Penile Anomalies and Conditions

Penile anomalies and conditions may be congenital or acquired and can affect the urethral opening, prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis, shaft, or glans or the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis. Examples include phimosis, paraphimosis, epispadias, hypospadias, balanitis, Peyronie disease, and priapism. The severity of clinical symptoms varies, but diagnosis of each of these conditions is usually based on the history and physical examination. Treatment varies from medical therapies to surgical intervention. These diagnoses are important to be aware of, since a few (such as phimosis and balanitis) are relatively common, while others (such as paraphimosis and priapism) can have severe complications if not treated in a timely fashion.

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Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

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Overview

Anatomy

The penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis is made up of:

  • Glans:
    • Also known as the head of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis
    • Covered by the prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis, also known as foreskin foreskin The double-layered skin fold that covers the glans penis, the head of the penis. Penis
  • Penile shaft:
    • Made up of 2 columns of erectile tissue known as: 
      • Corpus cavernosa
      • Corpus spongiosum
    • These regions are covered by a membrane: tunica albuginea
  • Urethra: 
    • Runs from the bladder through the center of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis 
    • Terminates with an opening at the tip of the glans, called the urethral meatus

Classification

Penile conditions can be classified on the basis of the affected region of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis:

  • Glans and prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis:
    • Balanitis
    • Phimosis
    • Paraphimosis
  • Penile shaft:
    • Peyronie disease
    • Priapism
  • Urethra:
    • Epispadias
    • Hypospadias

Epispadias and Hypospadias

Epispadias

  • Definition: abnormal opening of the urethra on the dorsal surface of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis 
  • Epidemiology:
    • Very rare
    • Reported incidence: < 1 in 100,000 male newborns
  • Etiology:
    • Defective migration of the genital tubercle → incomplete urethral tubularization
    • Associated with abnormal abdominal wall defects, such as exstrophy of the bladder
  • Clinical presentation: 
    • Meatal opening on the dorsum of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis
    • Ventrally hooded prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis
    • Associated with curvature of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis (chordee)
  • Diagnosis: often made at the time of newborn Newborn A neonate, or newborn, is defined as a child less than 28 days old. A thorough physical examination should be performed within the first 24 hours of life to identify abnormalities and improve outcomes by offering timely treatment. Physical Examination of the Newborn genital examination
  • Management: surgical closure and reconstruction
  • Complications: 
    • ↑ Frequency of urinary tract infections Urinary tract infections Urinary tract infections (UTIs) represent a wide spectrum of diseases, from self-limiting simple cystitis to severe pyelonephritis that can result in sepsis and death. Urinary tract infections are most commonly caused by Escherichia coli, but may also be caused by other bacteria and fungi. Urinary Tract Infections (UTIs)
    • Urinary incontinence Urinary incontinence Urinary incontinence (UI) is involuntary loss of bladder control or unintentional voiding, which represents a hygienic or social problem to the patient. Urinary incontinence is a symptom, a sign, and a disorder. The 5 types of UI include stress, urge, mixed, overflow, and functional. Urinary Incontinence
    • Urinary reflux
    • Infertility Infertility Infertility is the inability to conceive in the context of regular intercourse. The most common causes of infertility in women are related to ovulatory dysfunction or tubal obstruction, whereas, in men, abnormal sperm is a common cause. Infertility
Male baby with epispadias

Male baby with epispadias

Image: “Male baby with epispadias” by Department of Pediatric Urology, University Medical Center Regensburg, Germany. License: CC BY 2.0

Hypospadias

  • Definition: abnormal opening of the urethra on the ventral surface of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis 
  • Epidemiology:
    • Most common congenital penile defect
    • Occurs in approximately 1 in 250 male newborns in the United States
  • Etiology: 
    • Due to failure of urethral folds to close
    • Cause is multifactorial 
    • May be associated with the following factors:
      • Environmental
      • Genetic
      • Endocrine
  • Clinical presentation:
    • Meatal opening on the ventral surface of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis
    • Deficient foreskin foreskin The double-layered skin fold that covers the glans penis, the head of the penis. Penis on the ventral surface → incomplete foreskin foreskin The double-layered skin fold that covers the glans penis, the head of the penis. Penis closure around the glans
    • Associated with chordee
  • Diagnosis: often made at the time of newborn Newborn A neonate, or newborn, is defined as a child less than 28 days old. A thorough physical examination should be performed within the first 24 hours of life to identify abnormalities and improve outcomes by offering timely treatment. Physical Examination of the Newborn genital examination
  • Management: 
    • Surgical correction
    • Not warranted for those with mild defects
  • Complications:
    • ↑ Risk of UTIs
    • Urinary incontinence Urinary incontinence Urinary incontinence (UI) is involuntary loss of bladder control or unintentional voiding, which represents a hygienic or social problem to the patient. Urinary incontinence is a symptom, a sign, and a disorder. The 5 types of UI include stress, urge, mixed, overflow, and functional. Urinary Incontinence
    • Urinary obstruction
    • Infertility Infertility Infertility is the inability to conceive in the context of regular intercourse. The most common causes of infertility in women are related to ovulatory dysfunction or tubal obstruction, whereas, in men, abnormal sperm is a common cause. Infertility

Phimosis and Paraphimosis

Phimosis

  • Definition: inability to retract the prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis over the glans
  • Epidemiology: 1 of the most common penile abnormalities
  • Etiology:
    • Physiologic:
      • Occurs in almost all male newborns
      • Due to normal development of congenital adhesions between the prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis and glans
    • Pathologic: 
      • Nonretractable prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis secondary to scarring
      • Usually a result of infection and inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation
  • Clinical presentation:
    • Nonretractable prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis
    • Physiologic: “ballooning” of the prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis with urination
    • Pathologic:
      • Contracted, white, fibrous ring → scarring
      • Pain
      • Skin irritation
      • Hematuria
  • Diagnosis: physical examination
  • Management:
    • Physiologic phimosis is self-limiting.
    • Good hygiene
    • Gentle dilation and stretching
    • Topical corticosteroid cream
    • Circumcision 
  • Complications: 
    • Paraphimosis
    • Balanitis
    • ↑ Risk of penile cancer Penile cancer Malignant lesions of the penis arise from the squamous epithelium of the glans, prepuce, or penile shaft. Penile cancer is rare in the United States, but there is a higher prevalence in lower socioeconomic regions. The most common histologic subtype is squamous cell carcinoma. Penile Cancer
Phimosis

An erect penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis with phimosis

Image: “Example of an erect penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis with Phimosis. The foreskin foreskin The double-layered skin fold that covers the glans penis, the head of the penis. Penis will not retract.” by Andrew1985. License: Public Domain

Paraphimosis

  • Definition: condition in which the prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis of an uncircumcised penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis gets trapped behind the glans and cannot be reduced:
    • Can lead to strangulation and infarction of the glans 
    • Considered a urologic emergency
  • Etiology: several potential predisposing factors:
    • Phimosis 
    • Trauma
    • Sexual activity
    • Procedures (usually due to not replacing the prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis over the glans after the procedure): 
      • Cystoscopy 
      • Catheterization
  • Clinical presentation:
    • Swelling
    • Pain and tenderness
    • Constricting band of tissue proximal to the glans
    • Retracted prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis does not easily reduce manually
    • If not corrected: ischemia or necrosis → blue or black discoloration 
  • Diagnosis: physical examination
  • Management:
    • Pain control
    • Manual reduction
    • Surgical intervention:
      • Dorsal slit reduction (incision of the constricting band) 
      • Emergent circumcision
  • Complications: necrosis of the glans
Paraphimosis complicated by glans gangrene

Paraphimosis complicated by glans gangrene:
This condition is caused by impairment of lymphatic and venous blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure, leading to swelling and compromise of arterial blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure.

Image: “Demonstrating penile glans gangrene with paraphimotic constriction ring with purulent urethral discharge” by Balkan Medical Journal. License: CC BY 2.5

Balanitis

Definition and epidemiology

  • Balanitis is inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation of the glans penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis.
  • Affects approximately 3% of uncircumcised males globally

Etiology

  • Predisposing factors:
    • Inadequate cleaning of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis and foreskin foreskin The double-layered skin fold that covers the glans penis, the head of the penis. Penis
    • Phimosis
    • Exposure to irritants:
      • Soaps
      • Lubricants
      • Latex
    • Dermatologic conditions:
      • Allergic reaction
      • Eczema Eczema Atopic dermatitis, also known as eczema, is a chronic, relapsing, pruritic, inflammatory skin disease that occurs more frequently in children, although adults can also be affected. The condition is often associated with elevated serum levels of IgE and a personal or family history of atopy. Skin dryness, erythema, oozing, crusting, and lichenification are present. Atopic Dermatitis (Eczema)
      • Psoriasis Psoriasis Psoriasis is a common T-cell-mediated inflammatory skin condition. The etiology is unknown, but is thought to be due to genetic inheritance and environmental triggers. There are 4 major subtypes, with the most common form being chronic plaque psoriasis. Psoriasis
      • Lichen planus Lichen planus Lichen planus (LP) is an idiopathic, cell-mediated inflammatory skin disease. It is characterized by pruritic, flat-topped, papular, purple skin lesions commonly found on the flexural surfaces of the extremities. Other areas affected include genitalia, nails, scalp, and mucous membranes. Lichen Planus
    • Trauma
    • Chronic medical conditions, such as: 
      • Diabetes
      • Obesity Obesity Obesity is a condition associated with excess body weight, specifically with the deposition of excessive adipose tissue. Obesity is considered a global epidemic. Major influences come from the western diet and sedentary lifestyles, but the exact mechanisms likely include a mixture of genetic and environmental factors. Obesity
      • Congestive heart failure Congestive heart failure Congestive heart failure refers to the inability of the heart to supply the body with normal cardiac output to meet metabolic needs. Echocardiography can confirm the diagnosis and give information about the ejection fraction. Congestive Heart Failure or other edematous conditions
  • Common infectious causes:
    • Candida Candida Candida is a genus of dimorphic, opportunistic fungi. Candida albicans is part of the normal human flora and is the most common cause of candidiasis. The clinical presentation varies and can include localized mucocutaneous infections (e.g., oropharyngeal, esophageal, intertriginous, and vulvovaginal candidiasis) and invasive disease (e.g., candidemia, intraabdominal abscess, pericarditis, and meningitis). Candida/Candidiasis 
    • Bacteria 
      • Neisseria Neisseria Neisseria is a genus of bacteria commonly present on mucosal surfaces. Several species exist, but only 2 are pathogenic to humans: N. gonorrhoeae and N. meningitidis. Neisseria species are non-motile, gram-negative diplococci most commonly isolated on modified Thayer-Martin (MTM) agar. Neisseria gonorrhoeae
      • Group B streptococci
      • Chlamydia Chlamydia Chlamydiae are obligate intracellular gram-negative bacteria. They lack a peptidoglycan layer and are best visualized using Giemsa stain. The family of Chlamydiaceae comprises 3 pathogens that can infect humans: Chlamydia trachomatis, Chlamydia psittaci, and Chlamydia pneumoniae. Chlamydia
      • Anaerobes
      • Treponema Treponema Treponema is a gram-negative, microaerophilic spirochete. Owing to its very thin structure, it is not easily seen on Gram stain, but can be visualized using dark-field microscopy. This spirochete contains endoflagella, which allow for a characteristic corkscrew movement. Treponema pallidum
      • Trichomonas
      • Herpes simplex
      • Scabies Scabies Scabies is an infestation of the skin by the Sarcoptes scabiei mite, which presents most commonly with intense pruritus, characteristic linear burrows, and erythematous papules, particularly in the interdigital folds and the flexor aspects of the wrists. Scabies

Clinical presentation

  • Erythema
  • Edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema of the glans
  • Pain and tenderness
  • Pruritus
  • Tight foreskin foreskin The double-layered skin fold that covers the glans penis, the head of the penis. Penis
  • Purulent exudate
  • Ulcerated lesions of the glans and prepuce Prepuce The double-layered skin fold that covers the glans penis, the head of the penis. Penis 
  • Urinary obstruction
  • Lymphadenopathy Lymphadenopathy Lymphadenopathy is lymph node enlargement (> 1 cm) and is benign and self-limited in most patients. Etiologies include malignancy, infection, and autoimmune disorders, as well as iatrogenic causes such as the use of certain medications. Generalized lymphadenopathy often indicates underlying systemic disease. Lymphadenopathy
Balanitis_caused_by_smegma

Erythema and edema of the glans penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis consistent with balanitis

Image: “Balanitis caused by smegma” by MFN24. License: CC0 1.0

Diagnosis

  • Physical examination
  • Further workup is determined based on the history, but may include:
    • Bacterial cultures
    • Herpes simplex virus Virus Viruses are infectious, obligate intracellular parasites composed of a nucleic acid core surrounded by a protein capsid. Viruses can be either naked (non-enveloped) or enveloped. The classification of viruses is complex and based on many factors, including type and structure of the nucleoid and capsid, the presence of an envelope, the replication cycle, and the host range. Virology: Overview testing
    • Potassium hydroxide preparation → assess for Candida Candida Candida is a genus of dimorphic, opportunistic fungi. Candida albicans is part of the normal human flora and is the most common cause of candidiasis. The clinical presentation varies and can include localized mucocutaneous infections (e.g., oropharyngeal, esophageal, intertriginous, and vulvovaginal candidiasis) and invasive disease (e.g., candidemia, intraabdominal abscess, pericarditis, and meningitis). Candida/Candidiasis
    • Gonorrhea Gonorrhea Gonorrhea is a sexually transmitted infection (STI) caused by the gram-negative bacteria Neisseria gonorrhoeae (N. gonorrhoeae). Gonorrhea may be asymptomatic but commonly manifests as cervicitis or urethritis with less common presentations such as proctitis, conjunctivitis, or pharyngitis. Gonorrhea and chlamydia testing

Management

  • Good hygiene, with adequate cleaning of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis and foreskin foreskin The double-layered skin fold that covers the glans penis, the head of the penis. Penis 
  • Avoid irritants
  • Treatment of underlying infection, if present 
  • Management of chronic, predisposing conditions

Complications

  • Phimosis
  • Paraphimosis
  • ↑ Risk of penile cancer Penile cancer Malignant lesions of the penis arise from the squamous epithelium of the glans, prepuce, or penile shaft. Penile cancer is rare in the United States, but there is a higher prevalence in lower socioeconomic regions. The most common histologic subtype is squamous cell carcinoma. Penile Cancer
  • Urethral stricture

Peyronie Disease

Definition

Peyronie disease is a penile deformity caused by fibrosis/scarring of the tunica albuginea.

Epidemiology

  • Prevalence: approximately 5% of men (likely underreported)
  • Mean age: approximately 68 years

Etiology

  • Fibrous plaques may occur because of:
    • Trauma
    • Tissue ischemia
  • Risk factors:
    • Urologic procedures:
      • Catheterization
      • Cystoscopy
      • Transurethral resection of the prostate Prostate The prostate is a gland in the male reproductive system. The gland surrounds the bladder neck and a portion of the urethra. The prostate is an exocrine gland that produces a weakly acidic secretion, which accounts for roughly 20% of the seminal fluid. Prostate and other Male Reproductive Glands
    • Connective tissue Connective tissue Connective tissues originate from embryonic mesenchyme and are present throughout the body except inside the brain and spinal cord. The main function of connective tissues is to provide structural support to organs. Connective tissues consist of cells and an extracellular matrix. Connective Tissue disorders
    • Genetics Genetics Genetics is the study of genes and their functions and behaviors. Basic Terms of Genetics
    • Hypogonadism Hypogonadism Hypogonadism is a condition characterized by reduced or no sex hormone production by the testes or ovaries. Hypogonadism can result from primary (hypergonadotropic) or secondary (hypogonadotropic) failure. Symptoms include infertility, increased risk of osteoporosis, erectile dysfunction, decreased libido, and regression (or absence) of secondary sexual characteristics. Hypogonadism
    • Diabetes
    • Tobacco use
    • Alcohol use
    • Older age

Clinical presentation

  • More often evident with an erection Erection The state of the penis when the erectile tissue becomes filled or swollen (tumid) with blood and causes the penis to become rigid and elevated. It is a complex process involving central nervous system; peripheral nervous systems; hormones; smooth muscles; and vascular functions. Penis
  • Deviation of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis to the affected side
  • Pain
  • Palpable nodules or plaques
Abnormal curvature of penis peyronie disease

Abnormal curvature of the penis Penis The penis is the male organ of copulation and micturition. The organ is composed of a root, body, and glans. The root is attached to the pubic bone by the crura penis. The body consists of the 2 parallel corpora cavernosa and the corpus spongiosum. The glans is ensheathed by the prepuce or foreskin. Penis secondary to Peyronie disease

Image: “Penile deformity secondary to Peyronie’s disease” by Tran VQ et al. License: CC BY 3.0

Diagnosis

The diagnosis is clinical.

Management

  • Observation (for those with mild, stable disease)
  • Medical therapy:
    • Pentoxifylline → prevents collagen deposition and fibrosis
    • Intralesional injections: 
      • Collagenase
      • Verapamil → ↑ collagenase activity, ↓ collagen production
  • Surgical repair for severe cases

Complications

  • Erectile dysfunction Erectile Dysfunction Erectile dysfunction (ED) is defined as the inability to achieve or maintain a penile erection, resulting in difficulty to perform penetrative sexual intercourse. Local penile factors and systemic diseases, including diabetes, cardiac disease, and neurological disorders, can cause ED. Erectile Dysfunction
  • Psychologic effects:
    • Anxiety
    • Low self-esteem

Priapism

Definition

Priapism is an abnormal, persistent erection Erection The state of the penis when the erectile tissue becomes filled or swollen (tumid) with blood and causes the penis to become rigid and elevated. It is a complex process involving central nervous system; peripheral nervous systems; hormones; smooth muscles; and vascular functions. Penis (usually > 4 hours) that is not associated with sexual stimulation.

Epidemiology

  • Incidence: 0.73 case per 100,000 men per year
  • Ischemic priapism is more common (approximately ⅔ of cases are associated with erectile dysfunction treatment).
  • Bimodal age distribution:
    • 5–10 years
    • 20–50 years

Etiology and pathophysiology

  • Ischemic:
    • Caused by failure of venous outflow, which can be due to: 
      • Excessive neurotransmitters
      • Blocked venules → prevent venous drainage
      • Prolonged smooth muscle relaxation
    • Can lead to compartment syndrome Compartment Syndrome Compartment syndrome is a surgical emergency usually occurring secondary to trauma. The condition is marked by increased pressure within a compartment that compromises the circulation and function of the tissues within that space. Compartment Syndrome → tissue damage
    • Causes:
      • Drugs (e.g., sildenafil, amphetamines, hydralazine, prazosin)
      • Sickle cell disease Sickle cell disease Sickle cell disease (SCD) is a group of genetic disorders in which an abnormal Hb molecule (HbS) transforms RBCs into sickle-shaped cells, resulting in chronic anemia, vasoocclusive episodes, pain, and organ damage. Sickle Cell Disease
      • Leukemia
      • Vasculitis
      • Hypercoagulable Hypercoagulable Hypercoagulable states (also referred to as thrombophilias) are a group of hematologic diseases defined by an increased risk of clot formation (i.e., thrombosis) due to either an increase in procoagulants, a decrease in anticoagulants, or a decrease in fibrinolysis. Hypercoagulable States state
  • Nonischemic:
    • Arterial fistula formation → abnormal arterial flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure into the corpora cavernosa 
    • No venous outflow obstruction
    • Usually from a direct injury

Clinical presentation

  • Ischemic:
    • Painful erection Erection The state of the penis when the erectile tissue becomes filled or swollen (tumid) with blood and causes the penis to become rigid and elevated. It is a complex process involving central nervous system; peripheral nervous systems; hormones; smooth muscles; and vascular functions. Penis
    • Rigid
    • If not treated → necrosis
  • Nonischemic:
    • No pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain with erection Erection The state of the penis when the erectile tissue becomes filled or swollen (tumid) with blood and causes the penis to become rigid and elevated. It is a complex process involving central nervous system; peripheral nervous systems; hormones; smooth muscles; and vascular functions. Penis
    • Less rigid
    • Not associated with necrosis

Diagnosis

The diagnosis is suspected on the basis of the history and physical exam. The following can help differentiate ischemic from nonischemic priapism:

  • Cavernosal blood gas:
    • Ischemic:
      • Blood sample will appear black
      • Hypoxia
      • Hypercarbia
      • Acidosis
    • Nonischemic:
      • Blood sample will be red
      • Normal oxygen
      • Normal carbon dioxide
      • Normal pH
  • Doppler ultrasound:
    • Ischemic: minimal or absent blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure in the cavernosal arteries Arteries Arteries are tubular collections of cells that transport oxygenated blood and nutrients from the heart to the tissues of the body. The blood passes through the arteries in order of decreasing luminal diameter, starting in the largest artery (the aorta) and ending in the small arterioles. Arteries are classified into 3 types: large elastic arteries, medium muscular arteries, and small arteries and arterioles. Arteries
    • Nonischemic: normal or high arterial blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure

Management

  • Ischemic:
    • Urologic consultation
    • Cavernosal aspiration
    • Intracavernosal phenylephrine injection → induce smooth muscle contraction Smooth muscle contraction Smooth muscle is primarily found in the walls of hollow structures and some visceral organs, including the walls of the vasculature, GI, respiratory, and genitourinary tracts. Smooth muscle contracts more slowly and is regulated differently than skeletal muscle. Smooth muscle can be stimulated by nerve impulses, hormones, metabolic factors (like pH, CO2 or O2 levels), its own intrinsic pacemaker ability, or even mechanical stretch. Smooth Muscle Contraction → ↑ venous outflow
    • Surgery 
  • Nonischemic:
    • May resolve spontaneously
    • Arteriography with embolization

Complications

Complications of ischemic priapism include:

  • Erectile dysfunction Erectile Dysfunction Erectile dysfunction (ED) is defined as the inability to achieve or maintain a penile erection, resulting in difficulty to perform penetrative sexual intercourse. Local penile factors and systemic diseases, including diabetes, cardiac disease, and neurological disorders, can cause ED. Erectile Dysfunction
  • Penile necrosis

References

  1. Fahmy, M. (2018). Congenital Anomalies of the Penis. Springer.
  2. Baskin, L.S. (2020). Hypospadias: pathogenesis, diagnosis, and evaluation. UpToDate. Retrieved August 10, 2021, from https://www.uptodate.com/contents/hypospadias-pathogenesis-diagnosis-and-evaluation
  3. Baskin, L.S. (2020). Hypospadias: management and outcome. UpToDate. Retrieved August 10, 2021, from https://www.uptodate.com/contents/hypospadias-management-and-outcome
  4. Wilcox, D. (2021). Care of the uncircumcised penis in infants and children. UpToDate. Retrieved August 13, 2021, from https://www.uptodate.com/contents/care-of-the-uncircumcised-penis-in-infants-and-children
  5. Tews, M. (2020). Paraphimosis: clinical manifestations, diagnosis and treatment. UpToDate. Retrieved August 13, 2021, from https://www.uptodate.com/contents/paraphimosis-clinical-manifestations-diagnosis-and-treatment
  6. Tews, M., Singer, J.I. (2020). Balanitis and balanoposthitis in children and adolescents: clinical manifestations, evaluation, and diagnosis. UpToDate. Retrieved August 19, 2021, from https://www.uptodate.com/contents/balanitis-and-balanoposthitis-in-children-and-adolescents-clinical-manifestations-evaluation-and-diagnosis
  7. Barrisford, G.W. (2021). Balanitis in adults. UpTodate. Retrieved August 19, 2021, from https://www.uptodate.com/contents/balanitis-in-adults
  8. Deveci, S. (2021). Priapism. UpToDate. Retrieved August 19, 2021, from https://www.uptodate.com/contents/priapism
  9. Brant, W.O., Bella, A.J., Lue, T.F. (2021). Peyronie’s disease: diagnosis and medical management. UpToDate. Retrieved August 19, 2021, from https://www.uptodate.com/contents/peyronies-disease-diagnosis-and-medical-management
  10. Rabinowitz, R., Cubillos, J. (2020). Penile and urethral anomalies. MSD Manual Professional Version. Retrieved August 19, 2021, from https://www.msdmanuals.com/professional/pediatrics/congenital-renal-and-genitourinary-anomalies/penile-and-urethral-anomalies
  11. Anand, S., Lotfollahzadaeh, S. (2021). Epispadias. StatPearls. Retrieved August 19, 2021, from https://www.ncbi.nlm.nih.gov/books/NBK563180/
  12. McPhee, A.S., Stormont, G., McKay, A.C. (2020). Phimosis. StatPearls. Retrieved August 19, 2021, from https://www.ncbi.nlm.nih.gov/books/NBK525972/
  13. Bragg, B.N., Kong, E.L., Leslie, S.W. (2021). Paraphimosis. StatPearls. Retrieved August 19, 2021, from https://www.ncbi.nlm.nih.gov/books/NBK459233/
  14. Ghory, H.Z., Sharma, R. (2017). Phimosis and paraphimosis. Medscape. Retrieved August 19, 2021, from https://emedicine.medscape.com/article/777539-overview

USMLE™ is a joint program of the Federation of State Medical Boards (FSMB®) and National Board of Medical Examiners (NBME®). MCAT is a registered trademark of the Association of American Medical Colleges (AAMC). NCLEX®, NCLEX-RN®, and NCLEX-PN® are registered trademarks of the National Council of State Boards of Nursing, Inc (NCSBN®). None of the trademark holders are endorsed by nor affiliated with Lecturio.

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