Persistent müllerian duct structures in cryptorchid male infants: Surgical dilemmas

Pappis, C; Constantinides, Constantinos; Chiotis, Dimitrios; Dacou-Voutetakis, Catherine

doi:10.1016/0022-3468(79)90002-2

Cited by 26 publications

(13 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Some authors believe that excision of müllerian duct structures and scrotal orchidopexy are not possible without sacrificing the vasa deferentia. 2 In our patient, it was possible to dissect the vasa deferentia from the rudimentary uterus and vagina, perform scrotal orchidopexy and excision of müllerian duct structures without risking the vas deferens. We believe that every effort should be made to preserve the vas deferens and testes for possible future fertility.…”

Section: Discussionmentioning

confidence: 83%

“…However, the malignancy incidence appears to be similar to that of a normal cryptorchid child. 2,10,11 In conclusion, a conservative surgical approach for this rare syndrome in the form of orchidopexy and partial excision of the müllerian duct remnants without risking the vas deferens is recommended. A long follow-up will be needed for assessment of the fertility in these patients.…”

Section: Discussionmentioning

confidence: 99%

“…Fertility has been reported rarely in a few cases. 2,9 Martin et al 3 had reported a 32-year-old man with transverse testicular ectopia and a persistent müllerian duct who had a normal sperm count, but the motility index was zero, implying an intrinsic defect in spermatogenesis. The other possible cause of low sperm counts and poor motility in most of these patients is the partial duct obstruction.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Persistent Müllerian Duct Syndrome Associated with Transverse Testicular Ectopia

Albassam

1997

Ann Saudi Med

View full text Add to dashboard Cite

AA Al-Bassam, Persistent Müllerian Duct Syndrome Associated with Transverse Testicular Ectopia. 1997; 17(2): 226-227 Persistent müllerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism, characterized by the presence of a uterus and fallopian tubes due to failure of müllerian duct regression in genotypically normal males.1,2 More than 150 cases have been recorded, most of them in adults. The association between the persistent müllerian duct and transverse testicular ectopia is even more uncommon. In 1886, von Cenhossek described the first case of transverse testicular ectopia and in 1895, Jordan described the syndrome of transverse testicular ectopia with persistent müllerian ducts. 3 Case ReportA 10-month-old boy presented with a left inguinal hernia and absence of the right testis since birth. Examination of the groins showed a normal phallus, left inguinal hernia, empty right hemiscrotum, impalpable right testis and normal left testis.Urinalysis, complete blood count and serum electrolytes were normal. At exploration through the left inguinal incision, two spermatic cords were found separated by a rudimentary uterus, thick-walled vagina and ligamentous structure corresponding to the broad ligament. The two vasa deferentia were adherent to the thickened vagina. The left testis was lying in the left hemiscrotum, but were pulled up into the operative field during dissection. The right testis ended at the left mid-inguinal canal ( Figure 1). The two testes were biopsied, the two cord structures were carefully separated from the persisting müllerian structures and limited resection of the rudimentary uterus and vagina was done. The associated inguinal hernia was repaired and bilateral orchidopexy with transposition of the right testis to the right hemiscrotum through a suprapubic subcutaneous tunnel was performed.The postoperative course was uncomplicated. The karyotype confirmed a male gender 46XY. Renal ultrasound was normal. DiscussionNormally, the testis is located in the scrotum at birth. Ectopic testes have been reported at different sites, including the superficial inguinal pouch, suprapubic, femoral and perineal areas, and at the base of the penis. Migration of the testis to the opposite side, where both testes pass through the same inguinal canal, is known as transverse testicular ectopia (TTE). Over 100 cases of TTE have been reported in the literature. 5,6 Persistent müllerian duct syndrome is rare, characterized by the presence of well-developed or rudimentary uterus, cervix, vagina and fallopian tubes in a normal 46XY male.The presence of PMDS with TTE is even more uncommon. In most cases, the PMDS is discovered during surgery for the inguinal hernia or cryptorchidism or by the presence of transverse testicular ectopia. Our patient was found to have PMDS with TTE during surgery for herniotomy in a child with contralateral undescended testis. PMDS represents only a small number of male pseudohermaphroditism. The external genitalia in these patients are normal in shape and size wit...

show abstract

Section: Discussionmentioning

confidence: 83%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Persistent Müllerian Duct Syndrome Associated with Transverse Testicular Ectopia

Albassam

1997

Ann Saudi Med

View full text Add to dashboard Cite

show abstract

“…Since no harm is derived from the presence of feminine structures, it seems to us that they can be respected. In addition, its removal constantly implies the sacrifice of deferent ducts because they adhere very closely to the uterine walls (4,12). If the testicles can be brought to the scrotum they must be treated as in regular cryptorchidism, and if this is impossible, an orchidectomy must bp erformed.…”

Section: Discussionmentioning

confidence: 98%

Cryptorchidism and Persistence of Müllerian Remnants in a Normal Male

Barriola¹,

Jiménez²,

Gutierrez-Hoyos³

et al. 1981

Eur J Pediatr Surg

View full text Add to dashboard Cite

A four-year-old boy was explored for bilateral cryptorchidism. Operation led to the discovery of two intraabdominal testes with their corresponding vasa, two Fallopian tubes and a small uterus. No urological anomalies were found except for a vesico-ureteral reflux. Cystourethroscopy revealed a normal male tract. Sexual chromatine was negative and karyotype was 46, XY. A normal rise in testosterone serum level was recorded after stimulation with CGH. At operation both gonads could be placed extra-abdominally and the female internal structures were biopsied and left in place. This very unusual condition is derived from the absence of regression of Müllerian structures during fetal life due to a deficit in testicular secretion of anti-Müllerian hormone. The current knowledge on this topic is updated.

show abstract

“…The reason for this is the role of AMH in testicular descent, which it will not fulfill when dysfunctional [12]. Moreover, testicular descent may further be hindered mechanically by the presence of remnant müllerian structures [18]. Infertility is the rule in PMDS [19].…”

Section: Discussionmentioning

confidence: 99%

Persistent Müllerian Duct Syndrome

Buchholz¹,

Biyabani²,

Herzig³

et al. 1998

European Urology

View full text Add to dashboard Cite

Persistent müllerian duct structures in cryptorchid male infants: Surgical dilemmas

Cited by 26 publications

References 15 publications

Persistent Müllerian Duct Syndrome Associated with Transverse Testicular Ectopia

Persistent Müllerian Duct Syndrome Associated with Transverse Testicular Ectopia

Cryptorchidism and Persistence of Müllerian Remnants in a Normal Male

Persistent Müllerian Duct Syndrome

Contact Info

Product

Resources

About

Persistent müllerian duct structures in cryptorchid male infants: Surgical dilemmas

Cited by 26 publications

References 15 publications

Persistent Müllerian Duct Syndrome Associated with Transverse Testicular Ectopia

Persistent Müllerian Duct Syndrome Associated with Transverse Testicular Ectopia

Cryptorchidism and Persistence of Müllerian Remnants in a Normal Male

Persistent M&uuml;llerian Duct Syndrome

Contact Info

Product

Resources

About

Persistent Müllerian Duct Syndrome