Atypical penile Mondor’s disease – involvement of the circumflex vein

Introduction

Mondor’s disease or superficial thrombophlebitis of subcutaneous veins is an uncommon entity, which typically involves the veins of the anterior chest wall. Other sites of usual infection include the axillae, groins, abdomen and arms. Penile Mondor’s disease is a relatively rarer condition and typically involves the dorsal vein of the penis. ¹ We report a peculiar case of penile Mondor’s disease in a young Indian man who developed superficial thrombophlebitis of the circumflex vein of the penis with sparing of the dorsal vein. To the best of our knowledge, such a pattern of penile venous involvement has not been described before. Thus, we propose the term ‘atypical penile Mondor’s disease’ for such a case.

Case

A 26-year-old unmarried Indian man presented with the complaint of a cord-like hard mass on the ventral surface of penis noticed by him two days before while bathing. The mass was painless at rest but became slightly painful on erection and self-manipulation. There was no history of any pre-marital sexual contact, vigorous masturbation or sexual activity, use of sex stimulants or sexual devices like the shaft vacuum. He denied indulgence in any vigorous physical activity or weight-training prior to noticing the lesion. He also denied history of dysuria, urethral discharge, genital ulcer, inguinal swelling or any other symptom suggestive of a urogenital or sexually transmitted infection (STI). He categorically denied intake of anabolic steroids or any other over-the-counter drugs. Conditions like diabetes, hypertension, coronary artery disease, deep vein thrombosis or tendency of venous thromboembolism, inherited coagulopathies, malignancy, venous disease and haematologic disorders in the patient and his first-degree relatives were ruled out by detailed history.

Examination of the genital region revealed that the patient was uncircumcised with a retractable healthy prepuce. Inspection and palpation of the penile shaft revealed a subcutaneous, indurated cord-like mass, approximately 3 cm in size over the ventral aspect of the penile shaft extending laterally towards the dorsal aspect in a curvilinear pattern (Figure 1). The skin overlying the mass was unremarkable. The rest of the genitourinary examination was normal. On closer examination, the mass seemed to be involving the superficial circumflex vein, rather than the dorsal vein. OPEN IN VIEWER

Colour Doppler study of penis revealed a tubular beaded hypoechoic structure in the subcutaneous plexus on the ventral aspect of penis consistent with the circumflex vein. The vein was non-compressible and showed no colour flow, suggestive of thrombosis (Figure 2). The dorsal vein revealed no abnormality. Haematological and biochemical evaluations including blood count, blood sugar, hepatic and renal function tests and coagulation studies were normal. We recommended warm compresses and oral non-steroidal anti-inflammatory drugs (NSAIDS) for five days. However, the patient reported back within three days with complete resolution of the lesion. OPEN IN VIEWER

Discussion

Mondor’s disease, first described by Henry Mondor in 1939 as the phlebitis of chest wall, is three times more common in women. However, Braun–Falco and Helm and Hodge later described the rare penile involvement. ¹ In a study on heterosexual patients of 18–45 years who visited an STI clinic, 1.39% patients reported Mondor’s disease of penis. Patients classically present with a palpable cord-like structure over the penile shaft often associated with mild pain, but may be asymptomatic. ²

The aetiology of penile Mondor’s remains speculative. Mechanical trauma, typically due to vigorous sexual activity, is the most common implicated factor. Prompt resolution with abstinence supports this concept. A close temporal association with STIs has been observed but a causative correlation is uncertain as frequent sexual activity predisposes to the acquisition of STIs as well as development of Mondor’s phlebitis. ²

Other implicated factors include surgery of the pelvic area, pelvic tumours, use of constrictive or vacuum erection devices during sexual activity, prostrate surgery, long flights and haematological diseases like sickle cell disease. However, similar to the present case, many cases remain idiopathic.^2,3

Diagnosis is primarily clinical and easily confirmed by Doppler ultrasonography of the penile venous system. Absence of colour flow on Doppler study suggestive of thrombosis was the finding in our case consistent with Mondor’s disease. ⁴

To the best of our knowledge, penile Mondor’s has only been reported to involve the dorsal vein. This is the first case report in which a circumflex vein of the penis was thrombosed with sparing of dorsal vein. The penis is drained by three venous systems – superficial, intermediate and deep (Figure 3). The circumflex veins form a component of the intermediate system and arise from the spongiosum along the ventrum of the penis, course laterally around the cavernosa and pass beneath the dorsal arteries and nerves before finally draining into the deep dorsal vein (Figure 3). ⁵ OPEN IN VIEWER

A close differential which needs to be considered in our case is sclerosing lymphangitis of penis. Sclerosing lymphangitis closely resembles the clinical presentation of Mondor’s disease but it presents classically as a translucent cord-like structure generally located just proximal to coronal sulcus. However, in our case the cord-like structure was seen in midshaft of the penis.^6,7 On Doppler ultrasonography, lymphangitis is seen as dilated serpiginous structure with anechoic content resembling rosary beads whereas in our case the cord-like structure was seen as a non-compressible thrombosed linear structure. Therefore, the findings favour the diagnosis of Mondor’s disease. ⁸ However, differentiation between Mondor’s and sclerosing lymphangitis of penis has been a controversial subject. In the past, different authors have tended to discredit the use of one of the terms. ⁹ Others have suggested them to represent the same entity. The issue has been further complicated with the suggestion of the term ‘sclerosing lymphangitis of Mondor’! ¹⁰ The most conclusive way to reach an appropriate differential diagnosis would be with immunohistochemistry for which biopsy from the penile shaft is required. However, conducting an invasive test for a condition (whether thrombophlebitis or lymphangitis) which is self-resolving or responsive to conservative management seems unjustified and hence even we did not conduct it in our patient.^6–8

Despite being a benign and self-resolving condition, penile Mondor’s disease is associated with significant psychological morbidity; in particular, the patient’s fear of having acquired some STI or sexual dysfunction. Counselling and reassurance about its benign nature, and temporary sexual abstinence constitute the mainstay of therapy. Thus, it is imperative for the physician to be aware of this benign entity to allay the concerns of the patient and also to avoid overaggressive treatment. Oral antibiotics, NSAIDS and anticoagulants may be required in some cases. Surgical intervention like thrombectomy or superficial penile vein resection is very rarely indicated only for clots persisting more than six weeks or lack of response to medical treatment.^3,11

In conclusion, the current case of ‘atypical penile Mondor’s disease’ is peculiar due to the involvement of circumflex instead of the dorsal vein, lack of any specific cause and extremely rapid self-resolution.