Pierre Robin Sequence | Page 23

Ioannis Iatrou, Nadia Theologie-Lygidakis, Ourania Schoinohoriti
Mandibular distraction osteogenesis (MDO) has been increasingly gaining interest over the last decade as a treatment alternative for the challenging airway management in infants with the Robin Sequence (RS). This paper is a case report of a male child diagnosed with RS, suffering from life-threatening airway obstruction and feeding difficulties, treated with tracheostomy and gastrostomy since infancy. After evaluation of the patient by a multidisciplinary team of specialists, MDO performed as soon as possible, was considered the optimal treatment, not only to address the severe micrognathia but also to allow early tracheal decannulation...
September 2010: Journal of Cranio-maxillo-facial Surgery
Kurt-W Bütow, Christiaan Frederik Hoogendijk, Roger A Zwahlen
PURPOSE: The aim of the study was to evaluate the largest number of Pierre Robin sequence (PRS) cases to date and its treatment outcome. DESIGN: This is a retrospective study. SETTINGS: The study was conducted in a tertiary care university hospital. PATIENTS: One hundred eighty-eight patients with PRS have been subjected to analysis, defined by the clinical triad of glossoptosis, retro/micrognathia, and cleft or agenesis of the palate, for incidence, risk factors, associated syndromes, other concomitant abnormalities, airway and feeding difficulties, and outcome regarding the neonatal and perinatal surgical and nonsurgical management...
November 2009: Journal of Pediatric Surgery
Michael R Rossi, Miriam S DiMaio, Bixia Xiang, Kangmo Lu, Hande Kaymakcalan, Margretta Seashore, Maurice J Mahoney, Peining Li
Variable clinical presentations of patients with chromosomally detected deletions in the distal long arm (q) of chromosome 4 have been reported. The lack of molecular characterization of the deletion sizes and deleted genes hinders further genotype-phenotype correlation. Using a validated oligonucleotide array comparative genomic hybridization (oaCGH) analysis, we examined two patients with apparent chromosomal deletions in the distal 4q region. In the first, oaCGH identified a 2.441 megabase (Mb) duplication and a 12...
December 2009: American Journal of Medical Genetics. Part A
Sami A Al-Ani, Martin Rees, Tristan M de Chalain
A patient with Klippel-Feil syndrome, which was associated with Pierre Robin sequence, bilateral mandibular duplication, and occipital clefts, is described. The management, surgery, and possible etiology are discussed in relation to the relevant literature.
November 2009: Journal of Craniofacial Surgery
Michael Krimmel, Susanne Kluba, Martin Breidt, Margit Bacher, Klaus Dietz, Heinrich Buelthoff, Siegmar Reinert
Newborns with Pierre Robin sequence (PRS) have mandibular hypoplasia, glossoptosis, and possibly cleft palate. Their facial appearance is characteristic. The further facial development is controversial. The aim of this study was to analyze the facial development of children with PRS.In a prospective, cross-sectional study, 344 healthy children and 37 children with PRS and cleft palate younger than 8 years were scanned three-dimensionally. Twenty-one standard anthropometric landmarks were identified, and the images were superimposed...
November 2009: Journal of Craniofacial Surgery
Masaru Horikiri, Susam Park, Mikio Kinoshita, Daisuke Matsumoto
BACKGROUND: The Pierre Robin sequence (PRS) is a relatively rare symptom complex characterised by glossoptosis, micrognathia and respiratory obstruction. The initial problem that children with PRS face is obstructive dyspnoea, which can result in death without appropriate respiratory management. We designed and used a modified airway with a Kirschner wire (K-airway) in children with PRS who suffered from dyspnoea that did not improve with conservative treatment. METHODS: The subjects were four children diagnosed with PRS at the Department of Plastic Surgery, Shizuoka Children's Hospital, from February 2007 to December 2008...
April 2010: Journal of Plastic, Reconstructive & Aesthetic Surgery: JPRAS
Woranuch Chetpakdeechit, Bengt Mohlin, Christina Persson, Catharina Hagberg
OBJECTIVE: To study the risks of having other birth defects in children born with an isolated cleft palate (iCP) when the length of the cleft was taken into account. The hypothesis was that a newborn with an extensive cleft lesion may have an increased risk of other birth defects compared to a child with a less extensive cleft of the palate. MATERIAL AND METHODS: All Caucasian children with iCP born between 1975 and 2005 in the southwestern region of Sweden were included...
March 2010: Acta Odontologica Scandinavica
Els-Marie Andersson, Leiv Sandvik, Frank Abyholm, Gunvor Semb
OBJECTIVE: To describe Norwegian individuals born with cleft of the secondary palate (CP) in relation to cleft severity, Pierre Robin sequence, associated anomalies, syndromes, and hypodontia and to present estimated longitudinal prevalences. PATIENTS: Nine hundred ninety-four individuals with CP born between 1960 and 2002 referred to the Oslo Cleft Team (OCT). METHOD: Data were collected retrospectively from the archives of the OCT. RESULTS: Among all CP, 7...
July 2010: Cleft Palate-craniofacial Journal
Gary F Rogers, Gary Rogers, Asher A T Lim, John B Mulliken, Bonnie L Padwa
PURPOSE: Despite its pathogenic heterogeneity, it is often assumed that the small mandible in a neonate with Robin sequence will not grow normally. The purpose of this study was to determine mandibular length and position in patients with nonsyndromic versus syndromic Robin sequence. PATIENTS AND METHODS: Lateral cephalograms of 51 children with Robin sequence were analyzed. Group I consisted of nonsyndromic (NS) subjects and group II consisted of subjects with the 4 most common syndromic (S) diagnoses...
November 2009: Journal of Oral and Maxillofacial Surgery
Joel E Portnoy, Sherard Tatum
Pierre-Robin Sequence, the triad of glossoptosis, micrognathia and cleft palate, provides a challenge in airway management both in and out of the operating room. Transnasal intubation is greatly preferred during its surgical intervention for maximum oral exposure in these very small patients without the added encumbrance of an oral endotracheal tube. From 2001 to 2009, three neonates with Pierre-Robin Sequence who underwent surgery to improve their airway had a novel method of securing a transnasal airway performed in the operating theater...
December 2009: International Journal of Pediatric Otorhinolaryngology
David G Genecov, Carlos Raúl Barceló, Diego Steinberg, Timothy Trone, Elizabeth Sperry
OBJECTIVES: The objective of this study was to evaluate the long-term success of mandibular distraction osteogenesis in patients with mandibular airway obstruction syndrome (MAOS), defined as obstructive sleep apnea, swallowing abnormalities, and failure to thrive in the presence of micrognathia, glossoptosis, gastroesophageal or laryngeal reflux, and microaspiration. This is a cross-sectional study at the International Craniofacial Institute in the Medical City Hospital, Dallas, TX. From January 1997 to July 2008, a group of 81 patients were examined for MAOS using medical history and physical examination...
September 2009: Journal of Craniofacial Surgery
Weimin Shen, Cui Jie, Jianbing Chen, Jijun Zou, Yi Ji
Airway management in neonates with Pierre Robin sequence is often challenging. Advancement of the tongue base using mandibular distraction in neonates can avoid tracheotomy and is increasingly propagated. The surgical indication is very important. We establish indication. The result of the preoperative peripheral oxygen saturation of about 40% in the prone position is considered as indication. The distance from postpharyngeal wall to lingual root of more than 5 mm is considered as contraindication; however, distance of less than 2 mm is considered needing performing mandibular distraction in preoperative lateral cephalometric radiograph...
September 2009: Journal of Craniofacial Surgery
Ming-Lee Lin, Vishal Jhanji, Rasik B Vajpayee
A 30-year-old female with known Pierre Robin sequence presented to the emergency department of the Royal Victorian Eye and Ear Hospital, Melbourne, with acute unilateral hydrops as her first presentation of bilateral keratoconus. Initial management consisted of eye patching, hypertonic saline and cycloplegia. However, the long-term prognosis for visual rehabilitation remained poor in this patient because of the presence of a central corneal opacity and inability to comply with contact lenses. She was not an appropriate candidate for keratoplasty due to severe mental retardation...
February 2010: Contact Lens & Anterior Eye: the Journal of the British Contact Lens Association
Ying-Lun Chen, Kuo-Hwa Wu
BACKGROUND: Airway management for patients with craniofacial abnormalities poses many challenges. It potentially has a high rate of morbidity and even mortality. METHODS: We reviewed our experience in administering anesthesia to patients with a diagnosis of mucopolysaccharidosis or Pierre Robin sequence in the past 10 years (July 1998 to October 2008). The anesthetic procedures, methods of airway management, and events of morbidity and mortality were evaluated. RESULTS: Thirty patients with mucopolysaccharidosis and 53 patients with Pierre Robin sequence were placed under general anesthesia...
September 2009: Journal of the Chinese Medical Association: JCMA
Winnie Pradel, Günter Lauer, Jürgen Dinger, Uwe Eckelt
PURPOSE: In the Pierre Robin sequence, retrognathia and glossoptosis lead to airway obstruction in infants with or without cleft palate. Mandibular distraction has gained acceptance for the treatment of airway obstruction. However, surgical interventions can result in complications. In our institution, mandibular traction is the standard treatment in cases of severe respiratory distress. PATIENTS AND METHODS: A retrospective study was performed of all infants treated for Pierre Robin sequence at our institution from 1979 to 2007...
October 2009: Journal of Oral and Maxillofacial Surgery
Ilza Lazarini Marques, Luiz Carlos Silveira Monteiro, Luiz de Souza, Heloísa Bettiol, Catarina Hissako Sassaki, Renise de Assumpção Costa
OBJECTIVE: To study the prevalence of abnormal gastroesophageal reflux in infants with Robin sequence who had severe respiratory obstruction treated with nasopharyngeal intubation and to evaluate the efficacy of nonsurgical treatment. DESIGN: Longitudinal prospective study. SETTING: Hospital de Reabilitação de Anomalias Craniofaciais, University of São Paulo, Brazil. PATIENTS: Twenty infants with severe isolated Robin sequence treated with nasopharyngeal intubation...
July 2009: Cleft Palate-craniofacial Journal
Cleide C S D Mondini, Ilza L Marques, Cassiana M B Fontes, Sandra Thomé
OBJECTIVE: To provide a detailed description of the nasopharyngeal intubation (NPI) technique and photographs, which should be helpful for those who may need to perform it for treating the airway obstruction in Robin sequence. DESIGN: To describe and illustrate the NPI technique and the necessary considerations for its application. SETTING: Hospital de Reabilitação de Anomalias Craniofacial of University of São Paulo, Brazil. RESULT: The NPI procedure involves the use of a whitish, Portex, number 3...
May 2009: Cleft Palate-craniofacial Journal
Pierre Nicolas, Aurélie Leduc, Stéphane Robin, Simon Rasmussen, Hanne Jarmer, Philippe Bessières
MOTIVATION: High-density oligonucleotide tiling array technology holds the promise of a better description of the complexity and the dynamics of transcriptional landscapes. In organisms such as bacteria and yeasts, transcription can be measured on a genome-wide scale with a resolution >25 bp. The statistical models currently used to handle these data remain however very simple, the most popular being the piecewise constant Gaussian model with a fixed number of breakpoints. RESULTS: This article describes a new methodology based on a hidden Markov model that embeds the segmentation of a continuous-valued signal in a probabilistic setting...
September 15, 2009: Bioinformatics
D Longo, L Menchini, L N Delfino, S Lozzi, G Seganti, F Diomedi-Camassei, S Bottero, S Malena, G Fariello
Heterotopic neuroglial tissue is a rare lesion, occurring more frequently in the nasal cavities. Other rare locations are the orbit, the scalp, the palate, the pharynx, the parapharyngeal space and the lungs. They are usually detected occasionally because they are often asymptomatic, but sometimes they might present with dyspnoea, feeding difficulty, snorting and nasal flaring. Respiratory symptoms occur when heterotopic neuroglial tissue is located in the parapharyngeal space. We report a case of an infant affected by Pierre Robin sequence (PRS) who was admitted to our Institution for a worsening respiratory distress that was not explainable only by PRS...
September 2009: International Journal of Pediatric Otorhinolaryngology
Ricardo Horta, Marisa Marques, Virgilia Gomes, Marco Rebelo, Jorge Reis, José Amarante
The triad of glossoptosis, micrognathia, and cleft palate characterizes the Pierre Robin sequence (PRS). Complications can be severe and the clinical manifestations are very heterogeneous, but airway obstruction is almost present and may be life threatening. To avoid tracheostomy or allow for early decannulation in severely affected children, external or bioresorbable internal distraction devices can be applied to repair mandibular deficiencies. We report a case of a children submitted to bilateral mandibular distraction with external nonresorbable devices...
June 2009: Congenital Anomalies
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