Penile reconstruction for a case of genital lymphoedema secondary to proteus syndrome.

[proteus syndrome]

To our knowledge penile lymphoedema secondary to Proteus syndrome has not previously been reported . Hence we report a case of a 16 -year -old male who was referred with features of right hemi-hypertrophy and severe lymphoedema affecting his scrotum and penis . He had previously undergone scrotal reduction surgery at the age of 13 , but had since developed worsening penile oedema . His main concern was that of cosmetic appearance prior to sexual debut , and he also complained of erectile dysfunction . An MRI confirmed gross oedema of the penile skin , but normal underlying cavernosal structure , and no other anatomical abnormality . Under general anaesthesia , the entire diseased penile skin was excised . Two full thickness skin grafts were harvested from the axillae , and grafted onto the dorsal and ventral penile shaft respectively . A compressive dressing and urinary catheter was applied for 7 days . Follow-up at 4 months confirmed complete graft take with minimal scarring , and the patient was very satisfied with the cosmetic outcome . He had also noticed a recovery in erectile activity , and feels psychologically and physically more prepared for sexual relations .