Gynaecological care for people with a background of cloacal anomaly

A cloacal anomaly describes when a child is born with a single opening on their perineum rather than separate openings for their urethra (the tube that carries urine out of the bladder), vagina, and rectum (back passage, where poo comes from). 

Inside the body, this single opening connects to a common channel that then divides into the urethra, vagina, and rectum.  
 
There can be differences in the length of the common channel and its position (closer to the front, or back).

Many people with a background of a cloacal anomaly will also have a difference in how their uterus (womb) and vagina has formed. Some people will also have differences in how the clitoris is formed. 

Cloacal anomalies affect one in 50,000 people. It’s not understood why some people are affected and others aren’t.

It’s common for someone with a background of cloacal anomaly to also be affected by differences with how other body parts formed before birth. Some people have a cloacal anomaly as part of a “VACTERL Association”.  

VACTERL Association is where someone has three or more differences with how these body parts formed before birth: 

V-Vertebral (Spine)
A-Anorectal (Anus – where poo comes out and Rectum- lower section of bowel)
C-Cardiac (Heart)
Te-Tracheo-oesophageal (Trachea – windpipe, Oesophagus – food pipe)
R-Renal (Kidneys and bladder)
L-Limbs

In order to protect the kidney from damage, and to allow a child to develop continence (the ability to control urine and bowel movements), surgery will usually be performed during childhood. 

Typically, babies will have an operation in the first few days of life to form a stoma. This diverts poo away from the common channel, in order to allow drainage of urine and protect the kidneys. Surgery may also be required to drain the upper vagina if this has become blocked to allow the kidneys to drain urine.

Surgery to reconstruct a separate urethra and anal opening is typically performed when a child is around 1–2 years of age. The exact timing and type of surgery will depend on each individual’s anatomy and overall health.

Around half (40–50%) are able to pass urine out of their urethra. This typically includes those with a shorter common channel and a well-developed urinary tract.

About a third (30–40%) will use a catheter (a special soft tube which is inserted into the urethra and up to the bladder). This is called “clean intermittent self-catheterisation” (CISC).

Less than a third (10–20%) will have a continent catheterisable channel, most commonly a Mitrofanoff. This is a channel created during surgery, often using the appendix, that connects the bladder to the surface of the abdomen. This allows the bladder to be emptied by inserting the catheter through the opening on the tummy into the bladder.

Around one in five (15–25%) are able to pass stool (poo) through the anus. This means they can stay clean and dry without needing regular bowel medications or interventions. This is more likely in those with shorter common channels and well-formed muscles (sphincters) around the anus. 

Around a third (30–40%) use regular oral laxatives – such as Movicol, Senna or Lactulose – to help empty their bowels and avoid constipation or accidents. 

About a quarter (20–30%) use a method called trans-anal irrigation (TAI). This involves gently flushing fluid into the rectum (bottom) through the anus using a special system (like Peristeen or Navina). This helps empty the bowel in a more controlled and predictable way. 

Around 10–15% use an ACE (antegrade continence enema), sometimes called a Malone or ACE stoma. This is a channel created during surgery, often made with the appendix, that allows fluid to be introduced from the opening on the tummy into the bowel to flush it out from above. It can help with independence and give better control for some people.

Around 1 in 5 to 10 people (10–20%) have a stoma, where the bowel opens onto the surface of the tummy and stool is collected in a special bag. This may be temporary – especially when used early in life – or sometimes permanent if managing bowel function in other ways is difficult.

Puberty is a change in the body and mind that is driven by hormones and that helps us develop from children into adults.

The development of ovaries is not usually affected in cloacal anomalies. Sometimes the ovaries may be situated in less typical locations inside the body (often higher than usual), but they produce hormones normally.

This means that puberty is expected at a typical age with changes starting from 8–12 years of age.

Not everyone with a background of cloacal anomaly will have periods: 

• A womb might not have formed before you were born 
• Period blood might be blocked and not be able to pass out of your body. This might be the case if there’s a difference in how the womb and vagina developed, or scarring from previous surgery. 

Whether or not someone has a functioning uterus (and the shape of the uterus) is difficult to assess until after puberty. This is because the uterus increases in size with puberty and the rising levels of oestrogen. An ultrasound scan or MRI scan will be arranged to check whether the uterus developed before birth. 

Generally, an examination under anaesthesia with vaginoscopy (long fine telescope inserted into the vagina) is arranged in puberty to check for any vaginal narrowing that could affect the passage of period blood.   
 
Periods tend to start one to two years after the start of puberty.  It’s normal for periods to be irregular for the first couple of years of starting whilst hormones settle into a rhythm. 

Having a double uterus (didelphys) can be associated with heavier periods, but it’s also common to have heavy periods with a typically shaped uterus.   

Tampon use can be an issue if someone has vaginal narrowing or a vaginal septum dividing vagina into left and right sides. Surgery can often be performed to help open up the vagina to allow tampon use. 

If someone has a unicornuate uterus, or if their uterus or upper vagina did not fully develop before birth, then there may be increasing pain with obstructed period blood. This needs to be treated at first with painkillers and hormone medications to temporarily stop further periods.

The surgery suggested to treat an obstruction will depend on the type of obstruction. This surgery could involve a vaginal procedure, laparoscopic (keyhole) procedure, or a larger cut on the tummy (laparotomy).  

If you are having issues with painful periods with no concerns of obstructed blood on your scan, then the team will review and consider whether or not you could have a common condition called endometriosis. Painful periods and endometriosis are treated with anti-inflammatory painkillers. Please be aware that some people with kidney problems are recommended to avoid these medications: please check with your team if this affects you. 

Painful periods may also be treated with hormone pills, contraceptive pills, patches, injections, or a hormonal intrauterine device (coil). Hormonal intrauterine devices may not be suitable for some people with a background of a cloacal anomaly due to the differences in their vaginal and/or uterus.

In some people affected by cloacal anomaly, a section of bowel may have been used to form a neovagina. This may, or may not, be connected to a uterus.

If you have a vagina formed from bowel, you may experience excessive mucus discharge from the vagina and need to routinely wear a pad. The bowel segment can also develop inflammation, and this may be associated with an increased cancer risk. Some people with a vagina formed from bowel need to perform vaginal washouts and a smaller number require procedures in which a miniature camera is inserted into the vagina (a vaginoscopy) to allow more extensive vaginal washouts.

Sex may be affected because of differences in the shape or size of your vagina, scarring from surgeries, or differences in your clitoris. These physical differences might make certain activities, like vaginal sex, feel different, or you might not be able to have this type of sex.

Vaginal dilators may be recommended to help open up any narrowing from scarring. Sometimes surgery may be an option to help open up scarring or, if needed, to create a new vagina that allows an option for vaginal sex.

It’s really important to remember that sex isn’t just about having vaginal sex. Sex is much more than that. It’s about how you feel physically and emotionally. Feeling confident and comfortable in your body, enjoying intimacy, and sharing a connection with someone else are all part of sex. How you feel about your body and your control over your bladder and bowels can also influence how you experience sex and intimacy.

Everyone deserves to feel safe, confident, and comfortable exploring their own body when they’re ready. If you have any questions or worries about sex or your sexual health, the team is here to support you. This includes our psychology team supporting you with thinking about how you feel about your body and your background of a cloacal anomaly, and how you feel about starting an intimate relationship with someone in the future.

Protecting yourself

Barrier contraception (e.g. condoms) are recommended to reduce the risk of sexually transmitted infections. 

Condoms can also help reduce the risk of an unplanned pregnancy, but more reliable contraception is recommended along with condoms.  

Most contraceptive options can be used, but intrauterine devices (coils) won’t be suitable for some people with a background of a cloacal.   

You can find more information about contraception options on the Contraception Choices website.  

Using lubricants

Our team will speak to you about the use of lubricants to help make sex more comfortable. There are different types of lubricants and you can ask the team for more advice on this.  

Generally, water-based lubricants are suitable for most people, but they don’t last as long and you might need to reapply them during sex. Some water-based lubricants can be drying and this can be irritating. The gynaecology team can help recommend water-based lubricants that are less likely to cause this.

Oil based lubricants last longer but can have a 'thicker' feel. They cannot be used with condoms.

Silicone lubricants last longer and shouldn't cause dryness. Some people can be irritated by silicone so if you haven't used before you should do a small patch test (not on your vulva). Silicone lubricants can cause break down of silicone toys.

Fertility can be affected, this can be because of differences in the uterus, the function of the Fallopian tubes or ovaries, or because of general health issues (eg. chronic kidney disease).  

Most people with a background of a cloacal anomaly have normally functioning ovaries. Ovaries can be in slightly different locations (often higher or more towards the front of the body). This can affect how fertility doctors access the ovaries if planning egg collection for fertility treatment.    

Previous scarring from surgeries, or when someone has had obstructed periods, can affect ovarian function and reduce the number of eggs someone’s ovaries have.  

Ovaries have a set number of eggs which slowly reduces over time – especially after the age of 35.  

Your doctor will check your hormone levels to get a sense of your ovarian function and reserve. This will help them to advise on potential fertility preservation options for you.   

The Fallopian tubes can also be affected by scarring from surgery or obstructed periods in the past. This can lead to the tubes being blocked or damaged which can make the chance of falling pregnant without fertility treatment lower. It can also increase the risk of ectopic pregnancy (where a pregnancy doesn’t implant inside of the uterus – most commonly in the tube).

Your doctor will explain how your background of a cloacal anomaly affects your fertility and what your routes to parenthood might be.  This might include adoption or surrogacy (when someone else carries an IVF pregnancy, which could be with an egg from your ovaries).

If you have had a bladder reconstructed using a section of bowel, then you need to be aware that this can affect the result of urinary pregnancy tests. This is because the proteins in the mucus from the bowel segment in the bladder can confuse the pregnancy test and this can lead to a pregnancy test being positive, even when you are not pregnant. You would need to have the result confirmed by a blood test.

Your doctor will let you know if this is something that affects you and will give you a letter to show to health care professionals outside of our team if you have a positive urine pregnancy test result.

Pregnancy is more complex and therefore described as a high-risk pregnancy, so it’s best to plan ahead. We recommend using reliable contraception to avoid an unplanned pregnancy. When you are thinking about starting to try for a pregnancy, you should contact your gynaecology and urology teams for advice. We will arrange a pre-conception review with our obstetric team at UCLH, where they will review your kidney function, plan with you how to optimise your health for pregnancy and give you an overview of how your pregnancy care will be. 

There are several reasons why pregnancy in someone with a background of cloacal anomaly might be more complicated for example: 

• A higher risk of urinary tract infection 
• A higher risk of bladder stones (if someone has had a bladder reconstruction) 
• More strain on your kidneys with reduced kidney function 
• A higher chance of blood pressure changes and a condition called pre-eclampsia (a condition of pregnancy linked to the placenta which can affect blood pressure, liver, and kidney function. Pre-eclampsia affects around 5% of all pregnancies) 
• A higher risk of a late miscarriage (the loss of a baby before 24 weeks of pregnancy), or preterm labour (when a baby is born early and needs extra support), or growth restriction (where the baby’s growth slows or stops during pregnancy). These risks are more likely when someone has a different shaped uterus 
• If someone has a stoma they might notice a change in their stoma output with pregnancy, and the appearance of their stoma might change. Generally, it’s rare to have a significant issue with a stoma with pregnancy.    

Because of the surgery to reconstruct the bowel, bladder, and vagina for someone with a background of cloacal anomaly, we would usually recommend a planned Caesarean section for delivery. This would take place at UCLH, where our team is best equipped to manage any complications that could arise. In very rare emergency situations – such as extremely early labour a vaginal delivery might be considered, but this would be carefully weighed against the potential risks. Pregnancy care would usually be shared between your local maternity service and the specialist team at UCLH.

Cervical screening, which used to be called a smear test, is a test used to check the health of the cervix and help prevent cervical cancer. In the UK invitations are sent out for those between the ages of 24.5 and 65 years of age. Cervical screening checks a sample of cells from your cervix for certain types of human papillomavirus (HPV). These types of HPV can cause abnormal changes to the cells in your cervix and are called "high risk" types of HPV. If high risk types of HPV are found during screening, the sample of cells is also checked for abnormal cell changes. If abnormal cells are found, they can be treated so they do not get a chance to turn into cervical cancer.

You can get HPV from any kind of skin-to-skin contact of the genital area. If you've never had any kind of sexual contact with anyone, you may decide not to go for cervical screening when you're invited, but you can still have a test if you want to.
  
If you do not have a cervix then your gynaecology doctor will explain this to you and write to your GP requesting that they remove you from automatic invitation for cervical screening.

Your gynaecology team will let you know whether you’d be able to have a smear with your GP or whether you would benefit from having your smear with the ‘colposcopy’ team. The colposcopy team has access to specialist equipment e.g. comfortable couches with leg supports, and different sized speculums (instruments to place in the vagina so the colposcopy doctor can get a view of your cervix). This equipment may make it a lot easier and more comfortable for you to have a smear taken. In some circumstances, the team can also arrange for the smear to be taken under sedation or general anaesthetic.

If you missed getting the HPV vaccination when you were 12 or 13 years old, the HPV vaccine is available for free on the NHS for people under 25 years old. You can ask your GP to check your vaccination record and arrange for you to have the vaccine if you have not already had it.

Our psychology team can help you explore how your experience of having a cloacal anomaly background may be affecting you. We recognise that it’s a complex condition and it can affect people in different ways. 

The psychology team can support you with:  

• Understanding how your background of cloacal anomaly may have affected you in the past and may be affecting you now 
• Exploring your surgical options and supporting you in making an informed decision about your treatment 
• Sharing information about your condition with other people 
• Enjoying intimacy 
• Exploring your thoughts, feelings, and options to become a parent. 

The psychology service offers targeted, short term individual therapy as well as an online support group. Appointments can be scheduled online or in person. 

If you want to link in with our psychology team ahead of your next clinic appointment, you can contact our team via email (uclh.pag.queries@nhs.net).

We hope that this has been helpful to read. It may have raised some questions and patients to our service can contact our team with these.

Email: uclh.pag.queries@nhs.net