A 3-year-old boy with ovotestes: gender reassignment and surgical management.
Autor: | Raygorodskaya NY; Department of Pediatric Endocrinology, Saratov Medical University, Saratov, Russian Federation. nraygorodskaya@gmail.com, Chernykh VB, Morozov DA, Olutoye OO, Bolotova NV, Averyanov AP, Maslyakova GN, Shilova NV, Zolotukhina TV |
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Jazyk: | angličtina |
Zdroj: | Journal of pediatric endocrinology & metabolism : JPEM [J Pediatr Endocrinol Metab] 2011; Vol. 24 (7-8), pp. 587-9. |
DOI: | 10.1515/jpem.2011.244 |
Abstrakt: | 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: | MEDLINE |
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