Abstract
Mullerian ducts are named after Johannes Peter Müller, a German physiologist who described these ducts in his text in 1830. Mullerian duct anomalies (MDA) are not so rare but are not always detected. Therefore, their true prevalence in the general population is unknown because many are asymptomatic and not noticed. In a recent systematic review, the mean prevalence of mullerian congenital malformations in the general population was up to 7 %. Renal tract anomalies are associated with up to 30 % of cases because of the close embryological relationship between the paramesonephric and mesonephric ducts; various types of uterine anomalies and their classification are reported; MDAs have been classified by the American Society of Reproductive Medicine according to anatomic findings. Müllerian Aplasia is the absence of the uterus, cervix, and upper vagina; it represents the most severe type of MDA, in consisting of segmental agenesis and variable degrees of uterovaginal hypoplasia. The lower third of the vagina is typically present secondary to normal formation of the sinus vagina, and the external genitalia are normal. Incidence of incomplete Müllerian fusion is difficult to estimate, as many patients are asymptomatic; however, the incidence quoted is between 0.1 and 3 %. Uterus didelphys is a symmetric anomaly in which two completely separate uterine cavities are identified. In bicornuate uterus, two symmetric cornua are fused caudally, with communication of the endometrial cavities, most often at the level of the uterine isthmus.
Neonatal Uterine Prolapse is a rare condition; it is most commonly associated with anomalies of the central nervous system, especially the meningomyelocele.
Several case reports of congenital absence of the cervix exist; the cervix may be absent in lieu of a normal uterus and vagina.
Fallopian tube anomalies are rarely observed without an associated uterine anomaly, except for those cases with bifid, uni, or bilateral tube, which are relatively frequent as casual findings in laparoscopy or laparotomy.
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Mullerian Duct Anomalies (MDA)
Connell MT, et al. Genetic syndromes and genes involved in the development of the female reproductive tract: a possible role for gene therapy. J Genet Syndr Gene Ther. 2013;4(2):127.
Woolf RB, Allen WM. Concomitant malformations: the frequent, simultaneous occurrence of congenital malformations of the reproductive and urinary tracts. Obstet Gynecol. 1953;2:236–65.
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;14:415–29.
Junqueira BL, Lisa M, Allen LM, Rachel F, Spitzer RF, et al. Müllerian duct anomalies and mimics in children and adolescents: correlative intraoperative assessment with clinical imaging. Radiographics. 2009;29:1085–103.
The American Fertility Society classifications of adnexal adhesions, distal tubal obstruction, tubal occlusions secondary to tubal ligation, tubal pregnancies, Müllerian anomalies and intrauterine adhesions. Fertil Steril. 1998;49:944–955.
Vallerie AM, Breech LL. Update in Müllerian anomalies: diagnosis, management, and outcomes. Curr Opin Obstet Gynecol. 2010;22:381–7.
Moore KL, Persaud TVN. The developing human. 7th ed. Philadelphia: WB Saunders; 2003. Figure 13–43, p 321.
Müllerian Duct Agenesis (Müllerian Aplasia)
Pittock ST, Babovic-Vuksanovic D, Lteif A. Mayer-Rokitansky-Kuster-Hauser anomaly and its associated malformations. Am J Med Genet A. 2005;135:314–6.
Poulakou-Rebelakou E, Tsiamis C, Tompros N, Creatsas G. The lack of a child, the loss of a throne: the infertility of the first royal couple of Greece (1833–62). J R Coll Physicians Edinb. 2011;41(1):73–7.
Sultan C, Biason-Lauber A, Philibert P. Mayer–Rokitansky–Kuster–Hauser syndrome: recent clinical and genetic findings. Gynecol Endocrinol. 2009;25(1):8–11.
Incomplete Müllerian Fusion
Troiano RN, McCarthy SM. Müllerian duct anomalies: imaging and clinical issues. Radiology. 2004;233(1):19–34.
Rock JA, Breech LL. Surgery for anomalies of mullerian ducts. In: Rock JA, Jones III HW, editors. TeLinde’s operative gynecology. 9th ed. New York: Lippincott Williams & Wilkins; 2003. p. 732–6.
Duplication of Mullerian System
Zurawin RK, Dietrich JE, Heard MJ, Edwards CL. Didelphic uterus and obstructed hemivagina with renal agenesis: case report and review of the literature. J Pediatr Adolesc Gynecol. 2004;17(2):137–41.
Gilsanz V, Clevland RH. Duplication of the Müllerian ducts and genitourinary malformations. Radiology. 1982;144:793–6.
Neonatal Uterine Prolapse
Findley P. Prolapse of the uterus in nulliparous women. Am J Obstet Dis Wom. 1917;75:12.
Malpas P. Genital prolapse and allied conditions. London: Harvey and Blythe; 1955.
McGlone L, Patole S. Neonatal genital prolapse. J Paediatr Child Health. 2004;40:156–7.
Fahmy MAB. Neonatal genital prolapse. Paediatrics.me. 2007;12(4):4–8.
Bader D, Davidovich M, Berger A. Genital prolapse in a preterm female infant. J Perinatol. 1993;13:159–61.
Cervical Anomalies
Fujimoto VY, Miller JH, Klein NA, Soules MR. Congenital cervical atresia: report of seven cases and review of the literature. Am J Obstet Gynecol. 1997;177:1419–25.
Casey AC, Laufer MR. Cervical agenesis: septic death after surgery. Obstet Gynecol. 1997;90:706.
Tubal Anomalies
Lineberry TD, Rodriguez H. Isolated torsion of the fallopian tube in an adolescent: a case report. J Pediatr Adolesc Gynecol. 2000;13(3):135–7.
Krissi H, Shalev J, Bar-Hava I, et al. Fallopian tube torsion: laparoscopic evaluation and treatment of a rare gynecological entity. J Am Board Fam Pract. 2001;14(4):274–7.
Paratubal Cysts
Vlahakis-Miliaras E, Miliaras D, Koutsoumis G, et al. Paratubal cysts in young females as an incidental finding in laparotomies performed for right lower quadrant abdominal pain. Pediatr Surg Int. 1998;13(2–3):141–2.
Barloon TJ, Brown BP, Abu-Yousef MM, et al. Paraovarian and paratubal cysts: preoperative diagnosis using transabdominal and transvaginal sonography. J Clin Ultrasound. 1996;24(3):117–22.
Salamon C, Tornos C, Chi DS. Borderline endometrioid tumor arising in a paratubal cyst: a case report. Gynecol Oncol. 2005;97(1):263–5.
Benign Tubal Tumors
Baeyens K, Fennessy F, Bleday R, et al. CT features of a tubal lipoma associated with an ipsilateral dermoid cyst (2004:6b). Eur Radiol. 2004;14(9):1720–2.
Nielsen VT. Lipoma in the uterine tube. Is it an rarity-or an overlooked condition? Ugeskr Laeger. 1984;146(39):2946–7.
Author information
Authors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Fahmy, M.A.B. (2015). Uterus. In: Rare Congenital Genitourinary Anomalies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43680-6_13
Download citation
DOI: https://doi.org/10.1007/978-3-662-43680-6_13
Published:
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-662-43679-0
Online ISBN: 978-3-662-43680-6
eBook Packages: MedicineMedicine (R0)