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Polyorchidism
Polyorchidism By Nuri Kale and A. Can Bagaklar Ankara, Turkey l Polyorchidism, defined as the presence of more than two histologically proven testes, is a rare entity. A review of the literature has yielded 46 cases of true polyorchidism suitable for consideration in this review, and we add two more, one of them being the 12th case of complete duplication of the testis reported in the literature. Copyright o 1991 by W.B. Saunders Company INDEX testis.
WORDS:
Polyorchidism;
triorchidism,
supernumerary
P
OLYORCHIDISM is an unusual genital abnormality in which supernumerary testes are present. The location of the extra testis varies from below the normal testicle within the scrotum along the inguinal canal to retroperitoneum. To date, 46 histologically proven cases of true polyorchidism have been documented in the English language literature”’ and we add two more. Complete duplication including a totally separate testis, epididymis, vas deferens, and blood supply has been noted in 11 cases*4’5’“-‘4 One of the presented cases is the 12th case of complete duplication of the testis reported in the literature. The recent attendance by the authors of two patients with three testes has stimulated this case report and review of the literature. CASE
Histologically, the excised and biopsied tissues showed normal testicular tissue in both cases. DISCUSSION
The first living case confirmed histologically was reported by Arbuthnot Lane in 1895, though as early as 1670 and 1880, two previous cases were noted at autopsy by Blasius and Ahfeld, respectively.‘4 In the previous reviews of the literature, Boggon,’ Mehan et al,* Pelander et aJ3 and Hancock and Hodgins’ described 62 cases including their own patients. However, Hancock and Hodgins have accepted 40 of these cases suitable for consideration because of the inadequate information and the lack of histological proof.’ Since then, 6 further cases have been reported, increasing the total number of the reported cases to 46 and we add two more.4’6-‘o The youngest patient in whom polyorchidism has been reported was a 4-year-old boy, whereas the oldest was 75 years old.3 The accessory testicle can present as a scrotal mass or a mass in the inguinal canal due to the testicular mass itself or the associated with indirect inguinal hernia. The most common presentation is the presence of a scrotal mass. According to Hancock’s classification, the site of the accessory testicular mass was scrotum in 30, inguinal canal in 9, and retroperitoneum in 5 of the reported cases, including our
REPORTS
Two male children, 6 and 5 years old, respectively, with left-sided undescended testis were scheduled for orchiopexy. On examination, the left hemiscrotum was empty in both children and the gonads were palpable within the inguinal canal. When the inguinal canal was exposed in the first case, two testes with completely separate epididymii, spermatic cords and vessels, and a hernia sac were identified (Fig 1). The spermatic cords and the vessels of both testes were remaining separate as high as the limits of the surgical field. One of the testes was quite small and orchiectomy was performed on this testis. The other testis was biopsied and orchiopexy was performed. The hernia was repaired by high ligation. In the second case, both of the testicular masses were equal in size, shared a common epididymis, and drained by a common vas deferens (Fig 2). Incisional biopsy specimens were obtained and individual orchiopexy was then performed.
From the Department of Pediatric Surgery, Gazi of Medicine, Ankara, Turkey. Address reprint requests to A. Can Basaklar, Caddesi 488/15, Kizilay, Ankara, Turkey. Copyright o 1991 by W B. Saunders Company 0022-3468/91/2612-0028$03.00/0
1432
University MD,
School
MithatpaJa Fig 1. spermatic
Complete
duplication
of the left testis,
epididymis,
and the
cord in case 1.
JaurnalofPediatricSurgery,
Vol26,
No 12 (December),
1991: pp 1432-1434
1433
POLYORCHIDISM
48
cases
I nadequate nformation
I
/
L
retroperitoneal
scrotal
5
30 inguinal
9 Fig 2. epididymis
Both testes were of equal size and spermatic cord in case 2.
and
shared
a common
patients (Fig 3). In 32 of the 44 cases, involvement was on the left side and bilateral involvement was present in one report.6 In 15% to 50% of the cases, the accessory testicle is associated with cryptorchidism and in 30% with indirect inguinal hernia.3S’6 Torsion of either or both testes also may occur in 13% of the patients.‘,2,‘0,‘6-19 Other entities associated with polyorchidism include hydrocele in 9%, epididymitis, varicocele, and malig-
Fig 4. Hancock’s classification of reported cases of polyorchidism (including the present cases) based on the anatomic relationships among the testes, epididymii, and was deferens; rps, retroperitoneal space. (Modified with permission.‘)
Fig 3. Classification of reported cases of polyorchidism :he present cases) based on site of accessory testis.
(including
rant neoplasms in 1% of the cases.“.13.20 However, the Jreoperative diagnosis in these patients requires a righ index of suspicion. Details of the anatomic relations between the :estis, epididymis, and vas deferens of the reported :ases are shown in Fig 4. Two masses of testicular issue were joined together in some fashion by a single epididymis and drained by a single vas deferens i n 17 patients. Two separate and noncommunicating
1434
KALE AND
masses of testicular tissue with varying anatomic relationships of the epididymis and vas deferens were present in 24 cases. Among this group, only one of the testis was drained by an epididymis and vas deferens and the other testes lacked an epididymis or vas deferens or both in 9 patients. In the remaining 15 patients, each mass of testicular tissue was drained by its own epididymis and vas deferens, the vas deferens remaining separate as high as the limits of the surgical field in 12 patients and joining together within the operative field to make a single vas deferens in 3 patients. Necessary details of anatomic relationships
BASAKLAR
between the testis and its structures were not available in 7 of the reported cases. The removal of both masses or, more commonly, the smaller mass of the testicular tissue was practiced in the earlier reports, but biopsy and replacement of both masses of testicular tissue is the current practice. Orchiopexy should be performed to prevent torsion and to facilitate subsequent examination for malignant changes. To maximize the potential for fertility in young patients, all normal-appearing gonads should be left in place.
REFERENCES 1. Boggon RH: Polyorchidism. Br J Surg 20:630,1933 2. Mehan DJ, Chehval MJ, Ullah S: Polyorchidism. J Urol 116:530-532,1976 3. Pelander WM, Luna G, Lilly JR: Polyorchidism: Case report and literature review. J Urol 119:705-706,1978 4. Al-Habbal Z, Izzidien AY: Polyorchidism: Case report and review of the literature. J Pediatr Surg 19:212,1984 5. Hancock RA, Hodgins TE: Polyorchidism. Urology 24:303307,1984 6. Snow BW, Tarry WF, Duckett JW: Polyorchidism: An unusual case. J Urol133:483,1985 7. Gandia VM, Arrizabalaga M, Leiva 0, et al: Polyorchidism discovered as a testicular torsion associated with an undescended atrophic contralateral testis: A surgical solution. J Ural 137:734735,1987 8. Haddock G, Burns HJG: Polyorchidism. Postgrad Med J 63:703-705,1987 9. Giyanani VL, McCarthy J, Venable DD, et al: Ultrasound of polyorchidism: Case report and literature review. J Urol 138:863864,1987 10. Rogus BJ: Polyorchidism and testicular torsion. Urology 31:137,1988
11. Thiessen NW: Polyorchidism: Report of a case. J Urol 49:710-714,1943 12. Wescott JW, Dykhuizen RF: Polyorchidism. J Urol 98:497, 1967 13. Grechi G, Zampi GC, Selli C, et al: Polyorchidism and seminoma in a child. J Urol 123:291-292,198O 14. Lane A: A case of supernumerary testis. Trans Clin Sot Lond 28:59,1895 15. Necks BN: Polyorchidism with normal spermatogenesis and equal sized testes: A theory of embryological development. J Urol 120:638-639,1978 16. Feldman S, Drach GW: Polyorchidism discovered as testicular torsion. J Urol130:976, 1983 17. Wilson WA, Littler J: Polyorchidism: Report of two cases with torsion. Br J Surg 41:302-303,1953 18. Waite VC: Polyorchidism: A case report. Surgery 36:971, 1954 19. Corwin SH, Addonizio J, Nagamatsu GR: Polyorchidism with anomalous urogenital union. J Urol104:346, 1970 20. Scott KWM: A case of polyorchidism with testicular teratoma. J Urol124:930,1980
WORDS:
Polyorchidism;
triorchidism,
supernumerary
P
OLYORCHIDISM is an unusual genital abnormality in which supernumerary testes are present. The location of the extra testis varies from below the normal testicle within the scrotum along the inguinal canal to retroperitoneum. To date, 46 histologically proven cases of true polyorchidism have been documented in the English language literature”’ and we add two more. Complete duplication including a totally separate testis, epididymis, vas deferens, and blood supply has been noted in 11 cases*4’5’“-‘4 One of the presented cases is the 12th case of complete duplication of the testis reported in the literature. The recent attendance by the authors of two patients with three testes has stimulated this case report and review of the literature. CASE
Histologically, the excised and biopsied tissues showed normal testicular tissue in both cases. DISCUSSION
The first living case confirmed histologically was reported by Arbuthnot Lane in 1895, though as early as 1670 and 1880, two previous cases were noted at autopsy by Blasius and Ahfeld, respectively.‘4 In the previous reviews of the literature, Boggon,’ Mehan et al,* Pelander et aJ3 and Hancock and Hodgins’ described 62 cases including their own patients. However, Hancock and Hodgins have accepted 40 of these cases suitable for consideration because of the inadequate information and the lack of histological proof.’ Since then, 6 further cases have been reported, increasing the total number of the reported cases to 46 and we add two more.4’6-‘o The youngest patient in whom polyorchidism has been reported was a 4-year-old boy, whereas the oldest was 75 years old.3 The accessory testicle can present as a scrotal mass or a mass in the inguinal canal due to the testicular mass itself or the associated with indirect inguinal hernia. The most common presentation is the presence of a scrotal mass. According to Hancock’s classification, the site of the accessory testicular mass was scrotum in 30, inguinal canal in 9, and retroperitoneum in 5 of the reported cases, including our
REPORTS
Two male children, 6 and 5 years old, respectively, with left-sided undescended testis were scheduled for orchiopexy. On examination, the left hemiscrotum was empty in both children and the gonads were palpable within the inguinal canal. When the inguinal canal was exposed in the first case, two testes with completely separate epididymii, spermatic cords and vessels, and a hernia sac were identified (Fig 1). The spermatic cords and the vessels of both testes were remaining separate as high as the limits of the surgical field. One of the testes was quite small and orchiectomy was performed on this testis. The other testis was biopsied and orchiopexy was performed. The hernia was repaired by high ligation. In the second case, both of the testicular masses were equal in size, shared a common epididymis, and drained by a common vas deferens (Fig 2). Incisional biopsy specimens were obtained and individual orchiopexy was then performed.
From the Department of Pediatric Surgery, Gazi of Medicine, Ankara, Turkey. Address reprint requests to A. Can Basaklar, Caddesi 488/15, Kizilay, Ankara, Turkey. Copyright o 1991 by W B. Saunders Company 0022-3468/91/2612-0028$03.00/0
1432
University MD,
School
MithatpaJa Fig 1. spermatic
Complete
duplication
of the left testis,
epididymis,
and the
cord in case 1.
JaurnalofPediatricSurgery,
Vol26,
No 12 (December),
1991: pp 1432-1434
1433
POLYORCHIDISM
48
cases
I nadequate nformation
I
/
L
retroperitoneal
scrotal
5
30 inguinal
9 Fig 2. epididymis
Both testes were of equal size and spermatic cord in case 2.
and
shared
a common
patients (Fig 3). In 32 of the 44 cases, involvement was on the left side and bilateral involvement was present in one report.6 In 15% to 50% of the cases, the accessory testicle is associated with cryptorchidism and in 30% with indirect inguinal hernia.3S’6 Torsion of either or both testes also may occur in 13% of the patients.‘,2,‘0,‘6-19 Other entities associated with polyorchidism include hydrocele in 9%, epididymitis, varicocele, and malig-
Fig 4. Hancock’s classification of reported cases of polyorchidism (including the present cases) based on the anatomic relationships among the testes, epididymii, and was deferens; rps, retroperitoneal space. (Modified with permission.‘)
Fig 3. Classification of reported cases of polyorchidism :he present cases) based on site of accessory testis.
(including
rant neoplasms in 1% of the cases.“.13.20 However, the Jreoperative diagnosis in these patients requires a righ index of suspicion. Details of the anatomic relations between the :estis, epididymis, and vas deferens of the reported :ases are shown in Fig 4. Two masses of testicular issue were joined together in some fashion by a single epididymis and drained by a single vas deferens i n 17 patients. Two separate and noncommunicating
1434
KALE AND
masses of testicular tissue with varying anatomic relationships of the epididymis and vas deferens were present in 24 cases. Among this group, only one of the testis was drained by an epididymis and vas deferens and the other testes lacked an epididymis or vas deferens or both in 9 patients. In the remaining 15 patients, each mass of testicular tissue was drained by its own epididymis and vas deferens, the vas deferens remaining separate as high as the limits of the surgical field in 12 patients and joining together within the operative field to make a single vas deferens in 3 patients. Necessary details of anatomic relationships
BASAKLAR
between the testis and its structures were not available in 7 of the reported cases. The removal of both masses or, more commonly, the smaller mass of the testicular tissue was practiced in the earlier reports, but biopsy and replacement of both masses of testicular tissue is the current practice. Orchiopexy should be performed to prevent torsion and to facilitate subsequent examination for malignant changes. To maximize the potential for fertility in young patients, all normal-appearing gonads should be left in place.
REFERENCES 1. Boggon RH: Polyorchidism. Br J Surg 20:630,1933 2. Mehan DJ, Chehval MJ, Ullah S: Polyorchidism. J Urol 116:530-532,1976 3. Pelander WM, Luna G, Lilly JR: Polyorchidism: Case report and literature review. J Urol 119:705-706,1978 4. Al-Habbal Z, Izzidien AY: Polyorchidism: Case report and review of the literature. J Pediatr Surg 19:212,1984 5. Hancock RA, Hodgins TE: Polyorchidism. Urology 24:303307,1984 6. Snow BW, Tarry WF, Duckett JW: Polyorchidism: An unusual case. J Urol133:483,1985 7. Gandia VM, Arrizabalaga M, Leiva 0, et al: Polyorchidism discovered as a testicular torsion associated with an undescended atrophic contralateral testis: A surgical solution. J Ural 137:734735,1987 8. Haddock G, Burns HJG: Polyorchidism. Postgrad Med J 63:703-705,1987 9. Giyanani VL, McCarthy J, Venable DD, et al: Ultrasound of polyorchidism: Case report and literature review. J Urol 138:863864,1987 10. Rogus BJ: Polyorchidism and testicular torsion. Urology 31:137,1988
11. Thiessen NW: Polyorchidism: Report of a case. J Urol 49:710-714,1943 12. Wescott JW, Dykhuizen RF: Polyorchidism. J Urol 98:497, 1967 13. Grechi G, Zampi GC, Selli C, et al: Polyorchidism and seminoma in a child. J Urol 123:291-292,198O 14. Lane A: A case of supernumerary testis. Trans Clin Sot Lond 28:59,1895 15. Necks BN: Polyorchidism with normal spermatogenesis and equal sized testes: A theory of embryological development. J Urol 120:638-639,1978 16. Feldman S, Drach GW: Polyorchidism discovered as testicular torsion. J Urol130:976, 1983 17. Wilson WA, Littler J: Polyorchidism: Report of two cases with torsion. Br J Surg 41:302-303,1953 18. Waite VC: Polyorchidism: A case report. Surgery 36:971, 1954 19. Corwin SH, Addonizio J, Nagamatsu GR: Polyorchidism with anomalous urogenital union. J Urol104:346, 1970 20. Scott KWM: A case of polyorchidism with testicular teratoma. J Urol124:930,1980