Transverse testicular ectopia with persistent müllerian duct syndrome

doi:10.1016/S0022-3468(97)90323-7

Journal of Pediatric Surgery

Volume 32, Issue 9, September 1997, Pages 1362-1364

https://doi.org/10.1016/S0022-3468(97)90323-7 Get rights and content

Abstract

Transverse testicular ectopia is rarely associated with persistent müllerian duct syndrome. The ninth pediatric case of transverse testicular ectopia with persistent müllerian duct syndrome is reported. The clinical and operative findings and treatment are discussed. The importance of abdominal exploration in the presence of two gonads in one inguinal side and the avoidance of dissection of müllerian structures has been stressed.

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Cited by (56)

Nonneoplastic Diseases of the Testis
2020, Urologic Surgical Pathology
Changes in our understanding of pathology have accelerated in recent years. The ability to approach a diagnostic problem from different points of view—clinical, morphologic, and molecular genetic—has led to an integrative approach to pathology. Nontumoral pathology of the testis has not been an exception. In this chapter, we start from a histophysiologic base that includes the development of the testicle, the descent of the prepubertal testis, and the adult testicle. The chapter continues with congenital anomalies of the testis and the alterations in sexual development, the classifications for which have not been uniformly accepted by those who study these matters. We focus on the undescended testis, a source of controversy regarding its best treatment—surgical versus hormonal. The problem of the infertile patient is addressed, bearing in mind not only the primary alterations, be these chromosomal or molecular, but also considering that efficient testicular function is only acquired when there is a harmonious interaction between different glands and organs. Vascular pathology of the testis is always interesting, whether focusing on a dramatic situation such as testicular torsion, or considering the relationship between varicocele and infertility. In the section on Inflammation and Infection, besides the classical processes, other lesser known processes are included in the section Other Testicular and Epidydima Lesions. Compared with the previous edition, this chapter incorporates a significant number of figures that properly document the text.
Transvers testicular ectopia: A case report and literature review
2019, International Journal of Surgery Case Reports
Citation Excerpt :
The effect of PMDS on the embryological development of TTE may be explained by mechanical effects. Persistent Mullerian duct may interfere with the expected descent of the testicles mechanically or it may push both testicles towards the same hemiscrotum, and cause the development of TTE [11]. The development of malignancy after puberty has been reported in previous studies.
Transverse testicular ectopia (TTE) is a rare anomaly characterized by the presence of both testicles in the same hemiscrotum or inguinal region. The most common clinical findings of TTE are unilateral nonpalpable testis in the scrotum and inguinal hernia on the side of palpable testis in the scrotum. It should be kept in mind that TTE may coexist with Persistent Mullerian Duct Syndrome (PMDS). Therefore, appropriate treatment should be performed considering PMDS.
Type 2 transverse testicular ectopia was diagnosed in one patient who was operated with bilateral undescended testis. He was treated with transseptal orchiopexy and excision of mullerian structures.
In case of TTE with PMDS, optimal surgical approach with orchiopexy and excision of Müllerian duct is necessary. Intraoperative aggressive dissection of vas deferens and testicular vessels should be avoided in TTE patients. They should be closely followed because of the increased risk of malignant transformation in the postoperative period.
An investigation of transvers testicular ectopia should be performed in all nonpalpable undescended testis anomalies.
Testicular ectopia: Why does it happen and what do we do?
2017, Journal of Pediatric Surgery
Citation Excerpt :
Several theories have been postulated to explain the pathogenesis of perineal testicular ectopia, including congenital obstruction of the ‘secondary external inguinal ring’ and subsequent migration of testis to the perineal pouch [14], an abnormal interplay between androgen and calcitonin gene-related peptide (CGRP) released from the genitofemoral nerve (GFN) [12], or aberrant gubernacular stabilization caused by an anomaly at the distal extremity of the gubernaculum [13]. Transverse testicular ectopia (TTE) is the result of both testes ultimately descending through the same inguinal canal to sit in a common hemiscrotum [15–17]. Prevailing mechanistic theories to explain TTE included both testes being derived from the same germinal ridge (i.e., duplication of the gonadal primordium) [15], mechanical effect of persistent Müllerian duct structures (persistent Müllerian duct syndrome; PMDS) preventing testicular descent or causing both testicles to descend toward the same hemiscrotum [17], and defective gubernacular formation [16].
Testicular ectopia is rare, but the large range of anatomical locations described in the literature has spawned an abundance of possible theories to explain etiology. However, as the anatomical characteristics of normal testicular descent have only been elucidated recently, many of the theories of testicular ectopia do not incorporate this new perspective. In this study we aimed to determine what was in the literature about ectopic testis since 1980, and then try to explain the different anatomical variants in the light of current knowledge about testicular descent.
A literature search was performed and all articles in English published since 1980 about testicular ectopia using several key words were identified.
A total of 271 articles in English were found, of which 31 addressed the pathophysiology and are the primary focus of this study. Case reports and reviews described perineal ectopia (× 4), transverse testicular ectopia (× 11), and abdominal ectopia (× 2), along with 3 reviews/case reports addressing diagnosis and management. A range of proposed causes were found, including obstructed ‘third inguinal ring’ at neck of scrotum, abnormal CGRP function, aberrant distal gubernacular attachment, mechanical hindrance from retained Müllerian ducts, defective gubernacular formation or disruption of the gubernacular attachment to the testis.
After reviewing the proposed theories, we propose a unifying theory, based on current knowledge of testicular descent, where testicular ectopia can be explained by a) anomalous attachment of the gubernaculum to the anterior abdominal wall during transabdominal descent, or b) aberrant migration of the gubernaculum during the inguinoscrotal phase of testicular descent.
Rare case of transverse testicular ectopia – Case report and review of literature
2017, International Journal of Surgery Case Reports
This case report has been reported in line with the SCARE criteria; Consensus-based surgical case report guidelines of International Journal of Surgery 2016.
Transverse Testicular Ectopia (TTE) is a rare condition which manifests with unilateral undescended testis and contralateral hernia. Till now around 100 cases have been described in the literature. The management depends on the anatomy of the vas, vessels and testis found on surgical exploration. An algorithm exists for its management and we propose a modified algorithm for management of TTE.
Five year male presented with complaints of unilateral undescended testis on the right and hernia on the left. Clinically the right testis was impalpable and left testis palpable in the left hemiscrotum and fluid hernia on the same side.
Transverse testicular ectopia is a rare condition presenting with UDT and contralateral inguinal hernia. Although more than 100 cases have been described in the literature so far, those managed with a transeptal contralateral orchidopexy are two cases to the best of our knowledge. TTE was first described by Von Lenhossek in 1886. The mean age of presentation is around 4 years and most of the cases are diagnosed on surgical exploration. The management of TTE remains controversial even though an algorithm has been described for its management due to its varied presenting scenarios.
TTE is a rare condition which requires high index of suspicion for diagnosis preoperatively. Whenever suspected we recommend an USG and/or MRI prior to diagnostic laparoscopy and proceed with orchidopexy. Diagnostic laparoscopy is both helpful in diagnosis and management. Transeptal contralateral orchidopexy gives good tension free fixation of testes in the scrotum.
Transverse testicular ectopia with scrotal hypospadias but without inguinal hernia – Case report of a rare association
2017, International Journal of Surgery Case Reports
Citation Excerpt :
Fusion of the two spermatic cords in the inguinal canal is common and is usually inseparable [10,12]. Familial occurrences have been reported, and it can be associated with other anomalies like persistent Mullerian duct syndrome (PMDS), true hermaphroditism, inguinal hernia, hypospadias, pseudohermaphroditism, seminal vesicle cysts, renal agenesis and scrotal anomalies [3–6]. Ipsilateral inguinal hernia is invariably present in these cases but in our patient there was no evidence of inguinal hernia neither on clinical examination nor on ultrasonography of the inguinoscrotal region.
Transverse testicular ectopia is an extremely rare anomaly, in which both the testis migrate towards the same hemiscrotum through the same inguinal canal. It is usually associated with other abnormalities such as persistent Mullerian duct syndrome, true hermaphroditism, inguinal hernia, pseudohermaphroditism, and scrotal anomalies; the association with inguinal hernia being the commonest. We, here, report a case of transverse testicular ectopia in a 12 years old boy having the ectopic testis in contralateral hemiscrotum without any inguinal hernia but having scrotal hypospadias with severe chordee. The diagnosis was made on clinical examination and confirmed on ultrasonography. Trans-septal orchiopexy was done for ectopic testis and hypospadias and chordee were managed by staged repair.
Persistent Müllerian ducts syndrome: One case of late hypofertility
2015, Morphologie
Nous rapportons le cas d’un patient de 35 ans présentant un syndrome de persistance des canaux mésonéphrotiques de Müller (PMDS) de type féminin (groupe A). Le diagnostic a été posé à l’âge adulte au cours d’un bilan d’infertilité. L’examen clinique retrouvait un scrotum vide, une verge normale et des masses kystiques inguinales bilatérales. Le spermogramme retrouvait une azoospermie. L’imagerie par scanner et IRM pelvienne retrouvait la présence d’un utérus, de deux trompes et de deux testicules polykystiques en position inguinale. Une prise en charge chirurgicale a été réalisée pour biopsie chirurgicale des testicules. L’examen anatomopathologique retrouvait alors une formation kystique pluriloculaire d’origine mésothéliale, avec testicule atrophique en involution sertolienne et sans spermatozoïdes. Le syndrome PMDS est une forme rare de pseudo-hermaphrodisme interne caractérisé par la présence chez un homme de l’utérus, des trompes et de la partie supérieure du vagin avec des organes génitaux externes masculins aux caractères virilisés. Environ 200 cas sont rapportés dans la littérature. Le diagnostic est souvent fait chez l’enfant en peropératoire au cours d’une cure d’ectopie testiculaire. Le caryotype est de type 46 XY. La pathogénie est relative à un déficit en hormone anti-müllerienne (AMH) ou à une résistance tissulaire à son action par anomalies des récepteurs. La non-régression des dérivés du canal de Müller peut donner trois types de PMDS : un type masculin, un type féminin et un type transverse. La prise en charge chirurgicale reste difficile mais nécessaire en raison du risque d’hypofertilité et de dégénérescence des testicules ectopiques.
We report the case of a 35-year-old patient with a syndrome of persistent Müllerian ducts (PMDS) of the female type (group A). The diagnosis was made in adulthood during an infertility workup. Clinical examination revealed an empty scrotum, a normal penis and bilateral inguinal cystic masses. The spermogram found azoospermia. Imaging using MRI and tomotensidometry found the presence of an uterus, two fallopian tubes and two inguinal positions of polycystic testes. A surgical management was performed for surgical testicular biopsy. Histological examination then found a cystic formation of multi-celled mesothelial origin, with atrophic testis Sertoli cell involution and without sperm. PMDS is a rare form of pseudo-internal hermaphroditism characterized by the presence in a man of the uterus, fallopian tubes and upper vagina with external male genitalia and virilized characters. About 200 cases are reported in the literature. The diagnosis is often made in children intraoperatively during a cure of testicular ectopia. The karyotype is 46 XY type. The pathogenesis is related to a deficiency of anti-Müllerian hormone (AMH) or tissue resistance to its action by receptor abnormalities. The regression of the Müllerian duct derivatives can give three types of PMDS : masculine type, feminine type and a transverse type. Surgical treatment is difficult but necessary because of the risk of infertility and ectopic testicular degeneration.

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Case reportTransverse testicular ectopia with persistent müllerian duct syndrome

Abstract

J Pediatr Surg

J Pediatr Surg

Urology

Urology

J Pediatr Surg

J Urol

Case report
Transverse testicular ectopia with persistent müllerian duct syndrome