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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]

Introduction
Polyorchidism, a rare anomaly, signifies presence of more than two testes. Since the first case, found on a routine autopsy by Blasius in 1670 [1], further 191 cases in a mixed adult and pediatric population were reported until recently [2]. 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.

 

Case report
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 [3] as type 3, to Singer [4] as type 1, to Thum [5] as type II and to Bergholz [6] 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)

 

 

Literature review
 
Methods
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.

 

Results
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%).

 

Discussion
Polyorchidism is a rare congenital malformation. The left-sided predominance encountered in adults  (91.65% of cases) [2] 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 [7]. In a more recent meta-analysis by Bergholz [2] 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 [2].
The reported presence of painless scrotal mass in children was high (38%), which is in accordance with earlier reported results [3] but in contrary to the recent meta-analysis [2] 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 [14], 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 [8], 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.

 

References
 
1.  Ahlfeld F. Die Missbildungen des Menschen. Grunow, Leipzig 1880: 126.
2. Bergholz R, Wenke K. J Urol.  2009  182 : 2422-2427.
3. Leung AK. Am Fam Physician. 1988  38 : 153-156.
4. Singer BR, Donaldson JG, Jackson DS. Urology.  1992  39 : 384-388.
5. Thum G. Polyorchidism: case report and review of literature. J  Urol.  1991 145 : 370-372. 6. Bergholz R, Koch B, Spieker T, Lohse K. Polyorchidism: a case report and classification.J Pediatr Surg.  2007 42 : 1933-1935.
7. Yeniyol CÖ, Nergiz N, Tuna A. Abdominal polyorchidism:A case report and review of the literature. Int Urol Nephrol. 2004  36: 407-408.
8. Repetto P, Ceccarelli P, Bianchini A, Durante V, Biondini D, Cacciari A. Three small testes in left hemiscrotum: a rarer case of polyorchidism. J Pediatr Surg.  2010 45: E21-E23.
9. Savas M, Yeni E Ciftci H, Cece H, Topal U, Utangac MM.  Polyorchidism:  a three-case report and review of literature. Andrologia.  2009  42 : 57-61.
10. Danrad R, Ashker L, Smith W. Polyorchidism: Imaging may denote reproductive potential of accessory testicle. Pediatr Radiol.  2004  34 : 492-494.
11.Hunald FA, Rakototiana AF, Razafimanjato N, Tsiaviry P, Ahmad A, Rantomalala HYH. Un cas de polyorchidie: revue de la littérature.  Arch Pediatr.  2008  15: 1430-1432
12. Bhogal HR, Palit A, Prasad KK. Conservative management of polyorchidism in a young man: a case report and review of literature. Pediatr Surg Int.   2007  23 : 689-691.
13. Park HY, Moon HS. Polyorchidism.  Kor J Urol.  2005 46 : 536-538.
14. Khedis M, Nohra J, Dierickx L et al. Urol Int. 2008 80: 98-101.

 
Date added to bjui.org: 02/08/2011 


DOI: 10.1002/BJUIw-2011-043-web

 

Incomplete bladder duplication in a male child

We present a case of incomplete bladder duplication in a male child with no other associated anomalies and review the literature pertaining to this rare anomaly.

Authors: Dushi, Gezim; Ramseyer, Pascal; Osterheld, Maria-Chiara; Meyrat, Blaise; Frey, Peter. CHUV, Lausanne, Switzerland.

Corresponding Author: Peter Frey, MD, BSc, CHUV, Pediatric Urology, Lausanne, Switzerland.    Email: [email protected]

 

Introduction
Incomplete bladder duplication is an extremely rare condition. Since Cattirri reported the first case in 1670 [1] only 7 further cases have been reported in the literature [1-3]. Incomplete bladder duplication is often associated with other genitourinary anomalies [2,3] and can even be associated with bladder exstrophy [4]. We present a case of incomplete bladder duplication in a male child with no other associated anomalies and review the literature pertaining to this rare anomaly.

This case resembled closely that of a patient we attended not too long ago. He had bladder complications that seemed to have developed from not going to the bathroom as often as he needed. He was as frequent visitor of the casino SBOBET that is nearby to our medical center. We helped him with his condition and of course gave him multiple instructions to follow so that this would not happen again.

Case report
A 9 year-old-boy presented at the out-patient clinic after two episodes of asymptomatic lower urinary tract infections. However, he was suffering from voiding difficulties, characterized by urge incontinence, poor urinary stream and the necessity to raise the abdominal pressure in order to initiate voiding. Physical examination was uneventful. A single urethral meatus was observed. Ultrasound, showed two hypoechogenic structures in the pelvis,  communicating with each other. The kidneys appeared of normal size and morphology. Voiding cysto-urethrography confirmed the presence of these two communicating structures, lying adjacent to each other in the coronal plane; the left structure was slightly postero-lateral to the structure on the right, which was supposed to be the main bladder, the latter in communication with a single urethra (Figure 1 A).

 

Figure 1. A. Preoperative VCUG showing the two bladder structures in a latero-lateral position, the right-sided structure being in communication with a single urethra. B. VCUG, showing the reconstructed main bladder after excision of the left-sided structure and re-implantation of the left ureter in a coronal and C. in a sagittal view.
A. Preoperative VCUG showing the two bladder structures in a latero-lateral position, the right-sided structure being in communication with a single urethra. B. VCUG, showing the reconstructed main bladder after excision of the left-sided structure and re-implantation of the left ureter in a coronal and C. in a sagittal view.

 

No vesicoureteral reflux was seen. During this procedure the boy could not void despite a filling volume of 600 ml and the entire urethra, therefore, could not be imaged. To further delineate the nature of the left structure we performed magnetic resonance imaging (MRI) (Figure 2 A and B).

 

Figure 2. MRI: A.Coronal, B.Saggital T2-weighted view of the latero-lateral incomplete bladder duplication.
 MRI: A.Coronal, B.Saggital T2-weighted view of the latero-lateral incomplete bladder duplication.

 

This structure measured 11 cm in the cranio-caudal axis, 11,2 cm in the antero-posterior and 6 cm in the transverse axis. The communication between the two structures was located on the left infero-lateral side of the supposed main bladder and measured 1,5 cm in diameter.  Implantation site of the ureters could not be distinguished.
Cystoscopy was performed. The presence of a single urethra was confirmed and no posterior urethra valves could be detected. The ureteral meatus could not be identified on either side.
On surgery the two structures were identified, each containing a separate ureteric orifice meatus. The right ureteric orifice, draining into the main bladder, lay in a normal position. The left ureteric orifice was identified to be lying in a postero-superior part of the left structure (Figure 3 and 4).

 

Figure 3. Intraoperative view of the latero-lateral bladder structure and its ureter
Intraoperative view of the latero-lateral bladder structure and its ureter

 

Figure 4. Schematic presentation of incomplete bladder duplication
Schematic presentation of incomplete bladder duplication
The left ureter was completely mobilized and thereafter re-implanted into the right bladder, applying a modified Cohen procedure. The left structure was excised and the isthmus closed with two layers of running sutures. The excised specimen was fixed in formalin for histologic evaluation. Histology showed the presence of urothelial, smooth muscle and serosal layers. Immunohistochemically the smooth muscle layer stained positive with anti-a-smooth muscle actin (Figure 5).

 

Figure 5. Immunohistology of the excised left bladder structure, applying monoclonal anti-smooth muscle a-actin antibodies – peroxidase reaction to demonstrate the presence of smooth muscle. (X100)

 

Three months after surgery, voiding cystourethrography was repeated. It showed the absence of vesicoureteral reflux and a bladder capacity of approximately 250 ml. The presence of a slightly ectatic prostatic urethra was noted and voiding was complete (Figure 1B and C). The patient showed normal urinary continence and no further urinary tract infection was detected.

 

Discussion and literature review
In 1961, Abrahamson [1] classified bladder duplication as complete or incomplete. With complete duplication, two bladders are present with separated walls of mucosal and muscular layers, and each one empties through its own urethra. The final diagnosis is made by simultaneous retrograde urethrocystograms [5]. In incomplete bladder duplication, two bladders communicate with each other and drain into a common urethra. Duplication of the bladder may occur in a sagittal or coronal plane given the axis of the septum. The sagittal variant appears to be predominant compared to the coronal one, with a ratio of 2.5:1. According to published literature, the male to female ratio of this anomaly is described to be equal [1, 5-8]. Females are more likely to present the sagittal type of duplication and in males there is no predominance.
The aetiology of bladder duplication, despite the hypotheses proposed by Abrahamson [1], remains obscure. More recently Voigt [8] raised the question of whether the aetiology can be explained by the existence of a human homologue of the mouse mutant disorganization gene first described by Hummel in 1959. [9]
Complete bladder duplication is, in the majority of cases, associated with other anomalies. 40 cases of complete bladder duplication were reviewed by Kossow and Morales in 1973 [10]. They found associated duplication of the lower gastrointestinal tract in 42%, external genitalia in 90% and spina bifida, meningocele or myelomengocele in 15% of cases. Similar findings, except a lower percentage of associated duplication of the external genitalia (30%), were reported by Berrocal et al [11]. Associated urogenital or non-urogenital anomalies seem to depend on the axis of the septum. Non-urogenital anomalies seem to be predominantly present in complete bladder duplication with a saggital septum, while urogenital anomalies are more common in complete duplication with a coronal septum [6, 11-13]. After literature review, only six cases of complete bladder duplication without any associated anomalies were reported [7].
Of the previously reported 8 cases of incomplete bladder duplication, five presented with other anomalies and three were anomaly-free. In our patient, the ninth reported case, no any additional anomalies were present either. The histology of the excised left structure from our patient showed the typical findings of the bladder wall, comprising urothelial, smooth muscle and serosal layers. In particular the existence of bladder smooth muscles, could be proven by positive specific anti-a-smooth muscle-actin immunohistochemistry findings. Furthermore primary urothelial and smooth muscle cell cultures could be established in-vitro with the excised tissue, thus also proving the existence of bladder duplication.
The treatment of bladder duplication cannot be standardized, due to the great variations in presentation. We agree with statements in the literature, that treatment should aim at optimization of bladder function, addressing urinary continence and minimising the risk of infections [1, 2, 7, 8], which was the initial presentation in our case.

 

References
1.  Abrahamson J. Double bladder and related anomalies: clinical and embryological aspects and a case report. J Urol.  1961 33:195-212.
2.  Evangelidis A, Murphy JP, Gatti JM.  Incomplete bladder duplication presenting antenatally. Urology.  2004 64: 589.e3-589.e5.
3.  Metzger R, Schuster T, Stehr M, Pfluger T, Dietz HG. Incomplete duplication of the bladder.  Eur J Pediatr Surg. 2004 14: 203-205.
4.  Perren F, Frey P. The exstrophy-epispadias complex in the duplicated lower urinary tract. J Urol.  1998 159: 1681-1683.
5.  Dajani AM, El-Muhtasseb H, Kamal MF. Complete duplication of the bladder and urethra. J  Urol.  1992 147: 1079-1080.
6.  Bae KS, Jeon SH, Lee SJ, Lee CH, Chang SG, Lim JW, Kim JI. Complete duplication of bladder and urethra in coronal plane with no other anomalies: case report with review of the literature. Urology.  2005 65: 388.e12-388e13.
7.  Coker AM, Allshouse MJ, Koyle MA. Complete duplication of bladder and urethra in a sagittal plane in a male infant: case report and literature review. J Pediatr Urol.  2008 4: 255-259.
8.  Voigt HR, Wentzel SW. Complete duplication of the bladder, urethra and external genitalia in a male neonate with an imperforate anus.  Int  J Urol.  2005 12: 702-704.
9.  Hummel KP.  The inheritance and expression of disorganization, an unusual mutation in the mouse. J Exp Zool.  1958 137: 389–423.

 

Date added to bjui.org: 14/04/2011 


DOI: 10.1002/BJUIw-2010-095-web

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