bluuebunny/crossref_metadata_2025_split huggingface dataset:
Functional Analysis of Cleft Lip and Palate Patients with Modified Rakosi Method
Authors: Akcam M. Okan , Altiok Tunç , Özdiler F. Erhan 
Published: October 2018

Objective The aim of this study was to investigate the mandibular functions of patients with cleft lip and palate (CLP) by means of modified Rakosi Functional Analysis and to test whether there was a risk of tendency to temporo-mandibular joint disorder when compared with Angle Class I non-CLP patients. Design Fifteen patients with unilateral CLP with a mean age of 12.2 ± 1.91 years and randomly selected 15 Angle Class I orthodontic patients with a mean age of 10.5 ± 2.01 years as a control group. None of the subjects had complaint of symptoms of temporomandibular joint disorder in their histories. All subjects were evaluated using modified Rakosi Functional Analysis method in accordance with a scoring system of the findings. Main outcome Measures Intra- and extracapsular clinical functional surveys were taken. A Mann-Whitney U test was used to evaluate the differences between the CLP and non-CLP groups. Results and Conclusion There was a significant difference (p < .01) between the distributions of the total scores of the two groups. A significant difference (p < .05) in right lateral movement was also observed between the two groups. However, there were no significant but borderline differences in clicking, crepitus, and maximum mouth opening findings between the two groups. Because these results do not explain the reason for the aforementioned differences, further studies seem essential.
Airway Obstruction following Palatoplasty: Analysis of 247 Consecutive Operations
Authors: Antony Anuja K. , Sloan Gerald M. 
Published: October 2018

Objective and Methods Between February 1987 and September 1997, 247 patients underwent primary repair of a cleft of the secondary palate by one surgeon, using the double-opposing Z-plasty (Furlow) technique. This retrospective study reviews perioperative and postoperative airway compromise among these patients. The purposes of this study were to identify factors associated with airway obstruction following palatoplasty and to analyze the management of those patients. Although infants experiencing airway problems following Wardill-Kilner and Von Langenbeck palatoplasty have been described, airway complications in a group of Furlow repair patients has not been previously reported. Results Fourteen patients (5.7%) had airway problems. The average age of these patients was 18 months, which was not significantly different from those without airway problems. Airway obstruction occurred as late as 48 hours after the completion of surgery. Twelve of the 14 patients had severe airway compromise requiring continued postoperative intubation, reintubation, or tracheostomy (one). There were no deaths. Thirteen of the 14 patients with postoperative airway problems (93%) had other congenital anomalies in addition to clefting, a named congenital disorder, or both. Seven of those 13 had Pierre Robin sequence. In contrast, only 40 of the 233 patients without airway problems (17%) had additional congenital anomalies or named disorders. Presence of other congenital anomalies was associated with a significantly increased risk of airway obstruction (p = .005). Conclusion Patients with cleft palate with the Pierre Robin sequence or other additional congenital anomalies had an increased risk of airway problems following palatoplasty. Awareness of this risk permits identifying those patients prior to surgery so that they can be monitored and managed appropriately, minimizing the likelihood of major complications or death.
Beyond Easy Answers: Prenatal Diagnosis and Counseling during Pregnancy
Authors: Strauss Ronald P. 
Published: October 2018

Objective The advancing sophistication and availability of prenatal diagnostic technologies, such as transvaginal ultrasound, chorionic villus sampling, amniocentesis, and alpha feto-protein testing, have increased the medical capacity to detect genetic and congenital conditions during pregnancy. This paper raises many social and ethical questions about how families, craniofacial teams, and society respond when a prenatal diagnosis is made and considers the ethical and social issues around counseling, managing information, and making decisions. Design Ethical and sociological analysis. Setting Implications examined on the societal, health professional, and family level. Results Families and health professionals often manage ambiguity, uncertainty, and complex decision making in facing a prenatal diagnosis. Embedded in parental and clinical decisions are values about children with birth defects. Families are making decisions about whether to bear or abort an affected fetus on the basis of their perceptions of the impairment and on their expectation of the burden involved for the family and the child. On a broader, societal level, pressures to conform and minimize human differences are apparent in bio-medical interventions, the Human Genome Project, advertising and media images, and social pressures to normalize disabilities. How society deals with prenatal diagnosis will impact upon social values; moral, legal, and ethical perspectives; and on health policy. Conclusion Prenatal diagnostic technologies raise complex ethical, family, policy, and legal issues that have broad implications for the lives of children born with special health care needs, including children with cleft lip and palate.
Prenatal Diagnosis of Cleft Lip and Palate: Detection Rates, Accuracy of Ultrasonography, Associated Anomalies, and Strategies for Counseling
Authors: Jones Marilyn C. 
Published: October 2018

Objective As ultrasound becomes more widely utilized in pregnancy and imaging technology improves, cleft lip and palate will become more commonly identified in prenatal life. In efforts to meet the needs for information regarding cause and management, pregnant women and their partners are increasingly referred to cleft and craniofacial treatment programs. This group of patients provides unique challenges to professionals unfamiliar with the issues inherent to this population. Information regarding the extent of the defect and the absence of associated abnormalities is usually incomplete. Treatment teams may be uncomfortable with the possibility that couples may choose not to continue a pregnancy on the basis of what they hear. Currently between 14% and 25% of cleft lip, with or without cleft palate, is detected antenatally. About 12% of presumably isolated clefts will be one feature in a broader pattern of malformation. This article reviews the current status of ultrasound in the detection of clefts during pregnancy and outlines a strategy for counseling based on the author's experience in both a prenatal diagnosis program and a cleft-craniofacial treatment team.
Dental Development of Permanent Lateral Incisor in Complete Unilateral Cleft Lip and Palate
Authors: Ribeiro Luciana Lourenço , Neves Lucimara Teixeira Das , Costa Beatriz , Gomide Marcia Ribeiro 
Published: October 2018

Objective The objective of this retrospective study was to verify whether the root development of the permanent lateral incisor on the cleft side was delayed, compared with its contralateral tooth in subjects with complete unilateral cleft lip and palate. Setting Craniofacial Anomalies Rehabilitation Hospital (HRAC)-University of São Paulo-Bauru, Brazil. Patients A sample of 98 panoramic radiographs of HRAC patients with complete unilateral cleft lip and palate was selected, regardless of sex and race. Main outcome Measure Panoramic radiographs were analyzed and the root development stage of the cleft-side permanent lateral incisor was compared with its contralateral tooth. Results Overall, the permanent cleft-side lateral incisor was significantly delayed in root development in relation to the noncleft side contralateral tooth (p < .05). However, no significant differences were observed between boys and girls. Conclusions Root development of the permanent cleft-side lateral incisor was delayed, compared with its contralateral tooth. The cleft itself may play an important role in this delay because the results from boys and girls were very similar.
Modifying the Two-Stage Cleft Palate Surgical Correction
Authors: Chait Laurence , Gavron Gerald , Graham Cathy , Noik Errol , De Aguiar Gavin 
Published: October 2018

Objective This paper reports the experience with a two-stage approach to surgical correction of the complete cleft palate, wherein timing of the second stage is dependent on the judgment of the speech pathologist and the orthodontist together with the surgeon. Patients Of a total of 35 patients having complete unilateral clefts a sample of 22 were available for postsurgical assessment. The first-stage repair of the palate was carried out at an average age of 10.7 months (range 6 to 17 months), and the second-stage repair of the residual cleft was completed at an average age of 32.7 months (range 26 to 34 months). Interventions The first-stage repair of the soft palate defect involved mobilizing two short posteriorly based flaps, which extend onto the posterior quarter of the hard palate thus including up to 1 cm of mucoperiosteum. Careful freeing of the muscle is followed by an intravelar veloplasty. The later closure of the residual cleft involved turnover hinge flaps and small mucoperiosteal flaps. Results Eighty-seven percent of the sample had good to excellent speech as assessed by the Great Ormond Street screening method. Only two patients showed evidence of recessive maxillae with Class III malocclusions. Conclusions A two-stage surgical closure of the palate using this procedure would appear to confer several valuable advantages to the patient. These include favorable outcomes for speech in the large majority of cases and minimal adverse effects on the growth of the midface region.
The Correlation between Nasalance and a Differentiated Perceptual Rating of Speech in Dutch Patients with Velopharyngeal Insufficiency
Authors: Keuning Kornelis H.D.M. , Wieneke George H. , Van Wijngaarden Hans A. , Dejonckere Philippe H. 
Published: October 2018

Objective: The correlation between the nasalance score and the perceptual rating of several aspects of speech of speakers with velopharyngeal insufficiency (VPI) by six speech-language pathologists was evaluated. Procedure: The overall grade of severity, hypernasality, audible nasal emission, misarticulations, and intelligibility were rated on visual analog scales. Speech samples with a normal distribution of phonemes (normal text [NT]) and those free of nasal consonants (denasal text [DT]) of 43 patients with VPI were used. Mean nasalance scores were computed for the speech samples, and Spearman correlation coefficients were computed between the mean nasalance score and the five parameters of the differentiated rating. Setting: The Institute of Phoniatrics, Utrecht University Hospital, The Netherlands. Results: The correlation coefficient between the mean nasalance and the perceptual rating of hypernasality ranged among judges from .31 to .56 for NT speech samples and .36 to .60 for DT speech samples. Only small differences were found between speech pathologists with and without expertise in cleft palate speech. The rating of the overall grade of severity appeared to correlate quite well with the rating of the intelligibility (rNT = .77, rDT = .79). Lower correlation coefficients, ranging from .34 to .71, were found between overall grade of severity and hypernasality, audible nasal emission, and mis-articulations. Conclusions: A low correlation between the nasalance and the perceptual rating of hypernasality was found. The parameter overall grade of severity appeared to be determined mainly by the parameter intelligibility. Expertise in rating of cleft palate speech does not guarantee a high correlation between instrumental measurement and perceptual rating.
Spectral Analysis of Prespeech Sounds (Spontaneous Cries) in Infants with Unilateral Cleft Lip and Palate (UCLP): A Pilot Study
Authors: Wermke Kathleen , Hauser Christine , Komposch Gerda , Stellzig Angelika 
Published: October 2018

Objective: The objectives of the present study were: (1) to analyze the cry features of infants with cleft lip and palate (UCLP) by means of spectral analysis, (2) to describe changes of the acoustic parameters from birth until 9 months of age, and (3) to compare these data with existing cry data of infants without cleft (control group). Design: The study was designed on a interdisciplinary, prospective, and longitudinal basis. Setting: Interdisciplinary study: (1) Institute of Anthropology at the Humboldt-University, Berlin; (2) Heidelberg University Hospital: Interdisciplinary Cleft Palate and Craniofacial Center. Patients and Method: The cry parameters of five patients with complete unilateral cleft lip, alveolar ridge, and hard and soft palate were analyzed from birth to 9 months of age. The patients were treated with the same protocol. At the age of 24 months, sensomotor development was assessed using the KIPHARD test. Perceptual judgment of speech, performed after 36 months of life, included nasal resonance, nasal emission of air, articulation disorders, and speech intelligibility. Main Outcome Measure: The cry parameters of fundamental frequency (F0), pitch period perturbation quotient (PPQ), and cry duration (Tsam) were analyzed. Results: Contrary to the expectation that laryngeal parameters are not affected by vocal tract malformations, differences of cry parameters were found between the patients with UCLP and the noncleft group. Particularly, the F0 and its short-time variability (PPQ) were affected. Conclusions: The preliminary results of this study showed that F0 and PPQ of spontaneous cries are influenced in patients with UCLP, and a cry analysis might become a noninvasive tool for early detection of an at-risk status for neuromuscular development and prediction of an at-risk status for later speech and language acquisition in infants with cleft lip and palate. Future research strategies are outlined.
Incidence and Severity of Obstructive Sleep Apnea following Pharyngeal Flap Surgery in Patients with Cleft Palate
Authors: Liao Yu-Fang , Chuang Ming-Lung , Chen Philip K.T. , Chen Ning-Hung , Yun Claudia , Huang Chiung-Shing 
Published: October 2018

Objective: To investigate the incidence and severity of obstructive sleep apnea (OSA) associated with pharyngeal flap surgery in patients with cleft palate at least 6 months postoperatively and to determine whether age or the flap width had an effect on them. The hypothesis tested in this study was that the severity of OSA associated with pharyngeal flap surgery is greater in children than in adults. Subjects: Ten adults, six men and four women, with a mean age of 28.0 years at pharyngeal flap (adult group). Twenty-eight children, 13 boys and 15 girls, with a mean age of 6.3 years at pharyngeal flap (child group). Design: A prospective analysis. Main Outcome Measures: An overnight polysomnographic study was used to determine the incidence and severity of OSA 6 months after pharyngeal flap. Results: The incidence of OSA following pharyngeal flap was high but not significantly different between these two groups (90% in adults and 93% in children, p = 1.000). When OSA was stratified into different levels of severity according to the values of respiratory disturbance index, there were noticeable differences between these two groups (p = .022). In the adult group, eight patients (89%) had mild OSA and 1 patient (11%) had moderate to severe OSA. In the child group, 11 patients (42%) were found to have mild OSA, and 15 patients (58%) had moderate to severe OSA. No relation was found between the flap width and the incidence (p = .435 in adults and .640 in children) or the severity (p = .325 in adults and .310 in children) of OSA in each group. Conclusions: Six months following pharyngeal flap surgery, more than 90% of the patients with cleft palate still had OSA. The severity of OSA associated with pharyngeal flap surgery tended to be greater in children than in adults. The flap width was unrelated to the incidence and severity of OSA, no matter in adults or in children.
Occlusal Relationship in Patients with Bilateral Cleft Lip and Palate during the Mixed Dentition Stage: Does Neonatal Maxillary Arch Configuration Predetermine Outcome?
Authors: Perlyn Chad A. , Brownstein Jeffrey N. , Huebner Donald V. , Marsh Jeffrey L. , Nissen Richard J. , Pilgram Thomas 
Published: October 2018

Objective: To determine whether there is any correlation between initial maxillary arch dysmorphology patterns in complete bilateral cleft lip/palate (BCLP) and the subsequent occlusal relationship in early mixed dentition. Design: This retrospective study evaluated 41 consecutive patients with complete BCLP who were managed by a single tertiary cleft team between 1983 and 1992. All patients had bilateral synchronous lip adhesions with placement of a passive alveolar molding appliance at 4 to 8 weeks of age; definitive modified Manchester lip repair at 6 to 8 months of age; and a one-stage “two-flap” palatoplasty at 14 months of age. Neither gingivoperiosteoplasty nor early alveolar bone grafting was performed. Four patients required early surgical premaxillary setback because of adverse social reactions to a very protrusive premaxilla. All patients (7 to 9 years) were assessed prior to initiation of first-phase orthodontics (maxillary expansion) in anticipation of alveolar bone grafting. Cleft team records were reviewed to determine initial maxillary arch dysmorphology, treatment, and occlusal relationships in early mixed dentition stage. The initial maxillary dysmorphology was classified using a new grouping based on orientation of the vomer, the intercanine distance, and its symmetry. Patterns of mixed dentition occlusion were then compared with group type, and predication tables were generated. Because midface retrusion is the major dentoskeletal morbidity in cleft patients, angle Class I and II occlusions were designated as “favorable” and Class III as “unfavorable.” Results: In early mixed dentition, approximately three-fourths of patients with complete BCLP had favorable occlusion and one-fourth unfavorable occlusion. The occlusal status seemed unrelated to the initial maxillary arch dysmorphology, the use of early passive alveolar molding appliance, or surgical premaxillary setback. Conclusion: Patterns of variation within the initial dysmorphology of the maxillary arch in infants with complete BCLP cannot be used to predetermine subsequent molar occlusal relationships in the mixed dentition.
Toward Pathogenesis of Apert Cleft Palate: FGF, FGFR, and TGFβ Genes Are Differentially Expressed in Sequential Stages of Human Palatal Shelf Fusion
Authors: Britto Jonathan A. , Evans Robert D. , Hayward Richard D. , Jones Barry M. 
Published: October 2018

Objective: Critical cellular events at the palatal medial edge epithelium (MEE) occur in unperturbed mammalian palatogenesis, the molecular control of which involves a number of growth factors including transforming growth factor β3 (TGFβ3). Apert syndrome is a monogenic human disorder in which cleft palate has been significantly correlated to the fibroblast growth factor receptor (FGFR) 2-Ser252Trp mutation. We report the relative expression of these genes in human palatogenesis. Methods: The expression of the IgIIIa/b and IgIIIa/c transcript isoforms of FGFR2 and the proteins FGFR1, FGFR2, and FGFR3 was studied in situ throughout the temporospatial sequence of human palatal shelf fusion and correlated with the expression of TGFβ3. In addition, the immunolocalization of the ligand FGFs 2, 4, and 7 was undertaken together with the intracellular transcription factor STAT1, which is activated by FGFR signaling. Results: FGFRs are differentially expressed in the mesenchyme and epithelia of fusing palatal shelves, in domains overlapping those of their ligands FGF4 and FGF2 but not FGF7. Coexpression is seen with TGFβ3, which is implicated in MEE dynamics and FGF and FGFR upregulation, and STAT1, an intracellular transcription factor that mediates apoptosis. Conclusions: The coregulation of molecules of the FGFR signaling pathway with TGFβ3 throughout the stages of human palatal fusion suggests their controlling influence on apoptosis and epitheliomesenchymal transdifferentiation at the MEE. Experimental evidence links FGFR2-IgIIIa/b loss of function with palatal clefting, and these correlated data suggest a unique pathological mechanism for Apert cleft palate.
Midline Skeletodental Morphology in Holoprosencephaly
Authors: Kjær Inger , Keeling Jean W. , Hansen Birgit Fischer , Becktor Karin B. 
Published: October 2018

Objective: The purpose of the present study was to analyze histologically the midline structures in a human fetus with holoprosencephaly and a single median maxillary central incisor. Methods: A human male fetus, CRL 137 mm, postconceptional age 18 weeks, with a diagnosis of holoprosencephaly, the cebocephalic type (one nostril), and alobar brain development, was investigated. After radiography, the cranial midline structures were examined histologically. Results: The histological examination revealed partial absence of the intermaxillary suture (in the region anterior to the central incisor), absence of the internasal suture, and partial absence of the metopic suture (caudal part). A single midline nasal bone was observed instead of two bilateral nasal bones, and a midline maxillary central incisor was found instead of two bilateral incisors. A short nasal septum, absence of crista galli, and partial absence of cartilaginous tissue anterior to the sella turcica in the region of the presphenoid bone were recorded. Conclusions: The study shows that in the affected fetus, there were malformations of midline structures anterior to the sella turcica comprising suture development and cartilage development.
Prenatal and Perinatal Factors Associated with Isolated Oral Clefting
Authors: Wyszynski Diego F. , Wu Tianxia 
Published: October 2018

Objective: To describe medical service utilization and maternal morbid conditions of women who carry offspring with oral clefts (OCs), to describe maternal and offspring complications during birth, and to evaluate postnatal characteristics of their newborns with isolated OCs. Methods: Two thousand four hundred thirty-seven patients with isolated OCs and 4871 unaffected matched controls meeting inclusion criteria were selected from the U.S. Natality database for 1997. Matching variables were mother's and father's race and child's race, sex, county of birth, and month of birth. Patients and controls were compared in terms of maternal demographic characteristics, gestational complications, physical characteristics of the newborns, maternal exposure to potential risk factors, and adequacy of prenatal care. Results: Although the quality of care was very good in both groups, low gestational age, low birth weight, and low 5-minute Apgar score are more frequent among newborns with OCs than in unaffected controls. Mothers of offspring with OCs are at increased risk, compared with mothers of controls, of having hydramnios or oligohydramnios, eclampsia, and abruptio placenta. Obstetric procedures, such as amniocentesis, electronic fetal monitoring, induction of labor, tocolysis, and ultrasound, and repeat cesarean deliveries are more frequent among mothers of patients than those of controls. At birth, new-borns with isolated OCs are at risk of having hyaline membrane disease and of requiring assisted ventilation, independently of gestational age. Conclusion: The results of this large population-based, case-control study suggest that the presence of an OC in the offspring is associated with increased risk for prenatal and perinatal complications in the mother.