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Anorectal Malformation, aka Imperforate Anus, is a spectrum of abnormalities of the rectum and anus.

There are many possible abnormalities as follows: 1. The absence of an anal opening. 2. The anal opening in the wrong place. 3. A connection, or fistula, joining the intestine and urinary system. 4. A connection joining the intestine and vagina. 5. In females, the intestine can join with the urinary system and vagina in a single opening, known as a cloaca.

There is no known cause for anorectal malformation and most cases are isolated and do not run in families. This birth defect occurs in one in every 5,000 live births. It is more common among Asians and is somewhat more common in boys than in girls.

Answers to Common Questions


What are symptoms of anorectal malformation?
If there is a fistula to the skin, urethra or vagina, a newborn will pass meconium (a baby's first stool) and the anorectal malformation may not be suspected. If there is no anal opening and there is no fistula, the baby will not be able to pass stool after birth and this will lead to a swollen or "enlarged" abdomen and vomiting. The specific anatomic abnormality will vary, and it is important for you to understand your child's particular anatomy.

How is the diagnosis of anorectal malformation made?


The diagnosis is made by physical examination. If the anal opening is absent or in the wrong spot, it can be seen on examination. If there is stool coming out of the urethra, or vagina, it will be visible. In females with anorectal malformation, careful examination of the vestibule (area between the labia) must be made to ensure separate openings of the urethra and vagina. In the male with anorectal malformation, there can be a fistula to the urethra and stool may come out of the penis. If this is not observed directly, and stool is found in the diaper, the diagnosis may be missed.

What other anomalies are associated with anorectal malformation?


Abnormal vertebrae (including scoliosis, kyphosis and particularly abnormal sacral vertebrae) Anomalies of the urinary system Anomalies of the heart A tethered cord Abnormal pelvic muscles Cloacal anomalies

What is the treatment for anorectal malformation?


In general your child will need 3 operations to correct the anorectal malformation, The first to create a stoma, the second to pull the intestine to the skin through the anus and the third to close the stoma. Some children will only need one operation but this is less common. As the anatomy varies with each child the surgical plan will be determined after your child is carefully evaluated by the surgeon. If there is an opening for stool to drain, but it is in the wrong location, it will be closed and a new anal opening will be made in the correct location, within the anal sphincter. If there is no opening for stool to drain and the rectum is low and near the anal sphincter a new anal opening will be made in the correct location, within the anal sphincter. If there is no opening for stool to drain and if the rectum is far from the skin and the intestine ends high in the pelvis, surgical repair will be done in stages. First a stoma, or connection of the intestine to the abdomen, is made. This will allow stool to pass and baby can go home to recover and grow. A second stoma will be made near the stoma that drains stool. This is called a mucous fistula. This allows the other end of the intestine to drain as needed. In the next operation, a new anal opening will be made in the correct location, within the anal sphincter. If a fistula is found to the urinary system, during the operation, it will be closed. The stoma draining stool will be left in order to allow the surgical site to heal. Two weeks after the operation to make a new anal opening, anal dilations will need to begin. This will be discussed below. When the new anus has been adequately dilated and is soft, the stoma will be closed.

How long will my child be in the hospital after the operation?


Children can go home once they are feeding normally, having bowel movements per stoma or anus, are comfortable on pain medication by mouth and do not have a fever.

Do I see the surgeon again after the operation?


Bring your child to the surgeon's office for a postoperative visit two weeks after the operation. During this visit we check the stoma or, if there is no stoma, the size of the anal opening. Most children have to be dilated, or have the anus stretched, each day to prevent the anus from becoming too tight. You will be given a set of anal dilators to take home with you

from the hospital. Bring them to your baby's first surgical appointment and if dilations are necessary, we will show you how to do them. Dilations usually last a few months.

When do I call the surgeon's office?


Call our office at 415-476-2538 for the following:

Any concerns you have about your child's recovery A temperature of 101F or higher A red incision Increasing pain and tenderness at the incision Any liquid coming out of the incision

Definitions of medical terms

Stool- Poop, Feces. stools may be: o Liquid - diarrhea o Pasty - sticky; putty like o Formed - sausage links-like o Constipated stool - hard stool, stone-like New stool - stool from food eaten in the past 48 hours. Old stool - stool from food eaten more than 48 hours ago Bolus of feces - balls of feces Bulky stool - several balls of feces Infrequent stooling - bowel movements less than 2 a day (infants 0-6 months old); less than 1 a day (6 months to 3 years); less than 1 every 2 days (more than 3 years) Constipation - delay or difficulty in defecation resulting from failure to empty the rectum completely. This results in an increase in the hardness and size of stool. Passing stool frequently in small amounts throughout the day may also be constipation. The longer the stool remains, the harder it becomes. The longer the rectum remains distended, the less the feeling of urgency, the less the propulsive power of the rectum. The longer the period of constipation, the more an effective Valsalva is needed (ineffective gastro-colic reflex) with relaxation of the sphincters. Incontinence - absence of voluntary (conscious) control of bowel movements; results in unconscious (involuntary) leakage of stool. Inability to consciously contract the pelvic muscles (pelvic squeeze) and the anal sphincters (buttock squeeze) to "hold" stool. Inability to do an effective Valsalva (i.e. paralyzed abdominal muscles) in coordination with relaxation of the pelvic muscles and anal sphincters resulting in ineffective emptying of the rectum. True incontinence exists in conditions such an Meningomyelocele with paraplegia. Encopresis - Soiling/"accidents" - Poop in pull-ups or underwear, occurring spontaneously, during physical activity, coughing/sneezing, or overnight; involuntary (unconscious) leakage of stool; Incontinence/pseudo-incontinence may cause soiling. Soiling does not necessarily mean incontinence. Pseudo-Incontinence - unconscious passage of watery stool around constipated stool. If abdominal muscles, pelvic muscles and anal sphincters are neurologically intact and the patient can do an effective Valsalva with relaxation of the sphincters, he will be clean of stool with disimpaction, laxatives and timed toilet training. Continence - voluntary (conscious) control of bowel movements, resulting in an empty rectum after an effective bowel movement. It involves holding the stool

by Pelvic squeeze, contracting the pelvic muscles, and/or Buttock squeeze, contracting the anal sphincters Good/Effective bowel movement - Emptying the rectum of stool, coordinated with relaxation of the pelvic muscles and anal sphincters by doing a Valsalva, ake a deep breath, hold, then push. Or rolling contraction of abdominal muscles from top to bottom, hold, then push. Coordinated with a relaxation technique. Recto-anal coordination - the contraction of abdominal muscles and relaxation of pelvic muscles and anal sphincters (all voluntary) in coordination with rectal peristalsis (involuntary; gastro-colic reflex). Used in conjunction with timed toilet training. Timed toilet training - Educating the child to attempt a bowel movement at least twice a day, 30 minutes after breakfast and 30 minutes after dinner to take advantage of the gastro-colic reflex; or to take advantage of the circadian rhythm and the physiologic giant migrating contractions of the colon upon waking up.Teaching the child to push at a level of 5-7 (on a scale of 10 maximum effort) for 5 minutes; relax and repeat as needed and educating the child to empty the rectum and not to stop after the first stool is passed. Colon - Large intestine/large bowel. This is divided into the right or ascending colon, the middle or transverse colon, the left or descending colon, and the S-shaped or sigmoid colon. Rectum - The end of the large intestine, below the sigmoid colon and above the anal canal. Pelvic Muscles - Pubo-coccygeus muscle including the pubo-rectalis, pubo-urethralis, and pubo vaginalis (in females); under voluntary or conscious control. Anal sphincters - External anal sphincters are composed of the deep anal sphincter, which overlaps with the pubo-rectalis muscle, the superficial and the subcutaneous anal sphincters which are all under voluntary or conscious control. Valsalva - Take a deep breath, hold, contract abdominal muscles including diaphragm, and push against a closed glottis. Also called "straining at stool". Alternative Valsalva - Rolling contraction of abdominal muscles (mainly the recti muscles) from top to bottom, hold, and push. Simulates giant migrating contraction (GMCs; involuntary) from the left colon down to the rectum. Similar to "bellydancing". Relaxation technique for pelvic muscles - Do a Valsalva then while pushing, exhale slowly blowing bubbles through a straw under water in a glass. Continue blowing until you have exhaled completely. Repeat. Alternatively, you may blow soap bubbles.

The physiology of pooping


Pooping is a complex mixture of voluntary (conscious) actions and involuntary (unconscious) colonic contractions. It includes a conscious contraction of the abdominal muscles (recti, internal and external oblique muscles and the diaphragm), in coordination with relaxation of the pelvic muscles (pubo-coccygeus, especially the pubo-rectalis) and the anal sphincters (especially the deep external anal sphincter). The anal canal is responsible for propioceptive sense which allows an individual to differentiate stool, solid from liquid, or air (gas). Rectal distention elicits a perception of fullness. It is not clear whether the stretch receptors of the rectum are in the smooth muscles

or the perirectal tissue. What is known is that rectal distention stimulates the stretch receptors to give a perception of fullness. Voluntary actions If it is socially appropriate, the person goes to a restroom, does a Valsalva (push/straining at stool), relaxes his pelvic muscles and anal sphincters, stool moves down and out, producing a bowel movement. When it is socially inappropriate, the person consciously contracts his pelvic muscles and anal sphincters (voluntary) and keeps stool in. The abdominal muscles relax, breathing is controlled to limit diaphragmatic movement, the stool moves up, rectal distention diminishes and the urge to pass a bowel movement disappears. If there is a cough/sneeze at this point, there is reflex contraction of the anal sphincters to prevent leakage of stool. Involuntary colonic contractions The intrinsic physiologic colonic contractions (involuntary) include the individual phasic contractions (IPC) and the giant migrating contractions (GMC). The IPCs are intermittent and create a to-and-fro movement in the intestine which allows maximal absorption of water in the right and transverse colon. The GMCs are stronger but less frequent and are more regular in the descending and sigmoid colon. They follow a circadian rhythm, most prominent after waking up. The GMC creates a mass movement of stool down toward the rectum, causing rectal distention, stimulating the stretch receptors and giving a perception of fullness. This is amplified after a meal when gastric distention initiates the gastro-colic reflex. The top to bottom rolling contraction of the abdominal muscles (belly dancing) which is voluntary, simulates the movement of stool down toward the rectum. Timed toilet training Timed toilet training consists of educating the child to attempt a bowel movement at least twice a day, upon waking up (circadian rhythm); or after breakfast and after dinner, to take advantage of the gastro-colic reflex. A Valsalva, at a level of 5 to 7 (level 10 - maximum effort of straining) should be done. This is repeated until the rectum is empty. Prevention and treatment of constipation Prevention of constipation is the best approach for young children. In post-operative patients with anorectal malformation and Hirschsprung's Disease, pasty/sticky stool should be recognized and treated early with increased fluid intake and oral laxatives. Infrequent stooling should be treated with stimulant laxatives; osmotic laxatives and fiber for softening the new stool; rectal suppositories or retrograde enemas to soften the old stool. Digital rectal exam is needed to rule out impaction. If enemas fail to disimpact, it might be necessary to disimpact under anaesthesia. Abdominal Xrays are necessary to identify stool in the right colon. The use of enemas in young children will be considered carefully to avoid aversion. If needed, parents will be trained to do enemas before the child turns 1yr. Retrograde enemas should be reserved for soiling ("accidents") and severe diaper rash in the early post-op period or in patients with

abdominal Xrays showing solid stool in the right colon. In patients who do not know how to do a Valsalva, a high colonic irrigation would be more effective.

Home Care After anorectal malformation Repair


Home care immediately after surgical repair
Pain Management Prescription pain medication is not routinely required after hospital discharge. Most children are comfortable using Acetaminophen (Tylenol) or Ibuprophen (Motrin) once they are at home. Follow the dosage directions on the label. If your child is still uncomfortable, call our office. Care for Dressings The incision(s) will be covered with plastic film dressing. Under this are pieces of white tape called Steri-strips. The clear plastic dressing may be removed in 2-5 days, as instructed by the surgeon and then your child can bathe or shower with the Steri-strips in place. These strips fall off on their own or can be removed when they become loose. Under the steri strips is the incision. This is usually closed with dissolvable suture. There will be no visible stitches to remove because they are under the skin. The stitches will dissolve after several weeks. In some children these stitches may come through the incision, about a month postoperatively. This may be associated with a little local redness and pus and it may involve an end of the incision or a larger portion. This is normal and is best treated by gently cleansing the area with soap and water and waiting. When the suture falls out or completely dissolves, the wound will heal. If your child has worsening redness, swelling pain of the incision and a fever within 2 weeks of the operation, please call our office. Healing Ridge After the incisions are healed you will be able to feel a firm ridge just underneath the incision. This is called a healing ridge and it is normal to find this under an incision after an operation. The healing ridge usually lasts for several months before it softens and disappears. Bathing Restrictions Your child may bathe or shower as between 2 and 5 days after the operation, as directed by the surgeon. Bathing may be done without restriction. The anus can be cleaned (washed and patted dry) like a normal anus. If your child has a stoma, the pouch can be removed and your child, and his or her stoma, can be immersed in the bath. This will not harm the stoma and is recommended. The stoma does not feel any pain and can be cleaned (washed and patted dry) and the skin around it wiped like a normal anus. Activity Restrictions There are no specific activity restrictions following surgery. Babies, even with stomas and pouches can be carried and cuddled like a normal baby.

Diaper Skin Care After the operation to bring the intestine to the anal sphincter, very frequent bowel movements occur and can cause severe diaper rash. Begin using a protective skin cream as soon as bowel movements start. A diaper rash can occur rapidly and take days or weeks to heal. We recommend that you continuously apply the recommended protective skin care products. Begin as early as the day after surgery. Do not stop using the skin protective products until the number of bowel movements becomes less, usually after many weeks. If your child develops a rash that does not get better, please call our office. Here is a suggested routine. 1. Remove all butt balm (see below) cream, paste or ointment daily with mineral oil applied gently with cotton balls. 2. Soak your child's bottom in a tub of warm water after applying the mineral oil. 3. Clean gently with soap and water, do not scrub skin. Avoid making the skin bleed. 4. At the end of the bath, pat at the skin dry. 5. Apply Cavilon 3M No Sting Barrier Film by swab or spray to all affected skin in the diaper area. 6. Let dry for 60 seconds 7. Apply butt balm or other barrier paste over the 3M product. 8. After each bowel movement, clean the poop off the butt balm and do not rub the butt balm from the skin. 9. Reapply the butt balm to the diaper area. 10. Repeat after each bowel movement. 11. Repeat mineral oil and bath each day. 12. Apply Cavilon 3M no sting barrier film to skin around anus 13. Butt Balm Recipe 14. The recipe for butt balm is: a four ounce tube of Desitin ointment, a one ounce bottle of Stomahesive powder, mixed well. These products are available in stores or on-line. No prescription is needed. Colostomy Care Colostomy requires routine care to be taken with the pouch and a sigmoid colostomy requires additional instructions for diaper management. For info on homecare for a colostomy please refer to our Colostomy Care page.

Why does my child need anal dilators?


You will receive a set of anal dilators before you leave the hospital. Bring these to your first surgical appointment. Some anal incisions heal by contracting and becoming tight. If this happens, passing bowel movements will be very difficult. Stretching the anal incision gently with anal dilators will be required by your child's pediatric surgeon. Please follow instructions carefully. This is essential to prevent a stricture or narrowing of the anal opening. If you are having problems with anal dilation contact the surgical nurse practitioner. Supplies Gather dilator, K-Y lubricant, clean diaper, wipes and someone to help you, if needed.

Directions for anal dilations at home 1. Position your child on his/her back, holding the feet toward the head with knees flexed. 2. Lubricate the dilator and gently insert into the anus as instructed. 3. A very small amount of bleeding may occur, this can be normal. 4. Withdraw the dilator. Clean with soap and water. 5. Continue the dilations, at home using starting dilator size as directed by surgeon. 6. Use twice daily, for one week. 7. Every week, change to a dilator that is one size larger. 8. Use twice daily for one week. 9. Stop increasing the size once you get to the maximum size as directed by your surgeon. Tapering dilations 1. 2. 3. 4. 5. Continue twice daily dilations for 2 weeks using dilator size as directed by surgeon. Decrease frequency to once daily for 2 weeks. Decrease to every Monday, Wednesday and Friday for 2 weeks. Decease to once a week for 2 weeks. Stop dilations.*

*Do not discard your dilators. They are to be cleaned with soap and water and saved in the event they need to be used again. Regular follow up is recommended for bowel management of children with anorectal malformation and Hirschsprung's Disease in our LIFE clinic. Guidelines for sizing anal dilators:

1-4 Months of age 12 4-8 Months of age 13 8-12 Months of age 14 1-3 Years of age 15 3-12 Years of age 16 > 12 years of age 17 to 18

What ongoing colonic care will my baby need?


Babies with anorectal malformation are special babies who need special parents. They will need colon care throughout life but with personalized training, they can become very independent. Laxatives and, if needed enemas, will be a continuing resource for preventing constipation and maintaining a clean colon. Laxatives soften new stool and enemas clean out residual stool. Occasionally, when the residual stool builds up (stool in the right colon seen on X-ray), there may be a need for a laxative clean out, to treat the stool back up. This emphasizes the need for regular colonic care by use of laxatives or enemas, in order to avoid constipation.

The first thing to remember is that children with anorectal malformation may have a limited capacity to sense the need to pass a bowel movement. Therefore bowel training has to be coordinated with the normal colonic activity that produces a bowel movement. Normal intestinal activity is stimulated with every feeding by gastric distention or stomach stretching. This is called the gastrocolic reflex. This is why a baby poops after every feeding. Your baby's first year During the first year, bowel training is as simple as pressing the lower half of the abdomen with the open palm while raising both legs before changing the diaper following a poop. This simulates the Valsalva maneuver (conscious straining/pushing at stool) which the baby will learn to use at a later age. As the baby shifts from breast milk to formula and on to solids, the stools will be more bulky and bowel movements will be less frequent. Watch for pasty (sticky) stools as this is the first sign of constipated stool. Increase fluid intake (juice/water) in between milk/solids feedings at the first sign of pasty stools. Schedule a clinic visit if this persists. Oral laxatives may be necessary to prevent pasty stools. Prevention of constipation, from the time of the operation, is the foundation of a comfortable, well adjusted and independent life. A diaper rash may occur if the buttocks remain moist in between the change of diapers. This may be controlled with a cream/ointment the LIFE clinic NP will teach you to use. If the rash occurs around the anus, soft or liquid stool is leaking from the anus. This may occur with or without constipated stool. If the rash is severe, enemas may be needed to clear up the rash in addition to the cream/ointment. The NP will teach you how to do this during a clinic visit. Again, it helps to press the abdomen with an open palm while raising both legs after the enema to simulate the Valsava and pushing/straining at stool. If there is no diaper rash, the retrograde enema should still be learned at about 6 months of age since oral laxatives and retrograde enemas are the mainstay of long term colonic care for babies with anorectal malformation. Starting the enemas after one year is traumatic to the baby and may actually make colonic care more difficult for both parent and child. Your baby's second year In the second year, moderate straining is normal with bulky bowel movements. Pressing the abdomen with open palm while the baby is straining/crying helps to stimulate the baby to contract the abdominal muscles reflexively to help pass a bowel movement. In time, this reflex will become a conscious effort. Formed to firm bowel movements may require more straining. If this is noted, increase fluid intake (juice/water) in between feedings and schedule a clinic visit and an abdominal Xray. The dose of oral laxatives may be increased even before the clinic visit. Blood streaks lining the firm stools may mean anal fissures which are cracks or tears near the anus. Retrograde enemas may be required based on the results of the abdominal Xray. Enemas may be difficult and traumatic in the presence of fissures. Thus, firm stools should be treated aggressively before fissures develop. Timed toilet training is started when the child can sit at the toilet. Breakfast (or the first large feeding for the day) is given at a convenient time so the parent can stay with the child at the potty after breakfast. This is to take advantage of the gastrocolic reflex, a reflex in babies that makes them stool after eating. Sit the child on the potty with the upper body slightly leaning forward. Press the abdomen with an open palm to simulate Valsalva and pushing/straining

stool. The first few days, spend enough time to ensure a good bowel movement. Repeat the Valsalva and wait for a large bowel movement, rather than stopping after a small bowel movement. This is to avoid giving the child the impression that a small bowel movement is adequate. Small bowel movements lead to a building up of stool in the colon especially at an age when playing is more interesting than a moving the bowels. Dinner (or the last large feeding before bedtime) is also given at a convenient time, so the training can be repeated. Since most of the activities are during the day, timed toilet training twice a day allows the child to be clean in between two big meals. Soiling at night may need to be managed by colonic irrigation before bedtime.

Directions for timed toilet training


Parent awareness and patient education is the key to long term colonic care. Children with anorectal malformation and Hirschsprung's Disease are special people who need specialized care. They often need anal dilation post-operatively and are particularly prone to constipation or diarrhea. Colonic care will always be a part of their lives and laxatives and enemas will be normal for daily care. Corrective surgery for anorectal malformation places the colon through the pelvic muscles (primarily the pubo-rectalis muscle) and the anal sphincters (primarily the deep external anal sphincter). These are striated muscles and are under voluntary control; however, a child needs to learn how to control them. Even adults may not be aware of how to consciously relax and contract the pubo-rectalis muscle. There are 2 exercises to practice. The first is stopping and starting the urinary stream. The pelvic muscles that stop the urinary stream are the same muscles that stop a bowel movement. This can help develop awareness of the sensation of pelvic muscle contraction and relaxation. Thus, if the child practices stopping and starting the urinary stream regularly, it increases awareness and skill at voluntary control of this muscle. The second exercise is the contraction of the gluteus muscles, the "buttock squeeze", which also causes the external anal sphincters to contract. Thus, doing the "buttock squeeze" increases the awareness and skill at voluntary control of this muscle. Parents must be aware of these exercises themselves in order to teach their child how to do the exercises. A good bowel movement is produced with a Valsalva maneuver coordinated with relaxation of the pelvic muscles and anal sphincters. Therefore the next skill for a child to learn is the Valsalva maneuver. This is accomplished by taking a deep breath, holding, and pushing while keeping the glottis closed and the sphincters open. This is known as straining to stool. Alternatively, a rolling contraction of the recti muscles from top to bottom (belly-dancing), holding, and pushing achieves the same effect of pushing stool in the left colon down to the rectum. Occasionally, a child may know how to contract the pelvic muscles and anal sphincters but not know how to relax them. A helpful relaxation technique is to blow bubbles with a straw under water in a glass. This is done while exhaling slowly. Alternatively, the child may blow soap bubbles through a hoop. This is done while doing a Valsalva or a rolling contraction. When the rectum fills with stool and becomes distended, the child will feel a sensation of fullness. The pelvic muscles and anal sphincters can be consciously contracted with relaxation of the abdominal muscles and the diaphragm (short, shallow breathing). This will

relieve the sensation of fullness and allow bowel control. The feeling of fullness may not be present in some children with anorectal malformation. If so, the child must learn to keep his pelvic muscles and anal sphincters contracted, or squeezed closed, at all times to avoid soiling. For these children without rectal sensation, soiling may be present at night because the sphincters do not remain contracted while asleep. Staying clean is important to avoid teasing and bullying from peers. If your child cannot control bowel movements, even with practicing these exercises, contact our office. Muscles of the colon are smooth muscles and are involuntary, and cannot be consciously controlled. As such, peristaltic waves can not be controlled consciously. Peristaltic waves are muscular contractions that occur intermittently throughout the day, moving stool to and fro within the colon, allowing water to be absorbed. Giant migratory contractions (GMC) occur less frequently, usually on waking up in the morning and occur mainly in the left or descending colon. After meals gastric distention initiates the gastrocolic reflex and the GMCs move stool down to the rectum. Timed toilet training takes advantage of this activity and helps the child learn to pass a bowel movement on the toilet at a specific time (after breakfast and after dinner). Using the Valsalva helps relax the pelvic muscles and anal sphincters and evacuate the rectum. "Staying clean" is important to avoid teasing and bullying from peers. Since "dryness" and "smelling good" depends on avoidance of "accidents" (soiling), a daily regimen of sitting down at the toilet twice a day, after breakfast and after dinner, is recommended. Using the Valsava intermittently, and straining at a level of 5-7 (maximum level of 10) for 5 minutes is suggested. The child should be taught not to stop at the first stool passed but repeat until the rectum is empty. This requires concentration so the child must not be distracted by other activities. If this is successful, the rectum should be empty between the 2 timed bowel movements. Occasionally, there may be soiling in between the 2 timed bowel movements especially when the child is active in sports. This is managed with colonic irrigation, modifying diet and eating habits, and a reminder to keep the pelvic muscles and anal sphincters closed. If the soiling is at night, colonic irrigation should be done at bedtime since the pelvic muscles and anal sphincters are relaxed while asleep. This routine establishes 2 things: 1. The gastrocolic reflex initiates the bowel movement with or without the feeling of rectal fullness. 2. The child is clean the whole day and the whole night. Since eating and drinking distends the stomach (ie, with gas as in sodas) a bowel movement may be stimulated and the possibility of accidents exists. This reinforces the need to keep the pelvic muscles and anal sphincters contracted. The earliest time to teach a child timed toilet training would be when he/she can sit at the toilet. This is the same time when it is ideal to start toilet training in a normal child. Be patient, your child may achieve control only much later. If timed toilet training fails after a reasonable effort, an enema regimen will be initiated, or restarted.

What colonic care will my child need as they grow into adolescence?

The success of a Colonic Care Program is based on the determination and the perseverance of the young adult to be independent and responsible for his own well-being. If constipation is avoided during the first few years after the corrective operation(s) and the young adult has learned how to do an effective Valsalva (pushing/straining) with relaxation of the pelvic muscles and anal sphincters, a Colonic Care Program will be successful. In anorectal malformation, the measure of success is being clean and smelling good. 1. The Colonic Care Program will be tailored to the young adult's needs and consists of 8 activities: 2. Regularly timed voluntary bowel movement, twice a day, after breakfast and after dinner. 3. Prevention of constipation with oral laxatives. 4. Cleaning out residual stool with a laxative clean out and retrograde/antegrade enemas. 5. Exercises to strengthen the abdominal muscles to maximize the Valsalva maneuver; for example, diaphragmatic breathing exercises, sit-ups, athletic sports, and calisthenics. 6. Belly-dancing for an alternative/adjunct to the Valsalva maneuver. 7. Exercises to train the pelvic muscles and anal sphincters to contract. Examples: the pelvic squeeze and the buttock squeeze. 8. Relaxation techniques for pelvic muscle and anal sphincter relaxation. 9. Modifying diet and eating habits. Soiling (or accidents) may be a problem between the twice a day, timed bowel movements. There are 5 factors to consider: 1. Inadequate or asymmetrical pelvic muscles and sacral cord anomalies can be assessed by pelvic MRI. Abnormal muscles and nerves cannot be replaced. 2. Ability to sense the presence of stool in the rectum. This is either present or absent and cannot be learned. If absent, conscious control of the pelvic muscles and the anal sphincters can still be learned if there are normal muscles and nerves. 3. Chronic constipation may over-stretch the rectum and its stretch receptors. This will decrease the ability to sense the presence of stool. Relief of constipation may reverse this loss of sensation in some patients. 4. Increased physical activity, particularly sports. Physical activity causes the abdominal muscles to contract to keep the body erect. This increases the intra-abdominal pressure. The greater the activity (especially in sports), the greater the increase in pressure. To remain clean, the sphincters have to remain closed. If prolonged control is not adequate, the colon has to be empty before any activity to avoid accidents (soiling). To achieve this, the maximum dose of oral laxatives is given at bedtime. This allows the laxative to work overnight. After breakfast, the young adult has the regular timed bowel movement. If there is soiling during sports, it may be necessary to do colonic irrigation before physical activity. 5. The effect of select foods and timing of eating. Eating and drinking causes the stomach to stretch. This gastric distension stimulates the colon to actively contract. This is known as the gastro-colic reflex and is responsible for the urge to have a bowel movement after eating. The amount of food and the kind of food eaten determines the intensity of the response. Carbonated drinks (soda, "sparkling" drinks) produce gas which distends the stomach, stimulating the gastro-colic reflex. Eating and drinking in small amounts and avoiding soda, minimizes the gastro-colic reflex. Certain foods may produce more colonic activity than others. Fried foods (burgers,

French fries, chips) produce more prolonged activity than boiled, broiled or baked foods; sweet foods more than bland foods. Foods that are best tolerated will be learned through trial and error. It may be necessary to avoid eating and drinking before moderate physical activity, as it is difficult to contract the sphincters for long periods during active exercise (increased intra-abdominal pressure). If there is soiling during active exercise, it may be necessary to do colonic irrigation before the exercise to ensure the colon is clean. This will reduce the need to contract the sphincters during exercise. Colonic irrigation until the return flow is clear is more effective than retrograde enemas to avoid accidents.

Types of laxatives and how they work


Laxatives given by mouth are most effective for new stool but do not soften old, hard stool. Therefore they are best used to prevent constipation because soft, large stool (new stool) is easier to expel than small, hard stool. Laxatives given per rectum (Glycerin, Bisacodyl) are for small, hard stool (old stool).An early sign of constipation is the passage of sticky/pasty stool. The following is a summary of the types of laxatives commonly used, how they work within the intestine. Stimulant laxatives Stimulants work by increasing peristaltic activity (muscular contractions) of the intestinal muscles or by local irritation of the mucosal lining of the intestine.. Examples: Senna (Little Tummys, Ex-Lax, Pedia-Lax, Senokot); Bisacodyl (Dulcolax, Correctol, Bisco-Lax) available in oral and rectal preparations; Ricinoleic Acid (Castor oil) is a powerful purgative. Osmotic laxatives Osmotic laxatives retain fluid in the lumen of the colon, softening new stool. Examples: Polyethylene Glycol 3350 (Miralax, Glycolax); Polyethylene Glycol Electrolyte Solution (GoLYTELY; NuLYTELY); Hyperosmotic laxatives Hyperosmotic laxatives attract water from the bowel wall, increase intra-luminal pressure and stimulate peristalsis. Oral preparations are poorly absorbed (sugars, disaccharides) and make new stool soft. Saline laxatives (Milk of Magnesia) attract water from the bowel wall and stimulate peristalsis. Rectal preparations increase intra-luminal pressure and stimulate peristalsis. Examples of poorly absorbed sugars are Sorbital and Lactulose. Saline laxative: Milk of Magnesia; Examples of rectal preparations are the phosphate enemas. Lubricant laxatives Lubricants coat the bowel wall and stool with a waterproof film; inhibits colonic absorption of water; emulsification softens stool (Glycerin- best used per rectum); lubricant effect eases passage of stool. Example: Mineral oil.

Oral Mineral oil for children more than 1 year old: 1 - 3 mL per Kg per day

Oral high-dose Mineral oil for disimpaction: 15 - 30 mL per year of age per day; Maximum of 240mL (8oz) for 3 or 4 days Mineral oil may also be given per rectum 6mL/Kg; Max of 30 mL once a day. Glycerin may also be given per rectum 6mL/Kg; Max of 30 mL once a day.

Stool Softeners Softeners facilitate admixture of fat and water to soften stool. Example: Docusate; for use in the presence of anal fissures. FIBERS Insoluble fiber (does not dissolve in water) The unprocessed fiber found in grains, skin of fresh fruits, and vegetables; not absorbed; provides the bulk for new stool producing soft stools. When processed they become powders and become soluble. Examples: Psyllium (Metamucil); Methylcellulose (Citrucel); Polycarbophil (Fibercon) may be beneficial for both constipated and diarrheic stools. Soluble fiber (dissolves in water) Processed fiber; found in food such as legumes, oats, barley and fruit containing pectin (apple peel); acts like osmotic laxative, absorbs water from the lumen of bowel producing soft, bulky stools. Examples: Gaur Gum, Wheat Dextran (Benefiber ), Inulin (Fibersure) and Pectin ("Sure Jell", "Ball" or "'Certo").

Directions for retrograde enemas (for old stool)


Indications for enemas are impacted stools, persistence of stool in the right colon on abdominal Xray, soiling (or accidents, which results from unconscious passage of stool), and severe perianal rash that does not resolve with topical agents. The successful evacuation of stool with enemas is dependent on the ability to do an effective Valsalva with relaxation of the sphincters to push out gas, fluid and stool. If the patient can not do an effective Valsalva, colonic irrigation is more appropriate. Stimulant laxatives (Bisacodyl) and lubricant/emollients (Glycerin, Mineral oil) can also be given per rectum. In general, enema training should be started before the child turns 1 year old to avoid trauma and enema aversion. Types of enema solutions

Isotonic - Normal Saline (0.9% saline) Hypotonic (Hypo-osmolar) - Tap Water w or w/o Castille soap Hypertonic (Hyperosmolar) - Sodium phosphate (Fleet Enemas) Lubricant/Emollient (Glycerin, Mineral oil) and stimulant laxatives (Bisacodyl) can be given per rectum.

Volume of enema fluid

Infants less than 1 year: 6 mL per Kg (0.2oz/Kg); Maximum of 135 mL (4-5oz) daily

Children more than 1 year: 6 mL per Kg; Maximum of 135 mL; can be given 2 - 3 times a day. or, 10-15 mL per Kg; Maximum of 500 mL given once a day Hypertonic/hyperosmolar enemas are given in smaller volumes. Lubricant/emollients and stimulant laxatives (Bisacodyl) are also given in smaller volumes.

Directions for retrograde enemas Successful retrograde enemas are dependent on the ability to empty the rectum after the infusion of fluid. A child must know how to do a Valsalva (push/strain at stool) while relaxing his pelvic muscles and anal sphincters in order to empty his rectum for retrograde enemas to be successful. If the patient can not do an effective Valsalva, colonic irrigation is more appropriate. It is recommended that you keep a diary of enema details (amount of fluid and additives) and results as you get started. This will help you track changes and results more effectively. It may take several weeks of practice and adjustments to get good results. The goal is for your child to be accident free (no soiling) for 24 hours, between enemas. Gather supplies: These supplies will be ordered by the surgical nurse. Once the order is approved and filled, supplies will be sent to your home. 1. 2. 3. 4. 5. 6. Reusable enema bottle or Enema bag (Feeding bag acceptable) Silicone Foley Catheter - 20-24 French with 30 cc balloon Water soluble lubricant 30 cc slip tip syringe 60 cc catheter tip syringe Tap water or Saline as ordered by the surgical team

Getting started: 1. 2. 3. 4. Fill enema bag/bottle with __________ mL water or saline as instructed. Position your child, either bottom up or lying on the side. Test the balloon before using to make sure it is functional. Lubricate catheter and insert into anus about 4-6 inches, hold steady and inflate balloon with 10-30 mL of air to, as instructed, then pull back to secure balloon against anal opening. 5. Attach enema bag tubing to catheter and start flow of water or saline - This should take between 5-10 minutes. 6. Raising the bag will make the flow go faster, and lowering the bag will make it go slower. Proceed at a rate that is comfortable for your child. 7. Maintain some tension on the catheter to minimize leaking. 8. It is best if your child can hold the enema solution for 5-15 minutes. 9. Next have your child sit on the toilet, withdraw the air from the balloon and have him or her push out the catheter. 10. It is recommended that your child sit on the toilet for 30-45 minutes to allow all the water/stool to be pushed out. Repeatedly doing the Valsalva maneuver may shorten the waiting time.

11. Avoid letting the child do any other activity, i.e. watch TV, play games, or do his homework so he can concentrate on the Valsalva. 12. Clean the outside of the catheter in running water and flush the inside of the catheter with the 60 cc catheter tip syringe to clear out any stool. Store for use again. 13. Repeat each day, at the same time. 14. If your child has accidents (soiling) in between each enema, contact the surgeon or surgical nurse. 15. If an abdominal Xray shows stool in the right colon, a high colonic enema may be needed. To do this, gently insert the catheter to its full length. The balloon is not inflated and no tension is applied when infusing the fluid. Continue this as directed by the surgical team. Recipe for normal saline solution The safest normal saline solution (isotonic; 0.9%) to use is commercially prepared and purchased from a pharmacy. Saline solution can be made at home, but if mixed incorrectly using too much or too little salt, it is dangerous and can cause dehydration, seizures, lung edema or coma. If mixing at home follow this recipe exactly: 1. 1 teaspoons of salt 2. 1 liter (or 1000 ml) tap water 3. Stir until dissolved.

Directions for antegrade enemas (ACE Malone)


The success of Antegrade Enemas (ACE Malone) is dependent on previous successful retrograde enemas. The Ace Malone is designed to facilitate easy introduction of enema fluid. It is not an alternative to failed retrograde enemas. Successful enemas are dependent on the ability to empty the rectum after the infusion of fluid. A child must know how to do a Valsalva (push/strain at stool) while relaxing his pelvic muscles and anal sphincters in order to empty his rectum for retrograde enemas to be successful. It is recommended that you keep a diary of enema details (amount of fluid and additives) and results as you get started. This will help you track changes and results more effectively. It may take several weeks of practice and adjustments to get good results. The goal is for your child to be accident free (no soiling) for 24 hours, between enemas. Gather supplies: 1. 2. 3. 4. Enema bag (Feeding bag acceptable) 12-14 silicone Mentor catheter or extension set for MIC-KEY button or Chait tube. Water soluble lubricant for catheter Saline

Getting started: 1. Fill enema bag with saline, as instructed, with 20ml/kg of saline. 2. Have your child sit on the toilet.

3. Insert the catheter into the ACE stoma or attach the extension set to the MIC-KEY button or Chait tube 4. Start the flow of saline 5. This should take between 5-10 minutes to complete 6. Raising the bag will make the flow go faster, and lowering the bag will make it go slower. Proceed at a rate that is comfortable for your child. 7. It is recommended that your child sit on the toilet for 30-45 minutes to allow all the water/stool to be pushed out. Repeatedly doing the Valsalva maneuver will shorten the waiting time. 8. Avoid letting the child do any other activity, i.e. watch TV, play games, or do his homework so he can concentrate on his Valsalva. 9. Wash the catheter in running water; flush with saline; store for use again. 10. Repeat daily at the same time. 11. If your child has accidents (soiling) in between each enema, contact the surgeon or surgical nurse. 12. The ACE Malone can also be used to instill laxatives directly into the colon. 13. Optional agents to add to the saline to improve the effectiveness of the enemas. Discuss use of these additives with the surgical nurse before starting.: o Liquid glycerin o Castille Soap

Directions for colinic irrigations


These are given through the stoma (colostomy) or anus in patients who cannot do an effective Valsalva. In contrast to enemas which are given in one instillation and evacuated, colonic irrigations require flushing the colon with saline and withdrawing the fluid several times in a row (flush and withdraw) until the return flow is clear. As such, the total volume may vary from day to day. The total volume of normal saline prepared is 20ml/kg/irrigation. Flush and withdraw in 10-20ml increments until the return flow is clear. It is not necessary to use up 20ml/kg. The end point is when the withdrawn fluid is clear. It is recommended that you keep a diary of irrigation details (total amount of saline flushed and withdrawn) and results as you get started. This will help you track changes and results more effectively. It may take several weeks of practice and adjustments to get good results. The goal is for your child to be accident free (no soiling) for 24 hours, between irrigations. Gather supplies These supplies will be ordered by the surgical nurse. Once the order is approved and filled, supplies will be sent to your home. 1. 2. 3. 4. 5. Silicone Catheter - 20-24 French with additional side holes up to middle Water soluble lubricant 60ml catheter tip syringe Basin Normal saline solution (0.9%)

Getting started: 1. Prepare 20ml/kg of normal saline as instructed.

2. Your child should be seated on the toilet for colon irrigations per anus; or on his or her back for a stoma irrigation. 3. Lubricate catheter and gently insert the whole length into anus gently. If there is any resistance, remove and reattempt gently. It should advance without resistance or discomfort. If this cannot be done without pain or resistance, stop and contact our surgical nurse for advice. 4. Do not inflate the balloon. 5. If in stoma, just go beyond the stoma surface. The last hole should be inside the stoma. 6. Using the catheter tip syringe, flush and withdraw the solution in 10-20ml increments. 7. Empty into the toilet. 8. Repeat until the fluid returns clear of stool. 9. Clean the outside of the catheter in running water and flush the inside of the catheter with 60ml catheter tip syringe to clear out any stool. Store for use again. 10. Repeat each day, at the same time. 11. If your child has accidents (soiling) in between each irrigation, contact the surgeon or surgical nurse. Recipe for normal saline solution The safest normal saline solution (isotonic; 0.9%) to use is commercially prepared and purchased from a pharmacy. Saline solution can be made at home, but if mixed incorrectly using too much or too little salt, it is dangerous and can cause dehydration, seizures, lung edema or coma. If mixing at home follow this recipe exactly: 1. 1 teaspoons salt 2. 1 liter (or 1000 ml) tap water 3. Stir until dissolved.