Chapter 22 Perinatal Urology 387

TABLE 22.7 Causes of Scrotal Masses in the Neonate or swelling), and the testis will be tender. In prenatal torsion, the testis
will likely appear hyperechoic and avascular. In both prenatal and
Extravaginal torsion postnatal torsion, the testis can appear heterogeneous in echotexture.
Appendage torsion Bilateral or unilateral hydrocele may be present. Doppler studies will
Incarcerated hernia demonstrate absence of flow in the affected testis (Arena et al., 2006).
Hydrocele In the case of a nonsalvageable prenatal torsion, a contralateral
Epididymal cyst
orchidopexy may be performed on an urgent basis to avoid the
devastating consequence of asynchronous contralateral torsion. There
Meconium
is no uniform agreement among pediatric urologists in regard to the
Testis tumors urgency or ultimate need to explore these children. Generally, the
Trauma risk for extravaginal torsion is thought to be present for only
Hematoma from adrenal hemorrhage the first few weeks of life, and intervention should be pursued before
Tumor seeding (renal or adrenal tumors) this window closes.
For postnatal testicular torsion, management should be similar
to what is recommended for acute testicular torsion that appears
later in life, recognizing that the timing of presentation varies. The
decision to proceed with immediate surgical exploration should
catheterization, and polycythemia. Up to 50% of neonates with be measured in which the parents are aware of the low salvage rate
RVT have a prothrombotic disorder and should undergo hematologic for the affected testis (Brandt et al., 1992; Sorenson et al., 2003).
evaluation (Kuhle et al., 2004). Treatment consists of aggressive The main rationale behind intervention for postnatal torsion is the
hydration and expectant management. Anticoagulation should prevention of asynchronous contralateral torsion. A spinal anesthetic
be considered, and consultation with pediatric nephrology should can be employed to avoid neonatal exposure to inhalation anesthetic.
be obtained. To identify a scrotal mass, a thorough physical examination should
Renal artery thrombosis (RAT) is chiefly caused by umbilical be undertaken. Transillumination can assist in identifying a hydrocele.
artery catheter placement. The clinical presentation includes Gentle palpation of the mass may unveil a reducible inguinal hernia
hypertension and congestive heart failure. A renal scan may reveal or a communicating hydrocele. An abdominal radiograph may reveal
lack of perfusion to areas of the cortex. The umbilical artery bowel gas in the scrotum if herniated bowel is present. The presence
catheter should be removed and thrombolytic therapy may be of stippled calcifications suggests a history of meconium peritonitis
initiated (Schmidt and Andrew, 1995). (Kenney et al., 1985). Wilms tumor or neuroblastoma may seed the
Renal cortical necrosis can result from any condition in which testis through a patent processus. Scrotal hematoma has been reported
perfusion to the kidneys is decreased. Risk factors include hypotension, to masquerade as neonatal torsion (Diamond, 2003). Testis tumors
anoxia, severe hemorrhage, necrotizing enterocolitis, sepsis, placental in this age group are rare and include yolk sac tumors, teratomas,
abruption, or twin-twin transfusion syndrome (Jernigan, 2014). Renal juvenile granulosa cell tumors, and gonadal stromal tumors (Levy
ultrasonography will demonstrate loss of corticomedullary differentia- et al., 1994). Although rare, it is important to include possible
tion with a hypoechoic rim at the edge of the renal cortex (Sefczek malignancy in the differential diagnosis of a scrotal mass and perform
et al., 1984). Treatment is supportive. Neonates who experience renal an abdominal ultrasonography when suspected.
cortical necrosis carry a lifetime risk for renal failure.

Scrotal Mass in the Neonate KEY POINTS

The assessment of a newborn scrotal mass should discern the acuity • A urologic source for abdominal distention in the
of the condition. A thorough discussion with the nursing staff and newborn includes urinary ascites, urinary obstruction
delivery team combined with palpation should allow for this assess- (upper and lower tract), renal mass (cystic and solid), and
ment. The differential diagnosis is listed in Table 22.7. rarely a retroperitoneal mass (adrenal).
• Urinary retention may occur with LUTO and may occur in
Testicular Torsion males with PUV or females with urogenital sinus or
cloacal anomaly.
Testicular torsion in the newborn may be time-sensitive depending • Renal tumors are uncommon in the newborn period, and
on the acuity of the presentation. Typically, torsion in the newborn the most common is mesoblastic nephroma.
period is extravaginal, which involves twisting of the tunica vaginalis • The newborn with ambiguous genitalia should prompt
and the spermatic cord contained within. Extravaginal torsion may an evaluation for electrolyte disturbance caused by
be categorized into two separate phenomena, prenatal torsion (in classical salt-wasting CAH. Gender assignment should be
utero) and postnatal torsion (acute) (during the first 30 days of life). deferred.
This distinction is important because the salvage rate of a prenatally • Gross hematuria in the neonate is not common and
torsed testis is nearly nonexistent, whereas a postnatal extravaginal should be evaluated with urine analysis and Doppler
torsion carries a salvage rate of up to 44% (Brandt et al., 1992; abdominal ultrasonography.
Sorensen et al., 2003). Indeed, a survey of pediatric urologists found • The acuity of neonatal testicular torsion will dictate the
that 43% of respondents would treat a prenatal torsion as a surgical need for emergent exploration.
emergency, whereas 93% would immediately explore a neonate with
postnatal torsion (Broderick et al., 2013).
A neonate with prenatal testicular torsion will present with an
enlarged discolored scrotum accompanied by a nontender, solid testis
that is fixed to the scrotal wall. In contrast, postnatal torsion will REFERENCES
present with a postnatal change in scrotal examination (erythema and/ The complete reference list is available online at ExpertConsult.com.
 Chapter 44 Management of Abnormalities of the External Genitalia in Boys 893

in about 30% of cases (Belman, 2001; Bayne et al., 2008; Cozzi

et al., 2008; Ferro et al., 1995; Nagar and Kessler, 1998). These BOX 44.2 Differential Diagnosis of Pediatric
noncommunicating scrotal masses are tense and extend into the Adolescent Acute Scrotal Pain
abdomen, where they may be palpable. Ultrasound may aid in
Appendage torsion
defining the proximal extent (Belman, 2001). Abdominoscrotal
hydroceles usually manifest in infancy as such or as scrotal hydroceles Appendix testis
that enlarge over time (Celayir et al., 2001; Cuervo et al., 2009), Other appendage (epididymis, paradidymis, vas aberrans)
improve (Cozzi et al., 2008), or resolve spontaneously (Upadhyay Spermatic cord torsion
et al., 2006). Associated diagnoses include cryptorchidism, contra- Intravaginal, acute or intermittent
lateral hernia, hydrocele, or vanishing testis. Extravaginal
The most likely cause is enlargement and extension of a scrotal Epididymitis
hydrocele into the retroperitoneal or properitoneal space after closure Infectious
of the processus. Bayne et al. (2008) found that abdominoscrotal Urinary tract infection
hydrocele fluid was exudative and theorized that increasing size and Sexually transmitted disease
pressure would lead to lymphatic obstruction and progression. Massive
?Viral
enlargement could extend into the upper abdomen and be associated
with hydroureteronephrosis, lower extremity edema, or appendicitis Sterile or traumatic
(reviewed by Cuervo et al., 2009). Chamberlain et al. (1995) first Scrotal edema or erythema
reported dysmorphic elongation of the testis; this was subsequently Diaper dermatitis, insect bite, or other skin lesions
confirmed (Bayne et al., 2008) but found to be reversible in most Idiopathic scrotal edema
cases (Cozzi et al., 2008). Orchitis
The traditional surgical approach is an inguinal incision with Associated with epididymitis with or without abscess
proximal dissection of the sac from its abdominal attachments and Vasculitis (e.g., Henoch-Schönlein purpura)
distal complete or partial mobilization, with or without orchidopexy. Viral illness (mumps)
Some authors advocate orchidopexy to avoid iatrogenic cryptorchidism Trauma
(Bayne et al., 2008; Nagar and Kessler, 1998). Aspiration of the
Hematocele or scrotal contusion or testis rupture
scrotal component may facilitate the proximal dissection (Cuervo
et al., 2009). Alternative approaches include a midline abdominal Hernia or hydrocele
approach for large bilateral cases (Serels and Kogan, 1996) or lapa- Inguinal hernia with or without incarceration
roscopic decompression of the abdominal component followed by Communicating hydrocele
inguinal excision (Abel et al., 2009). To avert injury to the spermatic Encysted hydrocele with or without torsion
cord or vas, a strip of the lining of the sac may be left along the Associated with acute abdominal pathology (e.g.,
cord (Cuervo et al, 2009; Ferro et al., 1995). Tightening of a patulous appendicitis, peritonitis)
internal ring is described but may be unnecessary because the proces- Varicocele
sus vaginalis is invariably closed. Intrascrotal mass
Belman (2001) described a primary scrotal approach with drainage, Cystic dysplasia or tumor of testis
excision, and extensive plication with limited dissection. Cozzi et al.
Epididymal cyst, spermatocele or tumor
(2008) reported reduced morbidity and similar efficacy for the scrotal
approach (5 patients) compared with the inguinal approach (13 Other paratesticular tumors
patients). Persistent scrotal swelling, hematoma, and undescended Musculoskeletal pain from inguinal tendonitis or muscle strain
and/or hypoplastic testis was reported in 11 inguinal cases and Referred pain (e.g., ureteral calculus or anomaly)
infection in 2 scrotal cases.

ACUTE SCROTUM scrotal pain and who overwhelmingly (96%) felt increased public
awareness of the condition was needed. In a survey of more than
Acute scrotum refers to the constellation of new onset of pain, 500 parents in a nonurgent setting, Friedman et al. (2016a) found
swelling, and/or tenderness of intrascrotal contents. There is a 34% had heard of testicular twisting/torsion, most commonly through
limited differential diagnosis (Box 44.2) with considerable overlap friends, relatives, or knowing someone with torsion (35%) and only
of signs and symptoms, which may affect the ability to make a 17% from their pediatrician. Only 13% of parents had spoken with
definitive diagnosis; some reliable clinical features exist, and adjuvant their children about torsion. In response, these authors presented
use of scrotal imaging is helpful in making a diagnosis. Torsion of an electronic module aimed at educating health care providers on
the appendix testis is the most common diagnosis (40% to 60%), the evaluation and differential diagnosis of acute scrotal pain that
followed by spermatic cord torsion (20% to 30% excluding neonates), was learned within 30 minutes, increased confidence in assessment
epididymitis (5% to 15%), and other or no pathology (10%) (Friedman et al., 2016b), and produced a publicly available educa-
(Anderson and Giacomantonio, 1985; Mäkelä et al., 2007; Mushtaq tional video regarding testis torsion aimed at patients and families
et al., 2003; Murphy et al., 2006; Sidler et al., 1997; Van Glabeke (https://www.healthychildren.org/English/health-issues/conditions/
et al., 1999). Although all of these diseases can occur at any time genitourinary-tract/Pages/Testicular-Torsion.aspx).
during childhood, appendage torsion is typically most common
after infancy and before puberty, whereas epididymitis and spermatic Spermatic Cord Torsion
cord torsion are most common in the perinatal and pubertal periods.
Torsion of an appendage and epididymitis are managed conservatively Acute Intravaginal Spermatic Cord Torsion
with limited consequence; prompt surgical exploration for testicular
torsion is imperative because the gonad is at considerable risk of Predisposing Factors. Intravaginal torsion is commonly attributed
ischemic damage or loss, particularly when there is a delay in presenta- to excess mobility of the testis within a “bell-clapper deformity,”
tion, evaluation, or management. wherein the tunica vaginalis abnormally fixes proximally on the
Delay in presentation for medical attention may stem from patient cord. Although found in 12% of males at autopsy (Caesar and Kaplan,
embarrassment or denial but also from lack of public awareness 1994a), the prevalence of torsion is much lower: 8.6 per 100,000
regarding torsion and the possible consequences. Ubee et al. (2014) males aged 10 to 19 per year in the United States (Mansbach et al.,
surveyed parents of 62 boys undergoing emergent scrotal exploration, 2005). There is evidence for a familial predisposition (Collins and
among whom 66% did not fully appreciate the implications of acute Broecker, 1989; Cubillos et al., 2011; Cunningham, 1960) for which
894 PART III Pediatric Urology

the transmission is unknown. Cubillos et al. (2011) found a family not readily available, and used ionizing radiation, and is currently
history (various relatives) in 10% of probands, including one family rarely used. Ultrasound offered a rapid, available, and safe modality
with three generations of torsion. The inciting event for torsion is to assess testicular architecture, intraparenchymal blood flow, and
unknown but may include cold temperature (Chiu et al., 2012; other anatomic details (hydrocele, scrotal thickening).
Lyronis et al., 2009; Srinivasan et al., 2007) even in tropical countries CDUS findings consistent with testicular torsion include reduced
(Gomes et al., 2015) or a change in temperature (Chen et al., 2013) or absent Doppler color or waveforms and parenchymal heterogeneity
activating the cremasteric reflex, and/or rapid testicular growth at compared with the contralateral testis (Fig. 44.32). Kaye et al. (2008b)
puberty; yet torsion may occur at rest or at sleep. Cryptorchid testes and later Chmelnik et al. (2010) found that all testes with hetero-
are at increased risk of torsion and difficult to assess because of geneous echogenicity were necrotic, whereas homogeneous
the high position. Torsion after previous orchidopexy may be related echogenicity predicted a lower risk of orchiectomy. Lack of
to failure of suture (absorbable or nonabsorbable suture) to fix the demonstrable intratesticular flow on CDUS is 86% sensitive, 100%
testis in place (Frank and O’Brien, 2002; Mor et al., 2006; Redman specific, and 97% accurate in the diagnosis of torsion and ischemia
and Barthold, 1995). in painful scrotum (Burks et al., 1990) and its use spread (Kass
Clinical Presentation. Intravaginal testicular torsion may occur at et al., 1993b); even current studies reflect the accuracy of this
any age, but the vast majority of cases occur after age 10 years with approach. Altinkilic et al. (2013) prospectively assessed the diagnostic
a peak at 12 to 16 years (Anderson and Giacomantonio, 1985; value of CDUS in patients with clinical suspicion of torsion who
Mäkelä et al., 2007; Mansbach et al., 2005; Murphy et al., 2006; were explored by a surgeon blinded to the CDUS results. The sensitiv-
Mushtaq et al., 2003; Sidler et al., 1997). The prevalence of testicular ity, specificity, and positive and negative predictive values of CDUS
torsion is 1 in 4000 (Williamson, 1976) with left-sided predominance for detecting testicular torsion were 100%, 75.2%, 80.4%, and 100%,
and rare bilaterality. Classically, boys complain of acute, severe scrotal respectively. The authors concluded that routine surgical exploration
pain that occurs at rest (even sleep) or with physical activity or after was unnecessary if CDUS reveals normal intratesticular perfusion.
trauma. A history of prior episodes may be elicited. Alternatively, However, in other studies the sensitivity in confirming decreased or
patients may have milder, less acute, or even absent scrotal pain or absent blood flow in proven cases of spermatic cord torsion was
may have inguinal or abdominal pain. Nausea and vomiting occur only 63% to 90%, possibly because of enhanced detection of flow
in 10% to 60% of boys (Jefferson et al., 1997; Knight and Vassy, with newer equipment and/or user-dependent characteristics (Bentley
1984; Mäkelä et al., 2007; Sessions et al., 2003; Williamson, 1976). et al., 2004; Kalfa et al., 2004; Karmazyn et al., 2005; Stehr and
Scrotal edema and erythema may be present, depending on the Boehm, 2003; Steinhardt et al., 1993). Cassar et al. (2008) evaluated
duration or degree of torsion. Dysuria and fever are uncommon. Doppler waveforms in cases of torsion with decreased or preserved
The most common physical findings are generalized testicular testicular flow and observed subtle waveform abnormalities, including
tenderness, abnormal orientation of the testis, and absent cremasteric increase or decrease in amplitude relative to the normal testis and
reflex. Inspection may identify the high-riding testis from a fore- reversal of diastolic flow. Increased epididymal size and/or echo-
shortened cord and horizontally oriented testis. The genitofemoral genicity and altered epididymal vascularity, usually absent or reduced
reflex arc, normally present after age 2 years (Caesar and Kaplan, but occasionally increased, may provide additional support for the
1994b), is elicited by scratching the inner thigh with resultant testis diagnosis of torsion (Nussbaum et al., 2006).
elevation. Some studies report reduced or absent reflex in all cases High-resolution (10- to 20-mHz probe) ultrasonography (HRUS)
of testicular torsion (Caldamone et al., 1984; Kadish and Bolte, 1998; of the length of the spermatic cord may enhance the ability to diagnose
Rabinowitz, 1984), but it was intact in up to 10% of proven cases torsion. Using HRUS to directly image the cord proximal to the
of torsion in other series (Hughes et al., 2001; Karmazyn et al., 2005; testis, Kalfa et al. (2004) visualized the cord twist as a 1- to 3-cm
Murphy et al., 2006; Nelson et al., 2003). The presence of a crem- snail-shaped mass in 43 patients and a completely linear cord in
asteric reflex correlates with intact testicular blood flow but does nontorsion cases (Fig. 44.33). In a multi-institutional retrospective
not unequivocally indicate normal testicular perfusion, especially series of 919 cases of acute scrotum with cord imaging by HRUS, a
if the clinical presentation is otherwise suggestive of torsion. cord twist was seen in 96% of cases of surgically proven torsion,
Although anterior epididymal position, thickening of the cord, and HRUS carried 99% specificity when the cord was linear (Kalfa
testicular induration, loss of boundaries between the testis and et al., 2007). However, Karmazyn et al. (2005) observed normal
epididymis, scrotal edema, and/or erythema may be present, land- testicular blood flow and no visible spermatic cord twist in 2 of 41
marks become obliterated and the examination less reliable as the boys with partial or intermittent torsion.
duration of torsion increases. Potentially useful diagnostic modalities include contrast-enhanced
Several efforts have been made to offer a better clinical assess- pulse-inversion ultrasonography (CEUS), infrared thermography and
ment of testicular torsion in patients with the acute scrotum. spectroscopy, and scrotal MRI. Pulse-inversion imaging in a rabbit
Using a standardized history and physical examination form, model showed superior quantitative assessment of perfusion of the
Srinivasan et al. (2011) found that absence of ipsilateral cremasteric experimentally torsed testis as compared with conventional CDUS
reflex, nausea or vomiting, and scrotal skin changes on multivariate (Paltiel et al., 2006). CEUS was performed in 50 patients with acute
analysis were predictive of testicular torsion. According to use of a scrotal pain or trauma in whom testicular lesion of undefined nature
decision tool, patients with acute (<72 hours) scrotal pain and all was found at CDUS. Sensitivity and specificity were 76% and 45%
of the following had no risk of testicular torsion (100% sensitivity for CDUS and 96% and 100% for CEUS, respectively (Valentino
and negative predictive value): normal testicular lie, lack of nausea et al., 2011). Infrared thermography showed significant reduction in
and vomiting, and age 0 to 10 years (Shah et al., 2013). Barbosa scrotal temperature by 1 hour after 720-degree torsion and prompt
et al. (2013) developed a scoring system based on testicular swelling, normalization with detorsion in a sheep model (Capraro et al., 2008).
hard testicle, absent cremasteric reflex, nausea or vomiting, and Transscrotal near infrared spectroscopy was able to reliably discrimi-
high-riding testis, which was validated by Sheth et al. (2016). In an nate torsion from nontorsion in a select group of children with
effort to reduce in-hospital delay, a standardized process was used acute testis pain (Schlomer et al., 2017). MRI has been used in a
at Texas Children’s Hospital that included a sign and symptom scoring small series of testicular torsion and may have a role in difficult
tool for stratifying risk and improving communication that allowed diagnostic cases with use of dynamic contrast-enhanced MRI, which
significantly reduced time from emergency department or ultrasound requires gadolinium (Mäkelä et al., 2011), or diffusion-weighted
suite to the operating room (Afsarlar et al., 2016). imaging with findings of a lower testicular apparent diffusion coef-
Diagnostic Studies. Urinalysis is of limited usefulness in cases of ficient (ADC) without the need for contrast (Maki et al., 2011).
testicular torsion but is used to identify pyuria and/or bacteriuria
associated with epididymitis or hematuria, implicating a urinary Management and Surgical Treatment
tract calculus. Before the advent of reliable and rapid scrotal imaging,
immediate scrotal exploration was routine. Radionuclide imaging Testicular torsion is a true surgical emergency because testis viability
carried about 90% sensitivity and specificity but was lengthy, was is inversely related to duration of torsion. Visser and Heyns (2003)
 Chapter 44 Management of Abnormalities of the External Genitalia in Boys 895

A1 A2 B1 B2

A3 B3

C1 C2
Fig. 44.32. Imaging of intravaginal spermatic cord torsion. (A) Intermittent torsion. Color Doppler ultra-
sonography (CDUS) demonstrates preserved arterial flow to testis with 12 hours of pain and then loss of
flow and parenchymal heterogeneity when child returned later with worsening pain. (B) Acute torsion with
reduced arterial flow. (C) Prolonged torsion. CDUS shows heterogeneous testis without arterial or venous
flow and a hyperechoic parenchymal ring.

Fig. 44.33. Snail sign. B-Mode (left) and color Doppler ultrasonography (right) images showing hyper-
echoic central body of the snail and coiling of blood within the distal spermatic cord around the central
echogenic “body.”
896 PART III Pediatric Urology

amassed data from a published series including 1140 patients and in warm soaked gauze, and observed for improvement in color,
found that the risk of orchiectomy was approximately 5%, 20%, 40%, whereas the contralateral testis is fixed with nonabsorbable suture
60%, 80%, and 90% at 0 to 6, 7 to 12, 13 to 18, 19 to 24, more than to reduce the risk of metachronous torsion. The affected testis
24, and more than 48 hours after onset of pain, respectively. The is re-examined for potential viability, and the largely subjective
degree of torsion may provide incomplete vascular occlusion, helping decision for orchidopexy or orchiectomy is made. A Doppler flow
to explain the variability of these data or have a multiplicative effect probe or incision of the tunica albuginea (Arda and Ozyaylali,
on the time course to testis loss (Dias et al., 2016). 2001) with assessment of bleeding may document intratesticular
Orchiectomy after surgical detorsion occurs in 30% to 70% in flow after detorsion; however, the reliability of these assessments
large studies (Kaye et al., 2008b; Mäkelä et al., 2007; Murphy et al., lacks validation. If the testis is to be retained, it is fixed either via
2006; Sessions et al., 2003) and in 32% to 42% in database reviews dartos pouch or directly to the dartos with nonabsorbable suture.
(Cost et al., 2011; Mansbach et al., 2005; Zhao et al., 2011). Sessions Kutikov et al. (2008) have suggested that a compartment syndrome
et al. (2003) found that the median degree of rotation was 540 contributes to testicular injury based on the improved appearance
degrees in orchiectomy testes and 360 degrees when the testis and lower intraparenchymal pressures seen after detorsion and tunica
was salvaged, with a range of 180 to 1080 degrees in both groups. albuginea incision in three cases. A patch of vascularized tunica
The risk of delayed atrophy after orchidopexy was less than 10%, vaginalis was placed in the tunica albuginea defect to maintain lower
40%, and 75% after less than 12, 12 to 24, and more than 24 hours intraparenchymal pressure and to reduce the likelihood of ongoing
of pain, respectively (Visser and Heyns, 2003). Partial (<25%) testicular ischemia (Fig. 44.34). Figueroa et al. (2012) applied this technique
atrophy may occur after operative detorsion even after 4 hours after to 11 of 28 testes deemed nonsalvageable at surgery and found
the onset of pain (Anderson and Williamson, 1986; Krarup, 1978; that 55% recovered. The long-term outcome of these patients is not
Sessions et al., 2003; Tryfonas et al., 1994). Among 37 patients with available; however, in a rat model of testicular torsion, no significant
at least 6 months’ follow-up, Lian et al. (2016) found that 54% histologic differences were found between a detorsion group and
developed testicular atrophy (>50% compared with contralateral detorsion with tunica vaginalis flap group after 4 weeks, despite
testis) even when deemed viable intraoperatively; duration of pain reduction of intratesticular pressure in both groups (Józsa et al., 2016;
of more than 1 day and sonographic parenchymal heterogeneity Oktar et al., 2013).
were predictive of subsequent atrophy. Preoperative manual cord Risk factors for orchiectomy include young age, African-American
detorsion may relieve symptoms and allow delayed orchidopexy race, and being on Medicaid or lacking insurance (Cost et al., 2011;
but may incompletely untwist the cord. Sessions et al. (2003) found Zhao et al., 2011). Zhao et al. (2011) identified surgery at a children’s
that lateral twisting (rather than medial untwisting) occurred in unit to increase the risk of orchiectomy. This may reflect the transfer
33% and manual detorsion failed to completely relieve torsion in of patients, which delayed treatment by 75 minutes in the study by
32% of orchidopexy cases. Bayne et al. (2010), but did not affect the orchiectomy rate in the
There are two management approaches to the acute scrotum. study by Ramachandra et al. (2015). Orchiectomy is performed by
One is to avoid delay and perform exploration in almost all boys dividing the cord into segments, each of which is ligated with
to confirm absence of torsion; this implies many unnecessary surgeries nonabsorbable suture. In cases of orchiectomy, prosthesis placement
(Anderson and Giacomantonio, 1985; Mäkelä et al., 2007; Mushtaq is usually offered after complete healing or later in puberty; however,
et al., 2003; Sidler et al., 1997; Watkin et al., 1996). The other Bush and Bagrodia (2012) demonstrated the feasibility of performing
approach is more selective of patients for surgical exploration concurrent prosthetic placement and orchiectomy.
(Caldamone et al., 1984; Kass et al., 1993b; Kalfa et al., 2004; Lam
et al., 2005) based on the history, physical examination, and CDUS Prognosis
findings, which may lead to testis loss because of atypical presentation
and/or false-negative imaging. When findings support or raise Although the impact of testicular torsion on fertility is poorly
suspicion for spermatic cord torsion, emergent scrotal exploration understood given the inherent difficulty of long-term follow-up in
is indicated and should not be delayed. these patients, the few available studies suggest that subtle abnormali-
Surgical exploration of the testis through a hemiscrotal transverse ties of semen quality are common. Semen density is often within
(dartos pouch) or midline raphe incision should first address the the normal range but correlates with shorter duration of torsion
affected side. The testis is delivered and the tunica vaginalis opened and reduced atrophy (Anderson et al., 1992; Arap et al., 2007; Brasso
to note the color of the testis, the number of rotations, and the et al., 1993; Fisch et al., 1988; Puri et al., 1985). The observation
anatomy of the tunica vaginalis. The testis is untwisted, wrapped that increasing duration of torsion inversely correlates with semen

A B C

Fig. 44.34. Tunica vaginalis vascularized patch. Tense congestion of the testis (A) was relieved with
incision of the tunica albuginea (marked). The tunica vaginalis flap on a vascularized pedicle is harvested
(B) and used to fill the defect in the tunica albuginea (C). (Courtesy Douglas Canning, MD, and Jason
Van Batavia, MD.)
 Chapter 44 Management of Abnormalities of the External Genitalia in Boys 897

quality and limited contralateral testicular biopsy data suggest that

global testicular dysfunction may exist after torsion (Visser and Heyns,
2003). The hypothesis of an autoimmune phenomenon (Anderson
and Williamson, 1990) was dispelled by analysis of antisperm
antibodies in individuals with torsion (Anderson et al., 1992; Arap
et al., 2007; Brasso et al., 1993; Puri et al., 1985). Available animal
and human data support a role for ischemia-reperfusion injury after
release of testicular torsion (Kehinde et al., 2003; Turner et al., 2004).
Additional clinical data are needed to determine long-term outcome
after testicular torsion and the efficacy of any adjunctive treatment.
Romeo et al. (2010) assessed serum levels of FSH and LH (before
and after GnRH stimulation), testosterone, and inhibin B in 20
patients 5 years (mean) after testicular torsion and in 15 age-matched
controls; 12 patients were treated with detorsion and orchidopexy,
and 8 underwent orchidectomy. Serum FSH, LH, and testosterone
were within the reference range. Inhibin B levels were significantly
reduced in the two torsion groups compared with the controls but
not between each other. Fig. 44.35. Extravaginal spermatic cord torsion in a neonate. Transverse
ultrasound image demonstrates enlarged and heterogeneous left testis and
bilateral hydroceles.
Intermittent Intravaginal Spermatic Cord Torsion
Episodes of self-limited acute scrotal pain precede acute testicular
torsion in 30% to 50% of patients (Stillwell and Kramer, 1986;
Williamson, 1976). These episodes, single or multiple, typically begin series (Baglaj and Carachi, 2007; Yerkes et al., 2005) and may occur
and resolve acutely with durations of minutes to hours. Hayn et al. concurrently or metachronously. Several series indicate that bilateral
(2008) observed that the frequency of these episodes correlated with metachronous torsion may occur in boys in whom the primary event
the risk for eventual persistent torsion and testicular loss; 71% of occurs prenatally or postnatally (Al-Salem, 2007; Beasley and McBride,
patients were previously diagnosed with epididymitis or appendage 2005; John et al., 2008; Yerkes et al., 2005).
torsion, and 53% had acute or delayed testicular loss. Nausea and/ Scrotal imaging may be obtained in cases of suspected perinatal
or vomiting or notation of scrotal swelling may or may not be present. torsion, but its usefulness and reliability are questionable. Prenatal
A normal vertical testicular orientation is most common (Hayn et al., ultrasonography may show torsion (Herman et al., 2002). Postnatal
2008), but a horizontal lie may be present (Schulsinger et al., 1991). ultrasound may reveal parenchymal heterogeneity, calcification, and
Physical findings consistent with torsion depend on whether the absent blood flow (Fig. 44.35; Arena et al., 2006). Reported cases
testis is twisted at the time of the examination. This includes the of testicular blood flow suggest that postnatal CDUS may be unreliable
degree of tenderness and cremasteric reflexes. Eaton et al. (2005) (Al-Salem, 2007; Cuervo et al., 2007; John et al., 2008; Yerkes et al.,
reported absent cremasteric reflex in 3 of 15 patients and reduced 2005). Ultrasound helps to differentiate tumors from torsion (Kaye
or absent intratesticular blood flow by CDUS in only 5 of 12 patients. et al., 2008a), but its reliability in this has also been challenged
A whirlpool sign or an abnormal boggy cord and pseudomass forma- (Al-Salem, 2007; Calonge et al., 2004).
tion below the twisted spermatic cord may also signify intermittent There is no consensus regarding the best treatment of perinatal
torsion (Munden et al., 2013). testicular torsion (Snyder and Diamond, 2010). One side advocates
There are challenges to confirming the diagnosis of intermittent elective exploration because of the unsalvageability in most cases,
torsion. The diagnosis requires a high index of suspicion unless the the rarity of metachronous torsion, and the increased anesthetic
testis is noted to untwist during an examination or an ultrasound risk (Brandt et al., 1992; Das and Singer, 1990; Kaye et al., 2008a;
study shows absent or decreased flow before and normal to increased Stone et al., 1995). Others advocate for immediate exploration to
flow after marked improvement of symptoms. Once the condition offer possible partial or complete testicular salvage (Al-Salem, 2007;
is confirmed or highly suspected, elective bilateral orchidopexy Cuervo et al., 2007; Sorensen et al., 2003) and point to cases of
is indicated to avert torsion and possible organ loss. Patients and unexpected contralateral torsion or atrophy found at exploration
parents should know that absolute confirmation of the diagnosis (Abraham et al., 2016; Al-Salem, 2007; Baglaj and Carachi, 2007;
may not be possible and that symptoms may persist postoperatively. John et al., 2008; Roth et al., 2011; Yerkes et al., 2005). Among 110
pediatric surgeons and urologists surveyed in the United Kingdom and
Extravaginal Spermatic Cord Torsion Ireland, few (10.9%) used Doppler ultrasound to guide management
(Perinatal Testicular Torsion) or to exclude tumor. Although most (74.5%) performed ipsilateral
orchiectomy and contralateral orchidopexy (71.9%), few operated
Perinatal spermatic cord torsion is a term applied to infants regardless emergently because a viable testis was seldom found (10%) and
of whether the event occurred prenatally (hours, days, weeks, months), bilateral torsion was even rarer (7 cases). Some (21.8%) avoided
during delivery, or postpartum. Torsion of the entire cord occurs contralateral orchidopexy because of concern for iatrogenic injury
before fixation of the tunica vaginalis and dartos within the scrotum (Rhodes et al., 2011). If torsion is suspected after a normal postnatal
(extravaginal). This event most commonly occurs well before delivery, scrotal examination, then prompt exploration should be performed
yielding a “vanishing” testis or a hemosiderin-containing nubbin as for intravaginal torsion. Some surgeons use a scrotal approach,
in the scrotum or less commonly in the inguinal canal. The testis whereas others advocate an inguinal approach to ligate a patent
that sustains loss of blood supply close to delivery is a hard, painless processus vaginalis and avoid the theoretic risk of trans-scrotal surgery
testis fixed to the overlying erythematous or dark scrotal skin with if a tumor is found.
or without edema. A coexisting hydrocele complicates the examina-
tion. The estimated incidence is 6.1 per 100,000 births (John et al., Torsion of the Appendix Testis and Epididymis
2008), and familial cases are reported (Castilla et al., 1975). Predispos-
ing factors such as high birth weight and/or difficult delivery are Appendage torsion is the most common cause of acute scrotum
suggested (John et al., 2008; Kaye et al., 2008a) but unconfirmed in prepubertal children. The appendix testis and appendix epididymis
by controlled studies. In a minority of cases, symptoms occur after are vestiges of embryologic development without known function.
a documented normal scrotal examination. Rarely, neonatal intra- The appendix testis (from the müllerian duct) and appendix epi-
vaginal torsion occurs or infarction occurs without torsion (John didymis (from the wolffian duct) are present in 76% to 83% and
et al., 2008). Bilateral torsion was noted in 5% and 22% in two 22% to 28% of testes, respectively (Dresner, 1973; Jacob and