Diagnosis and Therapy of Female Genital Malformations (Part 1). Guideline of the DGGG, OEGGG and SGGG (S2k Level, AWMF Registry Number 015/052, May 2019)

Abstract

Objectives Female genital malformations may take 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 adnexa, the clinical picture of malformations may vary greatly. Depending on the extent of the malformation, organs of the urinary system or associated malformations may also be involved.

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 using a structured consensus process with neutral moderation and voted on.

Recommendations The guideline is the first comprehensive presentation of the symptoms, diagnosis and treatment options for female genital malformations. Additional chapters on classifications and transition were included.

Key words

guideline - genital malformation - genital reconstruction - MRKH - vaginal septum - subseptate uterus - bladder exstrophy - cloacal malformation

Publikationsverlauf

Eingereicht: 28. März 2021
Eingereicht: 02. Dezember 2020

Angenommen: 28. März 2021

Artikel online veröffentlicht:
08. Dezember 2021

• References/Literatur

• 1 Byrne J, Nussbaum-Blask A, Taylor WS. et al. Prevalence of Mullerian duct anomalies detected at ultrasound. Am J Med Genet 2000; 94: 9-12
• 2 Saravelos SH, Cocksedge KA, Li TC. Prevalence and diagnosis of congenital uterine anomalies in women with reproductive failure: a critical appraisal. Hum Reprod Update 2008; 14: 415-429
• 3 Oppelt P, Renner SP, Brucker S. et al. The V-C-U-A-M-Classification (Vagina-Cervix-Uterus-Adnex-associated Malformation) A new classification for genital malformations. Fertil Steril 2004; 84: 1493-1497
• 4 Grimbizis GF, Gordts S, Di Spiezio Sardo A. et al. The ESHRE-ESGE consensus on the classification of female genital tract congenital anomalies. Gynecol Surg 2013; 10: 199-212
• 5 OʼDey DM, Bozkurt A, Pallua N. The anterior Obturator Artery Perforator (aOAP) flap: surgical anatomy and application of a method for vulvar reconstruction. Gynecol Oncol 2010; 119: 526-530
• 6 Höckel M, Dornhöfer N. Vulvovaginal reconstruction for neoplastic disease. Lancet Oncol 2008; 9: 559-568
• 7 Haddad B, Louis-Sylvestre C, Poitout P. et al. Longitudinal vaginal septum: a retrospective study of 202 cases. Eur J Obstet Gynecol Reprod Biol 1997; 74: 197-199
• 8 Fedele L, Arcaini L, Parazzini F. et al. Reproductive prognosis after hysteroscopic metroplasty in 102 women: life-table analysis. Fertil Steril 1993; 59: 768-772
• 9 Bermejo C, Martinez-Ten P, Recio M. et al. Three-dimensional ultrasound and magnetic resonance imaging assessment of cervix and vagina in women with uterine malformations. Ultrasound Obstet Gynecol 2014; 43: 336-345
• 10 Graupera B, Pascual MA, Hereter L. et al. Accuracy of three-dimensional ultrasound compared with magnetic resonance imaging in diagnosis of Mullerian duct anomalies using ESHRE-ESGE consensus on the classification of congenital anomalies of the female genital tract. Ultrasound Obstet Gynecol 2015; 46: 616-622
• 11 Zhang H, Qu H, Ning G. et al. MRI in the evaluation of obstructive reproductive tract anomalies in paediatric patients. Clin Radiol 2017; 72: 612.e7-612.e15
• 12 Dietrich JE, Millar DM, Quint EH. Obstructive reproductive tract anomalies. J Pediatr Adolesc Gynecol 2014; 27: 396-402
• 13 Cetin C, Soysal C, Khatib G. et al. Annular hymenotomy for imperforate hymen. J Obstet Gynaecol Res 2016; 42: 1013-1015
• 14 Williams CE, Nakhal RS, Hall-Craggs MA. et al. Transverse vaginal septae: management and long-term outcomes. BJOG 2014; 121: 1653-1658
• 15 Preibsch H, Rall K, Wietek BM. et al. Clinical value of magnetic resonance imaging in patients with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome: diagnosis of associated malformations, uterine rudiments and intrauterine endometrium. Eur Radiol 2014; 24: 1621-1627
• 16 Hall-Craggs MA, Williams CE, Pattison SH. et al. Mayer-Rokitansky-Kuster-Hauser syndrome: diagnosis with MR imaging. Radiology 2013; 269: 787-792
• 17 Frank R. The formation of an artificial vagina without operation. Am J Obstet Gynecol 1938; 35: 1053
• 18 Soong YK, Chang FH, Lai YM. et al. Results of modified laparoscopically assisted neovaginoplasty in 18 patients with congenital absence of vagina. Hum Reprod 1996; 11: 200-203
• 19 Callens N, De Cuypere G, De Sutter P. et al. An update on surgical and non-surgical treatments for vaginal hypoplasia. Hum Reprod Update 2014; 20: 775-801
• 20 Schatz T, Huber J, Wenzl R. Creation of a neovagina according to Wharton-Sheares-George in patients with Mayer-Rokitansky-Kuster-Hauser syndrome. Fertil Steril 2005; 83: 437-441
• 21 Gurbuz A, Karateke A, Haliloglu B. Abdominal surgical approach to a case of complete cervical and partial vaginal agenesis. Fertil Steril 2005; 84: 217
• 22 Brucker SY, Gegusch M, Zubke W. et al. Neovagina creation in vaginal agenesis: development of a new laparoscopic Vecchietti-based procedure and optimized instruments in a prospective comparative interventional study in 101 patients. Fertil Steril 2008; 90: 1940-1952
• 23 Rall K, Schickner MC, Barresi G. et al. Laparoscopically assisted neovaginoplasty in vaginal agenesis: a long-term outcome study in 240 patients. J Pediatr Adolesc Gynecol 2014; 27: 379-385
• 24 Lee CL, Jain S, Wang CJ. et al. Classification for endoscopic treatment of mullerian anomalies with an obstructive cervix. J Am Assoc Gynecol Laparosc 2001; 8: 402-408
• 25 Pavone ME, King JA, Vlahos N. Septate uterus with cervical duplication and a longitudinal vaginal septum: a mullerian anomaly without a classification. Fertil Steril 2006; 85: 494.e9-494.e10
• 26 Grimbizis GF, Tsalikis T, Mikos T. et al. Successful end-to-end cervico-cervical anastomosis in a patient with congenital cervical fragmentation: case report. Hum Reprod 2004; 19: 1204-1210
• 27 Fraser IS. Successful pregnancy in a patient with congenital partial cervical atresia. Obstet Gynecol 1989; 74 (3 Pt 2): 443-445