| Imperforate hymen | |
|---|---|
| Specialty | Gynaecology  |
An imperforate hymen is a congenital disorder where a hymen without an opening completely obstructs the vagina. It is caused by a failure of the hymen to perforate during fetal development. It is most often diagnosed in adolescent girls when menstrual blood accumulates in the vagina and sometimes also in the uterus. It is treated by surgical incision of the hymen.
Affected newborns may present with acute urinary retention. [1] In adolescent females, the most common symptoms of an imperforate hymen are cyclic pelvic pain and amenorrhea; other symptoms associated with hematocolpos include urinary retention, constipation, low back pain, nausea, and diarrhea. [2] Other vaginal anomalies can have similar symptoms to an imperforate hymen. Vaginal atresia and a transverse vaginal septum require differentiation. [3] A strong urge to defecate has been observed in a few women. [4]
If untreated or unrecognized before puberty, an imperforate hymen can lead to peritonitis or endometriosis due to retrograde bleeding. Additionally, it can lead to mucometrocolpos (dilatation of the vaginal canal and uterus due to mucus buildup) or hematometrocolpos (dilatation due to buildup of menstrual fluid). Mucometrocolpos and hematocolpos can in turn cause urinary retention, constipation, and urinary tract infection. [1]
An imperforate hymen is formed during fetal development when the sinovaginal bulbs fail to canalize with the rest of the vagina. [5] Although some instances of familial occurrence have been reported, the condition's occurrence is mostly sporadic, and no genetic markers or mutations have been linked to its cause. [6]
An imperforate hymen is most often diagnosed in adolescent girls after the age of menarche with otherwise normal development. [7] In adolescent girls of menarcheal age, the typical presentation of the condition is amennorhea and cyclic pelvic pain, indicative of hematocolpos secondary to vaginal obstruction. An imperforate hymen is usually visible on vaginal inspection as a bulging blue membrane. [7] If hematocolpos is present, a mass is often palpable on abdominal or rectal examination. The diagnosis of an imperforate hymen is usually made based purely on the physical exam, although if necessary the diagnosis can be confirmed by transabdominal, transperineal or transrectal ultrasound. [2]
An imperforate hymen can also be diagnosed in newborn babies and it is occasionally detected on ultrasound scans of the foetus during pregnancy. [7] In newborns the diagnosis is based on the findings of an abdominal or pelvic mass or a bulging hymen. [7] Examination of the normal neonatal vagina usually reveals a track of mucus at the posterior commissure of the labia majora; an absence of mucus may indicate an imperforate hymen or another vaginal obstruction. [1]
A similar condition, cribriform hymen, is diagnosed when the hymen contains many minute openings. [8]
Before surgical intervention in adolescents, symptoms can be relieved by the combined oral contraceptive pill taken continuously to suppress the menstrual cycle or NSAIDs to relieve pain. [9] Surgical treatment of the imperforate hymen by hymenotomy typically involves making cruciate incisions of the hymen, excising segments of hymen from their bases, and draining the vaginal canal and uterus. [10] [11] For affected girls who wish (or whose parents wish) to have their hymens preserved, surgical techniques to excise of a central flange of the hymen can be used. [12] The timing of surgical hymen repair is controversial: some doctors believe it is best to intervene immediately after the neonatal period, while others believe that surgical repair should be delayed until puberty, when estrogenization is complete. [13]
Imperforate hymen is the most common vaginal obstruction of congenital origin. [1] Estimates of the frequency of imperforate hymen vary from 1 in 1,000 to 1 in 10,000 females. [14] [15]
The hymen is a thin piece of mucosal tissue that surrounds or partially covers the vaginal opening. A small percentage are born with hymens that are imperforate and completely obstruct the vaginal canal. It forms part of the vulva and is similar in structure to the vagina. The term comes straight from the Greek, for 'membrane'.
Dyspareunia is painful sexual intercourse due to medical or psychological causes. The term dyspareunia covers both female dyspareunia and male dyspareunia, but many discussions that use the term without further specification concern the female type, which is more common than the male type. In females, the pain can primarily be on the external surface of the genitalia, or deeper in the pelvis upon deep pressure against the cervix. Medically, dyspareunia is a pelvic floor dysfunction and is frequently underdiagnosed. It can affect a small portion of the vulva or vagina or be felt all over the surface. Understanding the duration, location, and nature of the pain is important in identifying the causes of the pain.
Vaginoplasty is any surgical procedure that results in the construction or reconstruction of the vagina. It is a type of genitoplasty. Pelvic organ prolapse is often treated with one or more surgeries to repair the vagina. Sometimes a vaginoplasty is needed following the treatment or removal of malignant growths or abscesses to restore a normal vaginal structure and function. Surgery to the vagina is done to correct congenital defects to the vagina, urethra and rectum. It may correct protrusion of the urinary bladder into the vagina (cystocele) and protrusion of the rectum (rectocele) into the vagina. Often, a vaginoplasty is performed to repair the vagina and its attached structures due to trauma or injury. Labiaplasty, which alters the appearance of the vulva, can be performed as a discrete surgery, or as a subordinate procedure within a vaginoplasty.
Rectal tenesmus is a feeling of incomplete defecation. It is the sensation of inability or difficulty to empty the bowel at defecation, even if the bowel contents have already been evacuated. Tenesmus indicates the feeling of a residue, and is not always correlated with the actual presence of residual fecal matter in the rectum. It is frequently painful and may be accompanied by involuntary straining and other gastrointestinal symptoms. Tenesmus has both a nociceptive and a neuropathic component.
Cryptomenorrhea or cryptomenorrhoea, is a medical condition in which menstrual bleeding occurs but remains hidden due to a congenital septum or atresia blocking the vagina, resulting in symptoms of menstruation without external bleeding. It is commonly seen in cases of imperforate hymen. Specifically the endometrium is shed, but a congenital obstruction such as a vaginal septum or on part of the hymen retains the menstrual flow. A patient with cryptomenorrhea will appear to have amenorrhea but will experience cyclic menstrual pain. The condition is surgically correctable.
Vaginal bleeding is any expulsion of blood from the vagina. This bleeding may originate from the uterus, vaginal wall, or cervix. Generally, it is either part of a normal menstrual cycle or is caused by hormonal or other problems of the reproductive system, such as abnormal uterine bleeding.
The cystocele, also known as a prolapsed bladder, is a medical condition in which a woman's bladder bulges into her vagina. Some may have no symptoms. Others may have trouble starting urination, urinary incontinence, or frequent urination. Complications may include recurrent urinary tract infections and urinary retention. Cystocele and a prolapsed urethra often occur together and is called a cystourethrocele. Cystocele can negatively affect quality of life.
Vaginal atresia is a condition in which the vagina is abnormally closed or absent. The main causes can either be complete vaginal hypoplasia, or a vaginal obstruction, often caused by an imperforate hymen or, less commonly, a transverse vaginal septum. It results in uterovaginal outflow tract obstruction. This condition does not usually occur by itself within an individual, but coupled with other developmental disorders within the female. The disorders that are usually coupled with a female who has vaginal atresia are Mayer-Rokitansky-Küster-Hauser syndrome, Bardet-Biedl syndrome, or Fraser syndrome. One out of every 5,000 women have this abnormality.
Vaginal discharge is a mixture of liquid, cells, and bacteria that lubricate and protect the vagina. This mixture is constantly produced by the cells of the vagina and cervix, and it exits the body through the vaginal opening. The composition, amount, and quality of discharge varies between individuals and can vary throughout the menstrual cycle and throughout the stages of sexual and reproductive development. Normal vaginal discharge may have a thin, watery consistency or a thick, sticky consistency, and it may be clear or white in color. Normal vaginal discharge may be large in volume but typically does not have a strong odor, nor is it typically associated with itching or pain. While most discharge is considered physiologic or represents normal functioning of the body, some changes in discharge can reflect infection or other pathological processes. Infections that may cause changes in vaginal discharge include vaginal yeast infections, bacterial vaginosis, and sexually transmitted infections. The characteristics of abnormal vaginal discharge vary depending on the cause, but common features include a change in color, a foul odor, and associated symptoms such as itching, burning, pelvic pain, or pain during sexual intercourse.
Uterine prolapse is a form of pelvic organ prolapse in which the uterus and a portion of the upper vagina protrude into the vaginal canal and, in severe cases, through the opening of the vagina. It is most often caused by injury or damage to structures that hold the uterus in place within the pelvic cavity. Symptoms may include vaginal fullness, pain with sexual intercourse, difficulty urinating, and urinary incontinence. Risk factors include older age, pregnancy, vaginal childbirth, obesity, chronic constipation, and chronic cough. Prevalence, based on physical exam alone, is estimated to be approximately 14%.
Hematocolpos is a medical condition in which the vagina is pooled with menstrual blood due to multiple factors leading to the blockage of menstrual blood flow. The medical definition of hematocolpos is 'an accumulation of blood within the vagina'. It is often caused by the combination of menstruation with an imperforate hymen. It is sometimes seen in Robinow syndrome, uterus didelphys, or other vaginal anomalies.
Vaginal hypoplasia is the underdevelopment or incomplete development of the vagina. It is a birth defect or congenital abnormality of the female genitourinary system.
Hematometra is a medical condition involving collection or retention of blood in the uterus. It is most commonly caused by an imperforate hymen or a transverse vaginal septum.
Fertility testing is the process by which fertility is assessed, both generally and also to find the "fertile window" in the menstrual cycle. General health affects fertility, and STI testing is an important related field.
Postcoital bleeding (PCB) is non-menstrual vaginal bleeding that occurs during or after sexual intercourse. Though some causes are with associated pain, it is typically painless and frequently associated with intermenstrual bleeding.
Cervical agenesis is a congenital disorder of the female genital system that manifests itself in the absence of a cervix, the connecting structure between the uterus and vagina. Milder forms of the condition, in which the cervix is present but deformed and nonfunctional, are known as cervical atresia or cervical dysgenesis.
Pediatric gynaecology or pediatric gynecology is the medical practice dealing with the health of the vagina, vulva, uterus, and ovaries of infants, children, and adolescents. Its counterpart is pediatric andrology, which deals with medical issues specific to the penis and testes.
Herlyn-Werner-Wunderlich syndrome, also known as OHVIRA is an extremely rare syndrome characterized by a congenital birth defect of the lower abdominal and pelvic organs. It is a type of abnormality of the Müllerian ducts.
Vaginal anomalies are abnormal structures that are formed during the prenatal development of the female reproductive system and are rare congenital defects that result in an abnormal or absent vagina.
Vaginal cysts are uncommon benign cysts that develop in the vaginal wall. The type of epithelial tissue lining a cyst is used to classify these growths. They can be congenital. They can present in childhood and adulthood. The most common type is the squamous inclusion cyst. It develops within vaginal tissue present at the site of an episiotomy or other vaginal surgical sites. In most instances they do not cause symptoms and present with few or no complications. A vaginal cyst can develop on the surface of the vaginal epithelium or in deeper layers. Often, they are found by the woman herself and as an incidental finding during a routine pelvic examination. Vaginal cysts can mimic other structures that protrude from the vagina such as a rectocele and cystocele. Some cysts can be distinguished visually but most will need a biopsy to determine the type. Vaginal cysts can vary in size and can grow as large as 7 cm. Other cysts can be present on the vaginal wall though mostly these can be differentiated. Vaginal cysts can often be palpated (felt) by a clinician. Vaginal cysts are one type of vaginal mass, others include cancers and tumors. The prevalence of vaginal cysts is uncertain since many go unreported but it is estimated that 1 out of 200 women have a vaginal cyst. Vaginal cysts may initially be discovered during pregnancy and childbirth. These are then treated to provide an unobstructed delivery of the infant. Growths that originate from the urethra and other tissue can present as cysts of the vagina.
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