Circumcision

Question 1

Describe the anatomical components of the penis.

Answer 1

The penis is composed of paired corpora cavernosa and a corpus spongiosum.

Corpora cavernosa are comprised of spongy erectile tissue surrounded by the tunica albuginea. The corpus spongiosum is located ventrally and surrounds the urethra. These structures are surrounded by Buck’s fascia, dartos fascia, and skin.

Question 2

Define the blood and nerve supply to the penis.

Answer 2

Penile blood supply originates from the internal pudendal artery which gives rise to the bulbar artery, urethral artery, and common penile artery. The common penile artery branches into the dorsal penile artery as well as the cavernosal artery (Figs. 49.1 and 49.2). The penile skin and prepuce are supplied by the external pudendal artery. The dorsal neurovascular bundle contains the deep dorsal vein, the dorsal penile artery, and the dorsal nerves of the penis (Fig. 49.1).

Question 3

What are the medical indications for circumcision?

Answer 3

Circumcision is commonly performed for the management of phimosis, recurrent episodes of inflammation/infection of the prepuce (e.g. balanoposthitis, posthitis, or balanitis), penile cancer, and balanitis xerotica obliterans (BXO). Non-medical reasons include religious or parental preference.

Question 4

Should neonatal circumcision be performed routinely?

Answer 4

This is a controversial topic with practices varying widely. The benefits and poten- tial risks to circumcision are important to discuss. One benefit of neonatal circum- cision is decreased risk of UTI within the first year of life and a decreased risk of penile cancer in circumcised men. In addition, population-based studies have demonstrated protective effect towards sexually transmitted infections in circum- cised men and their female partners [1, 2]. The American Academy of Pediatrics recognizes these health benefits but states that circumcision requires a shared decision-making process with the parents.

Question 5

What are the major contraindications to newborn circumcision?

Answer 5

• Abnormal prepuce (e.g. incomplete foreskin)
• Hypospadias
• Significant chordee or angulation of penis
• Penoscrotal webbing
• Congenital concealed penis
• Small anatomy so commonly used clamps do not fit well (e.g. prematurity,
micropenis)
• Coagulopathy (hemophilia, Von Willebrand’s disease, omission of newborn
vitamin K administration).

Question 6

What are the risks of circumcision?

Answer 6

• Bleeding
• Infection
• Removing too much or too little skin • Secondary phimosis or scar formation • Meatal stenosis
• Poor cosmetic outcome
• Injury to the glans or urethra
• Penile adhesions.

Question 7

How is circumcision performed?

Answer 7

Neonatal Circumcision is performed using a Gomco clamp, Mogen clamp, or Plastibell typically prior to 6 weeks of life.

Local anesthesia for this procedure includes a dorsal penile block.

Free hand circumcision is the most common method utilized outside of the neonatal period, typically completed after 6 months of age under general anesthesia in conjunction with caudal block or penile block.

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In neonates and infants up to 3–6 months of age, circumcisions are most commonly performed with a Gomco clamp or Plastibell.

The majority of these are performed by pediatricians, obstetricians, and other healthcare providers, rather than pediatric surgeons or urologists.

In older children, a free-hand circumcision allows for accurate removal of the appropriate amount of foreskin.

Alternatively, a clamp technique can be used. The foreskin is dilated, mobilized, and reduced over the glans. The frenulum is divided with bipolar cautery. The excess foreskin is then pulled upward, and amputated over an atraumatic clamp. Bleeders on the shaft are controlled with bipolar cautery.

It is preferable not to use monopolar electrocautery, as penile devascularization has been reported.

The edges of the mucosa and foreskin are then sutured together with 8–16 interrupted, fine, fast-absorbing sutures.

Sutureless circumcision using skin glue is faster and results in excellent results.

The author’s practice is to place four corner stitches to approximate the skin, and then apply tissue glue to the four quadrants.

A free-hand circumcision should be used in cases of BXO or revisions of a previous circumcision.

Sherif

Question 8

What is chordee?

Answer 8

Chordee is defined as ventral (downward) or dorsal (upward) curvature of the penis, typically due to disproportionate growth of the corporal bodies. It is often associated with hypospadias but can be an isolated finding. Lateral penile curva- ture (i.e. left or right) is typically due to corpora cavernosa length disproportion. The need to treat chordee is based on the degree of penile curvature, particularly if functional limitations are likely (e.g. inability to direct the urinary stream, unable to participate in penetrative intercourse later in life).

Question 9

Describe penoscrotal webbing.

Answer 9

Penoscrotal webbing occurs when the ventral junction of the scrotum and penis meets distally along the penile shaft, rather than the normal anatomic location at the base of the penis (Fig. 49.4). This is often a congenital finding, but can be seen with hypospadias, or as a result of excessive removal of ventral penile shaft skin during circumcision.

Question 10

What is a micropenis? Contrast this to a buried or concealed penis?

Answer 10

Micropenis is defined as a penile length measuring more than 2.5 standard devia- tions below the mean in a newborn male. In a term newborn male, this equates to a stretched penile length of less than 2 cm [3]. Newborn babies with micropenis require endocrine evaluation as this is typically due to an endocrinopathy (hypo- gonadotropic or hypergonadotropic hypogonadism). In contrast, a buried penis is one that appears small but has a normal stretched penile length. The most common etiology of buried/concealed penis is a large prepubic fat pad. To accurately meas- ure the penile length, the prepubic fat must be pushed back prior to measuring.

Question 11

What is Phimosis?

Answer 11

Phimosis is defined as the inability to retract the foreskin (Fig. 49.5). Phimosis is a normal finding in newborns and requires no intervention. When the phimotic ring is significantly tight, it can lead to ballooning of the foreskin during voiding, chronic irritation, and/or recurrent infections. Foreskin should probably be retract- able by the time a child is fully potty trained, so as to encourage good voiding and hygiene habits.

Question 12

What are the treatment options for phimosis?

Answer 12

Treatment options for phimosis include observation for spontaneous resolution, application of topical steroid cream, a dorsal slit procedure, or circumcision.

If parents/patients wish to avoid a procedure, a trial of topical steroids cream may facilitate the foreskin to become more elastic and allow for resolution of phimosis in over 75% of patients.

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Phimosis presenting outside the emergency setting can be treated medically or surgically.

Medical treatment involves application of steroid cream once or twice per day for four to eight weeks.

Success rates up to 90% have been reported.

This has also been proposed for cases of BXO, although it is unclear how this diagnosis can be confirmed without a surgical specimen from a circumcision.

In my experience, the success rate of steroid treatment, even with multiple courses, is much lower. Moreover, phimosis is a spectrum that varies from redundant foreskin with a pinhole opening to a wide prepuce with adhesions to the glans or corona.

It is this latter type that seems most amenable to steroid application, as the adhesions will often release with steroids.

Adhesions can also be released in the clinic by applying a topical anesthetic to the area and using a Q-tip to release the foreskin from the corona.

Symptomatic boys who do not respond to one or more courses of steroids should be circumcised.

The end result of a long-standing untreated phimosis may be BXO, as described in the index case presented.

BXO is also a spectrum and histologic changes characteristic of BXO have been reported in up to one-third of circumcision specimens, even when not diagnosed preoperatively.

Sherif

Question 13

What is paraphimosis?

Answer 13

Paraphimosis is when the foreskin is stuck in the retracted position and can- not be pulled forward/reduced due to distal penile edema. This is an emergency. Paraphimosis causes a tourniquet effect on the distal penis which can result in tissue ischemia. The goal of treatment is to reduce the foreskin into its normal position via manual compression and reduction, a dorsal slit, or emergent circumcision.

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Another emergent presentation in uncircumcised boys is paraphimosis.

This is one of the most serious conditions encountered as it essentially represents incarceration of the glans by the retracted foreskin, with possible glandular ischemia.

Urgent reduction of the prepuce over the glans is necessary.

This is usually accomplished by squeezing the glans and pushing it through the preputial ring.

A penile block or intravenous sedation may be necessary. A number of topical agents used as adjuncts, the most common of which is hyaluronidase, have also been used to resolve the edema of the glans and successfully reduce the paraphimosis.

Emergent circumcision is required if reduction cannot be achieved by noninvasive measures.

Even if successfully reduced, a single episode of paraphimosis is a reasonable indication for circumcision.

Sherif

Question 14

What is balanitis xerotica obliterans (BXO)?

Answer 14

Balanitis xerotica obliterans, or lichen sclerosis, is a chronic, inflammatory vascu- litis. It is most often associated with chronic inflammation secondary to phimosis. It results in white discoloration of the involved tissues, which can include the prepuce, glans, meatus, and urethra.

Treatment involves surgical excision of the affected skin (circumcision) or application of steroid cream to decrease the inflammatory response.

When BXO involves the meatus or urethra, this can lead to urethral stricture disease.

Question 15

What is meatal stenosis?

Answer 15

Meatal stenosis, narrowing of the urethral meatus, is thought to be due to chronic irritation of the urethral meatus. It is most often observed in circumcised patients. The most common symptom of meatal stenosis is change in urinary stream such as spraying or deviation of the stream. Treatment is recommended when changes in the urinary stream are noted and bothersome. This involves either a meatotomy or a meatoplasty.

Question 16

What is hypospadias?

Answer 16

Hypospadias is the second most common congenital abnormality of the urinary tract. It is seen in approximately 1 in 300 live birth males. It is characterized by a urethral meatus which opens on the ventral surface of the penis (Figs. 49.7 and 49.8). The etiology of hypospadias is multifactorial with genetic factors, inade- quate hormonal stimulation, maternal/placental factors, and environmental factors implicated.

Question 17

What are the typical physical findings in patients with hypospadias?

Answer 17

• Urethral meatus located in an abnormal location on the ventral surface of penis • Dorsally hooded foreskin (with deficiency of ventral foreskin) (Fig. 49.9)
• Chordee.

Question 18

What is epispadias?

Answer 18

Epispadias is defined as a urethral meatus that opens on the dorsal aspect of the penis. The opening can be as distal as the dorsal aspect of the glans and as proximal as the bladder neck (Fig. 49.10).

Mnemonic to differentiate hypospadias versus epispadias: The urinary stream is directed toward the heels in hypospadias and toward the eye in epispadias.

Question 19

What congenital anomalies are associated with epispadias?

Answer 19

• Diastasis of the symphysis pubis
• Bladder exstrophy
• Renal agenesis
• Ectopic/pelvic kidneys
• Vesicoureteral reflux.

Question 20

What is urethral duplication?

Answer 20

Urethral duplication is a congenital anomaly in which two urethras developed. There are multiple types of configurations (Fig. 49.11). The ventral urethra is usu- ally normal caliber and location, and the dorsal urethra is typically an accessory urethra that is stenotic/hypoplastic.

Question 21

What is aphallia?

Answer 21

Aphallia, or penile agenesis, is a rare congenital absence of the penis with an esti- mated incidence of 1 in 10 to 30 million live births. This is due to maldevelopment of the genital tubercle. The urethral meatus is often times located in the scrotum, perineum, or within the anal ridge. It is commonly associated with other genitouri- nary and anorectal abnormalities.

Question 22

What is diphallia?

Answer 22

Diphallia, penile duplication, is a rare congenital malformation with estimated incidence of 1 in 5 million live-births. Diphallia is classified into true diphallia or a bifid phallus. Each classification is further divided into complete or partial dupli- cation (Fig. 49.12).

Question 23

What is priapism? What is the most common cause?

Answer 23

Priapism is a prolonged erection lasting more than 4 hours. The most common cause of priapism in children is sickle cell disease. Additional etiologies include other hemoglobinopathies, leukemia, and trauma.

Question 24

What size Foley catheter should be used in a child?

Answer 24

Either weight or age are typically used to determine the appropriately sized Foley catheter to use in children. Table 49.1 demonstrates one method for determining catheter size. One formula to estimate catheter size in children is [5]:

Urinary catheter (FR) = (Weight(kg)/3) + 4

Question 25

Recommended catheter sizes for children?

Answer 25

Term newborn to 6 months (0-6kg)
- F6

6-9 months (5-9kg)
- F6-8

1-6 years (10-20kg)
- F8-F10

8-12 years (20-40kg)
- F10-12

14+ years (45kg)
- F12-14

Question 26

A 15-month old uncircumcised male patient is brought to the hospital by his parents with complaints of penile swelling and change of skin color in the affected area. On examination, he is afebrile, and the remainder of the systemic review is unremarkable. However, on the genitourinary examination, there is significant erythema, swelling, tenderness, and preputial purulent discharge. What is the first line management option for this patient?

A. Apply topical antibiotics like Mupirocin
B. Circumcision
C. Start oral antibiotics
D. Oral paracetamol and observe

Answer 26

C. Start oral antibiotics

Question 27

What is the prepuce?

Answer 27

The prepuce is the anatomic covering of the glans involving an outer and inner layer with attachments to both the shaft and corona.

Contributing to the debate concerning the appropriateness of routine circumcision is a poor understanding of its function.

The prepuce is specialized junctional mucocutaneous tissue that has both somatosensory and autonomic innervation.

Innervation of the prepuce differs from the glans, which is innervated by free nerve endings with protopathic sensitivity.

As a result of these differences, the inner mucosa of the prepuce is felt to be a part of penile erogenous tissue.

Given the possible relationship between the prepuce and sexual satisfaction, studies have looked into outcomes following circumcision.

Problems with sexual dysfunction (inability to ejaculate, lacking interest, premature ejaculation, pain during sex, not enjoying sex) appear to be slightly more prevalent among uncircumcised men.

For sexually active adult males undergoing circumcision, there does not appear to be any adverse, clinically important effects on sexual function or satisfaction.

Other studies, however, have shown difficulty with sexual enjoyment, erectile function, and a decrease in masturbatory pleasure following circumcision.

This is thought to be related to the keratinization of the glans and loss of specialized sensory tissue in the prepuce.

A recent systematic review comparing perceived sexual function in circumcised and noncircumcised males, before and after circumcision, showed no inferior sexual function after circumcision.

Additionally, using quantitative sensory protocols to assess touch and pain thresholds, penile sensitivity does not appear to differ across circumcision status.

These mixed results hold true for homosexual men as well.

The effects on female partners of adult males who are circumcised are similarly mixed. Some report dyspareunia, orgasm difficulties, and incomplete needs fulfillment, while others report no significant problems at all.

H&A

Question 28

What is the stand of the American Academy of Pediatrics on circumcision?

Answer 28

Circumcision (removal of the redundant prepuce) is one of the most frequently performed surgical procedures in the world.

There is a wide variability in the rate of circumcision among different populations.

A lack of consensus regarding the function of the foreskin and uncertainty regarding the benefits of circumcision has led to controversy regarding the appropriateness of elective circumcision.

The most recent policy statement from the American Academy of Pediatrics (AAP) states, “Although health benefits are not great enough to recommend routine circumcision for all male newborns, the benefits of circumcision are sufficient to justify access to this procedure for families choosing it.”

Circumcision has been practiced since ancient times. A common reason for elective circumcision centers on religious beliefs.

The Bible declares circumcision to be the sign of the covenant between God and the people of Israel.

In the Muslim faith, circumcision is recommended, but not obligatory.

Circumcision is common in the United States, areas of Africa, Australian aborigines, and portions of the Middle East. In contrast, routine circumcision is rarely performed in Europe, Asia, and Central and South America.

This variation in incidence likely reflects religious and cultural differences.

H&A

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the AAP’s current position is that the health benefits of routine circumcision outweigh the risks and that access to this procedure should be available for parents who choose it.

The rationale behind the current AAP position is the high-level evidence generated since the turn of the century indicating a significant decrease in the incidence of urinary tract infections and sexually transmitted diseases, particularly human immunodeficiency virus (HIV) infection, in circumcised males.

Interestingly, reviewing the same evidence, the Canadian Pediatric Society (CPS) has maintained its position that routine male circumcision is not indicated.

The CPS’s rationale is the reasonably high number needed to treat to realize each potential benefit of circumcision.

Furthermore, the CPS cites the low incidence of HIV infection in Western populations and the specific risk factors that allow targeting of specific populations for interventions as additional reasons not to recommend routine circumcision.

This controversy is likely to continue.

Sherif

Question 29

What are medical indications for circumcision?

Answer 29

The inability to retract the foreskin of a newborn is the result of incomplete keratinization of the glans and is not pathologic.

Phimosis is the inability to retract the foreskin.

True phimosis is associated with a white, scarred preputial orifice, most common just before puberty, and is an indication for circumcision.

Balanitis xerotica obliterans is a ring-like distal sclerosis of the prepuce with whitish discoloration or plaque formation that may involve the prepuce, glans, or urethral meatus, and is also an indication for circumcision.

Paraphimosis occurs when the foreskin has been retracted behind the corona and is unable to be brought back over the glans. This is also an indication for circumcision, though ardent opponents of circumcision would offer preputial stretching, plasty, or topical steroid creams as alternative therapy.

Balanitis is an infection of the glans, and posthitis is an infection of the prepuce.

Recurrent infection with scarring of the prepuce is also an indication for circumcision.

H&A

Question 30

Should circumcision be performed routinely at birth?

Answer 30

Circumcision is performed on the eighth day in the Jewish faith, traditionally by a mohel, a member of the faith trained in circumcision.

In Islamic countries, when performed, there is a wide variability in age at circumcision.

The appropriateness of routine circumcision of healthy newborn males is an emotional and contentious issue.

Underlying the argument is a poor understanding as to the function of the prepuce.

Opponents have presented circumcision as a symbol of the “therapeutic state,” a mutilating procedure, and have questioned the ethics and legality of newborn circumcision, especially with respect to human rights considerations and the notion of respect for bodily integrity.

Opponents have also cited an association between circumcision and subsequently developing poor breast-feeding outcomes, delayed cognitive abilities, and neonatal jaundice.

However, these concerns have not been substantiated in other studies.

One urologic study among children without urinary complaints found a correlation between circumcision and meatal stenosis. In this study, 20% of boys seen between 5 and 10 years of age who had undergone neonatal circumcision were found to have meatal stenosis.

Proponents for routine newborn circumcision generally cite three advantages:

1) prevention of urinary tract infections (UTIs),
2) prevention of sexually transmitted disease (STD), and
3) prevention of cancer, both penile and prostate.

Data regarding the effect of circumcision on the risk of UTIs is largely retrospective and from case-control studies.

A Cochrane Review in 2012 investigating the effect of routine neonatal circumcision for the prevention of UTIs in infancy showed there were no randomized controlled trials to date.

A systematic review involving 12 studies showed circumcision significantly reduces the risk of UTIs with a reduction in odds of 90%.

Given the low risk of UTIs in boys of approximately 1%, the number needed to circumcise to prevent one UTI is 111.

With a circumcision complication rate of 2%, a risk–benefit analysis did not favor routine circumcision for reducing the UTI risk alone.

In this study, the authors recommended circumcision in boys with recurrent UTIs.

A relatively recent prospective study found a reduced incidence of asymptomatic UTIs in circumcised boys.

Finally, in another, there was a 10-fold increase in the cost of managing UTIs in uncircumcised compared with circumcised infants.

An increased incidence of UTIs in uncircumcised males is believed to be secondary to adherence of pathogenic bacteria to the prepuce.

Proponents point to the 10% incidence of concurrent bacteremias and long-term sequelae of renal scarring after UTIs.

Critics have countered that these studies are retrospective analyses and that other studies have found genitourinary infection with circumcised children.

Additionally, they argue that colonization of the prepuce by nonmaternal uropathic bacteria could be prevented by strict rooming-in with the mother.

There are many studies examining the relationship between circumcision and STDs. The benefits of circumcision relative to STDs are a strong factor in the support of circumcision in the latest AAP statement.

Studies have shown that uncircumcised individuals have an increased risk of acquiring human immunodeficiency virus (HIV), human papillomavirus (HPV), and genital herpes.

However, other studies have found little support for or have refuted these findings altogether.

There is also evidence that circumcision is associated with a decreased risk of cervical cancer in women.

It is possible that the protective effect of circumcision against STDs may differ between developed and developing nations with poor hygiene.

Possible mechanisms for differing rates of STDs in relation to circumcision status include a more easily traumatized mucosa and epithelium of the uncircumcised phallus, the foreskin environment being more conducive to pathogens, or nonspecific balanitis in uncircumcised men predisposing to certain STDs.

Behavior and sexual practice still represent the greatest risk factors in STD transmission.

Epidemiologic studies of HIV and acquired immunodeficiency syndrome (AIDS) have raised another argument for prophylactic circumcision.

There is a substantial amount of evidence linking uncircumcised men with an increased risk of HIV infection that is independent of the increased risk of genital ulcers in uncircumcised men.

The inner surface of the foreskin contains Langerhans cells with HIV receptors. This explains the rational for circumcision to decrease HIV infection in men.

Three randomized controlled trials in Africa showed circumcision had an estimated relative risk reduction of 50–60% in contracting HIV. This was approximately a 1.8% absolute risk reduction. They concluded that for every 56 men who get circumcised, this will prevent 1 HIV infection.

Similar findings have been noted in the United States, with uncircumcised homosexual men having a twofold increase in the risk of HIV infection.

An uncircumcised male partner also appears to be associated with an increased risk of transmission of HIV to heterosexual contacts.

The most recent two Cochrane Reviews cite a protective association between circumcision and HIV acquisition, and recommend routine circumcision among heterosexual men while stopping short of recommending the same in homosexual men.

Circumcision may act as a protective measure against cancer, both prostate and invasive penile. It is believed that these cancers have infectious etiologies and that rationale underlies the reasoning that circumcision before first sexual intercourse may be associated with a decreased risk of prostate cancer.

The uncircumcised state has been strongly associated with invasive penile cancer in multiple case series, especially given its strong association with HPV.

The incidence of penile cancer in the United States is approximately 1 case per 100,000, with nearly all cases occurring in uncircumcised men.

The protective effect against penile cancer is diminished or lost when circumcision is performed after the newborn period.

Other factors associated with invasive penile cancer include smoking, a history of genital warts, penile rash or tears, multiple sexual partners, and poor penile hygiene.

Critics cite equally low rates of penile cancer in developed countries with low circumcision rates and feel that the incidence of penile cancer does not justify routine neonatal circumcision.

There is no definitive answer to the question of the appropriateness of routine newborn circumcision. Current studies do not provide conclusive evidence definitively for or against routine newborn circumcision.

Males not circumcised at birth have between a 2% and 10% likelihood of needing circumcision.

A longitudinal study comparing circumcised with uncircumcised males showed a higher risk of penile “problems” in infancy in the circumcised group. However, there was a higher rate of “problems” in the uncircumcised group after infancy. By 8 years, the uncircumcised group had experienced 50% more penile “problems.”

The previous circumcision policy statement from the AAP in 1999 acknowledged the potential medical benefits of newborn male circumcision, but did not recommend routine neonatal circumcision.

The 2012 statement states that the preventive health benefits outweigh the risks of the procedure, recommending access to the procedure but stopping short of recommending routine circumcision.

In the United States, the parental decision for newborn circumcision seems to be based more on social than medical reasons.

H&A

Question 31

What are the goals of circumcision?

Answer 31

Circumcision has been practiced for centuries.

Common to all methods, the goal is removal of an adequate amount of prepuce to uncover the glans, treat or prevent phimosis, and eliminate the possibility of paraphimosis.

Whichever method is chosen, the surgeon must be familiar with and adept at the technique with a resultant low complication rate.

Informed consent should always be obtained.

H&A

Question 32

What are techniques for circumcision in the newborn?

Answer 32

Circumcision is the most frequently performed male operation in the United States, with 64% of newborn male infants circumcised in a study from 1995.

Newborn circumcision is most frequently performed with a device, which may be a shield, used in traditional Jewish circumcision, a Mogen clamp, a Gomco clamp, or a Plastibell.

Prior to the procedure, the penis should be inspected for any contraindication to circumcision including a short or small phallus, hypospadias, chordee with no hypospadias, hooded prepuce, dorsal penile cutaneous hump, penile curvature or torsion, penoscrotal fusion, or large inguinal hernias or hydroceles that engulf the penis.

There is agreement for the need for adequate analgesia as studies have shown infants circumcised without analgesia have a stronger pain response to vaccination at 4 and 6 months of age compared with those receiving analgesics.

Another study, looking at pain in relation to timing, recommended circumcision before 8 days of age. Effective relief of circumcision pain has been found with acetaminophen, topical lidocaine–procaine cream, and local nerve blocks.

One study showed a subcutaneous ring block with 1% lidocaine without epinephrine to be the most effective pain relief.

Sucrose on a pacifier can also provide additional pain control.

Even though many circumcisions are performed outside the operating room, antisepsis is critical as infection is a serious potential complication.

In performing a Gomco circumcision, the field is sterilely prepped, and adhesions between the glans and inner surface of the prepuce are bluntly separated.

The extent of foreskin to be excised is marked with a pen or a crush of the dorsal prepuce with a straight clamp.

A dorsal slit allows the appropriate-sized bell to be placed over the glans, inside the prepuce.

The bell and foreskin are then brought through the opening in the clamp, placed in the yoke, and then tightened.

The excess foreskin distal to the base of the clamp is excised after waiting for several minutes.

Electrocautery should never be used because of transmission of the electrical current to the penis.

The bell is released and removed, taking care not to disrupt the weld between the shaft skin and the remnant of the inner surface of the prepuce.

A Plastibell allows strangulation of the distal foreskin, with a resulting slough of the tissue.

After sterile prep and dorsal slit, the appropriate-sized Plastibell is placed over the glans inside the prepuce.

A string is then tied around the prepuce and positioned over a groove in the bell.

The excess foreskin is trimmed, and the handle is broken off the bell.

The distal foreskin remnant and ring typically slough off in 7–14 days.

H&A

Question 33

What are indications for revision circumcision?

Answer 33

Following recovery from any of the foregoing procedures, there may be redundancy or asymmetry of residual preputial skin that may not meet the cosmetic expectations of the family.

In situations where the family feels the redundant, residual prepuce is unsightly, or with a postoperative phimosis, they may seek an opinion regarding revision.

As with primary circumcision, there is controversy surrounding the medical necessity and ethics of revision.

One study reported 46 revisions where the indications were primarily preputial redundancy, residual cicatrix, preputial-glandular bridges, a sebaceous cyst, and urethrocutaneous fistula.

Two other reported studies of circumcision revision both reported using the freehand or sleeve technique, with the most common indication being residual or redundant preputial skin.

H&A

Question 34

How is freehand circumcision performed?

Answer 34

In older patients, circumcision is usually performed in the operating room and devices are less often used.

As stated previously, in cases of circumcision revision, the freehand technique is the preferred method.

After prepping the field, any remaining adhesions between the glans and foreskin are bluntly lysed.

After marking the subcoronal sulcus, the foreskin is incised along the base of the glans with the foreskin in its normal position.

Less skin is excised from the ventral surface.

Dissection is carried down to Buck’s fascia.

The prepuce is then retracted and an incision made in the subcoronal sulcus, leaving a generous cuff of subcoronal skin.

Injury to the urethra must be avoided ventrally.

The collar of foreskin that is isolated is then excised, and electrocautery is used to obtain hemostasis.

The shaft skin is approximated to the subcoronal skin using absorbable sutures.

A recent prospective randomized trial compared the use of 2-octyl cyanoacrylate (glue) skin adhesive to hydrophobic ointment at the time of circumcision for the prevention of postoperative penile adhesions.

Patients <7 years of age undergoing circumcision were randomized to the glue around the sutures and the corona of the penis or antibiotic ointment.

The primary outcome variable was postoperative penile adhesions.

Over a 3.5-year period, 409 patients were enrolled in this study. Adhesion data was available on 243.

There was no difference between glue (16.8%) and those with antibiotic ointment (15.2%; P = 0.88), or in parental satisfaction among all areas measured.

The 165 patients who were lost to follow-up were evenly distributed between the two groups.

H&A

Question 35

What are complications of circumcision?

Answer 35

When performed by experienced hands under sterile conditions, circumcision has a low complication rate between 2% and 10%.

Bleeding and infection are the most frequent complications and are generally minor.

Although adhesions between the foreskin remnant and the glans are common, most will resolve with time.

However, serious complications can develop, including necrotizing fasciitis, sepsis, Fournier’s gangrene, and meningitis.

Other complications include skin bridges, inclusion cysts, iatrogenic hypospadias or epispadias, partial glans amputation, and catastrophic loss of the penis when electrocautery is used with a metal device.

There can be excision of too much or too little of the foreskin, with resultant postoperative phimosis or concealed penis.

As discussed previously, revision may sometimes be necessary later in childhood.

H&A

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Most complications of circumcision are mild and related to postoperative bleeding. It is therefore imperative to confirm excellent hemostasis before leaving the operating room.

Bleeding in the recovery room can usually be controlled with pressure.

Bismuth subgallate paste is a topical hemostatic agent that is also quite effective in controlling bleeding from a circumcision site.

Meatal stenosis is estimated to occur in approximately 10% of circumcised boys due to irritation from the urine ammonia contents.

A simple meatotomy under general anesthesia is usually curative.

A more serious complication of circumcision is trapped penis. This risk is especially significant in obese children who have a significant amount of prepubic fat and a short phallus.

A free-hand revision circumcision is necessary. The scarred ring of foreskin is removed, and the skin is degloved from Buck’s fascia all the way to the base of the penis. Two or three anchoring stitches are then placed between the dermis and Buck’s fascia at the base of the penis to avoid recurrent migration of the skin.

An example of this complication and its treatment in a 10-year-old boy is shown in Figure 50.8.

Buried penis may be seen in patients with large hydroceles and inguinal hernias, as in a neonate with large bilateral inguinal hernias.

Observation until the hydroceles resolve or repair of the inguinal hernias constitutes the appropriate treatment of this condition.

Sherif

Question 36

A 7-year-old boy of East Asian origin is being seen in the pediatric surgery clinic the day after he presented to the emergency department with dysuria and difficulty urinating.

The boy had a known history of phimosis and had been treated with three courses of 0.05% betamethasone cream over 6 months.

He was able to void in the emergency department and the bladder was nonpalpable post voiding.

A urinalysis showed 3–5 red blood cells per high-power field, but was otherwise normal.

The emergency department physician discharged the patient and arranged for follow-up the following day.

In the clinic, the child appears uncomfortable but is not in any acute distress. The abdominal exam is normal and there are no palpable masses. The scrotal exam is normal with bilateral descended testicles. The penile appearance is shown in Figure 50.1.

What is your diagnosis?

[Sherif]

Answer 36

The urethral meatus is poorly visualized. The prepuce is nonretractable and its distal portion demonstrates a white sclerotic ring. The glans cannot be separated from the prepuce.

This patient has classic findings of lichen sclerosis et atrophicus, which is called balanitis xerotica obliterans (BXO) when it involves the glans, prepuce, or external urethral meatus.

BXO is only found in uncircumcised boys. The exact etiology is not known, and there may be some genetic factors that predispose to it. Links to infectious causes, such as human papilloma virus and B. burgdorferi, have never been proved. It is considered a chronic progressive dermatitis.

In addition to urinary retention, difficulty voiding, and dysuria, BXO may also precipitate recurrent urinary tract infections. In addition, severe foreskin narrowing can result in a ballooning phenomenon during voiding. The urine collects in the space between the urethral meatus and the foreskin and then starts to dribble out once a certain volume threshold is reached.

Circumcision is the appropriate treatment in this patient who already failed several courses of topical steroids.

A free-hand circumcision should be performed under general anesthesia. The preputial scarring and adhesions should be carefully freed from the glans until the corona is visualized circumferentially.

Circumferential incisions should then be made in the foreskin proximally and distally, leaving more skin on the ventral surface of the penis.

The cuff of foreskin is then removed with sharp dissection and bipolar cautery, followed by approximation of the margins of foreskin and mucosa (nonkeratinized skin) with a fine fast-absorbing suture.

Sherif

Question 37

What are the histologic features of balanitis xerotica obliterans?

Answer 37

The lesion is characterized by an atrophic epithelium frequently intermixed with hyperplastic areas, vacuolar alteration of the basal layer (A), and a thickened lamina propria with the classic hyalinization/sclerosis (B).

A variable amount of band-like lymphoid (lichenoid) infiltrate is seen beneath the area of hyalinization (C).

Because of marked basal cell vacuolar alteration, severe cases also show dermal-epidermal cleftings (D).

Sherif

Question 38

Are antibiotics needed for balanitis xerotica obliterans post-circumcision?

Answer 38

No. The risk of infection post circumcision, even in difficult cases of BXO, is extremely low.

A local antibiotic ointment is prescribed for 1 week after surgery, and the patient is started on warm Sitz baths the day after surgery.

Question 39

What complications can be anticipated for balanitis xerotica obliterans post-circumcision?

Answer 39

Severe BXO can also involve the urethral meatus and urethra, resulting in stricture of either structure or both.

The absence of a significant stricture, as evidenced by Foley catheter probing at the time of surgery is reassuring.

However, meatal stenosis can develop in up to one-third of patients with BXO following circumcision.

The patient should therefore be followed for several months to confirm that voiding problems have resolved and that there is no evidence of meatal stenosis.

Sherif

Question 40

Which percentage of boys not circumcised at birth will require circumcision for preputial pathology, most commonly phimosis?

Answer 40

Approximately 2%–10% of boys who are not circumcised at birth will require circumcision for preputial pathology, most commonly phimosis.

The diagnosis of phimosis is also somewhat controversial. In order to appropriately define phimosis, the surgeon must understand the normal anatomy and evolution of the prepuce.

The prepuce is the anatomical covering of the glans. The inner mucosa of the prepuce is considered part of the penile erogenous tissue.

The prepuce is not retractable in the majority of neonates.

Smegma, or sloughed epithelial debris, starts to form between the foreskin and glans and helps induce the separation of the foreskin.

Approximately 50% of boys will have retractable foreskin by age 6 years and 95% by the beginning of puberty.

The prepuce is also sensitive to androgens, which may help achieve a retractable foreskin with increasing time and androgen levels.

A nonretractile foreskin by a specific age is often referred to as phimosis.

However, phimosis should only be diagnosed when the nonretractile foreskin is responsible for symptoms. These include difficulty voiding, urinary tract infections without other predisposing lesions, ballooning of the foreskin during voiding, paraphimosis, or infections of the glans or prepuce.

The most severe form of phimosis is BXO, as described in the index case.

Sherif

Question 41

For how long should medical treatment be attempted in phimosis?

Answer 41

Phimosis presenting outside the emergency setting can be treated medically or surgically.

Medical treatment involves application of steroid cream once or twice per day for four to eight weeks.

Success rates up to 90% have been reported.

This has also been proposed for cases of BXO, although it is unclear how this diagnosis can be confirmed without a surgical specimen from a circumcision.

In my experience, the success rate of steroid treatment, even with multiple courses, is much lower.

Moreover, phimosis is a spectrum that varies from redundant foreskin with a pinhole opening to a wide prepuce with adhesions to the glans or corona, as shown in Figure 50.5.

It is this latter type that seems most amenable to steroid application, as the adhesions will often release with steroids.

Adhesions can also be released in the clinic by applying a topical anesthetic to the area and using a Q-tip to release the foreskin from the corona.

Symptomatic boys who do not respond to one or more courses of steroids should be circumcised.

The end result of a long-standing untreated phimosis may be BXO, as described in the index case presented.

BXO is also a spectrum and histologic changes characteristic of BXO have been reported in up to onethird of circumcision specimens, even when not diagnosed preoperatively.

Sherif

Question 42

A 12-year-old-boy is brought to the office with a red penile lesion on his foreskin. He is uncircumcised on a physical exam.
Viral PCR is negative for HPV 6, 11, 16, and 31. He denies pruritus and dysuria. The patient denies any additional bothersome symptoms. The father, present upon examination, states that his son is not sexually ac-tive. Which of the following is the most appropriate management strategy for this patient?
Choices:
1. Complete excision
2. Topical 5-fluororacil
3. Topical Imiquimod
4. Surveillance

Answer 42

Answer: 4 - Surveillance
Explanations:
• The treatment for lichen sclerosus, or BXO, in an asymptomatic patient is no therapy with surveillance.
• With symptoms, topical steroids could be utilized to relieve itching and burning.
• Avoid excision in this condition as recurrence is high. Yearly follow-up is required because of the risk of malignancy.
•Complete excision is the treatment of choice is Buschke-Lowenstein Tumor, which can be premalignant and locally invasive. HPV has been found in these tumors. Tends to recur with inadequate excision. Topical 5-fluorouracil is the treatment for Bowenoid papu-losis, which is generally considered benign but has premalignant potential. A topical treatment like 5-FU can be utilized for this con-dition. If carcinoma in situ is suspected, HPV will likely be positive.
Treatment options include topical therapy with 5-FU or Imiqui-mod, wide local excision, or laser ablation.

StatPearls

Question 43

A two-month-old male is receiving inpatient care for a severe urinary tract infection. During a counseling session, his parents are informed about the benefits of surgical removal of the foreskin.
Circumcision would be most beneficial for which of the following anatomical abnormalities?
Choices:
1. Posterior urethral valves
2. Hypospadias
3. Epispadias
4. Vesicoureteral reflux

Answer 43

Answer: 1 - Posterior urethral valves
Explanations:
• Uncircumcised children with anatomic urinary tract abnormalities (posterior urethral valves, vesicoureteral reflux, etc.) are prone to recurrent UTIs and even renal damage due to periurethral flora.
• For male patients with posterior urethral valves, circumcision significantly reduces the incidence of urinary tract infections (UTIs).
• The benefit of circumcision in reducing urinary tract infections is greater in children with significant urological diseases than in healthy males.
• Infants born with genital abnormalities like hypospadias, chordee, and webbed or buried penis, will likely require the foreskin during surgical repair. These patients are not candidates for routine circumcision.

StatPearls

Question 44

Which of the following is the commonest reason of circumcision in the world?

A. Religious reasons.

B. Social reasons.

C. Urinary tract infection.

D. Phimosis.

E. Para-phimosis.

Answer 44

A

In the world, the commonest reason for circumcision is religious. All Muslims and Jews gets circumcised. Some part of world, especially Africa, circumcision is also done for social and cultural reasons. In the developed world, one of the common reasons is phimosis.

Syed/MCQ

Question 45

The following are complications of circumcisions except:

A. Haemorrhage.

B. Dilated meatus.

C. Sepsis.

D. Fistula formation.

E. Excision of too much or too little skin.

Answer 45

B

Meatal stenosis is one of the complication, not the dilatation.

Syed/MCQ

Question 46

Which of the following is true regarding circumcision in children?

A The incidence of circumcision in the United States has gone up since 1960.

B Circumcision is uncommon in Muslims, Jews and some tribes in Africa.

C It is believed that 6%–7% children in the UK are circumcised by the time they are 15 years old.

D Europeans have the highest incidence of circumcision in the world.

E Among Europeans, Sweden has the highest annual rate of circumcision.

Answer 46

C

Circumcision is the most commonly performed operation in males.

It was initially performed for religious, ritual or cultural reasons and did not become ‘medicalised’ until the nineteenth century.

Ritual circumcision is practised in Jews, muslims, Aboriginals and certain tribes in Africa.

The prevalence of routine neonatal circumcision in the united States has dropped from close to 90% in the mid-1960s to an estimated 64% in 1995.

The American Academy of Pediatrics task force report on circumcision (1999) supports a growing trend away from neonatal circumcision.

This task force report acknowledges potential medical benefits to neonatal circumcision; however, it concludes that routine circumcision in neonates is not necessary.

Currently in England, approximately 21,000 childhood circumcisions are performed annually for medical reasons (compared with 1.2 million in the united States).

It is estimated that 6%–7% of boys are circumcised before their 15th birthday, which is significantly less than the 24% reported in the 1950s.

In Scandinavian countries, particularly Sweden, the circumcision rate is the lowest among Western cultures.

SPSE 1

Question 47

Regarding the development and retraction of prepuce:

A the developing prepuce cannot be identified until late in gestation

B preputial development is independent of glans development

C the foreskin is retractile in 80% of the boys at birth

D nearly 10% boys can retract their foreskin by 3 years of age

E the prepuce grows over developing glans more quickly on the ventral aspect than on the dorsum side.

Answer 47

C? D?

By 8 weeks of gestation, there is a thickened ridge of epidermis proximal to the glans, which is prepuce that grows forwards over the developing glans penis more quickly on the dorsum than on the ventral side.

The circumferential full development is dependent upon final formation of the glanular urethra.

The epithelium on the inner surface of the prepuce fuses with the developing glans and full separation takes place only later in life.

Four percent of foreskins are retractile at birth and 10% of boys can retract their foreskin by the age of 3 years.

Spontaneous separation of the prepuce from the glans penis usually proceeds proximally due to desquamation of cells at a variable rate and only 1% of the foreskin remains non-retractile at 16 years of age.

SPSE 1

Question 48

Which of the following is not true for balanitis xerotica obliterans (BXO)?

A Pathological phimosis due to BXO is uncommon before 5 years of age.

B BXO is a contraindication for circumcision.

C The aetiology of BXO is not known.

D BXO can occur simultaneously to glans and meatus.

E Histologically BXO is similar to vulval lichen sclerosis.

Answer 48

B

When the foreskin is non-retractile, it may be long and during attempted retraction it may exhibit ‘flowering’, and there may be blanching of the preputial skin proximal to the preputial orifice. It is called ‘physiological phimosis’.

When the preputial orifice itself is abnormal and scarred, it is known as pathological phimosis.

The scarring is called BXO. The incidence peaks in early adolescence and is approximately 1.5% at the age of 17 years. It is rare before the age of 5 years.

SPSE 1

Question 49

Regarding indications for circumcision, which of the following is true?

A Recurrent balanoposthitis and paraphimosis are not the indications for circumcision.

B Circumcision can provide protection against diseases, e.g. penile cancer, sexually transmitted diseases.

C AIDS is less common in uncircumcised males as the AIDS virus attaches to Langerhans’s cells, which are deficient in foreskin.

D Circumcision is indicated in boys with hypospadias/severe chordee.

E Circumcision is indicated in buried penis or penoscrotal web.

Answer 49

B

medical indications for circumcisions are few and can be divided into absolute and relative.

The absolute indication for circumcision is phimosis secondary to BXO, which is identical to vulval lichen sclerosis et atrophicus. The aetiology of BXO is unknown but it has some resemblance to autoimmune collagenosis.

BXO may simultaneously affect the glans penis and urethral meatus much more commonly in adult men than boys.

Meatal disease (BXO) can occasionally occur de novo after a circumcision; however, it has been suggested, with no proof, that BXO never follows neonatal circumcision.

Relative indications include balanoposthitis (erythema and oedema of the prepuce, purulent discharge from the preputial orifice, dysuria in older children, with 20% incidence of recurrence) and paraphimosis (tight preputial ring proximal to the coronal sulcus resulting in oedema and swelling of the distal penis).

Prevention of penile and cervical cancer, prevention of sexually transmitted disease (particularly HIV/AIDS), and prevention of urinary tract infection (UTI) are also relative indications.

Circumcision has been found to be directly related to reducing the risk of HIV acquisition by reducing the ability of the virus to attach to and enter the langerhans’s cells. (The inner layer of the foreskin contains a high density of these cells, a target cell for HIV infection.)

As the foreskin is more susceptible to trauma, this may predispose to HIV infection during sexual activity.

A systematic review summarising studies from sub-Saharan Africa, has shown an adjusted relative risk of HIV acquisition of 0.42 (95% confidence interval) in circumcised, compared with uncircumcised male subjects.

Tears, zip injury or pressure injuries usually heal well with some scarring and in such cases a circumcision is necessary only when the foreskin becomes scarred and non-retractile.

Circumcision is contraindicated in hypospadias, chordee, penoscrotal fusion, buried penis, micropenis, bleeding disorders, and megameatus intact prepuce variant of hypospadias.

SPSE 1

Question 50

Which of the following statement is true regarding the protective effect of circumcision on UTI?

A Circumcision does not provide protection against UTI.

B Circumcised infant boys are 3–10 times less likely to get a UTI than uncircumcised infants.

C Neonatal circumcision offers no reduction of renal scarring in children with vesicoureteric reflux.

D Circumcision has no role in reducing UTI in children with posterior urethral valves or neuropathic bladder.

E UTI in male infants is less common in uncircumcised boys.

Answer 50

B

Circumcision reduces the risk of UTI.

The risk of UTI in normal boys is approximately 1%.

Almost 20 years ago Wisewell et al. found that uncircumcised infant boys were 3–10 times more likely to develop UTI than circumcised male infants, secondary to an increased rate of periurethral and inner preputial skin colonisation with bacteria.

This association has also been shown in a report published in Sweden (Jakobsson et al., 1999), which showed a preponderance of UTI in male infants where newborn circumcision is unusual.

In boys with high-grade vesicoureteric reflux (VUR) the risk of UTI recurrence in uncircumcised and uncircumcised boys is 10% and 30%, respectively.

It is a common practice by urologists to offer a circumcision to a boy with recurrent UTI, or a boy who develops a UTI despite conservative treatment in the presence of serious underlying urinary tract abnormality including VUR, posterior urethral valves, neuropathic bladder and so forth.

It is also interesting to note that a controlled trial by Kwak et al. could find no benefit for circumcision when it was done at the same time as antireflux surgery for severe VUR, irrespective of age. one randomised controlled study showed (Nayir, 2001) significant reduction of renal scarring on DmSA scan following neonatal circumcision.

SPSE 1

Question 51

Regarding complications following circumcision, which of the following is true?

A Bleeding following circumcision is an uncommon complication.

B Necrotising fasciitis, Fournier’s gangrene are common following circumcision.

C Meatal ulceration can be found in 20%–30% of infants following circumcision and can cause meatal stenosis.

D There is a high incidence of amputation of glans during circumcision

E Penile lymphoedema is commonly seen following circumcision.

Answer 51

C

The risk of complication following circumcision is 0.2%–5%.

The most common complication following circumcision is bleeding (occurring in 0.1%), which is usually self-limiting, but some studies have suggested that haemorrhage requiring readmission and operation varies from 1.4% to 20%.

Infection usually responds to local care and rarely requires antibiotics; however, such infections can occasionally cause necrotising fasciitis, Fournier’s gangrene and tetanus.

Such severe sepsis is quite rare following circumcision but when it occurs it usually results in death.

Other complications include recurrent phimosis or buried penis, which may require a re-circumcision when too much skin is left behind.

macarthur reported 1% revision of skin complications following freehand circumcision.

Excessive loss of skin can either be due to local infection or secondary to diathermy injury.

In smaller children these usually heal well while in older children it may require skin grafting.

other complications include glanular adhesions (adhesions of circumcision scar to the traumatised ulcerated glans), epidermal inclusion cysts, and penile lymphoedema – rarely seen and of unknown aetiology.

Amputation of part or all of the glans is rare but can happen if it is caught within the jaws of the circumcision clamp.

Pressure necrosis of the glans is also rare but is usually seen with Plastibell circumcision especially when the bell of the Plastibell is too small.

Injury to the urethra can be caused by overzealous haemostasis with diathermy on the ventral surface of the penis in the region of frenular artery.

This can also be secondary to circumcision performed with a Gomco or Plastibell clamp. These techniques can cause subcoronal fistula, which requires a secondary repair.

meatal ulceration has been documented in 20%–30% of infants within 2–3 weeks following circumcision. This is thought to be due to chemical and physical irritation within the wet nappy.

The incidence is lower (4%) when the circumcision is performed in older boys.

The meatal ulcer will usually heal without sequel but chronic irritation or scarring can lead to meatal stenosis, which is reported in 2%–12% of patients following circumcision.

The other proposed theory for meatal stenosis is relative ischaemia to the meatus following division of the frenular artery.

Berry Croft studied the urethral meatus in children and adults and found that 60% of circumcised men had a meatus of 20Fr or less compared with only 25% of uncircumcised men.

Rarely the bladder and upper tracts are secondarily affected by meatal stenosis causing bladder hypertrophy and upper tract dilatation.

SPSE 1

Question 52

Which of the following is true regarding concealed penis?

A Concealed penis is because of congenitally short penis.

B Concealed penis is due to inadequate attachment of the dartos layer and Buck’s fascia.

C Concealed penis is a self-limiting condition and gets better as the child gets older.

D Concealed penis is secondary to childhood obesity.

E Balanitis, psychological stress and poor self-esteem are contraindications for surgical correction.

Answer 52

B

Concealed or buried penis appears small; it is secondary to inadequate attachment of the dartos or spermatic fascia to the deeper Buck’s fascia.

The diameter and stretched penile length are normal.

Earlier thinking was that this condition was due to childhood obesity (prominent prepubic fat) or inadequate or overzealous circumcision.

It was believed that it would correct itself over time with growth; however, experience has suggested that this retrussive penile appearance cannot and does not correct itself.

Balanitis, urinary tract infections, skin adhesions, deflected urinary stream, psychological stress about the appearance of the penis, and boys who have poor self-esteem and are socially withdrawn are indications for correction.

Various surgical techniques have been described to correct this penile abnormality.

They include attaching dartos layer to Buck’s fascia after degloving the penis.

Dorsally Scarpa’s fascia superficial to the pubis is pulled down and attached to the Buck’s fascia with non-absorbable sutures, safeguarding dorsal penile nerves.

other techniques described are prepubic liposuction and lipectomy with Z-plasty for penoscrotal webbing to correct concealment.

SPSE 1

Question 53

Regarding micropenis, which of the following is true?

A Stretched penile length less than 2 cm in newborns is normal.

B Micropenis may be secondary to panhypopituitarism or due to syndromes such as Klinefelter’s or Prader–Willi’s syndrome.

C Micropenis can not be associated with testicular dysgenesis or 5α-reductase deficiency.

D Evaluation of testicular function is not necessary in the management of micropenis.

E Gender reassignment should be offered to those who respond to androgen stimulation.

Answer 53

B

Micropenis is defined as a normally formed penis with a stretched penile length >2–2.5 standard deviations below the mean length for age.

In a newborn, stretched penile length less than 2 cm is considered abnormal.

It may be associated with undescended testes.

Aetiology of micropenis includes idiopathic, gonadotropin deficiency with/ without pan hypopituitarism deficiency of growth hormone, testicular dysgenesis, 5α-reductase deficiency and partial androgen insensitivity syndrome.

It may be present in conditions such as Klinefelter’s syndrome, laurence–moon–Biedl’s syndrome, Prader–Willi’s syndrome and Robinow’s syndrome.

Evaluation of these children includes karyotyping, and measurement of serum glucose, electrolytes, cortisol, growth hormone and thyroid function tests.

Testicular dysfunction as a cause of micropenis should be ruled out by measuring serum luteinising hormone, follicle-stimulating hormone, and measurement of testosterone before and after human chorionic gonadotropin stimulation (hCG) – 500–1500 IU of hCG is given alternate days for 5–7 days and testosterone is measured 24–48 hours after the last dose.

Androgen stimulation therapy is the treatment of choice.

Testosterone 25mg is given by intramuscular injection every month for several months in infants to assess the penile growth.

Palpable testes and significant response to hCG stimulation test suggest there will be long-term penile growth.

It has been shown that in peripubertal boys in whom testosterone therapy has failed, dihydrotestosterone cream can be an effective alternative.

Gender reassignment was advised in the past with failed response to testosterone but this has been questioned in genetic males with functioning testes.

Adequate sexual function and clear male identity has been reported in some patients raised as males with persistent small penis; therefore, reassignment of gender should be performed with caution and should be accompanied by expert, patient and family counselling.

SPSE 1

Question 54

Regarding priapism, which of the following statements is true?

A Priapism is an involuntary, prolonged and painful erection not resulting from sexual desire.

B Priapism is uncommon in children with sickle-cell disease.

C Tricorporeal priapism does not involve tumescence of the spongiosum.

D Priapism may not be secondary to perineal trauma or haematological diseases.

E Ischaemic (veno- locclusive) priapism is secondary to perineal trauma.

Answer 54

A

The term is derived from the Greek god Priapus, son of Aphrodite. He became famous for his giant phallus.

Priapism is defined as an involuntary, prolonged, painful erection that does not result from sexual desire.

It has been classified into primary or idiopathic, and secondary.

Haemodynamically, it can be classified as veno-occlusive (ischaemic) and arterial (non- ischaemic).

Non-ischaemic priapism is secondary to trauma and rarely is seen in sickle-cell disease.

The ischaemic (low-flow) variety of priapism is commonly seen in children with sickle-cell disease.

The reported incidence of priapism in sickle-cell disease varies from 2% to 35%.

Stuttering episodes of priapism in patients with sickle-cell disease are common and usually these episodes last for less than 24 hours.

Commonly tumescence involves only the corpora cavernosa but occasionally it may also involve the corpus spongiosum and is then known as tricorporeal priapism.

There is a long list of causes of priapism.

Although demographically the incidence of priapism varies, nearly one-third of cases are idiopathic, 21% are caused by alcohol abuse or drug therapy, 12% following perineal trauma and 11% are due to sickle-cell disease.

The secondary causes for priapism include haematological disorders (sickle-cell disease), drugs (anticoagulant, antihypertensive, drugs acting on central nervous system and so forth), metabolic disorders (amyloidosis, gout, diabetes, nephrotic syndrome and so forth), trauma, malignancy (leukaemia) and Kawasaki’s disease.

It has been suggested that ischaemic priapism is due to failure of the detumescence mechanism from causes including excessive release of neurotransmitters, blockage of draining venules and prolonged relaxation of the intracavernous smooth muscle.

In sickle-cell disease, intravascular thrombosis and haemolysis is caused by hypoxia. This in turn causes release of haemoglobin and l-arginase in the extracellular space.

The free arginase consumes l-arginine, which is a substrate for the endothelial synthesis of nitric oxide.

Nitric oxide is consumed in the oxidation of haemoglobin to methaemoglobin and in the neutralisation of heme and ferrous ions.

This process leads to deficiency of nitric oxide along with release and activation of inflammatory and thrombogenic factors and a tendency to vasoconstriction.

Nitric oxide is the main physiologic mediator of the detumescence mechanism; hence its deficiency in sickle-cell disease may cause priapism.

SPSE 1

Question 55

Which of the following statements is true regarding priapism?

A Arterial priapism is due to excessive release of neurotransmitters.

B Ischaemic priapism is due to intracavernosal thrombosis and haemolysis due to hypoxia.

C Low-flow priapism is characterised by alkalosis, hypocarbia on penile blood.

D Surgical treatment is never required for priapism.

E Hydration and blood transfusion to decrease haemoglobin S concentration below 30% is not indicated for the treatment of priapism due to sickle-cell disease.

Answer 55

B

Electron microscopic studies have shown that interstitial oedema with destruction of sinusoidal endothelium causing exposure of the basement membrane, occurs with 12 hours of priapism.

Thrombocytes then adhere to basement membrane within 24 hours.

By 48 hours, thrombi have been shown in the sinusoidal spaces along with some degree of smooth muscle cell necrosis.

It is an interesting fact that even after many days of low-flow priapism, there is a lack of thrombosis of penile blood. This is probably due to ×3 increase in activity of fibrinolysis locally in the cavernosal blood compared with peripheral blood.

In low-flow priapism, penile blood gas analysis is characterised by acidosis, hypercarbia and hypoxaemia.

The usefulness and reliability of penile nuclear scans and Doppler studies in children remains unclear.

Treatment of low-flow priapism lasting for more than 4 hours in sickle-cell disease includes intracavernosal aspiration of blood and irrigation with sympathomimetic drugs.

This treatment should be carried out concurrently with hydration and blood transfusion to achieve the haemoglobin S concentration of less than 30%.

Exchange transfusion has been associated with acute neurologic events termed ASPEN syndrome (Association of Sickle cell disease, Priapism, Exchange transfusion and Neurologic events).

The morbidity of ASPEN can be reduced by gradual or partial exchange transfusion, close monitoring and recognising the early prodromal symptoms such as headache.

Surgical intervention is required when medical and intracavernosal therapies fail.

For low-flow priapism, distal shunt procedures are recommended. These include Ebbehoj’s, Winter’s and Al Ghorab’s procedures.

Ebbehoj’s shunt is the simplest, between glans spongiosum and the corpora cavernosa.

Winter described a similar technique using a Trucut biopsy needle instead of the knife, obtaining a core from the tunica albuginea of the corpora cavernosa.

Al Ghorab’s procedure is a modification of Winter’s procedure. In this technique the glans is incised on the dorsum at corona level to expose bulging cavernosal bodies. A 5 × 5 mm segment of tunica albuginea is excised from the tip to create a cavernous–glanular shunt. The glans incision is then closed.

In long-standing priapism, distal shunts do not work very well and more proximal shunts such as Quackles’s (cavernospongiosum) and Grayhack’s (cavernosaphenous) shunts should be created.

In arterial priapism (non-ischaemic), which is secondary to perineal trauma, the tumescence is compressible because of open venous channels. Pain in this condition is less, as it is not ischaemic.

For high-flow priapism, initial treatment involves ice packs and site-specific compression. If this fails then selective arterial embolisation of the ruptured artery is indicated.

SPSE 1

Question 56

Which of the following statements is true about penile torsion?

A Penile torsion can be associated with functional abnormality.

B Penile torsion is common in the general population.

C Penile torsion is almost always counterclockwise and can be associated with hypospadias and chordee.

D Surgical correction of penile torsion is not advised even when it is more than 60–90 degrees.

E Deviation of ventral penile raphae is always associated with penile torsion.

Answer 56

C

Penile torsion is a congenital rotational abnormality of the penis. It is almost always in counterclockwise direction.

It is often an isolated abnormality or can be associated with hypospadias or chordee.

The true incidence in normal males is 1.5% and with torsion of more than 90 degrees has been seen in 0.7%.

Deviation of ventral midline raphe was noted in 10% of newborns without any rotational deformity.

There is no functional abnormality associated with this condition and correction is indicated if the deformity is 60–90 degrees or more or the parents want it corrected.

There are various techniques described to correct the anticlockwise rotation of penis including, penile skin degloving and realignment of penile skin, dorsal dartos flap counter-rotation, suturing penile tunica albuginea to pubic periosteum, and by removing angular ellipses of corporal tissue and plication.

SPSE 1