Diagnosis and Therapy of Female Genital Malformations (Part 2). Guideline of the DGGG, OEGGG and SGGG (S2k Level, AWMF Registry Number 015/052, May 2019).

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This S2k guideline provides a comprehensive summary of clinical examinations, diagnostic workups, and treatment options for female genital malformations across all ages, including complex urogenital anomalies and tumor risks.

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This S2k-level guideline provides a comprehensive summary of the diagnosis and therapy for female genital malformations ranging from infancy to adulthood. Developed through a structured consensus process by representative members of German gynecological societies, it covers clinical examinations, diagnostic workups, and treatment options for various anatomical anomalies. The recommendations additionally address complex urogenital malformations, vascular issues, psychosomatic care, and associated tumor risks. This paper is centrally about endometriosis — specifically, it serves as a diagnostic framework for congenital anomalies that can present with symptoms overlapping those of endometriosis, such as pelvic pain and infertility, though the primary focus remains on structural birth defects rather than the disease pathology itself.

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Abstract

Objectives Female genital malformations may be present in the form of individual entities, they may involve neighboring organs or they may occur in the context of complex syndromes. Given the anatomical structures of the vulva, vagina, uterus and uterine appendages, the clinical picture of malformations varies greatly. Methods This S2k-guideline was developed by representative members from different medical specialties and professions as part of the guidelines program of the DGGG, SGGG and OEGGG. The recommendations and statements were developed and voted on using a structured consensus process with neutral moderation. Recommendations This guideline is the first comprehensive summary of female genital malformations from infancy to adulthood which covers clinical examinations, diagnostic workups and treatment options. Additional chapters have been included on complex urogenital malformations, vascular malformations, psychosomatic care, and tumor risk.
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Abstract

Objectives Female genital malformations may be present in the form of individual entities, they may involve neighboring organs or they may occur in the context of complex syndromes. Given the anatomical structures of the vulva, vagina, uterus and uterine appendages, the clinical picture of malformations varies greatly.

Methods

This S2k-guideline was developed by representative members from different medical specialties and professions as part of the guidelines program of the DGGG, SGGG and OEGGG. The recommendations and statements were developed and voted on using a structured consensus process with neutral moderation. Recommendations This guideline is the first comprehensive summary of female genital malformations from infancy to adulthood which covers clinical examinations, diagnostic workups and treatment options. Additional chapters have been included on complex urogenital malformations, vascular malformations, psychosomatic care, and tumor risk. Key words guideline - genital malformations - subseptate uterus - septate uterus - bicornuate uterus - unicornuate uterus - malformations of the uterine adnexa - uterine vascular malformationsPublication History Received: 28 March 2021 Received: 02 December 2020 Accepted: 28 March 2021 Article published online: 08 December 2021 © 2021. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany - References/Literatur - 1 Hall-Craggs MA, Kirkham A, Creighton SM. Renal and urological abnormalities occurring with Mullerian anomalies. J Pediatr Urol 2013; 9: 27-32 - 2 Wu CQ, Childress KJ, Traore EJ. et al. A Review of Mullerian Anomalies and Their Urologic Associations. Urology 2020; - 3 Ledig S, Wieacker P. Clinical and genetic aspects of Mayer-Rokitansky-Küster-Hauser syndrome. Med Genet 2018; 30: 3-11 - 4 Oppelt P, von Have M, Paulsen M. et al. Female genital malformations and their associated abnormalities. Fertil Steril 2007; 87: 335-342 - 5 Carlson BM. Human Embryology and developmental Biology. 6th ed. St. Louis, MO: Elsevier; 2019 - 6 Edmonds DK. Congenital malformations of the genital tract and their management. Best Pract Res Clin Obstet Gynaecol 2003; 17: 19-40 - 7 Acién P, Acién M. The presentation and management of complex female genital malformations. Hum Reprod Update 2016; 22: 48-69 - 8 Weijenborg PT, ter Kuile MM. The effect of a group programme on women with the Mayer-Rokitansky-Küster-Hauser syndrome. BJOG 2000; 107: 365-368 - 9 Heller-Boersma JG, Schmidt UH, Edmonds DK. A randomized controlled trial of a cognitive-behavioural group intervention versus waiting-list control for women with uterovaginal agenesis (Mayer-Rokitansky-Küster-Hauser syndrome: MRKH). Hum Reprod 2007; 22: 2296-2301

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