(1)
Pediatric Surgery, Al Azher University, Cairo, Egypt
Abstract
Penile lymphedema or Saxophone penis is a potentially disfiguring disorder, characterized by impaired lymphatic drainage of the penile or penoscrotal skin, and manifested by a redundant, thick and oedematous non compressible genital skin, it is secondary to parasitic infestation in older children from endemic area, but in neonate and western countries it is either congenital or idiopathic, treatment of such condition is challenging, with the need to excise the whole redundant defective skin and grafting the penis with a local flap or free skin graft.
Keywords
Penile lymphedema or Saxophone penisElephantiasisMilroy’s diseaseMeigs’ diseaseNomenclature
Saxophone penis, Genital lymphedema, Penile elephantiasis.
Definition
Penile lymphedema (Fig. 18.1) is an uncomfortable and potentially disfiguring disorder, characterized by impaired lymphatic drainage of the penile or penoscrotal skin, and manifested by a redundant, thick and oedematous non compressible genital skin.
Fig. 18.1
Penile lymphedema, confined to penis only and sparing the scrotum giving penis the shape of Saxophone
18.1 Incidence
The incidence of primary penile lymphedema account for approximately 1:60,000 live births. Scrotal lymphedema is rare outside endemic filariasis regions in Africa and Asia [1].
18.2 Classification
Primary: Penile lymphedema (usually in western countries) it is congenitally inherited in 15 % of the cases, in either an autosomal dominant form (Milroy’s disease), or a sporadic form (in 85 % of the cases) that occurs at puberty (Meigs’ disease).
Secondary: Usually caused by acquired infection in Africa and Asia e.g., lymphogranuloma venereum, chlamydia trachomatis or filarial infestation with Wuchereria bancrofti [2].
18.3 Presentation
Primary lymphedema of the genitalia may be presented in a neonate and children as:
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Isolated Penile lymphedema (Saxophone penis) Fig. 18.1.
Penoscrotal lymphedema Fig. 18.3.
Fig. 18.3
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