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Polyorchidism
0022-534 7/78/1192-028~02. 00/0 The Journal of Urology Copyright © 1978 by The Williams & Wilkins Co.
Vol. 119, February
Printed in U.SA.
POL YORCHIDISM ROBERT E. BUTZ
AND
JOHN H. CROUSHORE
From the Department of Urology, Grandview Hospital, Dayton, Ohio
ABSTRACT
The thirty-seventh case of polyorchidism is reported. A complete compilation of all cases of this rare congenital anomaly, including a brief historical review of the more important ones, is presented for future reference. Polyorchidism is a urologic curiosity. Previously reported cases have included patients with 3, 4 and even 5 testes. However, only cases of triorchidism have been verified histologically. Holder described a typical case of polyorchidism that was verified only clinically. His patient not only had 5 testes but also 3 scrotums, including 2 normal scrotums with 2 testes and a third rudimentary scrotum with 1 testis. 2 Clinical examination is inadequate for verification of SU· pernumerary testes. An intrascrotal mass, such as a spermatocele, cyst of Morgagni, testicular tumor or aberrant epididymis, can be mistaken for a third testis. Consequently, only histologically proved cases can be accepted as examples of polyorchidism.
tient urologic evaluation had yielded a positive culture of prostatic secretions. Chronic prostatitis was believed to be the etiology of the discomfort. Family history included a son who had chronic prostatitis and the anomaly of unilateral renal agenesis. Physical examination was normal except for the external genitalia, which included a normal right hemiscrotum and an abnormal left hemiscrotum with 2 masses. One mass was believed to be an atrophic testis and the other, an associated epididymal cyst. Laboratory examinations were within normal limits, including excretory urography and chromosomal analysis. Semen analysis revealed 116 times 106 sperm per cc with essentially normal morphology and 80 per cent motility. Cystoscopic
Fm. 1. Tangential ultrasonographic view of left hemiscrotal contents. Superior testis is on left and inferior testis is on right
Historically, Ashfeld generally is accepted as reporting the first histologically proved case of polyorchidism, which was discovered postmortem. 3 In 1895 Lane reported the first histologically proved case found at operation. 4 Numerous reviews of the incidence of polyorchidism have appeared in the literature with varying degrees of accuracy. The most enlightening ones have been by Edington and Blacklock,3 Lowsley and Porras, 5 and Wilson and Littler. 1 Since the review of 21 cases by Wilson and Littler in 1953 there have been 15 additional cases reported. CASE REPORT
W. C., a 44-year-old married laborer with 4 children, was hospitalized because of chronic perineal rliscomfort. Out_paAccepted for publication May 20, 1977.
examination revealed the positive findings of acute posterior urethritis and acute prostatitis. Transillumination of the left hemiscrotum at the time of cystoscopy failed to verify the presence of any cystic mass. Therefore, further evaluation of the left hemiscrotal masses was done, using a Litton ultrasound unit with a gray scale sonovision. A 13 mm. 2.5 MHz. near field transducer focused at 5 cm. was used. The inferior left hemiscrotal mass was reported as being an atrophic testis, while the superior mass was reported as cystic, measuring 1.7 cm. in depth by 2.5 cm. in diameter (fig. 1). Exploration of the left hemiscrotum revealed 2 testes within a single tunica vaginalis. Interconnecting the testes was a single cord-like structure believed to be an epididymis. The inferior testis had a vas deferens associated with it, while the superior testis had only a vascular supply to it (fig. 2). Biopsies of both testes revealed histologic verification oftestic289
290
BUTZ AND CROUSHORE
Reported cases References
Involved Side
Histology
Wilson and Littler' Holder Boggan, R. H.: Brit. J. Urol., 20: 630, 1953 Ashfeld: Cited by Edington and Blacklock3 Lassen: Cit.ed by Edington and Blacklock• Marriotti: Cited by Edington and Blacklock3 Lerat: Cit.ed by Edington and Blacklock3 Haas: Cit.ed by Edington and Blacklock3 Jeanin and Delat.er: Cited by Edington and Blacklock3 Lecene: Cited by Edington and Blacklock3 Oudendal: Cit.ed by Edington and Blacklock3 Edington and Blacklock3 Lane• King, E. S. J.: J. Anat., 65: 427, 1931 Goldstein, H. H. and Casilli, A. R.: J. Urol., 41: 583, 1939 Chevasu: Cited by Mehan and associates• Cranz: Cited by Mehan and associates• Thiessen, N. W.: J. Urol., 49: 710, 1943 Handley, R. S. and Crawford, T.: Brit. J. Surg., 31: 300, 1944 Ranson, F. T.: Brit. Med. J., 2: 137, 1949 Lowsley and Porras" Smith, P. G., Evans, A. T. and Suder, G. L.: J. Urol., 64: 515, 1950 Anderson, P. S. G.: Lancet, 1: 826, 1953 Carey, V. F.: J. Urol., 72: 703, 1954 Wait.e, V. C.: Surgery, 36: 971, 1954 Darrow, R. P. and Humes, J. J.: J. Urol., 72: 53, 1954 Golji, H.: J. Urol., 74: 207, 1955 Wescott, J. W. and Dykhuizen, R. F.: J. Urol., 98: 497, 1967 Hochberg, K.: Bruns Beitr. Klin. Chir., 216: 364, 1968 Corwin, S. H., Addonizio, J. and Nagamatsu, G. R.: J. Urol., 104: 346, 1970 Adams, J. R. and Jordon, W. P., Jr.: South. Med. J., 65: 594, 1972 Smart, R. H.: J. Urol., 107: 278, 1972 Lazarus, B. A. and Tessler, A. N.: Urology, 3: 615, 1974 Hakami, M. and Mosavy, S. H.: Brit. J. Surg., 63: 633, 1975 Mehan, D. J. and associates" Present study
Lt. Lt. Lt.
No spermatogenesis Normal No spermatogenesis
Lt.
Unknown
Rt.
Normal
Lt.
No spermatogenesis
Lt.
No spermatogenesis
Lt.
No spermatogenesis
Lt.
Normal
Rt.
No spermatogenesis
Abdominal
No spermatogenesis
Lt. Rt. Lt.
Normal Normal Spermatogenic arrest
Rt.
Normal
Lt.
Decreased spermatogenesis Tubular atrophy
Intra-abdominal Rt.
Sertoli cell only
Rt.
Normal
Rt.
Normal
Rt. Lt.
Tubular atrophy No spermatogenesis
Lt.
Spermatogenic arrest
Lt.
Tubular atrophy
Lt.
Tubular atrophy
Rt.
Normal
Lt. Rt.
No spermatogenesis Normal
Lt.
Germinal aplasia
Rt.
Normal
Lt.
Normal
Lt.
Normal
Lt.
No spermatogenesis
Rt.
Normal
Rt. Lt.
Normal Normal
Fm. 2. Duplicated left testis exposed for excisional biopsy. Superior testis is on left and inferior testis is on right. ular material with active spermatogenesis (fig. 3). Convalescence was uneventful. COMMENT
Polyorchidism is an interesting urogenital anomaly. The embryological explanation of supernumerary testes has been discussed adequately in the past by numerous authors. As yet no chromosomal aberration has been isolated to explain the congenital anomaly. Review articles have appeared in the literature with varying degrees of accuracy. Since the review by Wilson and Littler there has not been an accurate accounting of the actual number of cases of polyorchidism. To date, 36 cases of polyorchidism have been reported in the literature (see table). However the most recent article tabulated only 28. 6 Of the 36 cases testicular duplication occurred on the left side in 21. Spermatogenesis was present in 15 instances. Normal spermatogenesis appeared to be related to the position of the supernumerary organ. In most cases scrotal placement seemed to ensure normal spermatogenesis. REFERENCES
1. Wilson, W. A. and Littler, J.: Polyorchidism: report of two cases with torsion. Brit. J. Surg., 41: 302, 1953. 2. Holder, H. G.: A probable case oftriorchidism. J. Urol., 13: 555, 1925. 3. Edington, G. H. and Blacklock, J. W. S.: A case of duplication or subdivision of testicle. Brit. Med. J., 1: 937, 1928. 4. Lane, W. A.: A case of supernumerary testis. Trans. Clin. Soc. Lond., 28: 59, 1895. 5. Lowsley, 0. S. and Porras, E.: Congenital anomalies of the testicle. J. Int. Coll. Surg., 15: 332, 1951. 6. Mehan, D. J., Chehval, M. J. and Ullah, S.: Polyorchidism. J. Urol., 116: 530, 1976.
POLYORCHIDISM
291
Fm. 3. A, microscopic view of biopsy specimen taken from superior testis shows evidence of active spermatogenesis. B, microscopic view of biopsy specimen taken from inferior testis shows normal spermatogenesis.
Vol. 119, February
Printed in U.SA.
POL YORCHIDISM ROBERT E. BUTZ
AND
JOHN H. CROUSHORE
From the Department of Urology, Grandview Hospital, Dayton, Ohio
ABSTRACT
The thirty-seventh case of polyorchidism is reported. A complete compilation of all cases of this rare congenital anomaly, including a brief historical review of the more important ones, is presented for future reference. Polyorchidism is a urologic curiosity. Previously reported cases have included patients with 3, 4 and even 5 testes. However, only cases of triorchidism have been verified histologically. Holder described a typical case of polyorchidism that was verified only clinically. His patient not only had 5 testes but also 3 scrotums, including 2 normal scrotums with 2 testes and a third rudimentary scrotum with 1 testis. 2 Clinical examination is inadequate for verification of SU· pernumerary testes. An intrascrotal mass, such as a spermatocele, cyst of Morgagni, testicular tumor or aberrant epididymis, can be mistaken for a third testis. Consequently, only histologically proved cases can be accepted as examples of polyorchidism.
tient urologic evaluation had yielded a positive culture of prostatic secretions. Chronic prostatitis was believed to be the etiology of the discomfort. Family history included a son who had chronic prostatitis and the anomaly of unilateral renal agenesis. Physical examination was normal except for the external genitalia, which included a normal right hemiscrotum and an abnormal left hemiscrotum with 2 masses. One mass was believed to be an atrophic testis and the other, an associated epididymal cyst. Laboratory examinations were within normal limits, including excretory urography and chromosomal analysis. Semen analysis revealed 116 times 106 sperm per cc with essentially normal morphology and 80 per cent motility. Cystoscopic
Fm. 1. Tangential ultrasonographic view of left hemiscrotal contents. Superior testis is on left and inferior testis is on right
Historically, Ashfeld generally is accepted as reporting the first histologically proved case of polyorchidism, which was discovered postmortem. 3 In 1895 Lane reported the first histologically proved case found at operation. 4 Numerous reviews of the incidence of polyorchidism have appeared in the literature with varying degrees of accuracy. The most enlightening ones have been by Edington and Blacklock,3 Lowsley and Porras, 5 and Wilson and Littler. 1 Since the review of 21 cases by Wilson and Littler in 1953 there have been 15 additional cases reported. CASE REPORT
W. C., a 44-year-old married laborer with 4 children, was hospitalized because of chronic perineal rliscomfort. Out_paAccepted for publication May 20, 1977.
examination revealed the positive findings of acute posterior urethritis and acute prostatitis. Transillumination of the left hemiscrotum at the time of cystoscopy failed to verify the presence of any cystic mass. Therefore, further evaluation of the left hemiscrotal masses was done, using a Litton ultrasound unit with a gray scale sonovision. A 13 mm. 2.5 MHz. near field transducer focused at 5 cm. was used. The inferior left hemiscrotal mass was reported as being an atrophic testis, while the superior mass was reported as cystic, measuring 1.7 cm. in depth by 2.5 cm. in diameter (fig. 1). Exploration of the left hemiscrotum revealed 2 testes within a single tunica vaginalis. Interconnecting the testes was a single cord-like structure believed to be an epididymis. The inferior testis had a vas deferens associated with it, while the superior testis had only a vascular supply to it (fig. 2). Biopsies of both testes revealed histologic verification oftestic289
290
BUTZ AND CROUSHORE
Reported cases References
Involved Side
Histology
Wilson and Littler' Holder Boggan, R. H.: Brit. J. Urol., 20: 630, 1953 Ashfeld: Cited by Edington and Blacklock3 Lassen: Cit.ed by Edington and Blacklock• Marriotti: Cited by Edington and Blacklock3 Lerat: Cit.ed by Edington and Blacklock3 Haas: Cit.ed by Edington and Blacklock3 Jeanin and Delat.er: Cited by Edington and Blacklock3 Lecene: Cited by Edington and Blacklock3 Oudendal: Cit.ed by Edington and Blacklock3 Edington and Blacklock3 Lane• King, E. S. J.: J. Anat., 65: 427, 1931 Goldstein, H. H. and Casilli, A. R.: J. Urol., 41: 583, 1939 Chevasu: Cited by Mehan and associates• Cranz: Cited by Mehan and associates• Thiessen, N. W.: J. Urol., 49: 710, 1943 Handley, R. S. and Crawford, T.: Brit. J. Surg., 31: 300, 1944 Ranson, F. T.: Brit. Med. J., 2: 137, 1949 Lowsley and Porras" Smith, P. G., Evans, A. T. and Suder, G. L.: J. Urol., 64: 515, 1950 Anderson, P. S. G.: Lancet, 1: 826, 1953 Carey, V. F.: J. Urol., 72: 703, 1954 Wait.e, V. C.: Surgery, 36: 971, 1954 Darrow, R. P. and Humes, J. J.: J. Urol., 72: 53, 1954 Golji, H.: J. Urol., 74: 207, 1955 Wescott, J. W. and Dykhuizen, R. F.: J. Urol., 98: 497, 1967 Hochberg, K.: Bruns Beitr. Klin. Chir., 216: 364, 1968 Corwin, S. H., Addonizio, J. and Nagamatsu, G. R.: J. Urol., 104: 346, 1970 Adams, J. R. and Jordon, W. P., Jr.: South. Med. J., 65: 594, 1972 Smart, R. H.: J. Urol., 107: 278, 1972 Lazarus, B. A. and Tessler, A. N.: Urology, 3: 615, 1974 Hakami, M. and Mosavy, S. H.: Brit. J. Surg., 63: 633, 1975 Mehan, D. J. and associates" Present study
Lt. Lt. Lt.
No spermatogenesis Normal No spermatogenesis
Lt.
Unknown
Rt.
Normal
Lt.
No spermatogenesis
Lt.
No spermatogenesis
Lt.
No spermatogenesis
Lt.
Normal
Rt.
No spermatogenesis
Abdominal
No spermatogenesis
Lt. Rt. Lt.
Normal Normal Spermatogenic arrest
Rt.
Normal
Lt.
Decreased spermatogenesis Tubular atrophy
Intra-abdominal Rt.
Sertoli cell only
Rt.
Normal
Rt.
Normal
Rt. Lt.
Tubular atrophy No spermatogenesis
Lt.
Spermatogenic arrest
Lt.
Tubular atrophy
Lt.
Tubular atrophy
Rt.
Normal
Lt. Rt.
No spermatogenesis Normal
Lt.
Germinal aplasia
Rt.
Normal
Lt.
Normal
Lt.
Normal
Lt.
No spermatogenesis
Rt.
Normal
Rt. Lt.
Normal Normal
Fm. 2. Duplicated left testis exposed for excisional biopsy. Superior testis is on left and inferior testis is on right. ular material with active spermatogenesis (fig. 3). Convalescence was uneventful. COMMENT
Polyorchidism is an interesting urogenital anomaly. The embryological explanation of supernumerary testes has been discussed adequately in the past by numerous authors. As yet no chromosomal aberration has been isolated to explain the congenital anomaly. Review articles have appeared in the literature with varying degrees of accuracy. Since the review by Wilson and Littler there has not been an accurate accounting of the actual number of cases of polyorchidism. To date, 36 cases of polyorchidism have been reported in the literature (see table). However the most recent article tabulated only 28. 6 Of the 36 cases testicular duplication occurred on the left side in 21. Spermatogenesis was present in 15 instances. Normal spermatogenesis appeared to be related to the position of the supernumerary organ. In most cases scrotal placement seemed to ensure normal spermatogenesis. REFERENCES
1. Wilson, W. A. and Littler, J.: Polyorchidism: report of two cases with torsion. Brit. J. Surg., 41: 302, 1953. 2. Holder, H. G.: A probable case oftriorchidism. J. Urol., 13: 555, 1925. 3. Edington, G. H. and Blacklock, J. W. S.: A case of duplication or subdivision of testicle. Brit. Med. J., 1: 937, 1928. 4. Lane, W. A.: A case of supernumerary testis. Trans. Clin. Soc. Lond., 28: 59, 1895. 5. Lowsley, 0. S. and Porras, E.: Congenital anomalies of the testicle. J. Int. Coll. Surg., 15: 332, 1951. 6. Mehan, D. J., Chehval, M. J. and Ullah, S.: Polyorchidism. J. Urol., 116: 530, 1976.
POLYORCHIDISM
291
Fm. 3. A, microscopic view of biopsy specimen taken from superior testis shows evidence of active spermatogenesis. B, microscopic view of biopsy specimen taken from inferior testis shows normal spermatogenesis.