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Persistent mullerian duct syndrome: A 24-year experience
Muhammad Saleem1, Uzma Ather2, Bilal Mirza1
1Department of Paediatric Surgery, The Children's Hospital & the Institute of Child Health Lahore, Pakistan.
Background:
Persistence of mullerian duct derivatives in otherwise normal male child is a very rare disorder. This may lead to diagnostic as well as management dilemma.
Materials And Methods:
The medical record of 27 cases of persistent mullerian duct syndrome (PMDS) operated in three teaching hospitals more than a period of 24years is retrieved and analyzed for demography, clinical presentation, investigations, and treatment.
Results:
There were a total of twenty seven male children with PMDS. The age was ranged between 3months and 19years. Ten patients presented with isolated bilateral UDT, six patients with bilateral UDT and unilateral inguinal hernia (4 left and 2 right sided inguinal hernia), and eight patients presented with right inguinal hernia and left sided UDT. Eight of 27 patients showed familial trends i.e. four pairs of brothers had PMDS in our series. In 21 patients, the diagnosis was made incidentally while operating for UDT and inguinal hernia. At operation 5 patients had female type of PMDS and 22 patients had male type PMDS. In 6 patients (male type), the PMDS was associated with transverse testicular ectopia. In 18 patients the initial operation was performed through inguinal incision with excision of mullerian remnants in the same settings in 12 patients. In 4 patients, straightforward laparotomy performed (familial cases) to excise mullerian remnants. In 5 patients, the PMDS was diagnosed on laparoscopy; initially biopsy of these remnants and gonads was done followed by excision of remnants by laparotomy approach. Biopsies taken from gonads in each patient revealed testicular tissue with variable degree of immaturity and dysplasia. The biopsy of mullerian remnants did not reveal any malignancy. All patients were genotypically male.
Conclusion:
Isolated undescended testes, left UDT and right inguinal hernia, bilateral UDT and unilateral inguinal hernia are the main presenting features of PMDS. About 30% of the patients showed familial tendency. Inguinal exploration for UDT or inguinal hernia, and laparoscopy for UDT reveal incidental findings of mullerian remnants. PMDS can be managed as single stage procedure however two stage procedure including gonadal biopsies in first stage followed by mullerian remnants excision and orchidopexy in the second stage can be opted if there is doubt about gonads and genotype.
Insights
Persistent Mullerian Duct Syndrome (PMDS) is a rare disorder in males presenting with undescended testes and inguinal hernias. Diagnosis often occurs during surgery for these conditions, with familial trends noted in some cases.
Area of Science:
- Pediatric Surgery
- Endocrinology
- Genetics
Background:
- Persistent Mullerian Duct Syndrome (PMDS) is a rare congenital disorder in males.
- It involves the presence of Mullerian duct remnants, typically found in females.
- This condition can present diagnostic and management challenges.
Purpose of the Study:
- To analyze the clinical presentation, diagnostic methods, and treatment outcomes of PMDS.
- To identify common presenting features and familial patterns in PMDS patients.
- To evaluate surgical management strategies for PMDS.
Main Methods:
- Retrospective analysis of medical records from 27 male patients with PMDS.
- Data collected over 24 years from three teaching hospitals.
- Included demography, clinical presentation, investigations, and surgical treatments.
Main Results:
- Twenty-seven male children diagnosed with PMDS, aged 3 months to 19 years.
- Common presentations included bilateral undescended testes (UDT), unilateral inguinal hernia, or both.
- Familial trends were observed in approximately 30% of cases; diagnosis was often incidental during surgery for UDT or hernias.
Conclusions:
- Key presenting features of PMDS are isolated UDT, left UDT with right inguinal hernia, or bilateral UDT with unilateral inguinal hernia.
- A significant familial tendency exists in PMDS cases.
- Management can be single-stage, but a two-stage approach may be preferred if gonadal or genotypic uncertainty exists.
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