Lymphedema is a chronic disabling condition affecting a growing number of patients worldwide. Although lymphedema is not life-threatening, several reports underlined detrimental consequences in terms of distress, pain, functional impairment, and infections with a relevant decrease in quality of life. Currently, there is no cure, and the therapeutic management of this condition aims at slowing down the disease progression and preventing secondary complications. Early diagnosis is paramount to enhance the effects of rehabilitation or surgical treatments. On the other hand, a multidisciplinary treatment should be truly integrated, the combination of microsurgical and reductive procedures should be considered a valid strategy to manage extremity lymphedema, and rehabilitation should be considered the cornerstone of the multidisciplinary treatment not only for patients not suitable for surgical interventions but also before and after surgical procedures. Therefore, a specialized management of Plastic Reconstructive Surgeons and Physical and Rehabilitative Medicine physicians should be mandatory to address patients’ needs and optimize the treatment of this disabling and detrimental condition. Therefore, the aim of this review was to characterize the comprehensive management of lymphedema, providing a broad overview of the potential therapy available in the current literature to optimize the comprehensive management of lymphedema and minimize complications.

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.