Duplication of the testis with contralateral anorchism.

Abstract: We describe the case of a newborn infant with duplication of the testis and absence of the contralateral testis. The patient also had bilateral indirect inguinal hernias and an ipsilateral undescended testicle.

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Key Points:

* A newborn infant was found to have duplication of the testis and absence of the contralateral testis.

* The duplicated testes were equal size.

* The patient also had bilateral inguinal hernias and an ipsilateral undescended testis.

Case Report

A white male infant was born at 35 weeks' gestation to a healthy 22-year-old mother (gravida 3, para 2). The pregnancy was complicated by antepartum hemorrhage as a result of placenta previa. The infant was delivered by cesarean section because of fetal distress. The Apgar scores were 3 at 1 minute and 7 at 5 minutes. Birthweight was 2,470 g, and length was 48 cm. At birth, the infant was noted to have a right inguinal hernia. He had a testis in the right side of the scrotum, but no testis was found in the left side. At 2 weeks of age, he had repair of the right indirect inguinal hernia. During herniorrhaphy, another testis was found in the right inguinal canal. The two testes were of equal size. They had a separate epididymis but a common blood supply and a common vas deferens. The undescended testis was brought down and anchored to the right side of the scrotum by a retention suture. Ultrasonography of the abdomen performed when the infant was 1 month of age showed absence of the left testis; the kidneys were normal. Results of urinalysis and urine culture were normal. The patient was seen again at 2 years of age because of a left inguinal hernia. During the herniorrhaphy, the left side of the abdomen was explored by laparoscopy, and no testis was found.

Duplication of the testis and anorchism are clinical rarities. We describe a white infant with duplication of the testis on the right side and absence of the testis on the left side. We have found only one similar case reported in the literature. (1) Our patient is the youngest with such an association described in the literature. The patient also had bilateral indirect inguinal hernias and an ipsilateral undescended testis.

Discussion

Testicular duplication is a rare congenital anomaly. Ahlfeld noted the earliest observation, by Blasius in 1670, (2) who published the first histologically proved case found at autopsy in 1880. Lane, (3) in 1895, was the first to report this finding discovered intraoperatively.

Testicular duplication may result from a duplication of the genital ridge or longitudinal or transverse division of the genital ridge. (4) In the reported cases that mention size, the supernumerary testis usually has been smaller than the normal one, and the combined bulk of the affected side has approximated that of the unaffected side. (4) It is unusual to find duplicated testes of equal size. (5-7) This report adds another such case to the literature.

Duplication of the testis is usually asymptomatic. The most common presentations are the presence of two scrotal masses, inguinal swelling, undescended testis, and torsion of the supernumerary testis. (7) It may also be an incidental finding at the time of surgical exploration, (8) as illustrated in the present case.

Approximately 50% of the cases have been diagnosed between 15 and 25 years of age. (9) Rarely, the diagnosis is made in infancy. (8,10) Nistal et al (10) described an instance of testicular duplication in a newborn infant; the diagnosis was made at autopsy. The patient described by Khan (11) had a left-sided scrotal mass, present since birth, that was thought to be a spermatocele; the diagnosis of testicular duplication was not made until 32 years later. We think that ours is the first case in which the diagnosis was made in a live infant in the neonatal period.

Testicular duplication has to be distinguished from transverse testicular ectopia. In the latter case, the ectopic testis and the normal testis descended through the same inguinal canal and lie in the same side of the scrotum. (12) In transverse testicular ectopia, the two testes have two different sets of blood vessels and vasa deferens.

The two anomalies most commonly associated with polyorchidism are indirect inguinal hernia (30%) (4,13,14) and maldescended testis (15-30%), (13,14) as is illustrated in the present case. Other associated anomalies and complications include testicular torsion (13%), (13) hydrocele (9%), (13) varicocele (<1%), (15) hypospadias (<1%), (14) anomalous urogenital union (<1%), (14) and malignancy (<1%). (16)

True testicular agenesis is rare. (17,18) A vascular catastrophe as a result of torsion or vascular occlusion at the neck of processus vaginalis has been postulated to be the probable cause. (17) It is estimated that the incidence of monorchism is 1 in 5,000 males and that of anorchism, 1 in 20,000 males. (18) However, 3.4% of full-term infants have cryptorchidism. (19) The incidence decreases to 0.8% by 6 months of age. (19) If the testis has not descended by 6 months, it will remain undescended. (19) Therefore, we must consider any patient without a palpable testis as having cryptorchidism until proved otherwise. Failure to find a testis in the inguinal canal likewise cannot be considered an adequate basis for the diagnosis of testicular agenesis. Laparoscopy or surgical exploration of the abdomen is necessary for localization of the undescended testis, identification of testicular remnants, and confirmation of testicular agenesis. The diagnosis of testicular agenesis in the present case was made by laparoscopic exploration of the abdomen. Kalani and Purohit (1) reported the first case of duplication of testis with an absence of testis on the contralateral side. We report the second case of this association.

References

(1.) Kalani BP, Purohit AD. Duplication of the testis. J Pediatr Surg 1972; 7:73.

(2.) Ahlfeld F. Die Missbildungen des Menschen. Leipzig, Grunow, 1880.

(3.) Lane WA. A case of supernumerary testis. Trans Clin Soc Lond 1895; 28:59 60.

(4.) Leung AK. Polyorchidism. Am Fam Physician 1988;38:153-156.

(5.) Thiessen NW. Polyorchidism: Report of a case. J Urol 1943;49:710-714.

(6.) Nocks BN. Polyorchidism with normal spermatogenesis and equal sized testes: A theory of embryological development. J Urol 1978;120:638-640.

(7.) Haddock G, Burns HJ. Polyorchidism. Postgrad Med J 1987;63:703-705.

(8.) Shah SN, Miller BM, Geisler E. Polyorchidism discovered as testicular torsion. Urology 1992;39:543-544.

(9.) Sujka SK, Relabate JA, Smith RA. Polyorchidism. Urology 1987;29: 307 309.

(10.) Nistal M, Paniagua R, Martin-Lopez R. Polyorchidism in a newborn: Case report and review of the literature. Pediatr Pathol 1990;10:601-607.

(11.) Khan C. Polyorchidism with normal spermatogenesis. Br J Urol 1988; 61:100.

(12.) Sastry SC, Venkateswarlu K, Hussain BA. Transverse testicular ectopia. Int Surg 1974;59:373-374.

(13.) Kale N, Basaklar AC. Polyorchidism. J Pediatr Surg 1991;26:1432-1434.

(14.) Besner GE, Qualman SJ, Gosche JR. Identification of a duplicated undescended testicle (polyorchia): An unexpected benefit of laparoscopic localization. J Laparoendosc Surg 1996;6(Suppl 1):S107-S114.

(15.) Tammela TL, Mattila SI, Hellstrom PA, Makarainen HP. Polyorchidism with normal spermatogenesis diagnosed preoperatively with ultrasound. Scand J Urol Nephrol 1989;23:71 73.

16. Kulkarni JN, Bhansali MS, Tongaonkar HB, Kamat MR, Borges AM. Carcinoma in the third testis in a case of polyorchidism and persistent Mullerian structure syndrome. Eur Urol 1992;22:174-176.

(17.) Rai M, Agrawal JK, Sasikumar V, Singh SK. Bilateral congenital anorchia in three siblings. Clin Pediatr (Phila) 1994;33:367-369.

(18.) Brothers LR III, Weber CH Jr, Ball TP Jr. Anorchism versus cryptorchidism: the importance of a diligent search for intra-abdominal testes. J Urol 1978;119:707 708.

(19.) Elder JS. Undescended testes, in Behrman RE, Kliegman RM, Jenson HB (eds): Nelson Textbook of Pediatrics. Philadelphia, W.B. Saunders Co., 2000, ed 16, pp 1650-1651.

From the Departments of Pediatrics and Surgery, University of Calgary, and the Alberta Children's Hospital, Calgary, AB, Canada.

Reprint requests to Alexander K.C. Leung, MBBS. No. 200, 233 16th Avenue NW, Calgary, Alberta T2M 0H5, Canada. Email: [e-mail]

Accepted March 18, 2002.