Journal of Pediatric Urology (2009) 5, 234e236
CASE REPORT
Transverse testicular ectopia and persistent ¨llerian duct syndrome Mu ´rgio Leite Ottoni, Antonio Macedo Jr*, Ubirajara Barroso Jr, Se Valdemar Ortiz Department of Urology, Federal University of Sa˜o Paulo, Sa˜o Paulo, Brazil Received 7 October 2008; accepted 28 November 2008 Available online 5 February 2009
Introduction Transverse testicular ectopia (TTE) is the rarest form of testicular ectopia with fewer than 100 cases reported in the literature [1]. TTE is associated with persistent Mu ¨llerian duct syndrome (PMDS) in approximately 20% of cases, but this association is even rarer in children [2]. Karnak et al. reported the ninth case of TTE and PMDS in children [1]. The main clinical characteristics are
unilateral cryptorchidism and a contralateral inguinal hernia. The diagnosis is often made incidentally during surgery. Dean and Shah emphasize the importance of laparoscopy in these cases [3]. Here, we report a case of a young boy with TTE and PMDS preoperatively diagnosed by ultrasound, and in whom treatment was assisted by laparoscopy.
Case report A 1-year-old boy presented with bilateral cryptorchidism. The left testicle was not palpable and the right one was
Figure 1 Sonogram showing two testicles on the right hemiscrotum. * Corresponding author. Rua Maestro Cardim, 560/215, 01323-000 Sa ˜o Paulo, Brazil. Tel.: þ55 11 32870639; fax: þ55 11 32873954. E-mail address:
[email protected] (A. Macedo Jr.).
Figure 2 The Mu ¨llerian remnant was excised through an inguinal incision, splitting both testes.
1477-5131/$34 ª 2008 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jpurol.2008.11.012
Mu ¨llerian duct syndrome
Figure 3
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Macroscopic aspect of Mu ¨llerian remnant and histological analysis of rudimentary uterus.
palpable in its ipsilateral inguinal canal. Ultrasound identified two testicles on the right hemiscrotum (Fig. 1). Laparoscopy revealed uterus and two testicles entering the right internal inguinal orifice and persistence of the peritoneal/vaginal conduit. An inguinal incision was performed and the Mu ¨llerian remnant was excised (Fig. 2). Two small scrotal incisions were made and a Kelly clamp was passed to the abdominal cavity through the left hemiscrotal incision in order to bring down the left testicle. This was guided by laparoscopy. On the right side the herniorrhaphy was performed. Both testicles were fixed to the scrotum after biopsy. Histological analysis confirmed the presence of a rudimentary uterus (Fig. 3) and no testicular histological abnormality. Postoperative course was uneventful.
procedure to correct the persistence of the peritoneum vaginal conduit. Extraction of the Mu ¨llerian structures is not mandatory because it can cause lesion of the spermatic chord. In our case, the uterus was not firmly attached to the cord and spermatic vessel and therefore was removed, although we admit that extensive dissection should be avoided. Transeptal fixation of testicle, keeping both the chord and spermatic vessels at the same inguinal canal, is a valid option, but can put both gonads and their structures at risk of damage by trauma and infection. We prefer to perform a translocation of the crossed testis to its inguinal canal. This maneuver was guided by laparoscopy and the testicle could be brought down to the scrotum easily.
Appendix Discussion TTE is a rare event and even rarer in children when associated with PMDS with only a few cases reported in the literature [4]. Hypotheses for PMDS etiology include failure of synthesis or release of Mu ¨llerian inhibiting substance (MIS), failure of end organs to respond to MIS, or a defect in the timing of release of MIS. The relationship between TTE and PMDS is not clear since the role of MIS in testicular descent is not well understood. It seems that MIS increases gubernaculum activity [2]. Inguinal hernia usually accompanies TTE, but the condition may be difficult to diagnose correctly. We report the case of a young boy with TTE suspected by ultrasound. Laparoscopy was performed to better evaluate the internal genital organs; it was useful also in passing the crossed testicle to its corrected side. We acknowledge that all dissection procedures could have been done by laparoscopy. However, the inguinal approach assisted by laparoscopy is a fast and straightforward
Figure A1 Laparoscopic view of the left testis (LT), Mullerian duct (MD) and right testis crossing the internal inguinal orifice.
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References [1] Karnak I, Tanyel FC, Akcoren Z, Hic ¸sonmez A. Transverse testicular ectopia with persistent mullerian duct syndrome. J Pediatr Surg 1997;32:1362e4.
A. Macedo Jr. et al. [2] Hutson JM, Hasthorpe S. Testicular descent and cryptorchidism: the state of the art in 2004. J Pediatr Surg 2005;40:297e302. [3] Dean GE, Shah SK. Laparoscopic assisted correction of transverse testicular ectopia. J Urol 1817;2002:167. [4] Hammoudi S. Transverse testicular ectopia. J Pediatr Surg 1989; 24:223e4.
