The Cleft Palate Craniofacial Journal

To present technical modifications to the original presurgical nasal remodeling appliance introduced in 1991. The purpose of the modifications is to improve the cleft nasal deformity before unilateral and bilateral cleft lip repair.

The principle behind this technique, known as dynamic presurgical nasal remodeling (DPNR), is the use of the force generated during suction and swallowing. A conventional intraoral plate is built with a nasal extension added to the labial vestibular flange. The nasal extension was modified and consists of three components. The palatal plate is left loose in the mouth to generate a discontinuous but controlled impact directed to the affected nasal structures during suction and swallowing. The principle aim of the DPNR technique in unilateral cases is to improve the deformation of nasal structures by straightening the columella, elevating the nasal tip, and remodeling the depressed cleft side alar cartilages. In bilateral cases, the aims are to elongate the columella and to obtain nasal tip projection.

The modifications introduced in the appliance enhance the original DPNR technique and are effective in ameliorating the initial cleft nasal deformity. This facilitates primary surgical cleft lip and nose correction and improves surgical outcomes in patients with complete unilateral and bilateral cleft lip and palate.

To describe a modified procedure consisting of a mucoso-periosteal flap palatoplasty with a marginal musculo-mucosal flap (3M flap). This is also the first report of a primary repair for complete cleft palate using the 3M flap. We describe the lengthening effect of the nasal mucous layer of the soft palate and evaluate the fistula formation rate associated with this method.

This procedure has been performed on 21 patients with unilateral complete clefts and on 27 patients with incomplete clefts. A mucoso-periosteal flap raised from the hard palate was used mainly for closure of the cleft and not for the push-back. The 3M flap repaired the deficit of the nasal mucosa, making sure that the soft palate was lengthened. Intravelar veloplasty was performed also.

The dimension of the nasal mucosal defect that can be filled with the 3M flap is 10 to 12 mm in length, oriented anterior-posterior, and 15 to 20 mm wide. Oronasal fistula formation was recognized in only 3 of 48 cases (2 of 21 complete clefts, 1 of 27 incomplete clefts) and were located at the hard-soft palate junction at the anterior portion of the 3M flap.

This method has the theoretical advantages of (1) preventing fistula formation by filling the tissue deficiency with the 3M flap; (2) achieving better velopharyngeal function due to elongation of the soft palate and retropulsion of the muscular bundle, utilizing the 3M flap; and (3) minimizing maxillary growth retardation by adopting a non–push-back method of hard palate repair.

To study the quality of scars following the use of butyl cyanoacrylate to close the skin during cleft lip repair.

Nonrandomized prospective study.

Tertiary care hospital.

Patients (n = 104) with cleft lip and palate in combination of various degrees of cleft with the age group ranging from 3 months to 5 years.

Use of butyl cyanoacrylate to close the skin during cleft lip repair after inserting dermal stitches with delayed absorbable sutures.

None of the 104 patients required revision of the vertical scar, which was excellent in nature. No patients had complications such as wound infection, dehiscence, nasal obstruction, or eyelid adhesion.

Quality of the scar following the closure of skin of cleft lip repair using butyl cyanoacrylate was considered excellent.

To study the effect of infant orthopedics on facial appearance.

Prospective two-arm randomized controlled trial in parallel with three participating academic cleft palate centers. Treatment allocation was concealed and performed by means of a computerized balanced allocation method.

Cleft Palate Centers of Amsterdam, Nijmegen, and Rotterdam, the Netherlands.

Infants with complete unilateral cleft lip and palate, no other malformations.

One group (IO⁺) wore passive maxillary plates during the first year, the other group (IO⁻) did not.

Two metrical response modalities were used (i.e., visual analog scales and reference scores) to score facial appearance. Full face and cropped photographs were compared with reference photographs and were judged. The photographs were judged by 45 judges, 24 laypeople, and 21 professionals. Transformation of the scores into z scores was applied to compare and to pool both response modalities. The validity of each individual judge was evaluated, as was the reliability of the scales. Differences between the treatment groups were evaluated by means of t tests.

Photographs were available of 41 subjects, 21 with and 20 without infant orthopedics. No significant differences were found between groups. Mean z-score values for the full-face photographs were: group IO⁺ = 0.10 (SD = 0.73) and group IO⁻ = −0.03 (SD = 0.48); for the cropped photographs were: group IO⁺ = 0.12 (SD = 0.71) and group IO⁻ = −0.06 (SD = 0.55).

Infant orthopedics have no effect on facial appearance.

Evaluation of the effect of infant orthopedics on maxillary arch dimensions in the deciduous dentition in patients with unilateral cleft lip and palate.

Prospective two-arm randomized controlled clinical trial with three participating cleft palate centers.

Cleft palate centers of the Radboud University Nijmegen Medical Center, Academic Center of Dentistry Amsterdam, and University Medical Center Rotterdam, the Netherlands.

Children with complete unilateral cleft lip and palate (n = 54) were included.

Patients were randomly divided into two groups. Half of the patients (IO+) had a presurgical orthopedic plate until surgical closure of the soft palate at the age of 52 weeks; the other half (IO−) did not undergo presurgical orthopedics.

Maxillary arch dimensions were assessed on dental casts at 4 and 6 years of age with measurements for arch width, arch depth, arch length, arch form, and the vertical position of the lesser segment. Contact and collapse were assessed also.

There were no clinically significant differences found between IO+ and IO− for any of the variables.

Infant orthopedics had no observable effect on the maxillary arch dimensions or on the contact and collapse scores in the deciduous dentition at the ages of 4 and 6 years. Considering the Dutchcleft results to date, there is no need to perform infant orthopedics for unilateral cleft lip and palate patients.

This article reports a case of bilateral cleft lip and alveolus (BCLA) for which excessive rapid palatal expansion with a Latham appliance was performed for preoperative alignment of the protruded premaxilla. Postoperative changes of maxillary width were investigated with serial plaster casts.

A 3-month-old girl presented with complete BCLA in which the premaxilla was markedly protruded. Preoperative alignment of the protruded premaxilla with a Latham appliance was planned to facilitate primary lip repair. The appliance was placed when the patient was 4.5 months old. The necessary palatal expansion was estimated to be 7.0 mm in order to move the premaxilla backward into the ideal position. After palatal expansion and posterior repositioning of the protruded premaxilla, the primary operation, including cheiloplasty and gingivoperiosteoplasty, was performed when the patient was 7 months old. Excessive maxillary expansion might be a cause of transverse maxillomandibular discrepancy. Measurement with serial plaster casts demonstrated that maxillary widths increased from 42.3 mm pretreatment to 49.0 mm after orthopedic treatment but relapsed markedly to 43.5 mm at 3 months after the primary operation. Therefore, the net change of maxillary widths was only 1.2 mm. After alignment of the protruded premaxilla, tension-free soft tissue repairs were performed, and a harmonious alveolar arch was obtained without change in maxillary width.

These results indicate that this method is useful for preoperative management of BCLA with protruded premaxilla.

To quantitatively compare the alveolar bone support ratio of the permanent canine in cleft patients who received secondary alveolar bone graft with that of the population without clefts.

Retrospective study utilizing periapical radiographs of the subjects with and without clefts.

Hospital and university based.

Eighteen unilateral and 9 bilateral cleft patients who had secondary bone graft procedures.

Alveolar bone support of the permanent canine utilizing the ratio of bone height to root length.

Average bone support for the permanent canine was 88.55% in patients with clefts and 95.59% in patients with no history of clefts. This difference was statistically significant. There was no statistically significant difference in alveolar bone support ratio between the unilateral and bilateral cleft patients.

Although alveolar bone support was significantly higher in the noncleft control group, a successful level of alveolar bone support was achieved for the permanent canine on the cleft site after secondary bone graft. There was no difference in alveolar bone support achieved for the permanent canine whether the type of the cleft was unilateral or bilateral.

The current study was designed to evaluate the neurobiology of reading in a group of men with nonsyndromic clefts of the lip or palate (NSCLP) compared with healthy controls by positron emission tomography.

Subjects included eight men with NSCLP compared with six healthy control men. By using radioactively labeled water (O¹⁵), regional brain blood flow was obtained during the performance of three simple reading tasks: reading unrelated words, reading unrelated sentences, and reading a story.

During each of the reading conditions, NSCLP subjects compared with healthy controls showed increased blood flow in areas previously reported to be involved in language processing and reading (inferior frontal lobe, cerebellum, and occipital lobe). The increased blood flow suggests a possible neural inefficiency. In contrast, when analyzing the brain regions involved in more complex language functioning (reading stories compared with reading only words), control subjects showed an increase in blood flow in a distributed neural circuit, whereas the NSCLP subjects showed a decrease in flow in these regions. Additionally, the NSCLP subjects had activation of several regions not activated in the healthy controls, suggesting a compensatory circuit used for this more complex reading task.

These results indicate that subjects with NSCLP show abnormalities in the function of the distributed neural circuitry used for oral reading.

Our objective was to estimate nasalance due to transpalatal transfer of acoustic energy during production of voiced stop consonants by noncleft speakers. We also determined the relationship between the transpalatal nasalance and fundamental frequency (F0) of the speakers.

Participants were 8 men and 10 women (mean age = 21.9 years, SD = 4.0) without cleft palate who produced voiced stop (/b d g/) and nasal (/m n η/) consonants in syllables embedded in a carrier phrase. Participants also read the Zoo Passage. A divided OroNasal Nasality System mask was used to simultaneously obtain acoustic nasalance and airflow during production of the consonants. Both F0-derived and first formant (F1)–derived nasalance were computed.

F0-derived and F1-derived peak nasalance across all speakers ranged from a low of 20% to a high of 80% during production of stop consonants. An estimate of error from the combined sources of transoral transfer of energy (5%) and acoustic crossover between microphones (15%) was no greater than 20%. Analysis of variance revealed no significant effects of the sex of the speakers for either F0-derived or F1-derived nasalance of stops. There was a significant effect of the place of stop production for F0-derived nasalance (p;th< .05). Nonsignificant but positive correlations were found between the F0 of the speakers and F0-derived (r = .25) and F1-derived (r = .45) nasalance.

Transpalatal transfer of oral acoustic energy accounts for most nasalance obtained during production of voiced stop consonants by noncleft speakers. F1-derived nasalance appears to better reflect transpalatal effects. Clinical implications are discussed.

To examine the natural history of feeding skills in babies with clefts and identify risk factors and predictors of poor feeding.

Sixty-two babies with clefts were examined at 2 weeks, 3 months, and 14 months of age.

Feeding ability, oral motor function, and feeding efficiency were assessed. Univariate analyses were used to determine whether oral motor function and sequelae varied according to feeding ability or cleft condition. Multivariable logistic regressions were used to determine risk factors for poor feeding.

Poor feeding skills were detected in one third of newborns. The prevalence of poor feeding reduced to 19% at 3 months of age and 15% at 14 months of age. Oral motor dysfunction and sequelae (particularly nasal regurgitation) were more commonly observed in babies with poor feeding skills irrespective of comorbidity. The main risk factor for poor feeding was a diagnosis of syndrome or Pierre Robin sequence (PRS). At 2 weeks of age, babies with syndrome or PRS were 15 times more likely to have poor feeding skills than their nonsyndromic counterparts. When syndrome or PRS was controlled for, babies with cleft palate and cleft lip and palate were equally likely to have poor feeding skills. Parental report of feeding efficiency was predictive of poor feeding in young babies.

Poor feeding skills are relatively common in newborns with cleft palate and cleft lip and palate. Treatment for feeding problems may be needed beyond the first year of life, especially for babies born with PRS or a syndrome.

The Dutch Cleft Palate Association (DCPA) registers all patients with cleft lip or palate and associated congenital anomalies in the Netherlands. The aim of this study was to assess if early registration of cleft patients leads to underreporting of associated congenital anomalies and, if so, whether reregistration is necessary.

The DCPA registration of the birth cohort 1997 to 2001 was compared with the medical files of these cleft patients for prevalence, type, and moment of registration of associated congenital anomalies. To assess possible long-term underregistration, a second birth cohort of 1990 to 1991 was analyzed.

The percentage of cleft patients with associated congenital anomalies was 26% in the DCPA database and 33% in the retrospective medical file review. A syndrome, sequence, or association was recognized in 8% of the cleft patients by the DCPA compared with 13% in our medical file review. Of all associated congenital anomalies diagnosed during a follow-up of 12 years, 53% were diagnosed in the first year of life. The cumulative percentage was 59% after 2 years, 62% after 3 years, 80% after 6 years, and 97% after 10 years.

Early registration of cleft patients leads to underreporting of other associated anomalies. For a complete registration of associated congenital anomalies in cleft patients, reregistration at a later age is necessary.

To evaluate the outcomes of care for children by type of oral cleft.

Data were collected through structured telephone interviews during 2003 in Iowa with mothers of 2- to 12-year-old children with oral clefts. Interviews with mothers of children with clubfoot and statewide data on Iowa children were used for comparison.

Participants included mothers of children in Iowa born between 1990 and 2000 with nonsyndromic oral clefts. Children were identified by the statewide Iowa Registry for Congenital and Inherited Disorders.

Rating of cleft care, severity of condition, health status, esthetic outcome, speech, and school performance were evaluated by type of oral cleft.

Children with cleft lip and palate were most likely to have their clefts rated as very severe. Children with palatal involvement were reported to have a lower health status and were almost twice as likely to be identified as having a special health care need compared with either children with cleft lip or children statewide. Children with cleft lip had more esthetic concerns; children with palatal involvement had the most speech concerns.

Although mothers generally believed their children had received high-quality care, ratings of the children's current health status and outcomes of care varied significantly by type of cleft (cleft lip, cleft palate, and cleft lip and palate). Differences observed in this population-based study support the proposition that cleft type should be considered when examining outcomes of care for children with oral clefts.

To assess the oral health knowledge of mothers of children with cleft lip and/or palate, analyzing the practical application of this information on the basis of the oral hygiene of the children and caries prevalence of mothers and children.

Cross-sectional.

Pediatric Dentistry sector of the Hospital for Rehabilitation of Craniofacial Anomalies.

Mothers (n = 300) and their children with cleft lip and/or palate, ages 3 years to 5 years 6 months.

An interview with questions related to dietary habits and oral health knowledge and habits was conducted. Children and their mothers also were evaluated by intraoral clinical examination.

Of the mothers, 47.3% displayed poor oral health status (Group A) and 52.7% had a satisfactory oral health status (Group B). Children in Group A presented a mean dmft of 6.0, whereas children in Group B had a mean dmft of 5.6. No significant differences were observed between groups. Mothers demonstrated a reasonable knowledge on prevention of dental caries and oral hygiene.

Even though the mothers interviewed had some knowledge on the causes and prevention of dental caries, other factors should be regarded as relevant in the caries process.

The purpose of this study was to investigate the effect of personal contact on the attitudes of individuals towards persons with cleft lip and palate (CLP) and the possible cultural differences in these attitudes for Chinese respondents living in Hong Kong.

Chinese parent, teacher, and employer groups were administered a cleft lip and palate attitudinal questionnaire and the Scale of Attitudes Toward Disabled Persons inventory.

Thirty-nine parents of children with CLP, 27 teachers of children with CLP, and 37 employers with no previous contact with individuals with CLP participated in the study.

The results showed significant intergroup differences on several items involving beliefs concerning emotional health, social development, and employment-related barriers for individuals with CLP, and whether such individuals should seek nonmedical help to treat speech problems. Employers showed a comparatively less favorable attitude to persons with CLP than the two other groups.

Parent, teacher, and employer groups in the Chinese community studied showed differences in their attitudes towards individuals with CLP. The findings suggest the need for further community health education to help develop more positive attitudes towards disabled persons in general, as well as programs targeted at reducing negative stereotyping of adults and children with CLP.

To characterize the craniofacial phenotype of a mouse model for Crouzon syndrome by a quantitative analysis of skull morphology in mutant and wild-type mice and to compare the findings with skull features observed in humans with Crouzon syndrome.

MicroCT scans and skeletal preparations were obtained on previously described Fgfr2^C342Y/+ Crouzon mutant mice and wild-type mice at 6 weeks of age. Three-dimensional coordinate data from biologically relevant landmarks on the skulls were collected. Euclidean Distance Matrix Analysis was used to quantify and compare skull shapes using these landmark data.

Obliteration of bilateral coronal sutures was observed in 80% of skulls, and complete synostosis of the sagittal suture was observed in 70%. In contrast, fewer than 40% of lambdoid sutures were found to be fully fused. In each of the 10 Fgfr2^C342Y/+ mutant mice analyzed, the presphenoid-basisphenoid synchondrosis was fused. Skull height and width were increased in mutant mice, whereas skull length was decreased. Interorbital distance was also increased in Fgfr2^C342Y/+ mice as compared with wild-type littermates. Upper-jaw length was shorter in the Fgfr2^C342Y/+ mutant skulls, as was mandibular length.

Skulls of Fgfr2^C342Y/+ mice differ from normal littermates in a comparable manner with differences between the skulls of humans with Crouzon syndrome and those of unaffected individuals. These findings were consistent across several regions of anatomic interest. Further investigation into the molecular mechanisms underlying the anomalies seen in the Crouzon mouse model is currently under way.