title: A 3-year-old boy with ovotestes: gender reassignment and surgical management.
abstract: OBJECTIVE
  We report a male patient with ovotesticular disorder of sex development (OTDSD), resulting from structurally abnormal Y chromosome.
  
  
  METHODS
  A 3-year-old boy was admitted to the Surgical Pediatric Department for masculinizing reconstruction. He had a clitorophallus, bifid scrotum, perineal hypospadias and bilateral impalpable gonads. Pelvic ultrasound and laparoscopy showed a uterus and two gonads with primary ovarian follicles. Chromosome analysis detected a mos 47,XX,mar/46,XX karyotype. Complex genetic evaluation revealed that the marker was Yp isochromosome. Surgical care included a feminizing genitoplasty and separation of the gonads with total excision of testicular tissue.
  
  
  CONCLUSIONS
  The presented case emphasizes the importance of a systematic approach to the investigation and management of the patients with ovotesticular DSD. It also raises the important issue about gender reassignment in intersex individuals in mid-childhood.
authors.affiliation: Department of Pediatric Endocrinology, Saratov Medical University, Saratov, Russian Federation. nraygorodskaya@gmail.com
authors.forename: Nadezhda Y
authors.initials: NY
authors.lastname: Raygorodskaya
authors.forename: Vyacheslav B
authors.initials: VB
authors.lastname: Chernykh
authors.forename: Dmitrey A
authors.initials: DA
authors.lastname: Morozov
authors.forename: Oluyinka O
authors.initials: OO
authors.lastname: Olutoye
authors.forename: Nina V
authors.initials: NV
authors.lastname: Bolotova
authors.forename: Andrey P
authors.initials: AP
authors.lastname: Averyanov
authors.forename: Galina N
authors.initials: GN
authors.lastname: Maslyakova
authors.forename: Nadezhda V
authors.initials: NV
authors.lastname: Shilova
authors.forename: Tatiana V
authors.initials: TV
authors.lastname: Zolotukhina
mesh_terms: D002675:Child, Preschool; D041322:Chromosomes, Human, Y / Q000235:genetics; D006801:Humans; D018404:Isochromosomes; D008297:Male; D009030:Mosaicism; D050090:Ovotesticular Disorders of Sex Development / Q000601:surgery* / Q000628:therapy*; D012729:Sex Chromosome Aberrations; D057829:Gender-Affirming Procedures; D057830:Gender-Affirming Surgery