|Year : 2019 | Volume
| Issue : 4 | Page : 288-290
Transverse testicular ectopia with fused vas deferens: A case series
Sivasankar Jayakumar, Meena Agrawal
Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St. Thomas' Hospitals NHS Foundation Trust, London, UK
|Date of Web Publication||29-Aug-2019|
Dr. Sivasankar Jayakumar
Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St. Thomas' Hospitals NHS Foundation Trust, London
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Transverse testicular ectopia (TTE) is a rare anomaly where both gonads descend towards the same side of the hemiscrotum. Although more than 100 cases of TTE are reported, TTE with fused vas are extremely rare. We present our series of 4 TTE patients with fused vas [4, 7, 9 and 26 months old]. Three cases were diagnosed at the time of inguinal exploration and one during a planned laparoscopic inguinal hernia repair. An indirect inguinal hernia with TTE was noted with a fused proximal vas deferens in all 4 cases [Figure 1]. Inguinal herniotomy, ipsilateral orchidopexy along with contralateral transeptal orchidopexy (testis brought down through the inguinal canal on the same side of hernia) [Figure 2] was performed in all patients without any complication. None of the patients had uterine tissue. Testicular biopsies, Array and karyotyping (46XY) were normal in all 4 patients. Follow-up ultrasound doppler scans revealed normal testes with good vascularity in all patients. Fused vas with TTE is rare but transeptal orchidopexy is feasible with excellent outcome.
Keywords: Crossed testicular ectopia, fused vas, trans-septal orchidopexy, transverse testicular ectopia
|How to cite this article:|
Jayakumar S, Agrawal M. Transverse testicular ectopia with fused vas deferens: A case series. J Indian Assoc Pediatr Surg 2019;24:288-90
|How to cite this URL:|
Jayakumar S, Agrawal M. Transverse testicular ectopia with fused vas deferens: A case series. J Indian Assoc Pediatr Surg [serial online] 2019 [cited 2020 Apr 9];24:288-90. Available from: http://www.jiaps.com/text.asp?2019/24/4/288/265694
| Introduction|| |
Transverse testicular ectopia (TTE), also called as crossed testicular ectopia, is a rare anomaly first described by Von Lenhossek in 1886. More than 100 cases have been reported in the English literature so far, but TTE with fused vas has rarely been reported. TTE was initially classified into three types [Table 1] based on the associated findings. Type 1 is the most common type (40%–50%) of TTE associated with inguinal hernia, followed by Type 2 (30%) TTE associated with persistent Mullerian ducts (PMDs) and the least common Type 3 (20%) TTE associated with other anomalies such as hypospadias. Preoperative ultrasound and magnetic resonance imaging can help diagnose TTE. However, many cases are often diagnosed intraoperatively during inguinal exploration for herniotomy or laparoscopy. We present a series of four cases with TTE with proximal fused vas, diagnosed intraoperatively and managed successfully with transeptal orchidopexy.
|Table 1: Classification of (crossed) transverse testicular ectopia (Esteeves et al., Sau Paulo Med J, 1995)|
Click here to view
| Methods|| |
This was a retrospective review of medical records of children with TTE and fused vas deferens managed between 2000 and 2015 at our institution. Data were collected on age at diagnosis, intraoperative findings, type of TTE, and follow-up.
| Case Series|| |
Four patients were identified (aged 4, 7, 9, and 26 months) during the study period of 5 years. All four patients presented with unilateral inguinal hernia; palpable ipsilateral undescended testis and impalpable contralateral testis. Preoperative karyotyping (46XY) and array were normal in all four patients. Preoperative ultrasound examination showed left-sided inguinal hernia in two patients and right-sided inguinal hernia in two patients with ipsilateral testis seen but failed to identify the contralateral impalpable testis in all four patients. Inguinal exploration was performed in three patients and the TTE diagnosed at the time of inguinal exploration. The last case was diagnosed during a planned laparoscopic inguinal hernia repair.
An indirect inguinal hernia with TTE was noted with an intraperitoneal contralateral testis and a fused proximal vas deferens in the pelvis [Figure 1] in all four patients. Biopsy of the impalpable testis was done for the first case which showed no evidence of malignancy and was reported as normal testicular tissue. All three patients underwent inguinal exploration, inguinal herniotomy, and ipsilateral orchidopexy along with contralateral trans-septal orchidopexy (testis brought down through the inguinal canal on the side of hernia but placed into the corresponding hemiscrotum) through an extended herniotomy incision [Figure 2]. In the last case, after diagnosis of TTE by laparoscopy, inguinal exploration was done and a similar procedure to previous three cases was performed. The patient characteristics of all four patients are presented in [Table 2]. Follow-up with Doppler ultrasound scans revealed normal testes with good vascularity in all four patients at 18 months, 3 years, 10 years, and 12 years.
|Figure 1: Intraoperative picture of case 1 during transverse testicular ectopia repair|
Click here to view
|Figure 2: Intraoperative picture of case 1 after trans-septal orchidopexy|
Click here to view
| Discussion|| |
Several theories have been postulated regarding the etiology of TTE. Berg suggested that both testes may arise from the same genital ridge and this leads to TTE. However, Gupta and Das suggested that mesonephric and Mullerian ducts adhere and fail to separate. Defective or ruptured gubernaculum was also suggested as a cause of TTE. Familial incidence of TTE has been reported between two brothers, suggesting a possible genetic etiology as well. The exact etiology of TTE still remains unclear. Karyotyping is essential, as XX maleness, ovotesticular disorders of sex development, and TTE are reported together.
Laparoscopy helps to establish the anatomy including identifying PMD remnants and fused vas, which could also be done by open inguinal exploration. Mobilization of fused cord or vas and excising PMD remnants are challenging, and extended inguinal exploration with trans-septal orchidopexy (Ombredanne or modified procedure) [Figure 3] is often necessary; however, this has been managed laparoscopically as well., Laparoscopy is now being increasingly used for diagnosis and correction of TTE and PMD remnants excision. Frequently, the vas and the PMD remnants are joined together, but laparoscopic dissection of these structures is feasible and can minimize the damage to vas. PMD remnants should always be excised as there is a risk of malignancy, and the testes should be monitored regularly as at least 6% of TTE is reportedly associated with testicular tumors, usually seminomas.
|Figure 3: (a) Illustration showing left testicular ectopia with right inguinal hernia, (b) Illustration showing left transverse testicular ectopia fixed by trans-septal (modified Ombredanne procedure) orchidopexy|
Click here to view
Usually, the cords of both testis are fused, which includes both the vas and testicular vessels. However, in our cases, the testicular vessels were separate, and only the proximal vas was noted fused. Only five cases of proximal fused vas have been reported so far (one of them not associated with TTE), and our case series reports four such cases of Type 1 TTE with proximal fused vas being seen in all four patients. Separation of the fused cord or vas is not advised, and a conservative approach is suggested with trans-septal orchidopexy alone. This has resulted in all our four cases with good vascularity seen on Doppler scan at follow-up even after 12 years.
| Conclusion|| |
Fused vas with TTE is rare, but trans-septal orchidopexy is feasible with excellent outcome. Long-term follow-up is essential as there is a small but known risk of malignancy.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Von Lenhossek MN. Ectopia testis transversa. Anat Anz 1886;1:376-81.
Esteves E, Pinus J, Maranhão RF, Abib Sde C, Pinus J. Crossed testicular ectopia. Sao Paulo Med J 1995;113:935-40.
Gauderer MW, Grisoni ER, Stellato TA, Ponsky JL, Izant RJ Jr. Transverse testicular ectopia. J Pediatr Surg 1982;17:43-7.
Bothra JM, Shah HS, Jayaswal S, Sandlas G. Transverse testicular ectopia: A rare anomaly. J Pediatr Neonatal Care 2014;1:12.
Hughes DT, Croitoru DP. Case report: Crossed testicular ectopia. J Pediatr Surg 2007;42:1620-2.
Berg AA. VIII. Transverse ectopy of the testis. Ann Surg 1904;40:223-4.
Gupta RL, Das P. Ectopia testis transversa. J Indian Med Assoc 1960;35:547-9.
Zivanović D, Slavković A, Madić J, Novaković D. Transverse testicular ectopia. Srp Arh Celok Lek 2004;132:438-40.
Hisamatsu E, Takagi S, Nakagawa Y, Sugita Y. Familial transverse testicular ectopia unrelated to persistent Müllerian duct syndrome. Indian J Urol 2011;27:397-8.
] [Full text]
Maciel-Guerra AT, de Mello MP, Coeli FB, Ribeiro ML, Miranda ML, Marques-de-Faria AP, et al.
XX maleness and XX true hermaphroditism in SRY-negative monozygotic twins: Additional evidence for a common origin. J Clin Endocrinol Metab 2008;93:339-43.
Kamble RS, Gupta RK, Gupta AR, Kothari PR, Dikshit KV, Kesan KK, et al.
Laparoscopic management of transverse testicular ectopia with persistent mullerian duct syndrome. J Minim Access Surg 2015;11:213-5.
Alamsahebpour A, Blachman-Braun R, Gupta A, Castellan M, Campos SJ, Gosalbez R, et al.
Laparoscopy and transseptal orchiopexy in the management of transverse testicular ectopia. Curr Urol Rep 2015;16:48.
Shinmura Y, Yokoi T, Tsutsui Y. A case of clear cell adenocarcinoma of the Müllerian duct in persistent Müllerian duct syndrome: The first reported case. Am J Surg Pathol 2002;26:1231-4.
Yamada K, Takahata A, Ichijo Y, Akazawa K, Goto M, Terayama K, et al.
Acase of testicular seminoma in persistent Mullerian duct syndrome with transverse testicular ectopia. Abdom Imaging 2015;40:475-9.
Chacko JK, Furness PD 3rd
, Mingin GC. Presentation of fused vas deferens. Urology 2006;67:1085.e17-8.
[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2]