersonal Support Evaluation List, and the Posttraumatic Growth Inventory were included along with a researcher-designed, cleft-specific questionnaire.

The cleft-specific questionnaire revealed that parents born with a cleft lip and/or palate reported feelings of guilt significantly more often than did parents without a cleft lip and/ or palate. Parents without a cleft reported feeling anxious significantly more often than did parents born with a cleft lip and/or palate. The qualitative aspect of the study yielded further between-group differences. The feeling that their own cleft-related experiences influenced their adjustment to having a child with a cleft emerged as a dominant theme for parents born with a cleft; whereas, parents without a cleft lip and/or palate highlighted the importance of accurate information and positive interactions with clinicians in facilitating adjustment to their situation. No significant between-group differences were found on the standardized measures; however, the study's small sample size increases the risk of type II error and may account for the lack of significant findings.

These findings appear to provide support for the widely held clinical opinion that parental diagnosis of cleft lip and/or palate impacts how parents cope with and adjust to their child's diagnosis.

To evaluate the current pattern of cleft lip and/or palate deformities in Lagos, Nigeria.

Descriptive epidemiology.

Statewide survey of patients.

All patients with cleft lip and palate deformities that were seen during a screening program for individuals with orofacial deformities in Lagos, Nigeria, between June 2006 and May 2008 participated in this study. Demographic data were collected and physical examinations carried out. Cleft lip and/or palate deformities were classified using the Kernahan classification for common cases and the Tessier classification for the atypical cleft defects.

A total of 225 patients were enrolled and completed the study. Cleft lip and palate was the most common presentation and was found in nearly 45% of the patients. Cleft lip was found in 61 (27%) patients. Twenty patients (8.89%) with different types of rare orofacial cleft deformities were seen. Cleft lip with or without cleft alveolus deformities occurred more commonly among males; whereas, cleft palate was more common among females. Cleft lip and/ or palate occurred more frequently among males. Cleft lip with or without cleft alveolus was found predominantly on the left side.

The pattern of cleft lip and/or palate deformities in Lagos, Nigeria, is different from those of other parts of Nigeria in some aspects. The proportion and varieties of atypical orofacial deformities in the study are striking. The prevalence of cleft lip and/or palate deformities in the study was 2.25 per 100,000. There may be an association between the prevalence of atypical deformities and the high level of environmental pollutants in the Lagos area.

To determine factors associated with orofacial clefts and postsurgical complications of cleft lip and palate repair surgeries in northwestern Tanzania.

This was a cohort study involving patients with orofacial clefts. Associated factors (family history of orofacial clefts, maternal use of alcohol and cigarette smoking during pregnancy) were obtained through interviews with accompanying parents. Antenatal cards were used to obtain maternal age at birth and birth weight.

Ninety-four patients with different orofacial clefts were seen. Among them, 46.8% (44/94), 13.8% (13/94), and 39.4% (37/94) had cleft lip, cleft palate, and cleft lip and palate, respectively. About 15% of orofacial cleft cases had a positive family history of orofacial clefts. Among these, 7.4% had an affected relative on the maternal side, 4.3% had an affected relative on the paternal side, and 3.2% had an affected sibling. This difference was statistically significant (chi-square = 27.7, p < .001). Orofacial cleft was significantly associated with order of birth (chi-square = 21.0, p < .001). Postoperative complications observed included palatal fistula and philtrum dehiscence.

Family history of orofacial clefts and order of birth were significantly associated with orofacial clefts in northwestern Tanzania. These factors have been associated with risk of orofacial clefts elsewhere and suggest a hereditary role in the etiology of orofacial cleft. Palatal fistula and philtrum dehiscence were postsurgical complications observed in orofacial clefts patients who had primary surgery past the recommended age. These complications could have resulted from delayed surgery and absence of presurgical procedures.

The purpose of this study was to examine the relation of perceptual ratings of nasality by experienced listeners, measures of nasalance, and the size of the nasal port opening for three simulated English corner vowels, /i/, /u/, and /a/.

Samples were generated using a computational model that allowed for exact control of nasal port size and a direct measure of nasalance. Perceptual ratings were obtained using a paired-stimulus presentation.

Five experienced listeners.

Measures of nasalance and perceptual nasality ratings.

Differences in nasalance and perceptual ratings of nasality were noted among the three vowels, with values being greater for the high vowels /i/ and /u/ compared to the low vowel Id. Listeners detected nasality for the high and low vowels simulated with nasal port areas of 0.01 and 0.15 cm², respectively. Correlations between ratings of nasality and nasalance were high for all three vowels.

Results of the present study show a high correlation between ratings of nasality and measures of nasalance for nasal port areas ranging from 0 to 0.5 cm². The correlations were based on sustained vowel samples. The restricted speech sample limits generalization of the findings to clinical data; however, the results are a demonstration of the usefulness of modeling to understand the perceptual phenomena of nasality.

To demonstrate the feasibility of simultaneous-acquired magnetic resonance imaging (MRI) and high-quality synchronized audio recording for evaluating velopharyngeal closure.

Institutional Review Board–approved case series.

Tertiary care hospital.

Three healthy adult volunteers with a normal speech pattern.

MRI with simultaneous recorded audio files evaluating velopharyngeal closure.

Precise imaging and audio coordination of specific phonatory tasks.

Synchronization of MRI and audio in all three adults.

Our novel imaging and audio protocol provides simultaneous acquired MRI with synchronized high quality audio for evaluating velopharyngeal closure. This technique may provide the opportunity to improve diagnosis and surgical planning in patients with velopharyngeal insufficiency.