Cases reported "Genital Diseases, Male"

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1/6. A case of acquired smooth muscle hamartoma of the scrotum.

    We report a case of acquired smooth muscle hamartoma (ASMH) of tunica dartos, believed to be only the fifth so far reported. A 24-year-old man had a 6-month history of an asymptomatic tight and thickened scrotum. The skin was difficult to pinch. A biopsy specimen from the skin showed increased and proliferated smooth-muscle bundles composed of well-differentiated and uniform spindle cells that showed typical features of acquired smooth muscle hamartoma. Interestingly, dilatations of the lymph vessels were noted in the upper dermis above the proliferated smooth muscles. It has been reported recently that long-standing severe lymphoedema may cause histological features mimicking ASMH. As the present case was not preceded by oedema of the scrotum, we consider this case to be true ASMH.
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2/6. Cutaneous manifestations and massive genital involvement in Hennekam syndrome.

    We describe a 16-year-old boy with intestinal lymphangiectasia, lymphedema of the limbs and genitalia, mild mental retardation, and facial anomalies (Hennekam syndrome) and cutaneous lesions. Severe edema in the genital area created a gigantic mass that included the scrotum and penis. Numerous grouped red-violaceous pseudo-vesicular lesions and plaques, as well as verrucous brown papules, were present on the penis and scrotum. The prepuce was hypertrophic, with severe phimosis. Histologic analysis revealed dilated lymphatic vessels lined by a discontinuous layer of flat endothelial cells in the papillary dermis and extending down to the reticular dermis. Dilated blood vessels were also present but no cellular abnormalities were identified. A diagnosis of superficial cutaneous lymphatic malformations was made. To the best of our knowledge, this is the first detailed cutaneous histologic investigation in a patient with Hennekam syndrome. We assume that the onset of cutaneous lesions in our patient was likely triggered by a generalized worsening of his lymphedema.
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3/6. Localized gangrene of the scrotum and penis: a complication of heroin injection into the femoral vessels.

    Long-term intravenous heroin abusers have problems of vascular access. After the accessible sites are sclerosed, the neck, axillae and groins are then used frequently. We report on 3 heroin abusers who presented with localized gangrene of the genitalia after injection into the femoral vessels. We postulate that the pathophysiology of this entity is related to arterial embolization of particulate matter into the microcirculation of the genitalia, which causes arterial thrombosis leading to localized gangrene. Of the 3 patients 2 were treated with local excision, debridement and primary closure. A fourth addict who injected heroin directly into the scrotum and perineum, presented with Fournier's gangrene, a completely different, more lethal entity.
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4/6. When to use the Chevassu maneuver during exploration of intrascrotal masses.

    As many as 50 per cent of explorations for intrascrotal masses result in orchiectomy for benign disease. The most common diagnoses are hydrocele, epididymitis and benign testicular tumors. Many of these diagnoses could be made before orchiectomy if the tunica vaginalis and, occasionally, the tunica albuginea were opened, and the lesion biopsied. However, violation of the testicular tunics traditionally has been considered taboo because of the dangers of tumor seeding. In 1906 Chevassu suggested inguinal exploration and occlusion of the testicular vessels before biopsy of suspicious lesions. We have added scrotal hypothermia, double ligation of the gubernaculum before its division and irrigation with distilled water to provide a procedure that adheres to the principles of good cancer surgery. Its use during inguinal explorations for suspicious intrascrotal masses in 5 patients led to a benign diagnosis and preservation of the testis in 3 instances without subsequent testicular atrophy. Its judicious use can decrease the incidence of orchiectomy for benign disease.
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5/6. thrombosis of the spermatic vein in children.

    We describe 3 children in whom thrombosis of the spermatic vein vessels caused swelling of the spermatic cord and epididymis with edema of the scrotum. Venography of the spermatic vein demonstrated the thrombosis and, therefore unnecessary exploration was avoided.
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6/6. Congenital lymphoedema of the genitalia.

    Isolated congenital lymphoedema of the external genitalia in boys is extremely rare. It can have major physical and emotional consequences for the children. Three male patients with primary lymphoedema of the penis and scrotum are described. The first case presented with lymphoedema of the prepuce and the phallic skin that persisted after the age of 1 year. Circumcision and excision of the subcutaneous tissues of the phallic skin were successful in producing the appearance of a normal circumcised penis. The second case presented with oedema of both phallic and scrotal skin. At age 1 year only a pastous thickening of the prepuce remained, scrotal and phallic skin normalised spontaneously. Routine circumcision was successful with a 5-year follow up. The third case presented with persistent oedema of both the scrotum and the phallus. He suffered leakage of lymph requiring incontinence pads, with complicating skin infections recurring every 4-6 weeks at age 8 years. After complete peeling of the scrotal and phallic skin from subcutaneous tissue some leakage persisted, infections subsided. The cause of this disorder remains unknown, although hypoplasia of the lymphatic vessels is reported in most cases. In the literature, several congenital malformations have been associated with primary lymphoedema. These were not noted in our patients. Rarely, the lymphoedema regresses spontaneously. The treatment of persistent lymphoedema is surgical and consists of meticulous excision of all subcutaneous layers of the affected skin, combined with reconstruction of the penis and/or scrotum. CONCLUSION: Primary lymphoedema of the male external genitalia is an extremely rare malformation of the lymphatic vessels of unknown origin. If persistent, surgical treatment is necessary.
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