Case Report: Corpor~ctomy for Intractable Sickle-Associated Priapism YIH-MING YANG, MD, CYNTHIA A. DONNELL, MD,* JOHN H. FARRER, MD, t VIPUL N. MANKAD, MD

ABSTRACT: Priapism is a complication of sickle cell disease, and for those with severe prolonged attacks, it is serious, often resulting in permanent sexual impairment. Treatment is frustrating, and psychological consequences may be profound. A 20-year-old sickle cell patient with intractable priapism associated with intense pain and penile gigantism underwent multiple but unsuccessful medical and surgical treatment regimens. Finally, corpora cavernosa corporectomy was performed. The pathologic findings of extensive vascular thrombosis and stromal fibrosis underscore the irreversibility of this process and explain the inevitable impotence. Pain and sexual impairment were associated with serious psychological difficulties and suicide attempts. Counseling and close follow-up have improved his outlook considerably. He has been pain-free for 1 year, and future management includes consideration of placement of a penile prosthesis. KEY INDEXING TERMS: Intractable sickle-associated priapism; Corporectomy. [Am J Med Sci 1990; 300(4):231-233.]

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riapism is a relatively common and distressing problem in male patients with sickle cell disease. 1 Severe prolonged attack of priapism, although uncommon, is one of the most challenging problems encountered in these patients. There is no satisfactory treatment for sickle-associated priapism. Supportive medical measures such as hydration and analgesia are usually applied initially. 2 Surgical procedures have been advocated but are associated with high morbidity, including penile deFrom the University of South Alabama Comprehensive Sickle Cell Center and Departments 01 Pediatrics, Pathology, * and Urology, t University 01 South Alabama Medical Center, Mobile, Alabama. Reprint requests: Yih-Ming Yang, MD, Departmental Pediatrics, USA Medical Center, 2451 Fillingim Street, Mobile, AL 36617.

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formities and impotence,3,4 although impotence often follows priapism regardless of the management applied. Treatment for severe prolonged major attack of priapism is therefore difficult and frustrating. We report a 20-year-old sickle cell patient with unusually severe, protracted priapism associated with extreme pain and penile gigantism. He failed to respond to aggressive medical management, corporalglanular shunt, cavernosal-spongiosum shunt, and pudendal artery embolization. A corpora cavernosa corporectomy was performed to reduce the extraordinary size and eliminate the pain. The implication of radical surgical management, pathological findings of resected corpora cavernosa, and psychological impact of severe intractable priapism are discussed. Case Report A 20-year-old black man with sickle cell disease was admitted to a local hospital for severe, persistent priapism associated with intense pain of 3 days duration. He was treated with analgesics and red blood cell transfusions with no improvement, and was transferred to our institution. His first episode of priapism occurred at age 15, followed by intermittent attacks that worsened during the 3 years prior to admission. He reported daily priapism of several hours duration, for which he was treated with analgesics. He had received sporadic red blood cell transfusions during the 5-year period. He had a cavernosal-spongiosum shunt and a corporal-glanular shunt at the ages of 16 and 18, respectively. His penis was noted to be significantly enlarged 1 year after the first episode of priapism, and it continued to enlarge. At age 18 the midshaft penis circumference was 20.3 cm. On admission, the patient was alert but in severe pain. He had an extremely enlarged, tender, firm penis, measuring 19 cm in length and 29 cm in midshaft circumference (Figure 1). He weighed 70 kg, was 180 cm in height, and physical examination showed no other abnormalities. Immediate treatment consisted of intravenous hydration, analgesia, and a partial red cell exchange transfusion. A corporalglanular shunt was performed on the second hospital day. Blood gases were obtained from a mid-shaft aspiration, and showed a normal pH and a PO z of 130 mm Hg. Using a Trucut biopsy needle, a corporotomy was made through the glanular incision. It was extremely difficult to do, as the tissue was very firm and fibrotic. Microscopic examination of the corpora cavernosa revealed fibrosis. A temporary, partial detumescence was noted after the procedure, but it failed to eliminate the enlargement or pain. Red blood cell transfusions and partial exchange transfusions were performed over a I-week period to decrease the blood hemoglobin S level to 30%.

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Corporectomy for Intractable Sickle-Associated Priapism

Figure 1. The patient's penis on the third hospital day, after corporal-glanular shunt was performed. The penile size was essentially the same on admission.

The priapism persisted and the pain worsened, necessitating continuous high-dose morphine infusion of up to 24 mg per hour. Cavernosal-spongiosum shunt was performed on the 12th hospital day, with no improvement. A mid-shaft blood aspirate revealed high P0 2 on the second hospital day. Although that observation could have been due to aspiration of an artery or an AV shunt, in the hope that it was a reflection of a high flow state, the blood supply ofthe cavernous sinus was embolized on the 16th hospital day. Again, no improvement was noticed. The patient continued to require intravenous morphine for pain control, and at one time, epidural morphine was administered for 2 days for extreme pain. He was supported by intravenous fluid, and was transfused periodically to keep his hemoglobin S concentration below 30%. He was unable to wear pants or ambulate due to the intense pain and penile size. After 1 month of hospitalization with vigorous therapy, it was believed that all medical and surgical options had been exhausted, with no success, and that impotence was inevitable. Corpora cavernosa corporectomy was suggested to the patient, and discussed in detail with him and his mother, allowing ample time for him to consider the procedure. Emotional support and counseling were provided as needed. Corpora cavernosa corporectomy was performed 6 weeks after he was hospitalized. The postsurgical course was uneventful. His penis was markedly reduced in size and flaccid. He was discharged 2 weeks after surgery on no opioid analgesics. He has been painfree for 1 year, and has continued to receive counseling for impotence and social anxiety. Pathologic Findings. The corpora cavernosa measured 29.5 X 7.0 X 4.0 cm in maximum dimensions. Gross examination showed dense fibrous stroma with many thrombosed cavernous sinuses. Microscopically, there was a significant increase in septal fibrous tissue with resultant separation of, and in many areas, compression of the cavernae. Some sinuses were reduced to slit-like spaces (Figure 2). Thrombi in various stages of organization, ranging from very recent to remote, were present in some of the vascular spaces (Figure 3). The resultant lumenal compromise ranged from minimal to almost complete. In areas, the only patent vessels were capillary in size, surrounded by fibrous stroma that completely obliterated the larger vascular structures (Figure 4).

Figure 2. A compressed cavernous sinus, left, with adjacent collagen and early fibrosis (X100).

sickle cell patients of all ages. 1 Sickle-associated priapism accounts for 3.7% of male sickle-related hospital admissions. 2,3 Among patients with priapism, sickle cell disease is the most frequently identified cause. Two patterns of priapism have been recognized. 1 One is stuttering priapism, characterized by short (less than 3 hours), repetitive episodes with normal subsequent sexual function. The other is major episode priapism, characterized by severe, prolonged (more than 24 hours) attacks, frequently followed by impotence. A major episode usually brings the patient to medical attention. Penile gigantism of the magnitude that occurred in our patient is a rare consequence of priapism in sickle cell patients. The cause of gigantism of our patient is not clear; however, frequent and repeated attacks of priapism over a prolonged period of time may be an important contributing factor.

Discussion

Priapism, a sustained and painful penile erection, occurs commonly in the ambulatory setting in male

232

Figure 3. An organizing thrombus with resultant near-occlusion of cavernous sinus (X100).

October 1990 Volume 300 Number 4

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Figure 4. Only capillary-sized vessels remain, surrounded by severe fibrosis (X100).

Initial treatment of priapism generally involves supportive measures for the management of acute sickle cell vasoocclusive pain crisis, including vigorous hydration and analgesia. 2,4,5 Simple red blood cell transfusion to raise hemoglobin to 10 gm/dl, or partial exchange transfusion to reduce hemoglobin S to 30% or less are suggested if the erection and pain persist despite therapy for 24 hours. 5 Medical management usually results in diminished pain and partial detumescence. Prolonged priapism produces persistent intravascular sludging and ischemia, which may result in cavernosal cell death, vascular thrombosis, stromal edema, fibrosis, and ultimately, impotence.6 Early surgical intervention is recommended if the patient fails to respond to medical management, preferably at 24 to 48 hours when symptoms, particularly pain, persist or worsen. 7 Cavernosal-glanular shunt followed by cavernosalspongiosum shunt in the event of medical treatment failure has been advocated as a surgical treatment of sickle-associated priapism. These are the most commonly used effective minor procedures, with an acceptably low complication rate. 7 Unfortunately, these interventions did not help our patient. Because of our patient's suspected high-flow-rate priapism, a left pudendal artery embolization was performed after surgical therapy failed. Again, his priapism persisted. Other conservative measures, such as intracorporeal norepinephrine injections, cold penile packs, hot compresses, Sitz baths, hypotensive anesthesia, amyl nitrite, ketamine, estrogen, vasodilators, inhalation

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of 100% oxygen, and local or systemic anticoagulants were not considered for our patient because their effects are less well-defined. Corpora cavernosa corporectomy was performed on our patient 6 weeks after the onset of priapism. At the time, he had a persistently turgid penis associated with severe pain in spite of intensive medical management and three surgical procedures. It was also evident that impotence was inevitable due to the severity and prolonged course of his priapism. The extensive fibrosis and vascular obliteration identified in the pathologic specimen confirmed the clinical impression of irreversibility. Young men whose physiologic sexual dysfunction begins at an early age are particularly at risk for emotional trauma, depression, and substance abuse in response to their impotence.8 In situations where family members are supportive and the young man is sexually informed, less damage is observed. Our patient manifested a variety of problems, including suicide attempts, violence and depression. A sex therapist or counselor is often necessary to assist in the patient's adjustment.8 Prosthetic surgery for post-priapism impotence is difficult due to the end-stage fibrosis characteristic of this disease process. Use of a semi-rigid prosthesis rather than an inflatable one, along with optimization of hematologic parameters and prophylactic antibiotics have been recommended. 9 Placement of a surgical prosthesis 2 years after the corporectomy has been recommended to our patient. References 1. Emond AM, Holman R, Hayes RJ, Serjeant GR: Priapism and impotence in homozygous sickle cell disease. Arch Intern Med 140:1434-1437,1980. 2. Karayalcin G, Imran M, Rosner F: Priapism in sickle cell disease: report of five cases. Am J Med Sci 264:289-293, 1972. 3. Howe GE, Prentiss RJ, Cole JW, Masters RH: Priapism: a surgical emergency. J Urol101:576-579, 1969. 4. Grace DA, Winter CC: Priapism: an appraisal of management of twenty-three patients. J Urol99:301-310, 1968. 5. Baron M, Leiter E: The management of priapism in sickie ceii anemia. J Urol119:610-611, 1978. 6. Winter CC, McDowell G: Experience with 105 patients with priapism: update review of all aspects. J Urol 140:980-983, 1988. 7. Noe HN, Wilimas J, Jerkins GR: Surgical management ofpriapism in children with sickle cell anemia. J Urol126:770-771, 1981. 8. Smith AD: Psychologic factors in the multidisciplinary evaluation and treatment of erectile dysfunction. Urol Clin North Am 15(1):41-51, 1988. 9. Bertram RA, Carson CC, Webster GD: Implantation of penile prostheses in patients impotent after priapism. Urology 26: 325-327, 1985.

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Corporectomy for intractable sickle-associated priapism.

Priapism is a complication of sickle cell disease, and for those with severe prolonged attacks, it is serious, often resulting in permanent sexual imp...
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