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Condition: Cloacal Anomalies (cloaca, high imperforate anus)
Overview ("What is it?")
- Definition: A cloacal anomaly is a result of abnormal development of the urinary, vagina and anal openings. Instead of three separate openings, there is only one hole where urine and stool comes out. This one opening connects to the urethra/bladder (urine), vagina/uterus and rectum (stool). This problem occurs only in girls. It is the most complex malformation of this area.
- Epidemiology: Occurs in 1 in 20,000 live births (rare).
Figure 1: http://www.cloaca.eu/wp-content/uploads/2014/05/10917445_10153524971574517_7007155572264479258_n.jpg
Signs and Symptoms ("What symptoms will my child have?")
- Early signs
- Abnormal appearance of the area where the vagina, urinary opening (urethra) and opening for stool (anus) looks abnormal. There is only one hole instead of three separate holes for these body functions
- Urine and stool coming from same one hole
- May have distended belly
Diagnosis ("What tests will be done to determine what my child has?")
- Labs and tests: Babies with cloaca have higher risk of certain types of other abnormalities. It is important to find out if the baby has these abnormalities so nothing is missed that can impact the baby’s health moving forward. The most common abnormalities are grouped into the “VACTERL” association (named after the first letter of the most common anomalies).
- Vertebral (spine): Spine X-ray to look for abnormalities of the bones and ribs. Spine ultrasound or MRI may be obtained.
- Anus: Baby has high imperforate anus/cloaca.
- Cardiac (heart): An ultrasound of the heart (echocardiogram) is needed to check for problems such as abnormal holes, problems with valves, etc.
- Tracheo-Esophageal fistula (TEF): Abnormal connection between airway (trachea) and esophagus (tube that connects mouth to stomach) and a blind-ending esophagus in neck.
- Renal (kidney): Ultrasound of kidneys to look for abnormalities
- Limbs (arms and legs): Examine arms and legs for deformity. Arms most common place for abnormal bones.
- Usually need ultrasound or MRI pelvis to look at ovaries, uterus
Treatment ("What will be done to help my child?")
- No medicine can fix cloaca, only surgery.
- Surgery: The ultimate goal is to separate the urinary tract, stool opening and vagina. These surgeries are done in stages, as they are so complicated that the best results are achieved when the baby is bigger.
- First Surgery
- It is important to define the anatomy, as cloacal anomalies are not all the same. This is done by placing a scope in the opening and following where the channels lead—the urinary tract (bladder), the vagina and uterus, and the intestinal tract (rectum)
- Separate urine flow from stool output, since mixing of stool and urine can lead to infections of the bladder and can damage the kidneys.
- If the drainage of the bladder is not adequate, a tube may be placed through the belly to drain the bladder (vesicostomy)
- Often, adequate passage of stool is accomplished by creating a colostomy. A colostomy is when the large intestine is brought out through the belly and stool comes out of the bag. A mucus fistula is another hole where the lower portion of the intestine can drain out.
- Sometimes, the vaginal opening is so narrow that the womb (uterus) is filled with fluid, making the belly big. If so, the womb also needs to be drained of fluid to allow it to normalize in size. If the drainage of the uterus is not adequate, a tube may be placed through the belly to decompress the womb (vaginostomy)
- Postoperative care
- Once stool and gas come out of the stoma, the baby is fed
- Teach parents how to take care of stomas
- Anesthesia: Baby will have to be completely asleep with anesthesia and breathing tube for surgery
- Infection: The baby will receive medicine to prevent antibiotics (medicine to fight infection)
- Possible dislodgment of tubes if these were placed
- Doctor has better idea of anatomy
- The urine flow is separated from the stool, decreasing chances of urinary infection
- Drainage of all three systems is ensured
- Second Surgery
- Repair of the urinary, vaginal and rectal anomalies
- A separate hole is planned for each system
- Depending on how complicated the abnormalities are, the repair may be done from the bottom only or a combination of bottom and belly approaches.
- Preoperative preparation
- The timing is dependent on many factors: baby must growing and gaining weight, other abnormalities may need to be addressed first (heart, spine, etc)
- Other studies may need to be done prior to the surgery. Your surgeon will decide on this.
- Antibiotics will be given
- A scope will be inserted look at the channels to the bladder, vagina and anus to make sure that there are no changes.
- Create three separate openings
- Make urethral opening (urine)
- Make vaginal opening (may need to use intestine to make vagina if the vagina is not long enough to reach)
- Make opening for stool evacuation (anus)
- May need to also perform laparotomy (incision on belly)
- Postoperative care
- Usually in hospital for pain control, antibiotics, wound monitoring
- Bladder catheter in place usually while in the hospital
- Stool comes out of the colostomy so that stool does not contaminate the new incisions.
- Nothing should be placed into the newly created openings as they are healing for the first 2-3 weeks.
- Return to clinic in 2-3 weeks to start learning dilations of anal opening. Parents will then perform dilations at home to keep the hole from closing up.
- Anesthesia: Baby will have to be completely asleep with anesthesia for surgery
- Small risk of bleeding
- Small risk of infection (that is one reason the colostomy stays in place to decrease this risk)
- Risk of damaging vagina or urethra when trying to separate and create the three holes
- Create three distinct holes for the child’s urinary and stool evacuation, and for eventual sexual function
- Third surgery
- Goals: Colostomy closure—reconnect the intestine so the baby can poop out of the new anal opening and not have to use a colostomy anymore.
- Preoperative preparation
- The time when the colostomy is closed is dependent on whether the sites for the previous operation are completely healed.
- Before surgery, will need study of the intestine leading to the anus to make sure there are no areas of narrowing and the anal opening empties well.
- Bowel prep day before surgery—usually clear liquids and magnesium citrate or golytely (depends on surgeon)
- Exam under anesthesia to look at anus
- Takedown ostomy: reconnect colostomy and mucus fistula
- Postoperative care
- Antibiotics after surgery
- May wash incision with soap and water
- Continue anal dilations
- Diaper rash cream to buttocks since baby’s bottom has not yet seen stool. The skin is prone to breakdown.
- Anesthesia: Baby will have to be completely asleep with anesthesia
- Small risk of bleeding
- Risk of infection: Baby will be on antibiotics after surgery
- Wound infection at incision: Baby will be on antibiotics after surgery. Will closely watch incision for signs of infection. May need dressing changes
- Risk of leak from anastomosis (connection of intestine): Go slow with removing NG/OG tube and advancing feeds
- Benefits: No more stoma. Baby’s intestine now all connected.
Home Care ("What do I need to do once I take my child home?")
- Diet: Your child may eat a normal diet after surgery.
- Activity: Your child should avoid straddling after the first two operations.
- Wound care: Surgical incisions should be kept clean and dry for a few days after surgery. Most of the time, the stitches used in children are absorbable and do not require removal. Your surgeon will give you specific guidance regarding wound care, including when your child can shower or bathe.
- Medicines: Medicines for pain such as acetaminophen (Tylenol) or ibuprofen (Motrin or Advil) or something stronger like a narcotic may be needed to help with pain for a few days after surgery. Stool softeners and laxatives are needed to help regular stooling after surgery, especially if narcotics are still needed for pain.
- What to call the doctor for: Call your doctor for worsening belly pain, fever, vomiting, diarrhea, problems with urination, decreased stool output or if the wounds are red or draining fluid.
- Follow-up care: Your child should follow up with his or her surgeon 2-3 weeks after surgery to ensure proper post-operative healing. IT IS IMPORTANT TO KEEP ALL APPOINTMENTS WITH THE SURGEON, AS HEALING IS CRITICAL IN THE FIRST FEW MONTHS AFTER SURGERY.
Long-Term Outcomes ("Are there future conditions to worry about?")
- Bowel obstruction: Baby will have scar tissue in belly from surgeries. Scar tissue can cause kinking or blockage of intestine. If baby has yellow or green throw up, big belly, or does not pass gas/stool from stoma/anus, bring baby to pediatrician or surgeon or ER. Sometimes this can be treated with NG tube and IV fluids and stay in hospital. Sometimes this needs surgery to open up scar tissue and fix obstruction.
- Incontinence (cannot control need to pee and poop): These babies often have problems with potty training both urine and stool. Kids may not be able to feel when they have to pee or poop. They may have to see a specialist (urologist) for problems with urine. For problems with controlling stool, may need to be on bowel management or require other surgeries to try to keep the child clean.
- 20% or fewer of girls with cloacal anomalies have good continence
- Prolapse of anal mucosa (inside lining of the intestines are sticking outside the anus) may occur.
Author: Grace Mak, MD
Editors: Patricia Lange, MD; Marjorie J. Arca, MD