Abhinav Sidana, MD
Anish K. Shah, MD
BASICS
DESCRIPTION
• Penile pain could be a result of multiple etiologies and present in both a flaccid and erect penis
– Penile pain: Flaccid penis
Usually secondary to inflammation of bladder and urethra with referred pain localized to meatus
Potentially secondary to paraphimosis
– Penile pain: Erect penis
May be secondary to priapism or Peyronie disease (PD)
• Acquired penile curvature in men, known as PD, is an inflammatory condition of the tunica albuginea usually associated with painful erection with curvature. If severe, it can cause dyspareunia
– Can be a result of complications of penile prosthesis implantation
– Congenital chordee may present as penile curvature in infants due to a deficiency in formation of the urethra or the Buck fascia ventrally
– Iatrogenic chordee secondary to circumcision or other penile skin procedures
EPIDEMIOLOGY
Incidence
• Common at all ages, but mean age: 53
• Mostly affects males 40–70 yr old, with 0.4–3.2% incidence
Prevalence
In general 5% of men have evidence of PD
RISK FACTORS
• Buckling erectile trauma
• Fracture of the tunica albuginea during sexual activity
• Intracavernous injection of vasoactive agents
• Priapism
• Urethral and penile surgery
• See also “Commonly Associated Conditions”
Genetics
• Associated with HLA (histocompatability B7) cross-reactive antigens
• PD less frequent in patients with Asian or African ancestry
PATHOPHYSIOLOGY
• Mechanical tunical stress forces causing microvascular hemorrhage and inflammation in the tunical wall or septum. The result is hypertrophic scar formation (plaque).
• Autoimmune components have been demonstrated in 38–75% of men with PD.
• Altered cell-mediated immunity and antielastin antibodies support an immunologic component.
• In the setting of erectile trauma, an altered immunologic response to wound healing may predispose a subpopulation to PD.
ASSOCIATED CONDITIONS
• Chronic intracavernous injection therapy
• Dupuytren contracture
• ED (venoocclusive dysfunction)
• Ledderhose disease (Plantar fasciitis)
• Paget disease
• Tympanic sclerosis
• Urethral stricture
• Hypospadias
GENERAL PREVENTION
Avoidance of penile trauma during intercourse
DIAGNOSIS
HISTORY
• Obtain a thorough medical, surgical, and sexual history (1)[A]
• It is critical to differentiate penile pain from urethral pain: History of voiding symptoms and/or recurrent UTIs
• Establish duration, degree, and location of curvature and pain
• Dyspareunia
• Painful erection (suggests PD)
• Painful ejaculation
• Nature of deformity: Curvature, indentation or instability (hinge effect)
• Condition causes distress to patient or partner
• Coexisting ED
• Palpable nodule
• History of penile prosthesis
• History of penile trauma
• History of penile surgery as a child
• Sickle cell disease suggests predisposition to priapism
• Insect bites
PHYSICAL EXAM
• Circumcised/uncircumcised; retractable foreskin
• Measurement of stretched penile length
• Palpation of stretched shaft to amplify plaque and determine its location and size
• Plaque tenderness
• Location of meatus for evidence of hypospadias
• Evidence of hematoma that suggests acute trauma
• Solitary or multiple plaques
• Erythema and crepitus with Fournier gangrene
• Eggplant sign with penile fracture
DIAGNOSTIC TESTS & INTERPRETATION
Lab
• Usually not useful unless for workup of infections
• Urinalysis with or without culture in cases of referred pain from other GU cause
• Serum-free testosterone (morning) and prolactin levels if erectile dysfunction coexists
• Corporal blood gas for priapism
Imaging
• Radiograph of penile shaft if calcification is suspected.
• Duplex Doppler US with intracavernous pharmacologic injection to assess cavernous arterial function, presence of venoocclusive dysfunction, and degree of erectile curvature, lateral indentation or circumferential wasting.
• Private self-photography of erect penis using instant (Polaroid) or digital film imaging is useful to classify the extent of the curvature.
Diagnostic Procedures/Surgery
• Uroflowmetry to rule out associated urethral stricture
• Bladder US for postvoid residual
• Retrograde urethrogram (if history and exam suggest urethral injury, such as blood at meatus. May be seen with penile fracture.)
• Urethroscopy for urethral pathology
Pathologic Findings
• PD is characterized by a fibrous noncompliant plaque within the tunica albuginea, which may calcify, preventing uniform expansion of the corpora cavernosa during erection.
– Microscopy: Affected tunica albuginea demonstrates nonpolarized arrangement of collagen fibers and disordered arrangement of elastin fibers in PD.
DIFFERENTIAL DIAGNOSIS
• Balanitis, balanoposthitis, paraphimosis
• Cellulitis
• Chordee
• Congenital penile curvature
• Epispadius
• Erectile dysfunction
• Fournier gangrene
• Hypospadias
• Idiopathic urethralgia
• Insect bite
• Penile ischemia (embolic, atherosclerosis)
• Leukemic infiltration of the penile shaft
• Penile cancer
• Penile fracture, trauma, or contusion
• Penile pain syndrome
• Penile prosthesis problem: S-shaped or sigmoid curvature with buckling of prosthesis cylinders that are too long; pain also suggests infected prosthesis components
• Priapism
• Psychiatric causes of pain
• Pudendal neuralgia
• Referred pain, GI (rectum, hemorrhoids, fistula, fissures)
• Referred pain, GU (cystitis, urethritis, prostatitis, retention, urolithiasis, urethral calculus)
• Reiter syndrome
• STD (herpes, chancroid)
• Torn frenulum
• Trauma (GSW, penile fracture)
• Urethral foreign bodies
• Urethral shortening following urethroplasty
• Urethral stricture
TREATMENT
GENERAL MEASURES
Identify the specific cause of the penile pain and/or curvature and treat accordingly
MEDICATION
First Line
• PD
– All oral agents yield insignificant therapeutic benefit:
Vitamin E (antioxidant), potaba (antifibrotic), colchicine (antifibrotic), tamoxifen (antifibrotic), L-carnitine (antioxidant), pentoxifylline
• Paraphimosis
– Urgent manual reduction with firm pressure ± local anesthetic (see section on paraphimosis for details)
• Priapism
– Irrigation and aspiration
– Intracavernosal phenylephrine injection (100–500 mcg/mL)
• Referred pain to penis or urethra should be aimed at treating primary problem
• Treat infectious causes (cellulitis, STD) with antimicrobials
Second Line
• PD intralesional therapy
– Collagenase clostridium histolyticum (CCH) (Xiaflex) (3)[A]
FDA approved curvature deformity of the penis due to the presence of a plaque in PD. Restricted distribution through Risk Evaluation and Mitigation Strategy (REMS) due to the risks of serious adverse reactions, including penile fracture and other serious penile injury
Maximum 4 treatment cycles. Each treatment cycle consists of 2 Xiaflex injection procedures (in which Xiaflex is injected directly into the collagen-containing structure of the penis) and 1 penile modeling procedure performed by the healthcare professional.
Breaks down collagen, promotes remodeling
– Other intralesional agents (off-label) Verapamil (antifibrotic), collagenase (antifibrotic), interferon-α (antifibrotic)
– Steroid therapy
Subcutaneous, nonintralesional injections of triamcinolone (50 mg) (2)[C]
SURGERY/OTHER PROCEDURES
• PD
– Plication of the corporal body
– Plaque incision with graft interposition
Synthetic (Dacron)
Autologous (buccal mucosa, dermis, tunica albuginea, tunica vaginalis)
Prepackaged biologic (porcine small intestine submucosa)
– Penile prosthesis with manual molding, if necessary (4)[A]
• Paraphimosis
– Dorsal slit or incision of constricting band
– Circumcision may be necessary
• Chordee and epispadias
– See specific topic for surgical correction
• Penile Fracture
– See specific topic for surgical correction
• Priapism
– Distal and/or proximal shunting procedures
– See specific topic for surgical correction
• Penile reconstruction of scars, contractures, or other deformities
– Z-plasty or other plastic operation
ADDITIONAL TREATMENT
Radiation Therapy
Ineffective in PD
Additional Therapies
N/A
Complementary & Alternative Therapies
• None have been shown to have convincing benefit
– Vitamin E (α-tocopherol)
– Potaba (potassium aminobenzoate)
– L-carnitine
• Acupuncture
ONGOING CARE
PROGNOSIS
• Prognosis dependent on primary etiology of penile curvature and/or pain.
– Surgical straightening of erection is predictably successful (>85%).
– Shortened penile length and sensory loss may be noted postoperatively.
COMPLICATIONS
• Residual pain and curvature
• Dyspareunia
• Erectile dysfunction and loss of penile length due to PD or surgical repair
FOLLOW-UP
Patient Monitoring
Periodic monitoring of erectile function, penile length, and sensory function
Patient Resources
• The Peyronie Disease Society (www.peyroniessociety.org)
• Urology Care Foundation (http://www.urologyhealth.org/urology/index.cfm?article=115)
REFERENCES
1. Delavierre D, Rigaud J, Sibert L, et al. Symptomatic approach to chronic penile pain. Prog Urol. 2010;20:958–961.
2. Dickstein R, Uberoi J, Munarriz R. Severe, disabling, and/or chronic penile pain associated with Peyronie’s disease: Management with subcutaneous steroid injection. J Androl. 2010;31:445–449.
3. Gelbard M, Mavuduru RM, Agarwal MM, et al. Clinical efficacy, safety, and tolerability of collagenase clostridium histoyliticum for the treatment of peyronie disease in 2 large double-blind, randomized, placebo controlled phase 3 studies. J Urol. 2013;190:199–207.
4. Segal RL, Burnett AL. Surgical Management for Peyronie’s Disease. World J Mens Health. 2013;31:1–11.
ADDITIONAL READING
• AUA Guideline on the Management of Priapism 2003 (http://www.auanet.org/education/guidelines/priapism.cfm)
• www.urologyhealth.org
See Also (Topic, Algorithm, Media)
• Chordee
• Epispadias
• Penile Prosthesis, Models and Descriptions (Table)
• Penile Prosthesis Problems (Infection/Extrusion/Malfunction)
• Penis and Corporal Body Mass
• Penis, Curvature and or Pain Image 
• Peyronie Disease
• Priapism, General
CODES
ICD9
• 605 Redundant prepuce and phimosis
• 608.89 Other specified disorders of male genital organs
• 752.63 Congenital chordee
ICD10
• N47.2 Paraphimosis
• N48.89 Other specified disorders of penis
• Q54.4 Congenital chordee
CLINICAL/SURGICAL PEARLS
• Focused history and physical exam allows for prompt diagnosis and effective treatment.
• High index of suspicion for emergent etiologies (Fournier gangrene, penile fracture, priapism, paraphimosis).
• New data suggests that CCH can significantly reduce the symptoms of Peyronie disease.