Circumcision helps reduce HIV @ Penile Cancer

Phimosis, Adult Circumcision, and Buried Penis
Author: Richard A Santucci, MD, FACS, Chief of Urology, Detroit Receiving Hospital; Specialist-in-Chief of Urology, Detroit Medical Center; Chief of Urologic Trauma Surgery, Sinai Grace Hospital; Director, The Center for Urologic Reconstruction; Clinical Professor of Urology, Michigan State College of Medicine
Coauthor(s): Ryan P Terlecki, MD, Clinical Instructor in Reconstructive Urology, Department of Urology, Division of Surgery, University of Colorado
Contributor Information and Disclosures

Updated: Apr 15, 2009

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Keywords
Introduction
Phimosis

Phimosis is defined as the inability of the prepuce (foreskin) to be retracted behind the glans penis in uncircumcised males.

Nearly all males are born with congenital phimosis, a benign condition that resolves in the overwhelming majority of infants as they transition into childhood. In 1949, Douglas Gairdner showed that only 4% of infants had a fully retractable foreskin at birth but that 90% did by age 3 years. Contemporary work demonstrates that only 1% of males aged 17 years still have an unretractable foreskin. Adult phimosis (ie, pathologic or true phimosis) may be caused by poor hygiene or an underlying medical condition (eg, diabetes mellitus).

Uncomplicated pathologic phimosis is usually amenable to conservative medical treatment. Failure of medical treatment warrants surgical intervention, usually in the traditional form of a circumcision or preputioplasty.

Although phimosis is the most common indication for adult circumcision, other reported indications include paraphimosis, balanitis without phimosis, condyloma, redundant foreskin, Bowen disease, carcinoma, trauma, disease prophylaxis (eg, HIV infection), and patient choice.

Buried penis

Buried penis was described in the early 20th century as a penis of normal size that lacks an appropriate sheath of skin and is located beneath the integument of the abdomen, thigh, or scrotum. This condition is more common in children, usually presenting in neonates or obese prepubertal boys; however, it can also be seen in adults and has been observed in both circumcised and uncircumcised individuals. Marginal cases may not be diagnosed until adulthood, when increased fat deposition accentuates the problem.

Several classification systems of buried penis have been proposed, although none has been universally adopted in the literature. Maizels et al (1986) differentiated among the terms concealed (before circumcision), trapped (cicatricial [scarred] after circumcision), and buried (associated with adolescence and obesity).1

In most congenital pediatric cases, the buried penis is self-limited. In untreated adults, however, the condition tends to worsen as the abdominal pannus continues to grow.

History of the Procedure
Circumcision and adult circumcision

Circumcision is one of the earliest elective operations known to man. Historically, this procedure has been performed for various religious reasons, social reasons, or both. The practice is considered a commandment in Jewish law and a rule of cleanliness in Islam, although it is not mentioned in the Quran. In biblical times, mothers were responsible for circumcising their newborn sons, with mohels gradually taking over. Priests in ancient Egypt would perform the procedure with their gold-impregnated thumbnail. Female circumcision, which is likely better termed "genital mutilation," has been practiced for centuries by some cultures but is an unacceptable practice and without medical benefit.

Adult circumcision for phimosis is described in textbooks dating from the early 19th century. Alternative procedures for adult phimosis were described as early as 1900 by Cloquet. Surgical attempts to restore the prepuce are also well documented, going as far back as the Old Testament. However, potential psychological and surgical complications have led to closer scrutiny of routine neonatal circumcision. Currently, the American Academy of Pediatrics (AAP) neither recommends nor condemns routine neonatal circumcision.

Studies suggest that circumcised boys are at a lower risk of urinary tract infections (UTIs). To put this in perspective, the approximate likelihood of a UTI occurring in the first year of life is 1 in 100 in uncircumcised boys and 1 in 1000 in circumcised boys. A lower risk of malignancy is also reported in studies of circumcised men, although the incidence is also rare in uncircumcised men. Of note, this decreased risk seems to be associated only with infant circumcision and not with adult procedures.

The theory that circumcision contributes to prevention of sexually transmitted diseases (STDs) was encouraged by a 19th-century report of lower rates of syphilis in Jewish men. Van Howe et al (1999), in an earlier literature review, found no clear evidence that circumcision prevents STDs.2 However, studies have demonstrated that human papillomavirus (HPV) infection, including oncogenic HPV infection, is more prevalent in uncircumcised men, regardless of demographics and sexual history.3 Citing a link between the intact prepuce and sexually transmitted infection, some authorities have gone as far as suggesting that circumcision protects against prostate cancer.4

A recent meta-analysis of 3 randomized controlled trials in South Africa,5 Kenya,6 and Uganda7 has demonstrated that circumcision decreases the risk of HIV infection among heterosexual men by nearly 60%.8 Data from a mathematical model suggest that routine circumcision in southern sub-Saharan Africa could prevent 2 million HIV infections over 10 years.9 The results of the African randomized trials have sparked speculative interest in male circumcision to reduce HIV infection in the United States, especially in areas such as New York City.10 Pask et al (2008) have suggested that the protective benefit of circumcision against HIV infection may result from removal of Langerhans cells and that enhanced keratinization conferred by topical estrogen may therefore represent an alternative to circumcision.11
Surgical correction of buried penis

The first description of the buried penis was in 1919 by Keyes. The first attempted surgical correction of this problem was by Schloss in 1959; in 1968, successful correction was performed in an adult by Glanz. Since then, numerous techniques to correct buried penis have been developed.

Problem
Phimosis

Phimosis is defined as a condition in which the foreskin cannot be retracted behind the head of the penis. Depending on the situation, this condition may be considered either physiologic or pathologic.

Physiologic, or congenital, phimosis is a normal condition of the newborn male. In 90% of cases, natural separation allows the foreskin to retract by age 3 years. However, phimosis persisting into late adolescence or early adulthood need not be considered abnormal.

The entity of pathologic, or true, phimosis is far less common and can affect children or adults. This is associated with cicatricial scarring of the prepuce that is often white in appearance. Phimosis may occur after circumcision if redundant inner prepuce slides back over the glans, with subsequent cicatricial scarring and contraction.

Buried penis

Buried penis is a true congenital disorder in which a penis of normal size lacks the proper sheath of skin and lies hidden beneath the integument of the abdomen, thigh, or scrotum. The literature, on occasion, also refers to this condition as a hidden or concealed penis. Trapped penis is a condition in which the penis becomes inconspicuous secondary to a cicatricial scar, usually after overzealous circumcision. Webbed penis is characterized by obscuration of the penile shaft by scrotal skin webs at the penoscrotal junction. Micropenis (also known as microphallus) represents a penis less than 2 standard deviations below the mean in length when measured in the stretched state. Diminutive penis is a penis that is small, malformed, or both secondary to epispadias, exstrophy, severe hypospadias, chromosomal abnormalities, or intersex conditions.

Frequency
Phimosis

Nearly all males are born with physiologic phimosis. Data have shown that the foreskin is retractable in 90% of boys by age 3 years. Only 1% of boys have physiologic phimosis that persists until age 17 years. Thus, most healthy adult men should not have phimosis; the presence of the disorder in an adult male should raise the suspicion of balanitis (infection of the foreskin), balanoposthitis (infection of glans and foreskin), diabetes, or malignancy.

Approximately 1 in 6 men in the world are circumcised. In the United States, circumcision is the fifth most common procedure; in 1992, the foreskin was removed in 62% of newborn males in the United States.

Buried penis

Congenital buried penis is uncommon. The incidence of buried penis in adulthood is unknown, but it is highly likely that many cases go unreported.

Etiology
Phimosis

Physiologic phimosis is the rule in newborn males. Formation of the prepuce is complete by 16 weeks' gestation. The inner prepuce and glans penis share a common, fused mucosal epithelium at birth. This epithelium separates via desquamation over time as the proper hormonal and growth factors are produced. Thus, neonatal circumcision is a surgical treatment of normal anatomy.

Pathologic, or true, phimosis has several different etiologies. The most common cause is infection, such as posthitis, balanitis, or a combination of the two (balanoposthitis). Diabetes mellitus may predispose to such infections.

Adult circumcision is most commonly performed to correct phimosis. When circumcision is performed for phimosis, 25%-46% of removed foreskins are histologically normal. Other indications for adult circumcision include balanitis xerotica obliterans (BXO), infection without phimosis; paraphimosis; Bowen disease; carcinoma; condylomas (warts); trauma; religious or social reasons; disease prophylaxis (eg, HIV infection); and personal preference.

Buried penis

Various etiologic factors have been proposed to explain congenital buried penis. Recent literature favors dysgenetic dartos tissue with abnormal attachments proximally and to the dorsal cavernosum. A prominent prepubic fat pad is also a common primary factor, in addition to dysgenetic dartos fascia. Secondary buried penis may be the result of an overzealous circumcision with subsequent cicatricial scar (trapped penis), a large hernia, or a hydrocele.

Adults with buried penis are commonly obese and often have a history of trauma or surgery. Adults with this condition may have undergone abdominoplasty with overzealous release of attachments between the Scarpa and dartos fasciae, penile-lengthening procedures, or other genitoinguinal surgeries.

Pathophysiology
Phimosis

The foreskin of an uncircumcised child should not be forcefully retracted. This may result in significant bleeding, as well as glanular excoriation and injury. Consequently, dense fibrous adhesions may form during the healing process, leading to true pathologic phimosis.

Circumcision has been promoted as a means of reducing the risk of UTIs, which are more common in uncircumcised males younger than 6 months. The risk in circumcised infants is approximately 1 in 1000, whereas the risk in uncircumcised infants is about 1 in 100. Some researchers contend that the risk of UTI in these children is not high enough to warrant mandatory circumcision. Interestingly, nongonococcal urethritis (NGU) may be more common in circumcised men. However, a multicenter study suggested that, if a child has associated vesicoureteral reflux (VUR), the benefit of reduced infection risk may be valid support for surgery.12

Another cited indication for circumcision is prevention of STDs. Numerous case-control studies concerning the relationship between the foreskin and HIV infection have been published, with inconsistent results and no definite link. Therefore, it seems that STD prevention is not a justification for routine circumcision. Of note, of the developed nations, the United States has one of the highest rates of STDs, HIV infection, and male circumcision.

Infant circumcision seems to decrease the risk of penile cancer,13 whereas later circumcision does not. Penile cancer is a rare disease in the United States, with an incidence of 1.5 per 100,000 people. In developing countries, the incidence is higher and accounts for up to 10% of malignancies in some African and South American nations. Although primarily a disease of older men, penile cancer has been reported in children. The lowest incidence has been reported in Jews, with a similar incidence in Muslims; both groups have high rates of neonatal circumcision.

Daling et al performed a population-based case-control study in 2005 that examined the importance of circumcision in patients with penile cancer. Preputial status was not found to be a statistically significant factor in penile cancer. The investigators concluded that the role of circumcision in penile cancer prevention is unclear.14 Several studies suggest that poor hygiene may be a stronger risk factor than circumcision status. Although smegma has been implicated as a carcinogenic agent, definitive evidence is lacking.

Adult phimosis may be caused by repeated episodes of balanitis or balanoposthitis. Such infections are commonly due to poor personal hygiene (failure to regularly clean under the foreskin).

Phimosis may be a presenting symptom of early diabetes mellitus. When the residual urine of a patient with diabetes mellitus becomes trapped under the foreskin, the combination of a moist environment and glucose in the urine may lead to a proliferation of bacteria, with subsequent infection, scarring, and eventual phimosis.

Buried penis

The penis is properly formed by 16 weeks' gestation. Congenital buried penis is caused by a developmental anomaly in which the dartos fascia has not developed into the normal elastic configuration to allow the penile skin to move freely over the deeper tissues of the penile shaft. Instead, the dartos layer is inelastic, which prevents the forward extension of the penis and holds it buried under the pubis.

Other possible contributing factors to congenital buried penis include excess prepubic fat, scrotal webbing, deficient penile skin, loose skin, an abnormally low position at which the crura separate, abnormal attachments between the Buck fascia and the tunica albuginea, and insufficient attachment of dartos fascia and skin to the Buck fascia.

The pathophysiology of buried penis in adults differs from that in children and includes iatrogenically induced scar contracture with concurrent descent of the abdominal fat pad. Because the penis is suspended from the pubis by the suspensory ligament, it remains fixed, unlike the prepubic fat. As fat descends over the penis, excessive moisture and bacterial overgrowth may occur. Chronic infection may lead to skin maceration and more scar contracture, further aggravating the problem. In many children, this condition is self-limited. However, in adults, total body fat content typically increases with age, causing the buried penis to worsen over time.


Presentation
Phimosis

Congenital or physiologic phimosis is clinically asymptomatic so is not a cause for concern. It is often associated with "ballooning" of the foreskin during voiding. This is a self-limited phenomenon that, in the absence of pathologic phimosis, does not indicate urinary tract obstruction. Pathologic, or true, phimosis is far less common. Symptoms include skin irritation, dysuria, bleeding, and occasionally enuresis or urinary retention. Physical examination usually reveals white cicatricial scarring at the preputial ring. Meuli et al (1994) devised the following scoring system to rate the severity of phimosis:15

Grade I - Fully retractable prepuce with stenotic ring in the shaft
Grade II - Partial retractability with partial exposure of the glans
Grade III - Partial retractability with exposure of the meatus only
Grade IV - No retractability
Pathologic phimosis may be due to BXO, a genital form of lichen sclerosus et atrophicus. This condition affects both men and boys and represents an absolute indication for circumcision, which may be curative. The etiology of BXO is unknown, and it may represent a premalignant state. Clinically, it presents as severe phimosis and possibly meatal stenosis, glanular lesions, or both.

In older men, when the phimosis is severe, the distal foreskin often appears swollen and erythematous with cracked fissures (see Image 1). Men who are affected report pain and discomfort during sexual activity or when they attempt to retract the foreskin. Unlike in the pediatric population, lower urinary tract voiding symptoms are absent. In older men, acquired phimosis is often associated with poor hygiene but may be a product of diabetes mellitus.

Phimotic foreskin. The distal foreskin is edematous, with cracked fissures. The patient was unable to retract the foreskin.
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Phimotic foreskin. The distal foreskin is edematous, with cracked fissures. The patient was unable to retract the foreskin.


All uncircumcised adult men should have the foreskin retracted to exclude occult carcinoma as a part of a complete urologic examination. Squamous cell carcinoma of the penis may manifest as an ulcerated fungating mass of the glans or the prepuce. Alternatively, carcinoma in situ or penile carcinoma may appear as a velvety macular lesion of the glans (erythroplasia of Queyrat) or the penile shaft (Bowen disease).

Buried penis

Most pediatric cases of buried penis present in neonates or prepubertal boys. The most common age range of patients at presentation is 6 months to 1 year. Adolescents who present with buried penis are usually obese, and weight loss should be advised. Patients may be uncircumcised or circumcised; the latter complicates repair. One series found that 77% of children presenting with buried penis had been previously circumcised, emphasizing a need for pediatric urologists to educate primary care physicians.

The reasons for presentation vary. Often, parents are concerned because they are unable to see the penis, which may also complicate proper hygiene. Occasionally, they may witness ballooning of the foreskin with voiding, and children may be persistently wet if they are voiding into the preputial sac.

Adolescents may report dysuria, dribbling between voids, trouble directing their urinary stream because of difficulty holding the penis, or embarrassment in the locker room. Some patients have a history of balanitis and balanoposthitis, and some have undergone a radical circumcision or even multiple circumcisions.

In addition to some of the symptoms seen in children, adults may present with sexual complaints. These include painful erection, sexual embarrassment, and difficulty with vaginal penetration, especially if the tip of the glans does not project past the male escutcheon. This condition may lead to the inability to void in a standing position and may cause the patient to soil himself while urinating in the seated position. Obesity and diabetes mellitus are commonly associated comorbidities.

On physical examination, the penis may be concealed because it is buried in prepubic tissues; buried and enclosed in scrotal tissue (penis palmatus); trapped by phimosis, traumatic scar tissue, or postcircumcision cicatrix; or hidden secondary to a large hernia or hydrocele. A smooth transition from prepubic to penile skin indicates a buried penis. Trapped penis demonstrates a circumferential groove at the base of the penis. Only Maizels (1986)1 and Burkholder (1983)16 have noted an association between buried penis and renal anomalies. Other genitourinary anomalies have not been associated with this condition.

Indications
Phimosis

The main medical indication for circumcision in children is pathologic phimosis. In a prospective long-term study, 40% of boys treated for phimosis were found to have BXO, which has been linked to the development of penile squamous cell carcinoma (SCC).17 Although potent topical steroids may allow improvement and slow progression, total circumcision is the treatment of choice for BXO and may be curative.

Recurrent balanoposthitis, which affects 1% of boys, is also considered a relative indication for circumcision. However, this condition tends to be self-limited, and even if balanoposthitis is recurrent, preputioplasty and topical steroids represent alternatives to circumcision.18,19,20,21 In patients with balanoposthitis who are sufficiently troubled to warrant surgical intervention, circumcision is always curative.

Paraphimosis results from abuse or accident, not disease, of the foreskin and can be seen at any age. It represents the second most common indication for adult circumcision. Infants may present with paraphimosis if their parents have retracted the prepuce and failed to pull it forward thereafter. Reduction of the foreskin under sedation is almost always possible. However, in some situations, a dorsal slit or circumcision is required (see Paraphimosis). Unrecognized chronic paraphimosis or delay in diagnosis may result in urinary retention or even penile autoamputation.

Other indications for circumcision that are less common include small preputial tumors, multiple preputial cysts or condylomas, and penile lymphedema. A reasonable case may be made for circumcising boys with VUR who suffer from UTIs. In addition, the foreskin may be removed to perform a biopsy of lesions hidden under the prepuce or for definitive radiation therapy for penile cancer.

Occasions arise in which urethral instrumentation—in the form of a cystoscopy or Foley catheterization—is necessary. This may be quite problematic in an adult affected by severe phimosis. In such instances, an emergency bedside dorsal slit can be performed safely and expeditiously. After being discharged, the patient may proceed to undergo formal circumcision.

Many circumcisions are performed for social or religious reasons. Interestingly, only 1 out of 6 schools of Islamic law consider circumcision obligatory, whereas others feel it is to be recommended. Among religious Jews, circumcision is felt to be a commandment from their creator.

In summary, common indications for circumcision include the following:

Phimosis
Paraphimosis
Recurrent balanitis or balanoposthitis
Social or religious reasons
Buried penis

The primary reason that children are referred for correction of the buried penis is cosmesis. In the neonate, observation seems to be a viable option. Children younger than 3 years have a 58% chance of spontaneous resolution. Some pediatric urologists insist that this condition is a developmental stage that will resolve by puberty and feel that correction should therefore be deferred. Evidence has shown, however, that spontaneous resolution does not always occur. Also, in men and adolescents, measures such as diet and exercise are unlikely to be effective.

Other authors feel that, after age 3 years, buried penis requires correction. The primary reason cited is the importance of being able to void while standing during the period of toilet training. There are numerous other indications for repair. For example, a concealed penis can hamper proper hygiene, trap urine, and complicate voiding. This can lead to repeated infections, secondary phimosis, or even urinary retention. In addition, numerous investigators feel that children with buried penis are at risk for psychological and social trauma, even from an early age. Obese boys with a buried penis may be ostracized by their peers and withdraw socially. Surgery often relieves anxiety and may improve self-image.

In adults, buried penis tends to worsen over time as they accumulate more fat. The cicatricial scar does not loosen on its own over time. Urinary and sexual complications can greatly affect daily life. Therefore, surgery is likely necessary in these patients.


Relevant Anatomy
The penis is composed of paired corpora cavernosa, the crura of which are attached to the pubic arch, and the corpus spongiosum (see Image 16). The proximal portion of the corpus spongiosum is referred to as the bulb of the penis, and the glans represents the distal expansion. The urethra traverses the corpus spongiosum to exit at the meatus. The cavernosal bodies produce the male erection when they are engorged with blood.

Cross-section through the body of the penis.
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Cross-section through the body of the penis.

The fascial layers of the penis are continuous with the fascial layers of the perineum and lower abdomen. Dartos fascia represents the superficial penile fascia. Deep to this lies the Buck fascia, which covers the tunica albuginea of the penile bodies. Proximally, the Buck fascia is in continuity with the suspensory ligament of the penis, which attaches to the symphysis pubis.

The penis is supplied by a superficial system of arteries that arise from the external pudendal arteries and a deep system of arteries that stem from the internal pudendal arteries. The superficial blood supply lies in the superficial penile fascia and supplies the penile skin and prepuce. The internal pudendal artery, which arises from the hypogastric artery, gives rise to the penile artery. The penile artery then gives rise to the bulbourethral artery, the urethral artery, and the cavernous artery (deep artery of the penis) before terminating as the dorsal artery of the penis (see Images 14-15).

The arterial blood supply of the penis arises from the internal pudendal artery. The internal pudendal artery gives off branches to the bulbar artery, cavernosal artery, and dorsal penile artery. The bulbar artery continues on as the bulbourethral artery to supply the urethra. The cavernosal artery gives rise to the helicine arteries that are end arteries. The dorsal artery of the penis gives branches off to the circumflex arteries.
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The arterial blood supply of the penis arises from the internal pudendal artery. The internal pudendal artery gives off branches to the bulbar artery, cavernosal artery, and dorsal penile artery. The bulbar artery continues on as the bulbourethral artery to supply the urethra. The cavernosal artery gives rise to the helicine arteries that are end arteries. The dorsal artery of the penis gives branches off to the circumflex arteries.

Dorsal view of the arterial and venous blood supply of the penis.
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Dorsal view of the arterial and venous blood supply of the penis.

Somatic nerve supply to the penis comes by way of the pudendal nerves, which eventually produce the dorsal nerves of the penis on each side. Although cutaneous innervation to the penis is primarily from branches of the pudendal nerve, the proximal portion is supplied by the ilioinguinal nerve after it leaves the superficial inguinal ring. The prepuce has somatosensory innervation by the dorsal nerve of the penis and branches of the perineal nerve. The glans is primarily innervated by free nerve endings and has poor fine-touch discrimination.

Contraindications
Circumcision is generally not performed in children born prematurely or those with blood dyscrasias. It should not be performed in children with congenital penile anomalies such as the following:

Hypospadias
Epispadias
Chordee
Penile webbing
Buried penis