bluuebunny/crossref_metadata_2025 huggingface dataset:
Five Cases of DeMyer Sequence: An Interophthalmic Dysplasia
Authors: Spolyar John L. , Eldis Frances , Benjamins David
Published: October 2018

Five cases with vomeroseptal-prolabium agenesis, four with premaxillary agenesis, and one with hemipremaxillary agenesis are presented. All five patients had complete clefts of the secondary palate. No known family history for craniofacial dysmorphia was reported. Height and weight ranged from 2nd to 10th centile. Psychological testing showed intelligent quotients of 121, 93, 72 for three patients; two were microcephalic with undetermined IQ, but severe generalized developmental delay. CT scans for the patient with the IQ of 72 and for one of the two microcephalic subjects showed normal brain structures. The other microcephalic patients had midline prosencephalic dysgenesis. Cephalometric analysis of the cranial base and interorbital dimension using normal standards and self Indexing showed hypotelorism and small anterior cranial bases to be apparently related to reduced size of the sphenoid. An intact ethmoid, nasal bone, and crista galli appear to represent key anatomic differences in these patients as compared to classical holoprosencephaly sequence subjects. It was suggested that this pattern of midline facial agenesis may range from a solitary central incisor to the most severe variant presented. Using the embryologic classification, these subjects fall best under the heading of craniofacial dysplasia rather than cerebral craniofacial dysplasia, as a different type of interophthalmic dysplasia or DeMyer sequence.
A Cineradiographic Study of Velopharyngeal Movements for Deviant versus Nondeviant Articulation
Authors: Henningsson Gunilla , Isberg Annika
Published: October 2018

Five patients who displayed both glottal stop compensations and normal articulatory placement and production during connected speech and also had weak pressure articulatory consonants and hypernasal resonance had cineradiographic study of their velopharyngeal movements. The results showed that velopharyngeal movements were impaired during weak pressure consonants whereas velopharyngeal valving was maximal when articulation was not impaired. Velopharyngeal movements were the worst when glottal stop substitutions were produced. Poor quality or absence of velopharyngeal movements in connection with deviant articulation could be erroneously interpreted as weakness or incapability of motor activity if nondeviant articulation is not observed in individual patients.
Fallibility of Clinical Research
Authors: Shprintzen Robert J.
Published: October 2018

Though clinical research has resulted in an abundance of literature over the past 30 years, many of the same questions asked in 1960 are being asked today. Why is it seemingly impossible to determine the proper age for palate repair? Why does it seem impossible to determine if maxillary deficiency in patients with cleft lip and palate is caused by the surgeon or is intrinsic? Though poor design of clinical research is in part responsible, even well-designed clinical research findings can be misinterpreted, well-designed studies can be poorly executed, or important findings can be completely overlooked because of experimenter bias. It is even possible that clinical research is well designed and executed, but the question asked is the wrong question. Agreement on major issues may seem impossible, but experience in other fields of clinical research shows that the problem is not universal and our failure to agree may have a major impact on the application of patient care.
Directions and Issues in Psychosocial Research and Methods as Applied to Cleft Lip and Palate and Craniofacial Anomalies
Authors: Strauss Ronald P. , Broder Hillary
Published: October 2018

Psychosocial research into cleft lip and/or palate and craniofacial conditions has historically employed a “medical model” of research that sought to clarify whether patients experienced psychopathology as a consequence of the birth defect. In recent years, this paradigm has been replaced by a “social science model” of research that posits that most individuals with clefts do not manifest psychological pathology. The “social science model” examines adjustment and adaptation in the patients and their families. Research questions may consider the social, cultural, and psychological ramifications of being different in terms of speech, appearance, or identity. The authors suggest that an expanded set of research questions be considered, and that researchers from sociology, anthropology, ethics, economics, health services research, as well as psychology, become engaged. This paper proposes a range of possible research topics and indicates likely trends in research design and methodology.
Data-Objective Diagnosis of Infant Cleft Lip, Alveolus, and Palate. Morphologic Data Guiding Understanding and Treatment Concepts
Authors: Kriens Otto
Published: October 2018

Maxillary plaster models of untreated infants with various cleft lip and palate forms have been studied under the Reflex Microscope. From the three- dimensional data processed by a PC, the specific dimensions of various cleft forms can be determined. Unexpected findings are that skeletal maxillary changes are present in infant velar clefts. The mid-sagittal maxillary length in particular becomes shorter with more severe clefting of the bony palate. From the morphologic point of view, Robin's sequence does not represent a specific entity, but usually a severe cleft of the hard palate. Unilateral clefts of lip, alveolus, and palate with a partial cleft lip reveal worse malformations than complete unilateral clefts. The morphologic changes recorded can be explained by a varying degree of lingual malposition. This reveals that displacements and malformations of infant cleft lip and palate are in fact secondary in nature. In correcting these secondary surface manifestations as soon as indicated by physiologic criteria one could hope for early normalization of sensation, perception, and function. Three-dimensional high-precision measurements are crucial for data-objective diagnoses, timing of therapy, choice of treatment, and in later evaluation of the results.
Dental Students' Knowledge of and Exposure to Cleft Palate
Authors: Vallino Linda D. , Lass Norman J. , Pannbacker Mary , Klaiman Paula , Miller Patricia
Published: October 2018

A questionnaire was administered to 108 dental students from West Virginia University to assess their knowledge of cleft palate. Results revealed areas of deficiency in basic knowledge and exposure to clefting. When these data were compared to those from an earlier study (Lass et al., 1973) differences were found of varying type. A comparison between dental and medical students revealed that the basic knowledge of cleft palate and associated problems were not different between the two groups. However, the dental students had done more reading than the medical students and had the topic of cleft palate included in their coursework.
Three-Dimensional Computer Reconstruction of the Eustachian Tube and Paratubal Muscles
Authors: Spauwen Paul H.M. , Hillen Berend , Lommen Etienne , Otten Egbert
Published: October 2018

A new method of anatomic dissection and image reconstruction using computer techniques for better understanding of eustachian tube (ET) functioning is presented. Coronal sections of two noncleft fetal skulls were photographed and projected on a graphic tablet. Contours of the pertinent structures were digitized using a mouse. Coordinates of all digitized points were entered into a special computer program. The data were transformed into three-dimensional representations of anatomic structures. The levator veli palatini muscle (M-LVP) was found to have a close relationship with the ET running underneath it and passing at the inside of the pharyngeal edge of the medial cartilage before entering the soft palate. On M-LVP contraction, this part of the medial ET cartilage appears to be the sole point of impact for ET opening. The tensor veli palatini muscle (M-TVP) is connected with the lateral cartilage of the ET and leaves the ET, rounding the pterygoid hamulus before entering the palatal aponeurosis. On this anatomic basis, action of the muscle by isotonic contraction appears to be more likely than isometric contraction.
Mechanical Contraction Property of the Levator Veli Palatini Muscle
Authors: Kogo Mikihiko , Inoue Kazuo , Matsuya Tokuzo , Nishio Juntaro , Hamamura Yasushi , Yasui Yasunobu , Miyazaki Tadashi
Published: October 2018

Twenty adult dogs anesthetized with sodium pentobarbital were used as experimental animals in an analysis of the mechanical contraction properties of the levator veli palatini muscle. The following results were obtained: the contraction time and half relaxation time of maximal isometric twitch of the muscle were revealed to be 43 msec and 33 msec, respectively; a summation curve was obtained with stimulation at 15 Hz and maximal tetanic tension was at 70 Hz. The average of the tetanic tension ratio was found to be 5.7. The maximum rate of tension rise was observed at 150 Hz, and the value was twice the rate of the twitch contraction. The duration time of the active state plateau showed values of 3.9 to 4.5 msec. The contraction time of the levator veli palatini muscle closely resembles that of the intrinsic laryngeal and the orbicularis oris muscle.
Increased Nasal Resistance Induced by the Pressure-Flow Technique and its Effect on Pressure and Airflow during Speech
Authors: Liu Hui , Warren Donald W. , Dalston Rodger M.
Published: October 2018

Although the validity of the pressure-flow technique has been verified in a number of laboratories, some questions still remain. The purpose of this study was to determine whether the procedures involved in estimating orifice size affect the pressure and airflow variables being measured. Twenty subjects with demonstrated velopharyngeal inadequacy on pressure-flow testing (VPO ≥ 0.10 cm2) were assessed under two contrasting conditions. Subjects were asked to produce /p/ in the word “hamper” with a) one nostril occluded by a cork as in pressure-flow testing and b) both nostrils patent. The results indicate that the increased nasal resistance resulting from occlusion of one nostril does not appreciably affect pressure and airflow associated with plosive consonant production in patients with velopharyngeal inadequacy.
Motor Control of Velopharyngeal Structures during Vowel Production
Authors: Moon Jerald B. , Jones David L.
Published: October 2018

This investigation was designed to measure the ability of normal adult speakers to exert voluntary control over velopharyngeal positioning. Speakers were asked to phonate the vowels [a] and [i] at 50 percent and 75 percent of complete velopharyngeal closure, using visual feedback of velopharyngeal opening and closing gestures from a phototransducer. The musculature of the velopharyngeal mechanism was hypothesized to act as a coordinated system that may demonstrate both motor flexibility and plasticity (Folkins, 1985) when forced to function in a novel way. Evidence of both motor system responses to a novel speaking condition was observed. Speakers were able either to phonate at intermediate closure levels without having to learn new motor rules, or to learn new rules for velopharyngeal muscle activation that resulted in the ability over time to position the velopharyngeal mechanism appropriately. As such, support is derived for the notion (Folkins, 1985) that speakers develop motor rules or coordinative structures involving the velopharyngeal mechanism that govern velopharyngeal movement. The characteristics of this coordinative structure framework has not yet been described, however, and are the subject of ongoing research efforts.
Questionnaire Survey of Attitudes and Concerns of Patients with Cleft Lip and Palate and Their Parents
Authors: Noar Joe H.
Published: October 2018

The self-perceived problems and concerns of patients with complete unilateral cleft lip and palate and their parents were investigated. Thirty-two patients ranging in age from 16 to 25 years (mean=19.9 years) and their parents were sent questionnaires by mail. Twenty-eight patients and 30 parents completed the questionnaires. The results suggest that the patients were satisfied with the treatment they had received. They believed that the efforts of the members of the cleft palate team had been successful, and they were generally satisfied with their overall facial appearance and speech. There were, however, specific aspects with which they were less satisfied; their nose, lip, profile, speech, and teeth. They did not feel significantly socially or emotionally handicapped, although they noted that they had been teased and that they found relations with the opposite sex difficult. Their parents were also generally satisfied with all aspects of treatment and their child's appearance and speech. They identified those areas that their child was most concerned with, but did not fully appreciate how satisfied their child was with his or her facial features. The parents felt that their children were socially and emotionally affected by their cleft and felt their school results had also been affected.
Use of the Branemark Implant in the Cleft Palate Patient
Authors: Verdi Frank J. , Lanzi Guy L , Cohen Stephen R. , Powell Richard
Published: October 2018

This paper describes the clinical and surgical management of a patient presenting with a unilateral alveolar cleft and associated oronasal fistula. After orthodontic expansion of the maxilla, a secondary osseous graft was placed. A single Branemark implant was subsequently utilized to allow for prosthetic restoration of the dental arch. Through case presentation, a detailed course of treatment is outlined that effectively restores the anatomy, integrity, and function of both the alveolar and dental arches.
Postpalatoplasty Decrease in Respiratory Dead Space in Young Children
Authors: Beppu Kazunori , Oka Masuichiro , Abe Kihachiro
Published: October 2018

In 20 children with cleft palates age 1.5 to 2 years, respiratory parameters were evaluated before, 15 minutes after, and 1 hour after palatoplasty. The dead space (VD) in the palatoplasty group decreased from 25.2 ± 4.7 ml preoperatively to 12.3 ± 9.1 ml postoperatively. This decrease improved respiratory efficiency. A significant difference was found postoperatively in the partial pressure of CO2 in arterial blood (PaCO2) between the palatoplasty and control groups. No significant differences were found in base excess (BE), arterial pH, pulmonary shunt ratio, and the alveolar-arterial O2 difference (A-aDO2) between the groups pre- and postoperatively. This finding indicated that temporary respiratory failure may exist postoperatively; the phenomena were improved in the recovery room. Since lung compliance and airway resistance may also participate in the decrease in VD, further evaluation of these factors are necessary.
Accuracy of Information Reported by Parents and Children Evaluated by a Cleft Palate Team
Authors: Paynter Earlene T. , Edmonson Terry W. , Jordan William J.
Published: October 2018

Children with palatal clefts and their parents were interviewed to determine what information they had about the cleft and its management. Their responses were compared with the standards of 17 team members from seven disciplines. Both parent and child were consistently provided information about clefts that they were expected to remember. The children's responses met the standards 67 percent of the time. Their parents' responses met the standards 80.6 percent of the time. Parents accurately predicted their children's responses 92 percent of the time.
Craniofacial Asymmetries in Shunt-Treated Hydrocephalic Children
Authors: Pirttiniemi Pertti M. , Huggare Jan Å. , Kantomaa Tuomo J. , Serlo Willy S.
Published: October 2018

The degree and direction of craniofacial asymmetry in the frontal plane was studied in a sample of 26 shunt-treated hydrocephalic children aged 5 to 18 years. Thirteen of the children had the regulating shunt fixed on the right side of the head and 13 on the left. The shunt had usually been inserted during the first years of life. An age- and sex-matched group of healthy school children and students served as controls for cephalometric assessment by means of linear and angular measurements. Considerable craniofacial asymmetry was found in the shunt-treated hydrocephalie children, the direction of asymmetry being related to the laterality of the regulating shunt device. The most marked asymmetric changes were recorded in the maxillary and mandibular regions, probably due to the adaptive nature of these regions.
Characteristics of New Patients at Illinois Cleft Palate Teams
Authors: Will Leslie A. , Parsons Robert W.
Published: October 2018

The Illinois Association of Craniofacial Teams (IACT) surveyed all new patient visits to member teams in an effort to assess the needs of patients in Illinois. The survey determined the number of patients, their age at initial visit, the location and status of their defect, and their history of team visits. Four hundred eighty new patient visits were recorded. The number of patients was substantially lower than expected, and the large majority of older children seeing a team for the first time had repaired defects. It is concluded that the main opportunity for improving the quality of care for patients with clefts in Illinois may be in increasing the use of cleft palate teams.
Pressure-Flow Measurements for Selected Oral and Nasal Sound Segments Produced by Normal Adults
Authors: Andreassen Meri L. , Smith Bonnie E. , Guyette Thomas W.
Published: October 2018

Pressure-flow data are often used to provide information about the adequacy of velopharyngeal valving for speech. However, there is limited information available concerning simultaneous pressure-flow measurements for oral and nasal sound segments produced by normal speakers. This study provides normative pressure, flow, and velopharyngeal orifice area measurements for selected oral and nasal sound segments produced by 10 male and 10 female adult speakers. An aerodynamic categorization scheme of velopharyngeal function, including one typical category and three atypical categories (open, closed, and mixed) is proposed.
Developmental Correlates of Midfacial Components in a Normal and Cleft Lip and Palate Human Fetal Sample
Authors: Siegel Michael I. , Mooney Mark P. , Kimes Kyle R. , Todhunter John
Published: October 2018

Midfacial hypoplasia in patients with complete clefts of the lip and palate is considered to be the result of congenital dysmorphogenesis combined with surgical effects. The present study describes congenital correlates of midfacial hypoplasia by examining the relationships between premaxillary size and a number of adjacent oronasal capsular components in a sample of second trimester normal and complete cleft fetal specimens. Midfacial relationships from previously published data from 3-D computer reconstructions of histologic material were assessed by multiple regression and path analyses. Stepwise multiple regression analysis revealed that the combination of age (body size) and nasal septal length were the best predictor variables of premaxillary length and accounted for 90 percent and 93 percent (p < .01) of the variance in normal and cleft fetal specimens, respectively. Tongue length was seen to slightly increase the R2 values in the cleft, but not the normal sample. Path analysis results revealed that with body size held constant, nasal capsule volume explained only 3 percent of premaxlllary variance, while septal length accounted for approximately 45 percent of the variance (p < .05). Results show that the relationships of the midfacial components in the cleft fetal sample closely followed normal second-trimester midfacial growth patterns and suggest that the septal-traction model may be a more parsimonious and primary explanatory mechanism of early midfacial growth than the functional matrix model in both normal and complete cleft fetal specimens.
Comparison between Multiview Videofluoroscopy and Nasendoscopy of Velopharyngeal Movements
Authors: Henningsson Gunilla , Isberg Annika
Published: October 2018

The videofluoroscopic and nasopharyngoscopic observations of velopharyngeal movements during speech were compared in 80 subjects with hypernasal speech. An endviewing flexible fiberoptic endoscope was used for nasopharyngoscopic examination. This failed to demonstrate movements of the lateral pharyngeal walls that were videofluoroscopically documented in one third of the patients. In the cases of disagreement, the angle of view was impaired because of the presence of adenoid tissue. The misinterpretation at nasopharyngoscopy was explained by an unfavorable observation position of the flexible scope and failure to pass it further into the pharynx. Conclusions are satisfactory that videofluoroscopy is an indispensable procedure for assessing velopharyngeal function.
Development of Scales of Severity of Facial Cleft Impairment
Authors: Tobiasen Joyce M. , Hiebert John M. , Boraz Robert A.
Published: October 2018

The reliability and validity of human judgments in rating the severity of cleft-related facial impairment were examined in this series of studies. The goal of the research was to develop scales for rating the severity of facial cleft impairment using psychophysical methods for measuring human judgment. The principle findings were: (1) Four photographic categorized scales with high inter-group reliability and satisfactory content and construct validity were developed; (2) Raters used objective stimulus characteristics of the face to scale impairment; (3) Ratings of facial attractiveness decreased as the severity of impairment increases; (4) Full face ratings of the severity of impairment correlated highly with ratings of the same photographs cropped below the eyes. The implications of these findings for clinical and research application are discussed.
Cleft Lip and Palate in Tehran
Authors: Taher Abbas A.Y.
Published: October 2018

Seventy-nine cleft lip and/or palate births were isolated from 21,138 live births between January 1, 1983 and December 31, 1988 in one hospital in Tehran. Among these, 21 (26.58 percent) were cleft lip (CL), 45 (56.96 percent) were cleft lip and palate (CLP), and 13 (16.45 percent) were cleft palate (CP). Chemical sulfur mustard gas was indicated as a major factor in 30 (37.97 percent) of the bilateral cleft lip and palate infants.
Long-Term Follow-Up after Division of a Pharyngeal Flap for Severe Nasal Obstruction
Authors: Caouette-Laberge Louise , Egerszegi E. Patricia , De Remont Anne-Marie , Ottenseyer Ilse
Published: October 2018

Between 1965 and 1986, nine patients were noticed to have significant nasal airway obstruction following surgery for velopharyngeal incompetence (VPI). All had a superiorly based pharyngeal flap. Division of the flap was recommended to correct the posterior obstruction. A complete section of the flap was done in seven cases and lateral port enlargement was done in the remaining two. The interval between flap elevation and transection ranged from 5 months to 5 years. Three patients required more than one operation to fully correct the obstruction.

All the patients were evaluated 2 to 14 years later to assess nasal breathing and speech and to document velopharyngeal function by nasoendoscopy and videofluoroscopy. One patient presented major symptoms of nasal obstruction at follow-up, while others reported snoring and occasional mouth breathing, although their nasal respiration appeared subjectively adequate. Four patients had normal speech, three were mildly hyponasal, one was moderately hyponasal, and the other was severely hyponasal. Intelligibility was good in all cases but one, although three patients had some articulation errors: two with persistent errors related to early VPI and one from dental malocclusion and tongue protrusion. Videofluoroscopy and nasoendoscopy showed that despite complete transection at the base of the flap in eight cases, five still had evidence of residual tethering. In one patient, the obstruction was almost complete and repeat division of the flap was recommended. Seven patients showed increased thickness of the soft palate in the midline where the flap had been anchored. Velopharyngeal closure was adequate in five cases, marginal in three, and obstructed in one.

The review of our cases showed that the velopharyngeal opening in these patients is not large and incompetent, but rather is contracted and the flap often reattaches posteriorly after division. We recommend a closure of all raw surfaces to be done when the flap is sectioned, adding Z-plasties when needed to prevent further V-P obstruction. Even in the presence of recurrent obstruction, the resection of the extra tissue contributed by the flap on the soft palate is not felt to be indicated.
Superior Orbicularis Oris Muscle Activity in Children with and without Cleft Lip and Palate
Authors: Carvajal Raul , Miralles Rodolfo , Cauvi Doris , Berger Barbara , Carvajal Andres , Bull Ricardo
Published: October 2018

An analysis of integrated electromyographic (IEMG) activity of the superior orbicularis oris muscle was undertaken in 15 children with cleft lip and palate who have undergone surgery compared to 10 children without clefts (control group). Bipolar surface electrodes were used for IEMG recordings of resting level activity and during the swallowing of saliva. Similar resting level activity was observed in both groups. During the swallowing of saliva, activity in children with cleft lip and palate was higher than in children without clefts (noncleft children). Moreover, in the cleft lip and palate group, children with abnormal lip seal showed the highest values for IEMG activity during the swallowing of saliva. This fact suggests that with each swallow of saliva, a greater counteracting effect of the superior orbicularis oris muscle could be produced on the growing maxilla. This may result in a significant long-term effect on the growth of the stomatognathic system, since the process of swallowing is a 24-hour function repeated between 600 and 2400 times each day.
Growth Rate of the Vomer in Normal and Cleft Lip and Palate Human Fetal Specimens
Authors: Kimes Kyle R. , Mooney Mark P. , Siegel Michael I. , Todhunter John S.
Published: October 2018

The present study, part of an ongoing investigation of normal and dysmorphic development of the human fetal oronasal capsule, examined the rate of growth of the vomer. For comparative purposes, 29 human fetal specimens (20 “normal” and 9 cleft lip and palate [CLP]) were celloidin embedded, sectioned, stained with hematoxylin and eosin, and serially digitized. The specimens ranged from 8 to 21 weeks in postmenstrual age. The application of a well-documented three-dimensional reconstruction technique provided quantification of several aspects of the vomer. CLP vomer length and volume were growing at a faster rate In the 8 to 21 week age range. Growth curves were produced by plotting length and volume against postmenstrual age and a significant difference was noted between the slopes (growth rate) of the linear component of the normal and CLP growth curves for vomer length (p < .001) and volume (p < .001). This study tested the hypothesis of a more rapidly growing 8 to 21 week CLP vomer and observed that the growth trends of the CLP vomer are similar to those of the CLP nasal septum, which also was found to possess a significantly larger (p < .001) volumetric growth rate throughout the course of the vomer. Comparative findings suggest that a pathogenetic correlate of CLP is the rapid enlargement of the midline structures of the oral and nasal capsules.
Patterns of Abnormal Myogenesis in Human Cleft Palates
Authors: Cohen Steven R. , Chen Lynn L. , Burdi Alphonse R. , Trotman Carroll-Ann
Published: October 2018

To test the hypothesis that soft palate muscles are abnormal in cleft palate, we compared soft palate morphogenesis in fetuses with cleft palate (n=4) to age-matched (n=3) and nonmatched (n=1) control specimens. The morphologic status of all soft palate and masticatory structures were classified into one of six stages based on the level of histogenesis. At 54 mm crown-rump length (CRL), the levator vell palatini (L), palatopharyngeus (PP), and palatoglossus (PG) in cleft subjects demonstrated mesenchymal condensation into myoblastic fields, lagging behind the control specimens (97 mm CRL), which displayed definitive fields of myoblasts and myotube formation. In the 175 mm and 225 mm cleft and the 170 mm and 192 mm control specimens, muscular morphology was similar and had reached its postnatal appearance for the tensor veli palatini (175 m only) and L, PP, PG (225 mm only). Muscle fiber directions were, however, disoriented and disorganized, especially close to the medial epithelial edge of the cleft. The levator veli palatini, could not be distinguished as a discrete muscle in the cleft specimens, and what we believed to be the PP and PG seemed “normal” at the level of light microscopy, but malpositioned in a superior direction. This preliminary study demonstrates for the first time that early myogenesis in cleft palates differs from normal.
Enhanced Prevalence of Ankyloglossia with Maternal Cocaine Use
Authors: Harris Edward F. , Friend Gerald W. , Tolley Elizabeth A.
Published: October 2018

A series of 500 term neonates was examined in a well-baby nursery, 68 of whom tested positive for maternal cocaine use. From logistic multiple regression equations, weight and crown-heel length were significantly smaller in the maternal cocaine-use subset of this case-control study. Partial ankyloglossia, with a prevalence of 4.4 percent in the overall series, was significantly more common in males than in females (6.0% versus 2.3%), while race (black or white) had no influence on trait frequency. Controlling for race and sex, ankyloglossia was 3.5 times more likely to occur in the drug-use series, perhaps as a function of diminished mitotic rates.
Popliteal Pterygium Syndrome with Special Consideration of the Cleft Malformation: Case Report
Authors: Koch Hubertus , Grzonka Magdalena , Koch Josef
Published: October 2018

This report describes a new case of popliteal pterygium syndrome (PPS) and also a treatment protocol. The patient presented with the complete complex of PPS and additional abnormalities that have not been described in the literature: a sinus of the upper lip, an extreme hypopoplastic prolabium with aplasia of the vestibule In this area, and a velar pterygium.
Craniofacial Screening Profile: Quick Screening for Congenital Malformations
Authors: Coston Gale Norman , Sayetta Rona B. , Friedman Harold I. , Weinrich Martin C. , Macera Caroline A. , Meeks Kathryn , Mcandrews Shawn K. , Morales Kandi S.
Published: October 2018

The correct identification of syndromes and other congenital malformations at an early age is critical for the child, family and care providers. Most specialists who conduct large screenings of young children are not adequately trained to recognize signs and symptoms that should lead to appropriate referral to the clinical geneticist and/or diagnostic team. A systematic approach for recognizing important signs is presented here; a Craniofacial Screening Profile. Following a brief training program, the Profile was validated by 39 speech-language pathologists in screening 3,539 kindergarten and first grade children. The results were excellent (specificity was 99.6%), demonstrating that with limited training, specialists can effectively screen for important signs and symptoms of a major group of syndromes and other congenital malformations.
Cranio-Orbito-Zygomatic Measurements from Standard CT Scans in Unoperated Crouzon and Apert Infants: Comparison with Normal Controls
Authors: Carr Michele , Posnick Jeffrey C. , Pron Gaylene , Armstrong Derek
Published: October 2018

Cranio-orbito-zygomatic measurements taken from standard axial computed tomographic (CT) scans of unoperated patients with Crouzon syndrome (6 males, 14 females) and Apert syndrome (6 males, 10 females) under 1 year of age were compared to each other and to normal controls (40 males, 35 females). Fifteen cranio-orbito-zygomatic variables were measured and repeated. Means and standard deviations were computed for each sex, and means were pooled since they were not significantly different. Correlation coefficients for repeat measures (intra-observer error) of each variable showed good reproducibility. Only the lateral orbital wall angle differed between the syndromes (p = .034), suggesting that upper facial differences (as measured by these variables) do not manifest themselves at this young age. All measurements for both syndromes, except one, were outside the 95 percent confidence range for age-matched controls. Measurements taken from standard CT scans provide an objective comparison between specific syndromes and the normal population.
Nasal Capsule Shape Changes following Septopremaxillary Ligament Resection in a Chimpanzee Animal Model
Authors: Siegel Michael I. , Mooney Mark P. , Eichberg Jorg W. , Gest Thomas , Lee D. Rick
Published: October 2018

Recent human data suggest a relationship between the disruption of the septopremaxillary ligament (SPL) attachment and lack of anterior midfacial growth in cleft lip and palate (CLP) individuals. Early SPL resection in chimpanzees resulted in premaxillary growth deficits through 1200 days. Since the SPL is also continuous with the nasal bones, the present study was undertaken to investigate compensatory nasal capsule shape changes following SPL resection in a chimpanzee animal model. The study used 17 chimpanzees (Pan troglodytes): seven unoperated controls, five sham surgical controls, and five animals with early (average 144 days) SPL resection. Lateral head x-ray films and dental study models were collected quarterly and landmarks representing boundaries of the nasal capsule were identified. Mean nasal capsule polyhedrons were constructed from linear measurements at 200, 600, and 1000 days. In animals with SPL resection, the nasal capsule appeared truncated, shortened anteroposteriorly, and nasion was displaced posteriorly compared to controls by 600 days. Tensor biometric analysis of the growth/shape changes of the nasal capsule triangles revealed no significant differences across age in SPL resection animals while significant (p < .05) age-related changes were noted in both control groups. Results showed that early SPL resection resulted in maintaining the neonatal nasal capsule morphology in the chimpanzee and suggested that such early growth mechanisms may be operating in complete CLP individuals as well. These data support the concept of early re-establishment of the SPL in primary nasolabial cleft repair to facilitate midfacial and nasal capsule growth.
Experiments of Nature: Premature Unicoronal Cranial Synostosis in Mantled Howler Monkeys (Alouatta palliata)
Authors: Corner Brian D. , Richtsmeier Joan T.
Published: October 2018

In 1960 Adolph Schultz described several cases of plagiocephaly in a collection of mantled howler monkeys (Alouatta palliata) from the forests of Central America. Since then several more specimens have been described. These individuals make up one of the largest samples of nonhuman primates that are affected by craniosynostosis. In this study, we used finite element scaling analysis (FESA) to quantify morphologic differences between adult normal (N = 19) and plagiocephalic female howler monkeys (N = 4). FESA utilizes three dimensional coordinate data to provide detailed information on size and shape differences local to biologic landmarks. The relatively large number of plagiocephalic howler monkeys provides a natural means for examining hypotheses concerning patterns of dysmorphology associated with plagiocephaly. Comparison of our results to observations from the clinical literature reveals similarities in neu-rocranial and facial morphology among plagiocephalic humans and howler monkeys, but the direction and magnitude of local form differences between howler monkeys and humans indicate species-specific responses to suturai constraint. Few cases of craniosynostosis in nonhuman primates are known. The number of plagiocephalic howler monkeys suggests these monkeys may be particularly susceptible to some defect that leads to premature suture closure. If this is the case, then howler monkeys may be a good model to study the cause(s) of craniosynostosis.
Comparison of Velopharyngeal Gap Size in Patients with Hypernasality, Hypernasality and Nasal Emission, or Nasal Turbulence (Rustle) as the Primary Speech Characteristic
Authors: Kummer Ann W. , Curtis Cindy , Wiggs Melissa , Lee Linda , Strife Janet L.
Published: October 2018

Velopharyngeal insufficiency was assessed using multiview videofluoroscopy on eight patients with hypernasality, 10 patients with hypernasality and audible nasal emission, and 10 patients with nasal turbulence (rustle). Patients demonstrating hypernasality, with or without audible nasal emission, were found to have a significantly larger velopharyngeal gap than those with nasal rustle. This finding suggests that the degree of the velopharyngeal insufficiency can be predicted to some extent based on perceptual assessment. If the presence of a nasal rustle suggests a small velopharyngeal gap, despite the severe distortion of speech, then speech therapy should be considered prior to surgery in those cases.
Survey of Services and Practices of Cleft Palate-Craniofacial Teams
Authors: Pannbacker Mary , Lass Norman J. , Scheuerle Jane F. , English Phylisa J.
Published: October 2018

Questionnaire data about services and practices of cleft palate-craniofacial teams were obtained from 63 teams in 35 states. Findings indicate the following: (1) research was the least frequently conducted activity, although 73 percent of the teams Indicated that they conducted research; and (2) the majority of those responding regarded cinefluoroscopy, endoscopy, perceptual assessment, oral examination, and videoendoscopy as important procedures, yet most patients (90%) seen by these teams did not receive instrumental assessment of velopharyngeal function.
Periodontal Conditions in Adult Patients with Cleft Lip, Alveolus, and Palate
Authors: Bragger Urs , Schürch Ernst , Salvi Gianni , Von Wyttenbach Thomas , Lang Niklaus P.
Published: October 2018

The present study assessed the progression rate of periodontal disease over 8 years in a group of 52 adult patients with various forms of cleft lip, alveolus, and palate considered at risk for progression of periodontal disease. Of special interest was the evaluation of periodontal disease progression at sites adjacent to cleft regions compared to changes found at control sites not directly affected by such defects. High incidences of generalized plaque accumulation and bleeding on probing were noted at both examinations in 1979 and in 1987. A mean apical shift of the clinical attachment level amounting to 0.2 mm had occurred over the 8-year observation period. A slight apical displacement of the mesial and distal mean crestal alveolar bone was also noted. The rate of progression of periodontal disease over the 8 years was not found to be different at statistically significant levels at cleft sites compared to control sites. However, the results of this study documented that the cumulative periodontal destruction at 26 to 28 years of age was statistically significant and more pronounced at cleft sites as revealed by greater probing pocket depth and loss of clinical attachment. The differences between test and control sites amounted to 0.3 and 0.4 mm respectively for probing depth and 0.6 mm for loss of clinical attachment. In addition, the discrepancy between alveolar bone height and the levels of the clinical attachment at cleft sites demonstrated the presence of a long supracrestal connective tissue attachment adjacent to cleft defects. Therefore, the alveolar bone height as visualized in radiographs at such sites was considered an unreliable diagnostic tool for the assessment of the degree of periodontal destruction.
Quintuplets with Clefts: Follow-Up at Ten Years of Age
Authors: Brin Ilana , Zilberman Yerucham
Published: October 2018

A set of quintuplets, three of which were born with various degrees of oral clefting, were followed up to the age of 10 years. Study models, as well as panoramic and intraoral roentgenograms were collected at ages 5 and 10 years. Cephalometrlc roentgenograms were added at the age of 10 years. Analysis of the growth charts for height and weight indicated normal somatic development, save for a persistent, albeit diminishing, lag for the two most severely affected siblings. The latter also demonstrated a more vertical craniofacial growth pattern. The dental arch dimensions were within normal range, except for the maxillary and mandibular widths in the two more affected siblings, who also exhibited a delay of one or two stages of dental development in an interslbling comparison. These findings indicate that in the more severely affected siblings, there was only partial growth catch-up at the age of 10 years.
Comparison of the Craniofacial Characteristics of Two Syndromes Associated with the Pierre Robin Sequence
Authors: Glander Karl , Cisneros George J.
Published: October 2018

The craniofacial characteristics of two syndromes commonly associated with Robin sequence were compared for 49 subjects. Lateral cephalograms were analyzed for four groupings: Group I—Stickler syndrome with versus without Robin, Group II—velocardlofacial (VCF) syndrome with versus without Robin, Group III—Stickler without Robin compared to VCF without Robin, and Group IV—Stickler with Robin compared to VCF with Robin. Thirty-two skeletal and 18 soft tissue measurements were compared. In Group I, three skeletal measurements were significantly different (SNA, SNB, and SNPg). In Group II, no significant difference was found for any of the 50 measurements. In Group III, a significant difference was demonstrated for seven parameters (one skeletal, six pharyngeal and airway). In Group IV, two skeletal and eight airway measures were significantly different. The findings Indicate that the relative maxillary and mandibular retrognathia observed in Stickler/Robin patients may predispose them to the Robin sequence and vice versa; the Robin features In VCF may be caused by hypotonia rather than any craniofacial or physical obstruction of the airway; Stickler and VCF are similar in craniofacial morphology but show marked differences in pharyngeal and airway morphology; and cephalometrics should not be the sole prognosticator of the Robin sequence and Its association with Stickler and VCF.
Effect of Glossopexy on Speech Sound Production in Robin Sequence
Authors: Leblanc Star M. , Golding-Kushner Karen J.
Published: October 2018

The management of Robin sequence may Include glossopexy, In which the tongue is attached to the lower lip and mandible for anterior lingual positioning to resolve upper airway obstruction. The procedure Is performed early In Infancy and reversed at the time of palatal repair near 1 year of age. This period Is critical to prespeech vocalization. The effect of glossopexy on speech sound production was investigated In 17 children with Robin sequence who had glossopexles. Analysis suggested that glossopexy may temporarily affect the development of prespeech skills and sound production. The onset of babbling and Initiation of first words was often delayed in the subjects in comparison with syndrome-matched children who had not undergone glossopexy. Tongue-tip sounds were often produced with the tongue blade, but production was perceptually normal. The early delays were temporary and the only long-term effect of glossopexy was a tendency to produce tongue-tip sounds with the tongue blade.
Effects of Pubertal Changes on the Speech of Persons with Cleft Palate
Authors: Lang Berdetta K. , Starr Clark D. , Moller Karlind
Published: October 2018

The effects of puberty on the speech of persons with cleft palate and normal to moderate degrees of resonance distortion were investigated. Listeners were rated for resonance, articulation, and speech acceptability from recordings made prepuberty and postpuberty for 18 males who received no surgery during the time they went through puberty. Results suggest that changes were minimal and did not always result in speech improvement or deterioration.
Nasopharyngeal Characteristics in Children with Cleft Lip and Palate
Authors: Šmahel Zbyněk , Müllerova Iva
Published: October 2018

Cephalometrlc assessment of the nasopharynx and its adjacent structures was carried out in two experimental groups of 5-year-old male patients with unilateral cleft lip and palate. The first group Included individuals who had not had surgery, and the second was comprised of Individuals who were at least 1 year post-palatoplasty with primary pharyngeal flap. They were compared with a control group of boys of comparable average age who did not have clefts. Both groups of patients with clefts showed a reduction of the nasopharyngeal bony framework related to the posterior position and decreased posterior height of the maxilla without hypertrophy of the adenoids. The smaller nasopharyngeal airway was consistent with the reduction of the size of the bony nasopharynx. There was shortening of the anterior cranial base located In the region of the middle cranial fossa. The height of the body of the sphenoid bone was reduced, but the angle of the cranial base was within normal limits.
Anthropometric Growth Study of the Head
Authors: Farkas Leslie G. , Posnick Jeffrey C. , Hreczko Tania M.
Published: October 2018

Five measurements of the head were taken between 1 year and 18 years of age in 1,537 North American Caucasians. By 1 year of age, the circumference (87.5%) and length (87.1%) of the head showed the highest levels of developmental level compared with their adult size. By 5 years, the developmental level of all measurements in head width, head length, and circumference increased, closely approaching maturation. Head length reached full maturation at 10 years in females (182.7 mm), and at 14 years in males (189.2 mm). In females, head width showed the most advanced maturation at 14 years (142.7 mm). In males, most of the head measurements matured at 15 years of age. Adult head height was approached at 13 years in both sexes (113.3 mm in males and 109.8 mm in females). Early rapid growth in head height and head length took place between 1 and 4 years of age, and between 1 and 6 years in forehead width. The head width and head circumference showed continuous but mild growth rates throughout this period.
Growth Patterns of the Face: A Morphometric Study
Authors: Farkas Leslie G. , Posnick Jeffrey C. , Hreczko Tania M.
Published: October 2018

Age-related growth changes in the face and the relationships between its parts were calculated by using three vertical, two horizontal, and two projective surface measurements from 1,594 healthy North American Caucasians between 1 and 18 years of age. By 1 year, the width of the mandible was highly developed (80.2%) while its height reached only 66.6% of the eventual adult size. The mandible's height and width showed significant development between 1 and 5 years, while the face height, upper face height, face width, and the two face depth measurements exhibited continuous gradual growth after 5 years of age. In general, the face matured between 12 and 15 years in males and 2 years earlier in females. At 12 years, the upper face height, the mandible height, and the width of the face reached their mature size in females. At 15 years, the face height, mandible height, face width, and the depth of the mandible reached maturity in males.