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/lp/springer-journals/management-of-a-very-rare-case-of-polyorchidism-a-case-report-WS8i0H10y1
- Publisher
- Springer Journals
- Copyright
- Copyright © The Author(s) 2023
- eISSN
- 2090-5394
- DOI
- 10.1186/s43159-023-00245-z
- Publisher site
- See Article on Publisher Site
Abstract
Background Polyorchidism is a rare genetic anomaly characterized by the patient having more than two testicles. In the literature, only around two hundred cases have been reported. We present an unusual new case which does not conform to the traditional classifications of polyorchidism or previously reported cases. We discuss our diagnostic techniques as well as management decisions for this case and aim to raise awareness about the management choices available. Case report A 3-year-old male presented to the clinic by his parents due to concern of a painless swelling in the left inguinal region. Physical examination revealed features of a left indirect inguinal hernia. Furthermore, scrotal examina- tion revealed a firm, non-compressible painless mass in the left scrotum which was inferior to the left testis. Ultra- sonography suggested the mass as an extra testis, and MRI was followed which showed no signs of malignancy. Due to the reassuring nature of these findings, a conservative approach was taken, with the patient referred for regular follow-up. Conclusion When facing cases of polyorchidism, physicians should move away from considering surgical excision and biopsy as exclusive first-line management. Instead, we place emphasize and raise awareness about the option of conservative management if imaging shows no abnormalities. Ultimately, the aim of this paper is to raise awareness among the paediatric surgeon community that while excision may be a valid option, it is not the only treatment. Keywords Polyorchidism, Scrotum, Testes, Ultrasound, Paediatric surgery, Urology mass near the left testis which on surgical exploration Background was revealed to be an additional testicle. We reviewed the Polyorchidism or supernumerary testicle is an uncom- literature for polyorchidism and discuss the current man- mon congenital anomaly characterized by more than two agement strategies and methods for diagnosis. testicles. In the literature, only around two hundred cases have been reported so far [1]. Hereby, we report a case of Case presentation a 3-year-old boy who presented with a left inguinal her- A 3-year-old male was brought to the clinic by his parents nia and was coincidentally found to have an additional with the concern of a painless swelling in the left inguinal region which was incidentally noticed by the father since Wissam Jamal Al Tamr and Kareem Omran should be considered joint first the age of 3 months. authors. Physical examination revealed features of left indi- *Correspondence: rect inguinal hernia. Furthermore, scrotal examination Kareem Omran revealed a firm, non-compressible painless mass in the ko378@cam.ac.uk NMC Royal (Al-Zahra) Hospital, Sharjah, United Arab Emirates left scrotum which was inferior to the left testis. The Department of Public Health and Primary Care, University of Cambridge, right testis was normal in size and position and in terms Cambridge CB2 1TN, UK 3 of consistency. No varicocele or lymphadenopathy was Al Zahra Hospital, Sharjah, United Arab Emirates detected on either side. © The Author(s) 2023. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http:// creat iveco mmons. org/ licen ses/ by/4. 0/. Al Tamr et al. Annals of Pediatric Surgery (2023) 19:11 Page 2 of 4 Fig. 1 An ultrasound examination of the scrotum revealed another small homogeneous tissue in the left scrotal sac measuring 0.67 × 3.48 cm near the left testis An ultrasound examination of the scrotum revealed with two testes in the left scrotum. The superior testis bilateral testicular microlithiasis with left inguino-scrotal was larger in size (0.90 × 0.94 × 1.19 cm) with a volume fatty hernia. It was noticed that there was another small of 0.52 ml. It did not have an epididymis and vas defer- homogeneous tissue in the left scrotal sac measuring 0.67 ens; however, it had a normal blood supply. The inferior × 3.48 cm (Fig. 1). An MRI of the abdomen and pelvis testis was smaller in size (0.67 × 3.48 cm) and had an followed. This was to check for any signs of malignancy, epididymis along with a vas deferens (Fig. 2). The patient but it revealed no additional abnormalities. An alpha underwent left inguinal hernia repair along with orchi- fetoprotein level was taken, as it may be a marker of dopexy for both testes in the left scrotum, to prevent malignancy; however, this was also normal. future cases of torsion. The patient was then taken for surgery to repair the left A biopsy was deemed to be unnecessary due to the inguinal hernia. It was observed to be an indirect hernia, small nature of the extra testis. Moreover, the lack of Fig. 2 During the surgery, we found a left indirect inguinal hernia along with two testes in the left scrotum. Superior testis was larger in size and was without epididymis or vas deferens but had a normal blood supply. The inferior testis was smaller in size and had an epididymis along with vas deferens A l Tamr et al. Annals of Pediatric Surgery (2023) 19:11 Page 3 of 4 any gross abnormalities of the tissue and normal imag- fixation of the testes to avoid torsion. Furthermore, it ing made conservative management more assuring. The can also provide information regarding if the testis has management plan was discussed with the parents prior an outflow tract and if the testis can contribute to the to surgery, and it was decided that close follow-up in the spermatogenesis [7]. Definitive indications of orchidec - future for the early detection of any associated complica- tomy are malignant or dysplastic changes on biopsy, tions was ideal. ultrasonography suggestive of malignancy, absent sper- matogenesis, or situations where regular follow-up is not reliable [7]. Non-scrotal location of polyorchid- Discussion ism is considered as the most important risk factor for Polyorchidism/supernumerary testes is defined as the malignancy [5]. As such, we suggest that when planning presence of more than two testes and is an extremely for management of this condition, surgeons and pae- rare entity. Triorchidism, i.e. three testes, is one of the diatricians alike must not only consider the radiological commonest forms of polyorchidism where the extra findings but also the gross anatomy as well as societal testis is usually found on the left side [2]. Furthermore, barriers and availability of follow-up. polyorchidism has been classified into four distinct types by Singer et al. [3], depending on the presence of an epididymis or a vas deferens. Although our index patient Conclusion carries similarities to a “type D” (the supernumerary tes- When facing cases of polyorchidism testes, physicians ticle has complete duplication of testicles), the lack of should move away from considering surgical excision epididymis and vas deferens makes it a unique case to the and biopsy as exclusive first-line management. Instead, literature. we place emphasize and raise awareness about the Polyorchidism is typically asymptomatic, with pres- option of conservative management if imaging shows entation to healthcare being attributed to a mass being no abnormalities, and the patient can be readily fol- detected incidentally. However, sometimes, supernu- lowed up. Furthermore, we would like to emphasize the merary testes are brought to clinical attention due to need for a scrotal examination in all paediatric patients associated complications such as maldescent (40%), tor- presenting with signs of inguinal hernias, as this is a sion (15%), inguinal hernias (30%), hydrocoele (9%), and common manifestation for polyorchidism. With the malignancy (6%) [3]. The majority of documented malig - varying nature of this anomaly, adequate and proper nancies are of the testicular origin and, in rare instances, imaging using MRI is essential for screening for malig- rhabdomyosarcoma of the cremasteric muscle [4]. Most nancy. However, the success of ultrasonography as both of these testicular malignancies were detected in the first-line imaging as well as a diagnostic tool for extra undescended extra testicle [5]. testes cannot be neglected. To investigate this genetic anomaly, first-line imaging is ultrasound [6]. Magnetic resonance imaging should Acknowledgements then be followed for better visualization of signs of malig- Not applicable. nancy. Clinical examination alone is not sufficient for a Authors’ contributions diagnosis to be made. On ultrasound, supernumerary Dr. Wissam Jamal Al Tamr: senior surgeon who operated the case and testes can be visualized as freely mobile, well-defined supervised the write up. Kareem Omran: editing/write-up of manuscript and literature review. Dr. Rajebhosale Prashan: senior physician involved in the care ovoid structure with an eco-structure like that of normal of patient and write up of manuscript. Dr. Agraw al Pooja: senior physician testes, and Doppler can be used to determine presence of involved in the care of patient and review of manuscript. The authors read and blood flow [6]. approved the final manuscript. Management of polyorchidism has been vastly dis- Funding cussed and debated. Some authors suggest for close Not applicable. follow-up, whereas others recommend excision of the Availability of data and materials extra testicle considering its malignant potential [4]. Available upon request. Recently, with advancement in monitoring through MRI and ultrasound imaging, a more conservative Declarations approach can be followed [5]. Conservative approaches involve the diagnosis and monitoring of the supernu- Ethics approval and consent to participate Not applicable. merary testis with the help of high-resolution sonogra- phy and MRI. If there is no concomitant disorder and a Consent for publication testicular tumor is ruled out on imaging, surgical explo- Written informed consent was obtained from the patient to publish this report in accordance with the journal’s patient consent policy. ration with biopsy is not required [7]. On the other hand, surgical exploration allows biopsy along with Al Tamr et al. Annals of Pediatric Surgery (2023) 19:11 Page 4 of 4 Competing interests The authors declare no competing interests. Received: 7 November 2022 Accepted: 11 February 2023 References 1. Artul S, Habib G. Polyorchidism: two case reports and a review of the literature. J Med Case Rep. 2014;8:464. 2. Arslanoglu A, Tuncel SA, Hamarat M. Polyorchidism: color Doppler ultra- sonography and magnetic resonance imaging findings. Clin Imaging. 2013;37(1):189–91. 3. Singer BR, Donaldson JG, Jackson DS. Polyorchidism: functional classifica- tion and management strategy. Urology. 1992;39(4):384–8. 4. Mandalia U, Pakdemirli E. A case of triorchidism. Radiol Case Rep. 2020;15(9):1643–5. 5. Bergholz R, Wenke K. Polyorchidism: a meta-analysis. J Urol. 2009;182(5):2422–7. 6. Aitharaju V, Drevna DW, Barr RG. Polyorchidism: a review of the literature and case report of a third testicle presenting as an inguinal hernia. Ultrasound Q. 2022;38(3):222–3. https:// doi. org/ 10. 1097/ ruq. 00000 00000 000584 PMID: 35001028. 7. Thum G. Polyorchidism: case report and review of literature. J Urol. 1991;145(2):370–2. Publisher’s Note Springer Nature remains neutral with regard to jurisdictional claims in pub- lished maps and institutional affiliations.
Journal
Annals of Pediatric Surgery – Springer Journals
Published: Mar 2, 2023
Keywords: Polyorchidism; Scrotum; Testes; Ultrasound; Paediatric surgery; Urology