Wein: Campbell-Walsh Urology, 9Th Ed

ACUTE SCROTUM A child or adolescent with acute scrotal pain, tenderness, or swelling should be looked on as an emergency situation requiring prompt evaluation, differential diagnosis, and potentially immediate surgical exploration. Adolescent boys do not always understand the potential significance of acute scrotal conditions, and evaluation in many cases is delayed. As a result, a subacute or even chronic scrotal condition may in certain situations merit prompt evaluation and intervention. Differential Diagnosis The list of differential diagnoses for an acute scrotum is extensive. In all instances it is imperative to rule out torsion of the spermatic cord, a clinical diagnosis requiring emergency surgical intervention ( Table 127-1 ).

Table 127-1 -- Differential Diagnosis of Acute/Subacute Scrotum Torsion of the spermatic cord Torsion of the appendix testis Torsion of the appendix epididymis Epididymitis Epididymo-orchitis Inguinal hernia Communicating hydrocele Hydrocele Hydrocele of the cord Trauma/insect bite Dermatologic lesions Inflammatory vasculitis (Henoch-Schönlein purpura) Idiopathic scrotal edema Tumor Spermatocele Varicocele Nonurogenital pathology (e.g., adductor tendinitis) Torsion of the Spermatic Cord (Intravaginal) Torsion of the spermatic cord is a true surgical emergency of the highest order. Irreversible ischemic injury to the testicular parenchyma may begin as soon as 4 hours after occlusion of the cord. Bartsch and colleagues (1980) demonstrated that although testes operated on less than 8 hours after the onset of symptoms of torsion retained normal testis size and showed just slight changes in testicular morphology, only 50% of men whose testes underwent detorsion less than 4 hours after symptoms began had normal semen analysis. It appears that the degree of torsion that occurs may have a significant influence on the potential for viability of the testis over time. The significance of this situation is magnified by the findings of Barada and coworkers (1989) , who reported that patients younger than 18 years were more prone to testicular loss after acute torsion because of a median delay in medical evaluation of 20 hours after the onset of scrotal pain, an indication of the need for improved awareness of the significance of scrotal pain in adolescents. Intravaginal torsion, or torsion of the cord within the space of the tunica vaginalis, may result from lack of normal fixation of an appropriate portion of the testis and epididymis to the fascial and muscular coverings that surround the cord within the scrotum. In effect, the normally segmental area of the free space between the parietal and visceral layers of the tunica vaginalis is expanded to surround the testis and epididymis and extends proximally up the cord for a variable distance. This creates an abnormally mobile testis that hangs freely within the tunica space (a “bell-clapper deformity”) ( Fig. 127-9 ).

Figure 127-9 Anomalies of the inguinal canal and scrotum that may result from anomalous closure of the processus vaginalis. (From Welch KA, Randolph JG, Ravitch MM, et al [eds]: Pediatric Surgery, vol 2, 4th ed. St Louis, Year Book, 1986, p 780.)

Although torsion of the cord does occur in prepubertal males, it appears that the added weight of the testis after puberty adds a physical dimension that may be more likely to allow the testis to twist on its vascular stalk. Torsion can occur in association with trauma or athletic activity, but in most cases spontaneous torsion of the cord is reported; in many cases the adolescent is awakened from sleep. It is thought that sudden contraction of the cremasteric muscle, which inserts onto the cord in a spiral configuration, is the inciting event in most cases and initiates a rotational effect on the testis as it is pulled upward. The cord may twist as many as several complete (360-degree) rotations. The classic manifestation of acute torsion of the spermatic cord is that of an acute onset of scrotal pain, but in some instances the onset appears to be more gradual, and in some boys the degree of pain is minimized. A large number of boys with acute scrotal pain give a history of previous episodes of severe, self-limited scrotal pain and swelling. It is likely that these incidents represent previous episodes of intermittent torsion of the cord with spontaneous detorsion. Nausea and vomiting may accompany acute torsion, and some boys have pain referred to the ipsilateral lower quadrant of the abdomen. Dysuria and other bladder symptoms are usually absent. The history is an important factor in the differential diagnosis of an acute scrotum, but the physical examination may perhaps be even more crucial in determining whether the diagnosis is torsion of the cord or otherwise (i.e., whether the patient does or does not require immediate surgical exploration). Inspection of the genitalia may prove helpful if the affected testis is riding high in the scrotum, perhaps indicating foreshortening of the spermatic cord as the result of twisting of the cord. In some cases, the affected testis has an abnormally transverse orientation, but in many cases, in particular when several hours have passed since onset, an acute hydrocele or massive scrotal edema obliterates all landmarks. The absence of a cremasteric reflex is a good indicator of torsion of the cord. Rabinowitz (1984) found 100% correlation between absence of a cremasteric reflex and the presence of torsion in 245 boys over a 7-year period. In some cases, assessment of this physical finding is difficult. When the patient is cooperative enough to allow examination of the affected hemiscrotum, effort should be made to assess anatomic landmarks to primarily look for an appreciation of normal structures in an attempt to identify a swollen and tender epididymis or a twisted appendix of the testis or epididymis. If torsion of the cord seems likely, manual detorsion should be attempted as part of the initial examination because the patient may be uncooperative with an extended examination because of discomfort. Classically, torsion of the cord occurs such that the anterior surface of each testis turns toward the midline as viewed from the patient's perspective ( Sparks, 1971 ). To accomplish detorsion of the cord, a rotational effort should be made in the opposite direction. Kiesling and associates (1984) described detorsion through two planes, with rotation in a caudal-to-cranial direction and simultaneous medial-to-lateral rotation. In actuality, the examiner should try to twist or “unscrew” the testis in one direction (usually outward, toward the thigh) and then in the opposite direction if the first attempt is unsuccessful. When detorsion is successful, the testis “flips” into a different rotation and pain relief may be almost instantaneous, with the cord appearing to lengthen and the testis dropping into the scrotum. If manual detorsion does not totally correct the rotation that has occurred, prompt exploration is indicated. However, when the patient becomes almost immediately comfortable, it can be assumed that blood flow to the testis has been restored, at least to a significant degree, yet this should not be used to defer exploration. When the diagnosis of torsion of the cord is suspected, prompt surgical exploration is warranted. Although adjunctive tests are commonly used to aid in the differential diagnosis of an acute scrotum, these tests are most appropriately performed when their purpose is to confirm the absence of torsion of the cord in cases in which surgical intervention is believed to be unnecessary. Doppler examination of the cord and testis to determine whether blood flow is present was once touted as a helpful diagnostic test, but false- positive and false-negative results have led most examiners to abandon this technique. Color Doppler ultrasound examination has become the adjunctive investigation of choice in many institutions for the evaluation of both acute and chronic scrotal conditions. Color Doppler studies allow an assessment of anatomy (e.g., presence of a hydrocele, swollen epididymis) while determining the presence or absence of blood flow to the testis. Baker and associates (2000) showed that in patients with acute scrotal swelling and an uncertain diagnosis, color Doppler examination had a diagnostic sensitivity of 88.9% and a specificity of 98.8%, with a 1% rate of false-positive results. Allen and Elder (1995) , however, reported five cases in which color Doppler interpretations were inconsistent with findings at surgery. It is clear that as in most clinical situations, ultrasound imaging is inherently operator dependent. Radionuclide imaging, originally the study of choice for assessment of an acute scrotum, is more limited because it allows evaluation of only testicular blood flow ( Kogan et al, 1979 ). Although Levy and associates (1983) found the study to have a positive predictive value of 75%, a sensitivity of 90%, and a specificity of 89%, a false impression of blood flow may result from hyperemia of the scrotal wall. In addition, children with small scrotal sacs and testes that are not dependent may be difficult to image with radionuclide techniques. Despite these shortcomings, Paltiel and colleagues (1998) found the efficacy of color Doppler imaging and radionuclide imaging to be equivocal in the diagnosis of torsion of the spermatic cord in boys with indeterminate clinical diagnoses. In most institutions, clinicians learn to place most trust in the imaging technique and in radiographers with the most interest, experience, and reliability in the differential diagnosis of an acute scrotum. When surgical exploration is elected, it should be performed promptly. A median raphe scrotal incision may be used to explore both sides, or a transverse incision following the skin creases may be placed in each hemiscrotum. The separate incisions are more appropriate for dartos pouch placement of the testes. The affected side should be examined first. After the skin has been incised, a dartos pouch may be created into which the testis can later be placed; then the tunica vaginalis is entered and the testis is examined. The cord should be detorsed to reestablish blood flow to the testis. Testes with marginal viability should be placed in warm sponges and re-examined after several minutes. A necrotic testis should be removed by dividing the cord into two or three segments and doubly ligating each segment with silk suture. Testes with marginal viability may be preserved, although there has been some concern about “sympathetic orchiopathy” in the contralateral testis secondary to circulating antibodies released from the injured testis ( Cosentino et al, 1982 ; Nagler and White, 1982 ). The validity of comparing animal studies with the human situation, however, has been called into question, and most urologists choose to preserve testes that seem marginally viable. If the testis is to be preserved, it should be placed in the dartos pouch with suture fixation. It has been shown experimentally that placing sutures through the tunica albuginea of the testis can produce local injury to the testis, and therefore fixation should be performed with fine, nonreactive, nonabsorbable sutures placed so that they avoid superficial blood vessels on the surface of the testis ( Bellinger et al, 1989 ). When torsion of the spermatic cord is found, exploration of the contralateral hemiscrotum must be carried out. In almost all cases a bell-clapper deformity is found. The contralateral testis must be fixed to prevent subsequent torsion. Intermittent Torsion of the Spermatic Cord A significant percentage of adolescents with acute torsion of the spermatic cord give a history of previous episodes of acute, self-limited scrotal pain that appear clinically to have been episodes of intermittent torsion with spontaneous detorsion ( Stillwell and Kramer, 1986 ). It is not uncommon to be asked to evaluate an adolescent for one or more episodes of acute scrotal pain that resolved spontaneously, was severe in nature, and in many cases was associated with vomiting or even a visit to the emergency room. At the time of evaluation the physical examination will be normal. If suspicion is strong that episodes of intermittent torsion and spontaneous detorsion have occurred, our experience has been that the finding of a bell-clapper deformity at exploration can be expected. Elective scrotal exploration should be undertaken, and scrotal fixation of both testes should be performed when bell- clapper deformities are identified ( Eaton et al, 2005 ). The purpose of prophylactic fixation of the testes is to prevent an episode of torsion that might lead to testicular atrophy. Torsion of the Testicular and Epididymal Appendages The appendix testis, a müllerian duct remnant, and the appendix epididymis, a wolffian remnant, are prone to torsion in adolescence, presumably as a result of hormonal stimulation, which increases their mass and makes them more likely to twist on the small vascular pedicle on which they are based. The symptoms associated with torsion of an appendage are extremely variable, from an insidious onset of scrotal discomfort to an acute condition identical to that seen with torsion of the cord. In this sense, torsion of an appendage and epididymitis might be difficult to distinguish clinically. When seen at an early stage, an adolescent with torsion of an appendage may have localized tenderness of the upper pole of the testis or epididymis, where a tender nodule may be palpated. In some instances, the infarcted appendage is visible through the skin as a “blue dot sign” ( Dresner, 1973 ). In cases in which the inflammatory changes are more significant, scrotal wall edema and erythema may be severe. The cremasteric reflex should be present, and the testis should be mobile. Radionuclide scans or color Doppler studies may show normal or increased flow, and ultrasound imaging may delineate the swollen appendage. Though often interpreted as “epididymitis,” it is not likely to be bacterial in origin. When the diagnosis of torsion of an appendage is confirmed clinically or by imaging, nonoperative management allows most cases to resolve spontaneously. Limitation of activity, administration of nonsteroidal anti-inflammatory agents, and observation permit most symptoms to subside as the acute changes of ischemic necrosis resolve. In an occasional clinical situation, acute exploration is performed because of suspicion of torsion of the cord, or delayed exploration is performed because of failure of spontaneous resolution of the inflammatory changes and discomfort. Simple excision of the twisted appendage in these cases is therapeutic. Epididymitis Inflammation or infection of the epididymis is an important part of the differential diagnosis of an acute scrotum. Epididymitis is reported to be a rare clinical diagnosis in the pediatric age group. Siegel and associates (1987) reported fewer than five cases per year at a major pediatric hospital, most documented at the time of scrotal exploration. Likitnukul and colleagues (1987) reported 35 cases in a 20-year retrospective review. The most common clinical symptoms at the initial encounter are scrotal swelling, erythema, and pain; these symptoms are found equally in boys subsequently determined to have anatomic anomalies of the urogenital system and in boys with normal anatomy. In fact, it appears that in many cases the diagnosis of epididymitis is a “wastebasket” diagnosis for patients without torsion of the spermatic cord who have swollen, painful scrotal contents. It is possible that some cases of appendage torsion, for example, are misdiagnosed as epididymitis. This results, in part, from the varied severity of symptoms of epididymitis: from localized epididymal tenderness, to tenderness and swelling of the entire epididymis, to a massively inflamed hemiscrotum with absence of definable landmarks and increased blood flow on scrotal scintigraphy or color Doppler study. Epididymitis is classically described as an indolent process, in contrast to the rather acute onset of torsion of the spermatic cord. In adolescents, however, clinical distinction between the two entities is frequently less easily categorized. The presence of dysuria and fever is more common in the epididymitis group, although many boys with clinical epididymitis have neither. A past history of urinary tract infections, urethritis, urethral discharge, sexual activity, urethral catheterization, or urinary tract surgery may indicate a higher likelihood for epididymitis. Epididymitis has been associated with Henoch-Schönlein purpura, presumably on a systemic inflammatory basis, and has been noted in boys treated with the antiarrhythmic agent amiodarone ( Hutcheson et al, 1998 ). Dysfunctional voiding has also been implicated as a potential cause of epididymitis, presumably as a result of urinary reflux into the ejaculatory duct ( Bukowski et al, 1995a ). Physical examination may reveal localized epididymal tenderness, a swollen and tender epididymis, or a massively swollen hemiscrotum with the absence of landmarks. The cremasteric reflex should be present in patients with epididymitis, and its absence is highly suggestive of torsion of the spermatic cord; however, it may be difficult to demonstrate in the acutely swollen scrotum of those with epididymitis ( Rabinowitz, 1984 ). The presence of pyuria, bacteriuria, or a positive urine culture is important evidence that epididymitis should be high on the list of differential diagnoses, although urine cultures may be sterile in 40% to 90% of patients. Normal urinalysis results do not rule out epididymitis. The most common finding in this age group is gram-negative bacteria ( Likitnukul et al, 1987 ; Siegel and Snyder, 1987 ). Our experience, in fact, would demonstrate that most boys with a clinical diagnosis of epididymitis have sterile urine ( Gislason et al, 1980 ; Likitnukul et al, 1987 ; Siegel and Snyder, 1987 ). Conversely, there is a low incidence of clinical epididymitis in patients who require nonsterile intermittent catheterization. Thirumavalavan and Ransley (1992) found the incidence in this group to be only about one episode of epididymitis per 10 patient-years. Bennett and colleagues (1998) documented a relationship between epididymitis in boys with infected urine and the presence of an uncircumcised penis. Scrotal imaging may be an important part of making the diagnosis of epididymitis and thus avoiding unnecessary surgery, especially in a patient with a massively swollen scrotum. Color flow Doppler and radionuclide imaging reveal increased blood flow; ultrasound may demonstrate a swollen testis or testis and epididymis, frequently with the presence of a hydrocele, which may contain echogenic debris when bacterial infection is present. In current practice, the trend toward imaging of an acute scrotum has made the finding of epididymitis at scrotal exploration less common. Radiographic imaging of the urinary tract is commonly performed during follow-up of boys in whom epididymitis has been diagnosed. Likitnukul and coworkers (1987) found radiographic abnormalities in four of five such boys with positive urine cultures. Abnormalities included urethral stricture, ureteral ectopia into the seminal vesicle, and reflux of contrast into the seminal vesicles in two patients. In Siegel and Snyder's series (1987) , 47% of prepubertal boys with epididymitis were found to have radiographic abnormalities, including ectopia of the vasa or ureter and urethral anomalies, all having the common end result of predisposing the genital duct system to reflux of urine. The younger the child with epididymitis, the more likely it is that a urinary tract infection, radiographic anomaly, or both will be found ( Merlini, 1998 ). Because the majority of boys with epididymitis have sterile urine and apparently radiographically normal urinary tracts, it would seem most appropriate to reserve renal and bladder ultrasonography and voiding cystourethrography for prepubertal boys with positive urine cultures. When epididymitis is diagnosed on color Doppler study, it is expeditious to proceed with imaging of the bladder and the upper urinary tract at the same sitting. Epididymitis in adolescents should be treated aggressively, whether in an early or advanced stage. Because all boys with acute scrotal swelling of any cause will clinically worsen when allowed to resume normal activities, limitation of activity, especially that of a strenuous nature, should be enforced. In many cases, bed rest for 1 to 3 days results in a less protracted clinical course of pain and scrotal swelling. Following this period with a more extended course of relative restriction (sports, gymnastics) continues to promote resolution of scrotal swelling and discomfort. Scrotal elevation, the use of an athletic supporter, and the application of cold or warmth to the area may prove beneficial in reducing discomfort. Prompt and aggressive parenteral antibiotic therapy should be instituted when urinary tract infection is documented or suspected. Urethral instrumentation should be avoided if at all possible. After the acute episode has subsided, prophylactic antibiotic therapy should be continued until a voiding cystourethrogram is performed. In boys with sterile urine, the same limitations in physical activity should be imposed. Oral nonsteroidal anti-inflammatory agents may promote resolution of the inflammation. Miscellaneous Causes of Acute Scrotal Swelling Scrotal swelling, erythema, or pain may be initiated by lesions primary to the scrotal contents, the scrotal wall or skin, or the inguinal canal. On occasion, pain thought to originate in the scrotum is found to have an extrascrotal origin. Acute idiopathic scrotal edema is a self-limited process of unknown cause that is not usually associated with scrotal erythema ( Qvist, 1956 ). Fever is not present, and scrotal tenderness is usually minimal, but pruritus may be significant. Although the process is considered to be idiopathic, allergic or chemical dermatitis, insect bites, trauma, and other known potential causes of scrotal inflammation may be responsible but undiagnosed. Examination should include a complete assessment of the perineum and perianal region to rule out scrotal edema secondary to a contiguous process (e.g., perirectal abscess). In most cases, the scrotal wall is thickened but the testes can be palpated. When in doubt about the cause of scrotal edema, ultrasound evaluation with color flow Doppler should be performed. No therapy is indicated. Henoch-Schönlein purpura is a systemic vasculitis that can cause scrotal swelling secondary to involvement of the testis, epididymis, or both ( Clark and Kramer, 1986 ). The cause of vasculitis is unknown. The purpura is a nonthrombocytopenic process that may be manifested as abdominal and joint pain, nephritis and hematuria, and skin lesions. Scrotal involvement is merely part of the systemic manifestation; it is seen in up to 35% of patients. Scrotal findings are generally diffuse and consist of swelling, erythema, and tenderness. Urinalysis may demonstrate hematuria and proteinuria. Color Doppler study or scintigraphy shows increased blood flow. Observation of the scrotal findings is a part of managing the systemic symptom complex, which is usually a self-limited process but may require steroid therapy. Perinatal Torsion of the Spermatic Cord Torsion of the spermatic cord may occur prenatally (months, weeks, or days before birth or during the process of labor) or in the immediate postnatal period. Although the term perinatal torsion has been used to group both prenatal and postnatal torsion into a single clinical diagnosis, they may in fact represent distinctly different pathophysiologic processes that should be approached very differently. The major points of contention that arise when perinatal torsion is discussed are the utility of prompt surgical exploration and the need for contralateral scrotal exploration and fixation of the testis. Prenatal (in utero) torsion is typified by the finding at delivery of a hard, nontender testis fixed to the overlying scrotal skin. The skin is commonly discolored by the underlying hemorrhagic necrosis. This clinical scenario is pathognomonic of a resolving infarction process, the acute phase of which occurred before delivery. Pathologic examination of testes that have undergone prenatal torsion reveals that in most cases, extravaginal torsion (torsion of the cord and its tunics) has occurred. Duckett (1991) argued that the incidence of prenatal torsion is probably much higher than usually quoted. He postulated that the blind-ending spermatic cord (“vanishing testis”) discovered on exploration for a nonpalpable testis is in many cases the result of antenatal torsion. This thought is corroborated by the common finding of hemosiderin in the pathologic examination of the distal sections of blind-ending spermatic cords removed surgically. Prenatal torsion may merely be a late gestational representation of the same process that if it had occurred earlier, would have produced a blind-ending spermatic cord ( Duckett, 1991 ). Classic teaching has held that testes found to be hard, nontender, and fixed to the skin at birth do not merit surgical exploration because of the delayed nature of the pathologic process at the time of initial evaluation. In fact, the reported salvage rate of testes presumed to have undergone torsion before birth is negligible. Despite prompt exploration, Brandt found no salvageable testes in 25 explorations, a finding confirmed by others ( Brandt et al, 1992 ; Stone et al, 1995 ). However, controversy has arisen regarding the need for prompt exploration of the contralateral testis. Contralateral scrotal exploration traditionally has not been recommended in cases of prenatal torsion because extravaginal torsion is not associated with the testicular fixation defect (bell- clapper deformity) that is recognized as the cause of intravaginal torsion. However, reports of asynchronous perinatal torsion have made the practice of avoiding prompt surgical exploration of the contralateral testis controversial ( Olguner et al, 2000 ). The postnatal manifestation of acute scrotal swelling may present a problem for urologists who are unsure of whether the process is truly a prenatal or a postnatal event. Postnatal torsion is usually associated with swelling and tenderness of the scrotum. Fixation of the skin is not typically present. Burge (1987) described 30 infants with acute scrotal swelling, 18 of whom underwent prompt surgical exploration. Ten were found to have extravaginal torsion, 3 had intravaginal torsion, 1 had torsion of an appendix testis, 1 had torsion of an undescended testis, and 1 had a normal testis. Pinto and Noe (1987) described salvage of 2 of 10 testes explored within 6 hours of discovery. The diagnosis may be aided by color flow Doppler examination, even in small neonates ( Stone et al, 1995 ). Prompt exploration of suspected postnatal torsion of the spermatic cord is indicated (in conjunction with exploration of the contralateral testis) when the patient's general condition and anesthetic considerations allow for a safe procedure. The 17% incidence of bell-clapper deformity and the 20% incidence of salvage of a solitary contralateral testis (prevention of anorchia) must be weighed against the risk associated with general anesthesia in neonates. Tiret and colleagues (1988) reported the incidence of major anesthetic-related complications in children older than 1 year to be 0.5 per 1000, and in those younger than 1 year it was 0.7 per 1000. Mortality occurred in 1 in 40,000 anesthesia procedures. Others showed that the incidence of intraoperative and postoperative complications was greatest in infants younger than 1 month ( Cohen et al, 1990 ). Clearly, the decision to subject a neonate with suspected torsion of the spermatic cord to surgery should be carefully considered by weighing the clinical assessment of the acuity of the torsion episode, the risk to the contralateral testis, and the risk related to general anesthesia. The decision may be even more difficult when the neonate is located at a distance from a tertiary referral center that can offer skilled pediatric anesthesia because both the risk associated with neonatal transport and the time lost in transport may be critical if acute postnatal torsion is to be salvaged. Clearly, if the cause of scrotal swelling appears to be related to an acute postnatal event, all efforts should be made to pursue prompt surgical intervention. Exploration, when elected, should be carried out through an inguinal incision to allow for the most efficacious treatment of other potential or unexpected causes of scrotal swelling. If torsion is confirmed, contralateral scrotal exploration with testicular fixation should be carried out ( Bellinger, 1985 ). The most effective and safest form of testicular fixation involves dartos pouch placement ( Bellinger et al, 1989 ). Email to Colleague Print Version Copyright © 2007 Elsevier Inc. All rights reserved. - www.mdconsult.com