Spot Diagnosis

Swelling since birth

Question :
This child presented with progressive enlargement of left hand and penis since birth.

What is the diagnosis?

Discussion :
Primary lymphedema. Primary lymphedema represents a developmental abnormality of the lymphatic system. Primary lymphedema has been further subdivided into 3 forms, including congenital lymphedema, lymphedema praecox, and lymphedema tarda, depending on age at presentation. Females are affected twice as often as males, and the lower extremity is involved 3 times more frequently than the upper extremity. The histology of the lymphatic channels often demonstrates an anaplastic pattern without subcutaneous lymphatic trunks but with normal dermal plexus.
Lymphedema praecox is the most common form of primary lymphedema. By definition, it becomes clinically evident after birth and before age 35 years.
Lymphedema tarda, also known as Meige disease, does not become clinically evident until age 35 years or older.
Lymphoscintigraphy has replaced lymphangiography as the investigation of choice.
The goal of conservative therapy is to eliminate protein stagnation and to restore normal lymphatic circulation. Initiate therapy as early as possible before extensive irreversible fibrosclerotic changes occur in the interstitium. Patients should use compression garments continuously during the day. Diuretics have no role in the treatment of lymphedema. Surgical treatment is palliative, not curative, and it does not obviate the need for continued medical therapy.

Correct Answers : yes  52%

Last Shown : Nov 2009
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