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SPONTANEOUS THROMBOSIS OF THE DEEP DORSAL PENILE VEIN IN A PATIENT WITH THROMBOPHILIA BENJAMIN A. SCHMIDT,* THOMAS SCHWARZ AND SEBASTIAN M. SCHELLONG From the Division of Angiology, Department of Medicine, University of Dresden, Dresden, Germany KEY WORDS: penis, thrombosis, thrombophilia While superficial dorsal penile vein thrombosis has been associated with inherited thrombophilia, 1 and deep dorsal penile vein thrombosis following trauma 2 and thrombosis of the corpora cavernosa have previously been described, to our knowledge there are no reports of spontaneous deep dorsal penile vein thrombosis. We report such a case and discuss the functional and therapeutic issues of this disorder. CASE REPORT A 60-year-old man presented with painless flaccid penile swelling, which had been massive at onset 1 week earlier. There was no history of penile trauma. The interval since sexual intercourse was 3 days. Erection was reported to be impossible because of severe pain. Symptoms had been preceded by a mild gastrointestinal infection. Repeat urological consultation had not established a diagnosis. Physical examination revealed moderate penile edema without discoloration, induration or tenderness to palpation. History was significant for spontane- ous calf vein and saphenous vein thrombosis, which had been treated with oral anticoagulants for 4 months in 1997. There was a family history of deep venous thrombosis in the mother of the patient and 1 of 4 of his siblings. Duplex sonography showed isolated occlusive thrombosis of the deep dorsal penile vein without involvement of iliac veins, inferior vena cava or superficial dorsal penile vein (see figure). Laboratory tests revealed resistance to activated pro- tein C ratio of 1.56 (normal greater than 2.2) and heterozy- gous factor V Leiden mutation but no prothrombin gene mutation G20210A. Tests were negative for antinuclear an- tibodies, antiphospholipid-IgG and lupus anticoagulant. Complete blood count, protein C, protein S, free protein S, thromboplastin time, activated partial thromboplastin time, antithrombin, fibrinogen and prostate specific antigen were all normal. IgM antibodies were 15.9 to phosphatidylserine (normal less than 15) and 60.3 to cardiolipin (normal less than 44) at presentation, and returned to normal 6 weeks later. Abdominal sonography, chest x-ray and a test for occult gastrointestinal blood provided no evidence of paraneoplastic thrombosis. Treatment with phenprocoumon was started, with dose adjustments toward International Normalized Ratio 2.0 to 3.0, and initially combined with nadroparin. Penile swelling resolved completely in 1 week. At 6-week followup painless tumescent erection was possible and at 12-week followup erectile function had improved to nearly normal. Followup duplex sonography showed thrombus retraction without re- canalization. Anticoagulant treatment is planned for 4 years for symptomatic thrombophilia, which corresponds to the treatment duration proved beneficial for recurrent deep ve- nous thrombosis of the leg. 3 DISCUSSION While no therapy is required for superficial vein thrombo- sis 2 unless risk factors for thromboembolism have been es- tablished, 1 complete and segmental penile thromboses have been treated with fibrinolytics and anticoagulation. 4 To our knowledge there have been no reports of spontaneous throm- bosis of the deep dorsal penile vein and no treatment recom- mendations. The deep vein drains the glans, corpus spongio- sum and distal two-thirds of the corpora cavernosa. Therefore, a relationship between deep vein thrombosis and complete or segmental penile thrombosis seems probable, and anticoagulation for these disorders is applicable to deep penile vein thrombosis. In our patient a thrombophilic disor- der seemed likely and long-term anticoagulation was consid- ered mandatory. Although 12 weeks of treatment did not achieve recanalization on imaging, function almost com- pletely improved with conservative management, which cor- responds to results reported for segmental penile thrombo- sis. 4 REFERENCES 1. Luzzi, G. A., Pattinson, J. and Wathen, C. G.: Factor V Leiden presenting with penile vein thrombosis. J Urol, 159: 2093, 1998 2. Evans, D. T. and Ward, O. E.: Dorsal vein thrombosis of the penis presenting to an STD clinic. Genitourin Med, 70: 406, 1994 3. Schulman, S., Granqvist, S., Holmstro ¨m, M. et al.: The duration of oral anticoagulant therapy after a second episode of venous thromboembolism. The Duration of Anticoagulation Trial Study Group. N Engl J Med, 336: 393, 1997 4. Machtens, S. A., Kuczyk, M. A., Becker, A. J. et al: Partial unilateral penile thrombosis: magnetic resonance imaging and management. J Urol, 160: 494, 1998 Accepted for publication June 16, 2000. *Requests for information on laboratory tests: Division of Angiol- ogy, Department of Medicine, University of Dresden, Fetscher- strasse 74, 01307 Dresden, Germany. Sonographic cross section through proximal penis shows incom- pressible vein (DDPV) dorsally, superficial to corpora cavernosa. CC, left corpus cavernosum. CS, corpus spongiosum. Inset shows throm- bosed deep dorsal vein (DDPV) and adjacent deep dorsal arteries (DDPA). 0022-5347/00/1645-1649/0 THE JOURNAL OF UROLOGY ® Vol. 164, 1649, November 2000 Copyright © 2000 by AMERICAN UROLOGICAL ASSOCIATION,INC. ® Printed in U.S.A. 1649

SPONTANEOUS THROMBOSIS OF THE DEEP DORSAL PENILE VEIN IN A PATIENT WITH THROMBOPHILIA

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SPONTANEOUS THROMBOSIS OF THE DEEP DORSAL PENILE VEIN INA PATIENT WITH THROMBOPHILIA

BENJAMIN A. SCHMIDT,* THOMAS SCHWARZ AND SEBASTIAN M. SCHELLONGFrom the Division of Angiology, Department of Medicine, University of Dresden, Dresden, Germany

KEY WORDS: penis, thrombosis, thrombophilia

While superficial dorsal penile vein thrombosis has beenassociated with inherited thrombophilia,1 and deep dorsalpenile vein thrombosis following trauma2 and thrombosis ofthe corpora cavernosa have previously been described, to ourknowledge there are no reports of spontaneous deep dorsalpenile vein thrombosis. We report such a case and discuss thefunctional and therapeutic issues of this disorder.

CASE REPORT

A 60-year-old man presented with painless flaccid penileswelling, which had been massive at onset 1 week earlier. Therewas no history of penile trauma. The interval since sexualintercourse was 3 days. Erection was reported to be impossiblebecause of severe pain. Symptoms had been preceded by a mildgastrointestinal infection. Repeat urological consultation hadnot established a diagnosis. Physical examination revealedmoderate penile edema without discoloration, induration ortenderness to palpation. History was significant for spontane-ous calf vein and saphenous vein thrombosis, which had beentreated with oral anticoagulants for 4 months in 1997. Therewas a family history of deep venous thrombosis in the mother ofthe patient and 1 of 4 of his siblings.

Duplex sonography showed isolated occlusive thrombosisof the deep dorsal penile vein without involvement of iliacveins, inferior vena cava or superficial dorsal penile vein (seefigure). Laboratory tests revealed resistance to activated pro-tein C ratio of 1.56 (normal greater than 2.2) and heterozy-gous factor V Leiden mutation but no prothrombin genemutation G20210A. Tests were negative for antinuclear an-tibodies, antiphospholipid-IgG and lupus anticoagulant.Complete blood count, protein C, protein S, free protein S,thromboplastin time, activated partial thromboplastin time,antithrombin, fibrinogen and prostate specific antigen wereall normal. IgM antibodies were 15.9 to phosphatidylserine(normal less than 15) and 60.3 to cardiolipin (normal lessthan 44) at presentation, and returned to normal 6 weekslater. Abdominal sonography, chest x-ray and a test for occultgastrointestinal blood provided no evidence of paraneoplasticthrombosis.

Treatment with phenprocoumon was started, with doseadjustments toward International Normalized Ratio 2.0 to3.0, and initially combined with nadroparin. Penile swellingresolved completely in 1 week. At 6-week followup painlesstumescent erection was possible and at 12-week followuperectile function had improved to nearly normal. Followupduplex sonography showed thrombus retraction without re-canalization. Anticoagulant treatment is planned for 4 yearsfor symptomatic thrombophilia, which corresponds to thetreatment duration proved beneficial for recurrent deep ve-nous thrombosis of the leg.3

DISCUSSION

While no therapy is required for superficial vein thrombo-sis2 unless risk factors for thromboembolism have been es-tablished,1 complete and segmental penile thromboses havebeen treated with fibrinolytics and anticoagulation.4 To ourknowledge there have been no reports of spontaneous throm-bosis of the deep dorsal penile vein and no treatment recom-mendations. The deep vein drains the glans, corpus spongio-sum and distal two-thirds of the corpora cavernosa.Therefore, a relationship between deep vein thrombosis andcomplete or segmental penile thrombosis seems probable,and anticoagulation for these disorders is applicable to deeppenile vein thrombosis. In our patient a thrombophilic disor-der seemed likely and long-term anticoagulation was consid-ered mandatory. Although 12 weeks of treatment did notachieve recanalization on imaging, function almost com-pletely improved with conservative management, which cor-responds to results reported for segmental penile thrombo-sis.4

REFERENCES

1. Luzzi, G. A., Pattinson, J. and Wathen, C. G.: Factor V Leidenpresenting with penile vein thrombosis. J Urol, 159: 2093, 1998

2. Evans, D. T. and Ward, O. E.: Dorsal vein thrombosis of the penispresenting to an STD clinic. Genitourin Med, 70: 406, 1994

3. Schulman, S., Granqvist, S., Holmstrom, M. et al.: The durationof oral anticoagulant therapy after a second episode of venousthromboembolism. The Duration of Anticoagulation TrialStudy Group. N Engl J Med, 336: 393, 1997

4. Machtens, S. A., Kuczyk, M. A., Becker, A. J. et al: Partialunilateral penile thrombosis: magnetic resonance imaging andmanagement. J Urol, 160: 494, 1998

Accepted for publication June 16, 2000.*Requests for information on laboratory tests: Division of Angiol-

ogy, Department of Medicine, University of Dresden, Fetscher-strasse 74, 01307 Dresden, Germany.

Sonographic cross section through proximal penis shows incom-pressible vein (DDPV) dorsally, superficial to corpora cavernosa. CC,left corpus cavernosum. CS, corpus spongiosum. Inset shows throm-bosed deep dorsal vein (DDPV) and adjacent deep dorsal arteries(DDPA).

0022-5347/00/1645-1649/0THE JOURNAL OF UROLOGY® Vol. 164, 1649, November 2000Copyright © 2000 by AMERICAN UROLOGICAL ASSOCIATION, INC.® Printed in U.S.A.

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