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craniofacial cleft

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
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
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
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
Woo Shik Jeong, Jang Yeol Lee, Jong Woo Choi
The surgery-first approach (SFA) is a new paradigm in orthognathic surgery. In our experience over the last 10 years, SFA, particularly for the correction of the class III dentofacial deformity and facial asymmetry, has demonstrated high success rates without any major complications. However, many craniofacial surgeons remain concerned about the skeletal stability of SFA. In the present study, the authors aimed to compare the traditional and SFA with regard to the long-term outcomes of vertical skeletal stability using large-scale data...
March 8, 2018: Journal of Craniofacial Surgery
Tarek Abulezz, Hussein A Fadaak
In poor communities, patients may suffer from health problems requiring special management that cannot be provided locally because of lack of equipment and/or expertise. Children with craniofacial anomalies represent one of these challenging problems. Visiting medical missionary teams have attempted to address these issues for a long time. This article highlights healthcare difficulties in one of the third-world countries with personally based trials for providing free surgeries in tough situation and with hardly available diagnostic and therapeutic facilities...
March 2018: Journal of Craniofacial Surgery
Mirta Basha, Bénédicte Demeer, Nicole Revencu, Raphael Helaers, Stephanie Theys, Sami Bou Saba, Odile Boute, Bernard Devauchelle, Geneviève Francois, Bénédicte Bayet, Miikka Vikkula
BACKGROUND: Oral clefts, that is, clefts of the lip and/or cleft palate (CL/P), are the most common craniofacial birth defects with an approximate incidence of ~1/700. To date, physicians stratify patients with oral clefts into either syndromic CL/P (syCL/P) or non-syndromic CL/P (nsCL/P) depending on whether the CL/P is associated with another anomaly or not. In general, patients with syCL/P follow Mendelian inheritance, while those with nsCL/P have a complex aetiology and, as such, do not adhere to Mendelian inheritance...
March 2, 2018: Journal of Medical Genetics
Willian Saranholi da Silva, Ana Lúcia Pompéia Fraga de Almeida, Maria Giulia Rezende Pucciarelli, Karin Hermana Neppelenbroek, Juliana Dreyer da Silva de Menezes, Renato Yassutaka Faria Yaedú, Thais Marchini Oliveira, Flavia M R N Cintra, Simone Soares
This retrospective observational study aimed to evaluate and identify the relapse rate after orthognathic surgery for maxillary advancement (Le Fort I maxillary osteotomy) in oral cleft patients through digitized cephalograms and 3D dental models, following 2 years. Lateral cephalograms and dental casts of 17 individuals, enrolled in Orthodontics Department in Hospital of Rehabilitation of Craniofacial Anomalies, were carried out. The digital cephalometric tracings were evaluated in: T1-before surgery, T2-immediate after surgery, T3-6-month to 1-year after surgery...
March 1, 2018: Odontology
Arun K Gosain, Harvey Chim, Walter M Sweeney
OBJECTIVE: The present study investigates the efficacy of performing a "palate rerepair" utilizing a double-opposing z-palatoplasty (DOZ) following primary Furlow palatoplasty. DESIGN: Retrospective study. SETTING: Tertiary referral academic center for craniofacial surgery. PATIENTS: 15 consecutive patients who presented with velopharyngeal insufficiency (VPI) after primary Furlow palatoplasty. MAIN OUTCOME MEASURES: All subjects were evaluated using the perceptual speech assessment (PSA) scale...
January 1, 2018: Cleft Palate-craniofacial Journal
Yongchu Pan, Dandan Li, Shu Lou, Chi Zhang, Yifei Du, Hongbing Jiang, Weibing Zhang, Lan Ma, Lin Wang
microRNAs (miRNAs) are widely involved in craniofacial development, and genetic variants of miRNAs may be associated with the risk of non-syndromic orofacial cleft (NSOC). Here, we systematically selected five single nucleotide polymorphisms (SNPs) of miRNAs and investigated the associations between these variants and NSOC susceptibility in a two-stage case-control study including 1,406 NSOC patients and 1,578 controls from the Chinese population. We found that compared with the C allele, the rs2910164 G allele of pre-miR-146a was associated with an increased risk of NSOC (additive model: odds ratio (OR) = 1...
February 27, 2018: Human Mutation
Leela S Mundra, Husain T AlQattan, Meghan G Janette, Carissa Patete, Seth R Thaller
BACKGROUND: Cleft lip and palate are the most common craniofacial congenital malformations. Timing of the nasal repair remains somewhat controversial. Some authors perform a combined nasal and lip repair in infancy while others advocate for a staged repair with the nasal component occurring later in childhood. Frequently, secondary repair is needed to address residual nasal deformities in early adulthood. Conchal cartilage has become increasingly popular as a source of cartilage for secondary reconstruction...
February 23, 2018: Journal of Craniofacial Surgery
J Marulanda, M Murshed
Craniofacial development is a delicate process that involves complex interactions among cells of multiple developmental origins, their migration, proliferation, and differentiation. Tissue morphogenesis of the craniofacial skeleton depends on genetic and environmental factors, and on specific signaling pathways, which are still not well understood. Developmental defects of the midface caused by the absence, delays, or premature fusion of nasal and maxillary prominences vary in severity; leading to clefts, hypoplasias, and midline expansion...
March 2018: Oral Diseases
Akiko Suzuki, Nada Abdallah, Mona Gajera, Goo Jun, Peilin Jia, Zhongming Zhao, Junichi Iwata
Cleft palate (CP) is the most prevalent craniofacial deformity, with ethnic and geographic variation in prevalence in humans. Mice have been used as an animal model to study the cause(s) of CP by several approaches, including genetic and chemical-induced approaches. Mouse genetic approaches revealed that significant amounts of genes are involved in the CP pathology. The aim of this study was to identify common features of CP-associated genes and to explore the roles of microRNAs (miRNAs) as important post-transcriptional regulators that may be involved in the regulation of CP genes...
February 20, 2018: Mechanisms of Development
Michelle Kornbluth, Richard E Campbell, John Daskalogiannakis, Elizabeth J Ross, Patricia H Glick, Kathleen A Russell, Jean-Charles Doucet, Ronald R Hathaway, Ross E Long, Thomas J Sitzman
OBJECTIVE: To compare dental arch relationship, craniofacial form, and nasolabial aesthetic outcomes among cleft centers using distinct methods of presurgical infant orthopedics (PSIO). DESIGN: Retrospective cohort study. SETTING: Four cleft centers in North America. PATIENTS: One hundred ninety-one children with repaired complete unilateral cleft lip and palate (CUCLP). MAIN OUTCOME MEASURES: Dental arch relationship was assessed using the GOSLON Yardstick...
January 1, 2018: Cleft Palate-craniofacial Journal
Fan Liang, Hyuma Leland, Breanna Jedrzejewski, Allyn Auslander, Seija Maniskas, Jordan Swanson, Mark Urata, Jeffrey Hammoudeh, William Magee
Alveolar cleft reconstruction has historically relied on autologous iliac crest bone grafting (ICBG), but donor site morbidity, pain, and prolonged hospitalization have prompted the search for bone graft substitutes. The authors evaluated bone graft substitutes with the highest levels of evidence, and highlight the products that show promise in alveolar cleft repair and in maxillary augmentation. This comprehensive review guides the craniofacial surgeon toward safe and informed utilization of biomaterials in the alveolar cleft...
February 14, 2018: Journal of Craniofacial Surgery
Nazlı Gülsüm Akyel, Ayşe Gül Alımlı, Tülin Hakan Demirkan, Mesut Sivri
INTRODUCTION: Persistent craniopharyngeal canal (PCC) is a rare anomaly of the base of the skull. PCC is defined as a well-corticated osseous canal, extending from the roof of the nasopharynx to the base of the sella over the course of the sphenoid corpus. We reported a PCC case with unique associations. The magnetic resonance imaging findings are discussed. CASE PRESENTATION: We report a case of a 2-year-old boy with persistent craniopharyngeal canal, bilateral microphtalmia with large colobomatous cyst, and ectopic adenohypophysis with Rathke cleft cyst...
February 14, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Marta Losa, Maurizio Risolino, Bingsi Li, James Hart, Laura Quintana, Irina Grishina, Hui Yang, Irene F Choi, Patrick Lewicki, Sameer Khan, Robert Aho, Jennifer Feenstra, C Theresa Vincent, Anthony M C Brown, Elisabetta Ferretti, Trevor Williams, Licia Selleri
Human cleft lip with or without cleft palate (CL/P) is a common craniofacial abnormality caused by impaired fusion of the facial prominences. We have previously reported that in the mouse embryo epithelial apoptosis mediates fusion at the seam where the prominences coalesce. Here, we show that apoptosis alone is not sufficient to remove the epithelial layers. We observed morphological changes in the seam epithelia, intermingling of cells of epithelial descent into the mesenchyme, and molecular signatures of Epithelial-Mesenchymal-Transition (EMT)...
February 5, 2018: Development
Michele Alves Garcia, Marilia Yatabe, Thais Ustulin Fuzer, Adriana Maria Calvo, Ivy Kiemle Trindade-Suedam
OBJECTIVE: To compare the bone morphology after secondary alveolar bone graft surgery (SABG) performed before and after permanent canine eruption. DESIGN: Cross-sectional study. SETTING: Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Bauru, SP, Brazil. PATIENTS: 25 cone-beam computed tomography (CBCT) scans of complete unilateral cleft lip and palate (CLP) individuals who underwent SABG before or after eruption of the permanent canine taken 2 and 6 months (T1 and T2) after SAGB, resulting in 50 CBCT scans...
March 2018: Cleft Palate-craniofacial Journal
Pang-Yun Chou, Rami R Hallac, Ellen Shih, Jenny Trieu, Anjani Penumatcha, Priyanka Das, Clark A Meyer, James R Seaward, Alex A Kane
BACKGROUND: Sculpted physical models and castings of the anatomy of cleft lip and palate are used for parent, patient, and trainee education of cleft lip and palate conditions. In this study, we designed a suite of digital 3-dimensional (3D) models of cleft lip and palate anatomy with additive manufacturing techniques for patient education. METHODS: CT scans of subjects with isolated cleft palate, unilateral and bilateral cleft lip and palate, and a control were obtained...
March 2018: Cleft Palate-craniofacial Journal
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