Evidence-Based Diagnosis And Treatment Of Torsion Of The Spermatic Cord In The Pediatric Patient

Transcription

1 Evidence-Based Diagnosis And Treatment Of Torsion Of The Spermatic Cord In The Pediatric Patient Abstract The incidence of acute torsion of the spermatic cord (TOSC) has been estimated to be 4.5 cases per 100,000 population. 1 Others have cited an annual incidence of 1 in 4000 males under While not especially common in the emergency department (ED), these cases are important to the patient, the clinician, and the consultants who might be needed. Sorting out the etiology can be vexing. Doing so frequently involves not only examination but also imaging and consultation with surgery or urology colleagues. When faced with an acutely swollen and painful scrotum, the surgeon must decide quickly whether or not to explore the scrotum, and if a testicular torsion is found, choose between testicular salvage and removal. Both decisions can have consequences for the patient. The outcome for the patient is as dependent on the time elapsed from the onset of the attack, as it is on the decisions of the surgeon. Case Presentation A 14-year-old, previously healthy male presents to the ED on a typically busy Friday evening complaining of scrotal pain that started 2 days ago. His mom is visibly upset and states that the patient was taken to his Authors October 2011 Volume 8, Number 10 Martin I. Herman, MD, FAAP, FACEP Professor of Pediatrics, Attending Physician, Emergency Medicine Department, Sacred Heart Children s Hospital, Pensacola, FL Jonathan Jacobs, MD, FAAP Pediatric Emergency Medicine Fellow, UT College of Medicine, Memphis, TN Peer Reviewers Amy Doolan Roy, MD Pediatric Emergency Medicine, Elliot Hospital, Manchester, NH Jatinder Singh, MD, FAAEM, FACEP Attending Physician, Department of Emergency Medicine, Nassau University Medical Center, East Meadow, NY CME Objectives Upon completion of this article, you should be able to: 1. Identify testicular torsion in the pediatric patient. 2. Make an evidence-based decision regarding whether or not to explore the scrotum. 3. Choose between testicular salvage or removal if a testicular torsion is found. Date of original release: October 1, 2011 Date of most recent review: September 10, 2011 Termination date: October 1, 2014 Medium: Print and Online Method of participation: Print or online answer form and evaluation Prior to beginning this activity, see Physician CME Information on the back page. AAP Sponsor Martin I. Herman, MD, FAAP, FACEP Professor of Pediatrics, Attending Physician, Emergency Medicine Department, Sacred Heart Children s Hospital, Pensacola, FL Editorial Board Jeffrey R. Avner, MD, FAAP Professor of Clinical Pediatrics and Chief of Pediatric Emergency Medicine, Albert Einstein College of Medicine, Children s Hospital at Montefiore, Bronx, NY T. Kent Denmark, MD, FAAP, FACEP Medical Director, Medical Simulation Center; Associate Professor of Emergency Medicine and Pediatrics, Loma Linda University Medical Center and Children s Hospital, Loma Linda, CA Michael J. Gerardi, MD, FAAP, FACEP Clinical Assistant Professor of Medicine, University of Medicine and Dentistry of New Jersey; Director, Pediatric Emergency Medicine, Children s Medical Center, Atlantic Health System; Department of Emergency Medicine, Morristown Memorial Hospital, Morristown, NJ Ran D. Goldman, MD Associate Professor, Department of Pediatrics, University of Toronto; Division of Pediatric Emergency Medicine and Clinical Pharmacology and Toxicology, The Hospital for Sick Children, Toronto, ON Mark A. Hostetler, MD, MPH Clinical Professor of Pediatrics and Emergency Medicine, University of Arizona Children s Hospital Division of Emergency Medicine, Phoenix, AZ Madeline Matar Joseph, MD, FAAP, FACEP Associate Professor of Emergency Medicine and Pediatrics, Assistant Chair for Pediatrics - Emergency Medicine Department, Chief - Pediatric Emergency Medicine Division, Medical Director - Pediatric Emergency Department, University of Florida Health Science Center Jacksonville, Jacksonville, FL Alson S. Inaba, MD, FAAP, PALS-NF Pediatric Emergency Medicine Attending Physician, Kapiolani Medical Center for Women & Children; Associate Professor of Pediatrics, University of Hawaii John A. Burns School of Medicine, Honolulu, HI; Pediatric Advanced Life Support National Faculty Representative, American Heart Association, Hawaii and Pacific Island Region Andy Jagoda, MD, FACEP Professor and Chair, Department of Emergency Medicine, Mount Sinai School of Medicine; Medical Director, Mount Sinai Hospital, New York, NY Tommy Y. Kim, MD, FAAP, FACEP Assistant Professor of Emergency Medicine and Pediatrics, Loma Linda Medical Center and Children s Hospital, Loma Linda, CA Brent R. King, MD, FACEP, FAAP, FAAEM Professor of Emergency Medicine and Pediatrics; Chairman, Department of Emergency Medicine, The University of Texas Houston Medical School, Houston, TX Robert Luten, MD Professor, Pediatrics and Emergency Medicine, University of Florida, Jacksonville, FL Ghazala Q. Sharieff, MD, FAAP, FACEP, FAAEM Associate Clinical Professor, Children s Hospital and Health Center/University of California, San Diego; Director of Pediatric Emergency Medicine, California Emergency Physicians, San Diego, CA Gary R. Strange, MD, MA, FACEP Professor and Head, Department of Emergency Medicine, University of Illinois, Chicago, IL Christopher Strother, MD Assistant Professor,Director, Undergraduate and Emergency Simulation, Mount Sinai School of Medicine, New York, NY Adam Vella, MD, FAAP Assistant Professor of Emergency Medicine, Director Of Pediatric Emergency Medicine, Mount Sinai School of Medicine, New York, NY Michael Witt, MD, MPH, FACEP, FAAP Medical Director, Pediatric Emergency Medicine, Elliot Hospital Manchester, NH Research Editor Lana Friedman, MD Fellow, Pediatric Emergency Medicine, Mount Sinai School of Medicine, New York, NY Accreditation: EB Medicine is accredited by the ACCME to provide continuing medical education for physicians. Faculty Disclosure: Dr. Herman, Dr. Jacobs, Dr. Roy, Dr. Singh, and their related parties report no significant financial interest or other relationship with the manufacturer(s) of any commercial product(s) discussed in this educational presentation. Commercial Support: This issue of Pediatric Emergency Medicine Practice did not receive any commercial support.

2 primary pediatrician 2 days ago for a separate complaint, but he denied having pain despite being asked if anything else was bothering him. When questioned, the patient states he did have scrotal pain at that time, but he was too embarrassed to say anything. Further history reveals that the pain was acute in onset, sharp, left-sided, and has progressively gotten worse. He denies dysuria or other urinary complaints, and there have been no fever or chills. The patient denies suffering any trauma. He admits to nausea but has not vomited. The remainder of the review of systems is negative. The physical examination is remarkable for a tender, swollen left hemiscrotum. The left testis is high-riding compared to the right, and the patient fails to display a cremasteric reflex on the affected side. The right testis is nontender, and the remainder of the examination is noncontributory. You inform the patient s mom of the likely diagnosis and of your plan to obtain an ultrasound and consult urology. As you walk down the hall, your mind is racing. Is this testicular torsion? What else could it be? How will you determine the diagnosis definitively? Is there something you should be doing right now? You have heard some people say that an attempt to manually detorse is indicated. Is that true? Can it make matters worse? If you do decide to do it, which way should you try to flip the testis? Will ultrasound guidance help you choose the best direction? You also realize the clock is ticking. How long has the problem been going on? When will the function of the organ become irreparably damaged? Is there a time limit on how long the testis itself can survive a torsion of its blood supply? What diagnostic studies should you order? Should you notify the urology Table Of Contents Abstract...1 Case Presentation...1 Introduction...2 Critical Appraisal Of The Literature...2 Epidemiology...3 Developmental Anatomy...3 Etiology...3 Presentation...4 Differential Diagnosis...5 Prehospital Care...6 Emergency Department Evaluation...7 Laboratory Studies...8 Imaging Studies...9 Treatment...10 Clinical Pathway: Treatment Of The Acute Scrotum...11 Outcomes...13 Medicolegal Concerns...15 Risk Management Pitfalls In The Emergency Treatment Of Torsion Of The Spermatic Cord Summary...16 Case Conclusion...16 References...17 CME Questions...18 service now or wait till you know the diagnosis? Are there any pharmaceuticals you should administer to minimize the injury? If this teen had spoken up sooner, would the outcome have been affected? What should you do first? Introduction Acute scrotal emergencies are not the most frequent crisis faced in an emergency department (ED). It is said that an acute scrotum occurs with 1/20th of the frequency of an acute abdomen. 3 Cincinnati Children s Hospital found that in a 2 year period, there were 238 acute scrotal emergencies which represented only 0.13 % of their ED patients. 4 The incidence of acute TOSC has been estimated to be 4.5 cases per 100,000 population. 1 Others have cited an annual incidence of 1:4000 males under 25, with torsion presenting most often during 2 time periods of a males life. The first peak in incidence involves those under a year of age, and the second peak occurs around age 13. 2,5 These cases are important to the patient, the clinician, and the consultants who might be needed. Sorting out the etiology can be vexing. Doing so frequently involves not only examination but also imaging and consultation with surgery or urology colleagues. When faced with an acutely swollen and painful scrotum, the surgeon must decide quickly whether or not to explore the scrotum. If a testicular torsion is found, the surgeon must choose between testicular salvage and removal. Both decisions can have consequences for the patient. This month s issue of Pediatric Emergency Medicine Practice will delve into the problem of the male with a possible TOSC. The authors examine existing literature to develop a strong strategy for clinicians that explains what to do and when to do it in the diagnosis and treatment of TOSC. Critical Appraisal Of The Literature Ovid MEDLINE and PubMed were searched for literature published in core English-language clinical journals from on the subject of human subjects aged 1 month to 18 years with TOSC. Search terms included torsion, testicular cord, and spermatic cord. Results containing patients with neonatal testicular torsion were excluded. This search resulted in 640 articles. Modifiers were added in order to filter the search. Diagnosis resulted in 160 articles, treatment in 119, incidence in 56, ultrasound in 65, risk factors in 19, and outcomes in 14. These lists were reviewed for appropriate articles as were the reference pages of thorough review articles and other papers related to testicular torsion. Pediatric Emergency Medicine Practice October 2011

3 Epidemiology As previously noted, TOSC is estimated to occur in 4.5 out of every 100,000 men in America. More specifically, the relative risk in the highest risk group, men between 10 and 19, was an estimated 8.6 cases per 100,000 men. 1 According to Williamson, the annual incidence of TOSC in Bristol was about 1 in 4000 males between the ages of 1 and 25, with almost 1 in every 160 developing a torsion by their 25 th birthday. 6 A retrospective analysis performed in Israel put the relative incidence of torsions of the spermatic cord at 3.25% of all scrotal emergencies even though others have estimated that torsions may represent between 17% and 72% of all acute scrotal emergencies. The authors point out that this difference is probably due to the selection bias of other studies because most other studies identifying > 17% of cases as torsions came from urology and surgery clinics who, by their very nature, would not reflect the same patient mix as an ED population. 7 Torsions of the spermatic cord are a rare occurrence as they seem to occur with about 1/20 th of the frequency of acute abdominal pain. 3 Developmental Anatomy Three types of testicular torsions have been described, extra-vaginal, intravaginal, and torsion between the testis and the epididymis. The term extravaginal torsion refers to a twist of the spermatic cord occurring outside the tunica vaginalis. These are almost exclusively found in neonates, many times occurring in utero. Patients with extravaginal torsion may present with a hard, dark mass in the scrotum or with an empty scrotum due to an absent gonad and a vas deferens that ends abruptly. Intravaginal torsions are twists of the spermatic cord that take place within the tunica vaginalis. These are made possible because the tunica vaginalis attaches high on the spermatic cord. This type of attachment is referred to as a bell clapper deformity and is the most common deformity encountered in cases of torsion. In cases of an acute scrotum, when scrotal exploration is performed, a bell clapper deformity may be found in up to 50% of cases, which would mandate orchiopexy of the surviving gonad. 8 Normally, the epididymis drapes over the top of the testis and then extends inferiorly. It is shaped like a cone, being larger superiorly and tapering as it descends, eventually merging to become the vas deferens. The epididymis is fixed posteriorly to the scrotal wall, along with the posterior aspect of the testis, by an area that is not usually covered by the tunica vaginalis. The tunica vaginalis is the lower portion of the processus vaginalis, which forms as an extravasation of the peritoneum prior to the descent of the fetal testis. Once the testis drops from the abdomen to the scrotum, the proximal portion of the tunica regresses, leaving a sac that partially surrounds the testis. 9 Imagine the testis is an egg on a string (the spermatic cord) resting in a very soft water balloon (the tunica vaginalis). The softer the balloon, the deeper into it the testis can penetrate, which results in the edges of the balloon (or in this case, the tunica vaginalis) reaching higher on the stalk. If it envelopes the testis completely, you have the so called bell clapper deformity. (See Figure 1, page 4, and Figure 2, page 5.) If it does not envelope the testis, a section of the testis and epididymis is uncovered and can become fixed to the posterior wall of the scrotum. This balloon (tunica vaginalis) has a parietal and a visceral layer, and if the visceral surface covers the testis (except the posterior region), the risk for an intravaginal torsion to take place is great. Etiology What causes the spermatic cord to twist? Torsions of the spermatic cord have occurred during normal daytime activities, shortly after exercise (especially bicycle riding), and while at rest or asleep. 10 Trauma is not usually associated with torsions; however, it may play a role. 11 Torsions have also been noted to occur more commonly at times when environmental temperatures are colder. 12 In 1982, Shukla et al reported their experience with patients in Dublin. A retrospective study of 46 patients was performed. They concluded that acute torsions of the spermatic cord were more likely when the temperature was below 2 C (35.6 F). 12 Almost immediately after this report, another researcher tried to confirm the findings. Preshaw s retrospective review of all cases (272) of TOSC treated in Calgary, Alberta between 1966 and 1982 was performed. The author looked at the month of the year when the patient was discharged. Mean daily temperature for the month was also charted. No significant difference was found for the coldest months of the year or any 6 month period, leading the author to conclude that there was not an association between the occurrence of torsion and the season of the year. 13 However, even in that paper, Preshaw points to at least 3 other papers that present a case for the role of temperature in the occurrence of a torsion and was unable to explain the difference in his results and those of the others. More recently, a study performed at the Schneider Children s Hospital in New York showed a favorable association between lower temperatures and the occurrence of TOSC. Srinivasan et al did a retrospective study involving all patients with testicular pain presenting to their facility between January 1999 and December Neonatal torsions and torsions of the appendix testis were excluded. These researchers recorded the month, season, daily temperature, humidity, and atmospheric pressure. The patients were stratified October Pediatric Emergency Medicine Practice 2011

4 into 2 groups, those presenting when the temperature was > 15 C (59 F) and those presenting when the temperature was < 15 C (59 F). Interestingly, no significant difference in occurrence was found when they examined just the month of the year that the patient presented, nor the individual seasons. However, when they reviewed the data, they noted that 67.2% of the cases presented in either the winter or spring when compared to the fall and summer. Furthermore, 81% of the cases presented when the temperature was < 15 C (59 F). 14 A Nigerian study, published in 2004, adds additional support for the hypothesis that cold weather has some impact on the incidence of TOSC. In that study (an 18-year collection of data from a teaching hospital in Nigeria), 178 men had been admitted with acute scrotum. Acute TOSC accounted for half the cases, and it was more common during the harmattan season, a time of cold air and low relative humidity. 15 Similar findings from Canada, Ireland, and Japan suggest that cold air can stimulate exaggerated contraction of the cremasteric muscle. Retraction of the testis may also be associated with exercise, coitus, or a sudden fright. 16 The higher incidence in the winter and spring should serve as reminder to remain vigilant for the presence of TOSC when seeing male patients with abdominal pain, nausea, vomiting, and scrotal complaints during colder temperatures. Torsions have also been linked with a surge in testosterone levels that are associated with elevation and rotation of the gonads. 8 Longo proposed the following sequence or explanation for when and why TOSC occurs. The explanation starts with males that are predisposed to torsion because of the bell clapper deformity. Next, Longo points to Masters Figure 1. Bell Clapper Deformity Testicle in horizontal plane Twisted spermatic cord Spermatic cord Reproduced with permission of The McGraw-Hill Companies from: Knoop KJ, Stack AB, eds. Atlas of Emergency Medicine. New York: McGraw-Hill; 1997:211. Figure 8.2. and Johnson s observations that during the sexual excitement phase, the right testis is elevated, and during the plateau phase, the testes rotate anteriorly 90 with full elevation and rotation of the left cord required for orgasm. Longo also notes that testosterone levels are highest in the early mornings (usually 6-9am), and newborn testosterone levels approach those of the adolescent male. When each of these factors are taken into consideration, it is possible that, in some males, rotation beyond 90 takes place and results in a full TOSC. 17 Interestingly, there is a case report of a male that presented several times to an ED with acute scrotal pain after engaging in intense but non-coital and non-ejaculatory sexual play with his girlfriend. 18 This case report, though an isolated case in the literature, adds some support to this theory put forth by Longo. Another case report, published in 1991, highlights the risk of testicular torsion in infants after receiving human chorionic gonadotropin for treatment of cryptorchidism. 19 This entire series of cases and events corroborates with other observations that many cases of TOSC happen after puberty and often in the early morning hours. 8 Presentation Scrotal pain and swelling are the typical complaints for patients with some type of scrotal emergency. The intensity of the pain may vary and seems to influence how soon the patient presents for help. Patients with intense pain are likely to present early, and it has been conjectured that the early presenters, who may have the worst pain, are patients with an acute TOSC. At least one study found that 75% of males who present to an ED within 6 hours of the onset of pain have TOSC. 20 The incidence of acute torsions is bimodal. That is, it occurs with relative frequency in the first year of life and again around age An acute scrotum in the first year of life, which has a significant chance of being associated with TOSC, would be more often associated with an extra-vaginal type of torsion. Unfortunately, these usually present long after the ischemic event starts, and the testis is often not able to be saved. 8 The presentation may be crying, fussiness, swollen scrotum, scrotal erythema, or simply a dark knot or mass in the scrotum. Unless the torsion occurred postnatally and the patient is brought in promptly, there is little hope of finding a viable testis. The second peak occurs in the year age range, median age being 14 years of age. 21 These boys present with a painful swollen scrotum. There may be nausea or vomiting. The authors have observed that their gait is abnormal in that they will walk (if allowed to) with a wide base trying to guard the scrotum from any contact with their thigh as it changes position in the swing phase of the gait. Patients may guard their scrotum and prefer to lie Pediatric Emergency Medicine Practice October 2011

5 still in a low-to-medium Fowler s posture, with their knees up and legs open. When asked about prior episodes, some patients may relate that they have had other self-limited episodes of pain. Each episode is similar to the one currently being evaluated (acute in onset, intense in quality, and possibly associated with swelling, etc). Intermittent torsions have been reported and are well recognized as warnings that the scrotal contents are not stable. Scrotal exploration and orchiopexy has been advocated for these patients. 3,22 Differential Diagnosis Knight and Vassey found that 96% of acute scrotal emergencies involved either a TOSC, a torsion of the appendix testis (TAT), or an acute epididymitis (AE). 1 Observers have found the mix of these entities to vary from 14% to 38% for TOSC, 14% to 46% for TAT, and 31% to 71% for AE. 3,4,21 However, all have reported that the 3 entities do indeed explain the majority of the patients pain. There are other causes, but they are typically easily discerned and diagnosed by a careful history and examination. The differential diagnosis for a patient pre- senting to the ED with scrotal pain and/or scrotal swelling includes TOSC, epididymitis/orchitis, TAT, trauma, hydrocele, varicocele, incarcerated inguinal hernia, Henoch-Schonlein purpura, testicular cancer, and idiopathic scrotal edema. For an extensive list of potential diagnoses, see Table 1, page 6. In addition, the patient can experience referred pain that will broaden the differential further to include appendicitis, nephrolithiasis, and other urinary and/or intra-abdominal pathology. Of this extensive list, 3 diagnoses are consistently most common: TOSC, epididymitis, and TAT. 4,20,21 A recent 19-year chart review from a single center found that, of 388 consecutive patients under the age of 17 presenting with an acute scrotum undergoing surgical exploration, 26% had TOSC, 45% had TAT, and 10% had epididymitis. 20 By contrast, Lewis et al found that 16% had TOSC, 46% had TAT, and 35% had epididymitis. 4 The differences in the literature reflect a difference in the approach to a patient with an acute scrotum. In these examples, the former surgically explored every patient while the latter used clinical examination and imaging in addition to surgery to make the diagnosis. While percentage numbers vary, these entities consistently represent the top 3 diagnoses of Figure 2. The Acute Scrotum In Children Reproduced with permission from: Knight P, Vassey L. The Diagnosis and Treatment of the Acute Scrotum in Children and Adolescents. Ann Surg. 1984;2005(5): October Pediatric Emergency Medicine Practice 2011

6 acute scrotal pain. More importantly, they are usually easily distinguished from the broader differential in terms of history, review of systems, and physical examination but can be difficult to distinguish from each other based on that information alone. While the presentations of TOSC, TAT, and epididymitis may be similar and hard to differentiate, the approach, treatment, prognosis, and sequelae differ immensely. Delay in reaching an accurate diagnosis may lead to testicular loss, while aggressive surgical exploration without an accurate diagnosis may lead to unnecessary risk and complications. In years prior to the advancements in and availability of different imaging techniques, this dilemma resulted in the surgical exploration of all patients that presented with an acute scrotum. Some investigators continue to promote this approach today. 23 However, most now support an approach that involves imaging unless the onset is acute (< 6 hours) and the history and physical examination clearly support TOSC. It is important to clinically differentiate TOSC, Table 1. Differential Diagnosis of Testicular Torsion Scrotal: Tunics: 1. Idiopathic edema of the scrotum 1. Rupture of tunica albuginea 2. Traumatic fat necrosis 2. Cremasteric spasms and 3. Cellulitis testodynia 4. Acute scrotal gangrene 3. Rupture of hydrocoele 5. Fulminating intertrigo 4. Torsion of spermatocele 6. Scrotal abcess 5. Acute hydrocele 6. Pyocele Testicular: Infective 1. Epididymous-orchitis 2. Mumps orchitis 3. Salmonella enteridis orchitis Neoplasms 1. Bleeding into testicular tumor 2. Myofibroblastic pseudotumor 3. Torsion of tumor of the testis Miscellaneous 1. Testicular microlithiasis 2. Polyorchidopathia 3. Ischemic necrosis 4. Traumatic dislocation of the testis Systemic Conditions: 1. Henoch-Scholein purpura 2. Familial Mediterranean Fever 3. Polyarteritis nodosa 4. Thromboangitis obliterans 5. Hypersensitivity angitis Abdominal and Retroperitoneal: 1. Incarcerated strangulated hernia 2. Neonatal adrenal hemorrhage 3. Tumors of the pancreas 4. Acute appendicitis 5. Hemoperitoneum Spermatic cord: Appendages: 1. Infarction of spermatic cord 1. Torsion of testicular appendages 2. Thrombophleitis of varicocele 2. Epidiymal dirofilariasis 3. Spermatic cord hematoma Latrogenic: 1. Latrogenic torsion of testis 2. Laparoscopic inguinal herniography Reprinted with permission from: Pentyala S, Lee J, Yalamanchili P, et al. Testicular Torsion: A Review. J Low Genit Tract Dis. 2001;5(1):38-47 (Table 1, page 44). epididymitis, and TAT for providers that do not have immediate access to imaging and to avoid ordering unnecessary tests for those that do. To this end, the remainder of this section will focus on the subtle clinical differences that can be used to differentiate these clinical entities. In terms of the history, some investigators have found that the duration of symptoms is useful in differentiating the cause of an acute scrotum. Mushtaq performed a retrospective review of 204 patients who presented with an acute scrotum. They found that patients with TOSC sought care earlier, a median of 9.5 hours compared to 48 hours with TAT (P < 0.005). 24 Another study found that when compared with epididymitis, a patient was more likely to present in < 12 hours if they had TOSC or TAT (31% vs 69% and 62%, respectively, [P < 0.05]). 21 These investigators found a statistical difference in age at onset between TOSC and TAT with those having TOSC being older (median age of 14 years vs 10 years [P < 0.05]). 21 Finally, the history is more likely to reveal dysuria, urinary frequency, urethral discharge, and/or fever in epididymitis than the other 2 considerations. More useful are findings on the physical examination. Those with epididymitis are more likely to have an isolated tender epididymis (97% vs 23%) and to have a normal testicular lie (100% vs 54%) than those with TOSC. (See Figures 3 and 4, page 7.) In addition, those with TOSC are more likely to have an absent cremasteric reflex than those with epididymitis (100% vs 14%). 21 Rabinowitz et al report 100% specificity for a positive cremasteric reflex ruling out TOSC; however, that same study demonstrated this reflex in only 51% of boys. 25 In epididymitis, tenderness is limited to the epididymitis unless complicated by an associated orchitis. By contrast, the tenderness associated with TAT is usually isolated to the superior pole of the testis. It is important to note that while absence of the cremasteric reflex is an important finding, there have been case reports of TOSC with a reflex being present. 26 The blue dot sign is a bluish hue found at the superior pole of the testis and is pathognomonic for TAT, although rarely present. It was found only in 23% of patients with TAT. 21 In addition, elevation of the affected side with subsequent relief, Prehn sign, implicates epididymitis as the etiology of the patient s pain. While useful, these findings are seldom present and in the case of the Prehn sign, very subjective. Therefore, they are not reliable for differentiating the differential. Prehospital Care Families often contact their emergency medical services (EMS) providers when faced with a perceived acute medical emergency, and scrotal emergencies are no exception. Acute onset of scrotal pain or Pediatric Emergency Medicine Practice October 2011

7 swelling will likely prompt a patient or care taker to call EMS to provide pain relief and transportation. When that happens, the care and management of an acute scrotal emergency can be started well before the emergency clinician has a chance to intervene. It is incumbent on the EMS operator to provide safe and sound advice and to avoid delays in getting appropriate care so as to avoid gonadal tissue loss. The first priority has to be to assure that the ABCs (Airway, Breathing, Circulation) have been addressed. Some patients stricken by torsion may vomit. If so, protecting their airway becomes a consideration, especially if they have self-medicated with analgesics like acetaminophen with hydrocodone, oxycodone, or codeine that could suppress their gag reflex. Usually an airway maneuver is not necessary. However, if vomiting is persistent, positioning them in the recovery position, on their side with their head turned to the side, is preferred to lying on their back. Once the ABCs have been addressed and are secure (typically with acute scrotal pain, no airway or circulatory issues exist), EMS can get more information about the complaint (onset of pain, nature of the pain, and where pain originated). It may be helpful for EMS to note and then report the time of the first call for help since the time from onset of ischemia of the testis to the relief of the same is vital to the successful management of TOSC. In the interval between dispatch and arrival of the EMS team, the patient should be advised to rest and to avoid eating or drinking anything, including chewing gum, hard candy, or even water since these may increase the risks associated with general anesthesia. 27,28 In the field, another intervention that might help alleviate the patient s suffering is placing them in a high Fowler position for transport, with a pillow placed between the patient s legs to provide support Figure 3. Acute Torsion (High-riding, Horizontal Lie) for the scrotum. Providing oxygen may be helpful in reducing ischemic pain and should be started in all suspected cases of TOSC. Intravenous or intramuscular administration of a potent analgesic would also be appreciated by the patient. The authors recommend morphine sulfate over meperidine because it can induce some degree of vasodilatation. If vomiting occurred and is recurrent, administering ondansetron would be preferred to promethazine so as to avoid sedating the patient and making the ED evaluation more difficult. If ondansetron is not available, the authors prefer that the EMS team simply transport the patient. In some cases, elevating the scrotum by some type of supportive sling may help alleviate the pain. The application of ice has been shown to be effective in protecting a testis that is subjected to ischemic stress, so applying ice may help reduce the injury pattern in men experiencing TOSC. 29,30 Manually rotating the testis in an attempt to untwist the torsion should be left to the emergency clinician s discretion, as field attempts will certainly delay transport and could make the patient worse. See the Nonsurgical Management section (page 12) for more details on how and why to detorse, including why some patients get worse after a detorsion procedure is performed, even when performed correctly. No other intervention is appropriate by the EMS team as expediting the patient s arrival at an ED capable of determining whether torsion exists or not is most important. Emergency Department Evaluation History The importance of a history of recurrent episodes of scrotal pain and swelling should not be discounted. Intermittent torsions have been the subject of conjecture for years. A retrospective study of 50 patients reported that patients who underwent surgery for Figure 4. Acute Scrotal Hematocele Image is courtesy of Dr. Martin Herman, MD. October Image is courtesy of Dr. Martin Herman, MD. 7 Pediatric Emergency Medicine Practice 2011

8 TOSC had a mean of 4.3 painful episodes (range 1-30). All had a horizontal lie to their testes, and all were confirmed to have the bell clapper deformity at surgery. 31 Another retrospective study involved 17 boys who underwent emergency surgery for TOSC and another 30 who underwent elective surgery for recurrent, episodic scrotal pain. These boys experienced an average of 2 to 3 episodes of pain prior to their surgeries. 32 These findings and those of other researchers led to the recommendation that eliciting a history of intermittent pain, similar in nature to that experienced with an acute TOSC, should prompt the provider to consult with a urologist who should consider electively performing scrotal exploration with orchiopexy bilaterally. Otherwise, there will remain a risk of TOSC occurring with potential loss of the affected gonad. This recommendation is even stronger if the testes are found to be horizontally oriented on examination. In the history, be sure to ask for the presence or absence of the various risk factors for TOSC, such as recent trauma, sexual activity, exposure to cold, cryptorchidism, recurrent episodes of testicular or scrotal pain, abdominal pain, nausea, and or vomiting. In addition, inquire about risks for other explanations for the patient s pain. Also ask about medication and food allergies, since surgery may be necessary. Note the time of the last meal so you can inform your surgical colleague and anesthesia provider whether or not the stomach is empty. An expanded problemfocused physical examination is indicated. Physical Examination Examination of the genitalia should always include an abdominal examination. Even when a patient presents with an acute scrotum, the provider must be sure that a ruptured appendix hasn t resulted in sufficient inflammation to affect the scrotum. 20 Likewise, there are other possible entities that can result in pain or swelling of the scrotum. A retrospective study looking at 353 cases that presented to the Bristol area between 1960 and 1974 reported 90% accuracy in detecting TOSC. However, they also found 10 cases of acute appendicitis and 3 cases of incarcerated inguinal hernias, hence the advice to examine the abdomen and inguinal areas as part of the assessment of the male patient. 6 The corollary also applies. That is, when evaluating someone for abdominal pain, also include an examination of the genitalia. Once the abdominal examination and inguinal examination have been completed, the examination of the male genitals continues with a careful inspection and then palpation of each of the structures accessible to the examiner s fingers. Transillumination can be helpful in some disorders, and occasionally, even auscultation of the scrotum can help by detecting bowel sounds when an inguinal hernia is present. To examine the male, start with the male stand- ing with legs apart. Observe the size and position of the hemiscrotum. Note that the left testis is generally situated lower than the right. This may be because the spermatic cord is longer on the left. If it is not, consider that a torsion or twist in the spermatic cord has shortened the cord. There should be rugae noted externally, the absence of which indicates an empty hemiscrotum. That should cause the examiner to consider a cryptorchid testis or an atrophic testis due to an in utero torsion. The testes should lie in the scrotum with the ventral and inferior pole anterior dependent relative to the spermatic cord. The upper pole is posterior in the scrotum, typically covered by the epididymis. The long axis of the testis is situated almost vertically, with slight angulation of the upper pole to the dorsal aspect. 9 Note any lesions of the scrotal wall (insect bites, hemangiomas, infectious lesions like herpetic vesicles, chancres, etc). Stretch the scrotal wall, noting the consistency and tone. Is there edema of the wall? Next, try to assess the tunica vaginalis. Is it thin and smooth, and does it roll easily between the examining fingers? Assess the testis. Is it lying normally with the long axis nearly vertical? Or is it more horizontal, suggesting a bell clapper deformity? 33,34 Recall that this deformity is associated with an increased risk of an intravaginal TOSC. Is the testis normal in size for the patient s age? For those who do not examine a large number of children regularly, you may need to refer to a reference chart to determine that. Is it tender? Is the cremaster reflex present bilaterally? Find the epididymis. Can you differentiate it from the testis? Is it swollen or tender? Assess the spermatic cord. Can you feel any twists in it? Is it swollen? Is there a spermatocele or varicose vein complex? Patients who present with an acute scrotum may have swelling, erythema, and tenderness. However, in some cases of TOSC, especially those presenting > 6 hours after the onset of pain, tenderness may not be present. 20 The findings on an examination of a patient with TOSC may include swelling of the hemiscrotum, an abnormal lie of the testis, an enlarged and tender testis, a palpable twist or knot in the spermatic cord, and erythema or change in the color of the scrotal wall. However, erythema is much more common in acute epididymitis. In addition to pain, other symptoms may include fever, nausea, vomiting, and abdominal pain. Patients with TOSC typically have a tender testis and an absent cremaster reflex (100% of cases in Kadish s study). 21 The affected testis seemed to be positioned more horizontally (abnormal lie) in 54% of the cases, and scrotal erythema was present in only 38% of cases. 21 Laboratory Studies Given the similarity in presentation of TOSC, epididymitis, and TAT in terms of the history, further Pediatric Emergency Medicine Practice October 2011

9 testing is often required in order to make a definitive diagnosis. A work up is reserved for equivocal or low-suspicion cases. Conversely, in cases where the timing, history, and physical examination highly suggest TOSC, urologic consultation and surgical exploration and correction is indicated, and a further work up is of no additional benefit. Ideally, a serum marker could be utilized for differentiating these entities as these types of tests are more likely to be available and no experience or added training is required to utilize them. However, only a few studies have been done investigating this diagnostic modality, and this research is in its infancy. Acute phase reactants such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) have been evaluated in several studies and appear to have some value in differentiating cases of epididymitis/orchitis from non-inflammatory causes of acute scrotal pain. This was true for CRP in particular. 35,36 In one study, 96% of patients with epididymitis had an elevated CRP. Further, the mean value of CRP for epididymitis was 68 mg/l while for those with TOSC and other non-inflammatory etiologies it was 9 and 7 mg/l, respectively. 36 In one small study, interleukin 6 (IL-6) was significantly elevated in epididymitis, pg/ml (IQR ), as compared to 1.03 pg/ml (IQR ) in TOSC. The positive predictive value of IL-6 in diagnosing epididymitis was 79% while the negative predictive value for TOSC with an elevated IL-6 was 100%. In this same study, interleukin 1 and creatine kinase were also investigated but showed no reliable diagnostic value. 37 This area of research is promising, but larger studies are required in order to recommend the routine use of inflammatory serum markers in the evaluation of the acute scrotum. Urinalysis is simple and can be of use in the work up of acute scrotal pain. While pyuria is suggestive of an infectious process such as epididymitis, it is important to remember that according to a review on the topic as many as 30% of patients with TOSC have this finding and therefore a positive result used alone cannot rule out torsion. 38 Imaging Studies In contrast to laboratory analysis, imaging for investigation of the acute scrotum has been extensively studied, and there is good evidence to support its routine use. The 2 modalities that have emerged as the most reliable and useful are color Doppler sonography and testicular scintigraphy. Testicular scintigraphy has been utilized since the 1970s and uses radionuclide imaging to demonstrate absence of blood flow to the testis caused by obstruction from TOSC. Obstructed flow leads to delay in radionuclide perfusion that translates into a photopenic image. By contrast, increased flow may indicate an inflammatory process such as epididymitis/orchitis. Sensitivity varies from 80% to 100%, but most studies show sensitivity being > 95%, especially in cases when imaging was performed within 24 hours of onset of symptoms Interestingly, this sensitivity is higher than reported when scintigraphy and sonography were studied together. The issue with scintigraphy is one of specificity, since there are other entities that can demonstrate a photopenic testis such as hydrocele, hematocele, spermatocele, or a scrotal hernia. Color Doppler sonography has gained momentum over the last 20 years and has become the imaging test of choice for most providers. The advantages lie both in its noninvasiveness and more importantly, in its ability to differentiate nonsurgical causes of acute scrotum as well as offer an alternative definitive diagnosis. While scintigraphy provides only a Yes or No answer for TOSC, ultrasound can do the same while also demonstrating a specific diagnosis for the No group. 43 While the literature is scattered with cases of false-negative results for sonography, at least 2 prospective trials have shown a similar sensitivity when compared with scintigraphy. 47,48 In the larger more recent of the 2, color Doppler sonography and scintigraphy both had a sensitivity of 78.6% (95% confidence interval [CI], 66.7%-90.5%). 48 At least 1 recent sonographic study may show superior sensitivity to that of scintigraphy, possibly reflecting an improvement in equipment and user technique. In this study, 298 boys with findings suspicious for TOSC had a color Doppler sonogram and underwent surgery regardless of the imaging results. Sonography showed an overall sensitivity, specificity, positive predictive value, and negative predictive value for TOSC to be 96.8%, 97.9%, 92.1%, and 99.1%, respectively. 49 Expanding sonographic evaluation to include the cord in order to demonstrate the torsion itself has been shown to improve sensitivity. In a multicenter retrospective assessment, 919 boys were evaluated both with traditional color Doppler sonography and high resolution sonography of the cord looking for a twist. 50 Of the original 919 patients, 208 had TOSC proved at surgery. While traditional ultrasound showed a sensitivity of 76% (near what other studies have shown), the sensitivity improved to 96% when the cord images were added. Further, ultrasound revealed a linear cord in all other cases of acute scrotum. That gives this modality a specificity of 99% in this study. 50 This technique is relatively new and further studies are required. Like sonography in general, it is limited by user technique and experience, but its routine use shows promise. It is important to note that most studies evaluating imaging include all cases of acute scrotum regardless of the clinical suspicion of TOSC. While sonographic use is limited to cases that are equivocal October Pediatric Emergency Medicine Practice 2011

10 or of low suspicion, it continues to prove its usefulness in what can be a difficult diagnostic dilemma. 51 As previously stated, using imaging in this setting makes more clinical sense since those patients whose timing, history, and physical examination are highly suggestive of TOSC warrant immediate surgical exploration and fixation as opposed to an extended work up. Some have suggested reserving scintigraphy in those cases when ultrasound is equivocal but TOSC is still suspected. 43 While both forms of imaging can be very useful, false-negatives do exist, and the decision-making process to operate should include all clinical, laboratory, and imaging data. When doubt exists, best practice requires consultation with urology for a definitive plan of care. The European Association of Urology has published guidelines on the approach to the acute scrotum in children, and a search of the National Guideline Clearing House will present a summary of that report. 52 Treatment Surgical Management As recently as 76 years ago, TOSC was managed by bed rest or manual detorsion. 53 These approaches were not found to be satisfactory, so management evolved to urgent, if not emergent, surgical exploration. In recent years, the approach to these patients has been to go to the operating room and explore the scrotum. If TOSC was found, the cord was returned to its anatomic neutral state, the viability of the testis was assessed, and the testis was pexed to the posterior scrotal wall if viable or removed if dead. (See Figure 5, page 12.) The contralateral scrotum was also explored and the testis pexed to its hemiscrotal wall. Given the developments in imaging, specifically the availability and reliability of color Doppler ultrasonography, some have challenged the dogmatic approach of exploring all inflamed scrotums. 54 Some authors have suggested that a surgical procedure on the contralateral unaffected testis is unnecessary and might induce injury and atrophy. However, Krarup s follow-up study published in 1978 clearly confirms the value of prophylactically performing an orchiopexy and refutes the alleged concerns regarding inducing atrophy. 22 Additionally, Knight and Vassey reported that 2 of 9 boys who experienced an acute torsion of 1 spermatic cord and did not have a prophylactic orchiopexy performed went on to experience a torsion of their contralateral spermatic cord within 5 years of their first episode. 3 Even if surgery and open interrogation of the scrotum is performed, there are still unresolved questions. Should the ischemic testis be retained after detorsion, even if it looks viable, since there is a risk of auto-immunization with damage to the contralateral testis ensuing? 55,56 Even if it is decided to retain the testis, how should it be fixed to the posterior scrotal wall? There is even controversy regarding the types of suture materials used to fix the testis when repairing TOSC. At least 2 references have implicated absorbable sutures as factors in the failure of prophylactic orchiopexy to provide protection of the paired gonad. 3,57 The actual point of fixation is also controversial. Mor et al undertook a retrospective study to examine these perplexing questions. They looked at 179 patients who had an operation performed because of a testicular torsion at their institution between 1991 and Patients ranged in age from neonate to 45 years of age, with the median age being 18. Repairs were performed following guidelines published in Campbell s Urology (the tunica albuginea was sutured to the dartos fascia using 2 sutures of either 3-0 chromic, 3-0 polyglactin, or 4-0 polypropylene). The earliest cases used the chromic, and the last case used the polypropylene. In this comprehensive retrospective search, 8 cases were found to have experienced another intravaginal torsion, even after having been fixed. Those with recurrences of an acute scrotum with TOSC occurred somewhere between 6 months and 23 years after their original surgery. Half occurred on the side of the original torsion and half involved the contralateral testis, even though it was fixed at the time of the original surgery. 57 As Mor et al s study indicates, recurrence of TOSC is a possibility in a patient who has undergone an open procedure to salvage a previously compromised testis. Currently, the preferred and recommended approach is to fix the testis by using a sutureless technique (fixing the testis by creating a dartos pouch). 58,59 A novel approach to the management of TOSC was recently published by a group in Philadelphia. 60 These surgeons noted that a dusky testis would improve in appearance when they incised the tunica albuginea. Following up on that, they measured intracompartmental (intratesticular) pressures in 3 cases. They then proposed that surgeons leave the tunica albuginea open, in effect, performing a testicular fasciotomy and using a flap from the tunica vaginalis to cover the exposed seminiferous tubules. Once that is done, the testicular compartment pressures should remain low and the perfusion to the organ uncompromised. More studies have to be done to see if this approach affords any advantage over other techniques already in use. Even though the technique for stabilizing the testis has changed and may be better than those used in years past, clinicians should remember that patients may present with recurrent torsions of the spermatic cord after orchiopexy, since some will have been repaired using older techniques, which as confirmed by Mor et al s study, have some risk of Pediatric Emergency Medicine Practice October 2011

11 Clinical Pathway: Treatment Of The Acute Scrotum Acute scrotum < 6 hours > 6 hours Signs of torsion of the spermatic cord? (tender testis, high transverse lie, absent cremasteric reflex) NO Perform color Doppler ultrasound. YES Is the history and physical examination consistent with torsion of the spermatic cord or equivocal? YES NO Perform either detorsion, orchiopexy, and contralateral orchiopexy or orchectomy and contralateral orchiopexy. History and physical examination definitively diagnose an entity other than torsion of the spermatic cord. Treat accordingly. Ultrasound shows signs of torsion of the spermatic cord (decreased or absent flow). Ultrasound results are equivocal. Ultrasound provides an alternative definitive diagnosis (epididymitis/orchitis, hydrocele, hematoma, or abscess). Exploration is indicated. Consult surgery/urology. Consult urology and consider scintigraphy, urinalysis, C-reactive protein, etc. Treat accordingly. October Pediatric Emergency Medicine Practice 2011

12 failure. Providers should also keep in mind the possibility that this latest technique may prove to have some failures at a later date. 60 Nonsurgical Management When faced with a male who complains of testicular or scrotal pain, pain control should be immediately instituted. This may be accomplished by administering meperidine, morphine, ketoralac, or nitrous oxide (via inhalation). Icepacks applied to the affected hemiscrotum may provide some protection to the stressed testis and afford some comfort. 29,30 Cooling the testis may provide some survival benefit by reducing its metabolic rate and oxygen demand. Supplemental oxygen has also been suggested to super saturate the blood and force more oxygen to the compromised tissue. Some authors have even looked at the use of hyperbaric oxygen therapy. 61 Pain relief is not the only goal in treatment, as testicular survival is known to be adversely affected by the duration of any torsion. Once the provider has identified that TOSC exists and is waiting for transition to the operating room, pain control and potentially enhanced survival can be accomplished by manually rotating the testis to resume a normal position and to untwist any twists in the spermatic cord. This technique was first publicized in 1893 by Nash. Since then, several people have reported their experience with the technique. 62,63 As an adjunct to the care of these patients, it has fallen into disuse because many physicians are not familiar with the technique. Reporting on their experience and outcomes, Cornel and Karthaus remarked that only 1 of 3 urologists performed the maneuver in their institution. 62 Even so, they did report that they were able to successfully detorse 14 of 17 cases. The literature Cornel and Karthaus cited to support the recommendation to try detorsion had a cumulative success rate of 65 of 74 patients. Detorsion Procedure Conventional wisdom is that the testis will tend to rotate lateral to medial in most cases of torsion. Hence, relief should come from rotating the affected testis medial to lateral. That is, counterclockwise for the right testis and clockwise for the left, when the patient is supine on the stretcher, and the provider is at the foot of the bed. A retrospective study on 186 cases found medial rotations only 67% of the time. The median for the degree of rotation in orchiectomy cases was 540, or 3 rotations. 64 The tighter the twist, the shorter the time to organ death, 65 so manually untwisting a compromised gonad may give the surgeon much needed time to finish the reduction and secure the testis before any chance of gonadal survival is lost. In manually relieving a twisted spermatic cord, use the relief of pain as a guide as to when to stop. However, even if complete pain relief is obtained, it is still wise to have a surgeon explore the hemiscrotum to confirm the reduction and to stabilize the gonad against future twists. 66 It has been reported that ultrasound guidance provided for confirmation of successful detorsion in 8 of 11 cases. 67 One important caveat when considering a manual detorsion is that not all twists occur lateral to medial, so if a reduction is attempted and the pain increases or if no relief is obtained, consider reversing the direction of the reduction. This is where ultrasound guidance may prove itself most beneficial. Since relief of pain is one of the parameters to gauge the success of the maneuver, blocking the pain via a spermatic cord block using lidocaine would seem counterproductive. Procedural sedation with a combination of fentanyl and propofol has been suggested as an alternative to a spermatic cord block. This approach would allow the patient to report the relief or persistence of scrotal pain after a manipulation is performed. 67 Note that detorsion should never interfere with the swift movement of the patient to the operating suite; however, while preparations are being made, an attempt to detorse is worthwhile. In some cases, the patient can be sent home after a successful detorsion to return the next day or so for an elective scrotal exploration and orchiopexy. 62 Pharmaceutical Treatment Ischemic injury to the testis is mediated via the release of several noxious chemicals. Studies of rats with torsions have demonstrated changes to the contralateral testis even after a twist is corrected. 68 Figure 5. Infarcted Testis Image is courtesy of Dr. Martin I. Herman, MD. Pediatric Emergency Medicine Practice October 2011

13 A multitude of researchers have tried administering pharmaceuticals such as alpha lipoic acid, n-acetylcysteine, sildenafil, immunoligands, and selenium to ameliorate the damage expected with a torsion in the rat model with some success At this time, no consensus exists on the use of these adjuncts. Perhaps one day they will be included in the treatment, but for now, the recommended approach is limited to cooling, detorsion, pain relief, and surgery. Outcomes In terms of outcomes after TOSC, fertility, hormonal changes, and sexual function are most significant as these complications can impact quality of life. Many studies have been performed on animals and show varying degrees of atrophy, aspermatogenesis, hormonal changes, and production of anti-sperm antibodies. While this research is important, the translation into relevance for human outcomes is unclear. It seems the consensus is that when scrotal contents are checked 2 or more years after a torsion is corrected by a surgeon, 2 out of 3 were atrophied, and the degree of atrophy was related to the length of time the testis was under stress. When testicular function was examined, at least 4 of 5 had abnormalities of semen volume, sperm counts, sperm motility, and percent of deformed spermatozoa. (See Figure 6.) Also of note, the contralateral testis was affected. The impact on the contralateral testis is mitigated by removal of the affected gonad. It is important to note that the contralateral testis has shown dysfunction or damage due to single testis torsion, and various mechanisms have been proposed to explain these changes. In one study, Nagler et al took Spraque Dawley rats and divided them into groups. 56 Groups 1 and 2 underwent a sham operation with opening of the scrotum only. Group 3 underwent torsion of the right spermatic cord. Group 4 underwent torsion and then detorsion of the right spermatic cord. Group 5 underwent torsion and then orchiectomy. Group 6 was the control group for administration of anti-rat lymphocyte globulin (ALG). Group 7 consisted of 15 rats who underwent intrascrotal right torsion and were given ALG on days -1, 0, and +3; their left testes were harvested on day 10. Group 8 underwent subcutaneous torsion. Group 9 underwent torsion and then orchiectomy followed by a splenectomy; their left testes were harvested at day 10. Group 10 underwent a torsion, were given ALG, and underwent splenectomy with contralateral testis harvesting on day 10. The outcomes were interesting to say the least. Animals that underwent the sham operation had no effects noted on the contralateral gonad. In Group 3 (torsion), 60% of the contralateral testes were azoospermic, and their tubular diameters were diminished. Group 4 (torsion and detorsion) demonstrated similar adverse effects on the left testis. That is, with detorsion, 14 of 18 (77%) animals had decreased spermatogenesis, compared to 12 of 20 in Group 3. Immunologic modulation with ALG did improve gonadal function, with all rats in Group 9 and all rats in Group 10 having no change in either tubular diameters or spermatogenesis. 56 It appears that in the aftermath of TOSC, something is released by the dying testis that affects the contralateral gonad. Reversing the twist is not sufficient to protect the contralateral gonads, as demonstrated by Group 4. However, removal of the injured testis and/or immunologic modulation may help preserve function. In another animal study, 50 rats were subjected to an experimental induced torsion and their inhibin Figure 6. Results Of Seminal Analysis After Unilateral Orchidopexy For Torsion Volume of semen (ml) 5 Sperm count (10 6 /ml) 50 % Motile sperm 50 % Degenerate sperm Shaded areas are in the normal range. From: Thomas W, Williamson R, Diagnosis and outcome of testicular torsion. Br J Surg. 1983;70(4): By permission of Butterworth & Co. 0 0 October Pediatric Emergency Medicine Practice 2011

14 B levels were assessed. Serum inhibin B is a marker for Sertoli cell function and the degree of spermatogenesis. When the testes were examined histologically, no changes were seen, even after 4 hours of torsion and even though this time frame exceeds the 3-hour period of rescue opportunity that Cosentino prefered. 71 However, when measured, the serum inhibin B levels were significantly decreased. This work suggests that neither orchiectomy nor orchiopexy can prevent the auto-immune damage to the contralateral testis endocrine function if the critical window of opportunity is exceeded. 72 Not all research has substantiated Nagle et al s work. Madgar et al attempted to show that a simulated TOSC in rats followed by either release of a ligature compromising blood flow or orchiectomy after 24 or 48 hours would affect testicular integrity or fertility. This group did not see any effects on the contralateral testis nor fertility. 73 This presents a surgical dilemma. Does relieving the compromised blood flow and subsequent salvage of the testis leave the patient at risk for some harm to the unaffected testis? Would the patient be better served if all testes that had been compromised were removed? There is some support for this notion. As indicated by Nagler et al s work, there seems to be some immunologic phenomenon that affects the testis of the other side. Damage to testes has been induced by subjecting rat testes to testicular antigens. A condition known as experimental allergic orchitis (EAO) is well described in the literature. 56 Hence the possibility that when a testis is ischemic, it releases antigens that then trigger an immunologic response, which subsequently attacks the contralateral testis. Caution is advised when performing a reperfusion technique such as detorsion since the rush of blood to damaged tissue may enhance the release of antigenic material, thus resulting in an increased risk of injury to the unaffected gonad. The idea of reperfusion being associated with an increased risk isn t new; cardiology colleagues have warned the emergency medicine world to be alert to reperfusion phenomenon when giving antithrombolytics to patients suffering from myocardial ischemia. Aside from concerns that reperfusion or delayed relief of torsion is associated with an enhanced immunologic response and that salvage may not prevent the contralateral injuries, we also know from observations of patients who have had testicular salvage that about 68% of the testes had rescued atrophy. With such a high rate of atrophy, it may not be worth the added risk. 74 Krarup published a paper in 1976 on 74 patients treated for torsion and reported that there was a testicular survival rate of 59% after detorsion. 79 In 1978, he published a follow-up study on 48 of the original patients. 22 He found that 19 of 48 saved testes were atrophic. This is a 68% rate of degradation, even higher than reported in a study by Macnicol. 75 Ten of Krarup s patients were treated with surgical detorsion and contralateral fixation; 13 were treated with surgical detorsion without contralateral fixation. Five had manual external detorsion; 9 had orchiectomy and contralateral fixation. Seven were treated with orchiectomy without contralateral fixation; 4 had surgical fixation for recurrent pain. Patients were interviewed and queried with respect to scrotal pain, potency, and fertility. Testicular volume was measured ultrasonagraphically and by orchidometry. Of the 28 saved testes, only 9 were of normal volume; the remaining 19 were atrophied, many of which had been under torsion for 4 hours or more. However, even early surgical salvage did not guarantee that a full-sized organ would result. One of the 5 patients treated non-operatively subsequently lost a testis due to a recurrence of torsion that was misdiagnosed. In patients who did not have the contralateral testis fixed at the time of the original torsion, 8 of 25 patients reported recurrent pain. Two of these had surgical fixation performed prior to the follow-up study, and 3 more signed up for elective fixation during the study. At least 2 patients of the original 74 experienced torsion of their contralateral testis. 79 Also reported in this follow-up were patients who were old enough to be sexually active. They each had a normal libido and potency. Some had fathered children, and 39 patients had not. Those patients were primarily unmarried and had not tried to impregnate their sexual partners. Sperm samples were submitted by 19 patients only one of which was normal. 22 Studies that have attempted to determine outcomes in patients that suffered from TOSC for the most part show a good prognosis in terms of fertility and hormonal function if the patient presents early and there is no delay in diagnosis and surgical intervention. Follicle-stimulating hormone was elevated in patients who underwent orchiectomy, presumably because the duration of torsion was longer and the tubular destruction was greater; however, the luteinizing hormones were also elevated and may have acted to buoy testosterone levels in these males. Authors of these studies commented that several other patients (not part of this study) reported alterations in sperm concentration; however, in this study the sperm counts remained normal even though the morphology of the sperm were different. Even so, the impact on fertility did not appear significant. 74 Sexual dysfunction has been reported; however, this complication appears rare and may not be the result of torsion. The clinical bottom line is this: time from onset to intervention remains the most critical prognostic factor in not only salvation of the affected testis but also for preservation of normal hormonal and fertility function. As presented in an article by Lewis, the longer the interval, the lower the testicular salvage rate. 3 (See Figure 7, page 16.) If there is any question Pediatric Emergency Medicine Practice October 2011

15 about viability, it is better to remove the testis. The one exception to this rule is for patients with torsion who only have one gonad. In that case, preserving the testis would not risk injury to a partner gonad, since it does not exist, and it is possible that the testis might survive. 76 Medicolegal Concerns From the first encounter of the patient with the medical team, the decisions made can play a significant role in the outcome for all concerned. While clinicians never want to practice just to avoid a lawsuit, there are liability issues when a patient presents with an acute scrotum. Poor outcomes are fairly common, and clinicians actions need to be above reproach. When a legal remedy has been sought by patients, the experience has been mixed for doctors and their insurance companies. An objective review of all cases involving testicular torsions that were closed by a major medical insurer in New Jersey was performed for the time period During this 18 year period, 39 cases were closed. Sixty-seven percent resulted in a payment to the plaintiff, with Risk Management Pitfalls In The Emergency Treatment Of Torsion Of The Spermatic Cord 1. I didn t think I needed to perform a genital examination in a crying infant. Remember that the incidence of torsion is high in the first year of life, decreasing in incidence until the pubertal years, 14 and up. 2. I didn t consider acute torsion in my patient with abdominal pain. Risk factors for TOSC include recent trauma, sexual activity, exposure to cold, cryptorchidism, recurrent episodes of testicular or scrotal pain, abdominal pain, nausea, and/or vomiting. 3. My patient has abdominal pain and vomiting, but he has a normal appearing scrotum, so it can t be torsion of a cryptorchid testis. Though the absence of rugae usually indicates cryptorchid testis, cases do exist where the patient s testes appeared normal. 4. I performed manual detorsion, and my patient immediately felt relief. The procedure was 100% successful. Even with ultrasound guidance and palpable relief of torsion, some twist may persist. Even if the twist is 100% relieved, the chance that the patient will have a bell clapper deformity of the involved testis is high, not to mention the chance that the contralateral testis is also abnormally secured in its hemiscrotum. 5. I untwisted a torsed spermatic cord after giving my patient an inguinal block with lidocaine. I thought that the pain relief signaled a successful procedure. Since the nerve is blocked, pain cannot be used as a guide to the success or failure of the procedure. 6. My patient has complained of pain before, but it was awhile ago and it doesn t happen regularly. Failure to recognize recurrent episodes of pain may herald torsion. Many patients have a history of similar pain that remits spontaneously before actually presenting with a twist requiring surgery. These patients may benefit from an orchiopexy even if they haven t had unremittant torsion. 7. My patient s testis was pink and vibrant in the scrotum after torsion, so the testis was left intact. Studies have demonstrated adverse effects on the contralateral testis presumably from antibodies against normal testicular cells formed after an ischemic event. 8. This patient had no cremaster reflex, so I ruled out torsion of the spermatic cord. While the presence of a cremaster reflex was 100% sensitive in one study, the sample size may not have been large enough to generalize these findings to all cases with all examiners. 21 The wiser choice is to back up your diagnosis with an imaging study like color Doppler sonography of the scrotum. 9. I never realized that pyuria does not rule out torsion. Yes, pyuria is found most often in patients with acute epididymitis; some patients with torsion may have pyuria. 10. I relied on Prehn sign to rule out torsion and rule in epididymitis. Prehn sign (relief of scrotal pain with elevation of the sac) is only 45% sensitive, meaning it can t rule out torsion in over half of the cases. October Pediatric Emergency Medicine Practice 2011

16 95% of these being settled without trial. In only 13 cases (33%), the jury found for the defendant physician. The median indemnity payment was $45,000. In these cases, the presentation was deemed atypical about half of the time. The losses were primarily due to successful complaints that alleged an error in making the diagnosis (74%), alleged harm as a result of not making or a delay in making the referral (48%), alleged failure to obtain radiologic imaging (19%), alleged failure to explore the scrotum (13%), alleged error in the performance of the surgery (13%), and alleged adulterated records (10%). In a conversation with the authors of this article, the physician-owned State Volunteer Mutual Insurance Company of Tennessee reported that from , there were 25 cases involving testicular torsions. An indemnity payment was made in 10 (40%) cases. In those cases where a payment was made, 1 patient was a newborn, 1 was between 0 and 10 years of age, 4 were between 11 and 20 years of age, 1 was between 21 and 30 years of age, 2 were between 31 and 40 years of age, and 1 was between 41 and 50 years of age. Fifty percent of the cases that resulted in an indemnity payment involved kids under 21. The loss for the company was between $14,375 and $105, Although these 2 insurance companies had small losses relatively speaking, when extrapolated to the entire country, it is easy see that missing or mishandling an acute scrotum can have significant monetary penalties for the providers and society at large. Patients may suffer by an unnecessary loss of a gonad. Physicians may suffer by being entangled Figure 7. Testicular Survival From Onset of Pain To Surgery in a lawsuit for years. Knowing what to do when a male patient presents with a painful swollen hemiscrotum and when to do it may help provide the best care for the patient and the optimum defense against accusations of negligence in the management of this challenging problem. Summary Time is testis. A similar expression, time is tissue, was first heard when reading papers on streptokinase and tissue plasminogen activator for myocardial infarction 25 years ago. Not too long after, we began to hear this same mantra when the management of thrombotic cerebrovascular disease was being discussed. It seems to make sense that when a tissue or organ system s vascular supply is restricted because of a thrombus, embolus, or stricture of the afferent blood supply, the time it takes to restore the blood flow is critical to the survival of the tissue, organ, or organism, and the sooner the better. Cases of TOSC are no different in that respect. They fall into the class of time critical emergencies in how they are handled. Not only is pain relief linked to the return of blood supply but the very survival of the organ is dependent on how quickly the perfusion is returned. As described in this article, the amount of time that has elapsed from the onset of the torsion to the relief of the torsion is important not only to the survival of the testes functionality but the very survival of the testis itself. Both function and organ survival is important for the patient and, by extension, should be important to the medical team. When a case presents to the ED, the clock has already begun ticking. If, in retrospect, a clinician s actions are perceived as having taken too long, they may be held against them, especially if the outcome of the case can be linked to a delay of care. So clinicians must choose wisely. If their actions are thought to have resulted in a delay and contributed to a poor outcome, the penalty can be severe. Hardly a week goes by in the ED that a male doesn t present with a painful, swollen hemiscrotum. Knowing what to do and when to do it may help provide the best defense against accusations of negligence in the management of this challenging problem. Case Conclusion From Lewis A, Bukowski T, Jarvis P, et al. Evaluation of Acute Scrotum in the Emergency Department. Journ Ped Surg., Vol. 30, No 2 (February), 1995:pp (Figure 2, page 279) Realizing the poor prognosis for the patient s affected testis, you obtain intravenous access, provide parenteral narcotics for pain relief, and defer a detorsion attempt. The diagnosis is confirmed with color Doppler sonography while the urologic service is consulted. As the patient waits to be taken to the operating room, you discuss the prognosis for the patient s affected testis. Mom asks what, if any, the difference in outcome would have been if the Pediatric Emergency Medicine Practice October 2011