A 3-year-old boy with ovotestes: gender reassignment and surgical management
| Autor: | Dmitrey A Morozov, Galina N. Maslyakova, Oluyinka O. Olutoye, N. V. Bolotova, Nadezhda V Shilova, A.P. Averyanov, Tatiana V Zolotukhina, Nadezhda Yur'evna Raygorodskaya, Vyacheslav B Chernykh |
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| Rok vydání: | 2011 |
| Předmět: |
Male
endocrine system medicine.medical_specialty Endocrinology Diabetes and Metabolism Isochromosome Y chromosome Perineal hypospadias Endocrinology Sex Reassignment Surgery medicine True hermaphroditism Humans Laparoscopy Sex Chromosome Aberrations Gynecology Chromosomes Human Y medicine.diagnostic_test Mosaicism urogenital system business.industry Sex reassignment surgery (female-to-male) Karyotype medicine.disease Ovotesticular Disorders of Sex Development Isochromosomes Testis determining factor Child Preschool Sex Reassignment Procedures Pediatrics Perinatology and Child Health business |
| Zdroj: | Journal of Pediatric Endocrinology and Metabolism. 24 |
| ISSN: | 2191-0251 0334-018X |
| Popis: | Objective We report a male patient with ovotesticular disorder of sex development (OTDSD), resulting from structurally abnormal Y chromosome. Case report 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. |
| Databáze: | OpenAIRE |
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