blind ending gonadal vessels. If a blind-ending vas is seen but not the vessels, one must continue to

explore for hidden testicular tissue since there is an unusual phenomenon of testicular–epididymal

dysjunction, where the testicle is disconnected from the wolffian structures (epididymis and vas). One

may have to look very far to locate the vessels, even going up behind the colon and near the lower pole

of the kidney. Leaving a testicular tissue trapped within the abdominal cavity will increase the risk of

germ cell malignancy. There are currently no reliable imaging studies that can obviate the need for

surgical exploration, and diagnostic laparoscopy remains the gold standard. Ultrasound is not

recommended. Although it is noninvasive, it suffers from the negative test result prediction concern.

Most importantly, the surgical decision and planning are not altered by ultrasound findings. For

bilateral nonpalpable testicles, biochemical markers – such as HCG stimulation test or serum MIS level –

may be useful to determine the presence or absence of functional testicular tissue.

Figure 104-15. Intraabdominal testicle is gently clipped to force its dependence on the vassal collateral circulation (first-stage

Fowler–Stephens orchidopexy).

Figure 104-16. During the second-stage orchidopexy, the testicle is dissected while being attached to the vassal collateral

circulation.

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Figure 104-17. Left intravaginal testicular torsion. The nontorsed contralateral testicle is also explored and secured to prevent

future torsion.

Torsion of Testicle and Appendages

Torsion of the testicle is the leading nontraumatic cause of loss of the testicle in boys (Fig. 104-17).17 It

is an emergent condition that occurs as result of the spermatic cord twisting. This results in occlusion of

the lymphatic vessels, veins and arteries leading to ischemia, necrosis, and atrophy. If there is complete

occlusion of blood flow, the testicle may be salvageable up to 6 hours after onset. After 6 hours the

odds of salvage diminish exponentially. Most torsion occurs in older boys and adolescents – although it

can occur at any age – with the torsion occurring inside the tunica vaginalis (intravaginal torsion). A

horizontal lie (known as the bell-clapper deformity) caused by poor posterior fixation is believed to

predispose toward torsion. Torsion can also occur among neonates, but this occurs due to poor

gubernacular fixation and twisting of the cord outside the tunica vaginalis (extravaginal torsion).

Neonatal torsion classically is associated with a prolonged labor. The testicle is very firm, and there can

be a dark ecchymotic look about the scrotum. Successful salvage is very rare in perinatal torsion, and

exploration is controversial due to potential anesthesia concerns. Because of the very low salvage rate,

some do not advocate an emergent exploration, while others point out that if it is anesthetically safe,

exploration is important because it establishes definitively not only the state of the suspected torsed

testicle but allows fixation of the contralateral testicle. Metachronous torsion can occur and is difficult

to monitor clinically. Exploration and preemptive fixation can prevent loss of the contralateral testicle.

The presentation is a sudden onset acute unilateral scrotal pain. The pain can be intense and lead to

nausea and vomiting. With time, there will be increasing swelling and discoloration. The signs and

symptoms will evolve over time. Initially there can be little swelling or redness. Later when the nerve

endings have died out, the testicle and scrotum may still be swollen and ecchymotic but less painful.

Various clinical clues may help guide physical examination. These include a noticeably shortened

spermatic cord, absence of a cremasteric reflex, and lack of relief of discomfort with elevation, but

these are often of limited practical use when faced with an anxious patient with a tender scrotum. If one

has a strong clinical suspicion based on presentation, and there are no other medical or anesthetic

reasons not to go to surgery, emergent exploration – without any further imaging – is appropriate.

Doppler ultrasound – now readily available in most modern emergency departments – can be useful but

should not delay exploration. Ultrasound can help identify other cases of acute scrotum such as torsed

appendix testis, epididymitis, hernia/hydrocele, and idiopathic scrotal edema. Exploration is done

through a transverse scrotal incision. If the testicle is necrotic, it should be removed to alleviate pain,

and the contralateral testicle explored and sutured inside the scrotum in 3 to 4 quadrants to prevent

future torsion. If a safe surgery is not an option, one may attempt a manual detorsion with a cord block.

Untwisting as if one were opening a book (inside out) is the most commonly recommend technique, but

it is by no means certain. Scheduling a prompt exploration is recommended even if there is relief with

manual detorsion.

When the müllerian ducts atrophy in males, they can leave a remnant as a testicular appendage on the

head of the testis. These can twist and mimic a full torsion. In pale-skinned patients, the torsed

appendage during early presentation can resemble a painful small blue dot that is seen through the

stretched skin. Because the appendage is vestigial and functionally useless, its torsion is managed with

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pain relief, anti-inflammatories and exertion restrictions.

9 Prepubertal Testicular Tumors

There is a separate chapter that discusses common childhood tumors.18 For this chapter, we will limit

our discussion to prepubertal testicular tumors. Most testicular tumors are nonseminomatous germ cell

tumors. They usually present as a distinct palpable painless mass in the scrotum. They can have a

reactive hydrocele, and it is important to palpate the entire testicle before assuming that the hydrocele

is harmless. The primary imaging study is a scrotal ultrasound and biomarkers. The two most useful

biomarkers are the alpha-fetoprotein (AFP) and beta-HCG. The most common types of prepubertal

tumors are teratoma and yolk sac. Teratoma is benign in prepubertal age group. Histologically they

contain all three germ layers (ectoderm, mesoderm, and endoderm). Due to the benign nature, testis

sparing approach is warranted. An enucleation of the mass through a tunica albuginea incision is

performed if the frozen section confirms the diagnosis (Figs. 104-18 and 104-19). Yolk-sac tumors are

the most common malignant prepubertal tumor. Most present early as stage 1 (limited to the testicle

and completely resected), and the AFP level will be elevated in the vast majority of cases. After radical

inguinal orchiectomy, most are observed with serial imaging and tumor markers, provided that other

staging work up is negative, with retroperitoneal lymph node dissection and systemic adjuvant

chemotherapy being reserved for those with advanced disease (persistent retroperitoneal lymph

adenopathy or increased serum markers). Seminomas and mixed germ cell tumors are extremely rare in

prepubertal children, and their management should be as in adults. There are gonadal stromal tumors

that can mimic a germ cell tumor. The most common are Sertoli cell tumors. They occur as painless

masse and usually have no major endocrine effect. Simple orchiectomy is all that is needed. Leydig cell

tumors are the second most common stromal tumor. They can be associated with precocious puberty,

gynecomastia, and elevated levels of 17-hydroxy ketosteroids. They can be similar to hyperplastic

adrenal nodules that are found in boys with poorly controlled congenital adrenal hyperplasia.

Figure 104-18. Testis-sparing enucleation of prepubertal testis tumor with cystic mass seen on the ultrasound and negative

markers, likely indicating a teratoma.

Figure 104-19. Histology of prepubertal testis tumor demonstrating mature teratomatous differentiation into ectodermal elements.

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Varicocele

Varicocele is a collection of engorged varicose veins in the spermatic cord and usually is most

prominent in the scrotum in the region of the pampiniform plexus. Varicoceles are graded according to

their size and prominence. Grade I are varicoceles only palpable with exertion or a Valsalva maneuver.

Grade II are varicoceles that can be palpated without exertion or a Valsalva maneuver and collapse

when supine. Grade III varicoceles are visibly bulging and do not collapse when supine. There is also a

subclinical (or grade 0) varicocele that is only detected on imaging and not on physical examination.

Varicoceles are most common on the left side comprising >90% of all varicoceles. The left-sided

predominance relates to the different nature of the left and right spermatic vein drainage. The right

spermatic vein is shorter and inserts directly into the inferior vena cava. About 10% are bilateral. The

solitary right varicocele is worrisome as it can be associated with a retroperitoneal tumor and when

found warrants further evaluation with abdominal ultrasound. Varicoceles come to attention because of

two major concerns. First, they can be quite sizable and uncomfortable. Second, varicoceles are

associated in some cases with infertility. This is believed to be the result of two effects. The pooling of

venous blood allows toxic waste products to accumulate. The pooled blood in addition may also raise

the temperature of the whole scrotum. Normally, the scrotum is several degrees cooler than the deep

core body temperature. At random, up to 10% to 20% of all adult males can be found to have some

form of varicocele. Among men who attend infertility clinics, varicocele-associated subfertility can be as

high as 40%. These observations suggest that there are many men in whom a varicocele is a trivial

minor finding, but there are some in whom it can be an important reproductive issue. For boys and

adolescence undergoing puberty and sexual maturation, semen analysis findings are variable and

unreliable in addition to ethical concerns. For this reason, the clinical indication for intervention in boys

is comparative testicular volume on ultrasound. In some, the involved testicle will have a smaller

volume or lag in growth. If there is a substantial volume discrepancy (20% or greater) between the

testicles, surgery is warranted. If the volumes are stable and there is no major discrepancy, observation

with annual ultrasounds is recommended until the age when a semen analysis can be obtained reliably.

There are several methods of treating varicocele; the most commonly used approach is via subinguinal

or inguinal incision. The vas deferens and spermatic artery are identified and spared. The varicocele

veins are then ligated and the segment between the ligatures is removed. The use of an operating

microscope allows sparing of the lymphatics thereby reducing the risk of postsurgical hydrocele to well

under 1%.

VAGINAL ANOMALIES

Labial Adhesions

Labial adhesions occur when edges of the labia minora attach in the midline and form a thin web of skin

(Fig. 104-20). This can obstruct the normal path of the urinary stream leading to postmicturition

spotting when the girl stands up. Treatment in very mild cases can be successfully achieved with

estrogen cream applied to the surface of the webbing over several weeks. For more severe or refractory

cases, surgical incision and separation under anesthesia are needed. Minor adhesion may be left alone

without clinical concerns.

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Figure 104-20. Labial adhesions.

Figure 104-21. Imperforate hymen.

Imperforate Hymen

Occasionally the hymen will be imperforate at birth. This will present with a bulging hydrocolpos and

can be confused with a prolapsing ureterocele or bladder tumor (such as sarcoma) (Fig. 104-21).

Ultrasound or MRI can usually identify the prominent fluid-filled vagina. Puncture and drainage are

usually all that is required. In some older patients the condition may not be diagnosed until after the

onset of menses. At this time, there may be cyclic discomfort due to the inability to allow the menstrual

flow to pass creating a hydrometrocolpos. After a complete workup surgical drainage in the operating

room is advisable to avoid introducing bacteria into the sterile collection; an infection can lead to

pyocolpos or pyometrium leading to scarring and affecting future fertility.

Urogenital Sinus Anomalies

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Between the fifth and seventh week of fetal development the urogenital sinus forms.19 The caudal

portion forms the lower-third of the vagina. The müllerian ducts form the upper two-thirds of the

vagina and the uterus as well as fallopian tubes. Anomalies can occur as part of DSD conditions resulting

in either vaginal agenesis or urogenital sinus (such as that seen in masculinized females with congenital

adrenal hyperplasia). Surgical reconstruction is usually required to create distinct urethra and vaginal

channels. Traditional surgical approach aimed at separating the vagina from the UG sinus tract and

bringing it down to the perineum, but this often led to vaginal vascular compromise and stenosis.

Modern techniques employ urogenital sinus mobilization en bloc, keeping the urethrovaginal confluence

intact. Total mobilization has been discouraged due to concern for future urinary incontinence and

pelvic prolapse. A combination of partial mobilization – leaving the anterior urethral suspensory

ligaments intact – and various perineal flaps can result in an excellent outcome. The Mayer–Rokitansky–

Kusterhauser syndrome occurs in normal genotypic females with vaginal and/or uterine dysgenesis.

About one-third of these patients will have associated urologic issues – the most common being

unilateral renal agenesis. The diagnosis is usually made incidentally or around the age of puberty due to

amenorrhea. Treatment is vaginal dilation with estrogen cream or surgical vaginoplasty if dilation fails.

There are also a variety of duplication anomalies and vaginal and/or uterine septums and consultation

with gynecology is advisable on the necessity and timing of intervention. Typically the goals are to

relieve any obstruction to restore normal menses and to preserve reproductive potential.

Figure 104-22. Prune-belly syndrome. Notice the wrinkled appearance of the abdominal wall due to deficiency of musculature.

Scrotum is empty due to bilateral intraabdominal testicles.

Prune-belly Syndrome

The prune-belly syndrome (PBS) is a constellation of malformations also known as the triad syndrome

or the Eagle–Barrett syndrome.20 The syndrome has three key components: severe deficiency or absence

of the abdominal wall musculature, bilateral hydroureteronephrosis, and bilateral undescended

intraabdominal testicles (Fig. 104-22). There are also other associated genitourinary conditions

including renal dysplasia, fusiform urethra, large bladder, patent urachus, hypoplastic prostate, and

dilated proximal urethra. The underlying cause is unknown, but the current theory is that this is

triggered by a severe distal urethral obstruction early in development. This theory is supported by the

observation of that many of the similar effects are seen in PUVs. The newborn will present with a lax

and wrinkled abdominal wall, along with empty scrotum. Renal dysplasia is frequent and when severe,

it can lead to pulmonary hypoplasia. Stillbirths and early neonatal demise often occur because of the

combination of poor renal and pulmonary function. Hydroureteronephrosis can be massive but is

typically nonobstructive, and no procedure should be done simply to improve appearance. Nearly all of

the patients are infertile with an anecdotal report of successful fertility using the assisted reproductive

technology. For infants who survive into childhood, bilateral orchidopexy and abdominal wall

reconstruction may be necessary. Evaluation for bladder function is also required since many will have a

poor bladder muscle tone and high post void residual.

10 Neurogenic Bladder

The nervous system plays an important role in modulating the normal behavior of the lower urinary

tract. There are nerve roots (emanating from the spinal cord) and peripheral pelvic nerves that carry

sensory and motor signals to the bladder and urethral sphincter. Injuries or anomalies to these nerves

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can cause the bladder to be denervated, leading to poor emptying. At a higher level, the spinal cord up

to the brain stem is critical to regulation of lower urinary tract function, especially the thoracolumbar

sympathetic and sacral parasympathetic segments. The brain stem acts as the coordinating center. With

filling the bladder musculature – under the influence of the sympathetic input – remains relaxed thereby

keeping bladder storage pressures low until emptying occurs (driven by parasympathetic input). The

higher centers in the cerebral cortex allow conscious control and inhibition of reflex activity. Without

this influence the bladder fills, and at a certain volume it spontaneously empties with coordination. This

is what occurs in infants prior to toilet training. When the higher centers are affected (such as due to a

cerebrovascular accident, tumor, traumatic brain injury, or multiple sclerosis), this control is lost or

diminished and many patients will report sudden urgency and loss of urinary control. When the spinal

cord is affected, the bladder will lose its inhibitory control by the brain stem and cerebral cortex and

will act autonomously. The bladder will contract earlier than full capacity, and it is termed

hyperreflexia. The loss of coordination between bladder muscle and sphincter, leading to poor emptying

is known as detrusor–sphincter–dyssynergia.

Patients with spinal cord injury, traumatic brain injury, and acquired conditions that affect the

nervous system (e.g., diabetes mellitus, multiple sclerosis, varicella zoster, brain tumors, and

encephalitis) should trigger awareness about possible urinary effects.21 Mere urine egress from the

bladder is not necessarily a sign of normal voiding. It can be due to overflow incontinence or some form

of dysfunctional voiding (Valsalva voiding). Likewise continence is not necessarily a sign of

neurologically coordinated normal bladder function. The storage pressures can be higher than normal.

When the bladder urine storage pressures are too high, it can overcome the peristaltic pressure driving

urine from the kidneys down the renal pelvis and ureter. Ideally the storage pressure should be below

40 cm H2O.22 Above this pressure, hydronephrosis, pyelonephritis, and loss of renal function will occur.

A urology evaluation with urodynamic pressure–volume assessment of the lower urinary tract should be

considered if there is any condition with significant effects on the central nervous system.

Spina bifida is the most common congenital cause of a neurogenic bladder in children. During

development the spinal column does not form properly resulting in an exposed spinal cord, and patients

are born with a neurogenic bladder. Surgical closure in utero is now being done in hopes that early

closure can lead to better motor and bladder function, although this has not been demonstrated to date.

Early evaluation of bladder storage pressures is crucial. Regular, proactive bladder pressure monitoring

with cystometrogram, along with ultrasound assessment of the upper tracts, has shown to reduce

urologic morbidity in these patients. Children who do not empty with a safe bladder pressure require

CIC. Patients with poor bladder compliance and high storage pressures may need antimuscarinic bladder

muscle relaxants (such as oxybutynin). For older children, achieving social continence becomes

important and a variety of surgical options may be considered in addition to CIC and antimuscarinics.

Surgical bladder augmentation using intestinal segments (enterocystoplasty) is the most effective way

to improve the bladder storage function (Fig. 104-23), but there are many well-known long-term

complications, such as metabolic acidosis, mucus production, UTI, bladder stones, spontaneous

perforations, and malignancy. In some patients who have difficulty with urethral CIC (such as those

with severe scoliosis or girls in wheelchair), surgical creation of alternate catheterizable neourethra is

an option. The Mitrofanoff procedure (the classic appendicovesicostomy where a collapsible supple

appendix is implanted into the bladder within the submucosal tunnel for continence) can improve the

quality of life significantly. If the bladder outlet is inadequate, surgical options include artificial urinary

sphincter, bladder neck reconstruction with sling and bladder neck closure. Similarly, many patients will

also choose the surgical option of Malone antegrade continence enema where a catheterizable channel is

created into the colon for easy, self-administration of enema for achieving fecal continence.

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Figure 104-23. Bladder augmentation using a patch of detubularized ileum.

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