0022-5347/91/1452-0370$03.00/0 Vol. 145, 370-372, February 1991


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POLYORCHIDISM: CASE REPORT AND REVIEW OF LITERATURE GABRIELE THUM From the Department of Urology, Krankenhaus Am Urban, Berlin, Germany


Polyorchidism is a rare anomaly with approximately 70 cases reported in the literature. It may result from transverse division of the urogenital ridge, a hypothesis that best explains the anatomical features of the supernumerary testicle. The primary accompanying disorders and anomalies include maldescended testis, inguinal hernia and torsion. Malignancy has been reported in 3 cases. In the absence of any concomitant disorder and if testicular tumor can be ruled out by magnetic resonance imaging and ultrasonography, surgical exploration with biopsy is unnecessary. KEY WORDS:

testis, testicular diseases, magnetic resonance imaging, ultrasonic diagnosis

Contrary to the frequency of testicular malpositions (greater than 5% in infants), 1 polyorchidism, that is the presence of more than 2 testes, is rare. The first case of a supernumerary testis confirmed at postmortem examination was reported in 18802 (the frequently cited 1670 reference to Blasius could not be verified) and the first case of triorchidism discovered intraoperatively and proved histologically was published in 1895. 3 Since then, at least 70 cases of polyorchidism have been reported, including 3 cases of 4 testes each4-6 and 1 case of 5 testes 7 (not histologically confirmed). Until recently, histology provided the only proof of polyorchidism but new imaging techniques, such as sonography and magnetic resonance imaging (MRI), allow a highly accurate diagnosis of supernumerary testes rendering biopsy unnecessary. The literature on polyor- . chidism is reviewed and a case is reported. CASE REPORT

A 34-year-old man presented with a 2-year history of recurrent pain in the right groin and right scrotal space. Two ovoid swellings were palpable in the right scrotum. Routine laboratory studies and semen analysis were normal. Ultrasonography demonstrated 2 parenchymal masses 2 em. in diameter each (fig. 1). Surgical exploration with inguinal exposure of the scrotal contents revealed 2 separate testicles with a common epididymis and vas deferens but separate vessels originating from the spermatic cord (fig. 2). Biopsies were taken and the testicle was fixed to the scrotal wall to prevent torsion. Histology of the biopsy specimens of both testes confirmed the presence of seminiferous tubules and intact spermatogenesis. At followup 14 months postoperatively the patient was asymptomatic and torsion had not occurred.

sexual organs. It is the extent and the locus of the division that determine the different types of polyorchidism. A functional classification based upon the embryogenic development is provided and is represented schematically in figure 3. Type I-the supernumerary testis lacks an epididymis and vas. The split-off part of the primordial gonad does not communicate with the mesonephric tubules from which the epididymis develops. Type II-the supernumerary testis is linked to the regular testis by a common epididymis and shares a common vas with it. The division of the genital ridge occurs in the region where the primordial gonads are attached to the mesonephric ducts, although the latter are not divided (incomplete division). Type III-the supernumerary testis has its own epididymis but shares the vas with the regular testis. This variant results from a complete transverse division of the genital ridge. Other authors classify the various forms of polyorchidism according to the topology of the supernumerary testis, that is scrotal, inguinal or intra-abdominal position. 25, 26 A shortcoming of this classification lies in the disregard for the developmental aspects of the supernumerary testis. MeVary and Maizels propose yet another rather complicated scheme that permits denotation of etiology and anatomical variants. 27 The 54 (57 supernumerary testes) cases, including the present case, of polyorchidism are summarized from the literature. 3-7,14-17, 19-63 Patient age at presentation ranged from 4 weeks to 75 years (median age 17 years). Most patients were seen during infancy or adolescence. Bilateral polyorchidism occurred in 3 patients (5 %). The location of the supernumerary testis was scrotal in 75% of the cases, inguinal in 20% and retroperitoneal in 5%. Evaluation of histological data revealed testicular tissue in 54 supernumerary testes. Unfortunately, the presence or absence of spermatogenesis was determined in only


The differential diagnosis of polyorchidism should include transverse ectopia, which has been reported in approximately 100 cases,8 and an accessory empty scrotum, which has occurred in approximately 50 cases. 9 - 13 The embryogenic development of polyorchidism may involve the presence of 2-fold primordial gonads or a transverse division of the genital ridge. 14-19 The hypothesis of 2-fold primordial glands is supported by 6 cases of supernumerary testes with their own epididymides and vasa.3, 20-24 These findings suggest a complete duplication that cannot be explained by the other hypothesis of transverse division of the genital ridge. The anatomical features of all other cases are compatible with the latter theory, which assumes the transverse division of primordial gonads and mesonephros during the early embryonic stages of development of Accepted for publication August 1, 1990.

FIG. 1. Sonogram of 2 parenchymal organs with regular echoic patterns.


FIG. 2. Surgical specimen of 2 testes with common epididymis (~) and common vas deferens (~). Each testicle is supplied by its own vessels from spermatic cord.

8 cases The of torsion can be ascribed to the unstable connection of the testis to the remaincontents. hernias occur more +...........,"""',,,...,....·t-1,,7 but are less serious. of testicular tumors has been teratoma and rh:abldo:m,ros,ar1co]ma of the cremasteric muscle in 1 case each. Int,er~~st]lng case of triorchidism was by Hakami and as "unusual cause for failure of vasectomy.,,24 Evaluation of their patient, after his wife became pregnant, revealed a supernumerary testis in the right scrotum with an intact vas, normal histology and normal spermatogenesis. During vasectomy only 1 of the 2 at no point communicating vasa had been resected. This finding may be explained by complete duplication of the primordial gonad. All but 1 author performed scrotal or inguinal exposure of the involved testis. Surgical intervention is indicated if torsion, testicular tumor, undescended testicle or inguinal hernia requires treatment. However, if clinical and sonographic examination leaves any doubt as to the malignancy of a suspected scrotal mass, MRI might help to rule it out, in which case surgical exposure is unnecessary. In our case polyorchidism had not been included in the differential diagnosis because testicu1ar tumor was strongly suspected and MRI was not used. In it is that this patient could have been the r.,....,..' ..... r.,.... .... vlll .... 'JI..:.J'lJv,"'1lI



Type I

2. 3.

4. 5. Type I I

6. 7.



10. Type I I I

Kleinteich, B.: Klinische problematik. In: Kongenitale Hodendystopien. Edited by B. Kleinteich, Hadziselimovic, V. Hesse and G. Schreiber. Leipzig: Thieme, chapt. 4, p. 27, 1979. Ahlfeld, F.: Die Missbildungen des Menschen. Leipzig: Grunow, p. 126,1880. Lane, W. A.: A case of supernumerary testis. Trans. Clin. Soc. Lond., 28: 59, 1895. Baker, L. L., Hajek, P. C., Burkhard, T. K. and Mattrey, R. F.: Polyorchidism: evaluation by MR. AJR, 148: 305, 1987. Singh, and Sobti, M. K.: Polyorchidism. Brit. J. Urol., 458, 1988. Snow, B. W., Tarry, W. F. and Duckett, J. W.: Polyorchidism: unusual case. J. Urol., 133: 483,1985. Day, One man with five testes: report of case. J.A.M.A., 2055,1918. Gauderer, M. L., Grisoni, E. R., Stellato, T. A., Ponsky, J. L. and Izant, R. J., Jr.: Transverse testicular ectopia. J. Ped. Surg., 43, 1982. Coupris, and Bondonny, M.: Le scrotum surnumeraire. propos de observations. Chir. Ped., 61, 1987. Kolandaivelu, Seeniraj, K., Bose, C. and Rajagopal, Accessory scrotum. 363, 1987. Szylit, Y., Olarte, Nagler, nevus and accessory scrotum. occurrence. Amer. Acad. Dermatol., 905,1986. .... ......y .... F. M. Khanna, S.: Accessory scrotum: a case report. Brit. Plast. Surg., 127, 1983. Yokokawa, Nakano, E. and Takaha, M.: Accessory scrotum: a 593, 1986. case report. Urol., Collste, L. G. and von Garrelts, B.: Polyorchidism. Scand. J. Urol. Nephrol., 8: 167, 1974. Haddock, G. and Burns, G.: Polyorchidism. Postgrad. Med. J., 63: 703, 1987. Lambrecht, W. and Babayan, R.: Polyorchidie. Klin. Pad., 195: 430,1983. Lorincz, G.: Zur Kenntnis des Doppelhodens. Drologe A, 22: 180, 1983. Stave, D.: Erkrankungen der Geschlechtsorgane. In: Handbuch der Kinderheilkunde. Edited by H. Opitz and F. Schmid. Heidelberg: Springer-Verlag, vol. 7, p. 1234, 1966. Wilson, W. A. and Littler, J.: Polyorchidism: a report of two cases with torsion. Brit. J. Surg., 41: 302, 1953. Al-Hibbal, Z. and Izzidien, A. Y.: Polyorchidism: case report and review of the literature. J. Ped. Surg., 19: 212, 1984. 'v ........ ,


Malformations and disorders accompanying 57 supernumerary testes (multiple anomalies in 7 cases)

13. 14. 15.

No.(%) Undescended testis Hernia Torsion Other None

13 12 8 8 23

(23) (22) (14) (14) (40)

16. 17. 18.

43 testes. Of the 57 supernumerary testes 47% were type II, 33% type III and only 5% type I, and complete duplication was


reported in 11 %. No details regarding the anatomical position were given in 2 cases (4 %). Malformations and disorders accom-




21. Darrow, R. P. and Humes, J. J.: Polyorchidism: a case report. J. Urol., 72: 53, 1954. 22. Smart, R. H.: Polyorchism with normal spermatogenesis, J. Urol., 107: 278, 1972. 23. Thiessen, N. W.: Polyorchidism-report of a case. J. Urol., 49: 710,1943. 24. Hakami, M. and Mosavy, S. H.: Triorchidism with normal spermatogenesis: an unusual cause for failure of vasectomy. Brit. J. Surg., 62: 633, 1975. 25. Corbi, P., Houlgatte, A., Auberget, J. L., Rodier, J. and Timbal, Y.: Un diagnostic rare de tumeur intrascrotale: la polyorchidie. Ann. Urol., 22: 140, 1988. 26. Hancock, R. A. and Hodgins, T. E.: Polyorchidism. Urology, 24: 303,1984. 27. McVary, K. T. and Maizels, M.: Duplex testis (polyorchidism): report of 2 cases, review of the literature, and proposal of a classification system. J. Urol., 139: 500A, abstract 1346,1988. 28. Adams, J. R. and Jordan, W. P., Jr.: Polyorchidism. South Med. J., 65: 594, 1972. 29. Anderson, P. S. G.: A case of polyorchidism. Lancet, 1: 826,1953. 30. Bindewald, H., Meister, H. and Heinrich, R.: Beitrag zum Thema Polyorchismus: Triorchismus beim Saugling. Z. Kinderchir., 19: 86,1976. 31. Boggon, R. H.: Polyorchidism. Brit. J. Surg., 20: 630, 1933. 32. Burgers, J. K. and Gearhart, J. P.: Abdominal polyorchidism: an unusual variant. J. Urol., 140: 582,1988. 33. Butz, R. E. and Croushore, J. H.: Polyorchidism. J. Urol., 119: 289,1978. 34. Carey, V. F.: Report of case of duplication of left undescended testicle. J. Urol., 72: 703, 1954. 35. Corwin, S. H., Addonizio, J. and Nagamatsu, G. R.: Polyorchidism with anomalous urogenital union. J. Urol., 104: 346,1970 36. Edington, G. H. and Blacklock, J. W. S.: A case of duplication or subdivision of the testicle. Brit. Med. J., 1: 937, 1928. 37. Feldman, S. and Drach, G. W.: Polyorchidism discovered as testicular torsion. J. Urol., 130: 976, 1983. 38. Gandia, V. M., Arrizabalaga, M., Leiva, O. and Diaz Gonzalez, R.: Polyorchidism discovered as testicular torsion associated with an undescended atrophic contralateral testis: a surgical solution. J. Urol., 137: 743, 1987. 39. Garat, J.-M., Marina, S., Sole-Balcells, F. and Ayala, G.: Polyorchidie. J. d'Urol., 87: 175, 1981. 40. Giyanani, V. L., McCarthy, J., Venable, D. D., Terkeurst, J. and Fowler, M.: Ultrasound of polyorchidism: case report and literature review. J. Urol., 138: 863, 1987. 41. Goldstein, H. H. and Casilli, A. R.: Rhabdomyosarcoma of the

42. 43. 44. 45. 46. 47. 48. 49. 50. 51. 52. 53. 54. 55. 56. 57. 58. 59. 60. 61. 62. 63.

cremasteric muscle and concomitant polyorchidism. J. Urol., 41: 583,1939. Gomez, M., Jimenez, F., Abos, P. and Sole-Balcells, F.: Polyorchidie. Acta Urol. Belg., 44: 74, 1976. Golji, H.: Polyorchidism: a case report. J. Urol., 74: 207, 1955. Grechi, G., Zampi, G. C., Selli, C., Carini, M. and Ucci, M.: Polyorchidism and seminoma in a child. J. Urol., 123: 291, 1980. Handley, R. S. and Crawford, T.: A case of polyorchidism. Brit. J. Surg., 31: 300, 1944. Hochberg, K.: Verdoppelung des linken, nichtdeszendierten Hodens: Triorchismus. Fallbericht. Bruns Beitr. Klin. Chir., 216: 364, 1968. Holder, H. G.: A probable case of triorchidism. J. Urol., 13: 555, 1925. Jichlinski, D. and Ward-McQuaid, N.: Duplication of the testis and infertility. J. Urol., 90: 583, 1963. Kay, S. and Coleman, F. P.: Duplication of left undescended testicle: report of a case. J. Urol., 75: 815, 1956. Khan, C.: Polyorchidism with normal spermatogenesis. Brit. J. Urol., 61: 100, 1988. Nacey, J. N. and Hay, D. U.: Polyorchidism. Brit. J. Urol., 59: 280,1987. Nocks, B. N.: Polyorchidism with normal spermatogenesis and equal sized testes: a theory of embryological development. J. Urol., 120: 638, 1978. Parks, T. G.: Chromosome studies in polyorchidism. Brit. J. Surg., 54: 113, 1967. Pelander, W. M., Luna, G. and Lilly, J. R.: Polyorchidism: case report and literature review. J. Urol., 119: 705, 1978. Renton, C. J. C.: A case of polyorchidism with intersex. J. Urol., 113: 720, 1975. Rogus, B. J.: Polyorchidism and testicular torsion. Urology, 31: 137, 1988. Scott, K. W. M.: A case of polyorchidism with testicular teratoma. J. Urol., 124: 930, 1980. Smith, P. G., Evans, A. T. and Suder, G. L.: Polyorchidism: case report. J. Urol., 64: 515, 1950. Sujka, S. K., Ralabate, J. A. and Smith, R. A.: Polyorchidism. Urology, 29: 307, 1987. Waite, V. C.: Polyorchidism: a case report. Surgery, 36: 971, 1954. Weidenmann, W.: Eine seltene Form der Hodentorsion im Kindesalter als Beitrag zur Klarung dieses Krankheitsbildes. Zbl. Chir., 82: 1114, 1957. Wescott, J. W. and Dykhuizen, R. F.: Polyorchism. J. Urol., 98: 497,1967. Yoshida, T., Yabumoto, H., Shima, H., Ikoma, F., Nonomura, N. and Okuyama, A.: Polyorchidism: preoperative diagnosis by ultrasonography. Urol. Int., 44: 47,1989.

Polyorchidism: case report and review of literature.

Polyorchidism is a rare anomaly with approximately 70 cases reported in the literature. It may result from transverse division of the urogenital ridge...
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