Saxophone penis: An idiopathic origin

Dear Sir, A 40-year-old married male, presented with swelling of scrotum and penis with thickening of overlying skin for 3 months. He had sense of heaviness, discomfort, and anguish about his condition. There was no evidence of any extramarital sexual contact, ulcer, injury, surgery, or radiation prior to the onset. His spouse was seemingly normal. Genital examination displayed a hard, nontender, large – curved enlarged penis of size 6 inches in length, 3 inches in diameter resembling like a "saxophone" [Figure 1]. Lymph nodes were normal. There was no substantiation of ulcer, scar, or inflammation. Scrotum was enlarged and transillumination test was negative. Both lower legs were normal. All routine tests, including complete blood count, serum protein, albumin/globulin ratio, Mantoux test, and chest X-ray, were normal. Ultrasound of the abdomen showed no significant abnormality. Penoscrotal ultrasound was advised to look for deeper lymphatic involvement, but our patient was unable to get that done. Venereal disease research laboratory, HIV-1 and 2 were negative. Midnight peripheral blood smears for microfilaria were negative. Serological tests such as complement fixation tests and immunofluorescence tests were not done due to the lack of facilities in our hospital.Figure 1: Grossly enlarged penis along the longitudinal axis resembling the shape of saxophoneLymphedema is a chronic condition, resulting in the accumulation of lymphatic fluid due to improper drainage of interstitial fluid by the lymphatics channel. Primary lymphedema can be (1) congenital or familial (Milroy's disease), (2) lymphedema praecox, occurring at puberty and (3) lymphedema tardum, occurring after 35 years of age.[1] Secondary lymphedema is more common than primary. In India, secondary lymphedema is more commonly caused by filariasis, but other infectious causes include sexually transmitted infections such as lymphogranuloma venereum and donovanosis are causative factors in significant cases.[2] Other causes include surgical removal of lymph nodes, local radiation, and malignancy. The term "saxophone penis" is defined as deformity and curvature of the penis along its longitudinal axis. It is also known as "ram horn penis." It occurs due to contracture or fibrosis of connective tissue on the dorsal side, producing a dependent ventral side which has more vascularity leading to edema and excessive dorsal curvature that finally giving rise to the configuration of saxophone.[3] Genital lymphedema causes discomfort and emotionally depressive due to its heaviness with limitation in movement and hurdle in maintaining genital hygiene, leading to difficulty in performing sexual activity. Surgical intervention for above-mentioned functional impairment will be the mainstay of treatment. Surgical excision of genital elephantiasis involves various modes of reconstruction with or without lymphangioplasty.[1] If performed, it will improve cosmetic outcome and quality of life of the patients with genital elephantiasis. General surgery referral was advised for further management. The patient was advised to review after surgery, but patient did not follow-up. Saxophone penis is uncommon in daily routine practice and that of idiopathic origin is of rarest. Usually, in India, saxophone penis occurs as a sequel to infections, ulceration, injury, surgery, or any malignancies. However, in our case, saxophone penis occurred without any primary etiology. In India, there is a paucity in reports of saxophone penis with idiopathic origin, we report this case for its uniqueness, as saxophone penis occurred due to idiopathic origin. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.