title: [Persistent mullerian duct syndrome : Rare incidental finding during treatment of pediatric inguinal hernia].
abstract: BACKGROUND
  Persistent mullerian duct syndrome (PMDS) is a rare, autosomal recessive disorder. It is a form of male disorder of sexual differentiation in which mullerian duct structures are present in male phenotypes and 46XY karyotypes. In affected individuals, uterus, fallopian tubes, cervix and vagina are present.
  
  
  METHODS
  A 2-month-old boy was admitted to hospital with a right-sided inguinal hernia. The physical examination showed a phenotypically normal boy with a right sided indirect inguinal hernia and impalpable testis. During herniotomy, a uterus and two fallopian tubes were found in the pelvic peritoneum adjacent to the two gonads. Initial biopsies were taken from the gonads and blood was sent for karyotyping.
  
  
  RESULTS
  The biopsy showed normal testicular tissue without any ovarian tissue and the karyotyping result was 46XY; therefore, the diagnosis of persistent mullerian duct syndrome (PMDS) was made. In a second laparoscopically assisted operation the uterus and fallopian tubes were dissected, an orchidopexy of the left testis and an orchiectomy of the right testis were performed. The postoperative course was uneventful.
  
  
  CONCLUSIONS
  In the case of an incarcerated inguinal hernia in combination with impalpable testis, a PMDS should be considered as a differential diagnosis.
authors.affiliation: Abteilung für Kinderchirurgie, Klinik und Poliklinik für Allgemein-, Viszeral-, Gefäß- und Kinderchirurgie (Chirurgie I), Zentrum Operative Medizin, Klinikum der Bayerische Julius-Maximilians-Universität, Oberdürrbacher Straße 6, 97080, Würzburg, Deutschland.
authors.forename: P
authors.initials: P
authors.lastname: Sperling
authors.affiliation: Abteilung für Kinderchirurgie, Klinik und Poliklinik für Allgemein-, Viszeral-, Gefäß- und Kinderchirurgie (Chirurgie I), Zentrum Operative Medizin, Klinikum der Bayerische Julius-Maximilians-Universität, Oberdürrbacher Straße 6, 97080, Würzburg, Deutschland. meyer_t@ukw.de.
authors.forename: T
authors.initials: T
authors.lastname: Meyer
mesh_terms: D003937:Diagnosis, Differential; D058490:Disorder of Sex Development, 46,XY / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D006552:Hernia, Inguinal / Q000175:diagnosis* / Q000209:etiology* / Q000601:surgery*; D059685:Herniorrhaphy / Q000379:methods; D006801:Humans; D033162:Incidental Findings; D007223:Infant; D010535:Laparoscopy / Q000379:methods; D008297:Male; D035583:Rare Diseases / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D016896:Treatment Outcome
keywords: Childhood; Disorders of sex development; Inguinal hernia; Persitent muellerian duct syndrom; Undescended testis