Lymphedema of the external genitalia is uncommon and broadly classified as either congenital or acquired. Chronic disabling lymphedema failing conservative measures may require surgical intervention. Common procedures include excision of subcutaneous tissue with skin preservation, full and split thickness grafts, and circumcision. Unfortunately, surgery has mixed results with one third successful, one third requiring revision, and one third requiring conversion to total excision and full thickness grafting. The aim of this study is to present an example of late-onset primary lymphoedema of the penis managed successfully with circumcision.

We describe the case of a 58-year-old male who presented with idiopathic lymphedema of the genitals (Figure 1), resulting in phimosis and lower urinary tract symptoms. On initial presentation, he was extensively investigated with a lymphoscintigraphy, radiological imaging, and laboratory tests with no specific aetiology identified. Informed written consent was obtained from the patient for clinico-pathological and radiological data to be presented.

After five years’ treatment with lymphoedema physiotherapy, he was referred to urology for worsening, pinhole phimosis with difficulty passing small amounts of urine (Figure 2). Circumcision with a standard tunnelling approach achieved good cosmesis (Figure 3). His resected foreskin was 95 x 53 x 56mm. Histopathology revealed benign squamous mucosa, conspicuous stromal fibrosis, oedema, and non-specific chronic inflammation, consistent with primary lymphedema. He made an uneventful recovery and continued lymphoedema physiotherapy. He noted a significant improvement in LUTS and was satisfied with the functional and cosmetic outcomes. He was subsequently referred to plastics for discussion about future curative interventions should the need arise.

This case demonstrates a unique pathology with a multidisciplinary approach in a rural setting with a successful outcome involving physiotherapy for ongoing symptom control, urology for functional voiding support, and plastics for further cosmesis and potential curative interventions.