bluuebunny/crossref_metadata_2025_split huggingface dataset:
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.