Mitrofanoff formation and continuing care – in children

Introduction

This leaflet provides information on the Mitrofanoff procedure including the operation itself and advice and information about caring for your child’s Mitrofanoff. This leaflet is intended to supplement advice already given by your doctors and nurse specialists. Alternative treatments are outlined and can be discussed in more detail by your child’s care team.

Please keep this leaflet for reference later as it contains information about continuing care and problem-solving at home.

What is a Mitrofanoff?

The Mitrofanoff (pronounced ‘my-troff-an-off) procedure (also known as the ‘continent urinary diversion’) creates a channel from the skin on the abdominal (tummy) wall into the bladder. A catheter can be passed through this channel to drain urine out of the bladder. This procedure was first described by Paul Mitrofanoff in 1980 and is designed to help people who cannot empty their bladder completely via their urethra.

Some children will require the bladder to be made larger (‘augmented’) at the same time as the Mitrofanoff formation. A separate leaflet is available on bladder augmentation; please ask if bladder augmentation has been advised for your child and you have not been given a copy of the leaflet.

Why might my child require Mitrofanoff formation?

Mitrofanoff formation may be suggested if your child has incomplete bladder emptying secondary to conditions such as:

• Congenital birth malformations such as spina bifida, neuropathic bladder or bladder exstrophy.
• Spinal cord injuries and paraplegia.
• Acquired or secondary non-neuropathic neuropathic bladder (Hinman syndrome).
• History of bladder cancer.

Before admission to hospital

Investigations

Before admission to hospital your child will undergo a variety of investigations which include:

• A recent ultrasound scan of the kidneys, ureters and bladder.
• A nuclear medicine scan which provides information on the function of the kidneys.
• Blood tests to identify the kidney function and any signs of anaemia or abnormal clotting.
• X-rays to identify any evidence of reflux (urine passing back up towards the kidneys).
• Bladder function assessment (‘urodynamics’).

Referrals to community specialists and teaching

To ensure appropriate support is available to your child after surgery, referrals will be made before the operation to your child’s community nursing team who will provide home visits, support and nursing care after discharge.

In order for your child to be supported at nursery or school, it will also be necessary to ensure that appropriate teaching has been provided to key people before surgery is undertaken. Such teaching is arranged by the community and hospital teams.

Purchasing suitable painkillers

It is important that you purchase some children’s pain killers such as paracetamol (eg Calpol) and/or ibuprofen before admission to hospital so that you have these available at home after discharge.

If your child becomes unwell

If your child has a cold, cough or illness such as chicken pox the operation will

be postponed to avoid complications. Please telephone us. (The telephone number is at the end of this leaflet.)

Assessment before the operation

Your child will be assessed in one of our pre-op assessment clinics which include assessment by the anaesthetist, blood and urine tests and also testing (via swabs) for MRSA, which can cause delayed healing.

What happens when my child is admitted to hospital?

You will be usually be asked to bring your child to one of our children’s wards one to two days before the day of surgery for ‘bowel preparation.’ Children’s bowels need to be clear of faeces (poo) before surgery to help reduce risks such as infection. This is called ‘bowel preparation’ or ‘bowel prep.’ Every child that requires bowel prep will have an individualised care plan written for them and you will be given a copy of this on admission to hospital. Bowel prep is likely to involve the following:

• Children receiving bowel prep can eat and drink normally until they are admitted but after admission may only drink clear fluids (water and squash drinks).
• Oral laxative medication is given to help with the process of clearing the bowel of faeces.
• Some children (particularly those prone to constipation and those also having bladder augmentation surgery) will also require bowel washouts via the rectum.

Bowel washouts are carried out on the ward by a nurse and you can be present during this. Bowel washouts are not painful; the nurse will ensure that your child is kept warm and as comfortable as possible.

Children lie on their left side so you will be able to position yourself so your child can see you and have a story read to them, for example.

Bowel washouts involve passing a tube into the rectum (‘back passage’). A syringe is attached to the other end of the tube and a small amount of warm salty water is then poured into a syringe.

The water flows by gravity down the tube and into the child’s bowel. Once the water is in the bowel the syringe and tube are lowered to enable the water, now containing faeces, to run back out and be collected in a container. This process is repeated several times.

The number of washouts required will be stated in your child’s individual care plan, although it does also depend on the result of each washout (how much of the faeces is cleared). As the aim of the bowel preparation treatment is for the bowel to be completely clear of faeces before the operation, it is important that when the last washout is performed the fluid that runs back out of the stoma/rectum is clear.

Your child will have a cannula (‘drip’) inserted and more blood tests taken.

A parent is welcome to accompany their child when she/he goes to the anaesthetic room to go to sleep for the operation and also be present in the recovery area when she/he wakes.

What happens during the operation?

The operation is performed under general anaesthesia. The operation takes approximately three to four hours.

Commonly the appendix is used as the channel between the abdominal wall and the bladder. The surgeon will remove the appendix from its normal position on the large bowel (whilst maintaining its blood supply) and open it up to form a tube (channel). One end of the channel is connected to the bladder and the other end of the channel is connected to the surface of the skin, either within the belly button, or just below it, or to the right of the belly button.

The opening at skin level is called a ‘stoma’. A catheter will be passed into the stoma, down the Mitrofanoff channel, to drain the urine from the bladder. The surgeon will create a valve where the tube joins the bladder which squeezes shut when the bladder fills with urine and so helps to prevent urine from leaking between catheterisations.

If your child’s appendix is found to be too short, or if your child has already had their appendix removed, a piece of small bowel can be removed and used to create the channel described above.

At the end of the operation a catheter is left in place via the Mitrofanoff channel and, in addition, a second catheter is also left in place so that if one catheter blocks, another is available to drain the urine and keep the bladder empty while your child’s wound heals.

What happens after the operation?

You will be contacted when your child is waking in the recovery area so that you can be with your child again. After a period of monitoring in the recovery area your child will be taken back to the ward or, if they require closer monitoring due to any underlying condition, to our children’s high dependency unit.

Your child will have a number of tubes to drain urine and administer medication and fluids:

• Catheter via the Mitrofanoff – this will stay in place for approximately four to six weeks whilst the wound heals.
• A second catheter which is passed either through the urethra or directly into the bladder (called a ‘supra-pubic catheter’). The second catheter, which also stays in place for approximately four to six weeks, is usually clamped off and only opened to drain urine if the Mitrofanoff catheter blocks.
• Cannula (‘drip’) through which intravenous fluids can be administered until your child is drinking again (usually one to three days).
• Second cannula through which medication (pain killers and antibiotics) can be given. This cannula is removed once children are having fluids by mouth.
• Naso-gastric tube – some children might need a naso-gastric tube (a tube which passes into the stomach via the nose) to help drain the stomach and prevent vomiting while the bowel recovers. The tube is removed once children can manage fluids by mouth again.

The nursing team will measure your child’s urine output carefully and will need to know the volume of any drinks taken.

You will be taught how to look after your child’s catheters at home and also taught about problem solving and reasons to call for advice.

What are the benefits of having a Mitrofanoff?

• Allows the bladder to be drained when this is not possible via your child’s urethra.
• Discrete; unlike other types of urinary diversion, no bag needs to be on the skin to collect urine and, after the initial four to six weeks, no catheter is left inside the body.
• Allows children, particularly those with dexterity problems, to be independent with their toileting.

What are the complications/risks?

All operations have a potential for complications and risks; however, the majority of patients do not experience any. The potential risks and complications related to Mitrofanoff surgery and in the longer term care are as follows:

Complications at the time of surgery and whilst indwelling catheters are in place:

• Bleeding.
• Infection in the surgical wound.
• Infection in the urine.
• Leakage of urine around the Mitrofanoff catheter.
• The catheter placed at the time of surgery may fall out, possibly requiring a further operation to replace it or re-fashion the channel.
• If the blood supply to the bowel used to form the Mitrofanoff channel is impaired the tissue can die, requiring further surgery to re-fashion it.

Complications in the longer term:

• Tract stenosis: this means that the Mitrofanoff channel becomes narrow and so the intermittent catheter becomes difficult, or even impossible, to insert.
• Creation of a false passage: this means that a passage outside the channel is formed, usually as a result of tract stenosis or difficulty with catheterisation. If a false passage is created more surgery will be required and more time with an indwelling catheter in place will be needed.
• Adhesions: this is scar tissue formation which can occur after any abdominal operation. It is a small but lifelong risk which may result in an obstruction (‘blockage’) of the intestine. Symptoms of an adhesion obstruction include cramping abdominal pain and green (bile) vomit.
• Leakage of urine through the Mitrofanoff between catheterisations: this may occur due to a poorly functioning valve, a small bladder or a high pressure bladder.
• Complications secondary to mucous within the bladder which continues to be produced by the appendix/ bowel used to create the Mitrofanoff channel including urine infections and formation of bladder stones. (See the section on bladder washouts below).

What are the alternatives?

Catheterisation via the urethra is the usual alternative to the Mitrofanoff procedure and, due to its potential risks and complications, formation of Mitrofanoff is usually reserved for those children in whom urethral catheterisation is not possible.

Doing nothing is usually not an appropriate alternative for children who need to catheterise because, without the catheterisation, their bladders do not completely empty their urine and so they are at risk of complications such as urine infections and kidney problems.

An alternative to the Mitrofanoff urinary diversion is the ileal conduit diversion. An ileal conduit diversion is formed by detaching the ureters (the tubes that drain urine from the kidneys) from the bladder and joining them on to a short piece of the small bowel (ileum). One end of this piece of ileum is sealed off and the other is brought to the surface of the abdomen as a small spout (‘conduit’) called a urinary stoma. Urine continuously drains directly from your child’s kidneys, down the conduit and out into a stoma bag that sticks on your child’s abdomen (tummy) over the stoma.

Ileal conduit diversions also have complications/ risks which include sore skin around the stoma site, stomal hernias, incisional hernia (where parts of the stoma or parts of the intestine push through the muscle wall), prolapse of the stoma (in which the stoma extends outwards), urinary tract infections, stone formation and stenosis (narrowing) of the ureter which can cause obstruction.

Occasionally it is appropriate to offer the insertion of a supra-pubic catheter as an alternative to Mitrofanoff formation. A supra-pubic catheter is a catheter which is inserted directly into the bladder via the abdominal wall. A catheter is left in place continuously and as such, it increases the risk of urine infections and, in the long term, can lead to complications such as bladder stone formation.

How long will my child stay in hospital for and can I stay with him/her?

Your child’s hospital admission will be in two stages:

1. Admission to prepare your child for the operation, to have the operation and recover. This stage is usually seven to 10 days in length.
2. Stage two is scheduled for approximately four to six weeks after the operation itself. Your child will be re-admitted to hospital for a day so that the indwelling catheters can be removed and you and our child can be taught to undertake intermittent catheterisation via the Mitrofanoff. The date for this will be arranged with you by the nurse specialist before you go home following the surgery itself.

Discharge advice – How do I look after my child at home?

Daily cleaning of the Mitrofanoff site

Before your child is discharged you will be shown how to clean and, where necessary, change dressings at your child’s Mitrofanoff site. Some leakage is to be expected in the days and weeks following surgery and, because of this, and because your child will have a catheter in place, it is important to keep the area clean to prevent infection.

Emergency care box

Before your child is discharged you will be provided with an ‘emergency care box’ which will contain items to be used in the unlikely event of the catheter displacing. It is essential that this care box is kept with your child at all times.

Ensure your child drinks well

It is essential that your child drinks an appropriate volume for their age every day in order to prevent complications such as infection and the formation of bladder stones. As a rule, the urine passed during daytime hours should be clear. If yellow urine is seen this indicates your child is not drinking sufficiently. Sometimes mucous will be seen in the urine; drinking well helps to clear this mucous.

The volume of fluid that should be taken by different aged boys and girls:

• Girls and boys aged 4 to 8 years - 1000 to 1400ml per day
• Girls aged 9 to 13 years - 1200 to 2100ml per day
• Boys aged 9 to 13 years - 1400 to 2300ml per day
• Girls aged 14 to 18 years - 1400 to 2500ml per day
• Boys aged 14 to 18 years - 2100 to 3200ml per day

Problem solving after discharge

Problem solving whilst indwelling catheter is in place

The indwelling foley catheter via the Mitrofanoff has fallen out

Resolution:

• Collect equipment from your emergency care box (new catheter, lubricating jelly, new catheter valve).
• Apply lubricating jelly to the tip of the catheter.
• Pass the catheter down the Mitrofanoff stoma until half of the catheter length has been inserted.
• Attach the new catheter valve.
• If the same size of catheter cannot be inserted, use a catheter a size smaller.
• Call your nurse specialist and arrange to return to hospital for review (number is at the end of the leaflet).

The suprapubic foley catheter has fallen out (Mitrofanoff catheter remains in place)

Resolution:

• Collect your emergency care box.
• Press firmly over the exit site for five minutes using the sterile gauze.
• Place a clean folded gauze square (approx 2cm square) over the wound and cover this with the dressing provided.
• Connect a leg bag onto the Mitrofanoff catheter so it drains freely.
• Inform the clinical nurse specialist (number is at the end of the leaflet).

The urethral catheter has fallen out (Mitrofanoff catheter remains in place)

Resolution:

• Collect equipment from your emergency care box.
• Connect a free drainage urine bag onto the Mitrofanoff onto the bladder can drain freely.
• Inform the clinical nurse specialist (number is at the end of the leaflet).

There is no urine draining into the urine bag

Resolution:

• Use items from your emergency care box to place a free drainage bag onto your child’s spare catheter.
• Inform the clinical nurse specialist (number is at the end of the leaflet).

Catheter valve on the indwelling catheter is broken

Resolution:

• Collect equipment from your emergency care box.
• Person (1) pinches the affected indwelling catheter.
• Person (2) pulls off the old catheter valve and pushes a new one tightly in place.
• Check that the tap is in the off position before person (1) stops pinching the tube/a drainage bag is attached.

Discharge is observed from the Mitrofanoff stoma site

Resolution:

• It is normal to get clear/slightly coloured discharge from the Mitrofanoff stoma whilst a catheter is continually in place.
• It is important to clean the Mitrofanoff site daily.
• If the site has pus discharge antibiotics may be required; discuss with your nurse specialist.
• If the discharge has the appearance of urine, inform the paediatric surgery team.

Child has acute, short lived, episodic pain (bladder spasm)

Resolution:

• Children with indwelling catheters can experience bladder spasm.
• Oxybutynin is a medication used to treat bladder spasm; administer this medication as directed.

Child has a Mitrofanoff catheter and suprapubic catheter but is passing urine per urethra

Resolution:

• Check that urine is draining into the urine bag as your child may pass urine via their urethra when the catheter is blocked. If there is no urine follow the instructions above (row 4).
• It is not uncommon for children to leak small amounts of urine through the urethra, often secondary to bladder spasm.
• Oxybutynin may relieve this; administer as directed.

Problem solving when clean intermittent catheterisation of the Mitrofanoff is taking place

Unable to pass the intermittent catheter through the Mitrofanoff channel

Resolution:

Following surgery it is not uncommon for the stoma to shrink a little (stenosis) resulting in difficulty passing the catheter.

Inserting the catheter every three to four hours during the day should prevent this.

If stenosis does occur use a smaller size catheter then go back to the normal size the following day. If this fails continue using the size smaller catheter but contact the nurse specialist to discuss the use of a tapered tip catheter. Occasionally an indwelling catheter needs to be reinserted for one to two weeks or, very occasionally, widening the tract is necessary under general anaesthetic.

Discharge is observed from the Mitrofanoff stoma site

Resolution:

• Assess whether the discharge is urine/mucous/pus.
• If the discharge appears to be urine, check whether the catheterisation was overdue.
• If the leakage of urine recurs contact the paediatric surgery nurse specialist team.
• Mucous discharge is common. Ensure the site is regularly cleaned and, if necessary, cover the stoma with a small dressing.
• If pus discharge is observed and there are signs of infection (spreading redness, tenderness, warmth at site) contact your nurse specialist or GP as antibiotics may be required.

The Mitrofanoff site is red, inflamed or bleeding

Resolution:

Inserting an intermittent catheter can sometimes cause this. Contact your GP or the paediatric surgery team for advice.

Blood is seen on the intermittent catheter/in the urine

Resolution:

Blood can be seen intermittently on the catheter/in the urine, especially when the tract is newly formed due to friction caused from the catheter. This normally resolves by itself. If the problem persists, contact the paediatric surgery nurse specialist team.

Signs of a urinary tract infection are suspected

Resolution:

• Collect a mid‑stream specimen of urine during catheterisation.
• Contact your GP and paediatric surgery nurse specialist team as antibiotics may be required.

Your child continues to be wet in between catheterisations

Resolution:

It can take some weeks for a routine to be established which keeps the child dry. If the child has been previously dry and suddenly becomes wet again, this can be a sign of:

• infection (check mid stream urine specimen)
• poor bladder emptying (check technique)
• a change in bladder behaviour (liaise with the paediatric surgery nurse specialist team).

How is intermittent catheterisation performed via a Mitrofanoff?

Intermittent catheterisation is a procedure whereby an intermittent urinary catheter (a catheter manufactured for single use only) is inserted into the bladder to drain urine (using a clean rather than sterile technique) and then the catheter is immediately removed.

Intermittent catheters are available in a range of sizes (widths and lengths). It is important that your child uses the size advised by their care team and, also, that you have a supply of catheters one size smaller in case the Mitrofanoff channel narrows (see ‘How do I care for my child at home’ section above).

Procedure

Before you start, ensure you and your child are in an appropriate area to maintain your child’s dignity and privacy and promote ‘normal’ toileting behaviours. Ensure good lighting is also available and that you have all necessary equipment at hand.

• Collect equipment (catheter of appropriate size, wipes for cleaning, gloves – optional; suitable receptacle/toilet).
• Check all expiry dates on relevant equipment.
• Wash your hands and, if it is your preference, put on disposable gloves.
• Prepare catheter as taught by your nursing team.
• Position your child on the toilet.
• Wipe Mitrofanoff stoma.
• Gently push the catheter into the Mitrofanoff channel until urine starts to flow. You might feel some slight resistance as the catheter enters the bladder. Make sure the coloured funnel at the end of the catheter is pointing down into the toilet/collection device.
• If the catheter is not passed directly into the stoma, a new catheter must be used for the second attempt.
• When urine starts to flow push the catheter in a further 1-2 cm.
• When urine stops flowing withdraw the catheter slowly to remove the last few drops of urine.
• Dry the Mitrofanoff area with toilet tissue.
• Dispose of equipment as you have been taught.
• Wash your hands.

How often should intermittent catheterisation be performed?

The frequency of intermittent catheterisation varies from child to child but is usually every two to three hours during your child’s daytime hours and always first thing in the morning when your child wakes and always before bed. The frequency of catheterisations during daytime hours should never exceed four hours.

Some children benefit from wearing a watch with an alarm to remind them of the need to catheterise.

What is a bladder washout and how do I perform one?

Your child’s Mitrofanoff has been made using their appendix/ part of their bowel which will continue to produce mucous. The mucous can cause problems such as urinary tract infections, poor drainage of urine, blocked catheters and the formation of bladder stones. Bladder washouts are used to clear this mucous and so try to prevent these complications. Your child’s nursing team will teach you how to perform the bladder washout if required by your child.

Procedure for bladder washout

• Collect equipment (catheter of appropriate size, wipes for cleaning, gloves – optional; suitable receptacle/toilet, bladder washout fluid).
• Check all expiry dates on relevant equipment.
• Wash your hands and, if it is your preference, put on disposable gloves.
• Prepare catheter as taught by your nursing team.
• Position your child on the toilet.
• Wipe Mitrofanoff stoma.
• Gently push the catheter into the Mitrofanoff channel until urine starts to flow. You might feel slight resistance as the catheter enters the bladder. Make sure the coloured funnel at the end of the catheter is pointing down into the toilet/ collection device.
• If the catheter is not passed directly into the stoma, a new catheter must be used for the second attempt.
• When urine starts to flow push the catheter in a further 1-2 cm.
• When urine stops flowing attach the bag of bladder washout fluid to the funnel end of the intermittent catheter.
• Insert the washout fluid through the catheter by gently rolling the washout bag up.
• Disconnect the washout fluid bag.
• Attach a catheter tipped syringe to the intermittent catheter.
• Gently withdraw fluid back into the syringe; look for any mucous within the syringe.
• If there is a large amount of mucous you may be advised to repeat the above steps to improve clearance of mucous.
• Withdraw the catheter slowly and completely.
• Dry the Mitrofanoff area with toilet tissue.
• Dispose of equipment as you have been taught.
• Wash your hands.

Other frequently asked questions

Do we need to keep the Mitrofanoff site covered?

In the first weeks after surgery most children will have a dressing over the Mitrofanoff site but thereafter the site does not need to be covered.

Can my child bath?

For the first week after surgery your child should not bath or shower. After then, whilst your child has their indwelling catheter in, showers rather than baths are fine. Once the indwelling catheters have been removed your child can shower and bath as normal.

Can my child go swimming?

Swimming can recommence once the indwelling catheters have been removed.

Is it painful?

Before surgery your child’s anaesthetist will discuss initial pain management plans with you. After surgery your child’s nurses, surgical team and the children’s pain team will review your child’s pain management regularly to ensure your child is comfortable and able to mobilise again as soon as possible. It is usual for children to receive intravenous painkillers initially and until your child is eating and drinking again. At the time of discharge children usually only require simple pain killers such as paracetamol and ibuprofen.

Children and their families often worry that performing clean intermittent catheterisation will be painful. However, some children (for example those with spina bifida) may not have any sensation and, for those children who do have sensation, whilst they may feel the catheter as cold as it passes into the tract, catheterisation via the Mitrofanoff is not painful.

Are my child’s activities going to be restricted if they have a Mitrofanoff?

Your child’s activities will be restricted whilst they have an indwelling catheter during the initial four to six weeks after surgery in order to prevent the accidental dislodgement of the catheter(s) for example:

• no physical education (PE)
• no swimming
• no baths
• careful attention during physiotherapy and any lifting and handling eg in and out of wheelchairs

Once your child is having intermittent catheterisation there are no restrictions on activities unless your child has also undergone bladder augmentation (see leaflet on bladder augmentation).

Where will my supplies come from?

For the majority of patients continuing supplies of equipment such as catheters and bladder washout equipment can be arranged via a home delivery company. In some areas the GP practices prefer patients to collect equipment from a specific pharmacy rather than receive it on home delivery. Your nurse specialist will inform you what is available in your area.

It is essential that you always have sufficient volumes of equipment at home remembering to:

• order in advance of any family holidays so you have enough to take with you
• ensure your child has sufficient volumes of equipment at school
• order in advance of any public holidays when home deliveries will not be made

Who should be aware that my child has a Mitrofanoff?

It is important that all the doctors who take care of your child know that your child has a Mitrofanoff. You should also tell the school nurse. They need this information to look after your child.

We also advise that you consider use of a medic alert bracelet for your child. A medic alert bracelet lets health care workers know of your child's Mitrofanoff if there is an emergency. Your nurse or doctor can help you with the forms needed to get this bracelet.

Follow-up

Your child will be readmitted approximately four to six weeks after their initial surgery; the date for this will be arranged with you before discharge.

Once your child has commenced clean intermittent catheterisation they will be reviewed in the outpatient clinic approximately three months later. You will receive a letter in the post giving details of the appointment. Please ensure you telephone the clinic if you are unable to attend.

Chaperoning

During your child’s hospital visits your child will need to be examined to help diagnose and to plan care. Examination, which may take place before, during and after treatment, is performed by trained members of staff and will always be explained to you beforehand. A chaperone is a separate member of staff who is present during the examination. The role of the chaperone is to provide practical assistance with the examination and to provide support to the child, family member/carer and to the person carrying out the examination.

Are there on line support groups available?

Please visit Mitrofanoff support for children (opens in a new tab).

Who shall I contact if I have any queries, concerns or questions?

For further information/queries please contact:

• Your nurse specialist (Mon to Fri 08:00 to 18:00)
  Telephone: 01223 586973 (office) or, for urgent problems: 01223 245151 (switchboard and ask staff to contact the nurse specialist via their team mobile phone.
• The ward your child was on
• Your community children’s nurse
• Medic alert foundation (opens in a new tab)
  Telephone: 0207 8333034