DOCSLIB.ORG

The Spine, Trauma and Infection

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
The Spine, Trauma and Infection Develop. Med. Child Neurol. 1982, 24. 202-218 Review Article Robert N. Hensinger Eric T. Jones Developmental Orthopaedics. 11: The Spine, Trauma and Infection Torticollis Congenital muscular torticollis is Torticollis, or wryneck, is a common believed to result from local trauma to the clinical sign in a wide variety of childhood soft tissues of the neck during delivery. illnesses. When recognized at or soon after Birth records of these children birth, the usual cause is congenital demonstrate a preponderance of breech or muscular torticollis. However, difficult forceps deliveries, or primiparous roentgenograms of the cervical spine births', '. A common misconception is that should be obtained to exclude other less the neck is contused during delivery and common congenital conditions, such as the the resultant hematoma leads to fibrosis fixed or bony torticollis associated with and contracture. However, experimental Klippel-Feil syndrome and/or anomalies of the atlanto-axial articulation (Table I). TABLE I Congenital muscular torticollis is Differential diagnosis of torticollis usually discovered in the first six to eight weeks of life. If the infant is examined Congenital Congenital muscular torticollis within the first month of life, commonly a Klippel-Feil syndrome mass or 'tumor' is palpable in the neck' Basilar impressions Atlanto-occipital fusion (Fig. 1). Generally there is a non-tender, Pterygium colli (skin webs) soft enlargement which is mobile beneath Odontoid anomalies the skin and attached to or located within Neurological the body of the sternocleidomastoid Ocular dysfunction muscle. The mass obtains maximum size Syringomyelia Spinal-cord or cerebellar tumors (posterior within the first month and then gradually fossa) regresses. If the child is examined at about Bulbar palsies four to six months of age the tumor Inflammatoi:,. usually will have disappeared and the only Lymphadenitis of the neck clinical findings are the contracture of the Spontaneous hyperemic atlanto-axial rotary subluxation sternocleidomastoid muscle and torticollis posture-head titled towards the involved Trauma Fractures. subluxation, dislocations of the side and chin rotated towards the opposite cervical spine (particularly CI-2) shoulder2* (Fig. 2). Correspondence to Robert N. Hensinger, M.D.. Associate Professor OfOrthopaedicSurgery, University of Michigan, University Hospital, Ann Arbor, Michigan 48109. 20 2 REVIEW ARTICLE work suggests that the fibrosis within the particularly asymmetrical findings such as muscle is due to venous occlusion from atrophy and hemihypertrophy, should be pressure on the neck in the birth canal” ’. suspected of having an underlying The clinical deformity is probably related vertebral deformity. to the ratio of fibrosis to remaining The young child with congenital functional muscle after the initial insult2.If there is sufficient normal muscle it will stretch with growth and the child will not develop the torticollis posture. The fibrotic area, however, has little elastic potential. Further support for intra-uterine molding is provided by additional factors: in three out of four children the lesions are on the right side,’. and 20 per cent of affected children have congenital dysplasia of the hip6. All children with torticollis should be evaluated with roentgenograms to exclude bony abnormality or fracture. Congenital muscular torticollis is painless and is associated with a contracted or shortened sternocleidomastoid muscle, and it is not accompanied by bony abnormalities or neurological deficit. Fig. 1. Six-week-old infant with swelling in region of sternocleidomastoid muscle. Congenital scoliosis Although congenital scoliosis seldom poses a serious problem for the neonate, early recognition of a vertebral anomaly is important. If not controlled, it can quickly lead to significant deformity, both cosmetic and life-threatening’ (Fig. 3a). In the neonate, there may be no visible curvature to suggest a vertebral anomaly, but there are other clinical and roentgenographic findings which should alert the examiner to such a possibility. Children with cutaneous abnormalities Tn the region of the back-dermal sinuses, hairy patches, dimples and hemangioma- should be carefully evaluated for the presence of associated anomalies of the cervical spine (Klippel-Feil), bony torticollis, congenital heart-disease ( 15 per Fig. 2. Six-month-old infant with right-sided cent), and Sprengel’s deformity8. Children congenital muscular torticollis, Note rotation of skull, asymmetry and flattening of face, with ‘Ongenital problems Of the lower depression adjacent to left eye (on side of extremities, foot deformities, and contracted sternocleidomastoid muscle). 203 DEVELOPMENTAL ORTHOPAEDICS. 11: THE SPINE, TRAUMA AND INFECTION scoliosis will seldom have a neurological It is presumed that the clinical problem, un1es.s there is associated consequences are due to the ‘tethering’ diastematomyelia, myelodysplasia or effect of the diastematomyelia on the sacral agenesis. Affected children have a 20 normal ascent of the spinal cord. The per cent incidence of associated anomalies diastematomyelia may act as a ‘check-rein’ of the kidney and bladder, which may only on the upward migration of the neural be discovered on intravenous elements, with progressive neurological pyelography’. This study should be routine signs in the lower extremities. It should be for all children who have vertebral emphasized that the greatest change in the anomalies (Fig. 3b). vertebral architecture occurs in the last six months of intra-uterine skeletal growth: Diastematomyelia differential growth during childhood is at a Diastematomyelia is an uncommon slower pace, with more time for adaptive congenital disturbance of vertebral changes in the spinal cord and nerve architecture in which the neural elements, roots’” 14. the spinal cord or intraspinal nerve-roots The majority of patients have a are split into two columns by a midline cutaneous abnormality in the midline of mass in the spinal canal, fixed anteriorly to the back’’, I5-l7 which can be an important the vertebral body. The mass may be an clue to the diagnosis. The form of the osseous or cartilaginous spicule of a cutaneous defect is extremely varied: a fibrous septum partially or completely patch of hair, a discreet dimple, dividing the neural canal. Diastema- subcutaneous fatty tumor, pigmented nevi, tomyelia may be localized at one vertebral and hemangiomas have all been level or extended over several segments, reportedl6-I9. Although infrequent, the and is commonly found in the lumbar association of a dermal sinus is of Fig. 3a. Newborn with multiple congenital Fig. 3b. Same infant as in Fig. 3a. Intravenous anomalies of the spine. Routine roentgenography pyelogram demonstrates absent right kidney. should include (left) antero-posterior and (rixht) lateral views of spine. 204 REVIEW ARTICLE particular concern and must be carefully erythematous, and the child may have low- searched for, as it may represent a portal grade fever22. The clinical and for entry of bacteria into the spinal canal2’. radiographic appearance is similar to bone The orifice may be quite small- and or joint infection, and the two problems innocuous in appearance, yet lead to are often confused, particularly in the child abscess formation and meningitis, or a with an epiphyseal separation. frank neural cutaneous fistula2’. Similarly, the incidental discovery of such a defect Cervical spine injuries in neonates should make one suspect diastema- The most commonly reported spine tornyelial6. injuries in the neonatal period are those that involve the cervical spinal cord. Fractures in neonates Cervical injury appears to be uncommon, The majority of fractures occur at the or at least not commonly recognized, and -time of delivery and $re either epiphyseal occurs principally in two areas- separations of the humerus or femur, or proximally near the occiput (the C1-2 midshaft fractures of the long bones, most articulation) and distally neal: the thorax commonly the clavicle2’322. The few studies (C6-7 or C7-T1)22. However, there is reported in recent times suggest that the seldom roentgenographic evidence of a over-all incidence of fracture is bony injury26*27.The neonatal spine is uncommon, occurring in less than 1 per more elastic than the spinal cord itself: cent of newborn infants2’. The vast Towbin noted that traction could lengthen majority are clavicular fractures, of which the newborn spine by two inches, but the only a small percentage are recognized2’. spinal cord by only one-quarter inch2’. Similarly, many epiphyseal separations are Thus, stretching forces can cause a only slightly displaced and may be complete transection of the spinal cord undetected22. In contrast, midshaft without roentgenographic evidence of fractures of the long bones generally are bony disruption. recognized immediately, often by the Studies of stillborn infants demonstrate obstetrician at the precise moment they that the maximum angulation of the occur23*24. vertebral column occurs at the As might be expected, injury to a bone or cervicothoracic junction, but without joint occurs more often with complicated vertebral disruption. Consequently, if a obstetrical procedures or breech deliveries dislocation or fracture of the cervical (6 per cent), and is rare in spontaneous vertebrae is discovered, it suggests a severe Injury is more common among transection-type injury to the spinal cord22. first-born infants, but with the exception of C1-2 injuries
Load more

Recommended publications
  • Neonatal Orthopaedics
    NEONATAL ORTHOPAEDICS NEONATAL ORTHOPAEDICS Second Edition N De Mazumder MBBS MS Ex-Professor and Head Department of Orthopaedics Ramakrishna Mission Seva Pratishthan Vivekananda Institute of Medical Sciences Kolkata, West Bengal, India Visiting Surgeon Department of Orthopaedics Chittaranjan Sishu Sadan Kolkata, West Bengal, India Ex-President West Bengal Orthopaedic Association (A Chapter of Indian Orthopaedic Association) Kolkata, West Bengal, India Consultant Orthopaedic Surgeon Park Children’s Centre Kolkata, West Bengal, India Foreword AK Das ® JAYPEE BROTHERS MEDICAL PUBLISHERS (P) LTD. New Delhi • London • Philadelphia • Panama (021)66485438 66485457 www.ketabpezeshki.com ® Jaypee Brothers Medical Publishers (P) Ltd. Headquarters Jaypee Brothers Medical Publishers (P) Ltd. 4838/24, Ansari Road, Daryaganj New Delhi 110 002, India Phone: +91-11-43574357 Fax: +91-11-43574314 Email: [email protected] Overseas Offices J.P. Medical Ltd. Jaypee-Highlights Medical Publishers Inc. Jaypee Brothers Medical Publishers Ltd. 83, Victoria Street, London City of Knowledge, Bld. 237, Clayton The Bourse SW1H 0HW (UK) Panama City, Panama 111, South Independence Mall East Phone: +44-2031708910 Phone: +507-301-0496 Suite 835, Philadelphia, PA 19106, USA Fax: +02-03-0086180 Fax: +507-301-0499 Phone: +267-519-9789 Email: [email protected] Email: [email protected] Email: [email protected] Jaypee Brothers Medical Publishers (P) Ltd. Jaypee Brothers Medical Publishers (P) Ltd. 17/1-B, Babar Road, Block-B, Shaymali Shorakhute, Kathmandu Mohammadpur, Dhaka-1207 Nepal Bangladesh Phone: +00977-9841528578 Mobile: +08801912003485 Email: [email protected] Email: [email protected] Website: www.jaypeebrothers.com Website: www.jaypeedigital.com © 2013, Jaypee Brothers Medical Publishers All rights reserved. No part of this book may be reproduced in any form or by any means without the prior permission of the publisher.
    [Show full text]
  • Arthrogryposis Multiplex Congenita
    American Research Journal of Pediatrics Volume 2, Issue 1, pp: 1-5 Research Article Open Access Arthrogryposis Multiplex Congenita – Case Report Faton Krasniqi1, Shpetim Salihu1, Isabere Krasniqi3, Edmond Pistulli2* 1University Clinical Center of Kosova- Neonatology Clinic, Prishtina 2Faculty of Technical Medical Sciences, University of Medicine, Tirana-Albania 3Main Head Family Center, Prishtina-Kosovo *[email protected] Abstract: Arthrogryposis multiplex congenita is a rare disorder that accompanies with multiple joint been reported from Asia, Africa and Europe. Incidence is about 1:3000 to 1:10.000 of all live newborns. The contractures, which can occur at delivery and are non progressive. It affects both sexes. Most of the cases have causes, for now are unknown. However, this disorder can be provoked from neuropathic and myopathic diseases or some another cause that decreases the mobility of fetal joints. Great joints of both extremities are more attacked. The muscles of the extremities that are attacked can be hypoplastic. Also, the IQ of these children multiplex congenita, that has been resuscitated in delivery room and endotracheal intubation was needed. can be affected. We have presented a newborn female, born in term, by normal delivery, with arthrogryposis In utero, there has been a suspicion from the gynecologists at the end of pregnancy, for esophageal atresia. After delivery, all the needed consults and examinations have been realized. After three weeks in Neonatal Intensive Care Unit, the baby has been discharged in a better general condition, with recommendations to further consultations with orthopedics, physiatrician and pediatrician-neurologists. Key words: arthrogryposis, multiple contractures of joints, congenital defect Introduction Arthrogryposis multiplex congenita refers to a heterogeneous group of congenital defects that manifests with multiple joint contractures (1).
    [Show full text]
  • Genetic Causes of Congenital Malformation in India
    International Journal of Human Genetics ISSN: 0972-3757 (Print) (Online) Journal homepage: http://www.tandfonline.com/loi/rhug20 Genetic Causes of Congenital Malformation in India Geeta Talukder & Archana Sharma To cite this article: Geeta Talukder & Archana Sharma (2006) Genetic Causes of Congenital Malformation in India, International Journal of Human Genetics, 6:1, 15-25, DOI: 10.1080/09723757.2006.11885942 To link to this article: https://doi.org/10.1080/09723757.2006.11885942 Published online: 04 Sep 2017. Submit your article to this journal Article views: 2 View related articles Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=rhug20 © Kamla-Raj 2006 Int J Hum Genet, 6(1): 15-25 (2006) Genetic Causes of Congenital Malformation in India Geeta Talukder1 and Archana Sharma2 1. Vivekananda Institute of Medical Sciences, 99 Sarat Bose Road, Kolkata 700 026, West Bengal, India E-mail: geetatalukdar @hotmail.com 2. CAS in Cell & Chromosome Research, Department of Botany, University College of Science, 35 Ballygunj Circular Road, Kolkata 700 019, West Bengal, India KEYWORDS Congenital malformations; neonates; stillbirths; prenatal detection; prevention ABSTRACT Congenital malformations are a major cause of death of neonates in India where prenatal detection and treatment are not adequate in many hospitals and health centers. Incidence is specially high in stillbirths. It is not realized that genetic causes - chromosomal, single gene and polygenic - are the main causes of many congenital defects and early detection and prevention should be essential to make the small family norm a success. INTRODUCTION Recently Patel and Adhia (2005) detected major malformations in 7.92% of 17653 births and Phenotypic changes of genetic diseases at were able to attribute chromosomal cause to birth include congenital malformations in 4%,polygenic to 45.1% and total genetic chromosomes and single gene defects.
    [Show full text]
  • Congenital Transverse Defects of Limbs and Digits* ('Intrauterine Amputation') by H
    Arch Dis Child: first published as 10.1136/adc.37.193.263 on 1 June 1962. Downloaded from CONGENITAL TRANSVERSE DEFECTS OF LIMBS AND DIGITS* ('INTRAUTERINE AMPUTATION') BY H. G. KOHLER From the Department ofPathology, Birmingham Maternity Hospital (RECEIVED FOR PUBLICATION OCTOBER 23, 1961) Intrauterine amputation of the extremities is an Thomas Bartholin of Copenhagen (1616-1680) uncommon and peculiar congenital deformity which is said to be the first to mention a congenitally is characterized by the absence of one or more distal deficient extremity. He lived at the very threshold limb portions. Termination of the proximal part of the modern scientific era and belonged to a family is usually abrupt and bears no apparent relation to well known for their contributions to medicine and anatomical boundaries. The term 'amputation' biology: Thomas Bartholin's son, Caspar Secundus, suggests separation, by mechanical force, of a limb was the first to describe the vestibulo-vaginal glands. already formed rather than a failure of develop- Thomas was a man of great learning and wide ment, but such a view of the pathogenesis of this interests (Rhodes, 1957). His textbook on anatomy abnormality is far from universally accepted. It was translated into English and used widely for a would probably be better to use a neutral term such long time. Bartholin's descriptions of monsters, as 'transverse defects', but for the conservatism of however, tend to be more imaginative than factual medical nomenclature. (Hendry and Kohler, 1956). The essay which copyright. Circular grooves around the digits or limbs, and contains a reference to limb defects bears the title similar soft tissue defects, also known as ring con- 'Gravidarum Imaginatio' and there, in passing, he strictions, are seen usually in association with 'intra- tells us of a deformed male infant with only one uterine amputation', but also on their own.
    [Show full text]
  • Amyoplasia Or Arthrogryposis Multiplex Congenita [AMC]
    Arthrogryposis (Amyoplasia or Arthrogryposis Multiplex Congenita [AMC]) David S. Feldman, MD Chief of Pediatric Orthopedic Surgery Professor of Orthopedic Surgery & Pediatrics NYU Langone Medical Center & NYU Hospital for Joint Diseases OVERVIEW The word “arthrogryposis” is actually a catch-all term used to describe instances of joint contractures that are present at birth. Treatment varies according to the cause and severity of the condition but when treatment begins at an early age, children can gradually become stronger and experience improved joint mobility and function that can last the rest of their lives. DESCRIPTION Arthrogryposis is a rare medical condition that only occurs in about 1 in 10,000 live births. The condition is characterized by malformed or stiff joints, muscles, and tendons that result in arms, legs, hands, and / or feet having limited to no mobility. The cognitive function of children with the condition is not affected. In fact, they are often extremely bright and communicative. CAUSES There is no one specific cause of arthrogryposis but the most common causes are genetics and intrauterine viruses. Genetic causes often only involve the hands and feet while other causes typically result in more generalized weakness and contractures. DIAGNOSIS METHODS Arthrogryposis tends to be found in its most severe form during newborn examinations. Given the various possible causes of arthrogryposis, proper diagnosis plays a very important role in determining treatment. Diagnosis methods may include MRI, muscle biopsies, blood tests, DNA testing, studies, and / or observations. TREATMENT Arthrogryposis may require very complex treatment and should therefore only be undertaken by health professionals who are not just familiar with the disease but have a high level of expertise in treating arthrogrypotic patients.
    [Show full text]
  • Arthrogryposis Multiplex Congenita Part 1: Clinical and Electromyographic Aspects
    J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.35.4.425 on 1 August 1972. Downloaded from Journal ofNeurology, Neurosurgery, anid Psychiatry, 1972, 35, 425-434 Arthrogryposis multiplex congenita Part 1: Clinical and electromyographic aspects E. P. BHARUCHA, S. S. PANDYA, AND DARAB K. DASTUR From the Children's Orthopaedic Hospital, and the Neuropathology Unit, J.J. Group of Hospitals, Bombay-8, India SUMMARY Sixteen cases with arthrogryposis multiplex congenita were examined clinically and electromyographically; three of them were re-examined later. Joint deformities were present in all extremities in 13 of the cases; in eight there was some degree of mental retardation. In two cases, there was clinical and electromyographic evidence of a myopathic disorder. In the majority, the appearances of the shoulder-neck region suggested a developmental defect. At the same time, selective weakness of muscles innervated by C5-C6 segments suggested a neuropathic disturbance. EMG revealed, in eight of 13 cases, clear evidence of denervation of muscles, but without any regenerative activity. The non-progressive nature of this disorder and capacity for improvement in muscle bulk and power suggest that denervation alone cannot explain the process. Re-examination of three patients after two to three years revealed persistence of the major deformities and muscle Protected by copyright. weakness noted earlier, with no appreciable deterioration. Otto (1841) appears to have been the first to ventricles, have been described (Adams, Denny- recognize this condition. Decades later, Magnus Brown, and Pearson, 1953; Fowler, 1959), in (1903) described it as multiple congenital con- addition to the spinal cord changes.
    [Show full text]
  • Treatment and Outcomes of Arthrogryposis in the Lower Extremity
    Received: 25 June 2019 Revised: 31 July 2019 Accepted: 1 August 2019 DOI: 10.1002/ajmg.c.31734 RESEARCH ARTICLE Treatment and outcomes of arthrogryposis in the lower extremity Reggie C. Hamdy1,2 | Harold van Bosse3 | Haluk Altiok4 | Khaled Abu-Dalu5 | Pavel Kotlarsky5 | Alicja Fafara6,7 | Mark Eidelman5 1Shriners Hospitals for Children, Montreal, Québec, Canada Abstract 2Department of Pediatric Orthopaedic In this multiauthored article, the management of lower limb deformities in children Surgery, Faculty of Medicine, McGill with arthrogryposis (specifically Amyoplasia) is discussed. Separate sections address University, Montreal, Québec, Canada 3Shriners Hospitals for Children, Philadelphia, various hip, knee, foot, and ankle issues as well as orthotic treatment and functional Pennsylvania outcomes. The importance of very early and aggressive management of these defor- 4 Shriners Hospitals for Children, Chicago, mities in the form of intensive physiotherapy (with its various modalities) and bracing Illinois is emphasized. Surgical techniques commonly used in the management of these con- 5Pediatric Orthopedics, Technion Faculty of Medicine, Ruth Children's Hospital, Haifa, ditions are outlined. The central role of a multidisciplinary approach involving all Israel stakeholders, especially the families, is also discussed. Furthermore, the key role of 6Faculty of Health Science, Institute of Physiotherapy, Jagiellonian University Medical functional outcome tools, specifically patient reported outcomes, in the continuous College, Krakow, Poland monitoring and evaluation of these deformities is addressed. Children with 7 Arthrogryposis Treatment Centre, University arthrogryposis present multiple problems that necessitate a multidisciplinary Children's Hospital, Krakow, Poland approach. Specific guidelines are necessary in order to inform patients, families, and Correspondence health care givers on the best approach to address these complex conditions Reggie C.
    [Show full text]
  • Evaluation of Fetal Orbits and Ears
    Evaluation of Fetal Orbits and Ears Maria A. Calvo-Garcia, MD. Associate Professor of Radiology Cincinnati Children’s Hospital Medical Center Disclosure • I have no disclosures Goals & Objectives • Review basic US anatomic views for the evaluation of the orbits and ears • Describe some of the major malformations involving the orbits and ears Background on Facial Abnormalities • Important themselves • May also indicate an underlying problem – Chromosome abnormality/ Syndromic conditions Background on Facial Abnormalities • Assessment of the face is included in all standard fetal anatomic surveys • Recheck the face if you found other anomalies • And conversely, if you see facial anomalies look for other systemic defects Background on Facial Abnormalities • Fetal chromosomal analysis is often indicated • Fetal MRI frequently requested in search for additional malformations • US / Fetal MRI, as complementary techniques: information for planning delivery / neonatal treatment • Anatomic evaluation • Malformations (orbits, ears) Orbits Axial View • Bony orbits: IOD Orbits Axial View • Bony orbits: IOD and BOD, which correlates with GA, will allow detection of hypo-/ hypertelorism Orbits Axial View • Axial – Bony orbits – Intraorbital anatomy: • Globe • Lens Orbits Axial View • Axial – Bony orbits – Intraorbital anatomy: • Globe • Lens Orbits Axial View • Hyaloid artery is seen as an echogenic line bisecting the vitreous • By the 8th month the hyaloid system involutes – If this fails: persistent hyperplastic primary vitreous Malformations of
    [Show full text]
  • GUIDE to ADAPTED SWIMMING CLASSIFICATIONS Swimming Is
    GUIDE TO ADAPTED SWIMMING CLASSIFICATIONS Swimming is the only sport that combines the conditions of limb loss, cerebral palsy (coordination and movement restrictions), spinal cord injury (weakness or paralysis involving any combination of the limbs) and other disabilities (such as Dwarfism (little people); major joint restriction conditions) across classes. Classes 1-10 – are allocated to swimmers with a physical disability Classes 11-13 – are allocated to swimmers with a visual disability Class 14 – is allocated to swimmers with an intellectual disability The Prefix S to the Class denotes the class for Freestyle, Backstroke and Butterfly The Prefix SB to the class denotes the class for Breaststroke The Prefix SM to the class denotes the class for Individual Medley. The range is from the swimmers with severe disability (S1, SB1, SM1) to those with the minimal disability (S10, SB9, SM10) In any one class some swimmers may start with a dive or in the water depending on their condition. This is factored in when classifying the athlete. The examples are only a guide – some conditions not mentioned may also fit the following classes. Locomotor Impaired (S1-S10): S1: Generally persons with complete spinal cord injuries below C4-C5 or cerebral palsy characterized by severe quadriplegia. Unable to catch the water. Severely limited propulsion from the arms due to muscle weakness, restricted range of motion or uncoordinated movements. No trunk control. No functional leg movements and significant leg drag. Assisted water start. Ordinarily uses the backstroke because of an inability to turn the head to breathe when swimming freestyle. S2: Generally persons with complete spinal cord injuries below C6-C7 or similar musculoskeletal impairment or cerebral palsy characterized by severe quadriplegia.
    [Show full text]
  • The Orthopaedic Management of Arthrogryposis Multiplex Congenita
    Current Concept Review The Orthopaedic Management of Arthrogryposis Multiplex Congenita Harold J. P. van Bosse, MD and Dan A. Zlotolow, MD Shriners Hospital for Children, Philadelphia, PA Abstract: Arthrogryposis multiplex congenita (AMC) describes a baby born with multiple joint contractures that results from fetal akinesia with at least 400 different causes. The most common forms of AMC are amyoplasia (classic ar- throgryposis) and the distal arthrogryposes. Over the past two decades, the orthopaedic treatment of children with AMC has evolved with a better appreciation of the natural history. Most adults with arthrogryposis are ambulatory, but less than half are fully independent in self-care and most are limited by upper extremity dysfunction. Chronic and epi- sodic pain in adulthood—particularly of the foot and back—is frequent, limiting both ambulation and standing. To improve upon the natural history, upper extremity treatments have advanced to improve elbow motion and wrist and thumb positioning. Attempts to improve the ambulatory ability and decrease future pain include correction of hip and knee contractures and emphasizing casting treatments of foot deformities. Pediatric patients with arthrogryposis re- quire a careful evaluation, with both a physical examination and an assessment of needs to direct their treatment. Fur- ther outcomes studies are needed to continue to refine procedures and define the appropriate candidates. Key Concepts: • Arthrogryposis multiplex congenita (AMC) is a term that describes a baby born with multiple joint contractures. Amyoplasia is the most common form of AMC, accounting for one-third to one-half of all cases, with the distal arthrogryposes as the second largest AMC type.
    [Show full text]
  • Natural History Study of Arthrogryposis Multiplex Congenita, Amyoplasia Type
    The Texas Medical Center Library DigitalCommons@TMC The University of Texas MD Anderson Cancer Center UTHealth Graduate School of The University of Texas MD Anderson Cancer Biomedical Sciences Dissertations and Theses Center UTHealth Graduate School of (Open Access) Biomedical Sciences 5-2011 Natural history study of arthrogryposis multiplex congenita, amyoplasia type Trisha Nichols Follow this and additional works at: https://digitalcommons.library.tmc.edu/utgsbs_dissertations Part of the Congenital, Hereditary, and Neonatal Diseases and Abnormalities Commons, Medical Genetics Commons, Musculoskeletal Diseases Commons, and the Pediatrics Commons Recommended Citation Nichols, Trisha, "Natural history study of arthrogryposis multiplex congenita, amyoplasia type" (2011). The University of Texas MD Anderson Cancer Center UTHealth Graduate School of Biomedical Sciences Dissertations and Theses (Open Access). 150. https://digitalcommons.library.tmc.edu/utgsbs_dissertations/150 This Thesis (MS) is brought to you for free and open access by the The University of Texas MD Anderson Cancer Center UTHealth Graduate School of Biomedical Sciences at DigitalCommons@TMC. It has been accepted for inclusion in The University of Texas MD Anderson Cancer Center UTHealth Graduate School of Biomedical Sciences Dissertations and Theses (Open Access) by an authorized administrator of DigitalCommons@TMC. For more information, please contact [email protected]. NATURAL HISTORY STUDY OF ARTHROGRYPOSIS MULTIPLEX CONGENITA, AMYOPLASIA TYPE by Trisha Nichols
    [Show full text]
  • Appendix 3.1 Birth Defects Descriptions for NBDPN Core, Recommended, and Extended Conditions Updated March 2017
    Appendix 3.1 Birth Defects Descriptions for NBDPN Core, Recommended, and Extended Conditions Updated March 2017 Participating members of the Birth Defects Definitions Group: Lorenzo Botto (UT) John Carey (UT) Cynthia Cassell (CDC) Tiffany Colarusso (CDC) Janet Cragan (CDC) Marcia Feldkamp (UT) Jamie Frias (CDC) Angela Lin (MA) Cara Mai (CDC) Richard Olney (CDC) Carol Stanton (CO) Csaba Siffel (GA) Table of Contents LIST OF BIRTH DEFECTS ................................................................................................................................................. I DETAILED DESCRIPTIONS OF BIRTH DEFECTS ...................................................................................................... 1 FORMAT FOR BIRTH DEFECT DESCRIPTIONS ................................................................................................................................. 1 CENTRAL NERVOUS SYSTEM ....................................................................................................................................... 2 ANENCEPHALY ........................................................................................................................................................................ 2 ENCEPHALOCELE ..................................................................................................................................................................... 3 HOLOPROSENCEPHALY.............................................................................................................................................................
    [Show full text]