Penis Disorders: Types and Clinical Representation
Table of Contents
Hypospadiasis
Definition
The term Hypospadiasis epitomizes a congenital abnormality secondary to an arrest in the normal formation of the urethra, ventral aspect of the penis and the foreskin. There is an abnormal opening of the penile urethra on the ventral surface of the penis due to the failure of fusion of the urethral folds.
Epidemiology
Hypospadiasis is present in 1 in every 125 male children.
Pathogenesis
The etiopathogenesis is influenced by multiple factors. Prominent among those are environmental factors implicated during pregnancy, like a predominantly vegetarian diet in the mother and exposure to female sex hormones such as estrogen and progesterone.
Equivocal evidence attests to the presence of certain risk factors summarized as below:
Paternal risk factors:
- Abnormal sperm morphological characteristics
- Abnormal sperm morphological characteristics
- Faulty spermiogenesis secondary to abnormalities of testes or scrotum
Fetal risk factor:
- Low birth weight
Maternal risk factors:
- Parity; primiparous at increased risk compared to multiparous
- Maternal age
There is aberrant complex interplay between epithelial and mesenchymal signaling pathways which manifests as displaced urethral outlet.
Clinical Presentation
More often, anxious parents first diagnose hypospadiasis when the child has an abnormally directed poor stream of urine or has a frank misplaced urethral opening. Depending on the grade and severity of disjunction present, the presentation varies from being minimally symptomatic to having gross penoscrotal defects.
Various clinical characteristics can be summarized as follows:
Classification
Hypospadiasis has been liberally classified by urologists over the years.
The significant relevant classifications are as follows:
Diagnosis
Hypospadiasis is mainly diagnosed clinically. Radiological imaging serves as ancillary tests to rule out complications and associated anomalies.
Therapy
The treatment of Hypospadiasis is purely surgical and the implications extend far beyond cosmesis. The ideal time is between 6 to 18 months of age. All the layers of the penis around the meatus are reconstructed with delicate precision. Many procedures have been described, but the underlying principle is to reconstruct the meatus on the penis and remedy the deformed structures around to ensure normal urination and sexual function.
Various surgical procedures performed are summarized as follows:
More common than epispadiasis, hypospadiasis may result in urethral obstruction and cystitis.
Epispadiasis
Definition
The abnormal epithelial mesenchymal opening of penile urethra on the dorsal surface of the penis due to malpositioning of genital tubercle is termed as “Epispadiasis”. It is associated with abnormal abdominal wall defects such as exstrophy of the bladder. The same is known as “Exstrophy-epispadiasis complex”.
Presentation
Most neonates present with a bifid penis with urethral abnormalities which necessitate surgical intervention emergently.
When chronic, males tend to develop an abnormal curvature of the penis: “chordee.”
Females present with the abnormal clitoris and urinary incontinence.
Treatment
Surgical intervention with comprehensive repair of urethral meatal opening, penile shaft reconstruction and associated mobilization of the corpora is complex. For optimum results, it is advisable to perform the genitourinary reconstruction within the first 7 years of life. The most prevalent and tested intervention is the “Modified Cantwell-Ransley” approach. Other procedures performed include “Complete penile disassembly”.
Phimosis
Definition
Failure to retract the foreskin beyond the glans penis is termed as “phimosis”. This is a normal finding in children less than 5 years of age.
Classification
Phimosis is segregated into congenital and acquired types. The latter being more common is a sequel of poor hygiene, infection, inflammation or trauma leading to smegma accumulation and fibrosis.
Complications
Inability to retract the prepuce over the glans leads to accumulation of smegma and secondary balanitis. A small risk of squamous cell carcinoma also exists.
Treatment
Phimosis is a relatively benign pathology with definite surgical treatment; namely circumcision. Circumcision has an additional benefit of a decreased risk of the spread of HPV and HIV.
Paraphimosis
Definition
Failure to advance back the foreskin over the glans after retraction is known as “paraphimosis.”
Paraphimosis is rather unfortunately iatrogenic most of the time and follows faulty urethral catheterization techniques. It is also seen in congestive types of ischemia.
Complications
Retraction of tight foreskin over the prepuce can lead to strangulation and infarction of glans penis.
Treatment
Circumcision is the definitive treatment for paraphimosis. An emergency dorsal slit technique is also described.
Peyronie Disease
Definition
Also named as “Induratio penis plastica,” it is characterized by chronic inflammation causing an increased thickness of tunica albuginea (CITA) surrounding the corpora cavernosa.
Presentation
Affecting about 5% of males, the presenting complaint is often disturbing abnormal penile curvature with subsequent trouble in maintaining an erection.
One of the differentials to be always considered in these patients is the rupture of corpora cavernosa due to penile fracture.
Treatment
About 12% of patients demonstrate spontaneous regression. However, half of the patients can considerably worsen and hence warrant treatment. There is no unequivocal evidence as to which treatment option is superlative to others. Options include:
- Vitamin E supplements
- Interferon –alpha-2b
- Collagenase injection
- Pentoxyphylline
- Dexamethasone
“Nesbit surgery” requires considerable expertise; hence, it is resorted to “at the end” only if other options fail.
A penile prosthesis may be indicated in advanced cases.
Priapism
Definition
Abnormal, persistent, painful erection lasting for more than 4 hours; or is unrelated to sexual stimulation is “priapism”. It demands urgent attention.
Classification
The two types of priapism can be described as follows:
Epidemiology
SCC is more common in Africa, Asia and South America (almost 10% as against 0.5 per 1,00,000 in the USA) and is most likely due to the unequivocal influence of predominant HPV 16, 18 infections. IHC detects altered p16INK4a protein in these patients.
Risk factors
An elderly, unmarried male in his sixth decade with a history of late circumcision is prone for SCC. Other predisposing factors cited include promiscuous sexual behavior, multiple sex partners, tobacco use and poor hygiene.
Distribution
The topographical distribution of SCC, along the penile length is as follows:
| Location | Percentage |
| Glans | 48% (most common) |
| Penile shaft | Less than 2 % (least common) |
| Glans and prepuce | About 9% |
| Prepuce | 21% |
| Coronal | 6% |
Lymphatic spread for SCC follows the normal drainage pattern of the penis. It transgresses the local limits and spreads to superficial nodes and then progressed to deep inguinal and subsequently the external iliac lymph nodes.
Grading systems
There are 2 grading mechanisms available for SCC.
They are as follows:
Broders grading system:
Based on keratinization, mitosis predominance and nuclear pleomorphism; SCC is segregated into 4 grades.
Maiche system score:
Based on a 5-year survival, there are 3 proposed levels:
Jackson’s classification:
The standard AJCC staging for penile cancer is the TNM staging.
Various treatment options for penile cancer can be summarized as follows:
Presence of any of the following factors after penile lesion excision and lymphadenectomy calls for adjuvant therapy:
- Extra nodal extension of disease
- More than 2 metastatic inguinal nodes
- Pelvic lymph node metastases
Distant metastases has dismal prognosis. Chemotherapy and palliative therapy are available treatment options with only about 30% response rate.
Summary
Penile disorders are a heterogeneous aggregation of conditions extremely distressing to the male population.
Hypospadiasis involves malposition of the urethral meatal opening on the ventral aspect of the penis. Treatment is essentially surgical. Many procedures have been described, the most commonly performed being TIP.
Epispadiasis is characterized by the dorsal opening of the urethral meatus. It is often associated with other genito-urinary defects like exstrophy of the bladder. Surgical repair is often necessary latest by 7 years of age.
Inability to retract the prepuce is known as “phimosis”, while failure to advance back the foreskin after retraction is termed as “paraphimosis”. Both these seemingly devastating conditions are definitively treated by circumcision.
Peyronie’s disease is chronic inflammation of the tunica albuginea surrounding the corpora cavernosa in the penis, leading to abnormal curvature or indentation of the penis with subsequent difficult and often painful erection. While spontaneous regression is often described, about half of the patients need treatment.
Priapism is abnormal, persistent, non-sexual erection. Ischemic priapism is a medical emergency. Non-ischemic priapism is often managed with careful observation.
Squamous cell carcinoma is the most common form of penile cancer. Penile cancer is often preceded by the presence of premalignant lesions. A local excision of these lesions can often evade the grave eventuality of SCC. Carcinoma in situ is a histological diagnosis and demands meticulous examination for basement membrane invasion. CIS can be treated by timely local intervention.
SCC is a frank, invasive, and most common penile cancer. Treatment is often tailored as per topographical distribution, local and distal progression and lymph node status.
Review Questions
The correct answers can be found below the references.
1. Surgical treatment for Peyronie’s disease is…
- Whipple’s surgery
- Nesbit surgery
- Hunter surgery
- Macmillan surgery
2. False about carcinoma in situ is:
- It seldom progresses to invasive carcinoma
- It is a histological diagnosis
- Local treatment is sufficient
- Bowen’s disease is a premalignant penile condition.
3. True about ischemic priapism is:
- It is often painless
- It is seldom associated with systemic malignancy
- It is a medical emergency
- It requires observation only.
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