Idiopathic Scrotal Elephantiasis

Lymphedema is a condition caused by extra-cellular fluids accumulating in subcutaneous compartments as a result of lymphatic obstruction. Etiologically it can be classified as primary (congenital) and secondary (acquired). Primary lymphedema can be seen to be associated with congenital lymphatic hypoplasia, as a component of a genetic syndrome such as Milroy’s disease or Meigs syndrome.1 Secondary lymphedema is usually seen following an obstruction. The most common cause of secondary lymphedema in the world is filariasis, but the number of lymphedema cases secondary to radiotherapy and lymph node dissections is also rapidly increasing. In males, genital lymphedema can be isolated to the penis or the scrotum, or affects both. This condition can be either idiopathic, or occur secondarily to a surgery, trauma, radiation, tumor or infection in lymph nodes where the major lymphatics of the genital region are drained.2 Once the flow of lymphatic fluid is disrupted, a pathophysiologic process that disrupts cellular migration in the immune system is initiated with the fluid accumulating in the distal tissues containing macromolecules and proteins. This results in cellular expansion, loss of elasticity, and a tendency to infection. Pervasive fibrosis develops in the region. Thus, recurrent erysipelas, lymphatic cysts, and lymphuria, giant mass decubitus ulcers, and hygienic problems are frequently encountered in enlarged genital organs. Additionally, micturition difficulty and sexual dysfunction are fundamental issues impacting a reduced quality of life.1 In this study we present a very rarely seen idiopathic case of isolated massive scrotal lymphedema. This case is of significance in that it demonstrates that an isolated lymphedema can grow into massive sizes and lead to partial systemic disorders even if the patient has not undergone surgery.