problendoПроблемы ЭндокринологииProblems of Endocrinology0375-96602308-1430Endocrinology Research Centre10.14341/probl2017633201-203problendo-8640Research ArticleКлинические случаиCase ReportsОвотестикулярное нарушение формирования пола у пациента с кариотипом 46,XYOvotesticular disorder of sexual development in a patient with 46,XY karyotypehttps://orcid.org/0000-0003-0361-5329РайгородскаяНадежда ЮрьевнаRaygorodskayaNadezda Y.

д.м.н.

MD, PhD

nraygorodskaya@gmail.comhttps://orcid.org/0000-0002-8148-526XБолотоваНина ВикторовнаBolotovaNina V.

д.м.н., профессор

MD, PhD, Professor

kafedranv@mail.ruhttps://orcid.org/0000-0001-8040-6948ЖарковДанил АнатольевичJarkovDanil A.

к.м.н.

MD, PhD

dadaj@ya.ruhttps://orcid.org/0000-0002-3889-5052ПалатоваТатьяна ВасильевнаPalatovaTatyana V.iamnot88@bk.ruhttps://orcid.org/0000-0002-3958-4299ДоровскаяНаталья СергеевнаDorovskayaNatalya S.PCSO@yandex.ru<p>ФГБУ ВО &laquo;Саратовский государственный медицинский университет им. В.И. Разумовского&raquo; Минздрава России</p>Россия<p>V.I. Razumovskiy Saratov State Medical University</p>Russian Federation<p>ГУЗ &laquo;Клинический перинатальный центр Саратовской области&raquo;</p>Россия<p>Clinical Center of Perinatology</p>Russian Federation201715062017633201203Copyright &#x00A9; Райгородская Н.Ю., Болотова Н.В., Жарков Д.А., Палатова Т.В., Доровская Н.С., 20172017Райгородская Н.Ю., Болотова Н.В., Жарков Д.А., Палатова Т.В., Доровская Н.С.Raygorodskaya N.Y., Bolotova N.V., Jarkov D.A., Palatova T.V., Dorovskaya N.S.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.probl-endojournals.ru/jour/article/view/8640

Овотестикулярное нарушение формирования пола 46,XY является редким вариантом патологической дифференцировки гонад. У пациента в возрасте 1 года 3 мес отмечено двойственное строение наружных половых органов, отсутствие пальпируемых гонад и кариотип 46,XY. При визуализации полости малого таза была обнаружена матка. Гонады располагались на маточных трубах и макроскопически выглядели как овотестис: каждая гонада состояла из двух отсеков, разделенных прослойкой соединительной ткани. При гистологическом исследовании часть гонады была представлена тканью яичника, имела деление на корковое и мозговое вещество, содержала примордиальные фолликулы с выраженными дистрофическими изменениями; другие участки представляли собой незрелый эпителий канальцев с пролиферативными изменениями. В данном случае обоснован выбор женского пола воспитания, определяющий возможность адаптации в период полового созревания. На основании гистологического исследования выбрана тактика хирургического лечения, принято решение о проведении гонадэктомии.

A 46,XY ovotesticular disorder of sexual development is a rare variant of pathological gonadal differentiation. A 15-month-old patient had ambiguous external genitalia, no palpable gonads, and the 46,XY karyotype. The uterus was detected by imaging of the lesser pelvis. Gonads resided on the fallopian tubes and macroscopically resembled ovotestes: each gonad consisted of two compartments separated by a connective tissue interlayer. Histological examination showed that one gonadal portion consisted for ovarian tissue, was differentiated into the cortical and medullary matter, and contained primordial follicles with pronounced dystrophic changes. The remaining portions consisted of immature tubular epithelium with proliferative cellular changes. The decision about bringing up as a female with possible adaptation during puberty was justified in this case. The surgical approach was selected on the basis of histological examination and a decision on performing gonadectomy was made.

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The authors declare that there are no conflicts of interest present.