Simple congenital lymphedema is a congenital form of chronic lymphedema of unknown etiology which affects a part or all of the one extremity, especially the lower extremities, at or immediately after birth.
A clinical classification of iymphedema was offered by Allen and Ghormley in 1935, based on a study of 300 cases of lymphedema of which 12 cases are simple congenital lymphedema. They classified the cases of lymphedema into two divisions: noninflammatory and inflammatory, with a tabulation of primary and secondary causes.
One caes I reporting is classified as being of congenital origin and naturally comes under the noninflammatory group.
On May 1960, a 3 year old girl entered to the Department of Orthopaedic Surgery, Kyungpook National University Hospital because of lymphedema of right leg.
Since at birth, there were no history of skin infection, cellulitis and lymphangitis etc. Gross appearance of the skin of the affecte leg revealed leather like consistency and thickening. One week of preoperative preparation of the patient was sustained absolute bed rest with elevation of affected limb to ease drainage of lymphatic fluid.
The two staged operation of Humans procedure were performed under general anesthesia, first to medical half and second to remaining lateral half of the affected leg. The end result was relatively good in decreasing the circumfence of affected leg but it was not quite acceptable in its cosmetic effect.
The literature on primary lymphedema is reviewed.
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