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Arman T Serebrakian, Michael S Golinko, Michael Alperovich, Christopher M Runyan, David A Staffenberg
Craniofacial microsomia remains the second most common craniofacial deformity after cleft lip and palate. Mandibular pathology has been classically scored from type I to type III by the modified Pruzansky-Kaban classification. The authors report a case of a 5-year-old patient with Goldenhar syndrome and bilateral type III craniofacial macrosomia. The patient had absence of bilateral glenoid fossas, condyles, coronoids, and rami as well as hypoplasia of the symphysis, parasymphysis, and mandibular body. Reconstruction was performed using 2 costochondral rib autografts to reconstruct a ramus and assist in the development of a neo-glenoid fossa at the cranial base...
October 2016: Journal of Craniofacial Surgery
Michael S Golinko, Etoile M LeBlanc, Andrew M Hallett, Michael Alperovich, Roberto L Flores
BACKGROUND: Cleft palate is present in one-third of patients with Treacher-Collins syndrome. The authors present long-term speech and surgical outcomes of palatoplasty in this challenging patient population. METHODS: A retrospective review of all patients with Treacher-Collins syndrome and cleft palate was conducted over a 35-year period at a single institution. Demographics, palatal, mandibular, airway, and surgical outcomes were recorded. Speech outcomes were assessed by the same craniofacial speech pathologist...
September 2016: Journal of Craniofacial Surgery
Frederik Nevens, Pietro Andreone, Giuseppe Mazzella, Simone I Strasser, Christopher Bowlus, Pietro Invernizzi, Joost P H Drenth, Paul J Pockros, Jaroslaw Regula, Ulrich Beuers, Michael Trauner, David E Jones, Annarosa Floreani, Simon Hohenester, Velimir Luketic, Mitchell Shiffman, Karel J van Erpecum, Victor Vargas, Catherine Vincent, Gideon M Hirschfield, Hemant Shah, Bettina Hansen, Keith D Lindor, Hanns-Ulrich Marschall, Kris V Kowdley, Roya Hooshmand-Rad, Tonya Marmon, Shawn Sheeron, Richard Pencek, Leigh MacConell, Mark Pruzanski, David Shapiro
BACKGROUND: Primary biliary cholangitis (formerly called primary biliary cirrhosis) can progress to cirrhosis and death despite ursodiol therapy. Alkaline phosphatase and bilirubin levels correlate with the risk of liver transplantation or death. Obeticholic acid, a farnesoid X receptor agonist, has shown potential benefit in patients with this disease. METHODS: In this 12-month, double-blind, placebo-controlled, phase 3 trial, we randomly assigned 217 patients who had an inadequate response to ursodiol or who found the side effects of ursodiol unacceptable to receive obeticholic acid at a dose of 10 mg (the 10-mg group), obeticholic acid at a dose of 5 mg with adjustment to 10 mg if applicable (the 5-10-mg group), or placebo...
August 18, 2016: New England Journal of Medicine
Ting-Chen Lu, Gavin Chun-Wui Kang, Chuan-Fong Yao, Eric Jein-Wein Liou, Ellen Wen-Ching Ko, Zung-Chung Chen, Philip Kuo-Ting Chen
BACKGROUND: Timing of surgical intervention in hemifacial microsomia (HFM) is controversial. Although mandibular osteodistraction in childhood for HFM is popular, recent data for single-stage distraction in growing HFM patients demonstrated long-term relapse. Literature suggests that adolescents in the late mixed dentition age or permanent dentition age (11-16-year-old) had more stable outcomes post-distraction than younger children. We present favorable experience using single-treatment simultaneous maxillo-mandibular distraction in early adolescent Pruzansky-Kaban type II HFM patients...
September 2016: Journal of Cranio-maxillo-facial Surgery
R Christian Solem, Antonio Ruellas, Arthur Miller, Katherine Kelly, Joni L Ricks-Oddie, Lucia Cevidanes
INTRODUCTION: Disordered craniofacial development frequently results in definitive facial asymmetries that can significantly impact a person's social and functional well-being. The mandible plays a prominent role in defining facial symmetry and, as an active region of growth, commonly acquires asymmetric features. Additionally, syndromic mandibular asymmetry characterizes craniofacial microsomia (CFM), the second most prevalent congenital craniofacial anomaly (1:3000 to 1:5000 live births) after cleft lip and palate...
August 2016: American Journal of Orthodontics and Dentofacial Orthopedics
Hemali Kothari, Dawn P Pruzansky, Jae Hyun Park
No abstract text is available yet for this article.
April 2016: Journal of Clinical Orthodontics: JCO
Jordan W Swanson, Brianne T Mitchell, Jason A Wink, Jesse A Taylor, Scott P Bartlett
BACKGROUND: Grading systems of the mandibular deformity in craniofacial microsomia (CFM) based on conventional radiographs have shown low interrater reproducibility among craniofacial surgeons. We sought to design and validate a classification based on 3-dimensional CT (3dCT) that correlates features of the deformity with surgical treatment. METHODS: CFM mandibular deformities were classified as normal (T0), mild (hypoplastic, likely treated with orthodontics or orthognathic surgery; T1), moderate (vertically deficient ramus, likely treated with distraction osteogenesis; T2), or severe (ramus rudimentary or absent, with either adequate or inadequate mandibular body bone stock; T3 and T4, likely treated with costochondral graft or free fibular flap, respectively)...
January 2016: Plastic and Reconstructive Surgery. Global Open
Dawn P Pruzansky, Brittany Ellis, Jae Hyun Park
No abstract text is available yet for this article.
January 2016: Journal of Clinical Orthodontics: JCO
Waldemar Pruzanski, Julia Kopilov, Arnis Kuksis
Mitogenesis of Vascular Smooth Muscle Cells (VSMC) plays an important role in atherogenesis. Until recently, the effect of lipid subfractions has not been clarified. Secretory phospholipases A2 (sPLA2's) hydrolyse glycerophospholipids and release pro-inflammatory lyso-lipids, oxidized and non-oxidized fatty acids and isoprostanes. They localize in the vascular wall. We hypothesized that structurally similar sPLA2's may exert different impact on VSMC. The influence of sPLA2's, IIA, V, X, HDL, LDL, and hydrolysis products was tested on mitogenesis of VSMC, i...
January 2016: Prostaglandins & Other Lipid Mediators
Steven M Koehler, Amanda Walsh, Andrew J Lovy, Jason S Pruzansky, Dave R Shukla, Michael R Hausman
HYPOTHESIS: We hypothesize that a technique for all-arthroscopic fixation of capitellum osteochondritis dissecans (OCD) lesions using suture fixation and autogenous iliac crest bone grafting offers a successful alternative to open internal fixation techniques as shown by 2-year validated patient-reported outcomes. METHODS: Our technique uses arthroscopic all-inside suture fixation with iliac crest autogenous bone grafting. The procedure was performed on 4 elite-level, adolescent athletes presenting with 5 unstable capitellum OCD lesions resulting in elbow pain, limited range of motion, and decreased ability to play...
October 2015: Journal of Shoulder and Elbow Surgery
Caroline Szpalski, Meredith Vandegrift, Parit A Patel, Geoffrey Appelboom, Mark Fisher, Jeffrey Marcus, Joseph G McCarthy, Pradip R Shetye, Stephen M Warren
Bilateral craniofacial microsomia causes obstructive sleep apnea (OSA). We hypothesize that unilateral craniofacial microsomia (UCFM) is an underappreciated cause of OSA. The records of all pediatric UCFM patients from 1990 to 2010 were reviewed; only complete records were included in the study. UCFM patients with OSA (apnea hypopnea index >1/hr) were compared to UCFM patients without OSA. Univariate and multivariate Fisher and χ(2) tests were performed. Of the 62 UCFM patients, 7 (11.3%) had OSA. All OSA patients had Pruzansky IIB or III mandibles...
June 2015: Journal of Craniofacial Surgery
W Pruzanski, J Kopilov, A Kuksis
OBJECTIVE: Investigation of the diversity of human secretory phospholipases A2 (sPLA2) on the migration of human vascular smooth muscle cells (VSMC). MATERIAL: We investigated the impact of sPLA2 IIA, V, and X and of oleic acid, linoleic acid and lysophosphatidylcholine on the migration of human VSMC. METHODS: Recombinant human sPLA2's and Boyden's chamber method were applied. RESULTS: sPLA2, IIA but not V or X enhanced migration of VSMC in a dose/time dependent manner...
July 2015: Inflammation Research: Official Journal of the European Histamine Research Society ... [et Al.]
Rafael Denadai, Cesar Augusto Raposo-Amaral, Celso Luiz Buzzo, Cassio Eduardo Raposo-Amaral
BACKGROUND: To report autologous free fat grafting as an isolated procedure to manage facial contour asymmetry of a subset of growing patients with craniofacial microsomia (CFM). METHODS: A retrospective analysis of CFM patients (n = 11) with low socioeconomic and intellectual status, poor oral hygiene, living far from our center, Pruzansky-Kaban I/II mandibles, without functional concerns, and with no craniofacial skeletal surgery who underwent isolated free fat grafting between 2012 and 2013 was conducted...
March 2016: Annals of Plastic Surgery
C Neiva, S Dakpe, J Davrou, P-A Dîner, B Devauchelle, M-P Vazquez, A Picard, N Kadlub
Dysmorphogenesis of the mandible complicates assessment of the course of the inferior alveolar nerve in patients with craniofacial microsomia. Our aim in the present study was to correlate the anatomical description of the course with the Pruzansky classification (which indicates the severity of hemifacial microsomia), in the mandibles of 22 affected patients using 3-dimensional computed tomography (CT). We measured the distance between fixed landmarks on the normal and the microsomic sides. The normal sides served as controls...
May 2015: British Journal of Oral & Maxillofacial Surgery
Lei Shi, Wei Liu, Lin Yin, Shi Feng, Shuo Xu, Zhi-Yong Zhang
BACKGROUND: Hemifacial microsomia is the second most common congenital craniofacial malformation after cleft lip/palate with a wide variety of pathologic expression in jaws, skeletal components, ears, and soft tissues. Among the deformities, mandibular hypoplasia is the most common and is the main component that affects facial asymmetry. Mandibular distraction osteogenesis is the mainstay treatment; however, the vector of device and osteotomy lines need to be well designed. We utilized the sagittal split osteotomy for mandibular distraction with rapid prototyping surgical guide plate, making a successful outcome...
March 2015: Journal of Craniofacial Surgery
Youssef Tahiri, Catherine S Chang, Jorien Tuin, J Thomas Paliga, Kristen M Lowe, Jesse A Taylor, Scott P Bartlett
BACKGROUND: Craniofacial microsomia encompasses a spectrum of diagnoses associated with variable degrees of mandibular hypoplasia, facial asymmetry, chin deviation, occlusal abnormalities, and potential airway compromise. This study presents one surgeon's experience with costochondral rib grafting for mandibular reconstruction in children with Pruzansky/Kaban type 2B and type 3 mandibular hypoplasia. METHODS: An institutional review board-approved retrospective chart review was performed of all patients with craniofacial microsomia who underwent costochondral rib grafting for mandibular reconstruction performed by the senior author (S...
February 2015: Plastic and Reconstructive Surgery
James Thomas Paliga, Youssef Tahiri, Jason Wink, Scott P Bartlett, Jesse A Taylor
Although facial asymmetry in hemifacial microsomia (HFM) is well documented in the literature, no studies have concentrated on the morphology of the cranial base. This study aimed to evaluate the endocranial morphology in patients with HFM. Consecutive patients with unilateral HFM treated at a craniofacial center from 2000 to 2012 were included. The patients were grouped according to severity on the basis of the Kaban-Pruzansky classification: mild (0-1), moderate (2a), and severe (2b-3). Skull base angulation and transverse craniometric measures were recorded and then compared with those of age-matched controls...
January 2015: Journal of Craniofacial Surgery
Jason S Pruzansky, Peter Goljan, David P Lundmark, Eon K Shin, Sidney M Jacoby, A Lee Osterman
BACKGROUND: Trigger digit is a common pathology encountered by hand surgeons, but there is a lack of evidence-based guidelines. We investigated the treatment preferences of hand surgeons and explored whether geographic location, type of residency training, or clinical experience is associated with differences in practice. METHODS: An online survey was distributed via email by the American Association for Hand Surgery to 615 members. The survey consisted of 17 questions related to conservative and operative management of trigger digits...
December 2014: Hand: Official Journal of the American Association for Hand Surgery
J R F Walters, I M Johnston, J D Nolan, C Vassie, M E Pruzanski, D A Shapiro
BACKGROUND: Bile acid diarrhoea is a common cause of chronic diarrhoea, occurring as a primary condition or secondary to ileal disease or resection. Many patients have reduced levels of the ileal hormone fibroblast growth factor 19 (FGF19), an inhibitory regulator of hepatic bile acid synthesis, secreted in response to farnesoid X receptor (FXR) activation. AIM: To investigate whether obeticholic acid, a potent FXR agonist, could increase FGF19 in patients with bile acid diarrhoea, and produce clinical benefits...
January 2015: Alimentary Pharmacology & Therapeutics
Jaehoon Choi, Sang Woo Park, Geun-Yong Kwon, Sang-Hyun Kim, Ji An Hur, Seung-Hak Baek, Jae Chan Kim, Tae Hyun Choi, Sukwha Kim
Facial muscles are of major importance in human craniofacial growth and development. The purpose of our study was to investigate whether congenital facial nerve palsy influences craniofacial growth in craniofacial microsomia. Fifty-one patients with unilateral craniofacial microsomia and no history of craniofacial skeletal surgery whose radiographs were taken after craniofacial growth was complete were included in this study. These patients were divided into groups in which the facial nerve was involved or uninvolved...
November 2014: Journal of Plastic, Reconstructive & Aesthetic Surgery: JPRAS
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