bluuebunny/crossref_metadata_2025 huggingface dataset:
A Method for Three-Projection Infant Cephalometry
Authors: Hermann N. V. , Jensen B. L. , Dahl E. , Darvann T. A. , Kreiborg S.
Published: October 2018

Objective:

To assess morphology and growth in infants and children with craniofacial anomalies based on comprehensive digitization of radiographic films in three, mutually orthogonal projections.

Method:

The method consists of (1) acquisition of radiographic films in a highly standardized three-projection (lateral, frontal, and axial) cephalometer, (2) marking and digitization of a total of 279 anatomical landmarks in the three projections, and (3) computation and presentation (tabular and graphical) of 356 linear and angular variables describing the craniofacial morphology, including soft tissue. Computation of statistical entities describing a patient, a group of patients, the differences between patients or groups of patients was carried out. Error assessment of the method involved investigation of error distribution among a number of error sources. Duplicate digitization of radiographic films from 30 randomly selected patients, and from 10 dry skulls, was carried out to determine the errors contributed by the procedure of landmark digitization and the distribution of error among landmarks and variables, as well as between projections.

Results:

The average error due to landmark digitization, s(i), determined by duplicate digitization and calculated by use of Dahlberg's formula was 0.8 mm for linear variables and 1.6 degrees for angular variables.

Conclusion:

This method of infant cephalometry has been shown to be highly accurate and reproducible, and it adds significant new potential for, e.g., asymmetry detection, population comparison, and growth measurements compared to other cephalometric techniques due to its standardized acquisition and digitization protocol, inclusion of an axial projection, and the large number of well-defined landmarks and variables involved.
Incisive Suture (Fissure) in the Human Fetus: Radiographic and Histologic Study
Authors: Vacher Christian , Sakka Michel , Dauge Marie-Christine
Published: October 2018

Objective:

An anatomic study, in man, of the structure commonly known as the incisive suture (sutura incisiva) or incisive fissure has been performed to determine whether this structure belongs to the morphofunctional concept of a facial suture.

Material:

Eighteen palates of human fetuses of 9 weeks to 29 weeks after conception.

Method:

Anatomic, radiographic examination of the palate. Histologic study of sagittal and parasagittal sections of the palates (3 mm each).

Results:

The histologic aspect is that of a facial suture, with very poor vascularization. This suture is partial, limited laterally by osseous trabeculae.

Conclusions:

This particular suture, whose function in growth of the palatal process of the maxilla is discussed, could represent the phylogenetic vestige of the incisive-maxillary suture present in all nonhuman mammals.
Food and Drug Administration Regulation of Orthotic Cranioplasty
Authors: Littlefield Timothy R.
Published: October 2018

Objective:

To present information regarding the current federal regulation of cranial orthotics used for the treatment of deformational plagiocephaly as well as to discuss concerns raised by the Food and Drug Administration regarding the safety and effectiveness of these devices.

Background:

Although first introduced in 1979, the use of orthotic helmets for the treatment of deformational plagiocephaly was slow to gain acceptance. However, with the recent increase in infants presenting with this condition, numerous orthotic treatment programs have been established throughout the country. Until recently, federal regulation of this “industry” was largely ignored.

Regulation:

In 1995 our office was served notice that our orthosis would require clearance from the FDA. Since the FDA had never approved a medical device of this kind, clearance presented a significant challenge. However, after 3 years of providing clinical data, clearance was finally granted, and a new device category known generically as “cranial orthosis” was created. A cranial orthosis is considered to be a Class II neurology device and requires both general and special controls in order to ensure its safety and effectiveness.

Orthotics used for the treatment of deformational plagiocephaly are regulated by the FDA and are considered Class II neurology devices. Submission of a premarket notification (510[k]) is required prior to placing these devices on the market.
Patterns of Body and Visceral Growth in Human Prenates with Clefts of the Lip and Palate
Authors: Spyropoulos Meropi N. , Burdi Alphonse R.
Published: October 2018

Objective:

To address the hypothesis that human prenates with birth defects such as clefts of the lip and palate show growth patterns in which body size and visceral growth are aberrant for prenatal age.

Methods:

Body size and organ weight measurements were derived from a cross-sectional population sample of 167 legally donated human embryos that were representative of key periods in visceral organogenesis and growth. Measurements included those of overall body size (i.e., length and weight) and weights of major organs. Data were analyzed for two comparative (age-matched) groups consisting of 120 typical-for-age “control” and 47 specimens showing the three typical cleft types. Organ weight data for the entire cleft group and for each of the three cleft subgroups were compared with those of the control group.

Results:

Cleft specimens showed crown-rump body sizes that were within one standard deviation of the control group. Among the entire cleft sample, each of the organ weight values was generally consistent with the organ weights observed for the age-matched control group. When organ weights for the cleft group differed from the controls, with one exception, some organs showed either increased or decreased organ weights. One remarkable exception was with lung weights, which were reduced for each of the prenates across the three cleft types. Among the three cleft groups, fetuses with cleft lip and palate showed the highest percentage of organ weight discrepancies (i.e., smaller than expected) as compared with the group with cleft palate, which showed the lowest.

Conclusions:

Organ weight aberrancies were observable (whether increased or reduced weights), and such aberrancies were not apparent until approximately 24 weeks (fertilization age).
Investigations of a Pressure-Sensitive Theory of Marginal Velopharyngeal Inadequacy
Authors: Karnell Michael P. , Schultz Kara , Canady John
Published: October 2018

Purpose:

This two-part project was designed to test a pressure-sensitive theory of marginal velopharyngeal inadequacy (MPVI). Specifically, are select subgroups of children with MPVI perceived as hypernasal because they fail to achieve consistent closure during vowels and semivowels while demonstrating adequate closure during pressure consonants?

Methods:

In part one, 36 children with cleft palate and other craniofacial anomalies were examined using a clinical assessment protocol that included nasometry and perceived ratings of hypernasal resonance. Children with nasalance percentages above threshold during low-pressure (LP) productions and below threshold for high-pressure (HP) productions were placed in one group (group 1), while children with nasalance percentages below threshold for both LP and HP sentences were placed in another (group 2). Children in the two groups were age- and sex-matched. In part two, endoscopic data were examined for 10 additional children who received nasometry, perceived hypernasal resonance scores, and videoendoscopy on the same day and who received higher mean nasalance measures during production of LP sentences than during production of HP sentences.

Results:

The results of part one confirmed that children in group 1 were perceived as being significantly more hypernasal than children in group 2 (meangroup 1 = 2.17, meangroup 2 = 1.50; t = 2.75, p = .01). However, results of endoscopic testing failed to demonstrate a consistent observable physiologic pattern of velopharyngeal inadequacy that would confirm the theory that some patients with MVPI are perceived as being hypernasal because of difficulty achieving velopharyngeal closure during vowels and semivowels.

Conclusions:

The findings provide partial support for a pressure-sensitive theory of MVPI and demonstrate the value of using both HP and LP sentences to evaluate patients with MVPI.
Quality of Life in Adults with Repaired Complete Cleft Lip and Palate
Authors: Marcusson Agneta , Akerlind Ingemar , Paulin Gunnar
Published: October 2018

Objective:

This study evaluated the quality of life in adult Swedish subjects with repaired complete cleft lip and palate (CLP).

Design/Patients:

Sixty-eight adults with repaired CLP were compared with a group of 66 adults without cleft matched by gender and age.

Outcome Measures:

The outcome measures included a self-report questionnaire concerning quality of life in general, well-being, and health-related quality of life.

Results:

The CLP group rated their quality of life significantly lower than did the control group in the areas of life meaning (p < .05), family life (p < .0001), and private economy (p < .01). There were no significant differences between the groups concerning well-being. In the CLP group, the health-related quality of life was significantly lower concerning global life (p < .0001), disturbance to life (p < .01), well-being (p < .0001), social contacts (p < .0001), and family life (p < .05) but significantly higher concerning ability to make the most of leisure time (p < .001) and to be active (p < .001).

Conclusions:

The CLP group perceived a marked impact of the handicap on their lives concerning global aspects, well-being, and social life. More practical and tangible aspects of their daily living, however, were not affected, and only some minor aspects of their quality of life in general were poor in comparison with the control group, which indicates a fairly good life adjustment in spite of the handicap.
Two-Stage Reconstruction of Bilateral Alveolar Cleft Using Y-Shaped Anterior-Based Tongue Flap and Iliac Bone Graft
Authors: Kim Myung-Jin , Lee Jong-Ho , Choi Jin-Young , Kang Nara , Lee Jong-Hwan , Choi Won-Jae
Published: October 2018

Objective:

When an alveolar cleft is too large to close with adjacent mucobuccal flaps or large secondary fistula following a primary bilateral palatoplasty exists, a one-stage procedure for bone grafting becomes challenging. In such a case, we have used the tongue flap to repair the fistula and cleft alveolus followed by bone grafting to the cleft defect performed several months later. The purpose of this article is to report on our experiences with the use of an anteriorly based Y-shaped tongue flap to fit the palatal and labial alveolar defects and on the ultimate result of the bone graft.

Patients:

A series of 14 patients were treated with this approach from January 1994 to December 1998. The average age of the patients was 15.8 years (range 5 to 28 years). The mean period of follow-up following the second stage bone graft operation was 45.9 months (range 9 to 68 months). In 9 of the 14 patients, the long-fork type of a Y-shaped tongue flap was used for extended coverage of the labial-side alveolar defects with the palatal fistula; in the remaining patients, the short-forked design was used.

Results:

All patients demonstrated a good clinical result after the initial repair of cleft alveolus and palatal fistula. There was no fistula recurrence, although partial necrosis of distal margin in long-forked tongue flap occurred in one patient. Furthermore, the bone graft, which was performed an average of 8 months after the tongue flap repair, was always successful. Occasionally, transferred tongue tissue bulging interfered with the hygienic care of nearby teeth; however, these problems could be solved with proper contour-plasty performed afterward. No donor site complications such as sensory disturbance, change in taste, limitations in tongue movement, normal speech impairments, or tongue disfigurement were encountered.

Conclusion:

This two-stage reconstruction of a bilateral cleft alveolus using a Y-shaped tongue flap and iliac bone graft was very successful. It may be indicated for a bilateral cleft alveolus patient in which the direct closure of the cleft defect with adjacent tissue or the buccal flap is not easy because of scarred fibrotic mucosa and accompanied residual palatal fistula.
Histological Investigation of the Palatine Bone in Prenatal Trisomy 21
Authors: Lauridsen Halfdan , Fischer Hansen Birgit , Reintoft Ingermarie , Keeling Jean W. , Kjær Inger
Published: October 2018

Objective:

The purpose of the present study was to investigate the horizontal part of the palatine bone in palates from human fetuses with trisomy 21 to improve the phenotypic classification of the genotypic anomaly.

Methods:

Material from 23 human trisomy 21 fetuses was included in the study. The crown rump lengths of the fetuses ranged from 80 mm to 190 mm, corresponding to about 12 to 21 weeks of gestational age. The material was examined histologically.

Results and Conclusions:

Histological examination demonstrated four different palatal phenotypes on the basis of the development of the horizontal part of the palatine bone: type I, palatine bone complete; type II, the mesial region of the horizontal part of the palatine bone is lacking; type III, complete absence of the horizontal part of the palatine bone; and type IV, auxiliary bones in the region of the transpalatine suture. This finding shows that different types of malformations may occur in the horizontal part of the palatine bone in human trisomy 21 fetuses.
A New Approach to Classify Cleft Lip and Palate
Authors: Ortiz-Posadas M. R. , Vega-Alvarado L. , Maya-Behar J.
Published: October 2018

Objective:

To propose a new method, which allows for a complete description of primary and secondary cleft palates, incorporating elements that are related to the palate, lip, and nose that will also reflect the complexity of this problem.

Method:

To describe the type of cleft, two embryonic structures were considered: (1) the primary palate, formed by the prolabium, premaxilla, and columella and (2) the secondary palate, which begins at the incisive foramen and is formed by a horizontal portion of the maxilla, the horizontal portion of the palatine bones, and the soft palate. Anatomical characteristics to be considered were defined, and a new method is proposed to more fully describe any cleft.

Results:

A description of five cases was made using the method proposed in this work and compared with other published methods for the classification and description of clefts.

Conclusions:

A mathematical expression was developed to characterize clefts of the primary palate, including the magnitude of palatal segment separation and the added complexity of bilateral clefts, yielding a numerical score that reflects overall complexity of the cleft. Clefts of the secondary palate are also considered in a separate score. Using this method, it is possible to incorporate elements that are not considered in other approaches and to describe all possible clefts that may exist.
Enhanced Activity of Osteoblast Differentiation Factor (PEBP2αA2/CBFa1) in Affected Sutural Osteoblasts from Patients with Nonsyndromic Craniosynostosis
Authors: Shevde Nirupama K. , Bendixen Amy C. , Maruyama Midori , Ling Li Bai , Billmire David A.
Published: October 2018

Objective:

Nonsyndromic craniosynostosis is characterized by premature closure of one or more cranial sutures in infants. The purpose of this investigation was to evaluate cellular and molecular events that lead to pathogenesis of nonsyndromic craniosynostosis.

Design:

This study utilized discarded samples of normal and affected cranial sutures from 12 patients (7 boys, 5 girls) with nonsyndromic craniosynostosis.

Results:

Histological evaluation of affected sutures revealed complete osseous obliteration instead of a zone of connective tissue and osteogenic cells as seen in normal sutures. Although proliferation of normal and affected osteoblasts did not vary substantially, elevated osteocalcin production and increased in vitro bone nodule formation indicated that the differentiation and the bone-forming potential of affected osteoblasts was significantly higher than that of normal cells. We therefore investigated the levels and activity of Cbfa1, a transcription factor that plays an integral role in osteoblast differentiation. Northern blot analysis of messenger RNA from both normal and affected sutural osteoblasts revealed a twofold increase in the expression of Cbfa1 in affected cells. This increase in the level of Cbfa1 transcript correlated with an increase in its transcriptional activity on the osteocalcin gene promoter, as assessed using gene transfer methods.

Conclusion:

Our results indicated that osteoblasts from synostosed sutures exhibit an increased potential for differentiation and bone formation. The increased level and activity of Cbfa1 could play a vital role in the aberrant function of these affected osteoblasts and may explain their altered behavior compared to the normal cells.
Comparison of the Effect of Supraperiosteal versus Subperiosteal Dissection on the Growing Rabbit Maxilla
Authors: Mannucci Nicola , D'orto Ornella , Biglioli Federico , Brusati Roberto
Published: October 2018

Objective

To achieve closure of wide unilateral cleft lip repair without tension of the cleft lip margins, a large undermining, especially of the anterolateral surface of the maxilla, is needed. Two types of dissection are feasible: supraperiosteal or subperiosteal. The aim of this study was to investigate whether there are differences in maxillary growth between healthy rabbits after supraperiosteal or subperiosteal dissection.

Methods

Twenty-four male 7-week-old New Zealand white rabbits were divided randomly into three groups: eight control animals (untreated); eight animals undergoing supraperiosteal dissection of the left surface of the maxilla, and eight animals undergoing subperiosteal dissection of the left surface of the maxilla. All of the treated animals were operated on by the same surgeon at age of 7 weeks and sacrificed at 27 weeks together with control group animals. Seven cephalometric measures (representing aspects of maxillary length, width, and height), on the left side, were taken on the cleaned skull of the rabbits, and the results were analyzed statistically.

Results

No significant differences in maxillary growth were noted across the three study groups.

Conclusions

The supraperiosteal or subperiosteal undermining of the anterolateral surface of the maxilla does not seem to interfere with the growth of the normal maxilla in the rabbit.
A Child with Saethre-Chotzen Syndrome, Sensorineural Hearing Loss, and a TWIST Mutation
Authors: Lee Samson , Seto Marianne , Sie Kathy , Cunningham Michael
Published: October 2018

Objective

Patients with syndromic craniosynostosis may have associated hearing deficits. A review of hearing loss associated with syndromic craniosynostosis as well as implications of cochlear implantation in the craniosynostosis patients is presented. In the literature, patients with Saethre-Chotzen syndrome have been shown to have conductive or mixed hearing losses. This case report describes a patient with Saethre-Chotzen syndrome caused by a mutation in the TWIST gene who exhibits a severe to profound sensorineural hearing loss.
The Impact of Early Palatal Obturation on Consonant Development in Babies with Unrepaired Cleft Palate
Authors: Hardin-Jones Mary A. , Chapman Kathy L. , Wright Jane , Halter Kelli Ann , Schulte Julie , Dean Jeffrey A. , Havlik Robert J. , Goldstein Jeffrey
Published: October 2018

Objective

The purpose of this investigation was to determine whether palatal obturators enhance consonant development during babbling for babies with unrepaired cleft palate.

Participants

Fourteen babies with cleft palate who had worn anterior palatal obturators prior to palatal surgery were matched to 14 unobturated babies according to cleft type, sex, and age at time of presurgical evaluation.

Main outcome Measures

Spontaneous vocalizations of the obturated and unobturated groups were compared to determine whether differences were evident in size of consonant inventory as well as place and manner of consonant production.

Results

Paired t tests revealed no significant differences between the groups in size of consonant inventory or place and manner of consonant production. There was a trend for babies in the obturated group to produce more glottal consonants.

Conclusions

In general, the findings of this study suggested that palatal obturators do not appear to facilitate production of anterior palatal consonants during babbling.
Prenatal Diagnosis: When the Clinician Disagrees with the Patient's Decision
Authors: Saal Howard M.
Published: October 2018

Objective

Recent reports of prenatal diagnosis of cleft lip and cleft palate resulting in pregnancy termination have raised questions about the appropriateness of such decisions. This review attempts to clarify the importance of nondirective counseling and define the role of the clinician, especially in the face of difficult and often controversial decisions.

Design

Genetic counseling literature was reviewed to clarify the role of genetic counseling in prenatal diagnosis within the framework of different counseling approaches.

Conclusions

The ultimate decision regarding pregnancy outcome and management, including termination, belongs to the pregnant couple. Clinicians will often have their own thoughts about what decisions they would make when faced with certain clinical situations. However, the responsibility of clinicians is to help the couple make their own decisions based upon their personal religious, moral, and cultural values.
Effects of Calcium Phosphate Ceramic Bone Graft Materials on Permanent Teeth Eruption in Beagles
Authors: Linton Jina Lee , Sohn Byung-Wha , Yook Jong-In , Le Geros Racquel Z.
Published: October 2018

Objective

The aim of this study was to evaluate the use of calcium phosphate ceramic (CPC) materials as a potential alternative to autogenous secondary alveolar bone grafting in cleft lip and palate patients who are in mixed dentition.

Sample

Four 12-week-old beagles and one 15-week-old beagle were used as subjects.

Interventions

In each experimental beagle, the third and fourth deciduous premolars were extracted. The sockets were filled with four different CPC materials and sutured.

Main outcome Measure

The beagles were fed a soft diet for the following 8 weeks and then sacrificed for clinical, radiological, histological, transmission electron microscope (TEM), and infrared (IR) absorption analysis.

Results

All four experimental graft materials allowed normal development and eruption of permanent premolars. In histological sections, small particles of biphasic calcium phosphate (BCP) and carbonate apatite (CO3-AP) were resorbed, and large particulate forms served as bone frames in cortical bones. Polymer coated with carbonate apatite (Poly/CO3-AP) did not cause inflammation but was pushed away to the soft tissue by erupting teeth. Alginate coated with carbonate apatite (alginate/CO3-AP) caused a severe inflammatory reaction to the point of destroying a part of the dental follicle and cortical bone. In TEM, resorption activity by phagocytic cells was observed only in CO3-AP. Direct bonding of CO3-AP to the bone was observed as the electron-dense interface between bone and CO3-AP.

Conclusion

BCP and CO3-AP proved to be suitable as alveolar bone graft materials in areas where tooth eruption occurs. Of the four materials tested, CO3-AP produced the best results.
Modified Z-Plasty Repair of Webbed Neck Deformity Seen in Turner and Klippel-Feil Syndrome
Authors: Hikade Katie R. , Bitar George J. , Edgerton Milton T. , Morgan Raymond F.
Published: October 2018

Objective:

Webbed neck deformity exists in many syndromes including Turner's or Klippel-Feil syndrome. Multiple problems are encountered with existing techniques to correct a webbed neck deformity. In Turner's syndrome, a subcutaneous band of thickened fascia and a low neck hairline present a challenge to the surgeon when designing a repair. The authors propose the following new technique that addresses both issues.

Material and Methods:

Five patients with webbed neck underwent this new procedure. A Z-plasty is performed with the midline arm down the length of the web. The subcutaneous fibrous band is excised, the shortened trapezius is released, and the hair-bearing flap is excised. The anterior flap is rotated and advanced to join its mate flap from the contralateral neck at the posterior midline. A resultant dog-ear near the acromion is corrected with an additional Z-plasty.

Results:

In all five patients, the functional and aesthetic results were very satisfactory to both patient and surgeon. An 11-year follow-up is presented with excellent correction of the webbing. Both limited range of motion and the cosmetic deformity are addressed by this technique.

Conclusion:

The results obtained by using the simplified modified Z-plasty technique for repair of webbed neck deformity are very satisfactory. We propose the use of this technique for correction of webbed neck deformities whenever the posterior surface of the neck web contains a significant amount of hair.
Efficacy of Continuous Positive Airway Pressure for Treatment of Hypernasality
Authors: Kuehn David P. , Imrey Peter B. , Tomes Lucrezia , Jones David L. , O'Gara Mary M. , Seaver Earl J. , Smith Bonnie E. , Van Demark D.R. , Wachtel Jayne M.
Published: October 2018

Objective:

To determine whether speech hypernasality in subjects born with cleft palate can be reduced by graded velopharyngeal resistance training against continuous positive airway pressure (CPAP).

Design:

Pretreatment versus immediate posttreatment comparison study.

Setting:

Eight university and hospital speech clinics.

Patients:

Forty-three subjects born with cleft palate, aged 3 years 10 months to 23 years 8 months, diagnosed with speech hypernasality.

Intervention:

Eight weeks of 6 days per week in-home speech exercise sessions, increasing from 10 to 24 minutes, speaking against transnasal CPAP increasing from 4 to 8.5 cm H2O.

Main Outcome Measure:

Pretreatment to immediate posttherapy change in perceptual nasality score based on blinded comparisons of subjects’ speech samples to standard reference samples by six expert clinician-investigators.

Results:

Participating clinical centers treated from two to nine eligible subjects, and results differed significantly across centers (interaction p = .004). Overall, there was statistically significant reduction in mean nasality score after 8 weeks of CPAP therapy, whether weighted equally across patients (mean reduction = 0.20 units on a scale of 1.0 to 7.0, p = .016) or across clinical centers (mean = 0.19, p = .046). This change was about one-sixth the maximum possible reduction from pretreatment. Nine patients showed reductions of at least half the maximum possible, but hypernasality of eight patients increased at least 30% above pretreatment level. Most improvement was seen during the second month when therapy was more intense (p = .045 for nonlinearity). No interactions with age or sex were detected.

Conclusion:

Patients receiving 8 weeks of velopharyngeal CPAP resistance training showed a net overall reduction in speech hypernasality, although response was quite variable across patients and clinical centers. The net reduction in hypernasality is not readily explainable by random variability, subject maturation, placebo effect, or regression to the mean. CPAP appears capable of substantially reducing speech hypernasality for some subjects with cleft palate.
Simultaneous Cortex Bone Plate Graft with Particulate Marrow and Cancellous Bone for Reliable Closure of Palatal Fistulae Associated with Cleft Deformities
Authors: Ishii Masatoshi , Ishii Yoshimasa , Moriyama Takashi , Gunji Akemi , Morita Keiichi , Imaizumi Fumiko , Enomoto Shoji
Published: October 2018

Objective:

The purpose of this study was to evaluate the effectiveness of simultaneous cortex bone plate (CBP) graft with particulate marrow and cancellous bone (PMCB) graft for reliable closure of palatal fistulae associated with alveolar clefts.

Design:

Following standard secondary bone graft preparation of the cleft site, CBP harvested from the medial iliac crest was inserted into the palatal deficiency. This was followed by suturing the palatal mucosa. PMCB was then packed between the cortical bone and the reconstructed nasal floor.

Setting:

Ten consecutive patients with palatal fistula were operated on at Tokyo Medical and Dental University Hospital from 1998 to 2000. Primary palatal repair was performed in 7 out of 10 patients at our center and in 3 out of 10 patients at other hospitals.

Patients:

Ten patients (6 boys and men, 4 girls and women) with a palatal fistula associated with an alveolar cleft were studied. Ages ranged from 12 to 26 years.

Interventions:

All patients underwent simultaneous CBP graft with PMCB graft for closure of palatal fistula under general anesthesia.

Results:

Complete closure of palatal fistulae were obtained in 8 out of 10 cases. A very small asymptomatic fistula remained in one patient. Total necrosis of the labial flap with a residual palatal fistula occurred in one patient.

Conclusions:

Simultaneous CBP graft with PMCB graft could be more reliable than PMCB alone for closure of a cleft associated palatal fistula.
Hereditary Cleft Lip/Palate and Wilms Tumor: A Rare Association
Authors: Yu Chung-Chih , Wong Fen-Hwa , Lo Lun-Jou , Chen Yu-Ray
Published: October 2018

Objective:

The association of cleft lip/palate (CLP) with other anomalies is not uncommon, but its association with Wilms tumor (WT) is very rare, especially in a familial pattern. In this report, we present a family in which six members in two generations were affected with CLP, WT, or both.

Patients and Results:

A male patient presented with right complete CLP. He had a family history of facial cleft and abdominal tumor. Lip repair was performed at 3 months of age. An abdominal mass was noticed at 12 months of age, which proved to be WT. Surgical excision of the tumor and chemotherapy were conducted. He subsequently underwent palate repair. His father had an unrepaired microform cleft lip. Three of his aunts were known to have similar problems: one had both facial cleft and WT, one had WT only, and the other had facial cleft only. One of his cousins also was affected with WT.

Conclusions:

This is a unique family affected with a rare association of CLP and WT. Pedigree study revealed an autosomal dominant hereditary pattern.
Endoscopic Partial Adenoidectomy for Children with Submucous Cleft Palate
Authors: Finkelstein Yehuda , Wexler David B. , Nachmani Ariela , Ophir Dov
Published: October 2018

Objective:

Children with submucous cleft palate who suffer from chronic nasal obstruction because of hypertrophic adenoids usually are not subjected to adenoidectomy because of the fear of postoperative velopharyngeal insufficiency. These patients present a therapeutic challenge because we are aware more than ever of the importance of normal nasal breathing and nocturnal respiration, especially during childhood. Our hypothesis was that transnasal endoscopic horizontal limited adenoidectomy may relieve nasal obstruction while preserving the function of the velopharyngeal valve. The objective of this study was to evaluate the efficacy of transnasal endoscopic horizontal partial adenoidectomy in patients with submucous cleft palate and adenoidal hypertrophy.

Setting:

Patients were either referred to the outpatient clinic of the Palate Surgery Unit (seven patients) or were patients referred to the senior author's (Y.F.) private clinic. All the patients had been operated on by this senior author (Y.F.).

Patients:

Ten children aged 3.5 to 13 years (six girls and four boys) with submucous cleft palate and hypertrophic adenoids were included in the study. All the patients were hyponasal and suffered nasal obstruction, loud snoring, and episodes of apnea.

Interventions:

Endoscopic partial adenoidectomy was accomplished to open the lower third of the choanae. Nasal breathing was achieved in all the patients, and only mild snoring remained in two patients. The hyponasality disappeared and speech intelligibility normalized. Mild hypernasality developed in two patients but was still perceived as an overall improvement in speech.

Conclusions:

Transnasal endoscopic horizontal partial adenoidectomy may be an effective surgical method for relief of nasal obstruction while preserving velopharyngeal valve function in patients with submucous cleft palate who suffer from obstructive adenoids.
A Local Perspective on the Initial Management of Children with Cleft Lip and Palate by Primary Care Physicians
Authors: Grow Jennifer L. , Lehman James A.
Published: October 2018

Objective:

To evaluate the frequency and referral patterns, need for continuing education, and information given to parents of children with cleft lip and palate by local primary care physicians.

Study Design:

A survey was sent to primary care physicians from the pediatrics, family practice, and internal medicine/pediatrics specialties in six surrounding counties of a regional craniofacial center located within northeastern Ohio with a population base of 1.5 million people. Particular aspects of care evaluated included protocols of care, information for parents, referral patterns, conferences attended, and literature read.

Results:

Nearly 30% of physicians had standard protocols when children are born with clefts. Eighty-seven percent of physicians referred children within 2 weeks of diagnosis, yet only 48% gave referrals to a multidisciplinary team. Physicians providing team referrals were nearly three times as likely to attend related conferences and approximately four times as likely to have a set plan at the time of diagnosis of the cleft. Forty-nine percent of physicians gave oral information, 35% gave oral and written information, and 10% did not give parents information. Ninety-four percent of physicians gave parents the option to call with concerns. Pediatricians were approximately four times as likely to attend cleft conferences and desire continuing education updates. However, family practitioners were over three times as likely to give proper referrals, compared with pediatricians.

Conclusion:

There is a necessity to educate local primary care physicians who manage children with cleft lip and palate regarding cleft information for parents, referral guidelines, the cleft team concept, and the role of primary physicians.
Onlay Bone Graft Augmentation for Refined Correction of Coronal Synostosis
Authors: Grant John H. , Roberts Theodore S. , Loeser John D. , Gruss Joseph S.
Published: October 2018

Objective:

The primary purpose of this study was to evaluate the long-term result of an onlay bone graft augmentation of the supraorbital ridge at the time of primary correction of coronal suture synostosis.

Design:

The study is a retrospective review of 62 consecutive patients treated for coronal synostosis from June 1991 through February 1997. The surgical technique utilized involved a standard bilateral fronto-orbital advancement and calvarial reshaping with the addition of an onlay bone graft in the supraorbital region.

Setting:

All patients were treated at a tertiary care craniofacial center.

Results and Conclusion:

A total of 62 patients were treated by this technique. Fifty patients underwent primary correction as infants (mean age 9.8 months). An additional 12 patients were older (mean age 8.2 years) and were treated for residual deformity having previously undergone correction by another technique. Results with follow-up as long as 7 years demonstrate stable forehead and orbital symmetry. Complications identified by chart review were minimal and not directly attributable to this modification in surgical technique.
Validity of Subjective Evaluations for the Assessment of Lip Scarring and Impairment
Authors: Ritter Kelly , Trotman Carroll-Ann , Phillips Ceib
Published: October 2018

Objective

In patients with cleft lip and palate, the aim of the study was (1) to determine and compare the level of agreement among examiners’ subjective evaluations of static and dynamic lip form; (2) assess possible bias of examiners’ subjective evaluations; and (3) determine the impact of lip scarring on an examiner's subjective assessment of dynamic lip form.

Setting

Patients and subjects were recruited from the University of North Carolina Cleft Lip and Palate Center and School of Dentistry.

Patients, Participants

Thirteen patients with unilateral cleft lip and palate and varying degrees of cleft scar severity were selected and one subject without cleft who wore artificial scars of varying severity.

Interventions

For the patients with cleft, a previously repaired complete cleft lip and palate. Photographs and videotape recordings were made of the patients with cleft and the subject without cleft, with and without the artifical scars, at rest and smiling.

Main Outcome Measure(s)

Rankings of cleft scar severity and impairment on a 6-point Likert scale by a lay and professional panel.

Results

Intra- and interexaminer reliability was good for the lower facial regions at rest but not during movement. Professionals gave ratings of greater severity and impairment than laypersons, and professionals agreed when rating the lower faces at rest more so than during movement. Lip scarring affected perceptions of impairment during movement by viewers in both panels.

Conclusions

Subjective assessments can be affected by methodological approaches, professional experience, and stimulus type. Future research should focus on establishing objective methods to evaluate patients with cleft lip and palate at rest and during function.
A Profile of the Features and Speech in Patients with Mandibulofacial Dysostosis
Authors: Vallino-Napoli Linda D.
Published: October 2018

Purpose

To present a profile of the features and speech in patients with mandibulofacial dysostosis (MFD). Data were collected on occlusion, palatal condition, hearing, resonance, voice, and articulation.

Patients

Thirty patients with MFD ranging in age from 1.6 to 21.0 years.

Study Design

Retrospective and prospective cross-sectional designs.

Setting

Pediatric tertiary care hospital.

Results

Sixty percent of the patients had an open bite. Isolated cleft palate was found in 37% with other types of cleft conditions occurring less frequently. Twenty-three percent underwent tracheostomy. All patients demonstrated hearing loss, 93% were conductive and 7% were mixed. Resonance, voice, and articulation were also affected. Seventy-seven percent had aberrant resonance including hypernasality, hyponasality, mixed hyper- and hyponasality or muffled resonance, which was found in 40% of the patients. Voice quality was abnormal in 63%. All patients had articulation errors. Although overlap between categories occurred, results showed that 60% had errors related to malocclusion, 30% demonstrated errors usually associated with velopharyngeal inadequacy and 50% had general articulatory or phonological errors that could be attributed to other causes.

Conclusions

The features and speech of patients with MFD are complex. The speech disorders may have multiple overlapping etiologies that require careful differential diagnosis. This is imperative to establish appropriate treatment regimens and evaluate clinical outcomes.
The Relationship between Arch Dimensions and the 5-Year Index in the Primary Dentition of Patients with Complete UCLP
Authors: Dibiase A.T. , Dibiase D.D. , Hay N.J. , Sommerlad B.C.
Published: October 2018

Objective

To compare dental arch dimensions of children in the primary dentition with repaired unilateral clefts of the lip and palate (UCLP) to a noncleft group of a similar age and determine how the dimensions of the cleft arches relate to an index of treatment outcome.

Method

Dental study casts of 44 5- to 6-year-olds with complete UCLP (22 boys and 22 girls) from a single center, whose primary surgery had been carried out by one surgeon, were matched for age, sex, and ethnicity with dental study casts from a longitudinal growth study. Analysis of variance was used to ascertain differences in arch dimensions between the two groups. The cleft group casts were then assessed with an established index of surgical outcome, the 5-year-old index. Spearman's rank correlation coefficient was used to see how the arch dimensions of the cleft group related to the categories of the index.

Results and Conclusions

Maxillary arch dimensions were significantly smaller in the cleft group than in the noncleft group, irrespective of sex (p < .05). In the mandibular arch, there was no difference between the cleft and noncleft groups (p > .05). Maxillary arch dimensions of the cleft group correlated significantly with the 5-year-old index for arch length and intercanine width (p < .05) but not intermolar width (p = .842). This would suggest that the 5-year-old index is a suitable tool for assessing the outcome of treatment in the primary dentition for anteroposterior and anterior transverse arch dimensions.
Volume and Shape of Masticatory Muscles in Patients with Hemifacial Microsomia
Authors: Takashima Mariko , Kitai Noriyuki , Murakami Shumei , Furukawa Souhei , Kreiborg Sven , Takada Kenji
Published: October 2018

Objective

To test the following hypotheses in hemifacial microsomia (HFM): (1) the volumes of the masseter, lateral and medial pterygoid, and temporal muscles are reduced on the affected versus unaffected side; (2) significant differences exist between the degrees of right-left disproportion in these four masticatory muscles; (3) circumferential shapes of the masticatory muscles are more irregular on the affected versus unaffected side; and (4) the degree of masticatory muscle right-left disproportion can be judged by the degree of ear, mandibular, or dental anomalies.

Subjects and Methods

Ten preadolescent patients with HFM were studied using facial photographs, dental casts, cephalometric and panoramic radiographs, and helical computed tomography scanning and three-dimensional reconstruction technique. Volumes of masseter, lateral and medial pterygoid, and temporal muscles on both sides were measured. Muscle volume disproportion was expressed as the affected/unaffected ratio. Muscle circumferential irregularity was expressed as the ratio between the total circumferential length and correspponding cross-sectional area.

Results

Masticatory muscle volumes were significantly smaller on the affected versus unaffected side. No significant differences were observed between the degrees of disproportion of the four masticatory muscles examined. Circumferential shapes of masticatory muscles were significantly more irregular on the affected versus unaffected side. There were no significant relationships of the degree of ear, mandibular, or dental anomalies in relation to masticatory muscle disproportion.

Conclusions

In HFM the masseter, lateral and medial pterygoid, and temporal muscles all have a significantly smaller volume on the affected versus unaffected side, and specific muscles were not severely affected in the present subjects. Furthermore, all four muscles showed a significantly more irregular shape on the affected versus unaffected side. Finally, the severity of masticatory muscle disproportion can probably not be judged by the degree of ear, mandibular, and dental anomalies in preadolescent patients with HFM.
Effects of Orthognathic Surgery on Speech and Breathing of Subjects with Cleft Lip and Palate: Acoustic and Aerodynamic Assessment
Authors: Trindade Inge E.K. , Yamashita Renata P. , Suguimoto Roberto M. , Mazzottini Reinaldo , Trindade Alceu S.
Published: October 2018

Objective

To evaluate the impact of orthognathic surgery on acoustic nasalance of subjects with cleft and investigate the causes of possible changes by analyzing velopharyngeal function and nasal patency.

Design/Patients

Nasalance was measured in 29 subjects with operated cleft palate ± lip before (PRE) and 45 days (POST1) and 9 months (POST2) after surgery, on average. In 19 of the patients, the minimum velopharyngeal (VP) and nasal cross-sectional (N) areas were also determined.

Interventions

Le Fort I osteotomy with maxillary advancement in combination with procedures involving the nose, maxilla, mandible or all three.

Main Outcome Measures

Nasalance, VP area, N area.

Results

We observed: (1) a significant (p < .05) increase in mean nasalance at POST1 and POST2, compared with PRE during the reading of oral sentences and nasal sentences; at POST2, high nasalance on the oral sentences was observed in 45% of the patients with normal nasalance at PRE, and 57% of patients with low nasalance on the nasal sentences at PRE no longer presented abnormal nasalance; (2) a significant increase in mean VP area at POST1; two borderline patients demonstrated deterioration of VP closure at POST2, compared with PRE; and (3) a significant increase in mean N area at POST2, with 73% of patients no longer presenting subnormal areas seen at PRE.

Conclusions

On a long-term basis, orthognathic surgery modifies speech nasalance of some subjects with cleft, perhaps because of an increase in internal nose size. This may also improve nasal patency for breathing.
Bilateral Superiorly Based Full-Thickness Nasolabial Island Flaps for Closure of Residual Anterior Palatal Fistulas in an Unoperated Elderly Patient
Authors: Erçöçen A.R. , Yilmaz S. , Saydam M.
Published: October 2018

Objective

Unoperated bilateral complete cleft lip and palate in an adult or elderly patient is seen rarely, and the existence of unoperated clefts is a result of unfavorable economic and social circumstances. We report an unoperated 65-year-old patient with bilateral complete cleft lip and palate and present our preference for the surgical management.

Interventions

Repair of the bilateral complete cleft lip and palate was successfully carried out using straight-line closure for the bilateral cleft lip and two-flap pushback palatoplasty with superiorly based lateral port control pharyngeal flap for the wide cleft palate at the first stage, and large residual anterior palatal fistulas were closed using bilateral superiorly based (retrograde flow) full-thickness nasolabial island flaps at the second stage.

Conclusions

To our knowledge based on a review of the literature, this is the first report of an elderly patient with bilateral complete cleft lip and palate and the first application of bilateral superiorly based (retrograde flow) full-thickness nasolabial island flaps for closure of large residual anterior palatal fistulas or alveolar clefts. The bilateral superiorly based (retrograde flow) full-thickness nasolabial island flap may be a good solution in large anterior palatal defects using unilaterally or bilaterally in a single stage with minimal donor site morbidity, in which there is not enough tissue for local repair or if previous attempts are unsuccessful.
Syngnathia and Van der Woude Syndrome: A Case Report and Literature Review
Authors: Puvabanditsin Surasak , Garrow Eugene , Sitburana Orawan , Avila Filipe M. , Nabong Marcelo Y. , Biswas Anjali
Published: October 2018

Objective

Syngnathia is a rare anomaly involving soft tissue or bony adhesions between the maxilla and mandible. We review the literature and present a case of syngnathia associated with Van der Woude syndrome. Syngnathia can have very different etiologies, but this has rarely been reported in Van der Woude syndrome. Treatment of this condition is rarely discussed in the literature because of a paucity of case reports.

Results

Oral adhesion (syngnathia) in our patient was caused by a mandibular to maxillary fibrous band. Surgical treatment was successful. Clinical implications and review of literature for the treatment of this rare association of syngnathia and Van der Woude syndrome are discussed.