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Treatment of Urethral Hemangioma by Selective Arterial Embolization
0022-534 7/86/1366-1304$02.00/0 Vol. 136, December
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright © 1986 by The Williams & Wilkins Co.
TREATMENT OF URETHRAL HEMANGIOMA BY SELECTIVE ARTERIAL EMBOLIZATION W. J. GREIG MURRAY, MATTHEWS. FLETCHER, HUW L. WALTERS
AND
DEREK A. PACKHAM
From the Departments of Urology and Radiology, King's College Hospital, London, England
ABSTRACT
Control of significant hematuria by embolization of angiographically demonstrated vascular malformations of the prostatic and membranous urethra is reported in a patient with multiple diffuse superficial hemangiomas. Angiomatous lesions of the urethra are extremely rare, especially in cases in which generalized cutaneous hemangiomas coexist. We describe the use of selective arterial embolization to terminate an episode of massive urethral bleeding in a patient with multiple hemangiomas. CASE REPORT
A 68-year-old man presented with spontaneous onset of blood-stained urethral discharge and massive hematuria. Physical examination revealed a tense, tender bladder. Generalized small hemangiomatous lesions were noted on the face, neck, trunk and limbs, in addition to more prominent hemangiomas on the glans, penile shaft and scrotum. The penile lesions were tortuous in places, whereas the scrotal lesions resembled more closely the punctate cutaneous lesions seen elsewhere on the body (fig. 1). The lesions had appeared during the fifth decade of life. Each had begun as a small spot, which had gradually enlarged in size. The more prominent lesions on the penis had been treated by local excision elsewhere 20 years previously. The patient suffered a single transient episode of hematuria 6 years later. Excretory urography (IVP) at that time was normal. Cystourethroscopy showed a single small lesion near the center of the trigone, the appearances of which were consistent with an angioma. The hematuria had ceased spontaneously and no treatment was deemed necessary. Subsequently, barium studies of the upper and lower gastrointestinal tract were performed to exclude any involvement by the angiomatous process, and both were normal. A typical lesion on the neck was biopsied. Histological examination revealed dilated thin-walled vascular channels in the upper dermis, which were lined by flat endothelial cells. This appearance confirmed the clinical diagnosis of small cutaneous hemangiomas. On direct questioning no relevant family history was present. A year before hospitalization the patient presented elsewhere complaining of rectal bleeding. According to the hospital records a large external pile was present, which was excised with the patient under general anesthesia. The operative note stated that a "profusion of tiny perianal veins was present". Postoperatively, a disproportionately large amount of hemorrhage ensued, and it is interesting to speculate whether hemangiomas had involved the perianal region. At the present hospitalization the degree of urethral bleeding was sufficient enough to produce symptoms and signs of peripheral circulatory failure. Pulse rate was 110 per minute and blood pressure was 90/60 mm. Hg. Hemoglobin concentration was 8.5 gm. per cent, and platelet count and prothrombin time were within normal limits. Intravenous fluid replacement was· begun before an emergency blood transfusion. A 3-way irrigating urethral catheter was inserted, and a bladder washout yielded large amounts of clot. An IVP showed normal upper Accepted for publication July 25, 1986.
tracts but appearances in the bladder were consistent with residual blood clot. After stabilization of the patient cystourethroscopy was performed. The entire urethra appeared to be rather vascular, and a solitary raised area was present just distal to the verumontanum. At its apex the source of hemorrhage could be seen clearly. The bladder contained some blood clot, which was evacuated. No mucosal abnormality was present. In an effort to control the bleeding point localized transurethral fulguration was attempted. However, this served only to exacerbate the degree of hemorrhage. A urethral catheter was inserted and angiography was performed via the left transfemoral route. The right internal iliac artery was catheterized and a vascular malformation was identified in relation to the prostatic and membranous urethra (fig. 2). The lesion was supplied almost exclusively by the internal pudenda! artery. This angiomatous bed was ablated by absorbable gelatin sponge embolization. Following this procedure the urethral catheter was removed and no further hematuria occurred. The patient was well with no further problems 1 year later. DISCUSSION
Hemangioma of the urethra was first reported in 1895 by Klotz. 1 In the 90 years since then less than 20 cases have been reported. 2 It has been stated that such lesions usually present during the third decade of life but, recently, several children have been described. 2- 4 The usual presentation is spontaneous onset of blood-stained urethral discharge and hematuria. The lesions may be single or multiple and papillary or sessile. Microscopically, they consist of thin-walled vascular spaces lined by endothelial cells. There is much controversy concerning their true nature. Some reports suggest that they are congenital,3 while others have implicated local varicosity of vessels5 or chronic irritation. This uncertainty in the etiology of such lesions is matched by the diversity of opinion concerning the most effective form of treatment. Whatever method is used a high local recurrence rate is characteristic of these lesions. 6 Single or localized lesions usually are excised or fulgurated. However, transurethral fulguration can be unsuccessful3 or, indeed, exacerbate the bleeding as in our case. Furthermore, extensive fulguration will predispose to stricture formation. 2 This cqmplication also has been reported following treatment of urethral hemangioma by radium. It generally is accepted that a radical surgical approach is necessary in more extensive cases. For such multiple hemanc giomas of the urethra a reconstructive operation with the Denis Browne-Swinney-Johanson technique has been advocated. With this combined 2-stage technique an artificial hypospadias is produced to suture the mucosal margins to the skin margins of the urethrotomy. The artificial hypospadias is closed 3 months later with operative tubularization of a buried strip of epithelium.3 A computer search of the world literature failed to reveal a previous report of selective arterial embolization in the treat1304
URETHRAL HEMANGWMA
1305
FIG. 1. A, typical punctate hemangiomatous lesions on hand and wrist. B, similar lesions on scrotum, C, prominent hemangioma on glans penis.
ment of urethral hemangioma. Another interesting feature of our case is the presence of associated hemangiomatous lesions on the external genitalia, limb and trunk. In 1930 Macalpine reported a case of bladder hemangioma associated with hemangiomas of the penis, scrotum, rectum, anus and thigh. 7 Fuleihan and Cordonnier reported a case of bladder hemangioma associated with multiple hemangiomas of the buttock, perineum, vagina and vulva. 8 It has been postulated by Klein and Kaplan that these cases are examples of the Klippel-Trenaunay syn-
drome, a rare condition characterized by cutaneous hemangiomas, varicose veins, and soft tissue and bony hypertrophy of the involved extremity. 9 The investigators also mentioned 6 other cases of bladder hemangiomas associated with angiomas of the external genitalia. Hemangiomas of the glans penis have been treated by cryosurgery 10 and argon laser therapy,1 1 as well as electrofulguration and surgical excision. As in the case of urethral hemangiomas their exact nature is unclear. When there is a well
1306
MURRAY AND ASSOCIATES
mangiomas in patients with gross hematuria. 9 Although the associated genital lesions gave a much higher index of suspicion in our case, the validity of this statement is borne out once again. With selective arteriography we were able not only to confirm the diagnosis but also terminate the urethral bleeding by embolization. REFERENCES
FIG. 2. Right internal iliac arteriogram shows vascular malformation in relation to prostatic and membranous urethra.
defined gap in the underlying corpus cavernosum, herniation of cavernous tissue has been postulated. Alternatively, it has been suggested that the angiomatous lesions may result from vascularization of a hematoma. 12 Finally, hemangioma of the scrotum also is a rare lesion. Scrotal hemangioma can be mistaken easily for a varicocele, but it tends to be more firm, and does not completely empty with the patient in the supine position and when the scrotum is elevated. 13 Simple excision remains the most popular method of treatment. It has been said that the presence of cutaneous hemangiomas should alert urologists to the possibility of urinary tract he-
1. McCrea, L. E.: Angioma of the male urethra: review of the literature and report of a case. Urol. Cutan. Rev., 52: 205, 1948. 2. Roberts, J. W. and Devine C. J., Jr.: Urethral hemangioma: treatment by total excision and grafting. J. Urol., 129: 1053, 1983. 3. Tilak, G. H.: Multiple hemangiomas of the male urethra-treatment by Denis Browne-Swinney-Johanson urethroplasty. J. Urol., 97: 96, 1967. 4. Begley, B. J.: Hemangioma of the male urethra: treatment by Johanson-Denis Browne technique. J. Urol., 84: 111, 1960. 5. Herbut, P. A.: Urological Pathology. Philadelphia: Lea & Febiger, vol. 1, p. 95, 1952. 6. Manuel, E. S., Seery, W. M. and Cole, A. T.: Capillary hemangioma of the male urethra: case report with literature review. J. Urol., 117: 804, 1977. 7. Macalpine, J. B.: Two cases of haemangioma of the bladder. Brit. J. Surg., 18: 205, 1930. 8. Fuleihan, F. M. and Cordonnier, J. J.: Hemangioma of the bladder: report of a case and review of the literature. J. Urol., 102: 581, 1969. 9. Klein, T. W. and Kaplan, G. W.: Klippel-Trenaunay syndrome associated with urinary tract hemangiomas. J. Urol., 114: 596, 1975. 10. Ohtsuka, H., Shioya, N. and Tanaka, S.: Cryosurgery for hemangiomas of the body surface and oral cavity. Ann. Plast. Surg., 4: 462, 1980. 11. Kopf, A. W. and Bart, R. S.: Laser treatment of penile hemangiomas. J. Derm. Surg. Oncol., 11: 20, 1985. 12. Gibson, T. E.: Angioma of glans penis. J. Urol., 20: 501, 1928. 13. Cooper, T. P., Anderson, R. G. and Chapman, W. H.: Hemangioma of the scrotum: a case report, review and comparison with varicocele. J. Urol., 112: 623, 1974.
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright © 1986 by The Williams & Wilkins Co.
TREATMENT OF URETHRAL HEMANGIOMA BY SELECTIVE ARTERIAL EMBOLIZATION W. J. GREIG MURRAY, MATTHEWS. FLETCHER, HUW L. WALTERS
AND
DEREK A. PACKHAM
From the Departments of Urology and Radiology, King's College Hospital, London, England
ABSTRACT
Control of significant hematuria by embolization of angiographically demonstrated vascular malformations of the prostatic and membranous urethra is reported in a patient with multiple diffuse superficial hemangiomas. Angiomatous lesions of the urethra are extremely rare, especially in cases in which generalized cutaneous hemangiomas coexist. We describe the use of selective arterial embolization to terminate an episode of massive urethral bleeding in a patient with multiple hemangiomas. CASE REPORT
A 68-year-old man presented with spontaneous onset of blood-stained urethral discharge and massive hematuria. Physical examination revealed a tense, tender bladder. Generalized small hemangiomatous lesions were noted on the face, neck, trunk and limbs, in addition to more prominent hemangiomas on the glans, penile shaft and scrotum. The penile lesions were tortuous in places, whereas the scrotal lesions resembled more closely the punctate cutaneous lesions seen elsewhere on the body (fig. 1). The lesions had appeared during the fifth decade of life. Each had begun as a small spot, which had gradually enlarged in size. The more prominent lesions on the penis had been treated by local excision elsewhere 20 years previously. The patient suffered a single transient episode of hematuria 6 years later. Excretory urography (IVP) at that time was normal. Cystourethroscopy showed a single small lesion near the center of the trigone, the appearances of which were consistent with an angioma. The hematuria had ceased spontaneously and no treatment was deemed necessary. Subsequently, barium studies of the upper and lower gastrointestinal tract were performed to exclude any involvement by the angiomatous process, and both were normal. A typical lesion on the neck was biopsied. Histological examination revealed dilated thin-walled vascular channels in the upper dermis, which were lined by flat endothelial cells. This appearance confirmed the clinical diagnosis of small cutaneous hemangiomas. On direct questioning no relevant family history was present. A year before hospitalization the patient presented elsewhere complaining of rectal bleeding. According to the hospital records a large external pile was present, which was excised with the patient under general anesthesia. The operative note stated that a "profusion of tiny perianal veins was present". Postoperatively, a disproportionately large amount of hemorrhage ensued, and it is interesting to speculate whether hemangiomas had involved the perianal region. At the present hospitalization the degree of urethral bleeding was sufficient enough to produce symptoms and signs of peripheral circulatory failure. Pulse rate was 110 per minute and blood pressure was 90/60 mm. Hg. Hemoglobin concentration was 8.5 gm. per cent, and platelet count and prothrombin time were within normal limits. Intravenous fluid replacement was· begun before an emergency blood transfusion. A 3-way irrigating urethral catheter was inserted, and a bladder washout yielded large amounts of clot. An IVP showed normal upper Accepted for publication July 25, 1986.
tracts but appearances in the bladder were consistent with residual blood clot. After stabilization of the patient cystourethroscopy was performed. The entire urethra appeared to be rather vascular, and a solitary raised area was present just distal to the verumontanum. At its apex the source of hemorrhage could be seen clearly. The bladder contained some blood clot, which was evacuated. No mucosal abnormality was present. In an effort to control the bleeding point localized transurethral fulguration was attempted. However, this served only to exacerbate the degree of hemorrhage. A urethral catheter was inserted and angiography was performed via the left transfemoral route. The right internal iliac artery was catheterized and a vascular malformation was identified in relation to the prostatic and membranous urethra (fig. 2). The lesion was supplied almost exclusively by the internal pudenda! artery. This angiomatous bed was ablated by absorbable gelatin sponge embolization. Following this procedure the urethral catheter was removed and no further hematuria occurred. The patient was well with no further problems 1 year later. DISCUSSION
Hemangioma of the urethra was first reported in 1895 by Klotz. 1 In the 90 years since then less than 20 cases have been reported. 2 It has been stated that such lesions usually present during the third decade of life but, recently, several children have been described. 2- 4 The usual presentation is spontaneous onset of blood-stained urethral discharge and hematuria. The lesions may be single or multiple and papillary or sessile. Microscopically, they consist of thin-walled vascular spaces lined by endothelial cells. There is much controversy concerning their true nature. Some reports suggest that they are congenital,3 while others have implicated local varicosity of vessels5 or chronic irritation. This uncertainty in the etiology of such lesions is matched by the diversity of opinion concerning the most effective form of treatment. Whatever method is used a high local recurrence rate is characteristic of these lesions. 6 Single or localized lesions usually are excised or fulgurated. However, transurethral fulguration can be unsuccessful3 or, indeed, exacerbate the bleeding as in our case. Furthermore, extensive fulguration will predispose to stricture formation. 2 This cqmplication also has been reported following treatment of urethral hemangioma by radium. It generally is accepted that a radical surgical approach is necessary in more extensive cases. For such multiple hemanc giomas of the urethra a reconstructive operation with the Denis Browne-Swinney-Johanson technique has been advocated. With this combined 2-stage technique an artificial hypospadias is produced to suture the mucosal margins to the skin margins of the urethrotomy. The artificial hypospadias is closed 3 months later with operative tubularization of a buried strip of epithelium.3 A computer search of the world literature failed to reveal a previous report of selective arterial embolization in the treat1304
URETHRAL HEMANGWMA
1305
FIG. 1. A, typical punctate hemangiomatous lesions on hand and wrist. B, similar lesions on scrotum, C, prominent hemangioma on glans penis.
ment of urethral hemangioma. Another interesting feature of our case is the presence of associated hemangiomatous lesions on the external genitalia, limb and trunk. In 1930 Macalpine reported a case of bladder hemangioma associated with hemangiomas of the penis, scrotum, rectum, anus and thigh. 7 Fuleihan and Cordonnier reported a case of bladder hemangioma associated with multiple hemangiomas of the buttock, perineum, vagina and vulva. 8 It has been postulated by Klein and Kaplan that these cases are examples of the Klippel-Trenaunay syn-
drome, a rare condition characterized by cutaneous hemangiomas, varicose veins, and soft tissue and bony hypertrophy of the involved extremity. 9 The investigators also mentioned 6 other cases of bladder hemangiomas associated with angiomas of the external genitalia. Hemangiomas of the glans penis have been treated by cryosurgery 10 and argon laser therapy,1 1 as well as electrofulguration and surgical excision. As in the case of urethral hemangiomas their exact nature is unclear. When there is a well
1306
MURRAY AND ASSOCIATES
mangiomas in patients with gross hematuria. 9 Although the associated genital lesions gave a much higher index of suspicion in our case, the validity of this statement is borne out once again. With selective arteriography we were able not only to confirm the diagnosis but also terminate the urethral bleeding by embolization. REFERENCES
FIG. 2. Right internal iliac arteriogram shows vascular malformation in relation to prostatic and membranous urethra.
defined gap in the underlying corpus cavernosum, herniation of cavernous tissue has been postulated. Alternatively, it has been suggested that the angiomatous lesions may result from vascularization of a hematoma. 12 Finally, hemangioma of the scrotum also is a rare lesion. Scrotal hemangioma can be mistaken easily for a varicocele, but it tends to be more firm, and does not completely empty with the patient in the supine position and when the scrotum is elevated. 13 Simple excision remains the most popular method of treatment. It has been said that the presence of cutaneous hemangiomas should alert urologists to the possibility of urinary tract he-
1. McCrea, L. E.: Angioma of the male urethra: review of the literature and report of a case. Urol. Cutan. Rev., 52: 205, 1948. 2. Roberts, J. W. and Devine C. J., Jr.: Urethral hemangioma: treatment by total excision and grafting. J. Urol., 129: 1053, 1983. 3. Tilak, G. H.: Multiple hemangiomas of the male urethra-treatment by Denis Browne-Swinney-Johanson urethroplasty. J. Urol., 97: 96, 1967. 4. Begley, B. J.: Hemangioma of the male urethra: treatment by Johanson-Denis Browne technique. J. Urol., 84: 111, 1960. 5. Herbut, P. A.: Urological Pathology. Philadelphia: Lea & Febiger, vol. 1, p. 95, 1952. 6. Manuel, E. S., Seery, W. M. and Cole, A. T.: Capillary hemangioma of the male urethra: case report with literature review. J. Urol., 117: 804, 1977. 7. Macalpine, J. B.: Two cases of haemangioma of the bladder. Brit. J. Surg., 18: 205, 1930. 8. Fuleihan, F. M. and Cordonnier, J. J.: Hemangioma of the bladder: report of a case and review of the literature. J. Urol., 102: 581, 1969. 9. Klein, T. W. and Kaplan, G. W.: Klippel-Trenaunay syndrome associated with urinary tract hemangiomas. J. Urol., 114: 596, 1975. 10. Ohtsuka, H., Shioya, N. and Tanaka, S.: Cryosurgery for hemangiomas of the body surface and oral cavity. Ann. Plast. Surg., 4: 462, 1980. 11. Kopf, A. W. and Bart, R. S.: Laser treatment of penile hemangiomas. J. Derm. Surg. Oncol., 11: 20, 1985. 12. Gibson, T. E.: Angioma of glans penis. J. Urol., 20: 501, 1928. 13. Cooper, T. P., Anderson, R. G. and Chapman, W. H.: Hemangioma of the scrotum: a case report, review and comparison with varicocele. J. Urol., 112: 623, 1974.