Presentation
Also emphasized are the limitations posed by late presentation and lack facilities in our setting. A 4-day-old girl presented with inability to pass meconium and urine from birth associated with progressive abdominal distention and bilious vomiting. [afrjpaedsurg.org]
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]
Entire Body System
- Neonatal Intestinal Obstruction
Keywords: Hydrometrocolpos, intestinal obstruction, neonate, urinary obstruction, vaginal anomaly How to cite this article: Ekenze SO, Ezegwui HU. [afrjpaedsurg.org]
- Wound Infection
Amnion has antimicrobial properties as it produces lysozyme, hence it decreases the chances of post operative wound infection. [ncbi.nlm.nih.gov]
Respiratoric
- Respiratory Distress
Clinical signs and symptoms in the neonate include an abdominal mass, sepsis, and respiratory distress. [emedicine.staging.medscape.com]
Symptoms and signs in the newborn can be sepsis, abdominal mass, and respiratory distress. [en.wikipedia.org]
Musculoskeletal
- 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]
- Back Pain
Patients usually present with cyclical lower abdominal pain, back pain, lump in abdomen and cryptomenorrhoea. [ijps.org]
Psychiatrical
- Suggestibility
The lower vagina was not visualised in its expected location in sagittal as well as axial images, suggesting vaginal atresia. [eurorad.org]
Information obtained with MRI was superior to ultrasound and CT and is suggested as the examination of choice prior to surgical correction. [ncbi.nlm.nih.gov]
Labial fusion may obscure the anatomy of some patients but is not a suggestive finding unless the fusion is posterior and the clitoris is enlarged anteriorly. This configuration suggests congenital adrenal hyperplasia. [emedicine.medscape.com]
The second patient is a 4-years-old girl with occluded vagina suggesting distal atresia. Saline injected through the membrane eliminates by urethra. Cystoureterography confirms urethrovaginal fistula. [adc.bmj.com]
Neurologic
- 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]
Urogenital
- 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]
Their complaints were amenorrhea and difficulty or pain on attempting sexual intercourse; absence of the vagina and failure to palpate the uterus rectally were features in all ... [ibis-birthdefects.org]
- 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]
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]
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]
- 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]
- Anorchia
Unilateral absence of the ovary may accompany ipsilateral absence of the fallopian tube. 76 This implies pathogenesis involves a vascular accident or torsion after completion of gonadal and ductal differentiation, perhaps analogous to the cause of anorchia [glowm.com]
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. [utexas.influuent.utsystem.edu]
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]
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]
Stenotic coronary lesions significantly affected the long-term prognosis. To assess the long-term outcome following coronary artery bypas... [researchmap.jp]
[…] 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]
Etiology
The other plausible explanation is genetic (etiologic) heterogeneity. [glowm.com]
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]
Epidemiology
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]
[…] 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]
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]
Genit Syst Disor S1. doi: 10.4172/2325-9728.S1-002 Abstract Vaginal Outlet Obstruction - A Review of Cases The paper focuses on anatomical varieties of developmental anomalies of female vagina’s outlet, emphasizing the role of their early recognition in preventing [scitechnol.com]
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]