medigraphic.com
Órgano Oficial del Hospital Angeles Health System

2011, Number 3

<< Back Next >>

Acta Med 2011; 9 (3)

Congenital lymphedema- Milroy disease

Domínguez-Carrillo LG, Armenta-Flores R, Domínguez-Gasca LG

Language: Spanish
References: 30
Page: 149-154
PDF size: 90.50 Kb.


ABSTRACT

Introduction: Congenital lymphedema is called Milroy disease, it is characterized by anaplasia or hypoplasia of lymphatic vessels, the gene for this disease is located in 5 chromosome and is a vascular endothelial growth factor receptor defect. Failure of adequate clearance of lymphatic fluid leads to accumulation of protein in the extracellular fluid, the fibroblasts are stimulated, and the swelling becomes organized and predisposes the skin to recurrent infections. Clinical case: A 16 years old adolescent male with congenital edema of right pelvic extremity, he presents cellulitis of antero-internal zone of leg, he was hospitalized and treated with antibiotics, lymphangiocintigraphy showed absence of lymphatic vessels in right leg. The patient was sent to rehabilitation for specific program achieving lymphedema reduction. Conclusion: Rehabilitation is an important part of the treatment of lymphedema, avoiding cellulitis recurrence, improving life quality and functionality. Cellulitis must be treated with hospitalized patient.


REFERENCES

  1. Nonne M. Vier Fälle von elephantiasis congenita hereditaria. Virchows Archiv für pathologische Anatomie und Physiologie und für klinische Medicin, Berlin. 1891: 189-196.

  2. Milroy WF. An undescribed variety of hereditary edema. New York Medical J 1892; 56: 505-508.

  3. Chronic hereditary edema: Milroy’s disease. Chicago Journal of the Am Med Assoc 1928; 91: 1172.

  4. Meige H. Dystrophie oedematose hereditaire. Paris, La presse médicale 1898; 6: 341-343.

  5. Le trophoedème chronique héréditaire. Paris, Nouvelle iconographie de la Salpêtrière 1899; 12: 453-480.

  6. Bollinger A, Isenring G, Franzeck UK, Brunner U. Aplasia of superficial lymphatic capillaries in hereditary and connatal lymphedema (Milroy’s disease). Lymphology 1983; 16: 27-30.

  7. Stanley G, Rockson SG. Diagnosis and management of lymphatic vascular disease. J Am Coll Cardiol 2008; 52: 799-806.

  8. Wheeler ES, Chan V, Wassman R, Rimoin DL, Lesavoy MA. Familial lymphedema praecox: Meige’s disease. Plast Reconstr Surg 1981; 67: 362-364.

  9. Levinson KL, Feingold E, Ferrell RE, Glover TW, Traboulsi EI, Finegold DN. Age of onset in hereditary lymphedema. J Pediatr 2003; 142: 704-708.

  10. Warren AG, Brorson H, Borud LJ, Slavin SA. Lymphedema: a comprehensive review. Ann Plast Surg 2007; 59: 464-472.

  11. Devillers C, Vanhooteghem O, de la Brassinne M. Lymphedema and cutaneous diseases. Rev Med Suisse 2007; 3: 2802-2805.

  12. Irrthum A, Karkkainen MJ, Devriendt K, Alitalo K, Vikkula M. Congenital hereditary lymphedema caused by a mutation that inactivates VEGFR3 tyrosine kinase. Am J Hum Genet 2000; 67: 295-301.

  13. Butler MG, Dagenais SL, Rockson SG, Glover TW. A novel VEGFR3 mutation causes Milroy disease. Am J Med Genet A 2007; 143A: 1212-1217.

  14. International Society of Lymphology. The diagnosis and treatment of peripheral lymphoedema. Consensus document of the International Society of Lymphology. Lymphology 2003; 36: 84-91.

  15. Wu JJ, Wagner AM. Verruciform xanthoma in association with milroy disease and leaky capillary syndrome. Pediatr Dermatol 2003; 20: 44-47.

  16. Karg E, Bereczki C, Kovacs J. Primary lymphoedema associated with xanthomatosis, vaginal lymphorrhoea and intestinal lymphangiectasia. Br J Dermatol 2002; 146: 134-137.

  17. Tiwari A, Myint F, Hamilton G. Management of lower limb lymphoedema in the United Kingdom. Eur J Vasc Endovasc Surg 2006; 31: 311-315.

  18. Samlaska CP. Congenital lymphedema and distichiasis. Pediatr Dermatol 2002; 19: 139-141.

  19. Berry FB, Tamimi Y, Carle MV, Lehmann OJ, Walter MA. The establishment of a predictive mutational model of the forkhead domain through the analyses of FOXC2 missense mutations identified in patients with hereditary lymphedema with distichiasis. Hum Mol Genet 2005; 14: 2619-2627.

  20. Papendieck C. Malformaciones venosas en pediatría. Rev Arg Cir Vasc 2004; 2: 167-183.

  21. Fakir E, Roberts T, Stephen L, Beighton P. Klippel-Trenaunay-Weber syndrome: Orodental manifestations and management considerations. Oral Surg Oral Med 2009; 107: 754-758.

  22. Witte CL. Advances in imaging of limph flow disorders. Radio Graphics 2000; 20: 1697-1719.

  23. Bollinger A, Amann-Vesti BR. Fluorescence microlymphography: diagnostic potential in lymphedema and basis for the measurement of lymphatic pressure and flow velocity. Lymphology 2007; 40: 52-62.

  24. Albornoz MA, Myers AR. Recurrent septic arthritis and Milroy’s disease. J Rheumatol 1988; 15: 1726-1728.

  25. Mendez R. Kapisform hemangioendothelioma associated with Milroy disease. J Ped Surg 2003; 38: 9-12.

  26. Rockson SG. Diagnosis and Management of lymphatic vascular disease. J Am Coll Cardiol 2008; 52: 799-806.

  27. Gordon KD. Mortimer PS. A guide to lymphedema. Expert Rev Dermatol 2007; 2: 741-752.

  28. Cheung L, Han J, Beilhack A, Joshi S, Wilburn P. An experimental model for the study of lymphedema and its response to therapeutic lymphangiogenesis. Bio Drugs 2006; 20: 363-370.

  29. Belcaro G, Errichi B. Lymphatic tissue transplant in lymphedema a minimally invasive, outpatient, surgical method: a 10- year follow-up pilot study. Angiolog 2008; 59: 77-83.

  30. Becker C, Assouad J. Postmastectomy lymphedema: Longterm results following microsurgical lymph node transplantation. Ann Surg 2006; 243: 313-315.




2020     |     www.medigraphic.com