bluuebunny/crossref_metadata_2025 huggingface dataset:
Alterations in Vascular Pattern of the Developing Palate in Normal and Spontaneous Cleft Palate Mouse Embryos
Authors: Amin Nurul , Ohashi Yasushi , Chiba Junichi , Yoshida Shigemitsu , Takano Yoshiro
Published: October 2018

The alterations in the vascularization of the developing palate were studied through the use of resin cast images of vascular networks in normal and spontaneous cleft lip and palate (CL/Fr) mouse embryos (crown rump [CR] Length 9-18 mm, body wt. 0.11-0.65 g). A more dense vascular plexus was observed in the oral side than in the nasal side before the reorientation of the palatal shelves. Prominent terminal dilatations of the vascular plexus were observed in the extreme medial edge of the elevating palatal shelves which may exert significant force for palatal shelf elevation. The vascular plexus showed a lateral extension. Many small spherical masses of resin were observed in the medial edge of the palatal shelves at the time of medial extension and during fusion, indicating some changes occurring in the capillary wall leading to the resin leakage. In the spontaneous cleft group, a similar vascular pattern was observed, but the greater palatine artery showed discontinuity in the premaxillary region at an early stage. At the same time, terminal dilatations were delayed and frequently absent in the contralateral shelf. After the reorientation of the palatal shelves, the vascular plexus formed an irregular lattice pattern. Dilated vasculature was apparent in the anterior nasopalatine region, indicating the persistence of a more primitive vascular structure in the spontaneous cleft embryos. However, the area where capillary dilatation occurred was different between unilateral and bilateral cleft embryos. In conclusion, blood vessels in the palatal shelves are underdeveloped and remained immature in cleft lip and palate embryos and showed different patterns in the anterior nasopalatine region between unilateral and bilateral cleft embryos. These variant vascular patterns may be due to the inadequate blood supply to the nasopalatine region from the early embryonic stages possibly resulting from the discontinuity of the greater palatine artery.
Measurement of Velopharyngeal Closure Force during Vowel Production
Authors: Moon Jerald B. , Kuehn David P. , Huisman Jessica J.
Published: October 2018

The purposes of this study were to (a) design and test a new velopharyngeal closure force sensing bulb, and (b) use the closure force bulb to gather additional information on the variations in closure force associated with different vowels. The closure force sensing bulb possessed a flat frequency response to 30 Hz. Its output was highly linear relative to applied gram force. Reliable placement of the bulb in human subjects was achieved following prescribed placement criteria. The bulb was sensitive to small variations in velopharyngeal closure force. In agreement with some previous reports, high vowels were associated with greater velopharyngeal closure forces than low vowels. The results of this Investigation support the notion that articulatory goals are specified for vowels. This vowel specificity was observed during production in both isolation and in context.
Cleft Palate with Autosomal Recessive Transmission in Brittany Spaniels
Authors: Richtsmeier Joan T. , Sack George H. , Grausz Hannah M. , Cork Linda C.
Published: October 2018

In the course of maintaining a large colony of Brittany spaniels for studying a dominantly inherited motor neuron trait, cases of sporadic complete cleft palate were observed. Without intervention, the pups with cleft palate that attempt to nurse, aspirate and die. In this study, we report on the incidence of cleft palate in this dog kindred, describe the gross morphologic characteristics of the cleft, and present a morphometric analysis of the skull of two of the cleft palate pups and one unaffected pup that died at birth. Our data thus far Indicate 26.9% incidence of cleft palate in the colony. Pedigree analysis indicates that this cleft palate trait Is Inherited as an autosomal recessive. High resolution computed tomography scans of the pup heads were used in morphometric comparison of normal and cleft palate pups. We found specific morphologic differences between the cranial base and palate of normal and cleft palate pups. Plans for future studies of the genetics and growth and development of this animal model are discussed. This canine cleft palate trait provides an ideal model for studying a malformation common in humans.
Dentofacial Form in the Five-Year-Old Child with Unilateral Cleft Lip and Palate
Authors: Mackay Frances , Bottomley John , Semb Gunvor , Roberts Christopher
Published: October 2018

It is generally believed that studies of outcome for children with clefts of the lip and palate should be based on patients who are in their teens. This means that health care workers who look after these children would have to wait many years until the quality of treatment could be evaluated. In this study, significant differences between two centers, Oslo and Manchester, in facial form at the age of 5 years were detected. Based on cephalometric analysis, children from Manchester were more likely to have a retrognathic maxilla with the upper lip significantly behind the esthetic plane. An important future step may be the setting up of “reference centers” with a large archive of database information for each racial group. This would assist smaller centers in comparing their outcomes.
Palatal Changes after Lip Surgery in Different Types of Cleft Lip and Palate
Authors: Kramer Gem J.C. , Hoeksma Jan B. , Prahl-Andersen Birte
Published: October 2018

This study concerns palatal development during 6 months following primary lip closure. The sample consisted of 75 children with different forms of cleft lip and palate and 51 noneleft children. The palate was measured at 3 months of age, just before lip surgery, after surgery at 6 months, and again at 9 months of age. The results showed that lip closure has a strong effect in the anterior alveolar region. This effect was restricted to 3 months after surgery. The changes in complete clefts were more explicit than in incomplete cleft forms. Furthermore, the data showed that arch depth reduction due to lip surgery was compensated for by continued anteroposterior palatal growth. Early orthopedics appeared to prevent major palatal collapse immediately after lip surgery. Finally simultaneous closure of the alveolar cleft at the nasal side resulted in continued reduction of anterior cleft width.
Orodigitofacial Syndromes Type I and II: Clinical and Surgical Studies
Authors: Martinot V.L. , Manouvrier S. , Anastassov Y. , Ribiere J. , Pellerin P.N.
Published: October 2018

Eleven patients were followed at the multidisciplinary facial cleft department from 1963 to 1993. Nine had orodigitofacial (ODF) I syndrome (Papillon-Leage, 1954) and two had ODF II syndrome (Mohr, 1941). The authors observed seven median clefts of the upper lip, eight atypical cleft palates, nine lingual tumors, ten polylobed tongues, four ankyloglossla. All vestibular frenula were hypertrophied. Facial analysis showed five facial hypertelorisms, eight epicanthal folds, ten anomalies of the forehead. Nasal anomalies were constant with ten alar hypoplasia and four nasal curves. Syndactyly was the most frequent anomaly of the hand. Seven patients were mentally retarded. Transmission of ODF I Is by X-linked dominant variable expression; ODF (I is autosomal recessive. The difference in transmission means a precise diagnosis is necessary. Bilateral duplication of the great toe is characteristic of type II, but not pathognomonic. Hair and cutaneous anomalies suggest type I. The authors proposed a surgical treatment involving three operating steps.
Phonetic Features by Bables with Unilateral Cleft Lip and Palate
Authors: O'gara Mary M. , Logemann Jeri A. , Rademaker Alfred W.
Published: October 2018

Twenty-three babies with nonsyndromlc unilateral cleft lip and palate were audiotaped at regular intervals from 5 to 35 months of age. Narrow phonetic transcription of their comfort-state vocalizations and word approximations was accomplished to describe phonetic development over time and according to the nonrandomized age of palatoplasty. The babies that had earlier palatal repair produced significantly higher percentages of oral stops after 12 months of age than babies with similar clefts that had later palatal repair. No significant differences are evident, however, according to age of palatoplasty, for mean frequency use of oral fricatives up to 3 years of age. For all 23 babies, regardless of the age of palatoplasty intervention, time is an even stronger variable than age of palatoplasty for development of palatal, alveolar and velar place features, oral stops, and oral fricatives.
Parent Questionnaire for Screening Early Language Development in Children with Cleft Palate
Authors: Scherer Nancy J. , D'antonio Linda L.
Published: October 2018

This study investigated the efficacy of a parent questionnaire as a component for screening early language development of children 16 to 30 months of age with cleft lip and palate. Thirty nonsyndromic children with cleft lip and palate and 30 children without clefts received the MacArthur Communicative Development Inventory: Toddler (CDI: Toddler), administered by a pediatrician. In addition, a speech-language screening was performed by a speech-language pathologist. Results of the two assessments indicated that the CDI: Toddler was a valid screener of language development when compared with a comprehensive speech-language screening. Language and speech characteristics of the subject populations are discussed. In particular, differences between the cleft and noncleft groups demonstrated evidence of delays in expressive language development in the children with cleft lip and palate.
Effects of Carbon Monoxide and Hypoxia on Cleft Lip in A/J Mice
Authors: Bailey L'tanya J. , Johnston Malcolm C. , Billet Josephus
Published: October 2018

Epidemiologic evidence indicates an increase in cleft lip with or without cleft palate [CL(P)] in infants of mothers who smoke cigarettes. It appears that the principle mechanism is through carbon monoxide (CO) decreasing the oxygen (O2) available to the embryo. Previous studies have shown that maternal respiratory hypoxia can increase the incidence of CL(P) in mice. The present investigation was designed to analyze the effects of altered levels of CO and O2 in respiratory gases on the incidence of CL(P) in genetically susceptible A/J mice. Results from blood gas analysis, after a 24-hour exposure of pregnant mice during the time of primary palate development, showed that CO levels of 180 ppm in air decreased oxyhemoglobin (%O2Hb) and increased carboxyhemoglobin (%COHb) to slightly above the high end of the range found in human studies of cigarette smokers. Interestingly, the control COHb levels were higher in our CL(P) sensitive mouse strain compared with those of the range of increases found in human smokers, versus nonsmoker studies, and that the increase for treated mice (3x) was at the low end of the range for smokers. Decreasing O2 levels to 10% from 21% (normal percentage in air) more severely decreased %O2Hb and moderately decreased %COHb. At 24 hours of exposure, the incidence of CL(P) and resorption was approximately the same for both the CO and the control groups, but there were significant increases in the incidence of resorptions in the hypoxia group and of CL(P) in relation to the CO group. A statistically significant increase in CL(P), but not resorptions was found for a longer CO treatment time (48 hours vs. 24 hours of exposure). Also, an increase in the severity of clefting was noted with the carbon monoxide treated groups having more bilateral CL(P) than unilateral CL(P). The low incidences of CL(P) in the control groups prevented statistical analysis, although results from other studies indicate that the increased severity is real. CO exposure longer than 48 hours seems to give no additional increase in clefting incidence. We conclude that chronic exposure to moderate levels of CO increases the incidence and severity of CL(P) in A/J mice. The possible additional effects of nicotine in cigarette smoke and of other confounding factors related to smoking and CL(P) are discussed.
Cranio-Orbito-Zygomatic Measurements from Standard CT Scans in Unoperated Treacher Collins Syndrome Patients: Comparison with Normal Controls
Authors: Posnick Jeffrey C. , Al-Qattan Mohammad M. , Moffat Stephanie M. , Armstrong Derek
Published: October 2018

Fourteen reproducible cranio-orbito-zygomatic measurements taken from 26 standard axial computed tomographic (CT) scans of unoperated individuals with symmetric forms of Treacher Collins syndrome (TCS) were compared to age-matched controls. The interorbital measurements of the TCS patients were at the mean when compared to their cohort group (medial and lateral orbital wall separation), while the zygomatic measurements were significantly less than normal confirming the extent of malar hypoplasia. The congenitally deficient lateral aspect of the orbits in TCS patients was confirmed by the greater than normal values measured for globe protrusion and medial orbital wall protrusion in conjunction with the diminished lateral orbital wall lengths, all of which use the lateral orbital rim as a reference point. The abnormal shape of the anterior cranial vault in patients with TCS was documented as a diminished intercoronal distance (width) and decreased cephalic length when compared to normal age-matched controls.
Hearing Levels in Pierre Robin Syndrome
Authors: Handžić Jadranka , Bagatin Marijo , Subotić Radovan , Ćuk Višeslay
Published: October 2018

Hearing was tested in 18 patients with Pierre Robin syndrome (PRS). These results were compared with those obtained for 243 patients with either cleft lip or cleft palate, or both. None of the PRS group patients had middle ear or inner ear malformations, or sensorineural hearing loss in speech frequencies. Hearing loss in PRS is usually conductive, bilateral, and more frequent in PRS patients (30 ears or 83.33%) than in patients who do not have PRS (290 ears or 59.67%). A significantly higher (p < .01) mean of hearing loss for air conduction in speech frequencies (MHLSF = 24.5 dB) was found in PRS patients than in patients without PRS (MHLSF = 17.8 dB). The ears of the PRS patients with hearing loss were examined, revealing middle ear effusion. In all cases, hearing was restored to a normal level through suction and the use of ventilation tubes.
HONC Measures in Men and Women: Validity and Variability
Authors: Sussman Joan E.
Published: October 2018

Horii's (1980) Oral Nasal Coupling index (HONC) was used in measures of 20 women and 20 men with normal speech to validate procedures used for assessment of disordered nasal resonance. Subjects produced sustained vowels [l] and [a], repeated single word productions of “baby” and “mamie,” and nonnasal and nasal sentences. Results showed that the correction factors generated during [m] calibration procedures differed significantly between women and men, but not over time within the single measurement session. Differences were also found for the decibel levels produced in the voice channel during the [m] calibration procedure: the women used higher dB levels, particularly towards the end of the session, than the men. In addition, dB levels differed over time. Differences of 13 dB (HONC) were found to separate nonnasal from nasal sentences supporting the validity of the HONC measure. Smaller differences were found between sustained vowel and repeated single words and nasal sentences. Greater variability found for vowel productions also suggests that the sentence stimuli may be more effective for demonstrating hyper- or hyponasality.
The Role of Maternal Factors in the Adaptation of Children with Craniofacial Disfigurement
Authors: Campis Leslie Biron , Demaso David Ray , Twente Allison White
Published: October 2018

This study hypothesized that maternal adjustment, perceptions, and social support would better predict child adaptation to craniofacial disfigurement than medical severity. Mothers of 77 children (ages 6-12) completed the Child Behavior Checklist, Beck Depression Inventory, Spielberger Trait Anxiety Scale, Social Support Questionnaire Revised, and Parenting Stress Index. Medical severity was assessed by the number of operations (craniofacial and other), comorbid medical conditions, and the Hay Attractiveness Scale. The children and mothers in our sample resembled a normal population in terms of their psychological functioning and quality of the mother-child relationships. Maternal adjustment and maternal perceptions of the mother-child relationship were more potent predictors of children's emotional adjustment than either medical severity or maternal social support.
Pfeiffer Syndrome: A Clinical Review
Authors: Moore Mark H. , Cantrell Stephen B. , Trott James A. , David David J.
Published: October 2018

The combination of bicoronal craniosynostosis, broad thumbs and great toes, and partial variable soft tissue syndactyly of the hands and feet (i.e., Pfeiffer syndrome) classically followed a benign clinical course. A review of the clinical features of those Pfeiffer syndrome patients presenting to our unit confirm another subgroup in whom the craniofacial and associated manifestations are more extreme, with a significant risk of early demise. The early aggressive surgical management of craniostenosis, hydrocephalus, exorbitism, faciostenosis, and upper airway obstruction has provided the potential for prolonged useful survival in these cases.
Planning Mandibular Distraction: Preliminary Report
Authors: Losken H. Wolfgang , Patterson Gary T. , Lazarou Spiros A. , Whitney Timothy
Published: October 2018

Normal lengths of the vertical ramus, body, and angle of the mandible at different ages are presented. Before mandibular distraction is embarked on, the extent of the deficiency of the mandible is assessed. The length of the vertical ramus and body are measured on cephalometric radiographs or three-dimensional computed tomography (3D CT) scans. Deficiency of the length of the mandible is calculated. The position of the pin placement angle (from the horizontal ramus) is calculated by means of the following formula: 180 degrees minus mandibular angle times vertical ramus deficiency divided by total deficiency. Placing the pins correctly will result in correction of the vertical ramus and body deficiency of the mandible and the excessively obtuse angle of the mandible will become more acute.
Kabuki Syndrome: Underdiagnosed Recognizable Pattern in Cleft Palate Patients
Authors: Burke Leah W. , Jones Marilyn C.
Published: October 2018

Eight cases of Kabuki syndrome in non-Japanese patients are presented. (Although the name Kabuki Make-up syndrome has been used to identify this condition, the authors recommend that the term “make-up” be deleted from the designation as it has caused problems with some of the families.) Clinical features include a characteristic facies, developmental delay, musculoskeletal abnormalities, and dermatoglyphic differences. The phenotype appears to evolve over time making the diagnosis difficult in infancy. The progressive changes in the facial characteristics as well as the musculoskeletal problems suggest an underlying defect of the connective tissue. Finally, the syndrome appears to be more common in the non-Japanese population than previously appreciated, particularly in the cleft palate population.
Psychological Interventions for the Adolescent with Cleft Lip and Palate
Authors: Kapp-Simon Kathleen A.
Published: October 2018

The normal developmental tasks of adolescence include individuation from family, development of sense of personal identity, and establishment of satisfactory peer relationships. Accomplishment of each of these tasks is potentially more difficult for the adolescent who is also coping with the ongoing treatment demands of cleft lip and palate or other craniofacial disorders (CFA). This article presents treatment strategies that can be used by multidisciplinary teams to assist the adolescent with CFA in mastering age appropriate developmental tasks.
Children's Attitudes to Physical Differences among Youth from Western and Non-Western Cultures
Authors: Harper Dennis C.
Published: October 2018

Children with visible physical impairments (cosmetic and orthopedic) are known to experience difficulties in social interaction with peers. Some of these social difficulties are related to attitudes toward visible physical features of children. Several research studies from Western and non-Western countries are reviewed, focusing on children's attitudes toward children with facial disfigurement. Specific social preferences for particular disabilities are noted which are related to cultural and economic factors. Children's attitudes toward facial disfigurement reflect generally low preference for social interaction in the majority of cultures studied. Children's attitudes reflect both positive and negative attributions toward facial disfigurement.
Speech Outcomes in Adolescents with Cleft Lip and Palate
Authors: Peterson-Falzone Sally J.
Published: October 2018

Speech results were surveyed in 110 adolescent patients with clefts: 53 with unilateral cleft lip and palate, 46 with bilateral cleft lip and palate, and 11 with isolated clefts of the secondary palate. Only 12 of the 110 teenagers had received consistent team care from infancy. The early physical management was impossible to reliably determine in the remaining 98. Speech was normal in 22.7%, characterized by a variety of problems in approximately 66%, and a complete habilitative failure in 10.9%. Cleft palate and craniofacial teams who first encounter incompletely managed cases in the teenage years are faced with complex and interrelated challenges of providing appropriate physical management, speech habilitation, and psychosocial support, including determination of that approach which will be most likely to assure future compliance with treatment recommendations to each child and family.
Nasometric Discrimination of Hypernasality and Turbulent Nasal Airflow
Authors: Karnell Michael P.
Published: October 2018

Agreement between nasalance measures and perception of nasality during speech is not consistently strong. A possible reason may be the complicating combined effects of nasal turbulent airflow and nasal resonance. The purpose of this preliminary study was to examine nasalance measures obtained during production of a low pressure speech sample designed to minimize or eliminate the effects of turbulent nasal airflow. The results showed that nasalance measures obtained as some of the subjects produced the new speech sample were significantly different from those obtained when the standard high pressure speech sample was used. Use of both the new low pressure samples and the standard high pressure samples may result in improved agreement between nasalance measures and ratings of speech nasality as well as assist in the identification of subgroups of patients with marginal velopharyngeal insufficiency.
Tooth Malalignments in Chilean Children with down Syndrome
Authors: Ondarza Alejandro , Jara L. , Bertonati M.I. , Blanco Rafael
Published: October 2018

The present study analyzes the frequencies and types of anomalies In tooth alignment in a sample of 136 children with Down syndrome, 147 mentally-Impaired individuals without Down syndrome, and 149 normal individuals. Patients with Down syndrome showed a higher frequency of malalignments in both the deciduous and permanent dentitions compared with the children in control groups. In the three groups studied, the frequency of malalignments was higher in the permanent than in the deciduous dentition. In the deciduous dentition, the frequency of malalignments in the three groups was similar in the maxilla and mandible, and in both boys and girls. In the permanent dentition, the frequency of malalignments was higher in Down and mentally-Impaired girls without Down syndrome, while the frequency of malalignments in the mandible was only Increased in mentally-impaired individuals who did not have Down syndrome. In the deciduous dentition, the Down group presented a higher frequency of malalignment in the upper central incisor, lateral incisor, and canine regions compared with the normal children. When comparing teeth of Down children with those of mentally-impaired individuals who did not have Down syndrome, differences in malalignment were observed only in upper central incisor and canine regions. In the permanent dentition, the Down group showed a higher number of tooth malalignments than the normals (13 out of 28 teeth). A comparison of Down with non-Down mentally-impaired individuals, revealed only 8 teeth out of 28 were different. The most frequent malalignments in the deciduous dentition in Down patients were mesiopalatal, mesiolingual, and mesiovestibular. In the permanent dentition, the most frequent malalignments were distopalatal or distolingual.
Growth of the Facial Skeleton following the Malek Repair for Unilateral Cleft Lip and Palate
Authors: Bruce Ross R.
Published: October 2018

Variations in the surgical procedures used to repair a complete unilateral cleft lip and palate have differing effects on growth of the facial skeleton. In this study, cephalometric radiographs of the cases of Professor Rene Malek were analyzed. The sample consisted of 35 male patients with complete unilateral cleft lip and palate at a mean age of 10.1 years. Previous data from the cases of Dr. W.K. Lindsay in Ross (1987). Cephalometric data were compared with our sample. Results showed that the Malek protocol for surgical repair, as performed by Malek, produces excellent midface growth at 10 years of age.
Pharyngeal Flaps after One- and Two-stage Repair of the Cleft Palate: A 25-Year Review of 520 Patients
Authors: Vedung Sigfrid
Published: October 2018

Between 1962 and 1976, a one-stage surgical procedure was performed on 328 cases of cleft lip and palate or isolated cleft palate. From 1977 to 1986, a two-stage surgical procedure was performed on 192 patients. After one-stage repair, 22 (6.7%) required a pharyngeal flap at the age of 6 years, and 13 (7%) required a flap after two-stage repair. When the patients were 14 years or older, 56 (17%) needed a flap after one-stage repair, and 14 (24%) required a flap after two-stage repair. Two-stage repair at 24 months and 5.2 years resulted in more patients in need of pharyngeal flaps than those who had repair at 12 to 18 months and 3 years. In both groups, at age 10, the incidence of flaps was approximately 20% after closure of isolated cleft palate involving the hard palate, with 11 to 12% in patients with unilateral cleft lip and palate (UCLP) or clefts of the soft palate only, and 14 to 16% in patients with bilateral cleft lip and palate (BCLP). The pharyngeal flap was more often used in girls than in boys, especially in cases with CP only. Approximately 85% of the flaps were carried out before the age of 10 years, and only a few flaps may have been related to involution of the adenoid. The incidence of fistulas was higher after one-stage repair of cleft lip and palate (CLP) (p < .05) and lower in patients with CP only (p < .05), compared with the results after two-stage repair. Pharyngeal flaps were slightly more common after two-stage repair. The possible advantages of two-stage repair in relationship to maxillary growth, which may justify the second operation, will be investigated when the patients are older.
Spinal Anomalies in Pfeiffer Syndrome
Authors: Moore Mark H. , Lodge Michelle L. , Clark Bruce E.
Published: October 2018

Review of the spinal radiographs of a consecutive series of 11 patients with Pfeiffer syndrome presenting to the Australian Craniofacial Unit was performed. The prevalence of cervical spine fusions was high, and the pattern of fusion complex. Isolated anomalies were evident at lower levels, including two cases of sacrococcygeal eversion. Spinal anomalies occur more frequently in the more severely involved cases of Pfeiffer syndrome emphasizing the generalized dysostotic nature of this condition.
Poly-(L-Lactic) Acid Membranes in Palatal Surgery in Beagle Dogs: Clinical and Histologic Evaluation
Authors: In De Braekt Marcel M.H. , Maltha Jaap C. , Kuijpers-Jagtman Anne M.
Published: October 2018

This article reports on the histologic findings from a larger study that was designed to investigate whether the attachment of scar tissue to underlying bone, which is normally found after palatal surgery, can be prevented by using biodegradable poly-(L-lactic) acid membranes. Von Langenbeck's procedure was simulated in 12-week-old beagle dogs without clefts. In one group normal wound healing was allowed. In two groups, membranes were inserted immediately after surgery or 3 weeks thereafter. Sham and control groups were also included. Histologic evaluation was carried out at regular intervals. Reports have been published on other aspects, such as clinical wound healing, contraction and maxillary arch development in beagle dogs following this treatment. After direct implantation of membranes, wound healing was retarded. Disintegration of the membranes started soon after implantation and remaining particles were surrounded by a fibroblastic sheath and a fibrous capsule. At sites where membrane particles persisted, attachment of the scar tissue to the underlying bone by Sharpey's fibers was prevented.
Axial Configuration of the Velopharyngeal Valve and Its Valving Mechanism
Authors: Finkelstein Yehuda , Shapiro-Feinberg Myra , Talmi Yoav P. , Nachmani Ariela , Derowe Ari , Ophir Dov
Published: October 2018

The variability of the normal velopharyngeal (VP) closure mechanism was studied by investigating VP anatomy in relation to its closure mechanism in 60 patients. The axial configuration of the VP isthmus, as observed in axial CT scans at rest, was found to be correlated with VP function in terms of its closure patterns in speech as observed by nasendoscopy. A flat VP isthmus was found to be closed mainly in the anteroposterior direction, forming the coronal closure pattern. A deep VP isthmus is closed by movement of the velum and medial movement of the lateral pharyngeal walls, forming the circular closure pattern. A flat VP isthmus occurs when the hamuli are posteriorly located and the muscular slings, therefore, open more posteriorly. We conclude that posterior insertion of the velar muscles on to the skull base results in a flatter, larger VP axial configuration, whereas an anterior insertion results in a VP axial configuration that is deeper and less flat. A flat VP isthmus contracts mainly in an anteroposterior direction, exhibiting a coronal closure pattern, while a deep or round VP isthmus contracts centripetally, exhibiting a circular closure pattern. Variability of the VP valving mechanism is of anatomic and not of functional origin.
Relationships between Cleft Severity and Attractiveness of Newborns with Unrepaired Clefts
Authors: Slade Pauline , Bishop Sarah C. , Jowett Rachel A.
Published: October 2018

Perceptions of attractiveness of newborns with unrepaired clefts were investigated by using a range of photographs with clefts of differing severity as ordered by a rule of thumb system commonly adopted by surgeons. The investigation involved a combination of factors such as completeness, unilaterality or bilaterality, and palate involvement. Orderings of attractiveness by surgeons, nursing staff, and adult groups unfamiliar with clefts were remarkably consistent. In addition, the responses reflected the rule of thumb system with ranked preferences of the raters being significantly related to the severity of cleft impairment. The scale devised could be used for comparative treatment outcome studies requiring assessments of initial infant attractiveness.
Follow-up of Electromyographic and Cephalometric Findings in Patients with Unilateral Cleft Lip and Palate after Fifteen Months of Continuous Wearing of a Special Removable Appliance
Authors: Carvajal Raúl , Miralles Rodolfo , José Ravera Maria , Carvajal Andrés , Cauvi Doris , Manns Arturo
Published: October 2018

A 15-month follow-up study was conducted on 13 children with unilateral cleft lip and palate. The patients presented with short upper lip, abnormal lip seal, and maxillary retrusion. All had undergone surgery in childhood. Cephalometric and electromyographic measurements were made prior to the start of treatment, 4 months into treatment, and after 15 months of continuous wear of a special removable appliance. The cephalometric measurements on paired comparison showed major improvement in both the sagittal maxillary and dentoalveolar positions. The electromyographic activity of the superior orbicularis oris muscle demonstrated no significant changes over the treatment period. Improvement in the sagittal maxillary and dentoalveolar positions seems to suggest that the elimination of the restrictive effect of the superior orbicularis oris muscle is a good therapeutic approach to promote normal growth potential in children with cleft lip and palate.
Craniofacial Deformities Associated with Juvenile Hyperthyroidism
Authors: Hirano Akiyoshi , Akita Sadanori , Fujii Tohru
Published: October 2018

Three juvenile cases of thyrotoxic craniosynostosis are reported. Two patients had small heads with posterior slanted foreheads. Skull expansion was performed in one patient. Various degrees of ocular proptosis and shallow orbits without enlargement of the extraocular muscles were seen in all cases. Zygomatic repositioning for zygomatic hypoplasia and exophthalmos was performed in two patients. The facial skeleton was involved in thyrotoxic craniosynostosis in these children, and their clinical features seemed to resemble craniofacial dysostosis.
Periodontal and Prosthetic Treatment of a Cleft Lip and Palate Patient: A Case Report
Authors: Santi Enrico , Weinberg Mea A. , Abitbol Thierry E.
Published: October 2018

Although cleft lip and palate patients are usually treated by a multidisciplinary team involving physicians and dentists, their periodontal condition may be overlooked. Crowded or malpositioned teeth, hypertrophic gingiva, orthodontic appliances, and prosthetic replacements can impede proper plaque removal and thus perpetuate periodontal disease. It is important to incorporate periodontal treatment into the comprehensive treatment as early as possible. This case report discusses the periodontal surgical procedures involved in eliminating a residual ridge defect and the fitting of the final prosthetic reconstruction. Also, the importance of the identification and management of periodontal conditions characteristic of cleft lip and palate patients before and after surgical, orthodontic, and prosthetic rehabilitation will be emphasized.
Wound Healing in Beagle Dogs after Palatal Repair without Denudation of Bone
Authors: Leenstra Thomas S. , Maltha Jaap C. , Marie Kuijpers-Jagtman Anne , Spauwen Paul H.M.
Published: October 2018

The connection of scar tissue to palatal bone by Sharpey's fibers, after cleft palate surgery, might lead to maxillary growth inhibition. The aim of this study, in beagle dogs, was to investigate the possibility of preventing the development of Sharpey's fibers by means of a modified surgical technique. In group 1, palatal repair according to von Langenbeck, was simulated. In group 2, palatal surgery was performed using a new partially split flap technique. The palates were histologically evaluated 12 weeks after surgery and compared with a control group. In group 1, the scar tissue was firmly attached to bundle bone by means of Sharpey's fibers. In group 2 and in the control group, this kind of attachment was not found; the bone was of the lamellar type. The partially split flap technique had led to the development of vaguely demarcated scar tissue and it had prevented, to a large extent, development of Sharpey's fibers.
Skull Morphology Affected by Different Sleep Positions in Infancy
Authors: Huang Chiung-Shing , Cheng Hsin-Chung , Lin Wen-Yuan , Liou Jein-Wein , Chen Yu-Ray
Published: October 2018

In Infancy, prior to cranial suture and fontanel calcification, the craniofacial skeleton can be easily deformed by an externally exerted force. In this study, the relationship between the sleep position and skull morphology was investigated. A group of 81 cleft lip and/or palate infants without other systemic anomalies was first seen in the craniofacial center at approximately 1 month of age. The sleep position of each infant was recorded as supine, prone, or mixed type. The body and skull growth were longitudinally measured at 1, 3, and 6 months of age. Infants sleeping in the supine sleep position tended to have a wider head width, shorter head length, and a larger cephalic Index by 6 months of age. The opposite phenomena were observed in the prone sleep group. The mixed sleep group tended to have head width, head length, and cephalic index between those of the supine sleep group and the prone sleep group. During the first 3 months of life, the sleep position could mold the skull primarily in the dimension of head width. In conclusion, the supine sleep position may promote brachycephaly and the prone sleep position dolichocephaly.
Cleft Width and Secondary Alveolar Bone Graft Success
Authors: Long Ross E. , Spangler Barbara E. , Yow Mimi
Published: October 2018

Fifty-six cleft sites were reviewed prior to alveolar bone grafting and subsequently evaluated for graft success using study models, periapical and occlusal radiographs from the Lancaster Cleft Palate Clinic. All patients in this sample had presurgical orthodontics to expand and align the maxillary arch prior to alveolar bone grafting. Ninety-five percent of the grafts were done using iliac crest, the remaining 5% were cranial grafts. The alveolar bone grafting technique used was as described by Boyne and Sands (1972, 1976). Cleft width was measured on a radiograph taken no more than 1 month preoperatively, following the completion of all orthodontic expansion. Cleft width was determined by inspection at its narrowest point. A distortion correction was attempted by determining the ratio of the radiographic width of the maxillary central incisor adjacent to the cleft compared with the actual width of this tooth measured on study models. The radiographic cleft width was then multiplied by this factor to approximate true cleft width. Alveolar contour was measured at least 6 months postoperatively using ratios of actual bone heights measured at the mesial, middle, and distal margin of the previous cleft compared with root length of adjacent teeth. This was to eliminate the radiographic distortion factors of foreshortening and elongation. Regression analysis was carried out to see if there was a correlation between preoperative cleft width and eventual success of the graft as measured on postsurgical radiographs. The success rate for achieving a bony bridge across the cleft was 91%. Mean alveolar crest height achieved was 93% of the anatomic root length of the tooth in the proximal segment and 96% of the anatomic root length of the tooth in the distal segment. There was significant, but low, negative correlation between presurgical cleft width and alveolar bone attachment of teeth adjacent to the grafted cleft site, after a mean radiographic follow-up of 3.1 years. Notching of the alveolar ridge in the region of the bone graft had a statistically significant low positive correlation with differing cleft widths.
Landmark Positioning on Maxilla of Cleft Lip and Palate Infant — a Reality?
Authors: Seckel Nicky G. , Van Der Tweel Ingeborg , Anne Elema G. , Specken Tom F.J.M.C.
Published: October 2018

In this study, we tested the precision of landmark positioning for a set of landmarks that can be used for the edentulous cleft lip and palate maxilla of the infant, by analyzing intraobserver and interobserver repositioning and measuring on a series of 121 (unilateral and bilateral) study casts. To date, no data on interobserver and only minimal data on intraobserver reproducibility are available. We found intraobserver reproducibility acceptable with total measurement errors ranging from 0.51 to 1.54 mm. Interobserver reproducibility was only slightly less with total measurement errors ranging from 0.63 to 1.57 mm. Total measurement errors were found to be relatively high for some variables. Both the intra- and Interobserver analyses show insight into expected precision of landmark positioning during placement of these points on casts. A learning effect for precise positioning has been demonstrated in both analyses. The points with the highest precision overall are Q and Q’, followed by the linear measurements PL and P'L’. Quality of regular study casts (impressions) is an important factor, with room for improvement. Reproducible landmark positioning on the cleft lip and palate infant's maxilla, can only be a reality if the quality of the cast is optimal and the investigator is experienced. Intraobserver and interobserver reproducibility are in the same range, which justifies the comparison of results from different studies and relieves future investigators of the need to have all measurements within a study conducted by the same investigator. Aspects of the biologic meaning of landmarks are also discussed.
Prosthodontic Abutment in Four Patients with Unilateral Cleft Lip and Palate
Authors: Suzuki Ruri , Taniguchi Hisashi , Ohyama Takashi
Published: October 2018

An examination of the relation between the number of teeth used as the abutment of a fixed prosthesis and the increase of the functional loading capacity of the upper jaw was conducted in four male patients with unilateral cleft lip and palate (age range, 19 to 21 years). This was measured at the maximum biting force at each two teeth adjacent to the cleft under various splint extension conditions and compared with that in the nonsplint condition. In all subjects, the maximum biting force under every splinted condition was significantly increased compared with the nonsplint condition. The increase was proportional to the number of teeth included in the splinting. Results indicated that two teeth should be used in each segment in the abutment of the splint across the cleft in order to increase the functional loading capability of the upper jaw comparable to that of the lower jaw. The results also indicated that inclusion of more than two teeth has no further advantageous effect on the functional loading capability.
Velopharyngeal Incompetence and Persistent Hypernasality after Adenoidectomy in Children without Palatal Defect
Authors: Ren Yan-Fang , Isberg Annika , Henningsson Gunilla
Published: October 2018

Persistent hyper nasal speech after adenoidectomy has been reported In children with palatal deficiency. Hypernasality after adenoidectomy can also occur in children with normal palatal function. The aim of the present study was to identify the cause of velopharyngeal Incompetence and hypernasality after adenoidectomy in children who did not have palatal defect as a predisposing factor. Sixteen children who developed hypernasality after adenoidectomy were included in the present study. Standard lateral cephalometry, videofluoroscopy, and nasopnaryngoscopy were performed to visualize the velopharynx and Its function during speech. The results showed that enlarged tonsils and prominent remaining adenoid tissue on the posterior pharyngeal wall were the causes of hypernasality in these children. Incomplete removal of the adenoid tissue should be avoided and enlarged tonsils should be removed at the time of adenoidectomy to prevent the risk for postoperative hypernasality.
Experiencing Clinical Ethical Dilemmas: Cases of Ethical Decision-Making in the Care of Children with Major Craniofacial Deformities
Authors: Strauss Ronald P.
Published: October 2018

This series of six papers considers ethical and health policy issues related to the care of children born with major craniofacial anomalies, using a real-life case-based approach. The papers are based upon the direct experiences of pediatricians, craniofacial surgeons, social workers, parents, and ethicists. Using a simple theoretic framework presented In the paper by Sharp, the authors “unpack” their cases and discuss the ethical, clinical, and social issues raised, considering policy implications.
Ethical Decision-Making in Interdisciplinary Team Care
Authors: Sharp Helen M.
Published: October 2018

Most clinical and ethical decisions are made between a patient and his or her physician. However, patients with complex or chronic medical problems are often cared for by a team of professionals from multiple disciplines. Little is known about the process of making ethical decisions between patients and teams of health care providers. The purposes of this article are to examine the process of collective decision-making in interdisciplinary patient care, to present a model for clinical and ethical decision-making, and finally, to discuss ethical decisionmaking in team care. The benefits and potential risks of interdisciplinary care are discussed and illustrated with case examples. Cleft palate and craniofacial teams are used as a model of interdisciplinary team care.
Neonatal Intensive Care as a Locus for Ethical Decisions
Authors: Saal Howard M.
Published: October 2018

Children born with congenital anomalies are usually cared for in the neonatal intensive care unit (NICU). Although most of these children will have conditons amenable to surgical correction, many will have serious underlying disorders that will alter the approach to management of the secondary birth defects and the child. The decision as to whether to treat or withhold treatment from a child with congenital anomalies lies with the parents or legal guardians with guidance and counseling from the health and medical care givers. The ability to make a rational decision about whether or not to offer treatment depends upon the ability to make a correct diagnosis, understand the implications of the diagnosis, and to be able to communicate this information to the patient's family. This responsibility, in many centers, falls upon the shoulders of the clinical geneticist. There is a critical need for clear communication among care givers who comprise the management team and between the management team and the family. Major obstacles that can arise include lack of communication among care givers and the reluctance of family members to make decisions regarding withholding treatment despite a diagnosis of a condition with a grave prognosis. As our technology improves, our ability to save the lives of the smallest and sickest infants will increase; the greatest dilemma, however, which we will face will not be whether we can treat, but rather, whether we should treat certain conditions and how these decisions will be made.
Common Molecular Pathways for Patterning of the Body Axis, Limbs, Central Nervous System, and Face during Embryonic Development
Authors: Stern Claudio D.
Published: October 2018

Many congenital anomalies affecting the face are known to appear as syndromes or associations, in combination with other defects. Often, these involve the limbs, eyes, central nervous system, and body axis. A general, and understandable, tendency among clinical researchers has been to seek a single cell type or definable embryologic process on which to ascribe the etiologic basis for such associations. The possibility of a gene, or group of genes, under coordinate control has not received much attention until recently. With the advent of recombinant DNA technology and the current explosion in basic research on the molecular bases of embryonic development, however, several possibilities are beginning to emerge. Here, I will list a few genes whose expression during development suggests that the molecules they encode are used as part of a coordinate molecular pathway, and that they play a role in the development of systems that often appear together in congenital associations or syndromes.