Torsion of the Cryptorchid Testis—Can It be Salvaged? Dorit Zilberman,* Yael Inbar, Zehava Heyman, Danny Shinhar, Ron Bilik, Itamar Avigad, Paul Jonas, Jacob Ramon and Yoram Mor From the Departments of Urology (DZ, PJ, JR, YM), Diagnostic Imaging (YI, ZH) and Pediatric Surgery (DS, RB, IA), Chaim Sheba Medical Center, Tel-Hashomer and Sackler School of Medicine, Tel-Aviv University, Tel Aviv, Israel
Purpose: The literature concerning undescended testis mainly concentrates on the increased risks of infertility and development of germ cell tumors. Yet the UDT also appears to be at higher risk for torsion compared to the normally descended testis, and this issue is relatively poorly addressed. We reviewed all cases of torsion of UDTs operated on at our hospital during the last 20 years in an attempt to characterize better this condition and its salvageability. Materials and Methods: In this retrospective clinical study we reviewed and analyzed all cases of testicular torsion involving UDT operated on at our hospital between 1984 and 2004. Results: A total of 11 children were operated on at our hospital for torsion of undescended testis between 1984 and 2004. Patient age ranged from 1 month to 18 years (median 7.5 months). In all cases unilateral torsion of undescended testis was diagnosed, with 73% of cases involving the left side. Clinical symptoms included inguinal swelling and erythema associated with a tender, firm mass palpated in the groin area and an empty ipsilateral hemiscrotum. Doppler ultrasound examination was routinely performed in the last 7 patients to confirm the diagnosis. During inguinoscrotal exploration severe ischemia or overt necrosis of the testis was found in 5 of 11 cases. Three of these 5 cases were managed by orchiectomy, while in the other 2 cases the testis subsequently vanished. In the 6 patients who exhibited some improvement following detorsion and warming of the tissue the testes were preserved and orchiopexy was performed. Followup was available in only 5 patients, with vanishing of the torsed testis observed in 4 and a normal testicle detected 21 years postoperatively in 1 patient who was diagnosed early. Conclusions: This series clearly demonstrates poor rates of surgical salvage, which we mainly attribute to delays in parental response and in primary physician referral to the hospital. Parents, who have a pivotal role in early diagnosis, were usually unaware of this urological emergency, and some were surprisingly unaware of the presence of cryptorchidism. By increasing the awareness regarding this entity among members of the medical community and parents, we hope that torsion of the cryptorchid testis (literally, “hidden testis”) will no longer necessarily be synonymous with “crypt-torsion” (“hidden torsion”). Key Words: cryptorchidism, testis, spermatic cord torsion
he incidence of cryptorchidism in full-term neonates is estimated to be 2.7% to 5.9% at birth but decreases to 1.2% to 1.8% by age 1 year.1 The literature concerning undescended testis mainly concentrates on the increased risks of infertility and germ cell tumor development as the primary sequelae of this condition.2 Yet the UDT also appears to be at higher risk for torsion compared to the normally descended testis. Williamson estimated torsion to be approximately 10 times more common in cryptorchidism.3 Still, this issue is currently poorly addressed and sometimes neglected even in contemporary articles concerning pediatric testicular problems.1 Not unexpectedly, there is not enough awareness among physicians or parents with regard to this urological emergency, and in most cases the diagnosis unfortunately is deferred. We reviewed all cases of torsion of UDT operated on at our hospital during the last 20 years in an attempt to characterize better this condition and its salvageability.
T
Submitted for publication October 5, 2005. * Correspondence: Department of Urology, Chaim Sheba Medical Center, Tel-Hashomer, Ramat-Gan, 55621 Israel (telephone: 972-35302231; FAX: 972-3-5351892; e-mail:
[email protected]).
0022-5347/06/1756-2287/0 THE JOURNAL OF UROLOGY® Copyright © 2006 by AMERICAN UROLOGICAL ASSOCIATION
PATIENTS AND METHODS We reviewed and analyzed all cases of testicular torsion involving UDT operated on at our hospital between 1984 and 2004. Data were collected from the original referral letters, emergency room charts, surgery reports and subsequent outpatient clinic files. If information was lacking, the patients were invited for further interview, repeat physical examination and ultrasound imaging, to document the outcome accurately.
RESULTS A total of 11 children were operated on at our hospital following torsion of UDT between 1984 and 2004. Patient age ranged from 1 month to 18 years (median 7.5 months). Torsion of the undescended testis was diagnosed in all children (73% on the left and 27% on the right side), with bilateral cryptorchidism noted in 3 patients. Clinical symptoms mainly included the appearance of inguinal swelling and erythema described as a tender, firm mass palpated in the groin region. It was usually accompanied by inconsolable crying in infants, poor nutritional intake and occasional vomiting in older children. Eight
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Vol. 175, 2287-2289, June 2006 Printed in U.S.A. DOI:10.1016/S0022-5347(06)00329-6
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patients (73%) were admitted to the hospital 4 to 8 hours after identification of initial symptoms by the parents. However, 2 infants unfortunately were referred to the hospital 3 days after the onset of symptoms due to misdiagnosis of upper respiratory tract infection associated with a “reactive hydrocele.” During the last decade Doppler ultrasound examination was routinely performed before surgical exploration in the last 7 cases to confirm the diagnosis. However, Doppler ultrasound failed to show perfusion to the involved testicles. Exploration was universally performed through an inguinal approach and delineated a spectrum of ischemic insults to the involved testicles, with 5 testicles appearing necrotic on gross inspection. In 3 patients with apparently longstanding necrosis orchiectomy was performed because no testicular viability was observed despite appropriate salvage attempts. However, in 1 patient presenting with necrotic tissue the testicle was preserved despite clear evidence of irreversible ischemia, due to the reluctance of the surgeon to perform orchiectomy in the presence of a contralateral UDT. In this case the necrotic testicle subsequently vanished. In another case that initially appeared to be necrotic the appearance significantly improved following detorsion, and, thus, the testicle was left in place, although physical examination and Doppler ultrasound performed 15 years postoperatively revealed marked testicular atrophy. The 6 remaining patients initially exhibited ischemic effects but achieved some improvement in testicular color following detorsion and warming of the tissue. In these cases the testes were preserved and orchiopexy was performed, either concomitantly or during a second stage operation. Followup, which was available in 5 patients, demonstrated the torsed testis had vanished (on physical examination and ultrasonography) in 4. One patient, whose grandfather was a physician, and whose family was highly aware of this possible complication and sought treatment immediately, had a testicle of normal size and texture 21 years postoperatively. DISCUSSION Cryptorchidism constitutes a common congenital surgical problem encountered in males in pediatric urology. A 10-fold increased rate of cryptorchidism is reported in premature, low birth weight infants and in those with neural tube defect disorders.1,4 A 53.8% incidence of cryptorchidism has been noted in patients with cerebral palsy, and some reports refer to torsion of cryptorchid testis in patients with spastic neuromuscular disease.5,6 Genetic predisposition also has a role in this disorder, which has been noted in 1% to 4% of siblings and 6% of fathers of children with cryptorchidism.4 The most serious complications of cryptorchidism, which are extensively discussed in the literature, are a high rate of infertility and a high incidence of testicular cancer.7 Early surgical intervention in cryptorchidism is currently aimed at preserving germinal epithelium, decreasing the rate of infertility, and situating the testis in a position that allows better access for self physical examination and earlier detection of testicular cancer. Torsion of the spermatic cord was first described by Delasiauve in 1840, and interestingly it was in a 15-year-old boy with UDT treated with orchiectomy.5,8 However, in 1857 Curling described a similar case in which the undescended
testis was untwisted for the first time, relocated to the scrotum and fixed to the dartos.9 It is noteworthy that in that era there was a remarkable awareness among physicians regarding that association, who believed that “50% of all torsions occur with incomplete descent of the testicle.”8,10 Nevertheless, Roche still expressed the hope that “treatment of torsion of the spermatic cord will improve with correct diagnosis, and the diagnosis will be more frequently made when the condition is recognized as not uncommon.”8 Unfortunately, although more than 70 years has elapsed, we find the statement still relevant in the discussion of torsion associated with cryptorchidism. A review of the contemporary literature shows that it is still generally believed that an undescended testis has an increased susceptibility of undergoing torsion based on the fact that cryptorchidism is encountered in up to 7% of male neonates, with 23% of torsions occurring in this population.11 Williamson estimated that overall 1 in 160 men is anticipated to have development of torsion of the testis or its appendages by age 25.3 However, he found that torsion was approximately 10 times more common in the presence of cryptorchidism. Yet with the modern tendency for earlier orchiopexy we suspect that these figures cited from historical series have not been updated and probably represent an overestimate of the current incidence (although this fact might be balanced by the current increased incidence of prematurity). The mechanism of torsion in the undescended testis is not well understood. It has been postulated that it is related to a greater relative broadness of the testis compared to its mesentery,7 which is a possible explanation for the reported association with testicular tumors.12,13 Another theory involving abnormal contractions of the cremasteric muscles, which are responsible for the spermatic twist,6 seems more likely in patients suffering from neuromuscular disease. The diagnosis of torsion of an undescended testis should be considered in every child presenting with unexplained groin or abdominal pain in the presence of tender groin swelling and an empty ipsilateral hemiscrotum.7,13 The use of Doppler ultrasound for the detection of torsion of an inguinal testis has been well documented.6,14,15 Although it might be technically challenging, it usually reveals the testis to be diffusely edematous and enlarged, and lacking Doppler signals. The management of torsed testis is by immediate surgical exploration, regardless of the location of the testis. Following detorsion the surgeon is confronted with the dilemma of whether to try to mobilize the testis and perform scrotal orchiopexy or postpone this definitive surgical treatment. Another somewhat controversial point is whether prophylactic fixation of the contralateral testis is indicated.5,6,16 While the reported salvage rate in testicular torsion ranges from 20% to 92%,6,17 there are no such data available concerning undescended testes, nor reports regarding the longterm outcome of the preserved testicles. It is noteworthy that patients with a history of cryptorchidism who have undergone orchiopexy rarely present with acute scrotum due to torsion,18,19 in contrast to those with normally located testes who have previously undergone orchiopexy for torsion.20 It seems that eversion of the tunica vaginalis during orchiopexy for undescended testis, as well as division of the cremasteric fibers, is essential to decrease the risk of future torsion.18,19
TORSION OF CRYPTORCHID TESTIS CONCLUSIONS Torsion of an undescended testis is a relatively rare phenomenon that should be suspected, diagnosed and treated without delay. This series clearly demonstrates younger age at onset and poor rates of surgical salvage (10%) compared to the higher figures reported in patients with torsion of a normally descended testis (56%).17 By increasing awareness regarding this entity, we hope that torsion of the cryptorchid testicle (literally, “hidden testis”) will no longer necessarily be synonymous with “crypt-torsion (“hidden torsion”). Moreover, with improved recognition of this entity and earlier referrals of patients with undescended testes by primary care physicians this entity might eventually be prevented.
Abbreviations and Acronyms
13.
14.
15.
16. 17.
18.
19.
UDT ⫽ undescended testis 20.
REFERENCES 1. 2. 3. 4. 5. 6.
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Pillai, S. B. and Besner, G. E.: Pediatric testicular problems. Pediatr Clin North Am, 45: 813, 1998 Lee, P. E.: Fertility after cryptorchidism: epidemiology and other outcome studies. Urology, 66: 427, 2005 Williamson, R. C. N.: Torsion of the testis and allied conditions. Br J Surg, 63: 465, 1976 Cilento, B. G., Najjar, S. S. and Atala, A.: Cryptorchidism and testicular torsion. Pediatr Clin North Am, 40: 1133, 1993 Schultz, K. E. and Walker, J.: Testicular torsion in undescended testes. Ann Emerg Med, 13: 567, 1984 Candocia, F. J. and Sack-Solomon, K.: An infant with testicular torsion in the inguinal canal. Pediatr Radiol, 33: 722, 2003 Schneck, F. X. and Bellinger, M. F.: Abnormalities of the testis and scrotum and their surgical management. In: Campbell’s Urology, 8th ed. Edited by P. C. Walsh, A. B. Retik, E. D. Vaughan, Jr. and A. J. Wein. Philadelphia: W. B. Saunders Co., chapt. 67, pp. 2353–2394, 2002 Roche, A. E.: Torsion of the spermatic cord. In: Urology in General Practice. London: H. K. Lewis and Co. Ltd., chapt. 36, pp. 238 –264, 1935 Noske, H. D., Kraus, S. W., Altinkilic, B. M. and Weidner, W.: Historical milestones regarding torsion of the scrotal organs. J Urol, 159: 13, 1998 O’Connor, V. J.: Torsion of the spermatic cord. Surg Gynecol Obstet, 57: 242, 1933 Johnson, J. H.: The undescended testis. Arch Dis Child, 40: 113, 1965 Rigler, H. C.: Torsion of intra-abdominal testes. Surg Clin North Am, 52: 371, 1972
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Johnson, J. H.: Abnormalities of the scrotum and the testes. In: Paediatric Urology, 2nd ed. Edited by D. I. Williams. London: Butterworth Scientific, chapt. 37, pp. 451– 465, 1982 Han, B. K.: Uncommon causes of scrotal and inguinal swelling in children: sonographic appearance. J Clin Ultrasound, 14: 421, 1986 Dravid, V. S. and Heyman, S.: Torsion of an undescended testis not detected on testicular imaging. Clin Nucl Med, 16: 275, 1991 Sheldon, C. A.: Undescended testis and testicular torsion. Surg Clin North Am, 65: 1303, 1985 Barda, J. H., Weingarten, J. L. and Cromid, W. J.: Testicular salvage and age-related delay in presentation of testicular torsion. J Urol, 142: 746, 1989 O’Shaughnessy, M., Walsh, T. N. and Given, H. F.: Testicular torsion following orchidopexy for undescended testis. Br J Surg, 77: 583, 1990 Nesa, S., Lorge, F., Wese, F. X., Njinou, B., Opsomer, R. J. and Van Cangh, P. J.: Testicular torsion after previous orchidopexy for undescended testis. Acta Urol Belg, 66: 25, 1998 Mor, Y., Pinthus, J. H., Nadu, A., Raviv, G., Golomb, J., Winkler, H. et al: Testicular fixation following torsion of the spermatic cord— does it guarantee prevention of recurrent torsion events? J Urol, 175: 171, 2006
EDITORIAL COMMENT This is an important review of a poorly appreciated topic. When it is remembered that close to 2% of all males have an undescended testis the risk of torsion in an undescended testis clearly needs to be made more evident to pediatric urologists and pediatricians. The poor testis salvage rate in this series, I am sure, is reflective of the late diagnosis resulting from lack of awareness of the risk of torsion in an undescended testis. We all need to start reminding our referring pediatricians and ourselves that the parents of any child seen with an undescended testis need to be informed that the development of any tenderness associated with the undescended testis means an emergent trip to an emergency room for evaluation, and hopefully earlier care resulting in more salvage of undescended testes that twist. For infants who are having frequent diaper changes it is a simple matter for a parent to determine if there is any tenderness associated with a testis outside the scrotum. When we make awareness of this entity more widespread in the urological profession as well as in pediatrics we should be able to improve testis salvage. Howard M. Snyder, III Division of Pediatric Urology Children’s Hospital of Philadelphia Philadelphia, Pennsylvania