Abstract
Lymphatic obstruction either congenitally determined (primary) or by a consequence of acquired lymphatic failure because of obstruction or damaged lymphatics (secondary). Milroy disease is an example of primary lymphoedema where edema is present at birth or soon thereafter. A 16 year old young boy presented with bilateral leg with scrotal oedema since birth with ups and downs of his symptoms. According to the statement of the mother the boy was seen by several physicians and also attended several tertiary hospitals in Dhaka city and eventually was diagnosed as a case of Milroy disease in 2003. Patient was in a reasonably stable condition till July 2008 when he developed massive pleural effusion on right side and chylous fluid was aspirated. After that he developed five episodes of right sided pleural effusion at one to three month interval. Thoracic surgeon was consulted and intra thoracic tube drainage and pleurodesis was done on 19. 02. 2009. After couple of weeks repeat chest x-ray revealed left sided pleural effusion and again left sided pleurodesis was performed. He is now clinically stable except mild oedema in his both feet. DOI: 10.3329/bsmmuj.v3i1.5514 BSMMU J 2010; 3(1): 40-43
Highlights
Lymphoedema (LE) is the abnormal buildup of fluid in an arm or leg due to a malfunction or malformation in the lymphatic system[1]
Primary LE is traditionally classified according to the time of onset: congenital LE, LE praecox or LE tarda
Most Lymphoedema in children occur as primary Lymphoedema
Summary
Lymphoedema (LE) is the abnormal buildup of fluid in an arm or leg due to a malfunction or malformation in the lymphatic system[1]. When there is an inability to pinch up a fold of skin between the second and third toe on the dorsum of the foot is called a positive Stemmer sign This sign is a sensitive test for LE that does not give false positive findings.[10,17] In a study of 71 patients from 10 families with Milroy disease had VEGFR3 mutations.[23] Brice et al described an onset of LE at birth in 97% of the patients; distal oedema (94%), bilateral (85%), unilateral (14%), hydrocele in males (37%), prominent veins (23%), cellulitis (20%) and ‘ski jump’ nails (14%). Case report: Md. Humayun Kabir, a 16 year old pleasant boy, hailing from Nawabgonj, Dhaka presented with swelling of both lower limbs and scrotum since birth with an exacerbation and remission of symptoms. He is clinically stable except mild oedema in both lower limbs
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