Polyorchidism in children
We report a case of a very rare right-sided polyorchidism in a child, review the reported pediatric cases and discuss the management of this anomaly.
Authors: Dushi, Gezim; Ramseyer, Pascal; Meyrat, Blaise; Frey, Peter. CHUV, Pediatric Urology, Lausanne, Switzerland.
Corresponding Author: Peter Frey, CHUV, Pediatric Urology, Lausanne, Switzerland. Email: [email protected]
Polyorchidism, a rare anomaly, signifies presence of more than two testes. Since the first case, found on a routine autopsy by Blasius in 1670 , further 191 cases in a mixed adult and pediatric population were reported until recently . Most of the cases presented were left sided triorchidism.
After literature review, we could not find any specific analysis of polyorchidism in the pediatric population. We therefore report a case of a very rare right-sided polyorchidism in a child, review the reported pediatric cases and discuss the management of this anomaly.
A 13 year-old-boy presented at the out-patient clinic for investigation of a painless soft tissue mass in the right scrotum. The ultrasound (fig. 1) revealed two normal homogenous structures, being testes, in the right scrotum. The cranial one was bigger than the caudal and measured 1.8x2x2.7cm compared to the caudal, measuring 1.3×1.4×1.6cm. An epididymis attached to each testis was seen. There was only one testis on the left side, which was normal on ultrasound. MRI (fig.2) showed that the epididymi of each right-sided testis were attached to each other and only one vas deferens was seen. This presentation of polyorchidism is classified according to Leung  as type 3, to Singer  as type 1, to Thum  as type II and to Bergholz  as type A3.
The tumor markers alpha-fetoprotein, prostate-specific antigen and beta-human chorionic gonadotropin were negative. There was no surgery performed. Patients were followed-up, every 6 months, clinical examination was uneventful and annual ultrasound was normal 3 years after the diagnosis of polyorchidism. Clinical and ultrasound examination will be further performed in the future.
Figure 1. Ultrasound: Presence of two homogenous testes. The caudal testis has its own epidydimis (circle or arrow)
Figure 2. MRI: Sagittal view T2-weighted. Double testes on the right, sharing the same vas deferens. (arrow)
Using the combinations of key words “polyorchidism”,” triorchidism”, “supernumerary testes” “boy”, “child” we searched the electronic databases MEDLINE® and EMBASE®. Histologically proven cases of polyorchidism or cases diagnosed by imaging techniques were included in our study, the latest entry being in February 2011.
47 cases of polyorchidism were reported in children aged from 1 month to 16 years ( mean 6.8 years). Only one case of bilateral testicular duplication was reported.
There is a predominant 83% left-side presentation. 21 (42.5%) were discovered during surgical intervention for testicular maldescent, hernia in three (6.5%), testicular torsion in two (4%), pain in two (4%) cases, hydrocele in one (2%) and scrotal lymphangioma in one (2%) case. In 18 (38%) children the polyorchidism was found during the evaluation of a palpable scrotal mass from which 10 (55%) were operated. Presence of a seminoma and a teratoma was reported in two respective cases (4%).
Polyorchidism is a rare congenital malformation. The left-sided predominance encountered in adults (91.65% of cases)  was confirmed in the pediatric population where it was found in 83% of cases. Earlier reports of abnormalities associated with polyorchidism showed an association of 40% with maldescended testis, 30% with hernia, 15% with torsion, 9% with hydrocele and 6% with tumors in a mixed adult and pediatric population . In a more recent meta-analysis by Bergholz  the reported percentages were almost equal.
In the pediatric group, however, in the literature this was shown to be true only in the case of maldescended testis (42.5%) and tumors (4%). In all other associated anomalies the percentages were much lower. In children, scrotal pain leading to the discovery of polyorchidism was low (4%) and comparable to the one reported in the mixed population .
The reported presence of painless scrotal mass in children was high (38%), which is in accordance with earlier reported results  but in contrary to the recent meta-analysis  where only 16% of the patients presented with this feature.
With the advances in imaging techniques (US and MRI) the former practice of removing the supernumerary testes has changed. The conservative surveillance of polyorchidism in cases with a normal radiological appearance and negative tumor-markers has started to be accepted, especially in the pediatric population [8-13].
Despite this fact, we found that 55% of cases of polyorchidism in children, diagnosed by ultrasound for a painless scrotal mass, were operated on.
There are mainly two possible classifications of polyorchidism in the literature: the etiological [3,5] and, since the etiology is not clearly understood, the anatomical [4,6] classification was developed.
We propose a rather simple pragmatic approach in children with the aim to preserve their reproductive capacity and to avoid potential operation-related complications.
This management strategy is similar to the one published by Khedis et al , however, it has been simplified and in particular suggests an ultrasound to be performed on a regular and compulsory basis. Also it differs from the strategy suggested by Repetto et al , as we do not suggest performing repeated MRI.
In cases of polyorchidism recognised by the presence of a scrotal mass, and clearly identified by imaging techniques and with negative tumor markers, we propose conservative treatment, performing clinical and ultrasound examination at 6 monthly and yearly intervals respectively.
In cases of polyorchidism discovered during surgical intervention, we advocate orchidectomy if the supernumerary testis is atrophic, separated from the normal testis, or without connection to the vas deferens. These were formerly classified according to Leung as type 1, to Singer as type 2, to Thum as type I and to Bergholz as type B1 and 2.
In all the other cases, formerly classified according to Leung type 2,3 and 4, to Singer as type 1; to Thum as type II and III, and to Bergholz as type A1,2 and 3 we propose conservative management and clinical follow-up as described above.
Conclusions: The optimal management of polyorchidism is still a matter of debate. After literature review in children we propose whenever possible conservative management, however, these patients should be very closely followed up.
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Date added to bjui.org: 02/08/2011