Outflow Obstructions Clinical Presentation

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

History

  • Although observation of an imperforate hymen can occur upon genital inspection with labial separation, most patients present with history of amenorrhea, recurrent lower abdominal cyclical pain, or lower abdominal midline mass.
  • Severe long-standing obstruction may cause urinary retention, constipation, and hydronephrosis.
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Physical

  • Physical examination reveals imperforation of hymenal tissue. Variations of an imperforate hymen include microperforate, septate, stenotic, and cribriform hymen.
  • If menstruation occurs and hematocolpos develops, the hymen may bulge and exhibit a bluish discoloration.
  • In cases of transverse vaginal septum, physical findings depend on the site of the septum (ie, low, mid, or high). If the site is low, a knee-chest examination may reveal the presence of the bulging septum. The vagina is observed as a blind pouch. Digital examination reveals the septum in the vagina. The most common location is the upper third of the vagina. Septa usually have microperforations; however, these are usually insufficient in preventing hematocolpos.
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Causes

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. Recently, interest has focused on abnormalities in the structure; expression or function of the mammalian HOX genes may be a possible etiology for such malformations.[2]

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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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.

  6. Cooper AR, Merritt DF. Novel use of a tracheobronchial stent in a patient with uterine didelphys and obstructed hemivagina. Fertil Steril. Feb 2010;93(3):900-3. [Medline].

  7. Davies MC, Creighton SM, Woodhouse CR. The pitfalls of vaginal construction. BJU Int. Jun 2005;95(9):1293-8. [Medline].

  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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