Persistent müllerian duct syndrome: How to deal with the müllerian duct remnants — a review

Abstract

Persistent müllerian duct syndrome (PMDS) is usually a surprise finding either during orchidopexy or during routine inguinal hernia repair in male patients. Often, the surgeon would face a dilemma about what is to be done with the remnants of müllerian duct, i.e. the fallopian tubes, uterus and proximal vagina. Till recently, it was advised to retain these structures whenever it was felt that the complete excision of these structures may jeopardise the blood supply and integrity of the vas deferens. Recent reports of malignancy in these retained structures would justify more aggressive approach. In our patients with PMDS, we have removed the mucosa of the retained müllerian structures, without compromising the integrity and vascularity of the vas deferens, thus reducing the chances of malignancy.

Management of the patients with persistent Müllerian duct syndrome: Is the ultimate goal testicular descent?

“…[5] The female type is bilateral intra-abdominal testes in a position analogous to ovaries. The male type, also called hernia uteri inguinale, is characterized by one testis along with the uterus and tubes in a hernia sac or scrotum.…”

Persistant Mullerian Duct Syndrome with Transverse Testicular Ectopia Presenting as Left Inguinal Hernia with Cryptorchidism, A Rare Presentation

“…Group 3 (10%) male type: Both the testes are located in the same hernial sac along with the Mullerian structures (transverse or crossed testicular ectopia). [3,4,5,6] Our patient belongs to the rarest group, transverse testicular ectopia associated with PMDS, presenting with left inguinal hernia and bilateral undescended testes. Upon exploration bilateral testes along with uterus and fallopian tubes were present as contents of left sliding inguinal hernia.…”

Persistent Müllerian Duct Syndrome (PMDS): a Rare Anomaly the General Surgeon Must Know About

“…3. Smallest group (10 %) where both the testes are located in the same hernial sac along with the müllerian structures (transverse testicular ectopia (TTE), male variant) [5] ( Fig. 5).…”

“…There have been reports of embryonal cell carcinoma, seminoma, choriocarcinoma and teratoma in patients with cryptorchidism and persistent Müllerian duct. Apart from malignancy, retained müllerian remnants which are in connection with the prostatic utricle are known to cause recurrent UTI, stones and voiding disturbances [5].…”

“…For TTE, transseptal fixation or modified Ombredanne technique, in which both cords and vessels of two testicles pass through the same inguinal canal, is recommended, but this carries a risk of damage to both vas and vessels of the two testes whenever there is trauma or infection [21]. Manjunath et al (2010) recommended stripping/destroying the mucosa of the retained müllerian remnants to reduce the risk of malignancy and, simultaneously, to prevent the damage to the vas deferens and disruption of collateral blood supply to the testes [5]. In both cases, we identified the vas deferens and blood supply to the testis which was running high up, and subtotal hysterectomy was done; both the testis were fixed in their respective hemiscrotum after adequate mobilisation.…”