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Outflow Obstructions

  • Author: Latha Chandran, MBBS, MD, MPH; Chief Editor: Andrea L Zuckerman, MD  more...
 
Updated: Aug 20, 2015
 

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. With appropriate treatment, the prognosis is excellent, and full recovery is typical.

For patient education resources, see the Women's Health Center, as well as Amenorrhea and Female Sexual Problems.

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Pathophysiology

Embryologically, the lower two thirds of the vagina develops 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 two 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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Etiology

The exact etiology of such genital tract anomalies is unclear. Any defect in the normal organogenesis involving the urogenital sinus or the müllerian duct can result in genital tract anomalies. Interest has been focused on abnormalities in the structure; expression or function of the mammalian HOX genes may be a possible etiology for such malformations.[1]

Among patients who have posttransplant graft versus host reactions, scarring of the vagina and vaginal agglutination can result in acquired outflow obstructions similar to those seen in patients with lichen planus lesions of the vagina.

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Epidemiology

Female genital malformations occur in 5-7% of the general population.[2, 3] 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. Imperforate hymen is the most common genital outflow tract anomaly.

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

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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.

Specialty Editor Board

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 Professor, Department of Emergency Medicine, Mercy St Vincent Medical Center; Chairman, Pediatric Institutional Review Board, Mercy St Vincent Medical Center, Toledo, Ohio

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

Disclosure: Nothing to disclose.

Chief Editor

Andrea L Zuckerman, MD Associate Professor of Obstetrics/Gynecology, 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, Massachusetts Medical Society, North American Society for Pediatric and Adolescent Gynecology

Disclosure: Nothing to disclose.

Additional Contributors

Elizabeth Alderman, MD Director, Pediatric Residency Program, Director of Fellowship Training Program, Adolescent Medicine, Professor of 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, Society for Adolescent Health and Medicine

Disclosure: Nothing to disclose.

Acknowledgements

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.

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

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

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

 
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