PERSISTENT MüLLERIAN DUCT SYNDROME ASSOCIATED WITH TRANSVERSE TESTICULAR ECTOPIA: REPORT OF TWO CASES

Öztürk, Hayrettin; Eroğlu, Muzaffer; Öztürk, Hülya; Uzunlar, Ali Kemal; Okur, Hanifi

doi:10.1080/15513810701394660

Cited by 7 publications

(4 citation statements)

References 18 publications

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“…TTE can be classified according to the presence of associated anomalies into 3 types; type 1 (associated with hernia only, 50% of the cases), type 2 (associated with PMDS, 30% of cases) and type 3 (associated with genitourinary abnormalities, 20% of cases) [ 5 ]. Our case belongs to type 2, which results from defects either in the synthesis or action of Müllerian inhibiting factor (MIF) and is characterized by the presence of uterus and fallopian tubes in otherwise phenotypically normal 46 XY males.…”

Section: Discussionmentioning

confidence: 99%

Transverse testicular ectopia with Müllerian duct remnant in an incarcerated congenital inguinal hernia – a case report

2020

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Background: Transverse testicular ectopia (TTE) is a rare anomaly characterized by both testes descending through a single inguinal canal. In this report, the diagnosis of TTE was discovered in the event of an incarcerated congenital inguinal hernia in a neonate. Case presentation: We present a case of TTE accompanied by persistent Müllerian duct structures (PMDS) that had been discovered incidentally during inguinal exploration of a 26-day-old boy who presented with an incarcerated congenital inguinal hernia on the right side and left cryptochidism on the left side. The pathogenesis, approach and proposed management of TTE are discussed. Conclusion: TTE is an extremely rare anomaly, especially in neonates, and should be suspected in patients presenting with inguinal hernia on one side and cryptorchidism on the other side.

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Section: Discussionmentioning

confidence: 99%

Transverse testicular ectopia with Müllerian duct remnant in an incarcerated congenital inguinal hernia – a case report

2020

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“…Type 2 is associated with persistent or rudimentary Mullerian canal remnants. Type 3 is the rare form where more abnormalities (inguinal hernia, hypospadias, pseudohermaphrodite and scrotal abnormality) than Mullerian remnants are observed [12]. Isolated left undescended testis plus right transverse ectopic testis were determined in our case.…”

Section: Discussionmentioning

confidence: 69%

A Case Presentation With Tranverse Testicular Ectopia

Cece

2012

Int J Clin Pediatr

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Ectopic testis is defined as the aberrant location of testis in places other than the scrotum during descent from the inguinal canal after the external ring. These aberrant locations include suprapubic site, medial of the thigh, perineum, anterior abdominal wall and contralateral scrotum. An interesting and rare form of ectopic testis is transverse testicular ectopia (TTE) or in other words, cross testicular ectopia. This abnormality presents with both testicles in the same hemiscrotum. In this manuscript, we present a four-year old male case who presented to our out patients clinic with left undescended testis and was diagnosed with transverse testicular ectopia.

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“…Though these imaging techniques are useful in diagnosis of PMDS, its pre-operative diagnosis is rarely performed due to the normal development of penis and scrotum. As the patients with PMDS are generally observed with bilateral undescended testes or inguinal hernia on one side and cryptorchidism on the other side, the disorder is usually diagnosed during surgery for inguinal hernia or bilateral undescended testes (6). In the present case report, the child was diagnosed during laparoscopic exploration, but after performing right testicle orchidopexy.…”

Section: Discussionmentioning

confidence: 99%

“…In the present case report, the child was diagnosed during laparoscopic exploration, but after performing right testicle orchidopexy. The fluctuation in serum AMH levels might also prove to be helpful in determining PMDS in case of infants and young boys (before puberty) (6). Studies report that patients with AMH gene defects are diagnosed with low AMH levels from birth.…”

Section: Discussionmentioning

confidence: 99%

Bilateral Cryptorchidism, a rare presentation for persistent Müllerian duct syndrome

Al-Faris¹,

Jabari²,

Al-Sayed³

et al. 2016

Electron physician

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Persistent Müllerian duct syndrome (PMDS) is a rare, sex-limited, autosomal recessive disorder representing male pseudo-hermaphroditism. It is observed in males with the presence of female reproductive organs such as the uterus, cervix, and bilateral fallopian tubes along with normally developed male reproductive organs. It generally occurs during embryogenesis due to mutation in anti-Müllerian hormone (AMH) gene, i.e., AMH gene or AMH receptor (AMHR2) gene. The present case reports a male infant with inflammation in the right groin who was admitted to Security Forces Hospital in 2015. During surgery, his testis was pulled down to the scrotum in the normal anatomical position, but full orchidopexy was not performed due to fragile capsule and edematous area. After a year, a right orchidopexy was performed. During left testis laparoscopic exploration, a small left intra-abdominal gonad in a position similar to the ovary, Müllerian structures in the form of a small uterus and vagina in the midline were observed. Biopsy of left gonad showed immature testicular tissues. The MRI findings of the pelvis showed normal male urethra with the presence of a vagina and small uterus, but no definite ovaries or testicle. Based on the clinical, laboratory, imaging, and primarily operative findings, the boy was diagnosed with PMDS. This was confirmed using genetic testing, which revealed biallelic mutations in the AMHR2 gene. The patient is currently under clinical observation to decide further management of PMDS through left testis orchidopexy, either with or without surgical excision of persistent Müllerian duct structures. The key message, which needs to be spread from this case report, is that the infant with bilateral undescended testes or inguinal hernia on one side and cryptorchidism on the other side should be thoroughly investigated to exclude any underlying disorder of sex development (DSD) before any further intervention.

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PERSISTENT MüLLERIAN DUCT SYNDROME ASSOCIATED WITH TRANSVERSE TESTICULAR ECTOPIA: REPORT OF TWO CASES

Cited by 7 publications

References 18 publications

Transverse testicular ectopia with Müllerian duct remnant in an incarcerated congenital inguinal hernia – a case report

Transverse testicular ectopia with Müllerian duct remnant in an incarcerated congenital inguinal hernia – a case report

A Case Presentation With Tranverse Testicular Ectopia

Bilateral Cryptorchidism, a rare presentation for persistent Müllerian duct syndrome

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