Persistent Mullerian Duct Syndrome Research Articles

Persistent Mullerian Duct Syndrome- a Rare Case with Review of Literature

Persistent Mullerian Duct Syndrome (PMDS) is a rare type of male pseudohermaphroditism. It is brought on by an insufficiency of anti-mullerian hormone (AMH) or a malfunction of its receptor (type II). Approximately 150 adult cases of PMDS have been documented overall, with the majority from the Middle East, USA and Europe. 44 publications (49 individuals) with 3 testicular tumors linked to PMDS were found using the Medline/Pubmed search; the majority (59%) of the patients presented with a big abdominal mass. In our case, the abdomen mass that was identified as seminoma testis in a 22-year-old male patient with PMDS. Our goal in presenting this case is to highlight how uncommon it is for PMDS patients to have a testicular tumor, and how the presence of an undescended testis raises the risk of testicular tumor development in PMDS patients. The clinicians should be aware of rare entity while dealing with the cryptorchidism with inguinal hernia and necessary management should be done to offer proper treatment.

A rare case of persistent mullerian duct syndrome with adenocarcinoma of ascending colon

Persistent mullerian duct syndrome (PMDS) is a rare congenital disorder. This condition results from a failure of Mullerian duct regression during fetal development, leading to the presence of uterus, fallopian tubes, and upper vagina in otherwise phenotypically male individuals. Radiological evaluation plays a crucial role in the diagnosis and management of PMDS. Various imaging modalities including ultrasound, magnetic resonance imaging, and computed tomography are employed to visualize and confirm the presence of Mullerian structures in affected individuals.

'Testicular masquerade': a case report of testicular malignancy with persistent Müllerian duct syndrome and transverse testicular ectopia.

Persistent Müllerian duct syndrome (PMDS) is a rare sexual development disorder. It is even more rarely associated with transverse testicular ectopia (TTE), a rare form of testicular ectopia, in which both testes descend through a single inguinal canal and are present in the same hemiscrotum. PMDS with TTE is associated with 18%-33% malignant transformation. Here we report the case of a 48-year-old man who presented with a large right inguinoscrotal swelling and on evaluation was found to have a large right testicular mass with complete right inguinal hernia, undescended left testis and a central abdominal mass. On evaluation with contrast-enhanced computed tomography abdomen and pelvis and image-guided biopsy he was diagnosed with mixed germ cell tumour of the right testis (predominantly a seminoma) with a retroperitoneal nodal mass and absent left testis, for which he received chemotherapy. Post-chemotherapy he underwent surgery and was diagnosed intraoperatively with PMDS along with TTE and testicular malignancy arising from the ectopic left testis. Postoperative recovery and follow-up were uneventful. Most cases of PMDS are diagnosed early in life. They present clinically with unilateral or bilateral undescended testis with inguinal hernia. In adults, PMDS is usually associated with male infertility. However, TTE is associated with an increased risk of testicular tumours if undiagnosed until adulthood. In adults PMDS with TTE is usually an intraoperative finding and is commonly associated with malignancy in the ectopic/undescended testis.

A novel technique in the management of crossed testicular ectopia in children

BackgroundCrossed testicular ectopia (CTE) is an extremely rare anomaly of urogenital development in which both testes are migrated and descend through a single inguinal canal, one or both testes may be ectopic in the abdomen, the inguinal region or descent to the hemi-scrotum with empty contralateral hemi-scrotum. The etiopathogenic mechanism of CTE is still unknown. CTE was classified into three types based on the associated findings. Most of the cases were found associated with other abnormality such as Persistent Mullerian Duct Syndrome, Inguinal hernia, Hermaphroditism, and other scrotal abnormality. The condition is mostly detected incidentally during repair of inguinal hernia or laparoscopic evaluation of impalpable undescended testis. We present our experience in management of seven cases with CTE including a novel technique named "Arabic eight figure orchiopexy". ConclusionsCTE should be considered in children presented with unilateral inguinal hernia or hydrocele with empty contralateral hemiscrotum. We present a novel technique in management of CTE named "Arabic eight figure orchiopexy".

Persistent Mullerian duct syndrome: An atypical clinical presentation

Abstract Introduction Persistent Müllerian duct syndrome (PMDS) is a rare sexual development disorder characterized by a normal male phenotype. Müllerian structures cannot regress due to the absence of anti-müllerian hormone (AMH) or resistance to AMH receptor. In addition to male internal genitalia, female reproductive organs, including the uterus, uterine tubes, and upper 1/3 of the vagina are also detected. We will present a patient with PMDS who presented with bilateral cryptorchidism. Clinical Case A 31-year-old single man was referred to our clinic because of bilateral undescended testes and a rudimentary uterus detected during the evaluation of the lower abdominal pain. On physical examination, his body mass index (BMI) was 21.2. His arm span was 170 cm, and his upper-lower segment ratio was 0.96. Beard, axilla, and pubic hair development were normal; however, testes were not in the scrotum bilaterally. His karyotype analysis was 46XY. His prenatal, natal, and postnatal histories were not remarkable. His parents were not consanguineous; the younger brother had a history of undescended testis operation at age one. In laboratory examination, blood glucose, renal and liver functions were normal. Androstenedione, DHEAS, total and free testosterone, FSH, LH, total-hCG, AFP were normal. AMH>9µg/L. In the spermiogram, no sperm was observed. A rudimentary uterus and proximal vagina were observed in the abdominal computed tomography (CT). Symmetric solid masses measuring 55×40 mm compatible with bilateral undescended testis were detected in the ovarian lodges. The mass extended to the inguinal canal on the left and the scrotum on the right. In association with these masses, there were also tubular structures with the biggest diameter of 16 mm on the right and 13 mm on the left. Prostate sizes were normal. Seminal vesicles were not observed. PMDS was considered owing to 46 XY karyotypes, normal testosterone level, normal sexual development, external genitalia findings, accompanying Mullerian structures, and a high level of AMH. AMH gene mutation was not detected. However, AMH receptor type 2(AMHR2) gene mutation could not be studied. Bilateral undescended testicles, uterus, uterine tubes, and the tubular structures in the abdomen were excised. Classical type seminoma with lympho-vascular invasion was detected in the right-side tubular structure. Tumor-negative testicular tissue was frozen for possible fertility in the future. The patient received one cycle of Carboplatin. No metastasis was detected in the post-operative PET-CT scan. In post-operative evaluation, FSH, LH levels were high, total and free testosterone levels were low. AMH < 0.01 µg/L. The patient was started on testosterone replacement therapy. Conclusion PMDS is a rare cause of male pseudo-hermaphroditism. Malignant degeneration and infertility are two critical points in terms of treatment. Early diagnosis with increased awareness is essential in preserving fertility and preventing malignant degeneration.

A Surgical and Clinical Approach to Persistent Müllerian Duct Syndrome: Laparoscopic, Histological, and Molecular Findings

Background: Persistent müllerian duct syndrome (PMDS) is characterized by the persistence of müllerian duct derivatives in otherwise normally virilized 46,XY males. Biallelic mutations of the anti-müllerian hormone (AMH) and AMH receptor type 2 (AMHR2) genes lead to PMDS type 1 and 2, respectively. Aim: The aims of the study were to report the clinical, hormonal, and genetic findings in a patient with PMDS and discuss surgical strategies to achieve successful orchidopexy. Results: A 4-year-old boy was evaluated after the incidental finding of müllerian derivates during laparoscopy for nonpalpable gonads. Karyotype was 46,XY and laboratory tests revealed normal serum gonadotropin and androgen levels but undetectable serum AMH levels. PMDS was suspected. Molecular analysis revealed a novel variant c.902_929del in exon 5 and a previously reported mutation (c.367C>T) in exon 1 of the AMH gene. Successful orchidopexy was performed in two sequential surgeries in which the müllerian duct structure was preserved and divided to protect the vascular supply to the gonads. Histological evaluation of the testicular biopsy showed mild signs of dysgenesis. Doppler ultrasound showed blood flow in both testes positioned in the scrotum 1.5 years after surgery. Conclusion: PMDS is a rare entity that requires a high index of suspicion (from surgeons) when evaluating a patient with bilateral cryptorchidism. Surgical treatment is challenging and long-term follow-up is essential. Histological evaluation of the testis deserves further investigation.

Persistent müllerian duct syndrome: a rare clinical image

Persistent müllerian duct syndrome (PMDS) is a rare form of internal male pseudo-hermaphroditism in which müllerian duct derivatives are seen in a male patient. The syndrome is caused by an insufficient amount of mullerian inhibiting substance (MIS) or due to insensitivity of the target organ to MIS. It is usually discovered during surgery for hernia or undescended testis, as “hernia uteri inguinalis”. Persistent müllerian duct syndrome is often familial, presenting as an autosomal recessive trait. It was characterized by the presence of müllerian structures (fallopian bed by Nilson in 1939, as “hernia uteri inguinalis”. It has fallopian tubes, uterus and upper part of vagina) in phenotypically and genotypically male (46XY). The gene responsible is chromosome 19. A 17-year-old male child presented with complaints of swelling in the right groin and associated with pain. On examination, an irreducible right inguinoscrotal swelling and right testis not palpable. Intraoperatively, the hernial sac was found to contain testis and uterus along with fallopian tubes and ovaries. The right testis was smaller compared to the opposite side. Excision of the müllerian duct derived structures was done. This was followed by orchidopexy of right testis and right inguinal hernioplasty. Postoperative recovery of the patient was uneventful. It presents as inguinal hernia or undescended testis. A high index of suspicion is required when a patient presents with bilateral inguinoscrotal problems. Whenever possible, excision of müllerian structures meticulously, avoiding injury to the vas, must be performed. The aim is to preserve fertility. Orchidopexy should be the goal of management.

Surgical resection therapy of a rare presentation of persistent Mullerian duct syndrome: a case review

Surgical resection therapy of a rare presentation of persistent Mullerian duct syndrome: a case review

Physiological role and diagnostic value of anti-Mullerian hormone in pediatrics

Background. Anti-Mullerian hormone (AMH) has now gained popularity as a marker of ovarian reserve. It is important to determine the place and role of AMH in children. The purpose of this work was to analyze the data of the scientific literature on the role of AMH in pediatric practice. Materials and methods. A review of the literature in PubMed was conducted, limiting itself to articles in English and updating the search in February 2022. The search term was “anti-Mullerian hormone”. A total of 437 manuscripts were found, including 37 review articles. The search was gradually narrowed with filters of clinical trials and systematic reviews to 75 articles. The references of the original and review articles were then checked to ensure a complete review. AMH is responsible for the differentiation of the gonads, provokes the regression of Mullerian ducts in the male fetus, correlates with karyotype, sexual development, levels of luteinizing hormone, follicle-stimulating hormone, and its serum levels reflect the ovarian reserve in women, even in childhood. Serum AMH is high from prenatal life to puberty. In postnatal period, the secretion of AMH by the testes is stimulated by follicle-stimulating hormone and strongly inhibited by androgens. AMH is of clinical value as a marker of testicular tissue in men with differences in sexual development and cryptorchidism, as well as in the assessment of persistent Mullerian duct syndrome. Determination of AMH is useful for assessing the function of the gonads without the need for stimulation tests and guides the etiological diagnosis of childhood male hypogonadism. In women, AMH is used as a prognostic marker of ovarian reserve and fertility. The use of criteria developed for adult women is problematic for adolescent girls, as clinical signs associated with polycystic ovary syndrome are normal phenomena of puberty. AMH can be used as an additional criterion in the diagnosis of polycystic ovary syndrome in adolescents. However, the lack of an international standard for AMH limits comparisons between AMH analyzes. Conclusions. AMH has broad clinical diagnostic utility in pediatrics, but interpretation is often complex and should be made in the context of not only the age and sex, but also the stage of development and puberty of the child. Recognition of the role of AMH beyond the development and maturation of the gonads may lead to new diagnostic and therapeutic applications that will further expand its use in pediatric practice.

Persistent Müllerian duct syndrome associated with genetic defects in the regulatory subunit of myosin phosphatase.

Can mutations of genes other than AMH or AMHR2, namely PPP1R12A coding myosin phosphatase, lead to persistent Müllerian duct syndrome (PMDS)? The detection of PPP1R12A truncation mutations in five cases of PMDS suggests that myosin phosphatase is involved in Müllerian regression, independently of the anti-Müllerian hormone (AMH) signaling cascade. Mutations of AMH and AMHR2 are detectable in an overwhelming majority of PMDS patients but in 10% of cases, both genes are apparently normal, suggesting that other genes may be involved. DNA samples from 39 PMDS patients collected from 1990 to present, in which Sanger sequencing had failed to detect biallelic AMH or AMHR2 mutations, were screened by massive parallel sequencing. To rule out the possibility that AMH or AMHR2 mutations could have been missed, all DNA samples of good quality were analyzed by targeted next-generation sequencing. Twenty-four samples in which the absence of AMH or AMHR2 biallelic mutations was confirmed were subjected to whole-exome sequencing with the aim of detecting variants of other genes potentially involved in PMDS. Five patients out of 24 (21%) harbored deleterious truncation mutations of PP1R12A, the gene coding for the regulatory subunit of myosin phosphatase, were detected. In addition to PMDS, three of these patients presented with ileal and one with esophageal atresia. The congenital abnormalities associated with PMDS in our patients are consistent with those described in the literature for PPP1R12A variants and have never been described in cases of AMH or AMHR2 mutations. The role of chance is therefore extremely unlikely. The main limitation of the study is the lack of experimental validation of the role of PPP1R12A in Müllerian regression. Only circumstantial evidence is available, myosin phosphatase is required for cell mobility, which plays a major role in Müllerian regression. Alternatively, PPP1R12A mutations could affect the AMH transduction pathway. The study supports the conclusion that failure of Müllerian regression in males is not necessarily associated with a defect in AMH signaling. Extending the scope of molecular analysis should shed light upon the mechanism of the initial steps of male sex differentiation. The study was funded by la Fondation Maladies Rares, GenOmics 2021_0404 and la Fondation pour la Recherche Médicale, grant EQU201903007868. The authors report no conflict of interest. N/A.

Squamous Cell Carcinoma Arising from Remnant of Mullerian Duct With 47XYY Karyotype – Rare Case

Persistent Mullerian duct syndrome (PMDS) is a rare type of male pseudohermaphroditism characterized by the presence of Mullerian derivatives (uterus, fallopian tubes and upper vagina), cryptorchidism either unilateral or bilateral along with inguinal hernia in a phenotypically and genotypically male person. PMDS is rarely associated with malignancies, the most common being testicular germ cell tumours. Occasional case of Adenocarcinoma arising in the PMD remnant structure is reported.
 We report an unusual case of male type of PMDS with squamous cell carcinoma (SCC) arising from PMD derivatives and presented with hematuria Also, this patient had karyotype of 47XYY (Jacob syndrome). Both these are hitherto unreported in English literature to the best of our knowledge.

101 A Case Report on Persistent Mullerian Duct Syndrome Presenting as Obstructed Inguinalhernia in an Adult Male

Abstract Persistent Mullerian Duct Syndrome (PMDS) is defined as the presence of müllerian derivatives, uterus, and Fallopian tubes in otherwise normally masculinized 46, XY subjects. It's more common in children with cryptorchidism and is a rare finding during hernia surgery among adults with a risk of malignant degeneration. Here we present the case of a 34-year-old adult male with a history of irreducible swelling in the right groin for 1 day and with a past surgical history of left sided hernia repair 10 years back. On examination a tender, irreducible swelling was noted in the right inguinoscrotal region, opposite side and groin area appeared normal with normal male pattern virilisation. A clinical diagnosis of irreducible right inguinal hernia was made, and the patient was taken up for surgery under spinal anaesthesia. Intraoperatively, right sided sliding hernia with an indirect hernia sac was noted with the contents being degenerated uterus, right fallopian tube connecting to the cornua of uterus along with right gonadal tissue at the fimbrial end within the broad ligament. Consent was taken and hernioplasty was performed after excision of the gonadal tissue along with the fallopian tube. On histopathological examination the gonadal tissue was found to be of testicular origin with fibrosis and degeneration. The diagnosis of persistent Müllerian duct syndrome was confirmed. This case report adds on to the current literature on incidence and presentation of PMDS amongst adults and highlights the importance of knowing the pattern of occurrence of this rare condition.

Diagnostic value of ultrasound in children with transverse testicular ectopia.

ObjectiveThe study aimed to investigate the diagnostic value of ultrasound in children’s transverse testicular ectopia (TTE).Materials and methodsWe retrospectively studies all TTE cases diagnosed in our hospital from January 2017 to December 2021. All cases were evaluated by ultrasound examination and compared to physical examination and diagnostic laparoscopy results.ResultsThis study included 14 TTE patients in total, with a median age was 1.08 years. In the 14 TTE, physical examination found 10 TTE cases, of which nine testes were located in the opposite scrotum, one testis was located in the opposite groin, and the other four testes were not observed by physical examination. All cases were diagnosed by preoperative ultrasound, and nine testes were located in the opposite scrotum, two testes were located in the opposite groin, and three testes were located next to the opposite iliac vessel in the abdominal cavity. Preoperative ultrasound showed the ectopic spermatic cord in six cases (6/14, 42.8%) and persistent Müllerian duct syndrome (PMDS) in one case (1/14, 7%). Diagnostic laparoscopy finally confirmed 14 cases of TTE, which was consistent with preoperative ultrasound, and the coincidence rate was 100% (14/14). Among the 14 cases of TTE, diagnostic laparoscopy showed that 12 cases had ectopic spermatic vessels and vas deferens (12/14, 85.7%), and six cases were associated with PMDS (6/14, 42.8%). When TTE was associated with the ectopic spermatic cord and PMDS, the diagnostic performance of diagnostic laparoscopy was better than that of preoperative ultrasound (P < 0.05). The testis volume of the affected side of TTE was less than that of the contralateral testis (P < 0.05).ConclusionUltrasonography is very helpful for the preoperative diagnosis of TTE in children, and it is suitable as a non-surgical method for locating ectopictestis. Preoperative assessment of the exact presence of PMDS is difficult and unclear. This may be related to factors such as pelvic developmental stages in infancy, examination techniques, and atypical imaging findings of PMDS.

Lessons learned from five patients of persistent Mullerian duct syndrome: A case series.

IntroductionPersistent Mullerian duct syndrome (PMDS) is a genetic disorder characterized by the persistence of Mullerian structures of fallopian tubes, uterus, and upper two-thirds of the vagina in a normal XY male. It is a rare genetic disorder that has been reported less than two hundred times. More rarely it may be seen in association with transverse testicular ectopia (TTE).Presentation of casesFour patients presented with swelling in the inguinal region and undescended testes. Pre-op ultrasound was done on three of these patients and it showed a hernia with testes inside the hernial sac. Hernia surgery was planned for these patients. One patient presented with a complaint of bilateral cryptorchidism that prompted laparoscopic exploration. In all five patients, on surgical exploration, Mullerian derivatives i.e., fallopian tubes, uterus, and vagina were found in the abdomen. Hernia surgery was done and Mullerian structures were excised. For undescended testes, patients had orchiopexy or orchidectomy depending on their respective age group.DiscussionPMDS is caused by failure of production of Mullerian inhibiting substance. Mullerian structures other than causing inguinal hernia are also at risk of malignant transformation, which is the most important significance of this condition. In light of the risk of malignant transformation, Mullerian structures must be excised.ConclusionTo prevent the risk of malignant transformation in PMDS, the Mullerian structures must be excised. If PMDS is associated with TTE, orchiopexy must be done for pediatric patients and orchidectomy for adult patients.

Persistent Mullerian Duct Syndrome with Malignant Conversion of Mullerian Remnant

A case report of a very rare entity of persistent Müllerian duct syndrome with malignant transformation of Mullerian remnant is presented. A twenty-two-year-old phenotypically normal male presented with complain of hematuria and lower abdominal pain. On examination patient had bilateral cryptorchidism and on imaging bilateral duplication of pelvicalyceal system and ureters and left sided ureterocoele. There was a malignant mass lesion with metastatic lymphadenopathy in pelvis. The diagnosis of persistent Mullerian duct syndrome was suspected on MRI pelvis and confirmed by genetic and pathological testings. Risk of malignant transformation of undescended testis and Mullerian remnants increases with age and have impact on psychological health of patient, so early diagnosis becomes crucial.

Reviewing recherche presentations of persistent Mullerian duct syndrome: case reports

BackgroundPersistent Mullerian Duct Syndrome (PMDS) is a type of pseudo-hermaphroditism occurring in males internally due to failure in the Anti Mullerian Hormone (AMH)-dependent sex differentiation pathway. Due to absent Anti Mullerian Hormone (AMH), the adult male has both the Wolffian and Mullerian duct derivatives. Considering the limited information available in the existing literature, malignant transformation in PMDS has rarely been reported.Case PresentationIn this article we present two cases of PMDS which have complicated in to malignancy with review of literature and histopathological correlation. Both of these patients presented with lower abdominal pain, for which computed tomography of abdomen and pelvis was performed following ultrasound of abdomen and pelvis Both the Wolffian and Mullerian duct derivates were visualized on the above mentioned scans in these genetically confirmed males (46 XY).ConclusionFamiliarity with PMDS is necessary to diagnose the condition. Hence, in this article, we report recherche presentations of Persistent Mullerian Duct Syndrome which had complicated into malignancies.

Persistent Mullerian Duct Syndrome with Transverse Testicular Ectopia Presenting as an Irreducible Inguinal Hernia

Persistent Mullerian Duct Syndrome with Transverse Testicular Ectopia Presenting as an Irreducible Inguinal Hernia

Mullerian ducts derivatives in abdominal cryptorchism in children

Disorder of sex determination is a condition associated with clinical and biochemical discrepancy between genetic, gonadal, and phenotypic sex of a child requiring detailed examination for final selection of sex. Indications for sex determination can arise both in infancy and during puberty. Several conditions pertaining to abnormal sex differentiation are manifested as normal male genitalia with Mullerian ducts derivatives.The study objective is to present clinical observations of children with persistent Mullerian duct syndrome.The study presents 2 clinical cases of children with disorders of sex determination. Both children were initially hospitalized at the surgical facility with cryptorchism diagnosis, Mullerian duct derivatives were found intraoperatively. During examination of the children (karyotyping, histological and immunohistochemical examination) in one case persistent Mullerian duct syndrome was diagnosed, in the other - chromosomal abnormality of sex formation (45,X/46,XY), mixed dysgenesis of the gonads.If Mullerian duct derivatives are found during diagnostic laparoscopy, resection biopsy of the ambiguous gonad and intraoperative single-step urethrocystoscopy for visualization of the seminal colliculus and catherization of the uterus for its identification from the abdominal side should be performed. Further examination includes karyotyping, hormonal examination, consultations with an endocrinologist and a geneticist, and genetic examination if necessary.

A 35-year-old father with persistent Mullerian duct syndrome and seminoma of the right undescended testis: a rare case report

BackgroundA persistent Müllerian duct syndrome is a rare disorder of sexual differentiation characterized by the presence of the female reproductive system in a normal male.Case presentationHerein, we report a case of a 35-year-old father with persistent Müllerian duct syndrome and seminoma in the right undescended testis. The exploratory laparotomy was performed and revealed a mass in the right undescended testis and Müllerian duct structures.ConclusionsFor patients with cryptorchidism and inguinal hernia, the persistent Müllerian duct syndrome should be considered, and radiological evaluation of the genitourinary system is recommended for early diagnosis of persistent Müllerian duct syndrome. The persistent Müllerian duct syndrome is usually detected during a surgical operation, and it is considered a risk factor for developing testicular malignancies.

Surgical management and molecular diagnosis of persistent Müllerian duct syndrome in Chinese patients.

Persistent Müllerian duct syndrome (PMDS) is a rare clinically and genetically overlapping disorder caused by mutations in the anti-Müllerian hormone (AMH) gene or the anti-Müllerian hormone receptor type 2 (AMHR2) gene. Affected individuals present uterus and tubes in normally virilized males and are discovered unexpectedly during other surgeries. Since it is rare and complex, a definitive clinical diagnosis can be missed, and there are no guidelines regarding how to deal with the uterus. In the present study, exome sequencing and Sanger verification were performed for causal variants in 12 PMDS patients. Preoperative diagnoses were made by positive exome sequencing in 8 patients. Of them, 7 patients evoked on the basis of ultrasound indicating bilateral testes on the same side of the body. Twelve different AMH variants (2 frameshift/nonsense, 1 deletion, 8 missense, and 1 in-frame) in 9 patients and 6 different AMHR2 variants (5 missense and 1 splicing) in 3 patients were identified. Seven variants were classified as “pathogenic” or “likely pathogenic”, and 4 of them were novel. All but two patients with AMH defects showed low serum AMH concentrations, but all patients with AMHR2 defects showed elevated AMH levels. During surgery, an abnormal vas deferens was observed in half of the patients. Eight patients underwent orchidopexy with uterine preservation. Of them, 2 patients presented complications including irreducible cryptorchidism, and 3 patients developed Müllerian remnant cysts. Three patients underwent subtotal hysterectomy. Of them, one patient had complication of injury to the vas deferens, and one had hemorrhage after operation. This is the first report of PMDS involving a large Chinese population. The present study not only expands the variation spectrum but also provides clinical experience about the management of the uterus.