title: Persistent mullerian duct syndrome: A 24-year experience.
abstract: 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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Paediatric Surgery, The Children's Hospital & the Institute of Child Health Lahore, Pakistan. Electronic address: msalimc@yahoo.com.
authors.forename: Muhammad
authors.initials: M
authors.lastname: Saleem
authors.affiliation: Department of Gynaecology, Al-Noor Medical Centre, Lahore. Electronic address: atheruzma@yahoo.com.
authors.forename: Uzma
authors.initials: U
authors.lastname: Ather
authors.affiliation: Department of Paediatric Surgery, The Children's Hospital & the Institute of Child Health Lahore, Pakistan. Electronic address: bl_mirza@yahoo.com.
authors.forename: Bilal
authors.initials: B
authors.lastname: Mirza
authors.affiliation: Department of Paediatric Surgery, The Children's Hospital & the Institute of Child Health Lahore, Pakistan. Electronic address: drssiqbal@yahoo.com.
authors.forename: Shahid
authors.initials: S
authors.lastname: Iqbal
authors.affiliation: Department of Paediatric Surgery, The Children's Hospital & the Institute of Child Health Lahore, Pakistan. Electronic address: profafzalsheikh@gmail.com.
authors.forename: Afzal
authors.initials: A
authors.lastname: Sheikh
authors.affiliation: Department of Paediatric Surgery, Mayo Hospital/KEMC, Lahore. Electronic address: mahshaukat@gmail.com.
authors.forename: Mahmood
authors.initials: M
authors.lastname: Shaukat
authors.affiliation: Department of Paediatric Surgery, Mayo Hospital/KEMC, Lahore. Electronic address: dr.tahir@gmail.com.
authors.forename: Muhammad Tahir
authors.initials: MT
authors.lastname: Sheikh
authors.affiliation: Department of General Surgery, BVH, Bahawalpur. Electronic address: farooqahmed56@yahoo.com.
authors.forename: Farooq
authors.initials: F
authors.lastname: Ahmad
authors.affiliation: Department of General Surgery, BVH, Bahawalpur. Electronic address: tmrehan@yahoo.com.
authors.forename: Tariq
authors.initials: T
authors.lastname: Rehan
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D058490:Disorder of Sex Development, 46,XY / Q000175:diagnosis / Q000601:surgery*; D005500:Follow-Up Studies; D005544:Forecasting; D006801:Humans; D010535:Laparoscopy; D007813:Laparotomy; D008297:Male; D009095:Mullerian Ducts / Q000000981:diagnostic imaging / Q000601:surgery*; D056126:Orchiopexy / Q000379:methods*; D012189:Retrospective Studies; D055815:Young Adult
keywords: Hernia utri inguinale; Persistent mullerian duct syndrome; Transverse testicular ectopia; Undescended testes