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
- Pain
Primary amenorrhea, periodic abdominalgia and abdominal pain are the main reasons for the post pubertal patients to visit doctors. [ncbi.nlm.nih.gov]
The interrogation raises the notion of cyclic chronic pelvic pain. [panafrican-med-journal.com]
(4) Missed menstrual period (3) Molluscum contagiosum (1) Myoma (benign muscle tumor) (4) Nipple leakage (2) Ovarian abscess (3) Ovarian cyst (4) Ovarian mass growth (3) Pain during sex (4) Pelvic pain (3) Periductal inflammation (1) Phimosis (1) Placenta [hulihealth.com]
- Surgical Procedure
This drainage did result in dramatically improved clinical status, and findings of local inflammation resolved within 4-6 weeks, which facilitated a later successful definitive surgical procedure. [ncbi.nlm.nih.gov]
For each malformation, it includes indications, timing, surgical illustration, contraindication, anaesthesia, surgical procedure, postoperative treatment and key points. [books.google.de]
Results The surgical procedure was successful and uneventful in all cases. [academic.oup.com]
Abstract Background: Many reconstructive surgical procedures have been used for vaginal agenesis. Almost all of them are surgically challenging, multi-staged, time consuming or leave permanent scars on abdomen or skin retrieval sites. [omicsonline.org]
Surgical Procedures When the atresia is due to urogenital anomaly, resulting in imperforate hymen, transverse vaginal septum, or complete atresia of the lower vagina, this is usually corrected by surgery, using the perineal approach. [news-medical.net]
- Sepsis
Drainage through the sinus was unsuccessful because the baby developed sepsis by trapping urine in the uterus. Finally an abdominoperineal vaginal pull-through was successfully done. The embryology and literature are reviewed. [utexas.influuent.utsystem.edu]
[…] anomalies of complete vaginal atresia and partial cervical agenesis, and highlight the limitations of magnetic resonance imaging for definitive initial diagnosis, and consequently the importance of early definitive management, to avoid life-threatening sepsis [ncbi.nlm.nih.gov]
Clinical signs and symptoms in the neonate include an abdominal mass, sepsis, and respiratory distress. [emedicine.staging.medscape.com]
- Asymptomatic
These patients are usually asymptomatic until menarche. There may be associated with renal and urinary tract anomalies. We describe two cases of young girls with occluded vagina associated with urethrovaginal fistula. [adc.bmj.com]
Children are usually asymptomatic, but they may present with amenorrhea and a distended upper vagina during adolescence. [aafp.org]
However, most neonates with vaginal atresia are asymptomatic, and palpation of an abdominal mass during the first few weeks of life or an abnormal result on ultrasonographic screening frequently raises suspicion of the diagnosis. [emedicine.staging.medscape.com]
We believe that, in the event of a diagnosis established by chance during an imaging examination carried out for another reason in asymptomatic prepubertal patients, the vaginal reconstruction should be postponed to pubertal age as soon as the hematocolpos [panafrican-med-journal.com]
- Swelling
[…] obstruction in newborn has also been reported to follow a high vaginal septum, cloacal malformation, or an imperforate hymen. [4] Presentation of hydrometrocolpos depends on the degree of compression of the surrounding structures by the uterovaginal swelling [afrjpaedsurg.org]
This fact was amply demonstrated in a young girl (Case 16) who presented at puberty with a painless cystic swelling rising upto the umblicus. She had marked difficulty in micturation. [jpma.org.pk]
Occasionally, the duct of the gland becomes obstructed, causing unilateral vulvar swelling. Bartholin's gland abnormalities are uncommon in children. [aafp.org]
On laparotomy the uterus and both ovaries were found to be of normal size (Fig.4) .There was cystic swelling at the region of cervix, which when incised anteriorly a dark brown coloured semiliquid material came out (Fig.5). [ispub.com]
Respiratoric
- Dyspnea
Browse recently published Learning/CME Learning/CME View all learning/CME CME A Trial of a Shorter Regimen for Rifampin-Resistant Tuberculosis Case 10-2019: A 69-Year-Old Man with Progressive Dyspnea Mucinous Ovarian Carcinoma Challenge Yourself Interactive [nejm.org]
If the patient develops dyspnea and pneumoderma during or after this procedure, a chest radiogram or thoracoabdominal CT should be taken for diagnostic purposes. [giornalechirurgia.it]
Gastrointestinal
- Abdominal Pain
Primary amenorrhea, periodic abdominalgia and abdominal pain are the main reasons for the post pubertal patients to visit doctors. [ncbi.nlm.nih.gov]
Clinical History A 15 years-old female patient referred to the hospital with delayed menarche, pelvic discomfort and lower abdominal pain increasing for the last 2 months. [eurorad.org]
- Abdominal Mass
A mullerian duct duplication with a vaginal atresia can present as a painful abdominal mass in a female caused by a hematometrocolpos and a hemosalpinx. [ncbi.nlm.nih.gov]
A large abdominopelvic mass displacing the bowel to the right hypochondrium was seen on plain abdominal radiograph. Ultrasonography was not done because this was not available as emergency service. [afrjpaedsurg.org]
Clinical signs and symptoms in the neonate include an abdominal mass, sepsis, and respiratory distress. [emedicine.staging.medscape.com]
Abstract Cystic lower abdominal masses in female infants may be due to enormous distention of the upper vagina, secondary to either atresia of the vaginal canal or to an imperforate hymen. [pediatrics.aappublications.org]
- Lower Abdominal Pain
Clinical History A 15 years-old female patient referred to the hospital with delayed menarche, pelvic discomfort and lower abdominal pain increasing for the last 2 months. [eurorad.org]
Primary amenorrhea and cyclic, cramping lower abdominal pain was the common symptoms of all the patients. In this work we report a new case of such pathology in a 23 old woman affected also by harmonic nanism. [ncbi.nlm.nih.gov]
Mayer-Rokitansky-Kuster, Kaufman-McKusick, Fraser, Winter, and congenital adrenal hyperplasia syndromes are the known examples of such associations. 2 A 15-year-old girl presented to our hospital with a complaint of lower abdominal pain. [nzma.org.nz]
Case history A 16 year old girl attended the gynaecology OPD with complain of cyclical lower abdominal pain for two months. She had not attended menarche. No similar history in her sibling. [ispub.com]
Skin
- Purpura
Haug Browse recently published Learning/CME Learning/CME View all learning/CME CME Case 3-2019: A 70-Year-Old Woman with Fever, Headache, and Progressive Encephalopathy Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura Randomized [nejm.org]
Eyes
- Prolapse
Postpartum surgical management of the uterine and vaginal prolapse is described. [ncbi.nlm.nih.gov]
[…] of uterus (3) Rectal prolapse (3) Recurrent miscarriage (3) Sagging eyelid (1) Supernumerary surgery (2) Thelarche (2) Thyroid cancer (1) Tongue tumor removal (1) Tuboovarian abscess (3) Uncontrolled menstruation (4) Urinary tract infection (UTI) (3) [hulihealth.com]
Focus on today's hot topics in urology, including stress and mixed urinary incontinence; prolapse therapy; therapy for overactive bladder; and targeted therapy for renal cell carcinoma. [books.google.com]
The only risk is of pain and potentially of vault prolapse in the long term. [news-medical.net]
Urethral prolapse and prolapsed ureterocoele can also mimic imperforate hymen. [6] Most of cases of the imperforate hymen present during puberty although diagnosis in utero and neonates and infancy are also documented. [1], [3], [7], [8] The incidence [ijhas.in]
Urogenital
- Amenorrhea
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, periodic abdominalgia and abdominal pain are the main reasons for the post pubertal patients to visit doctors. [ncbi.nlm.nih.gov]
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]
We report the uncommon case of a 14-year-old girl with a primary amenorrhea associated to a cyclical pelvic pain, in whom examination objectified a vaginal cup that replaced the introitus. [panafrican-med-journal.com]
- Imperforate Hymen
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]
Imaging also revealed imperforate hymen, vaginal atresia, and mucocolpos. [ncbi.nlm.nih.gov]
[…] of imperforate hymen is 0.014-1%. [3] Imperforate hymen can also present as pyocolpos in neonates. [11] Vaginal atresia, transverse vaginal septum and urogenital sinus need to be differentiated from imperforate hymen. [ijhas.in]
Clinical examination can reveal the imperforate hymen with a bulge. Imperforate hymen is the most frequent congenital abnormality of the female genital tract and its surgical treatment involves a simple hymenotomy. [eurorad.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]
Rarely, the diagnosis may be late for a woman with primary amenorrhea but who consults for difficult sexual intercourse because of insufficient vaginal penetration or who comes for primary infertility consultation when the vaginal cup has been sufficiently [panafrican-med-journal.com]
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]
- Pelvic Pain
The interrogation raises the notion of cyclic chronic pelvic pain. [panafrican-med-journal.com]
A 16-year-old girl with cyclic hematuria, periodic pelvic pain, and primary amenorrhea. Diagnostic studies, followed by an abdominoperineal-vaginal pull-through procedure. Reconstruction of the urethrovaginal fistula and vaginal route. [ncbi.nlm.nih.gov]
Differential diagnosis in peripubertal girls presenting with pelvic pain and pelvic mass can be varied. Some of the common causes would include an ovarian cyst or ovarian neoplasm, urinary retention and pelvic kidney. [eurorad.org]
(4) Missed menstrual period (3) Molluscum contagiosum (1) Myoma (benign muscle tumor) (4) Nipple leakage (2) Ovarian abscess (3) Ovarian cyst (4) Ovarian mass growth (3) Pain during sex (4) Pelvic pain (3) Periductal inflammation (1) Phimosis (1) Placenta [hulihealth.com]
- 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]
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]
Other presenting symptoms include the inability to use tampons or dyspareunia in cases of partial vaginal atresia. [emedicine.staging.medscape.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]
Prior to the operation, she received an informed consultation on the prognosis of her fertility. A verbal and written consent for the procedure was collected from her legal guardian. The patient underwent then a perineal vaginoplasty. [panafrican-med-journal.com]
[…] 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]
We also wish to bring to the light of obstetricians that numerous pelvic examinations or difficult vaginal delivery may lead to genital tract trauma, and thus, must be minimised in an attempt to prevent postpartum genital tract adhesions. pain reproductive [casereports.bmj.com]
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]