Genital Lymphedema and How to Deal with It: Pearls and Pitfalls from over 38 Years of Experience with Unusual Lymphatic System Impairment

Abstract

Background and Objectives: Conservative treatment represents an essential pillar of lymphedema management, along with debulking and physiologic surgeries. Despite the consistent number of treatment options, there is currently no agreement on their indications and possible combinations. When dealing with unusual lymphedema presentation as in the genitalia (Genital Lymphedema-GL), treatment choice becomes even more difficult. The authors aimed to present their targeted algorithm of single and combined treatment modalities for rare GL in order to face this paucity of information. Materials and Methods: Data were collected from a prospectively maintained database since January 1983, and cases of GL that were managed in the authors' department were selected. Only patients that were treated in the authors' institution and presented a minimum follow-up of 3 months were admitted to the current study. Results: From January 1983 to July 2021, 19 patients with GL were recruited. All the patients were male, and their ages ranged from 21 to 73 years old (average: 52). Ten cases (52.6%) presented with ISL (International Society of Lymphology) stage I, five (26.3%) were stage II and four (21.1%) were stage III. GL was managed with conservative treatment (12 cases), LVA (LymphaticoVenous Anastomosis) (3) or surgical excision (4). In a mean follow-up of 7.5 years (range: 3 months-11 years), no major complications occurred, and all cases reached improvements in functional and quality of life terms. Conclusions: Contrary to the predominant thought of the necessity to avoid surgery in unusual lymphedema presentations such as GL, they can be managed using targeted multimodal approaches or by adapting well-known procedures in unusual ways to achieve control of disease progression and improve patients' quality of life.

Keywords: debulking surgery; genital lymphedema; multimodal approach; physiologic surgery; rare lymphedema; targeted therapy.

Figure 1

Treatment algorithm applied to manage GL.

Figure 2

A male patient, 56 years old, presented to our unit with GL stage II involving the scrotal area, subsequent to prostatic cancer treated with prostatectomy, radiation and chemotherapy (a) The scrotal lymphedema did not respond to 6 months of CDT, so it was treated with LVA procedure: (b) during surgery, ICG was infused near the groin area and the lymphatic ducts were mapped with an ICG camera. Moreover, patent blue was injected to visualize the lymphatic vessels directly. One LVA was performed on each side of the groin area. Three months after surgery (c), there was remarkable improvement of swelling at the genital area, and the patient felt more comfortable, with easier passage of urine reported.

Figure 3

A male patient, 45 years old, presented to our unit with GL stage III involving the scrotal area after radiation for retroperitoneal malignancy. The scrotal lymphedema was almost completely hiding the penis. (a) Excisional surgery and scrotum reconstruction with meshed STSG were performed: the picture (b) shows indurated skin and subcutaneous tissue due to chronic inflammation that were excised. One year after surgery, there was no more inflammation and discharge in the genital area. The urine passage was smooth (c).