Priapism is an uncommon condition of prolonged erection. It is usually painful for the patient, and no sexual excitement or desire is present. The disorder is idiopathic in 60% of cases, while the remaining 40% of cases are associated with diseases (eg, leukemia, sickle cell disease, pelvic tumors, pelvic infections), penile trauma, spinal cord trauma, or use of medications (trazodone). Currently, intracavernous injection therapy for impotence may be the most common cause. Although the idiopathic type often is initially associated with prolonged sexual stimulation, cases of priapism due to the other causes are unrelated to psychic sexual excitement.
Priapism may be classified into high- and low-flow types. High-flow priapism (nonischemic) usually occurs secondary to perineal trauma, which injures the central penile arteries and results in loss of penile blood-flow regulation. Aneurysms of one or both central arteries have been observed. Aspiration of penile blood for blood–gas determination demonstrates high oxygen and normal carbon dioxide levels. Arteriography is useful to demonstrate aneurysms that will respond to embolization; erectile function is usually preserved.
The patient with low-flow priapism (ischemic) usually presents with a history of several hours of painful erection. The glans penis and corpus spongiosum are soft and uninvolved in the process. The corpora cavernosa are tense with congested blood and tender to palpation. The current theories regarding the mechanism of priapism remain in debate, but most authorities believe the major abnormality to be physiologic obstruction of the venous drainage. This obstruction causes buildup of highly viscous, poorly oxygenated blood (low O2, high CO2) within the corpora cavernosa. If the process continues for several days, interstitial edema and fibrosis of the corpora cavernosa will develop, causing impotence.
Ischemic priapism must be considered a urologic emergency. Epidural or spinal anesthesia can be used. The sludged blood can then be evacuated from the corpora cavernosa through a large needle placed through the glans. The addition of adrenergic agents administered via intracavernous irrigation has proved helpful. Monitoring intracavernous pressure ensures that recurrence is not imminent. Multiple wedges of tissue can be removed with a biopsy needle to create a shunting fistula between the glans penis and corpora cavernosa. This technique, which has been very successful, provides an internal fistula to keep the corpora cavernosa decompressed. To maintain continuous fistula drainage, pressure should be exerted intermittently (every 15 minutes) on the body of the penis. The patient can do this manually after he has recovered from anesthesia.
If the shunt described fails, another shunting technique may be used by anastomosing the superficial dorsal vein to the corpora cavernosa. Other effective shunting methods are corpora cavernosa to corpus spongiosum shunt by perineal anastomosis; saphenous vein to corpora cavernosa shunt; and pump decompression.
Patients with sickle cell disease have benefited from massive blood transfusions, exchange transfusions, or both. Hyperbaric oxygen also has been suggested for these patients. Patients with leukemia should receive prompt chemotherapy. Appropriate management of any underlying cause should be instituted without delay. Such treatment should not prevent aggressive management of the priapism if the erection persists for several hours.
Impotence is the worst sequel of priapism. It is more common after prolonged priapism (several days). Early recognition (within hours) and prompt treatment of priapism offer the best opportunity to avoid this major problem.
Peyronie's disease (plastic induration of the penis) was first described in 1742 and is a well-recognized clinical problem affecting middle-aged and older men. Patients present with complaints of painful erection, curvature of the penis, and poor erection distal to the involved area. The penile deformity may be so severe that it prevents satisfactory vaginal penetration. The patient has no pain when the penis is in the nonerect state.
Examination of the penile shaft reveals a palpable dense, fibrous plaque of varying size involving the tunica albuginea. The plaque is usually near the dorsal midline of the shaft. Multiple plaques are sometimes seen. In severe cases, calcification and ossification are noted and confirmed by radiography. Although the cause of Peyronie's disease remains obscure, the dense fibrous plaque is microscopically consistent with findings in severe vasculitis. The condition has been noted in association with Dupuytren's contracture of the tendons of the hand, in which the fibrosis resembles that of Peyronie's disease when examined microscopically.
Spontaneous remission occurs in about 50% of cases. Initially, observation and emotional support are advised. If remission does not occur, p-aminobenzoic acid powder or tablets or vitamin E tablets may be tried for several months. However, these medications have limited success. In recent years, a number of operative procedures have been used in refractory cases. Excision of the plaque with replacement with a dermal or vein graft has been successful, as has the use of tunica vaginalis grafts after plaque incision. Other authors have incised the plaque and inserted penile prostheses in the corpora cavernosa. Additional methods include radiation therapy and injection of steroids, dimethyl sulfoxide, or parathyroid hormone into the plaque. The success of such treatments is poorly documented.
Phimosis is a condition in which the contracted foreskin cannot be retracted over the glans. Chronic infection from poor local hygiene is its most common cause. Most cases occur in uncircumcised males, although excessive skin left after circumcision can become stenotic and cause phimosis. Calculi and squamous cell carcinoma may develop under the foreskin. Phimosis can occur at any age. In diabetic older men, chronic balanoposthitis may lead to phimosis and may be the initial presenting complaint. Children younger than 2 years seldom have true phimosis; their relatively narrow preputial opening gradually widens and allows for normal retraction of foreskin over the glans. Circumcision for phimosis should be avoided in children requiring general anesthesia; except in cases with recurrent infections, the procedure should be postponed until the child reaches an age when local anesthesia can be used.
Edema, erythema, and tenderness of the prepuce and the presence of purulent discharge usually cause the patient to seek medical attention. Inability to retract the foreskin is a less common complaint.
The initial infection should be treated with broad-spectrum antimicrobial drugs. The dorsal foreskin can be slit if improved drainage is necessary. Circumcision, if indicated, should be done after the infection is controlled.
Paraphimosis is the condition in which the foreskin, once retracted over the glans, cannot be replaced in its normal position. This is due to chronic inflammation under the redundant foreskin, which leads to contracture of the preputial opening (phimosis) and formation of a tight ring of skin when the foreskin is retracted behind the glans. The skin ring causes venous congestion leading to edema and enlargement of the glans, which make the condition worse. As the condition progresses, arterial occlusion and necrosis of the glans may occur.
Paraphimosis usually can be treated by firmly squeezing the glans for 5 minutes to reduce the tissue edema and decrease the size of the glans. The skin can then be drawn forward over the glans. Occasionally, the constricting ring requires incision under local anesthesia. Antibiotics should be administered and circumcision should be done after inflammation has subsided.
Although circumcision is routinely performed in some countries for religious or cultural reasons, it is usually not necessary if adequate penile cleanliness and good hygiene can be maintained. There is a higher incidence of penile carcinoma in uncircumcised males, but chronic infection and poor hygiene are usually underlying factors in such instances. Circumcision is indicated in patients with infection, phimosis, or paraphimosis (see preceding sections).
Acquired urethral stricture is common in men but rare in women. (Congenital urethral stricture is discussed earlier in the chapter.) Most acquired strictures are due to infection or trauma. Although gonococcal urethritis is seldom a cause of stricture today, infection remains a major cause—particularly infection from long-term use of indwelling urethral catheters. Large catheters and instruments are more likely than small ones to cause ischemia and internal trauma. External trauma, for example, pelvic fractures (see Chapter 17), can partially or completely sever the membranous urethra and cause severe and complex strictures. Straddle injuries can produce bulbar strictures.
Urethral strictures are fibrotic narrowings composed of dense collagen and fibroblasts. Fibrosis usually extends into the surrounding corpus spongiosum, causing spongiofibrosis. These narrowings restrict urine flow and cause dilation of the proximal urethra and prostatic ducts. Prostatitis is a common complication of urethral stricture. The bladder muscle may become hypertrophic, and increased residual urine may be noted. Severe, prolonged obstruction can result in decompensation of the ureterovesical junction, reflux, hydronephrosis, and renal failure. Chronic urinary stasis makes infection likely. Urethral fistulas and periurethral abscesses commonly develop in association with chronic, severe strictures.
A decrease in urinary stream is the most common complaint. Spraying or double stream is often noted, as is postvoiding dribbling. Chronic urethral discharge, occasionally a major complaint, is likely to be associated with chronic prostatitis. Acute cystitis or symptoms of infection are seen at times. Acute urinary retention seldom occurs unless infection or prostatic obstruction develops. Urinary frequency and mild dysuria may also be initial complaints.
Induration in the area of the stricture may be palpable. Tender enlarged masses along the urethra usually represent periurethral abscesses. Urethrocutaneous fistulas may be present. The bladder may be palpable if there is chronic retention of urine.
If urethral stricture is suspected, urinary flow rates should be determined. The patient is instructed to accumulate urine until the bladder is full and then begin voiding; a 5-second collection of urine should be obtained during midstream maximal flow and its volume recorded. After the patient repeats this procedure eight to ten times over several days in a relaxed atmosphere, the mean peak flow can be calculated. With strictures creating significant problems, the flow rate will be <10 mL/s (normal 20 mL/s).
Urine culture may be indicated. The midstream specimen is usually bacteria free, with some pyuria (eight to ten white blood cells [leukocytes] per high-power field) in a carefully obtained first aliquot of urine. If the prostate is infected, bacteria will be present in a specimen obtained after prostatic massage. In the presence of cystitis, the urine will be grossly infected.
A urethrogram or voiding cystourethrogram (or both) will demonstrate the location and extent of the stricture. Sonography has also been a useful method of evaluating the urethral stricture. Urethral fistulas and diverticula are sometimes noted. Vesical stones, trabeculations, or diverticula may also be seen.
Urethroscopy allows visualization of the stricture. Small-caliber strictures prevent passage of the instrument through the area. Direct visualization and sonourethrography aid in determining the extent, location, and degree of scarring. Additional areas of scar formation adjacent to the stricture may be detected by urethroscopy.
The stricture can be calibrated by passage of bougies à boule.
Benign or malignant prostatic obstruction can cause symptoms similar to those of stricture. After prostatic surgery, bladder neck contracture can develop and induce stricture-like symptoms. Rectal examination and panendoscopy adequately define such abnormalities of the prostate. Urethral carcinoma is often associated with stricture; urethroscopy demonstrates a definite irregular lesion, and biopsy establishes the diagnosis of carcinoma.
Complications include chronic prostatitis, cystitis, chronic urinary infection, diverticula, urethrocutaneous fistulas, periurethral abscesses, and urethral carcinoma. Vesical calculi may develop from chronic urinary stasis and infection.
Dilation of urethral strictures is not usually curative, but it fractures the scar tissue of the stricture and temporarily enlarges the lumen. As healing occurs, the scar tissue reforms.
Dilation may initially be required because of severe symptoms of chronic retention of urine. The urethra should be liberally lubricated with a water-soluble medium before instrumentation. A filiform is passed down the urethra and gently manipulated through the narrow area into the bladder. A follower can then be attached (see Chapter 10) and the area gradually dilated (with successively larger sizes) to approximately 22F. A 16F silicone catheter can then be inserted. If difficulty arises in passing the filiform through the stricture, urethroscopy should be used to guide the filiform under direct vision.
An alternative method of urethral dilation employs Van Buren sounds. These instruments are best used by an experienced urologist familiar with the size and extent of the stricture involved. First, a 22F sound should be passed down to the stricture site and gentle pressure applied. If this fails, a 20F sound should be used. Smaller sounds should be used with care, because they can easily perforate the urethral wall and produce false passages. Bleeding and pain are major problems caused by dilation.
Urethrotomy under Endoscopic Direct Vision
Lysis of urethral strictures can be accomplished using a sharp knife attached to an endoscope. The endoscope provides direct vision of the stricture during cutting. A filiform should be passed through the stricture and used as a guide during lysis. The stricture is usually incised circumferentially with multiple incisions. A 22F instrument should pass with ease. A catheter is left in place for a short time to prevent bleeding and pain. Results of this procedure have been satisfactory in short-term follow-up in 70–80% of patients, but long-term success rates are much lower. The procedure has several advantages: (1) minimal anesthesia is required—in some cases, only topical anesthesia combined with sedation; (2) it is easily repeated if the stricture recurs; and (3) it is very safe, with few complications.
If urethrotomy under direct vision fails, open surgical repair should be performed. Short strictures (≤2 cm) of the anterior urethra should be completely excised and primary anastomosis done. If possible, the segment to be excised should extend 1 cm beyond each end of the stricture to allow for removal of any existing spongiofibrosis and improve postoperative healing.
Strictures >2 cm in length can be managed by patch graft urethroplasty. The urethra is incised in the midline for the full length of the stricture plus an additional 0.5 cm proximal and distal to its ends. A full-thickness skin graft is obtained—preferably from the penile skin or buccal mucosa—and all subcutaneous tissues are carefully removed. The graft is then tailored to cover the defect and meticulously sutured into place (Figure 41–4).
Left: Urethrogram demonstrating multiple anterior urethral strictures. Right: Voiding cystourethrogram following a patch skin graft of 14 cm in the same patient. There are no residual strictures.
In very long, densely fibrotic strictures, the distal penile fasciocutaneous flap technique has been successful in >80% cases. This single-stage procedure can be combined with buccal mucosa grafting in panurethral strictures. In adults, grafts from buccal mucosa or penile skin should be applied with an onlay technique in the bulbar region of the urethra to maximize graft vascularization from the corpus spongiosum.
Strictures involving the membranous urethra ordinarily result from external trauma (see Chapter 17) and present problems in reconstruction. Most can be corrected by a perineal approach with excision of the urethral rupture defect and direct anastomosis of the bulbar urethra to the prostatic urethra (Figure 41–5). At times, partial pubectomy from the perineal approach can be done to improve urethral approximation without tension on the anastomosis. Rarely, total pubectomy combined with the perineal approach is required to accomplish the direct end-to-end anastomosis.
Voiding urethrogram following repair of traumatic posterior urethral stricture. Arrow indicates that area of repair is stricture free.
These single-stage procedures have a high success rate and create a urethra free of hair—a major problem seen with two-stage procedures. Although seldom required, two-stage procedures are important reconstructive techniques to be considered in complex urethral strictures.
Treatment of Complications
Urinary tract infection in patients with strictures requires specific antimicrobial therapy, followed by long-term prophylactic therapy until the stricture has been corrected. Periurethral abscesses require drainage and use of antimicrobial drugs. Urethral fistulas usually require surgical repair.
A stricture should not be considered “cured” until it has been observed for at least 1 year after therapy, since it may recur at any time during that period. Urinary flow rate measurements and urethrograms are helpful to determine the extent of residual obstruction.
Urethral Condylomata Acuminata (Urethral Warts)
Condylomata acuminata are uncommon in the urethra and are almost always preceded by lesions on the skin. They are wart-like papillomas caused by a papilloma virus and are usually transmitted by direct sexual contact but may be transmitted nonsexually.
Patients commonly complain of bloody spotting from the urethra and occasionally have dysuria and urethral discharge. Examination of the urethral meatus often reveals a small, protruding papilloma. If a lesion is not found in this location, the meatus should be separated with the examining fingers so that the distal urethra can be inspected. About 90% of such lesions are situated in the distal urethra. Complete urethroscopy must be done to be certain other lesions do not exist.
Lesions of the meatus can be treated by local excision. A local anesthetic is applied to the area at the base of the lesions, and the pedunculated lesions are sharply incised with small scissors. The area is then fulgurated by electrocautery. Meatotomy may be indicated for excision of lesions in the fossa navicularis and glandular urethra.
Deeper lesions may be fulgurated transurethrally with a resectoscope or Bugby electrode. Recently, lesions have been successfully destroyed using a carbon dioxide or holmium laser. Laser therapy does minimal damage to the urethral mucosa, and stricture formation seems less likely with its use.
Multiple lesions have also been treated with fluorouracil, 5% solution or cream. The drug is instilled in the urethra for 20 minutes twice a week for 5 weeks. Care must be taken to protect the penile skin and scrotum from coming in contact with the medication, since it may produce severe irritation.
Lesions may become infected and ulcerated. This suggests carcinoma, and histopathologic confirmation of the diagnosis should be obtained. Rarely, giant condylomata (Buschke-Löwenstein tumors) involving the glans penis and often the urethra may be seen. Such lesions suggest carcinoma and a biopsy must be done. Surgical excision is the treatment of choice.
To prevent recurrence of condylomata acuminata, the sexual partner must also be examined and treated if necessary.
Stenosis of the Urethral Meatus
Newborns are often suspected of having meatal stenosis of some degree. This condition is thought to be secondary to ammonia dermatitis following circumcision and resulting in prolonged irritative meatitis.
Calibration is important, since the visual appearance of the meatus does not correlate well with its actual size. The urethra should easily accept the tip of an 8F pediatric feeding tube. The significance of metal stenosis is debated, but a meatal caliber <5F in children <10 years of age is an indication for meatotomy.
Penile Phlebothrombosis and Lymphatic Occlusion
Superficial veins and lymphatic vessels of the dorsal penile shaft just proximal to the corona may become irritated and inflamed. A careful history usually indicates that minor trauma to the area (eg, from prolonged sexual intercourse) has occurred. Examination reveals a tender, indurated, cord-like structure on the distal penile shaft. Slight erythema may be present.
For clinical purposes, there is no need to distinguish lymphatic and venous causes, since both penile phlebothrombosis and lymphatic occlusion will resolve spontaneously. The patient must be reassured.