Outflow Obstructions 

  • Author: Latha Chandran, MBBS, MD, MPH; Chief Editor: Andrea L Zuckerman, MD   more...
 
Updated: Jul 6, 2011
 

Background

Genital tract outflow is important for the expulsion of normal secretions from the cervix and vagina. Outflow is also critical for menstrual efflux. Outflow obstruction may occur at different levels with resultant variations in clinical presentation.

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Pathophysiology

Embryologically, the lower two thirds of the vagina develop from the urogenital sinus. The upper vagina, cervix, uterus, fallopian tubes, and ovaries form from the Müllerian duct system. Failure of vertical fusion or canalization of the 2 systems in utero may result in bicornuate uterus, uterus didelphus, cervical stenosis or atresia, vaginal atresia, or longitudinal or transverse vaginal septa. In addition, hymenal tissue may be imperforate.

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Epidemiology

Frequency

United States

Female genital malformations occur in 5% of the general population.[1] The incidence of Müllerian agenesis is about one in 4500 women; 30-36% of such malformations are associated with other anomalies as well, particularly anomalies of the kidneys and skeleton.

International

Imperforate hymen is the most common genital outflow tract anomaly.

Mortality/Morbidity

Patients with undiagnosed imperforate hymen who have reached menarche may have recurrent, cyclic, lower abdominal pain.

Age

Although imperforate hymen is a congenital anomaly and can be detected early in life, imperforate hymen remains undetected until puberty in many patients. At birth, the presence of increased mucous secretions in the vagina secondary to maternal estrogen effects may result in a mucocele appearing as a bulging hymen, which is easily observed.

If not detected at birth, secretions resorb, and the condition may be undetected until puberty, when menstrual blood collects and causes hematocolpos, a bluish-colored bulging hymen.

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Contributor Information and Disclosures
Author

Latha Chandran, MBBS, MD, MPH  Professor of Pediatrics, Vice Dean for Undergraduate Medical Education, Stony Brook University School of Medicine, New York

Latha Chandran, MBBS, MD, MPH is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Coauthor(s)

Joseph A Puccio, MD, FAAP  Director, Division of Adolescent Medicine, Stony Brook University Hospital; Assistant Professor, Department of Pediatrics, Stony Brook University School of Medicine

Joseph A Puccio, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics and Society for Adolescent Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Elizabeth Alderman, MD  Director of Fellowship Training Program, Director, Adolescent Ambulatory Service, Professor, Clinical Pediatrics, Department of Pediatrics, Division of Adolescent Medicine, Albert Einstein College of Medicine and Children's Hospital at Montefiore

Elizabeth Alderman, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, North American Society for Pediatric and Adolescent Gynecology, and Society for Adolescent Medicine

Disclosure: Merck Honoraria Speaking and teaching

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Wayne Wolfram, MD, MPH  Associate Professor, Department of Emergency Medicine, Mercy St Vincent Medical Center

Wayne Wolfram, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Pediatrics, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Daniel Rauch, MD, FAAP  Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine

Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine

Disclosure: Baxter Honoraria Consulting

Chief Editor

Andrea L Zuckerman, MD  Assistant Professor of Obstetrics/Gynecology and Pediatrics, Tufts University School of Medicine; Division Director, Pediatric and Adolescent Gynecology, Tufts Medical Center

Andrea L Zuckerman, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, Association of Professors of Gynecology and Obstetrics, Massachusetts Medical Society, North American Society for Pediatric and Adolescent Gynecology, and Society for Adolescent Medicine

Disclosure: Nothing to disclose.

References
  1. Oppelt P, von Have M, Paulsen M, et al. Female genital malformations and their associated abnormalities. Fertil Steril. Feb 2007;87(2):335-42. [Medline].

  2. Burel A, Mouchel T, Odent S, et al. Role of HOXA7 to HOXA13 and PBX1 genes in various forms of MRKH syndrome (congenital absence of uterus and vagina). J Negat Results Biomed. 2006;5:4. [Medline].

  3. Nayci A, Avlan D, Oz U, et al. Does menstrual flow exclude hematometra? A rare case of uterine anomaly presenting with anorectal malformation. J Pediatr Surg. Apr 2002;37(4):666-7. [Medline].

  4. Kansagra AP, Miller CB, Roberts AC. A novel image-guided balloon vaginoplasty method to treat obstructive vaginal anomalies. J Vasc Interv Radiol. May 2011;22(5):691-4. [Medline].

  5. Cetinkaya SE, Kahraman K, Sonmezer M, Atabekoglu C. Hysteroscopic management of vaginal septum in a virginal patient with uterus didelphys and obstructed hemivagina. Fertility and Sterility. 2011/07;96:e16-e18.

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  8. Haddad B, Barranger E, Paniel BJ. Blind hemivagina: long-term follow-up and reproductive performance in 42 cases. Hum Reprod. Aug 1999;14(8):1962-4. [Medline].

  9. Pletcher JR, Slap GB. Menstrual disorders. Amenorrhea. Pediatr Clin North Am. Jun 1999;46(3):505-18. [Medline].

  10. Altchek A, Deligdish L. Congenital absence of the uterus and the vagina. In: The Uterus: Pathology, Diagnosis and Management. 1991:272-93.

  11. Edmonds DK. Congenital malformations of the genital tract. Obstet Gynecol Clin North Am. Mar 2000;27(1):49-62. [Medline].

  12. Emans SJ, Laufer MR, Goldstein DP. Structural abnormalities of the female reproductive tract. In: Pediatric and Adolescent Gynecology. 1998:303-62.

  13. Spence J, Gervaize P, Jain S. Uterovaginal anomalies: diagnosis and current management in teens. Curr Womens Health Rep. Dec 2003;3(6):445-50. [Medline].

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