114. Disorders of Lymphatics

  1. Alan D. Irvine MD, FRCPI, FRCP3,4,
  2. Peter H. Hoeger MD5,6 and
  3. Albert C. Yan MD, FAAP, FAAD7,8
  1. Sahar Mansour MD1 and
  2. Peter S. Mortimer MD, FRCP2

Published Online: 24 MAY 2011

DOI: 10.1002/9781444345384.ch114

Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition

Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition

How to Cite

Mansour, S. and Mortimer, P. S. (2011) Disorders of Lymphatics, in Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition (eds A. D. Irvine, P. H. Hoeger and A. C. Yan), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444345384.ch114

Editor Information

  1. 3

    Trinity College, Dublin, Ireland

  2. 4

    Our Lady's Children's Hospital, Dublin, Ireland

  3. 5

    University of Hamburg, Hamburg, Germany

  4. 6

    Catholic Children's Hospital Wilhelmstift, Hamburg, Germany

  5. 7

    University of Pennsylvania School of Medicine, Philadelphia, PA, USA

  6. 8

    The Children's Hospital of Philadelphia, Philadelphia, PA, USA

Author Information

  1. 1

    SW Thames Regional Genetics Service, St George's University of London, London, UK

  2. 2

    Cardiac and Vascular Sciences (Dermatology), St George's, University of London, London, UK

Publication History

  1. Published Online: 24 MAY 2011
  2. Published Print: 3 JUN 2011

ISBN Information

Print ISBN: 9781405176958

Online ISBN: 9781444345384



  • disorders of lymphatics;
  • lymphoedema, common disorder of lymphatic system;
  • lymphatic system, components - lymphatic vessels and lymph nodes;
  • PROX-1, transcription factor – mice, deficient in factor PROX-1;
  • lymphatics, two roles - homeostasis regulation;
  • lymphoscintigraphy (isotope lymphography) - investigation of choice, for lymphoedema;
  • primary lymphoedemas, congenital - primary abnormality in lymphatic system;
  • microcephaly–lymphoedema–chorioretinal dysplasia (OMIM 152950) - very rare autosomal dominant disorder;
  • Klippel–Trenaunay syndrome (KTS) - two major features;
  • congenital lymphatic malformations - lymphangioma/lymphangiectasia, lymphatic malformations


This chapter contains sections titled:

  • The lymphatic system

  • Lymphoedema

  • Congenital lymphatic malformations

  • Lymphatic disorders with later onset

  • Antenatal presentation and diagnosis of lymphatic disorders

  • Lymphatic tumours

  • References