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Cleft palat

Franz X Bauer, Veronika Heinrich, Florian D Grill, Felix Wölfle, Dennis M Hedderich, Andrea Rau, Klaus-Dietrich Wolff, Lucas M Ritschl, Denys J Loeffelbein
Nasoalveolar Molding (NAM) is associated with ambivalent acceptance regarding effectiveness and unknown long-term results. Our purpose was to analyze the stress distribution patterns within the viscero- and neurocranium of neonates during the first phase of NAM therapy. A finite element (FE) model of a healthy four-week-old neonate was generated, derived from a computed tomography scan allowing the implementation of a bone-density-dependent material model. The influence of dental germs with variable material properties, the cleft width and area of expected force application were analyzed in a worst-case scenario...
February 2, 2018: Journal of Cranio-maxillo-facial Surgery
Ana Paula Corrêa de Queiroz Herkrath, Fernando José Herkrath, Maria Augusta Bessa Rebelo, Mario Vianna Vettore
OBJECTIVES: To investigate the structural and intermediary determinants of health-related quality of life (HRQoL) and oral health-related quality of life (OHRQoL) among adults with cleft lip and/or palate (CL/P). DESIGN AND PARTICIPANTS: A cross-sectional study was conducted with patients enrolled at the referral center for craniofacial anomalies in Manaus, Brazil. Adults aged 18 years or more with nonsyndromic CL/P were selected. MAIN OUTCOME MEASURES: Both HRQoL and OHRQoL were assessed using the 36-item Short-Form Health Survey and the Oral Impacts on Daily Performance, respectively...
January 1, 2018: Cleft Palate-craniofacial Journal
Charlotta Gustafsson, Arja Heliövaara, Junnu Leikola, Jorma Rautio
OBJECTIVE: Speech-correcting surgeries (pharyngoplasty) are performed to correct velopharyngeal insufficiency (VPI). This study aimed to analyze the need for speech-correcting surgery in children with isolated cleft palate (ICP) and to determine differences among cleft extent, gender, and primary technique used. In addition, we assessed the timing and number of secondary procedures performed and the incidence of operated fistulas. DESIGN: Retrospective medical chart review study from hospital archives and electronic records...
January 1, 2018: Cleft Palate-craniofacial Journal
Sergey Y Turin, Cathy J Tang, Arun K Gosain
The anatomy of the greater palatine foramen has variation with regard to location, but there have been no reports on the absence of a complete foramen. We present 2 cases of pediatric patients with cleft palates possessing a greater palatine "notch" as opposed to a bony foramen. Awareness of this anomaly is advantageous in preventing injury to the greater palatine vessels during palatoplasty.
January 1, 2018: Cleft Palate-craniofacial Journal
Montserrat Diaz, Mar Llorente, Victor Asensi
Thomas Malthus (1766-1834), distinguished English professor, is the father of modern demography and the most famous harelip carrier of history. Much is known of his orofacial cleft but nothing regarding its mysterious surgical correction. An 1833 portrait of Malthus by John Linnell, finished when he considered himself "handsome enough," for sitting does not show any upper lip scar. When this surgery took place? Although technically feasible in the 19th century, surgery of cleft lip and palate was complicated...
January 1, 2018: Cleft Palate-craniofacial Journal
Øystein A Haaland, Rolv T Lie, Julia Romanowska, Miriam Gjerdevik, Håkon K Gjessing, Astanand Jugessur
Background: It is widely accepted that cleft lip with or without cleft palate (CL/P) results from the complex interplay between multiple genetic and environmental factors. However, a robust investigation of these gene-environment (GxE) interactions at a genome-wide level is still lacking for isolated CL/P. Materials and Methods: We used our R-package Haplin to perform a genome-wide search for GxE effects in isolated CL/P. From a previously published GWAS, genotypes and information on maternal periconceptional cigarette smoking, alcohol intake, and vitamin use were available on 1908 isolated CL/P triads of predominantly European or Asian ancestry...
2018: Frontiers in Genetics
Klaus Bsl Batista, Badri Thiruvenkatachari, Jayne E Harrison, Kevin D O'Brien
BACKGROUND: Prominent upper front teeth are a common problem affecting about a quarter of 12-year-old children in the UK. The condition develops when permanent teeth erupt. These teeth are more likely to be injured and their appearance can cause significant distress. Children are often referred to an orthodontist for treatment with dental braces to reduce the prominence of their teeth. If a child is referred at a young age, the orthodontist is faced with the dilemma of whether to treat the patient early or to wait and provide treatment in adolescence...
March 13, 2018: Cochrane Database of Systematic Reviews
Chihiro Tanikawa, Kae Hirata, Tomonao Aikawa, Jun Maeda, Mikihiko Kogo, Seiji Iida, Takashi Yamashiro
OBJECTIVES: To evaluate the effects of maxillary anterior segmental distraction osteogenesis (MASDO) in patients with cleft lip and palate (CLP) and to identify risk factors for increased relapse. DESIGN: A retrospective study. PATIENTS: Thirty-one Japanese patients with CLP who underwent MASDO were eligible for study inclusion. MAIN OUTCOME MEASURES: We evaluated lateral cephalograms obtained before (T1), at 3 months (T2), and at 1 year (T3) after MASDO, and measured changes from T1 to T2 (δT1T2), from T2 to T3 (δT2T3), and from T1 to T3 (δT1T3)...
January 1, 2018: Cleft Palate-craniofacial Journal
Min Jae Kang, Soo Min Ahn, Il Tae Hwang
Cornelia de Lange syndrome (CdLS) is a developmental disorder which is characterized by typical facial features, upper extremity malformations, and growth and cognitive delays. The genes involved in CdLS encode the cohesin complex and its associated proteins; and NIPBL mutations, which account for half of the cases, result in severe CdLS phenotypes. We describe a girl with CdLS, presenting with typical facial dysmorphism, cleft palate, hypertrichosis, upper limb hypertonicity, flexion contracture of elbows, micromelia, bilateral hearing loss, gastroesophageal reflux, and severe pyloric stenosis...
January 2018: Annals of Clinical and Laboratory Science
Joanna L Kaplan, Catherine T Gunther-Harrington, Jessie S Sutton, Joshua A Stern
BACKGROUND: The teratogenic effects of immunomodulatory and certain antimicrobial therapies are described in small rodents and humans. While the described teratogenic effects in small rodents have been extrapolated to make conclusions about its use in the pregnant dam, teratogenic effects of prednisone and doxycycline have not yet been reported in the dog. Here we report and describe midline defects observed in a litter of golden retriever puppies exposed to mid-gestational immunosuppressive and antimicrobial therapy...
March 12, 2018: BMC Veterinary Research
Marisabel Magnifico, Diana Cassi, Mauro Gandolfini, Andrea Toffoli, Piero A Zecca, Alberto DI Blasio
BACKGROUND: Moebius syndrome is a rare condition characterized by bilateral facial and abducens nerve paralysis. The aim of this study is to evaluate the main orthodontic features and the frequency of associated clinical characteristics in patients with Moebius Syndrome (MS). METHODS: According to Terzis classifications, 58 patients with MS aged 8 months to 46 years old underwent orthodontics and clinical examination. RESULTS: Strabismus, upper and lower limb malformations, lip and palatal cleft were frequently associated with MS...
March 8, 2018: Minerva Stomatologica
Jiu-Zhen Jin, Zhenmin Lei, Zi-Jian Lan, Partha Mukhopadhyay, Jixiang Ding
Numerous studies have been conducted to understand the molecular mechanisms controlling mammalian secondary palate development such as growth, reorientation and fusion. However, little is known about the signaling factors regulating palate initiation. Mouse fibroblast growth factor (FGF) receptor 2 gene (Fgfr2) is expressed on E11.5 in the palate outgrowth within the maxillary process, in a region that is responsible for palate cell specification and shelf initiation. Fgfr2 continues to express in palate on E12...
March 8, 2018: Reproductive Toxicology
Oliver C Thiele, Matthias Kreppel, Anton Dunsche, Andre M Eckardt, Michael Ehrenfeld, Bernd Fleiner, Volker Gaßling, Gerd Gehrke, Marcus Gerressen, Martin Gosau, Alexander Gröbe, Stefan Haßfeld, Max Heiland, Bodo Hoffmeister, Frank Hölzle, Cornelius Klein, Maximilian Krüger, Alexander C Kübler, Norbert R Kübler, Johannes J Kuttenberger, Constantin Landes, Günter Lauer, Markus Martini, Erich T Merholz, Robert A Mischkowski, Bilal Al-Nawas, Emeka Nkenke, Jörn U Piesold, Winnie Pradel, Michael Rasse, Martin Rachwalski, Rudolf H Reich, Daniel Rothamel, Jan Rustemeyer, Martin Scheer, Henning Schliephake, Rainer Schmelzeisen, Alexander Schramm, Wiebke Schupp, Wolfgang J Spitzer, Erwin Stocker, Christian Stoll, Hendrik Terheyden, Alexander Voigt, Wilfried Wagner, Dieter Weingart, Richard Werkmeister, Jörg Wiltfang, Christoph M Ziegler, Joachim E Zöller
The current surgical techniques used in cleft repair are well established, but different centers use different approaches. To determine the best treatment for patients, a multi-center comparative study is required. In this study, we surveyed all craniofacial departments registered with the German Society of Maxillofacial Surgery to determine which cleft repair techniques are currently in use. Our findings revealed much variation in cleft repair between different centers. Although most centers did use a two-stage approach, the operative techniques and timing of lip and palate closure were different in every center...
February 6, 2018: Journal of Cranio-maxillo-facial Surgery
Ryan H Belcher, David W Molter, Steven L Goudy
Despite humanitarian otolaryngology groups traveling in record numbers to resource-limited areas treating pediatric otolaryngology disease processes and training local providers, there remains a large burden of unmet needs. There is a meager amount of published information that comes from the developing world from an otolaryngology standpoint. As would be expected, the little information that does comes involves some of the most common pediatric otolaryngology diseases and surgical burdens including childhood hearing loss, otitis media, adenotonsillectomies, airway obstructions requiring tracheostomies, foreign body aspirations, and craniomaxillofacial surgeries, including cleft lip and palate...
March 7, 2018: Otolaryngologic Clinics of North America
Priyanka Kumari, Subodh Kumar Singh, Rajiva Raman
BACKGROUND: Genome-wide linkage analysis and whole genome sequencing in a Van der Woude syndrome (VWS) family revealed that the SNP, rs539075, within intron 2 of the cadherin 2 gene (CDH2) co-segregated with the disease phenotype. RESULTS: A study with nonsyndromic cleft lip with or without cleft palate (NSCL ± P) cases (N = 292) and controls (N = 287) established association of this SNP with NSCL ± P as a risk factor. RT-PCR based expression analysis of the SNP-harbouring region of intron 2 of CDH2 in the clefted lip and/or palate tissues of 16 patients revealed that the mutant allele expressed in all those individuals having it (hetero-/homozygous), whereas the wild type allele expressed in <50% of the samples in which it was present...
March 7, 2018: Gene
Maxime Cadieux-Dion, Nicole P Safina, Kendra Engleman, Carol Saunders, Elena Repnikova, Nikita Raje, Kristi Canty, Emily Farrow, Neil Miller, Lee Zellmer, Isabelle Thiffault
BACKGROUND: Ectodermal dysplasias (ED) are a group of diseases that affects the development or function of the teeth, hair, nails and exocrine and sebaceous glands. One type of ED, ankyloblepharon-ectodermal defects-cleft lip/palate syndrome (AEC or Hay-Wells syndrome), is an autosomal dominant disease characterized by the presence of skin erosions affecting the palms, soles and scalp. Other clinical manifestations include ankyloblepharon filiforme adnatum, cleft lip, cleft palate, craniofacial abnormalities and ectodermal defects such as sparse wiry hair, nail changes, dental changes, and subjective hypohydrosis...
March 9, 2018: BMC Medical Genetics
Daichi Morioka, Nirina Mandrano, Hiroki Fujimoto, Yasushi Koga, Nobuhiro Sato, Yasuyoshi Tosa, Fumio Ohkubo, Shinya Yoshimoto
The usefulness of three-dimensional (3D) stereophotogrammetry for treating cleft lip (CL) has been well documented. However, there are only a few reliable anthropometric analyses in infants with CL because at this age they cannot assume a resting facial position. Since 2014, we have used a handheld 3D imaging system in the operating room to obtain optimal images of infants with CL and palate under general anesthesia. Currently, 168 infants with a unilateral cleft, 50 infants with bilateral clefts, and 47 infants with an isolated cleft palate are being followed up in this way for a maximum of 30 months...
March 8, 2018: Journal of Craniofacial Surgery
Øivind Skare, Rolv T Lie, Øystein A Haaland, Miriam Gjerdevik, Julia Romanowska, Håkon K Gjessing, Astanand Jugessur
Background: Although both the mother's and father's alleles are present in the offspring, they may not operate at the same level. These parent-of-origin (PoO) effects have not yet been explored on the X chromosome, which motivated us to develop new methods for detecting such effects. Orofacial clefts (OFCs) exhibit sex-specific differences in prevalence and are examples of traits where a search for various types of effects on the X chromosome might be relevant. Materials and Methods: We upgraded our R-package Haplin to enable genome-wide analyses of PoO effects, as well as power simulations for different statistical models...
2018: Frontiers in Genetics
Anas Imran Arshad, Mohammad Khursheed Alam, Mohammad Fadhli Khamis
OBJECTIVES: The aim of this study is to assess the treatment outcome of complete unilateral cleft lip and palate (CUCLP) patients using modified Huddart/Bodenham scoring system (MHB). To determine whether there is an association of congenital and postnatal factors with the treatment outcome. DESIGN: Retrospective observational study. SETTING: Two regional cleft-referral centers. MAIN OUTCOME MEASURES: In the current study, 101 pairs of dental models of non-syndromic CUCLP patients were retrieved from hospital archives...
January 1, 2018: Cleft Palate-craniofacial Journal
Jordana da Silva Freitas, Maria Cristina de Almeida Freitas Cardoso
PURPOSE: Determine the occurrence of dysphagia symptoms in children with cleft lip and/or palate (CLP) pre- and post-surgical correction. METHODS: Quantitative observational cross-sectional study. Existence of clef lip and/or palate without association with other syndromes was the study inclusion and/or exclusion criterion. Parents and/or legal guardians responded to a recall questionnaire on the identification of occurrence of coughing, choking, vomiting, and nasal escape pre- and postoperatively and whether these symptoms disappeared after surgical correction...
March 5, 2018: CoDAS
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