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Vaginal Atresia


Presentation

  • RESULTS: Five of the seven patients who underwent anoplasty in childhood presented to us in their teens with primary amenorrhea and cyclical abdominal pain. All five had sigmoid colon neovaginoplasty.[ncbi.nlm.nih.gov]
Wound Infection
  • Amnion has antimicrobial properties as it produces lysozyme, hence it decreases the chances of post operative wound infection.[ncbi.nlm.nih.gov]
Severe Abdominal Pain
  • A 14-year-old girl with Robinow syndrome was admitted with severe abdominal pain that had recurred periodically during the last 6 months.[ncbi.nlm.nih.gov]
Short Arm
  • This disorder is caused by an implication in the WNT4 protein coding gene, which is found on the short arm (p) of chromosome 1.[en.wikipedia.org]
Neglect
  • Neglected foreign bodies in the vagina following induced abortion, pelvic inflammatory disease and radiotherapy have also been documented to result in a similar abnormality [ 4 ].[ncbi.nlm.nih.gov]
Amenorrhea
  • Primary amenorrhea, periodic abdominalgia and abdominal pain are the main reasons for the post pubertal patients to visit doctors.[ncbi.nlm.nih.gov]
  • Learn about this topic in these articles: amenorrhea In reproductive system disease: Amenorrhea A less rare abnormality is vaginal atresia, or closure, an obstruction of the vagina by a membrane just above the level of the hymen; menstruation occurs,[britannica.com]
Primary Amenorrhea
  • Primary amenorrhea, periodic abdominalgia and abdominal pain are the main reasons for the post pubertal patients to visit doctors.[ncbi.nlm.nih.gov]
  • It is considered the second most common cause of primary amenorrhea. The estimated Incidence is at 2 in 10000 women. The most common symptom of the disease is cyclic abdominal pain.[radiopaedia.org]
  • Presentation Vaginal atresia presents most commonly with primary amenorrhea in a girl with a normal XX genotype, ovarian and hormone function. In other patients, it may present with cyclical abdominal pain.[news-medical.net]
Dyspareunia
  • The third patient (with dyspareunia) resorted to paraurethral coitus for more than 2 years before seeking advice. The management of vaginal atresia proved difficult because of the recurrence of stenosis at the site of the excised membrane.[ncbi.nlm.nih.gov]
  • Other presenting symptoms include the inability to use tampons or dyspareunia in cases of partial vaginal atresia.[emedicine.staging.medscape.com]
  • The primary presenting signs and symptoms included primary amenorrhea (71.8%), periodic abdominalgia (41.0%), abdominal pain (36.0%), dyspareunia (10.3%), menstrual disorders (5.1%), and pelvic mass (5.1%).[link.springer.com]
Enlarged Uterus
  • The patient primarily presented with amenorrhea and cyclic abdominal pain; transabdominal ultrasonography revealed an enlarged uterus due to hematometra and absence of the lower segment of the vagina.[ncbi.nlm.nih.gov]

Workup

  • CONCLUSIONS: Vaginal atresia with imperforate anus is a rare and an extensive pre-operative workup of females with imperforate anus must include assessment of vagina patency.[ncbi.nlm.nih.gov]
  • Physical examination is a fundamental component of the workup, but the findings are often inadequate to establish a definitive diagnosis.[emedicine.staging.medscape.com]

Treatment

  • The treatment of imperforate hymen is well defined, but the treatment of vaginal atresia is more complex. We encountered two cases of hydrocolpos secondary to distal vaginal atresia, that were operated on in the first days of life.[ncbi.nlm.nih.gov]
  • There are several methods of treatment for individuals with vaginal atresia. The first method of treatment that is recommended would be self-dilation of the vagina.[en.wikipedia.org]

Prognosis

  • The prognosis for vaginal atresia is one that is complicated. There are variations in patients' anatomic findings as well as an absence in consistent surgical techniques which makes it difficult to give a prognosis for this condition.[en.wikipedia.org]
  • […] atresia is reported to be the second most common cause of primary amenorrhea in tertiary care centers. [7] Transverse vaginal septum, formerly called partial vaginal agenesis, is relatively uncommon, with a reported incidence of 1 in 70,000 females. [8] Prognosis[emedicine.medscape.com]
  • In acquired atresia the prognosis as to ultimate results is less favorable in proportion to the extent of the adhesions, and can only be said to be good when they are low in the vagina.[homeoint.org]

Etiology

  • Etiology The specific molecular mechanisms leading to failure of vaginal development have not been elucidated. Multiple developmental pathways share a critical event that may promote or interfere with normal development.[emedicine.medscape.com]
  • The other plausible explanation is genetic (etiologic) heterogeneity.[glowm.com]

Epidemiology

  • […] production of MIS in the female embryonic gonad Regional absence or deficiency of estrogen receptors in the lower müllerian duct structures Arrest of müllerian duct development by a teratogenic agent Mesenchymal inductive defect Sporadic gene mutation Epidemiology[emedicine.medscape.com]
  • Different classification systems have led to some uncertainty regarding epidemiology with some concerns that the classifications may lead to underdiagnosis or overdiagnosis. [ 4 ] Surgical techniques to correct genital malformations depend on the type[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • Given this developmental scheme, pathophysiologic events resulting in a septate uterus can be attributed to failure of the septum to regress between the fused müllerian ducts.[emedicine.medscape.com]
  • Bardet-Biedl syndrome: Genetics, molecular pathophysiology, and disease management. Indian J Ophthalmol. 2016;64(9): 620-627. Hufnagel RB, Arno G, Hein ND, et al.[rarediseases.org]

Prevention

  • This reconstruction technique should obviate dilatation in the older child and prevent further stenosis.[ncbi.nlm.nih.gov]
  • Early diagnosis allows appropriate therapeutic management and prevents subsequent complications. Keywords Hemivagina Herlin-Werner-Wunderlich Syndrome Ipsilateral renal agenesis uterus didelphys OHVIRA urethrovaginal fistula.[adc.bmj.com]
  • It also helps to prevent adhesions and rectal injury. Robotic surgery is now becoming increasingly used. References Callahan, T. L., & Caughey, A. B. (2009). Blueprints obstetrics & gynecology.[news-medical.net]

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