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CASE REPORT
Transverse testicular ectopia and persistent
Mu
¨llerian duct syndrome
Antonio Macedo Jr*, Ubirajara Barroso Jr, Se
´rgio Leite Ottoni,
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
¨l-
lerian 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.
Figure 2 The Mu
¨llerian remnant was excised through an
inguinal incision, splitting both testes.
* Corresponding author. Rua Maestro Cardim, 560/215, 01323-000
Sa
˜o Paulo, Brazil. Tel.: þ55 11 32870639; fax: þ55 11 32873954.
E-mail address: macedo.dcir@epm.br (A. Macedo Jr.).
1477-5131/$34 ª2008 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.jpurol.2008.11.012
Journal of Pediatric Urology (2009) 5, 234e236
palpable in its ipsilateral inguinal canal. Ultrasound iden-
tified 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 per-
formed 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 tes-
ticle. 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.
Discussion
TTE is a rare event and even rarer in children when asso-
ciated 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 accom-
panies 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
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 sper-
matic 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.
Figure 3 Macroscopic aspect of Mu
¨llerian remnant and histological analysis of rudimentary uterus.
Appendix
Figure A1 Laparoscopic view of the left testis (LT), Mullerian
duct (MD) and right testis crossing the internal inguinal orifice.
Mu
¨llerian duct syndrome 235
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.
[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 trans-
verse testicular ectopia. J Urol 1817;2002:167.
[4] Hammoudi S. Transverse testicular ectopia. J Pediatr Surg 1989;
24:223e4.
236 A. Macedo Jr. et al.
