bluuebunny/crossref_metadata_2025_split huggingface dataset:
Analysis of Speech Characteristics in Children with Velocardiofacial Syndrome (VCFS) and Children with Phenotypic Overlap without VCFS
Authors: D'Antonio Linda L. , Scherer Nancy J. , Miller Laura L. , Kalbfleisch John H. , Bartley James A. 
Published: October 2018

Objective: To address two questions of theoretical importance regarding the profile and course of communication impairment associated with velocardiofacial syndrome (VCFS): (1) do speech characteristics of children with VCFS differ from a group of children with some of the phenotypic characteristics of VCFS who do not have the syndrome, and (2) do younger children with VCFS demonstrate speech patterns that differ from older children with VCFS? Design: Prospective, cross-sectional study comparing two groups of children at two age levels. Patients: Thirteen children with VCFS and eight children with some of the phenotypic features of VCFS who did not have the syndrome. Children ranged in age from 3 to 10 years. Main Outcome Measure: (1) Broad phonetic transcription of speech yielding measures of number of consonant types, Percent Consonant Correct, and percentage of glottal stops used; and (2) composite ratings of velopharyngeal function made from perceptual, aerodynamic, and endoscopic evaluations. Results: Younger children with VCFS demonstrated greater speech impairment than older children with VCFS or the children without VCFS, such as smaller consonant inventories, greater number of developmental errors, greater severity of articulation disorder, and higher frequency of glottal stop use. The relationship between ratings of velopharyngeal function and the speech variables analyzed was not straightforward. Conclusions: Some young children with VCFS demonstrated speech impairment that is qualitatively and quantitatively different from older children with VCFS or children without VCFS. This finding supports the hypothesis that some children with VCFS demonstrate a profile of speech production that is different from normal but also may be specific to the syndrome.
Palatal Development in Twirler Mice
Authors: Gong Siew-Ging , Eulenberg Rachel L. 
Published: October 2018

Objective: The development of the secondary palate of Twirler (Tw) mice was characterized, and a quantitative coronal plane evaluation of the width and length of the craniofacial regions of homozygous mutant embryos was conducted. Results and Conclusions: The secondary palatal shelves were retarded in growth and the timing of elevation from a vertical to horizontal position. The homozygous Tw embryos, when compared with their wild-type littermates, also had a significantly wider midface. It is not surprising, therefore, that the short palatal shelves in a wide midface resulted in cleftings of the secondary palate observed in all homozygous Tw mice. These findings are consistent with similar studies in humans and other murine models.
New Design of a Palatal Lift Prosthesis Combined with a Palatal Bar
Authors: Ueda Naoto , Sato Yasumori , Senoo Yasuhiro , Mori Toshiko , Moriguchi Takahiko , Nakagawa Hirofumi 
Published: October 2018

Objective In the past, palatal lift prostheses (PLPs) have been problematic with respect to stability. This stability problem has been addressed by designing a PLP that includes a palatal bar. Structure A PLP combined with a palatal bar is functionally characterized by a palatal bar that holds down the posterior palatal plate. By making this location the fulcrum, the force exerted by the soft palate on the lamina actually functions to stabilize the prosthesis instead of acting to overturn it. Cases This new prosthetic design was applied to patients with cleft palate and those who had undergone cerebral contrusion operations. The experimental group was composed of a total of 13 patients (7 men and boys, 6 women and girls) who ranged in age at the time of introduction of the prosthesis from 4 years 6 months old to 54 years 5 months. The period of follow-up after prosthetic insertion ranged from 2 months to 4 years 1 month. In all cases, the stability of the prosthesis was sufficiently maintained. Conclusion Because of increased stability, this technique can be applied to not only adult patients but also children in whom insertion during the deciduous dentition period can be difficult. The new PLP was also found to be effective in patients with cleft palate with a short clinical crown.
Mandibular Asymmetry and Antigonial Notching in Individuals with Unilateral Cleft Lip and Palate
Authors: Kyrkanides Stephanos , Richter Liubov 
Published: October 2018

Objective To determine whether asymmetric antigonial notching is associated with the development of mandibular and lower facial asymmetry in individuals with unilateral cleft lip and palate (UCLP). Design Retrospective mixed-longitudinal investigation including UCLP and nonaffected orthodontic patients. Participants All (24) available patients with UCLP treated in our clinic with complete longitudinal records, and 72 skeletal maturation matched noncleft orthodontic patients that served as controls. Subject records included hand-wrist, panoramic, frontal, and 45° oblique cephalometric radiographs. Main outcome Measures The development of mandibular asymmetry, measured on oblique cephalometric radiographs, as well as lower facial asymmetry, measured on frontal cephalometric radiographs, was monitored in individuals with UCLP in relation to antigonial notching asymmetry, assessed on panoramic and oblique cephalometric radiographs. Results First, no statistical differences were found in antigonial notching asymmetry between patients with UCLP and noncleft controls. Second, antigonial notching asymmetry, observed on panoramic and oblique cephalometric radiographs, significantly correlated with the attendant development of mandibular and lower facial asymmetry in individuals with UCLP. Third, the development of mandibular and lower facial asymmetry at pubertal and post-pubertal growth spurt stages significantly correlated with antigonial notching asymmetry, measured on panoramic radiographs, at prepubertal and pubertal growth spurt stages, respectively. Conclusions The degree of antigonial notching noted on panoramic radiographs can be used as an early indicator of developing mandibular and lower facial asymmetry in individuals with UCLP.
Assessing Intelligibility in Speakers with Cleft Palate: A Critical Review of the Literature
Authors: Whitehill Tara L. 
Published: October 2018

Objective A literature review was conducted in order to investigate three research questions: how is speech intelligibility being measured in speakers with cleft lip and palate? Is intelligibility adequately being distinguished from related measures such as acceptability? Has there been an increased understanding of intelligibility deficits in speakers with cleft lip and palate? Fifty-seven relevant articles published between 1960 and 1998 were included in the analysis. Results The results showed an increase in the number of articles that included a measure of intelligibility or a similar measure. Several concerns were raised as a result of the review, including the reliability and validity of measures being employed, adequate definition and differentiation of terms, and the need to determine speech and nonspeech variables contributing to reductions in intelligibility. Relevant literature on intelligibility from fields outside cleft lip and palate is reviewed, and a number of recommendations are made regarding the measurement of intelligibility in speakers with cleft lip and palate.
Facial Appearance and Attachment in Infants with Orofacial Clefts: A Replication
Authors: Coy Katherine , Speltz Matthew L. , Jones Karen 
Published: October 2018

Objective The current study sought to replicate an unexpected finding reported by Speltz et al. (1997): relatively unattractive infants at age 3 months were more likely than attractive infants to show secure maternal attachment at age 12 months, a finding unaffected by the diagnosis of cleft lip and palate (CLP), cleft palate only (CPO), or the absence of a cleft condition. Design We evaluated the effects of diagnosis (CLP, CPO, or no diagnosis) and age (3, 12, and 24 months) on facial attractiveness ratings derived from a modified Q-sort method. Setting Craniofacial clinic in an urban children's hospital. Participants Infants with CLP and CPO and typically developing infants without clefts (n = 126) and their mothers. Ratings were made by 13 adults unfamiliar with cleft conditions. Main outcome Measures Facial attractiveness ratings. Results Infants with CLP were rated as the least attractive at all time points. At ages 12 and 24 months, infants in the CPO group were rated as less attractive than typical infants but more attractive than infants in the CLP group. Typical infants and those with CLP—utnot CPO—received higher attractiveness ratings with age. As hypothesized, less attractive infants, regardless of diagnosis, were more likely to show secure attachment than were more attractive infants. Conclusions We tentatively conclude that the perceived vulnerability of young infants, as indexed by atypical or unattractive facial characteristics, engenders extraordinary protectiveness and responsiveness in some mothers, leading to a higher probability of secure attachment. A test of this hypothesis with a new sample of infants is recommended.
Cephalometric Analysis in Submucous Cleft Palate: Comparison of Cephalometric Data Obtained from Submucous Cleft Palate Patients with Velopharyngeal Competence and Incompetence
Authors: Park Susam , Omori Makoto , Kato Kogo , Nitta Naotugu , Kitano Ichiko , Masuda Tomomi 
Published: October 2018

Objective The purpose of this study was to investigate the relationship between craniofacial and nasopharyngeal morphology and velopharyngeal function in submucous cleft palate. Design and Patients Fifty-two lateral cephalometric radiographs of 46 sub-mucous cleft palate (SMCP) patients with velopharyngeal competence (24 patients) and incompetence (22 patients) at 4 and 7 years of age were studied. The patients had not received any surgical or orthodontic treatment prior to cephalography being performed. Results Significant differences were found between cephalometric variables (N-Ba, N-S-Ba angle) in children with velopharyngeal competence and incompetence. However, the results of our study showed that cephalometric data alone are not useful for predicting velopharyngeal function and can not serve as an absolute prognostic indicator. Conclusion There are many factors that can influence velopharyngeal function in SMCP patients. Cephalometric data did not demonstrate a strong relationship to velopharyngeal function.
Neonatal Permanent Jaw Constriction Because of Oral Synechiae and Pierre Robin Sequence in a Child with van der Woude Syndrome
Authors: Denion E. , Capon N. , Martinot V. , Pellerin P. 
Published: October 2018

Objective To report a newborn with van der Woude syndrome, Pierre Robin sequence, and oral synechiae. Pierre Robin sequence is a rare manifestation of van der Woude syndrome as are oral synechiae. We speculate that the oral synechiae may be causally related to the development of Pierre Robin sequence in this patient.
A Cephalometric Study of Facial Growth in van der Woude Syndrome
Authors: Kane Alex A. , Liao Yu-Fang , Lo Lun-Jou , Huang Chiung-Shing , Huang Li-Min , Chen Yu-Ray , Noordhoff M. Samuel 
Published: October 2018

Objective To test the hypothesis that maxillary growth and lower lip form in patients with van der Woude syndrome (VDW) is different from patients with nonsyndromic cleft lip and palate. Design and Setting Retrospective, case-control study at a tertiary cleft center, Chang Gung Memorial Hospital, Taipei, Taiwan. Patients and Participants Records of 53 patients with VDW, who presented for treatment during the years 1968 through 1998 were obtained. Twenty-three of 53 patients had received at least one lateral cephalogram during the course of their treatment. Of these 23, in 17 it was possible to find nonsyndromic case controls with identical cleft type, sex, and method of cleft palate repair, with year of birth matching within 1 year of the corresponding VDW patient. For these 17 pairs of VDW and nonsyndromic cleft controls, cephalogram acquisition dates were checked to see how well the corresponding pairs matched. A total of 43 pairs of cephalograms were deemed to be acceptably matched because the ages at acquisition differed by less than 15% of the VDW patient's age. Main outcome Measures Thirteen measurements were derived from the 11 standard lateral cephalometric landmarks recorded on each cephalogram. The data were classified into five groups according to age at time of cephalogram, and sets of paired nonsyndromic cleft and VDW measurements were tested for differences using a Wilcoxon signed rank sum test in two ways, first including all cleft types and then including only those patients with complete bilateral cleft lip and palate. A longitudinal growth analysis considering the movement of the skeletal A and B points was performed on the patients with complete bilateral cleft lip and palate. Results For the osseous measurements, anteroposterior maxillary length as described by the anterior nasal spine (ANS)-posterior maxillary point distance was statistically significantly shorter in the VDW patients of age 13 years and older, by 5.3 mm. Maxillary height, as described by the nasion-ANS distance was shorter in the VDW patients, closely approaching statistical significance in the age range 7 through 11 years. The lip soft tissue measurements showed significantly greater protrusion over several age ranges in the VDW patients. The longitudinal growth analysis showed a significantly more inferior vertical position of the B point in the controls. Conclusions This study demonstrates a few statistically significant differences in maxillary growth and lip conformation between VDW and matching controls. Small sample sizes for each age group hamper the ability to fully interpret or generalize the pattern of these differences.
Clinical and Biochemical Findings of a Patient with Thanatophoric Dysplasia Type I: Additional Finding of Dicarboxylic Aciduria
Authors: Okajima Kazuki , Asai Kiyofumi , Niwa Toshimitsu , Ohki Shigeru , Sobajima Hisanori , Tyson Jess , Malcolm Sue , Wada Yoshiro 
Published: October 2018

Objective A long-surviving thanatophoric dysplasia type I patient to age of 6 years is presented. Results and Conclusions Molecular studies revealed a heterozygous point mutation, S249C in the fibroblast growth factor receptor 3 gene. Most of the clinical course was similar to previous reports, including hearing loss and acanthosis nigricans. Abnormal urinary excretion of dicarboxylic acids and 3-hydroxydicarboxylic acids was observed. We hypothesize that this was a consequence of the FGFR3 mutation.
Palate Height: Another Indicator of Surgical Outcome in Unilateral Cleft Lip and Palate?
Authors: Kharbanda Om P. , Shaw William C. , Worthington Helen 
Published: October 2018

Objective: To determine whether palate height and maxillary arch depth are systematically related to the surgical center at which primary repair in unilateral cleft lip and palate (UCLP) was carried out. Design: A retrospective comparison based on study casts of consecutive cases of UCLP obtained at age 9 years from six different centers. The observer who conducted measurements was blinded to the source of individual records. Setting: The patients whose records were analyzed received all their surgical care in a national health service setting in six different northern European centers and regions. Patients: Patients were consecutively treated Caucasian children with non-syndromic complete UCLP born in the period 1976 to 1979. Main Outcome Measures: The main outcome measures for the original study were craniofacial form, dental arch relationships, nasolabial appearance, and speech. This report focuses on measurements of anterior maxillary arch depth and palate height. Results: Anterior arch depth and anterior palate height showed some variation among the centers. There was a tendency for anterior arch depth and palate height to also be reduced at centers at which patients showed unfavorable dental arch relationships. Conclusions: Anterior arch depth and palate height might be considered in future studies of surgical outcome and in their possible relationship to problems of articulation.
Isolated Soft Tissue Cleft Lip: The Influence on the Nasal Cavity and Supernumerary Laterals
Authors: Hansen Ken , Mehdinia Maryam 
Published: October 2018

Objective: The purpose of this investigation was to estimate the vertical deviation of the nasal floor and to register dental disturbances (i.e., hypo- and hyperdontia) in patients with an isolated soft tissue cleft lip. Design: Thirty patients with isolated cleft lips were randomly selected from the files of the Department of Maxillo-Facial Surgery and Jaw Orthopedics. As a matched control group, 30 patients were selected from the files of the Department of Orthodontics. Panoramic radiographs were analyzed and the lowest point of the floor of the left and right nasal cavity was measured to the nearest 0.5 mm. The presence of supernumerary (deciduous or permanent) or missing lateral incisors was recorded from the patient's journals. Results: The vertical position of the nasal floor differed significantly between the cleft and the noncleft side (p <0.001). Supernumerary, deciduous, and permanent laterals were recorded in 73% of the subjects in the cleft lip group (22 patients). In the control group, no differences were found between the position of the nasal floor on the left and right side and no supernumerary laterals were recorded. Missing laterals were not noted in any of the individuals investigated. Conclusions: Isolated cleft lips were in most cases associated with: (1) lowering of the nasal floor on the cleft side and (2) supernumerary primary or permanent lateral incisors on the cleft side. Panoramic radiographs can be used to detect differences in the vertical position of the floor of the nasal cavity in patients with isolated cleft lips and to diagnose supernumerary laterals.
Visualization of Individual Growth-Related Craniofacial Changes Based on Cephalometric Landmark Data: A Pilot Study
Authors: Lux Christopher J. , Starke Jens , Rübel Jan , Stellzig Angelika , Komposch Gerda 
Published: October 2018

Objective: An approach based on Euclidean distances between cephalometric landmarks is presented (1) to visualize and localize the individual shape changes of the complex craniofacial skeleton during growth and (2) to depict the individual dynamic behavior of developmental size and shape changes. Patients and Method: Growth-related craniofacial changes were investigated exemplarily for two male orthodontically untreated subjects from the Belfast Growth Study on the basis of lateral cephalograms at 7, 9, 11, 13, and 15 years. The interlandmark distances among seven skeletal cephalometric landmarks served as a database for the study. A modified Karhunen-Loèvedecomposition based on orthogonal modes and time-dependent scalar amplitudes was used to describe the growth process. The individual shape changes of the various craniofacial regions were visualized by allocation of colors to the respective distances, and overdrawn representations were reconstructed by means of multidimensional scaling. Results and Conclusions: This visualization technique allows anatomical regions to be characterized with respect to reduced or strengthened growth, compared with pure size changes. The clinically relevant mechanisms of craniofacial changes are visualized (e.g., shifts in the anteroposterior or vertical dimensions of the jaws in relation to cranial base and structural imbalances during development). In addition, overdrawing the effects of shape change on the skeletal structures gives a more readily comprehensible impression of the growth process. Taking account of the methodical limitations of this approach (e.g., the restrictions concerning the number of landmarks), the clinician may take advantage of this technique in orthodontic or surgical diagnostics to gain additional insight into the individual complex size and shape changes during development along with their dynamic behavior.
Speech Outcomes in Isolated Cleft Palate: Impact of Cleft Extent and Additional Malformations
Authors: Persson Christina , Elander Anna , Lohmander-Agerskov Anette , Söderpalm Ewa 
Published: October 2018

Objective The purpose of the study was to study the speech outcome in a series of 5-year-old children born with an isolated cleft palate and compare the speech with that of noncleft children and to study the impact of cleft extent and additional malformation on the speech outcome. Design A cross-sectional retrospective study. Setting A university hospital serving a population of 1.5 million inhabitants. Subjects Fifty-one patients with an isolated cleft palate; 22 of these had additional malformations. Thirteen noncleft children served as a reference group. Interventions A primary soft palate repair at a mean of 8 months of age and a hard palate closure at a mean age of 4 years and 2 months if the cleft extended into the hard palate. Main outcome Measures Perceptual judgment of seven speech variables assessed on a five-point scale by three experienced speech pathologists. Results The cleft palate group had significantly higher frequency of speech symptoms related to velopharyngeal function than the reference group. There were, however, no significant differences in speech outcome between the subgroup with a nonsyndromic cleft and the reference group. Cleft extent had a significant impact on the variable retracted oral articulation while the presence of additional malformations had a significant impact on several variables related to velopharyngeal function and articulation errors. Conclusion Children with a cleft in the soft palate only, with no additional malformations, had satisfactory speech, while children with a cleft palate accompanied by additional malformations or as a part of a syndrome should be considered to be at risk for speech problems.
Constriction of the Maxillary Dental Arch by Mucoperiosteal Denudation of the Palate
Authors: Kim Takenori , Ishikawa Hiroyuki , Chu Soowon , Handa Asako , Iida Junichiro , Yoshida Shigemitsu 
Published: October 2018

Objective This study examined the influence of two factors in the constriction of the maxillary dental arch by mucoperiosteal denudation of the palate: (1) inhibition of lateral growth and (2) medial inclination of teeth. Method Thirty-five male 20-day-old Wistar rats were divided into experimental and control groups. The experimental group had bilateral mucoperiosteum excised in the lateral one third of the palate. Methyl methacrylate resin–embedded frontal sections were prepared from both groups after alternate weekly injections of tetracycline and calcein in the dorsal subcutaneous area. The sections were observed and photographed under either a confocal laser scanning microscope, a fluorescence microscope, or both. Chronological changes in lateral palatal growth, maxillary dental arch width, and inclination of the upper first molars were examined up to 8 weeks after the operation. Paraffin-embedded frontal sections were also made and stained with Elastica van Gieson stain. Results The scar tissue formed on the rat palate by the mucoperiosteal denudation was tightly connected to the palatal bone and teeth. The intervals between the labeling lines of the experimental group were less definite during the first 2 weeks after the operation. Increments of palatal and maxillary dental arch widths were smaller in the experimental group than in the control group. The upper first molars in the control group gradually inclined laterally, whereas those in the experimental group inclined medially with age. Conclusion Medial inclination of teeth is a stronger influence than inhibition of lateral growth on constriction of the rat maxillary dental arch.