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The Mitrofanoff procedure, also known as the Mitrofanoff appendicovesicostomy, is a
surgical procedure Surgery ''cheirourgikē'' (composed of χείρ, "hand", and ἔργον, "work"), via la, chirurgiae, meaning "hand work". is a medical specialty that uses operative manual and instrumental techniques on a person to investigate or treat a pat ...
in which the appendix is used to create a conduit, or channel, between the skin surface and the
urinary bladder The urinary bladder, or simply bladder, is a hollow organ in humans and other vertebrates that stores urine from the kidneys before disposal by urination. In humans the bladder is a distensible organ that sits on the pelvic floor. Urine enters ...
. The small opening on the skin surface, or the
stoma In botany, a stoma (from Greek ''στόμα'', "mouth", plural "stomata"), also called a stomate (plural "stomates"), is a pore found in the epidermis of leaves, stems, and other organs, that controls the rate of gas exchange. The pore is bor ...
, is typically located either in the
navel The navel (clinically known as the umbilicus, commonly known as the belly button or tummy button) is a protruding, flat, or hollowed area on the abdomen at the attachment site of the umbilical cord. All placental mammals have a navel, although ...
or nearby the navel on the right lower side of the abdomen. Originally developed by Professor Paul Mitrofanoff in 1980, the procedure represents an alternative to urethral catheterization and is sometimes used by people with urethral damage or by those with severe autonomic dysreflexia. An intermittent catheter, or a catheter that is inserted and then removed after use, is typically passed through the channel every 3–4 hours and the urine is drained into a toilet or a bottle. As the bladder fills, rising pressure compresses the channel against the bladder wall, creating a one-way valve that prevents leakage of urine between catheterizations.


Description of procedure

A surgeon creates a small channel using the appendix or in the absence of the appendix, a piece of small bowel. When bowel is used instead of appendix, it is called a Monti procedure. One end of the channel is sewn to the skin, creating an opening on the surface called a stoma. The other end of the channel is sewn to the bladder and a flap valve of tissue is created to prevent leakage from the stoma between catheterizations. Sometimes, the bladder is enlarged with bowel tissue to enable greater urine storage in an additional procedure called
bladder augmentation Bladder augmentation is a surgical alteration of the urinary bladder. It involves removing strips of tissue from the intestinal tract and adding this to the tissue of the bladder. This has two intended results: increased bladder volume; and ...
. The Mitrofanoff procedure is different from an indwelling catheter placement because the catheter is removed from the channel between urine drainage events. Some people with Mitrofanoff channels can also void urethrally, while others catheterize exclusively.


Relation to MACE

The ''
Malone antegrade continence enema A Malone antegrade continence enema is a surgical procedure used to create a continent pathway proximal to the anus that facilitates fecal evacuation using enemas. Description The operation involves connecting the appendix to the abdominal wall ...
'' (MACE), used to treat
fecal incontinence Fecal incontinence (FI), or in some forms encopresis, is a lack of control over defecation, leading to involuntary loss of bowel contents, both liquid stool elements and mucus, or solid feces. When this loss includes flatus (gas), it is referred ...
, is like the Mitrofanoff procedure as it uses the ''
Mitrofanoff principle In urology, the Mitrofanoff principle is the creation of a passageway for urine or enema fluid that, by its (surgical) construction, has a valve mechanism to allow continence. Procedures which make use of the Mitrofanoff principle: *Mitrofanoff pr ...
'' and, thus, can be considered an analogous procedure. As fecal and urinary incontinence frequently co-exist, a MACE is often created at the same time as a continent catheterizable urinary conduit.


Relation to Monti procedure

If the appendix is not available, due to
appendectomy An appendectomy, also termed appendicectomy, is a Surgery, surgical operation in which the vermiform appendix (a portion of the intestine) is removed. Appendectomy is normally performed as an urgent or emergency procedure to treat complicated acu ...
, or unusable for another reason, the Monti procedure is done.


Relation to bladder augmentation and neobladder construction

If the bladder is not sufficiently large, some people may need a bladder augmentation at the same time as a Mitrofanoff. Augmentation enlarges the bladder, making it possible to hold more urine and prevent backflow into the kidneys. This is usually done with one's own bowel tissue and typically bowel tissue produces mucus. Hence, regular washouts are usually required. Because bowel tissue aids in absorption, its use for an augmentation may result in metabolic imbalance and result in the need to monitor
vitamin B12 Vitamin B12, also known as cobalamin, is a water-soluble vitamin involved in metabolism. It is one of eight B vitamins. It is required by animals, which use it as a cofactor in DNA synthesis, in both fatty acid and amino acid metabolism. It ...
,
bicarbonate In inorganic chemistry, bicarbonate (IUPAC-recommended nomenclature: hydrogencarbonate) is an intermediate form in the deprotonation of carbonic acid. It is a polyatomic anion with the chemical formula . Bicarbonate serves a crucial biochemic ...
, and
chloride The chloride ion is the anion (negatively charged ion) Cl−. It is formed when the element chlorine (a halogen) gains an electron or when a compound such as hydrogen chloride is dissolved in water or other polar solvents. Chloride salts ...
. If bowel tissue is used to create an entirely new bladder, the resulting reservoir to hold urine is called a neobladder. Neobladders are usually created in instances of bladder removal.


History

The concept of
clean intermittent catheterization Intermittent catheterization is a medical technique used in conditions where patients need either short-term catheter-based management of the urinary bladder or as a daily habit for life. Intermittent catheterization is considered the "gold standa ...
via the urethra was widely introduced by Jack Lapides when he published a seminal paper on the subject in 1972. Clean intermittent catheterization provides an alternative to the sterile technique and allows individuals to self-catheterize after washing their hands, without the need for medical professionals and sterile equipment. In 1980, Professor Paul Mitrofanoff described a "trans-appendicular continent cystostomy," the technique that would later be named for him. Mitrofanoff's concept revolutionized clean intermittent catheterization because it allows urine to be drained via a route other than the urethra. However, the Mitrofanoff procedure was slow to be adopted until a pediatric resident named Marc Cendron translated Mitrofanoff's French language paper for the well-known pediatric urologist Dr. John Duckett Jr. in Philadelphia. The Mitrofanoff procedure is sometimes performed along with bladder neck closure, but Duckett advised against the closure of the bladder neck. Today, the Mitrofanoff procedure can be performed robotically or using laparoscopic techniques and it paved the way for the creation of other urinary conduits using Fallopian tubes, ureters, and segments of bowel, as in the Monti procedure.


Indications

The Mitrofanoff procedure is typically performed as an alternative for people who experience painful urethral catheterization and has been particularly useful for females. It is also used in people with
neurogenic bladder dysfunction Neurogenic bladder dysfunction, or neurogenic bladder, refers to urinary bladder problems due to disease or injury of the central nervous system or peripheral nerves involved in the control of urination. There are multiple types of neurogenic bladde ...
, urethral trauma, and spinal cord injuries. The procedure is sometimes recommended for those with spinal cord injuries who have severe autonomic dysreflexia. Wheelchair users who cannot use a toilet independently or who struggle to catheterize independently may get a Mitrofanoff to gain greater control over their care. For people who would otherwise leak via the urethra, the Mitrofanoff channel can provide continence and enable them to stop using diapers. Other conditions for which the procedure may be appropriate include urethral cancer, congenital absence of a urethra, Prune Belly syndrome, sacral agenesis, and traumatic loss of urethra from a gunshot. Appropriate candidates are prepared to commit to a lifetime of followup care.


Contraindications

People who have high pressure bladders, meaning their bladder pressure increases significantly with small increments of fluid, are not good candidates for the procedure due to the risk of damage to the kidneys. Also contraindicated are those who cannot hold large volumes of urine without refluxing into the kidneys. The procedure is not recommended for people with poor hand function, especially those without access to reliable assistance.


Presurgical testing

People who have been determined to be candidates for a Mitrofanoff surgery will need to undergo a variety of presurgical tests. Testing often includes blood tests such as a
complete blood count A complete blood count (CBC), also known as a full blood count (FBC), is a set of medical laboratory tests that provide cytometry, information about the cells in a person's blood. The CBC indicates the counts of white blood cells, red blood cell ...
, a
basic metabolic panel A basic metabolic panel (BMP) is a blood test consisting of a set of seven or eight biochemical tests and is one of the most common lab tests ordered by health care providers. Outside the United States, blood tests made up of the majority of the ...
, and an assessment of
cystatin c Cystatin C or cystatin 3 (formerly gamma trace, post-gamma-globulin, or neuroendocrine basic polypeptide), a protein encoded by the CST3 gene, is mainly used as a biomarker of kidney function. Recently, it has been studied for its role in predictin ...
. Additionally,
urodynamic testing Urodynamic testing or urodynamics is a study that assesses how the bladder and urethra are performing their job of storing and releasing urine. Urodynamic tests can help explain symptoms such as: * incontinence * frequent urination * sudden, stro ...
and a kidney bladder ultrasound are typically performed to assess characteristics of the urinary tract prior to surgery. The urodynamics test should be done within 2 years prior to the procedure and the ultrasound within 1 year. Prior to surgery, the bowels are typically cleared with a routine called bowel prep. Bowel prep can be performed at home the 1–2 days before surgery or in some instances, occurs in a hospital before the operation. Bowel prep may require magnesium citrate drink to empty the colon. Bowel prep is done to reduce infection risk.


Recovery from surgery and life with a Mitrofanoff


Hospitalization and learning to use the channel

The Mitrofanoff procedure is a major surgery and typically requires inpatient hospitalization for 5–7 days. Initially, eating and drinking by mouth is not permitted and fluids are delivered intravenously for a few days. Progression to a regular diet can be accomplished, starting with the consumption of clear fluids. After the surgery, a tube is placed in the newly created channel to keep it open while it heals. A tube is typically also placed in the urethra or through a
suprapubic The hypogastrium (also called the hypogastric region or suprapubic region) is a region of the abdomen located below the umbilical region. Etymology The roots of the word ''hypogastrium'' mean "below the stomach"; the roots of ''suprapubic'' mean ...
opening to ensure full urine drainage and to rest the bladder during recovery. The tubes are generally removed and the channel is ready to use with intermittent catheters in 4–6 weeks, provided that a medical professional first instructs on how to catheterize. Depending on one's neurological status, a person with a Mitrofanoff may or may not feel the sensation to urinate. Full recovery time varies from person to person and ranges from 3–12 months.


Types of catheters

There are three major types of intermittent catheters used with a Mitrofanoff. Intermittent catheters are used to drain the bladder at regular intervals. The three types are: * Non-coated: can be used with a lubricant * Hydrophilic: need to be activated with water by following the product instructions * Pre-lubricated: these come already soaked in lubricant


Dietary considerations

People with Mitrofanoff channels should eat a balanced diet that is high in fiber, including a recommended 5 servings of fruits and vegetables daily. Drinking plenty of fluids is recommended to flush out the kidneys and bladder and to lessen the chance of
urinary tract infection A urinary tract infection (UTI) is an infection that affects part of the urinary tract. When it affects the lower urinary tract it is known as a bladder infection (cystitis) and when it affects the upper urinary tract it is known as a kidney ...
. Recommended fluid intake is 2-3 liters of water a day, resulting in the production of healthy urine that is light in color. Most people with Mitrofanoff channels can drink alcohol in moderation provided that they follow a regular catheterization schedule to avoid overfilling the bladder.


Sex and pregnancy

Sexual activity is typically avoided for 6 weeks postoperatively. It is possible to have a healthy pregnancy after Mitrofanoff surgery but the pregnancy should be monitored closely by a urologist. A C- section may be considered. In individuals with a conduit made from bowel tissue, a standard pregnancy test will not be accurate in most instances and pregnancy can instead be confirmed by a blood test. Male fertility is typically unaffected.


Exercise and physical activity

For 2–3 weeks after surgery, extra care should be taken to prevent trauma to the surgical area. For this time period, physical education and activities such as bicycle riding are not advisable. It is possible to play sports with a Mitrofanoff, but high contact sports such as rugby are typically not advised. Light exercise following surgery can facilitate recovery; though specific exercise recommendations generally require consultation with a medical professional.


Bladder washouts

Bladder washouts are performed to prevent build up of mucus and debris that can lead to urinary tract infection and increase the chance of a
bladder stone A bladder stone is a stone found in the urinary bladder. Signs and symptoms Bladder stones are small mineral deposits that can form in the bladder. In most cases bladder stones develop when the urine becomes very concentrated or when one is d ...
. Bladder stones can stop the bladder from emptying completely during catheterization and cause infection. Those with an augmented bladder are more likely than those with a native bladder to require washouts. A washout, also called an irrigation, is performed by pushing saline or sterile water into the channel using a syringe connected to a catheter. The water is pulled back out when the syringe is withdrawn and the process is repeated until the mucus is cleared. Certain foods and drinks such as dairy products and soft drinks (soda) can increase mucus production. The frequency of bladder washouts is dictated by medical advice.


Annual follow up testing

People with Mitrofanoff channels can expect a lifetime of annual testing to evaluate their urological health. These tests may include: * a scan of the bladder, kidneys, and ureters * a
cystoscopy Cystoscopy is endoscopy of the urinary bladder via the urethra. It is carried out with a cystoscope. The urethra is the tube that carries urine from the bladder to the outside of the body. The cystoscope has lenses like a telescope or microscope ...
, an examination of the bladder using a camera * blood tests to check the kidney and liver


Risks and complications

Every surgery has some risks. Some possible complications are: * Bladder stones: Bladder stones are hardened masses of minerals usually form when the bladder has not emptied properly or mucus has built up. Left untreated, bladder stones can cause infection. * Stenosis: Stenosis of the channel occurs when it becomes narrower, making it difficult to pass a catheter. Additional surgery may be required to ensure the safe insertion of a catheter. If the bladder cannot be emptied via the urethra and the catheter cannot enter the channel, it is a medical emergency. * Leaking: Leakage of urine may occur from the stoma, particularly if the channel becomes stretched and the valve preventing such leaks fails to self- seal. Bladder spasms, painful contractions of the bladder, can cause leakage from the stoma or the urethra and may need to be treated with medication called an antispasmodic. * Urinary tract infections: Urinary tract infections can be a concern in people who use catheters due to incomplete emptying or catheter contamination from the hands. They can be prevented with proper fluid intake and careful hand hygiene.


References

{{reflist Urologic surgery