Testicular infarction associated with protein S deficiency.

Protein S deficiency is a rare inherited disease in which a defect in the natural coagulation inhibitor protein S predisposes patients to thrombosis. Manifestations of protein S deficiency commonly include superficial and deep venous thrombosis, and pulmonary embolism. Mesenteric, myocardial and cerebral infarctions have been reported less frequently. To our knowledge we report the first case of testicular infarction due to protein S deficiency which manifested as acute scrotal pain. Our case might indicate the need to extend the concept of classic risk factors for testicular infarction in young patients. CASE REPORT A 17-year-old man had protein S deficiency and was taking prophylactic warfarin anticoagulant therapy. He had had several episodes of lower leg deep vein thrombosis during the last 7 years. He was admitted to our hospital with multiple ulcerative wounds and a painful, erythematous and swollen lower left leg. Lower left abdominal cramping occurred followed by left scrotal pain and rapid swelling. Physical examination revealed a red, swollen and tender left testicle. Pain was exacerbated by lifting the testicle above the pubic symphysis. Urinalysis showed 25 to 50 red blood cells per high power field but no pyuria. White blood count was 13,530/mm. 3 (normal 4,000 to 10,000), prothrombin time 17.4 seconds (normal 10.7 to 13.0) and activated partial thromboplastin time 38.3 seconds (normal 25 to 33). Doppler ultrasonography revealed a swollen and heterogeneous texture of the left testis with no Doppler signal of blood flow (fig. 1). Exploratory surgery was performed since the possibility of testicular torsion could not be ruled out. The left testis was explored through a scrotal incision. On gross examination the testis and epididymis were edematous, congested and darkened but the spermatic cord was in the normal position without torsion. Therefore, left orchiectomy was performed. Hemorrhagic infarction was evident on pathological examination. Microscopic examination revealed that thrombosis of the spermatic artery had resulted in testicular infarction (fig. 2). DISCUSSION