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A rectovaginal fistula is a medical condition where there is a fistula or abnormal connection between the rectum and the vagina.
Rectovaginal fistula may be extremely debilitating. If the opening between the rectum and vagina is wide it will allow both flatulence and feces to escape through the vagina, leading to fecal incontinence. There is an association with recurrent urinary and vaginal infections. The fistula may also connect the rectum and urethra, which is called recto-urethral fistula. Either conditions can lead to labial fusion. This type of fistula can cause pediatricians to misdiagnose imperforate anus. The severity of the symptoms will depend on the size of fistula. Most often, it appears after about one week or so after delivery.
Anal fistulae can present with the following symptoms:
- skin maceration
- pus, serous fluid and/or (rarely) feces discharge — can be bloody or purulent
- pruritus ani — itching
- depending on presence and severity of infection:
Depending on their relationship with the internal and external sphincter muscles, fistulae are classified into five types:
- Extrasphincteric fistulae begin at the rectum or sigmoid colon and proceed downward, through the levator ani muscle and open into the skin surrounding the anus. Note that this type does not arise from the dentate line (where the anal glands are located). Causes of this type could be from a rectal, pelvic or supralevator origin, usually secondary to Crohn's disease or an inflammatory process such as appendiceal or diverticular abscesses.
- Suprasphincteric fistulae begin between the internal and external sphincter muscles, extend above and cross the puborectalis muscle, proceed downward between the puborectalis and levator ani muscles, and open an inch or more away from the anus.
- Transphincteric fistulae begin between the internal and external sphincter muscles or behind the anus, cross the external sphincter muscle and open an inch or more away from the anus. These may take a 'U' shape and form multiple external openings. This is sometimes termed a 'horseshoe fistula.'
- Intersphincteric fistulae begin between the internal and external sphincter muscles, pass through the internal sphincter muscle, and open very close to the anus.
- Submucosal fistulae pass superficially beneath the submucosa and do not cross either sphincter muscle.
Urethral diverticula are often asymptomatic and symptoms that are present tend to be nonspecific. They can co-occur with cancer, in approximately 6-9% of cases, most commonly adenocarcinoma, but also including squamous cell carcinoma and transitional cell carcinoma. Approximately 10% of cases co-occur with kidney stones.
There are 2 types of urethral diverticulums. Congenital and acquired. In infancy usually the urethral diverticulum is congenital but in rare instances acquired urethral diverticulum can be seen in infancy specially following traumatic catheterization.
Common symptoms of urethral diverticulum include incontinence, urinary frequency and urgency, pain during sex, and pain during urination. Other symptoms include pain localized to the urethra or pelvis and frequent urinary tract infection.
When urethral diverticulum becomes severe, a painful mass can sometimes be felt inside the introitus of the vagina, which can discharge pus. If the mass is hard or bleeds, complications like cancer or kidney stones may be present.
A ureterovaginal fistula is an abnormal passageway existing between the ureter and the vagina. It presents as urinary incontinence. Its impact on women is to reduce the "quality of life dramatically."
If a colostomy is not performed immediately after birth, patients with rectovestibular fistulae may present later in life with complications including severe constipation and megacolon (abnormal dilation of the colon), requiring colostomy or further surgery.
A urerovaginal fistula is a result of trauma, infection, pelvic surgery, radiation treatment and therapy, malignancy, or inflammatory bowel disease. Symptoms can be troubling for women especially since some clinicians delay treatment until inflammation is reduced and stronger tissue has formed. The fistula may develop as a maternal birth injury from a long and protracted labor, long dilation time and expulsion period. Difficult deliveries can create pressure necrosis in the tissue that is being pushed between the head of the infant and the softer tissues of the vagina, ureters, and bladder.
Radiographic imaging can assist clinicians in identifying the abnormality. A Ureterovaginal fistula is always indicative of an obstructed kidney necessitating emergency intervention followed later by an elective surgical repair of the fistula.
Rectovaginal fistulas are often the result of trauma during childbirth (in which case it is known as obstetric fistula) where improper medical interventions are used, such as episiotomy with forceps/vacuum extraction or in situations where there is inadequate health care, such as in some developing countries. Rectovaginal fistula is said to be known as the leading cause in maternal death in developing countries. Risk factors include prolonged labour, difficult instrumental delivery and paramedian episiotomy. Rates in Eritrea are estimated as high as 350 per 100,000 vaginal births. Fistulas can also develop as a result of physical trauma to either the vagina or anus, including from rape. Women with rectovaginal fistulae are often stigmatized in developing countries, and become outcasts.
Rectovaginal fistula can also be a symptom of various diseases, including infection by lymphogranuloma venereum, or the unintended result of surgery, such as episiotomy or sexual reassignment surgery. They may present as a complication of vaginal surgery, including vaginal hysterectomy. They are a recognized presentation of rectal carcinoma or rarely diverticular disease of the bowel or Crohn's disease. They are seen rarely after radiotherapy treatment for cervical cancer.
A urethral diverticulum is a condition where the urethra or the periurethral glands push into the connective tissue layers (fascia) that surround it.
Vesicovaginal fistula, or VVF, is an abnormal fistulous tract extending between the bladder (or vesico) and the vagina that allows the continuous involuntary discharge of urine into the vaginal vault.
In addition to the medical sequela from these fistulas, they often have a profound effect on the patient's emotional well-being.
A rectovestibular fistula, also referred to simply as a vestibular fistula, is an anorectal congenital disorder where an abnormal connection (fistula) exists between the rectum and the vulval vestibule of the female genitalia.
If the fistula occurs within the hymen, it is known as a rectovaginal fistula, a much rarer condition.
Various types of fistulas include:
Although most fistulas are in forms of a tube, some can also have multiple branches.
Fistulas can develop in various parts of the body. The following list is sorted by the International Statistical Classification of Diseases and Related Health Problems.
Vesicovaginal fistula (VVF) is a subtype of female urogenital fistula (UGF).
Symptoms of obstetric fistula include:
- Flatulence, urinary or fecal incontinence, which may be continual or only happen at night
- Foul-smelling vaginal discharge
- Repeated vaginal or urinary tract infections
- Irritation or pain in the vagina or surrounding areas
- Pain during sexual activity
Other effects of obstetric fistulae include stillborn babies due to prolonged labor, which happens 85% to 100% of the time, severe ulcerations of the vaginal tract, "foot drop", which is the paralysis of the lower limbs caused by nerve damage, making it impossible for women to walk, infection of the fistula forming an abscess, and up to two-thirds of the women become amenorrhoeic.
Obstetric fistulae have far-reaching physical, social, economic, and psychological consequences for the women affected.
According to UNFPA, “Due to the prolonged obstructed labour, the baby almost inevitably dies, and the woman is left with chronic incontinence. Unable to control the flow of urine or faeces, or both, she may be abandoned by her husband and family and ostracized by her community. Without treatment, her prospects for work and family life are virtually non-existent.”
Anorectal anomalies are medical problems affecting the structure of the anus and rectum. A person with an anorectal problem would have some sort of deformative feature of the anus or rectum, collectively known as an anorectal malformation.
Examples of anorectal anomalies include:
- Anal stenosis
- Imperforate anus
- Proctitis
- Anal bleeding
- Anal fistula
- Anal cancer
- Anal itching
- Hemorrhoid (piles)
Hydrocolpos is the distension of the vagina caused by accumulation of fluid due to congenital vaginal obstruction. The obstruction is often caused by an imperforate hymen or less commonly a transverse vaginal septum. The fluid consists of cervical and endometrial mucus or in rare instances urine accumulated through a vesicovaginal fistula proximal to the obstruction. In some cases it is associated with Bardet-Biedl Syndrome. If it occurs in prepubertal girls, it may show up as abdominal swelling. It may be detected by using ultrasound. It may also present at birth as a distended lower abdomen and vagina. It also associated with vaginal atresia.
Obstetric fistula is a medical condition in which a hole develops in the birth canal as a result of childbirth. This can be between the vagina and rectum, ureter, or bladder. It can result in incontinence of urine or feces. Complications may include depression, infertility, social isolation, and poverty
Risk factors include obstructed labor, poor access to medical care, malnutrition, and teenage pregnancy. The underlying mechanism is poor blood flow to the affected area for a prolonged period of time. Diagnosis is generally based on symptoms and may be supported by use of methylene blue.
Obstetric fistulas are almost entirely preventable with appropriate use of cesarean section. Treatment is typically by surgery. If treated early the use of a urinary catheter may help with healing. Counselling may also be useful. An estimated 2 million women in sub-Saharan Africa, Asia, the Arab region, and Latin America have the condition with about 75,000 new cases developing a year. It occurs very rarely in the developed world. It is considered a disease of poverty.
Thyroglossal duct cysts most often present with a palpable asymptomatic midline neck mass above the level of the hyoid bone. The mass on the neck moves during swallowing or on protrusion of the tongue because of its attachment to the tongue via the tract of thyroid descent. Some patients will have neck or throat pain, or dysphagia.
The persistent duct or sinus can promote oral secretions, which may cause cysts to become infected. Up to half of thyroglossal cysts are not diagnosed until adult life. The tract can lie dormant for years or even decades, until some kind of stimulus leads to cystic dilation. Infection can sometimes cause the transient appearance of a mass or enlargement of the cyst, at times with periodic recurrences. Spontaneous drainage may also occur. Differential diagnosis are ectopic thyroid, enlarged lymph nodes, dermoid cysts and goiter.
Thyroglossal cyst usually presents as a midline neck lump (in the region of the hyoid bone) that is usually painless, smooth and cystic, though if infected, pain can occur. There may be difficulty breathing, dysphagia (difficulty swallowing), or dyspepsia (discomfort in the upper abdomen), especially if the cyst becomes large.
The most common location for a thyroglossal cyst is midline or slightly off midline, between the isthmus of the thyroid and the hyoid bone or just above the hyoid bone. A thyroglossal cyst can develop anywhere along a thyroglossal duct, though cysts within the tongue or in the floor of the mouth are rare.A thyroglossal cyst will move upwards with protrusion of the tongue.Thyroglossal cysts are associated with an increased incidence of ectopic thyroid tissue. Occasionally, a lingual thyroid can be seen as a flattened strawberry-like lump at the base of the tongue.
An infected thyroglossal duct cyst can occur when it is left untreated for a certain amount of time or simply when a thyroglossal duct cyst hasn't been suspected. The degree of infection can be examined as major rim enhancement has occurred, located inferior to the hyoid bone. Soft tissue swelling occurs, along with airway obstruction and trouble swallowing, due to the rapid enlargement of the cyst.
With infections, there can be rare cases where an expression of fluid is projected into the pharynx causing other problems within the neck.
There are several forms of imperforate anus and anorectal malformations. The new classification is in relation of the type of associated fistula.
The classical Wingspread classification was in low and high anomalies:
- A low lesion, in which the colon remains close to the skin. In this case, there may be a stenosis (narrowing) of the anus, or the anus may be missing altogether, with the rectum ending in a blind pouch.
- A high lesion, in which the colon is higher up in the pelvis and there is a fistula connecting the rectum and the bladder, urethra or the vagina.
- A persistent cloaca (from the term cloaca, an analogous orifice in reptiles and amphibians), in which the rectum, vagina and urinary tract are joined into a single channel.
Imperforate anus is usually present along with other birth defects—spinal problems, heart problems, tracheoesophageal fistula, esophageal atresia, renal anomalies, and limb anomalies are among the possibilities.
A salivary gland fistula (plural "fistulae") is a fistula (i.e. an abnormal, epithelial-lined tract) involving a salivary gland or duct.
Salivary gland fistulae are almost always related to the parotid gland or duct, although the submandibular gland is rarely the origin.
The fistula can communicate with the mouth (usually causing no symptoms), the paranasal sinuses (giving rhinorrhea) or the facial skin (causing saliva to drain onto the skin).
The usual cause is trauma, however salivary fistula can occur as a complication of surgery, or if the duct becomes obstructed with a calculus.
Most parotid fistulae heal by themselves within a few weeks.
Hypospadias is a congenital disorder of the urethra where the urinary opening is not at the usual location on the head of the penis. It is the second-most common birth abnormality of the male reproductive system, affecting about one of every 250 males at birth. In roughly 90% of cases, the opening (meatus) is on or near the head of the penis (glans), referred to as distal hypospadias, while the remainder have proximal hypospadias with a meatus near or within the scrotum. Shiny tissue seen extending from the meatus to the tip of the glans, which would have made the urinary channel, is referred to as the urethral plate.
In most cases, the foreskin is also underdeveloped and does not wrap completely around the penis, leaving the underside of the glans penis uncovered. Also, a downward bending of the penis, commonly referred to as chordee, may occur. This is found in 10% of distal hypospadias and 50% of proximal hypospadias at the time of surgery. The scrotum may be higher than usual to either side of the penis, called penoscrotal transposition, adding to the abnormal overall appearance.
Hypospadias is thought to result from failure of the urinary channel to completely tubularize to the end of the penis; the cause is not known. Most often, it is the only abnormal finding, although in about 10% of cases, hypospadias may be part of a syndrome with multiple abnormalities.
The most common associated defect is an undescended testicle, which has been reported in around 3% of infants with distal hypospadias and 10% of those having proximal hypospadias. The combination of hypospadias and an undescended testicle sometimes indicates a disorder of sexual differentiation, and so additional testing may be recommended. Otherwise no blood tests or X-rays are routinely needed in newborns with hypospadias.
Hypospadias can be a symptom or indication of an intersex condition but the presence of hypospadias alone is not enough to classify as intersex. In most cases, hypospadias is not associated with any condition.
Fistulae between the trachea and esophagus in the newborn can be of diverse morphology and anatomical location; however, various pediatric surgical publications have attempted a classification system based on the below specified types.
Not all types include both esophageal agenesis and tracheoesophageal fistula, but the most common types do.
The letter codes are usually associated with the system used by Gross, while number codes are usually associated with Vogt.
An additional type, "blind upper segment only" has been described, but this type is not usually included in most classifications.
There are other forms of anorectal malformations though imperforate anus is most common. Other variants include anterior ectopic anus. This form is more commonly seen in females and presents with constipation.