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Chirurgische Behandlung weiblicher genitaler Fehlbildungen im Kindesalter

Eine Herausforderung

Pediatric surgery of complex female genital malformations

A challenge

  • Leitthema
  • Published:
Der Gynäkologe Aims and scope

Zusammenfassung

Störungen der sexuellen Differenzierung (DSD) können auf chromosomaler, gonadaler und phänotypischer Ebene ablaufen. Entsprechend ist das Spektrum der resultierenden anatomischen Verhältnisse breit, und die biologischen Zusammenhänge sind komplex. Hinzu kommt, dass in den vergangenen Jahrzehnten eine intensive Auseinandersetzung sowohl der behandelnden Mediziner und Ethiker als auch der Betroffenen mit der Thematik zu einem Umdenken bezüglich vorhandener Therapieempfehlungen geführt hat. Der State-of-the-Art-Artikel vermittelt eine Übersicht über das operative Management von Mädchen (46,XX DSD) mit konnatalem adrenogenitalen Syndrom, Sinus urogenitalis, Kloakalfehlbildungen sowie Blasen- und Kloakalekstrophie. Der Fokus liegt dabei auf der urogenitalen Rekonstruktion mit Darstellung der Kontroversen hinsichtlich Operationsindikation, Operationszeitpunkt und verschiedener operativen Korrekturmöglichkeiten sowie der Langzeitprobleme.

Abstract

Disorders of sexual differentiation (DSD) is an umbrella term for atypical development of chromosomal, gonadal or phenotypic sex. Resulting anatomic conditions and biological mechanisms vary widely. During the past few decades, an intense discussion among physicians, ethicists and concerned persons led to a paradigm shift regarding recommendations of therapy. This article provides a summary of the surgical management of 46,XX DSD girls with congenital adrenal hyperplasia, urogenital sinus, persistent cloaca, and bladder or cloacal exstrophy. The focus lies on surgical reconstruction including the contentious issues of indications for surgery, ideal time point of surgery, different surgical procedures and long-term complications. However, the paradigm of early gender assignment has been challenged by the results of clinical and basic science research, which show that gender identity development likely begins in utero.

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Authors and Affiliations

  1. Abteilung für Kinderchirurgie und Kinderurologie, Universitätsklinikum Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Deutschland

    J. Fuchs &  V. Ellerkamp

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Correspondence to J. Fuchs.

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J. Fuchs und V. Ellerkamp geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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T. Dimpfl, Kassel

S. Brucker, Tübingen

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Fuchs, J., Ellerkamp, V. Chirurgische Behandlung weiblicher genitaler Fehlbildungen im Kindesalter. Gynäkologe 49, 101–110 (2016). https://doi.org/10.1007/s00129-015-3824-7

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