Case Reports Elephantiasis of Penis and Scrotum NASEHI A, AZADI SH Department of Urology, Aiatollah Kashani Hospital, Tehran, Iran KEY WORDS: elephantiasis, penis, scrotum, lymphedema 55 Urology Journal UNRC/IUA Vol. 2, 55-57 Spring 2004 Printed in IRAN Introduction Lymphedema of penis and scrotum is rarely seen in countries in which filariasis is not endemic. The abnormal accumulation of lymphatic fluid in sub- cutaneous tissue of penis and scrotum could lead to swelling, pain, dysuria, and sexual dysfunction (impotency and erectile dysfunction). Lymphedema may be idiopathic or secondary to inflammation, surgery, malignancies, trauma, radi- ation, hypoproteinemia, and other medical disor- ders. Lymphatic obstruction is limited to penis and scrotum and is not seen in adjacent organs such as lower extremities, abdomen, and but- tock.(1) Regardless of the cause of scrotal and penile elephantiasis, this disease can lead to phys- ical and spiritual weakness and its treatment is difficult particularly in the aged.(2) Case Report A 16-year-old boy was referred to our hospital because of painless swelling of penis and scrotum (fig. 1). He reported a history of swelling since his childhood, which had been intensified during the past year. No history of irradiation, surgery, trauma, or infection was reported. Penis and scrotum was swelling and non tender. Testes in the scrotum were palpable with difficultly. Mild lower extremi- ties edema was present. Other systems were nor- mal. Ultrasonography of testes and lower urinary tract were normal. Abdominal and pelvic CT scan, as well as CBC, U/A, ESR and blood biochemical tests were nor- mal. Serologic study was negative for filariasis. With the diagnosis of idiopathic elephantiasis of penis and scrotum, surgery was planned in which extensive debridement of the involved tissue, scro- toplasty by the use of skin flaps of 1/3 of the pos- terior scrotum, and Z plasty by applying extra skin of penis region to repair its cover were to be per- formed (fig. 2, 3, 4). Pathology report was diffuse dermal edema with chronic inflammation around vessels, compatible with arteriovenous malformation (fig. 5). Our patient had primary or idiopathic penile or scrotal lymphedema. In one year follow-up after reconstructive surgery, recurrence was not seen Accepted for publication in January 2003 FIG. 1. Lymphedema or elephantiasis of penis and scrotum ELEPHANTIASIS OF PENIS AND SCROTUM and he had a normal sexual function. Discussion Genital elephantiasis is mostly developed in trop- ical regions. Degreef believes that about 20% of males in tropical regions develop penile and scro- tal elephantiasis.(3) Lymphedema has two types: Primary and sec- ondary. Primary lymphedema is subdivided into three categories: 1. congenital-inherited (Milory syndrome), 2. praecox (with early onset), and 3. tarda (with late onset).(1) Secondary lymphedema has four subtypes: 1. obstructive (secondary to neoplasm, radiation, sur- gical intervention, mechanical trauma, and chemi- cal agents injection), 2. inflammatory (parasitic, bacterial, and fungal infections), 3. phlebitis, and 4. angioneurotics.(1) Penile and scrotal lymphedema mostly occurs following an infection or as a reaction to trauma. Idiopathic lymphedema is rarely seen and is caused by a primary obstruction of lymphatic ves- sels of scrotum.(1) No effective medical treatment has been intro- duced; instead, different surgical methods for the treatment of chronic genital lymphedema have been reported in the literature. Two main methods are as follows: 1. Physiologic methods or lymphangioplasty through which lymphatic discharge from involved regions to new lymphatic channels is obtained. 2. Lymphangiectomy with reconstructive sur- gery.(2) Lymphangioplasty is used in the cases of recur- rent lymphedema; however, this method can not be successful in the cases of chronic fibrosis or lym- phedema caused by radiation because of the lack 56 FIG. 2. Freeing of testes and spermatic cord from the surrounding involved tissue through a horizon- tal incision on posterior part of the scrotum FIG. 3. Testes, spermatic cord and penis have been separated from the surrounding involved tissue through two longitudinal and spiral incisions. FIG. 4. Scrotoplasty and Z plasty in order to con- struct the cover of penis following extensive debridement of the involved tissue FIG. 5. A microscopic image from the involved tis- sue that shows diffuse edema in dermis, interstitial tissue, and muscle fibers ELEPHANTIASIS OF PENIS AND SCROTUM of appropriate lymphatic channels.(2) Lymphangiectomy includes the removal of super- ficial lymphatic network, which is located above the Buck's fascia which is derived from median raphae and prepuce lymphatics. These lymphatics drain to superficial posterior lymphatic channels. A deeper system is located beneath the Buck's fas- cia and is drained into deep inguinal lymph nodes.(4) This method of drainage leads to the success of this surgical method. It is essential to remove involved skin and subcutaneous tissue completely (reduction scrotoplasty) to prevent lymphedema recurrence followed by reconstructive surgery of penis and scrotum.(5,6) Different surgical techniques are used in lym- phangiectomy and repair of penis and scrotum which include Jourdan and Meller,(1) Dlepech,(2,5) Larrey,(5) Cadogan and Anderson,(7) Raghaviah,(8) Vaught,(9) Dandapat,(3,5) Morey,(10) Apesos,(2) and Malloy.(1) Surgical complications of elephantiasis or geni- tal lymphedema include hemorrhage, hematoma, urethral injury, infection, painful erection, decrease of sensation, and scar in suture line. These complications could be reduced by using a proper incision, use of Z plasty instead of longitu- dinal suture, separating of testes and cord by an external incision in scrotum before taking any measure, and removal of involved tissue.(2,3) References 1. Malloy TR, Wein AJ, Gross P. Scrotal and penile lymphede- ma: surgical consideration and anagement. J Urol 1983; 130: 263. 2. Apesos J, Anigian G. Reconstruction of penile and scrotal lymphedema. Ann Plast Surg 1991; 27: 570. 3. Dandapat MC, Mohaparto SK, Patro SK. Elephantiasis of the penis and scrotum (a review of 350 cases). Am J Surg 1985; 149: 686. 4. Brown WL, Woods JE. Lymphedema of the penis. Plast Reconstr Surg 1977; 59: 68-71. 5. Prpic I. Severe elephantiasis of penis and scrotum. Br J Plast Surg 1966; 19: 173-8. 6. Ketterings C. Lymphedema of penis and scrotum. Br J Plast Surg 1968; 21: 381-6. 7. Anderson BB, Cadogan CA. Scrotal lymphedema praecox: disease and treatment. J Natl Med Ass 1982; 74: 387. 8. Raghavaiah NV. Reconstruction of scrotal and penile skin in elephantiasis. J Urol 1977; 118: 128. 9. Vaught SK, Litvak AS, McRoberts JW. The surgical man- agement of scrotal and penile lymphedema. J Urol 1975; 113: 204. 10. Morey AF, Meng MV, McAninch JW. Skin graft reconstruc- tion of chronic genital lymphedema. Urology 1997; 50: 423. 57