Title: Quadruple Orchidopexy for Torsion Testis in an Adolescent with Polyorchidism: a Case Report

Transcription

1 Accepted Manuscript Title: Quadruple Orchidopexy for Torsion Testis in an Adolescent with Polyorchidism: a Case Report Author: Hany Ibrahim, Matthew J. Roberts, David Hussey PII: S (15) DOI: /j.urology Reference: URL To appear in: Urology Received date: Accepted date: Please cite this article as: Hany Ibrahim, Matthew J. Roberts, David Hussey, Quadruple Orchidopexy for Torsion Testis in an Adolescent with Polyorchidism: a Case Report, Urology (2015), /j.urology This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

2 Quadruple orchidopexy for torsion testis in an adolescent with polyorchidism: a case report Hany Ibrahim 1, Matthew J. Roberts 2-4, David Hussey 2 1 Department of General Surgery, The Prince Charles Hospital, Brisbane, Australia 2 Department of Urology, The Royal Brisbane and Women s Hospital, Brisbane, Australia 3 School of Medicine, The University of Queensland, Herston, Australia 4 Centre for Clinical Research, The University of Queensland, Herston, Australia Corresponding author: Dr Matthew Roberts Department of Urology, The Royal Brisbane and Women s Hospital, Brisbane, Australia, m.roberts2@uq.edu.au Abstract We report a case of testicular torsion in an adolescent with polyorchidism, specifically quadorchidism. Sonographic and intra-operative images are provided to describe the presentation and management of this case, while the challenges and pitfalls with this clinical scenario are discussed. Keywords; testicular torsion, congenital abnormality, polyorchidism Word Count Abstract 40; Manuscript 936 (excluding references, figure legends) 1 Page 1 of 8

3 Polyorchidism represents a congenital disorder resulting in the presence of supernumerary testicle/s, with the most commonly reported variant being tri-orchidism in asymptomatic patients. While the prevalence of torsion with anatomically normal testes is 0.025%, 1 testicular torsion in the presence of polyorchidism is exceedingly rare. Polyorchidism with four testes has been reported for eight patients to date. 2-4 Here, we report a unique case of testicular torsion in an adolescent with four testes which was successfully preserved intraoperatively. This case outlines the challenges faced with diagnosis and management of this condition. CASE REPORT A healthy 14-year-old boy presented to the Emergency Department with a two hour history of atraumatic, first-episode left scrotal pain following a football game. He denied any recent or concurrent fever, urinary symptoms, nausea and family or travel history. His birth history was uncomplicated following conception using in vitro fertilisation (IVF). On physical examination, his vital signs were within normal limits and he appeared well, alert and comfortable with a soft, non-tender abdomen without organomegaly or abdominal herniation. External genitalia examination revealed no hypospadias, rash or other abnormality, as well as no scrotal swelling, erythema, hydrocele or inguinal lymphadenopathy. On examination of the left hemiscrotum, the patient was uncomfortable 2 Page 2 of 8

4 due to moderate generalised tenderness, while two separate, mobile masses were palpable in each hemiscrotum, of which the left superior mass was firm. On repeated examination, the initially superior firm mass was palpated in the inferior hemiscrotum, indicating unrestricted movement. Leucocytosis (13.9 x 10 9 /L) and neutrophilia (11.24 x 10 9 /L) were demonstrated in otherwise normal serum investigations. A urinalysis was not performed. Scrotal ultrasonography demonstrated polyorchidism, specifically two testes located within each hemi-scrotum (Figure 1A). Vascularity was absent and reduced in the upper and lower left testes, respectively (Figure 1B and 1C). Following informed consent and general anaesthesia, scrotal exploration including complete opening of the tunica vaginalis was performed. Intraoperatively, the sonographic findings were confirmed to be a result of clockwise 720⁰ torsion of the upper left testis (Figure 2A), which appeared ischaemic. The testis was connected to a smaller inferior testis and ductus deferens by a long epididymis, with duplicated vascular supply observed (Figure 2B). This anatomical arrangement was present bilaterally (Figure 2B and 2C). Following de-torsion and warming, the left upper testis was deemed viable. Three-point orchidopexy was performed with a vertically-oriented (superior-, mid- and inferior-pole) approach using an absorbable braided suture (3.0 Vicryl ) on all four testes, which were fixed to the tunica vaginalis in close approximation nearest to the most natural configuration. The patient experienced an uncomplicated recovery and outpatient abdominal and renal tract ultrasound did not demonstrate further congenital or other abnormality. 3 Page 3 of 8

5 COMMENT The aetiology of polyorchidism may be from aberrant division of the genital ridge in early gonadogenesis before 8 weeks gestation (Figure 3). 2,5 Anatomical and functional classification models have been proposed based on the presence of testicular drainage by ductus deferens and arrangement of the epididymis. 2, 6 Anatomically, this case illustrates a bilateral type A3 (Bergholz) or type II (Leung) polyorchidism (Figure 2D). Based on the Leung classification, it is hypothesised that this case is the result of bilateral genital ridge division at the primordial gonads without affecting the mesonephros and metanephric ducts. 6, 7 Functionally, this case represents a Singer type 1A, being that the intrascrotal polyorchid testes are drained by a vasoepididymis and have reproductive potential. 6 While polyorchidism is rare, more than 200 cases have been reported to date. According to a meta-analysis of 191 cases in 2009, 2 triorchidism (95%) and left sided anomalies (65%) occurred most commonly, with malignancy detected in 5% of histologically confirmed cases, predominantly located in a maldescended position. Other associated anomalies include inguinal hernia (30%) and maldescended testis (22%), while other congenital anomalies (hypospadias, varicocoele and anomalous urogenital union) were uncommon. Testicular torsion occurred in 13% of cases, which considering the small population size, is considerably higher than general incidence. The bell clapper deformity, where the tunica vaginalis attaches higher than usual, is central to the aetiology of testicular torsion. This 4 Page 4 of 8

6 developmental defect may be more pronounced in polyorchidism with a greater testicular volume transmitted with the peritoneum through the inguinal canal. This developmental aberration intuitively predisposes these patients to a higher incidence of testicular torsion. 7 This was a feature of our case, with regular positional change of the testes between serial examinations. Furthermore, conception via IVF and later development of polyorchidism in this case may be associated, although evidence for this is scarce. Management of asymptomatic uncomplicated polyorchidism usually comprises a conservative approach with regular ultrasonography and follow-up. 3 As the reproductive potential of a supernumerary testis is estimated to be 50-65%, 8 testicular preservation is encouraged. 7 However, with concurrent undescended testes (inguinal or intra-abdominal; Singer type B), excision of the affected testis should be considered due to high risk of malignant transformation. When scrotal exploration is performed, orchidopexy is encouraged to minimise risk of future testicular torsion. In our case, orchidopexy of four testicles was challenging owing to uncertainty of important anatomical landmarks, such as the sinus of the epididymis, and available surface area of the tunica vaginalis. We experienced that allowing the testes to return to their resting position provided the most appropriate location and were accordingly fixated to the tunica vaginalis. Reconstructive vasoepididymal approximation using a non-absorbable suture has been suggested to facilitate later vaso-epididymostomy if infertility ensues in these cases. 7 In conclusion, torsion of a supernumery testis in the presence of polyorchidism represents a highly unusual but very serious clinical situation. An awareness of this congenital 5 Page 5 of 8

7 abnormality, the operative challenges and management options may assist urologists in managing these patients in their practice. Acknowledgements: MJR is supported by a Doctor in Training Research Scholarship from Avant Mutual Group Ltd., Cancer Council Queensland PhD Scholarship and Professor William Burnett Research Fellowship from the Discipline of Surgery, School of Medicine, The University of Queensland. Conflicts of interest: none References 1. Barada JH, Weingarten JL, Cromie WJ. Testicular salvage and age-related delay in the presentation of testicular torsion. J Urol. 1989;142: Bergholz R, Wenke K. Polyorchidism: a meta-analysis. J Urol. 2009;182: Alamsahebpour A, Hidas G, Kaplan A, McAleer IM. Bilateral polyorchidism with diffuse microlithiasis: a case report of an adolescent with 4 testes. Urology. 2013;82: Ellett JD, Rosoff JS, Prasad MM. Three testicles in one hemiscrotum: an unusual presentation of polyorchidism. BMJ Case Rep. 2015;2015: bcr Lawrentschuk N, MacGregor RJ. Polyorchidism: a case report and review of the literature. ANZ J Surg. 2004;74: Leung AK. Polyorchidism. Am Fam Physician. 1988;38: Page 6 of 8

8 7. Kajbafzadeh AM, Ghahestani SM, Zamani MM, Kajbafzadeh M, Kia MM. A novel approach in eight polyorchidism cases: vasoepidydimal or epididymo-epididymal approximation in single vas deferens duplicated testis. Urology. 2012;79: Spranger R, Gunst M, Kuhn M. Polyorchidism: a strange anomaly with unsuspected properties. J Urol. 2002;168: Page 7 of 8

9 Figure legends Figure 1: Scrotal ultrasonography; A left hemiscrotum (longitudinal section) with corresponding Doppler measurement, demonstrating absent blood flow in upper left testis and diminished vascularity in left lower testis; B right hemiscrotum (longitudinal section) demonstrating two testes; C transverse section across upper scrotum showing right and left upper testes, demonstrating normal and reduced/absent blood flow, respectively. Figure 2: Intraoperative photography; A left upper testis on initial scrotal exploration demonstrating 720⁰ torsion; B right hemiscrotal contents demonstrating polyorchidism; C left hemiscrotal contents following detorsion demonstrating polyorchidism and relationship of accessory structures (epididymis, ductus deferens, neurovascular bundles); D schematic representation of panel C demonstrating anatomical variation. Figure 3: Schematic drawing demonstrating the proposed anatomical development of polyorchidism in this case. The relationship of primitive embryonal structures (genital ridge, mesonephric tubules and duct, and Müllerian duct) and potential level of division to form a polyorchid hemiscrotum, containing duplicated testes sharing epididymis (adapted from Lawrentschuk et al. 5 ) 8 Page 8 of 8