Abstract

Case Report

Meige Trofoedema: A form of primary lymphedema

Carlos Al Sanchez Salguero*

Published: 24 April, 2018 | Volume 2 - Issue 1 | Pages: 010-014

The lymphatic system consists of small non-contractile lymph vessels which collect fluid from the interstitial space and carry it to the major contractile lymphatic vessels with valves that maintain a unidirectional flow to the lymph nodes and is responsible for returning proteins, lipids and water from the interstitium to the venous system near the junction of the subclavian vein and internal jugular vein on both sides. Lymphedema would be the result of the failure of transport with the further accumulation of fluid rich in protein at the interstitium, especially in limbs.

Primary lymphedema, which affects 1.15 / 100000 children (especially pre-pubescent girls), not always clinically evident at birth, is a genetic disorder that represents many different subsets of phenotypes: congenital or congenital lymphangiectasia, congenital trofoedema family or Meige, congenital amniotic band and essence or idiopathic.

The most common form of presentation is due to congenital absence or abnormality of the lymphatic tissue caused by a genetic mutation of the genes responsible for the development of lymphatic vessels that is characterized by the difficulty of draining lymph vessels. This genetic alteration may be sporadic or inherited.

Read Full Article HTML DOI: 10.29328/journal.acr.1001007 Cite this Article Read Full Article PDF

Keywords:

Lymphedema; Indirect radionuclide lymphoscintigraphy; Trofoedema; Meige´s disease

References

  1. Allevato MA, Bilevich DE. Linfedema. Act Terap Dermatol. 2008; 31: 294. Ref.: https://goo.gl/bYMfmp
  2. Tabel Y, Mungan I, Sigirci A, Gungor S. Prymary Lymphoedema at an unusual Location Triggered by Nephrotic Syndrome. Ann Acad Med Singapore. 2009; 38: 636-673. Ref.: https://goo.gl/9VXKc6
  3. Mortimer PS. The pathophysiology of lymphedema. Cancer. 1998; 15: 2798-2802. Ref.: https://goo.gl/gVaUey
  4. Smeltzer DM, Stickler GB, Schirger A. Primary lymphedema in children and adolescents: a follow-up study and review. Pediatrics. 1985; 76: 206-218. Ref.: https://goo.gl/8jRB9q
  5. Szuba A, Rockson SG. Lymphedema: classification, diagnosis and therapy. Vasc Med. 1998; 3: 145-156. Ref.: https://goo.gl/AbW7M5
  6. Blanco JM, Ruiz OC, Acedo MJM, Garcia EM, Gomez EF, et al. Linfedema primario. A propósito de un caso. Vox Paediatrica. 1999; 7: 36-38. Ref.: https://goo.gl/Dut7Rx
  7. Fischer M, Costanzo U, Hoffmann U, Bollinger A, Franzeck UK. Flow velocity of cutaneous lymphatic capillaries in patients with primary lymphedema. Int J Microcirc Clin Exp. 1997; 17: 143-149 Ref.: https://goo.gl/75VKuj
  8. Rezaie T, Ghoroghchian R, Bell R, Brice G, Hasan A. Primary non-syndromic lymphoedema (Meige disease) is not caused by mutations in FOXC2. Eur J Hum Genet. 2008; 16: 300-304. Ref.: https://goo.gl/haVUJP
  9. Dale RF. The inherirance of primary lymphoedema. J Med Genet. 1985; 22: 274-278. Ref.: https://goo.gl/EagFyX
  10. Wolfe JH. The prognosis and possible cause of severe primary lymphoedema. Ann Royal College Surg Engl. 1984; 66: 251-257. Ref.: https://goo.gl/sgwBgp
  11. Olmo JD, España A, Richter J. Utilidad de la linfogammagrafía isotópica en el estudio de linfedemas. Actas Dermo-sifiliograficas. 2005; 96: 419-423. Ref.: https://goo.gl/pMkjv6
  12. Fonkalsrud EW. Congenital malformations of the lymphatic system. Semin Pediatr Surg. 1994; 3: 62-69. Ref.: https://goo.gl/guB7qG
  13. Szuba A, Rockson SG. Lymphedema: classification, diagnosis and therapy. Vasc Med. 1998; 3: 145-156. Ref.: https://goo.gl/XxUQDB
  14. Browse NL. The diagnosis and management of primary lymphedema. J vasc Surg. 1986; 3: 181-184. Ref.: https://goo.gl/uetcui
  15. Huang A, Fruauff A, DiCarmine F, Schuss A, Losada R. Case report 861: Primary lymphedema of the left lower extremity. Skeletal Radol. 1994; 23: 483-485. Ref.: https://goo.gl/DK8gJs
  16. Yasuhara H, Shigematsu H, Muto T. A study of the advantages of elastic stockings for leg lymphedema. Int Angiol. 1996; 15: 272-277. Ref.: https://goo.gl/QdEZp1
  17. Ko DS, Lerner R, Klose G, Cosimi AB. Effective treatment of lymphedema of the extremities. Arch Surg. 1998; 133: 452-458. Ref.: https://goo.gl/CUFHFX

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