A mullerectomy is a surgical procedure involving the removal of persistent Müllerian duct structures in genetically male individuals. These structures are remnants of fetal ducts that would normally develop into the uterus, fallopian tubes, and upper vagina in a female. This intervention targets the non-regressed internal female anatomy to prevent future complications.
Persistent Müllerian Duct Syndrome
The necessity for a mullerectomy stems from a rare congenital anomaly known as Persistent Müllerian Duct Syndrome (PMDS). Individuals affected by PMDS are genetically male, possessing a 46,XY karyotype and normal male external genitalia. During typical embryonic development, the testes produce Anti-Müllerian Hormone (AMH), which signals the regression of the Müllerian ducts. In PMDS, this regression fails due to either a deficiency in AMH production or the target tissues’ inability to respond to the hormone signal.
This failure results in the development of a uterus, cervix, and fallopian tubes within the male pelvis or abdomen, alongside normal male reproductive ducts derived from the Wolffian ducts. The syndrome is typically discovered incidentally during childhood or adolescence, often during surgical correction of an inguinal hernia or undescended testes (cryptorchidism). The presence of these internal female structures complicates the descent of the testes, often anchoring them high in the abdomen.
The clinical presentation involves one or both testes being non-palpable or located within an inguinal hernia sac, which pulls the attached Müllerian structures. PMDS does not affect the production of testosterone or the development of secondary sexual characteristics. However, the anatomical disruption significantly increases the risk of infertility and malignancy.
Performing the Mullerectomy
The goal of the mullerectomy is two-fold: to remove the non-regressed Müllerian structures and to facilitate the repositioning of the testes into the scrotum (orchiopexy). Surgeons must dissect the rudimentary uterus and fallopian tubes from surrounding male reproductive structures. A challenge involves separating the Müllerian remnants from the vas deferens, the delicate tube that transports sperm.
The vas deferens is often embedded in the wall of the persistent uterus or cervix, requiring caution during dissection to avoid injury that could impair future fertility. Complete excision of the Müllerian remnants is generally recommended to remove any potential source of malignancy. However, some surgeons may leave small portions of tissue if the risk of damaging the vas deferens is too high. The surgery is commonly performed using minimally invasive techniques, such as laparoscopy or robotic-assisted surgery, which allow for better visualization and precise dissection of the pelvic structures.
Laparoscopic access is preferred, especially in younger patients, as it minimizes incision size and allows for assessment of the pelvic anatomy. The procedure often involves first securing the vas deferens and the testicular blood supply before excising the uterus and fallopian tubes down to the pelvic floor. Removal of these structures provides the necessary space to mobilize and bring the previously undescended testes into the scrotum during the concurrent or subsequent orchiopexy.
Postoperative Care and Short-Term Recovery
Following a mullerectomy, especially when performed laparoscopically, patients typically experience a hospital stay lasting one to two days. The initial focus of postoperative care is on managing pain, generally controlled with prescription or over-the-counter medications like acetaminophen. Patients are monitored for common surgical complications such as bleeding, infection at the incision sites, or delayed wound healing.
Minimally invasive incisions usually heal quickly, but physical activity restrictions are put in place for several weeks to ensure internal healing. Patients are advised to avoid heavy lifting or strenuous activity for about four to six weeks. This precaution prevents strain on the internal sutures and the surgical site in the pelvic region. Returning to normal daily activities, including school or light work, can usually be accomplished within one to two weeks, depending on the individual.
Long-Term Outcomes and Follow-Up
The long-term prognosis after a successful mullerectomy and orchiopexy is generally favorable, though ongoing monitoring is required. A primary benefit of removing the Müllerian remnants is the elimination of the risk of malignant transformation within that tissue, estimated to be around eight percent over a lifetime. However, the risk of testicular cancer, elevated due to the initial cryptorchidism, remains comparable to that of other individuals with a history of undescended testes.
Fertility potential is the long-term concern and depends on the function of the testes and the preservation of the vas deferens during the removal of the Müllerian structures. While the procedure aims to maximize the chance of fathering children, infertility remains common due to pre-existing testicular damage from being undescended. Patients require long-term urological and endocrinological follow-up to monitor testicular health and function, including potential testosterone replacement therapy if the testes do not function adequately after repositioning.