Crossed testicular ectopia, also named transverse testicular ectopia, is a rare but well-known congenital anomaly in which both gonads migrate toward the same hemiscrotum. It is usually associated with other abnormalities such as persistent Mu¨ llerian duct syndrome, inguinal hernia, hypospadias, pseudohermaphrodism, and scrotal anomalies. We report two cases of crossed testicular ectopia; a persistent Mullerian duct syndrome was associated in one case. We also performed a literature search for other reports of crossed testicular ectopia.Keywords: crossed testicular ectopia, ectopic testis, persistent Mullerian duct syndrome, testis, transverse testicular ectopi

Boukesra, Tarek

Dhaou, Mahdi B.

Jallouli, Mohamed

Kotti, Ahmed

Meftah, Souhir

Mhiri, Riadh

English

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Crossed testicular ectopia: what should be specified?Tarek Boukesraa, Ahmed Kottib, Mahdi B. Dhaoub, Souhir Meftahb,Mohamed Jalloulib and Riadh MhiribCrossed testicular ectopia, also named transversetesticular ectopia, is a rare but well-known congenitalanomaly in which both gonads migrate toward the samehemiscrotum. It is usually associated with otherabnormalities such as persistent Müllerian duct syndrome,inguinal hernia, hypospadias, pseudohermaphrodism, andscrotal anomalies. We report two cases of crossedtesticular ectopia; a persistent Müllerian duct syndromewas associated in one case. We also performed a literaturesearch for other reports of crossed testicular ectopia. AnnPediatr Surg 13:110–112 c 2017 Annals of PediatricSurgery.Annals of Pediatric Surgery 2017, 13:110–112Keywords: crossed testicular ectopia, ectopic testis, persistent Müllerianduct syndrome, testis, transverse testicular ectopiaaDepartment of Pediatric Surgery B, Children Hospital Bechir Hamza, Tunis andbDepartment of Pediatric Surgery, Hedi Chaker Hospital, Sfax, TunisiaCorrespondence to Tarek Boukesra, MD, Department of Pediatric Surgery B,Children Hospital Bechir Hamza, Tunis 1006, TunisiaTel: + 216 225 64473; fax: + 216 742 41384; e-mail: tarekboukesra@yahoo.frReceived 1 September 2014 accepted 22 June 2016IntroductionTesticular ectopia is an anomaly of testicular descentcharacterized by localization of the testis out of its normalmigration pathway from the retroperitoneum to thescrotum.In crossed testicular ectopia (CTE), the ectopic testis isfound in the opposite groin or hemiscrotum, beside theother testis. CTE is a very rare congenital anomaly, whichis characterized by a symptomatic inguinal hernia, with anonpalpable testis in the controlateral side. PersistantMüllerian duct syndrome (PMDS) is also a rare anomalythat may be associated with CTE. PMDS is an inheritedform of intersex disorder characterized by the presence ofMüllerian derivatives in otherwise phenotypically normalmales [1].We report two cases of CTE, in which diagnosis was madepreoperatively.Case 1A 1-year-old boy presented with a left inguinal hernia andright nonpalpable testis. A crossed testicular ectopia wassuspected clinically, and ultrasonography examination wasperformed. It showed crossed testicular ectopia on theleft side with hydrocele; no other anomalies weredetected. Left inguinal herniotomy showed a PMDS:two left testes, two fallopian tubes, and Müllerian cavities(Figs 1 and 2). Müllerian remnants were resected. Eachtestis had separate vessels, and both testes had passedthrough the left inguinal ring after ligation of theperitoneal sac. Fixation of the right testis was performedin the right hemiscrotum after trans-septal passage(Fig. 3). His karyotype was 46 XY.Case 2A 2-year-old boy presented with a right inguinal herniaand nonpalpable left testis. An ultrasound was performedfor nonpalpable testis, and it showed the absence of theleft testis in his normal pathway but the presence of bothtestes through the right inguinal canal.Exploration through the right inguinal incision showedtwo testes: one in the right scrotum and the second in thehernia sac. Both of them had a gubernaculum. The righttestis had a gubernaculum fixation on the iliopubicbranch. The lowest one had gubernaculum fixation on theright hemiscrotal cavity. No Müllerian remnants had beendetected. Each testis had its own vas deferens, but thespermatic cord fused together proximally. After ligatureof the hernia sac, the left testis was fixed by a trans-septal way after its passage through the right inguinalring. The patient was discharged 24 h later without anycomplication.DiscussionCrossed testicular ectopia is also called transversetesticular ectopia, or testicular pseudoduplication. Sincethe first case was reported by Von Lenhossek in 1886,about 170 cases were reported in the literature [2].Clinically, the most usual presentation is an inguinalhernia with ipsilateral palpable testis and nonpalpabletestis on the opposite side. The diagnosis is often madeincidentally during herniotomy or during exploration forundescended testis [3]. However, preoperative diagnosiscould have been made in some cases [4]. In our cases,diagnosis is made preoperatively by clinical examinationand confirmed by ultrasonography.Gauderer and colleagues has described a classificationsystem for crossed testicular ectopia based on thepresence of associated abnormalities: type 1 (40–50%)associated with inguinal hernia alone, type 2 (30%)associated with persistent or rudimentary Müllerianduct structures, and type 3 (20%) associated withother genitourinary abnormalities without Müllerianremnants [2].The etiology of CTE is unknown. Several theories havebeen advanced: Berg is the first to explain that bothtestes develop from the same genital ridge [5]. Kimuraproposed that if both vas deferens arose from one side,110 Case report1687-4137 c 2017 Annals of Pediatric Surgery DOI: 10.1097/01.XPS.0000489148.55669.f6Copyright r 2017 Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited.there had been unilateral origin; however, if there wasbilateral origin, one testis had crossed over [3,6].Many authors propose that abnormal or absent guberna-culum development results from an adherent developingwolf ducts. Most authors also agree, that later one testiscrosses to the opposite side; they believe that mechanicalcauses are important for this pathology. The association ofCTE with Müllerian duct abnormalities has led some tohypothesize a mechanical interruption of testiculardescent by persistant Müllerian duct [4,7].The treatment of CTE is focused on the detection ofassociated congenital abnormalities and placement ofectopic testicles into anatomical positions. Trans-septalorchiopexy via controlateral inguinal incision is thetreatment of choice if adequate length of the spermaticcord is present [7].Similarly, rodent studies show that transection of thegubernaculum can lead to accidental transverse ectopia,but in our second case gubernaculum of the controlateraltestis was fixed in the opposite iliopubic branch [8]. Inthis case, the adherence of developing wolffian ducts wasthe most interesting theory.The management of PMDS associated with CTE is stillcontroversial. Many authors prefer testicular biopsies;reintroducing Müllerian cavity, testes, and fallopian tubeto the abdominal cavity; herniorrhaphy; and reoperatingafter confirming the sexual orientation (karyotype, age,social). In the case of male orientation, Müllerian cavitywill be resected (to prevent mass syndrome and pain atpuberty) and both testes will be fixed in dartos.Management of testicular ectopia is either by trans-septal or transperitoneal transposition orchidopexy [9].An orchidectomy is indicated for testis that cannot bemobilized to a palpable location [7].Recently, with the experience of laparoscopic surgery forimpalpable testis, both diagnosis and management ofCTE are possible, as well as the associated anoma-lies [10,11].Patients with CTE are at an increased risk of malignanttransformation. In fact, the overall incidence of malignanttransformation of gonads is 18%. There are no reports ofmalignancy arising from the Müllerian remnants [4,9,12].ConclusionIt is important to specify the location of the gubernacu-lums. We must distinguish between simple CTE andPMDS. Management is still controversial in the formwith PMDS most author prefer a conservative approachto this rare syndrome without risking the vas deferens,but we prefer to resect Müllerian cavity to prevent painand mass syndrome later [13]. Trans-septal fixation is asafe option for contralateral orchidopexy.Long-term follow-up is needed for the assessment offertility in these patients.Conflicts of interestThere are no conflicts of interest.Fig. 3Both testis were placed in separate hemiscrotums, keeping their bloodsupply intact.Fig. 2Fallopian tube and testis in lateral view.Fig. 1Persistent Müllerian duct syndrome: right testis, fallopian tubes, lefttestis, spermatic cord, uterus.Crossed testicular ectopia Boukesra et al. 111Copyright r 2017 Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited.References1 Avolio L, Belville C, Bragheri R. Persistent Müllerian duct syndrome withcrossed testicular ectopia. Urology 2003; 62:350.2 Malik MA, Iqbal Z, Chaudri KM, Ahmed AJ, Malik NA. Crossed testicularectopia. Urology 2008; 71:984e5–984e6.3 Gauderer MW, Grisoni ER, Stellato AT, Ponsky JL, Izant RJ Jr. Transversetesticular ectopia. J Pediatr Surg 1982; 17:1–2.4 Karnak I, Tanyel FC, Akçören Z, Hiçsönmez A. Transverse testicular ectopiawith persistent Müllerian duct syndrome. J Pediatr Surg 1997; 32:1362–1364.5 Beberg AA 3rd. Transverse Ectopy of the Testis. Ann Surg 1904; 40:223–224.6 Kimura T. Transverse ectopy of the testis with masculine uterus. Ann Surg1918; 68:420–425.7 Hughes DT, Croitorub DP. Case report: crossed testicular ectopia. J PediatrSurg 2007; 42:1620–1622.8 Hutson JM, Hasthorpe S, Heyns CF. Anatomical and functional aspectsof testicular descent and cryptorchidism. Endocr Rev 1997; 18:259–280.9 Jouini R, Lefi M, Sami C, Manef G, Mohsen B, Nouri A. Transverse ectopictestis. Prog Urol 2002; 12:666–667.10 Zuckerman JM, Upadhyay J. Crossed-fused testicular ectopia: a case forthe use of laparoscopy to evaluate nonpalpable testicles. J Pediatr 2012;160:879.11 El Gohary MA. Transverse testicular ectopia with persistent Müllerian ductsyndrome. Ann ped surg 2012; 8:62–63.12 Mahfouz ES, Issa MA, Farag TI, Naguib KK, Al-Awadi SA, Schimke RN.Persistent Müllerian duct syndrome: report of two boys with associatedcrossed testicular ectopia. J Pediatr Surg 1990; 25:692–693.13 Beatty JS, Bhalla VK, Hatley RM, Pipkin WL, Howell CG. Neglectedcryptorchidism: delayed recognition of persistent Müllerian ductsyndrome and subsequent malignant degeneration. Urology 2013; 82:511–514.112 Annals of Pediatric Surgery 2017, Vol 13 No 2Copyright r 2017 Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited.

Crossed testicular ectopia: What should be specified?

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Crossed testicular ectopia: What should be specified?

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