Sunday, April 14, 2024

Hirschsprung’s Disease Treatment Without Surgery

Hirschsprung Treatment At Washington University

Hirschsprung Disease, Colitis, and Fecal Incontinence

Treatment for Hirschsprung disease is essential for a healthy, comfortable life. Infants and children who undergo these procedures often find relief from the discomfort and complications caused by this condition.

Surgeons like Dr. Shakhsheer and others at the Division of Pediatric Surgery diagnose and treat patients from birth and older who require medical attention for HD. After surgery, the patient should follow up with a gastroenterologist to help address chronic issues related to HD.

The Pediatric Colorectal Center addresses the treatment of HD and more in young patients. Washington University Pediatric Colorectal Surgeons provide high-quality care to children with complex colorectal issues, including Hirschsprung disease, anorectal malformations, and severe chronic constipation.

Surgical Treatment Of Adult Hirschsprungs Disease

Congenital Megacolon

Among the 41 patients with congenital megacolon, 23 underwent low anterior rectal resection and terminal ileostomy, six underwent pull-out anterior rectal resection and terminal ileostomy, and 12 underwent low anterior rectal resection. Moreover, prophylactic terminal ileostomy was performed in 29 patients due to the low position of anastomosis .

Figure 1. CT image of adult congenital megacolon resected specimen of adult congenital megacolon .

Adult Idiopathic Megacolon

Figure 2. X-ray image manifestations of adult idiopathic megacolon CT image of adult idiopathic megacolon photos after colostomy 1 week after colostomy 1 month after colostomy the intestine specimen after subtotal resection .

Ganglion Cell Deficiency

Figure 3. Intraoperative photo of ganglion cell deficiency specimen of ganglion cell deficiency.

Toxic Megacolon

Iatrogenic Megacolon

Figure 4. CT image of iatrogenic megacolon caused by colonic anastomotic stenosis CT images of iatrogenic megacolon after radiotherapy for rectal cancer.

How To Prevent Hirschsprungs Disease: A Guide For Parents

Hirschsprungs disease is a rare, congenital condition that affects the large intestine. It occurs in approximately 1 out of every 5000 live births and may be more common in males than females. Because it is a rare disorder, it is often difficult to diagnose Hirschsprungs Disease early. Thats why prevention is so important. Here are some tips to help you prevent Hirschsprungs Disease from happening to your child or loved one.

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Skin Care Of Diaper Area

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.

  • Remove all butt balm cream, paste or ointment daily with mineral oil applied gently with cotton balls.
  • Soak your child’s bottom in a tub of warm water after applying the mineral oil.
  • Clean gently with soap and water, do not scrub skin. Avoid making the skin bleed.
  • At the end of the bath, pat at the skin dry.
  • Apply Cavilon 3 M No Sting Barrier Film by swab or spray to all affected skin in the diaper area.
  • Let dry for 60 seconds
  • Apply butt balm or other barrier paste over the 3 M product.
  • After each bowel movement, clean the poop off the butt balm and do not rub the butt balm from the skin.
  • Reapply the butt balm to the diaper area.
  • Repeat after each bowel movement.
  • Repeat mineral oil and bath each day.
  • Butt Balm Recipe

    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.

    What Is Hirschsprung Disease In Children


    Hirschsprung disease is a rare birth defect. It affects the nerve cells in the large intestine. These nerve cells control the muscles that move food and waste, or stool, through the large intestine. The large intestine is the last part of the digestive tract.

    Babies with Hirschsprung disease are missing nerve cells in all or part of the large intestine. In most cases, only the end parts of the colon are affected. Without these nerve cells, the muscles cant move food and waste through that part of the large intestine. Stool cant move forward. It stays in the large intestine.

    The intestine can become partly or fully blocked. It begins to grow larger than normal. This can cause constipation, swelling, pain, and infection.

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    What Are The Symptoms Of Hirschsprung Disease In A Child

    Most babies with Hirschsprung disease have symptoms in the first few weeks of life. In some cases only a short part of the intestine may be affected. Then symptoms may not be seen for a few months or years.

    Each childs symptoms may vary. Symptoms in newborns may include:

    • Not having a bowel movement in the first 48 hours of life
    • Slow swelling or bloating of the belly
    • Vomiting green or brown fluid

    Children who dont show early symptoms may also have:

    • Constipation that gets worse over time
    • Loss of appetite
    • Small, watery, bloody stools

    Symptoms of Hirschsprung disease may seem like other health problems. See your child’s healthcare provider for a diagnosis.

    How Is Hirschsprung Disease Diagnosed

    If your baby is showing symptoms of Hirschsprung disease, your pediatric gastroenterologist or pediatric surgeon may recommend one of more tests be performed to evaluate for the possibility of Hirschsprung disease:

    • Contrast enema: X-ray study where a contrast dye is given by an enema.
    • Anorectal manometry: Test where a small balloon is placed in the rectum to measure the response of the wall of the colon to pressure.
    • Biopsy: Removing a sample of colon tissue to examine the structure of the nerves.

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    How Is Hirschsprung’s Disease Diagnosed

    Most newborns have a bowel movement in the first 1 to 2 days after birth. Hirschsprung’s Disease is usually suspected when a baby does not have a bowel movement for several days following birth or has bowel movements infrequently. Babies with Hirschsprung’s disease can have a large, swollen abdomen and may vomit green liquid after feeding.

    Late diagnosis

    While most babies are diagnosed very soon after birth, some children may not be suspected to have Hirschsprung’s disease until much later in life. These children often have severe constipation, with formed stool above the narrow segment of the intestine with watery stools passing around it. They are also prone to slow weight gain. Those patients diagnosed later in life often need to undergo a staged repair with a temporary stoma as described below.

    How Will Hirschsprung Disease Affect My Baby During And After Surgery

    Hirschsprung disease. Diagnosis, treatment and preventing fecal incontinence.

    Children with Hirschsprung disease also continue to be at risk of developing bowel infection after surgery, especially in the first year. Signs and symptoms of enterocolitis can include: bleeding from the rectum, diarrhea, fever, swollen abdomen, and vomiting. You should call your child’s doctor immediately if any of these signs and symptoms occur. Your child’s doctor may recommend home rectal irrigations to prevent or treat enterocolitis.

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    What Causes Hirschsprungs Disease

    Hirschsprungs disease is a disease of the enteric nervous system , the network of nerve cells throughout the gastrointestinal tract . The gastrointestinal tract controls how the body absorbs nutrients from food, pushes food and waste along the intestine and passes stool.

    Before a child is born, the nerve cells that form the ENS develop from a group of cells called neural crest cells. While a baby develops during pregnancy, neural crest cells travel from the esophagus to the stomach, small intestine and large intestine until they fill the entire length of the digestive tract. Hirschsprungs disease occurs when the neural crest cells fail to fill the entire digestive tract. This leaves the end of the intestine aganglionic .

    Usually the aganglionic area is found in the lower end of the digestive tract. Sometimes it can extend farther up the colon and into the small intestine. The absence of nerve cells keeps the digestive tract from working properly. This means your child cannot pass stool normally.

    The Classification And Surgical Treatments In Adult Hirschsprungs Disease: A Retrospective Study

    • Department of Colorectal Surgery, Changhai Hospital, Shanghai, China

    Purpose: To explore the treatments and short-term effects of different types of adult Hirschsprungs disease.

    Methods: 89 patients treated in Shanghai Changhai Hospital were retrospectively analyzed. According to the patients medical history, clinical manifestations, auxiliary examination and postoperative pathological results, the patients were divided into adult congenital megacolon, adult idiopathic megacolon, ganglion cell deficiency , toxic megacolon and iatrogenic megacolon, The Treatment methods and short-term prognosis of patients in each group were summarized.

    Adult Hirschsprungs diseases were divided into adult congenital hirschsprungs disease, idiopathic Hirschsprungs disease, ganglion cell deficiency, toxic hirschsprungs disease, and iatrogenic Hirschsprungs disease. Different types of surgical treatments for Hirschsprungs disease in adults should be selected according to the specific diagnosis. All patients with adult Hirschsprungs diseases have good short-term outcomes after surgical treatment.

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    What To Do If You Have Symptoms

    If you or your child has symptoms of Hirschsprungs Disease, its important to see a doctor right away. The sooner the diagnosis is made, the more likely it is that your child will have a normal life expectancy and wont need any bowel surgery.

    Some of the most common symptoms of Hirschsprungs Disease are:

    • Bowel movements that are difficult to flush away
    • Having an enema before going to school
    • Straining to pass a bowel movement
    • Passing a hard stool with streaks of blood or mucus on toilet paper

    Hirschsprung Disease Symptoms In Older Babies Children And Adults

    Hirschsprung Disease :: Nationwide Children

    Not all babies with Hirschsprung disease have symptoms right away, and some people are not diagnosed until later in life. But common symptoms seen in older babies and children include:

    • chronic constipation that does not improve with taking oral laxatives
    • malnutrition, which is also known as failure to thrive for infants or children weighing less than the expected weight at their age

    Keep in mind that for older children, the above symptoms can also be caused by other conditions. So healthcare professionals may not always immediately assume that Hirschsprung disease is the cause. Adult Hirschsprung disease is rare, but symptoms in this age group are the same as those seen in older babies and children.

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    What Is The Ongoing Research On Treating Hirschsprungs Disease

    There are many ongoing areas of research for Hirschsprungs disease, including:

    • New Treatment Options
    • Development of stem cell therapy for Hirschsprungs disease. The idea is that the adult stem cells or a patients own cells can be isolated from the intestine and grown in a dish. The cells could then be transplanted into the aganglionic intestine to replace the missing nerves. Several labs around the world are working on this as a possible new therapy for Hirschsprungs disease with the goal of avoiding the need for surgery and improving long-term health for patients. Scientists are still learning whether the transplanted cells can improve how the intestines work.
  • How to Prevent or Treat Enterocolitis
  • Understand what causes enterocolitis in the first place. Researchers have found disease-causing bacteria in the intestine of children with Hirschsprungs disease. Some scientists hope probiotics might lower the risk of enterocolitis by restoring normal, healthy gut bacteria. However, researchers are still looking for evidence that shows a connection between specific groups of bacteria and the development of enterocolitis.
  • How other abnormalities in the intestines of children of Hirschsprungs disease may contribute to the development of enterocolitis. Researchers are actively studying these abnormalities as well.
  • Quality of Life of Children with Hirschsprungs Disease
  • Involvement of mental health professionals to improve childrens social and emotional well-being.
  • When Do I Call The Surgeon’s Office

    • Any concerns you have about your child’s recovery
    • A temperature of 101°F or higher
    • Increasing pain and tenderness at the incision
    • Any liquid coming out of the incision
    • A change in the number of bowel movements each day
    • No bowel movement for one day
    • Foul smelling bowel movements
    • Any questions concerning your child’s stoma

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    Differences Among Treatment Modalities

    Subsequently, we divided the patients into two groups according to the surgical methods, which is 11 patients who underwent fecal diversion and 78 intestinal continuity restoration . Then, the clinical and pathological data, and the postoperative efficacy and complications within 30 days were analyzed . Categorized by the surgical method, the preoperative gastrointestinal organic lesions , the duration of obstruction and the incidence of postoperative complications were significantly higher in the fecal diversion group than that of the intestinal continuity restoration group.

    Table 3. Differences among treatment methods.

    Living With Hirschsprungs Disease

    Hirschsprung’s Disease, Causes, Signs and Symptoms, Diagnosis and Treatment.

    Hirschsprungs disease cannot be cured on its own. It can be fatal for children who do not have surgery.

    After surgery, most children lead normal lives. They may have minor health problems because of the disease. These include diarrhea, constipation, or other problems passing stool. Some children develop an infection called enterocolitis . This can be serious, so call your doctor right away if your child has the following symptoms:

    • Delayed toilet training

    Children may need to make some lifestyle changes to help manage their condition. For instance, eating foods that are high in fiber can help reduce constipation. The large intestine absorbs water and salt that the body needs. If your child has a large portion of the intestine removed, it will absorb less. Make sure your child gets plenty of fluids and salt to make up for this.

    Your baby and older children will feel better after ostomy surgery because they will be able to pass stool easily. Adjusting to life with an ostomy pouch will take time and may make your child feel different. Your child will need to learn how to care for the stoma and how to change the ostomy pouch. With a few lifestyle changes, children with ostomies can lead normal lives. An ostomy nurse can answer questions and show your child how to care for an ostomy.

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    Reoperative Surgery For Distention And Enterocolitis

    If after a pull-through for Hirschsprung disease a patient develops repeated episodes of abdominal distention, this may mean they are not adequately emptying the stool. This can lead to a dangerous condition called enterocolitis, in which the child can get severely dehydrated. When stool does not move through the colon, stasis of stool develops, which allows the bacteria in the colon to grow which leads to diarrhea, similar to what happens in a pond as compared to a flowing stream. If this is more chronic and occurs over many years, the child’s growth can be affected, which is called failure to thrive.

    If enterocolitis occurs in repeated episodes, after a year of age, or after 6-12 months following the pull-through, the cause could be due a problem with the anatomy of the end of the pull-through or could be related to a problem with the nerves at the end of the pull-through. In such a case, assessment begins with the patients medical and surgical history, and evaluation of the anatomy under anesthesia.

    We want to find the underlying cause of the abdominal distention and figure out why the pull-through does not empty well.

    All these problems can be corrected with a redo operation, which involves a transanal removal of the end of the pull-through which is problematic and pull through of the segment of bowel above this area that is functioning well. With such a redo pull through obstructive symptoms resolve in almost all cases.

    Nonoperative Treatment Of Hirschsprung’s Disease: A New Approach**

    The approach of the combined traditional Chinese and modern medicinal treatment of Hirschsprung’s disease has been applied in 90 cases. Long-term follow-up showed satisfactory results. Of these, 55 cases with a typical history, plus a positive ACHE and x-ray were selected for analysis and discussion, making an over all rate of 78.18%. This treatment is particularly useful for those babies under 1 year of age with a short narrow segment of the rectum. In addition, it would be a good trial procedure for those babies over 1 year old with mild symptoms and the narrow segment just beyond the sigmoidorectal junction.

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    Postoperative Situation And Perioperative Safety

    All patients recovered and discharged after the operation, and no death occurred. The median length of stay for all patients was 19.5 days. Complications occurred in 10 cases , including postoperative intestinal obstruction , severe hypoproteinemia , postoperative anemia and hypovolemia , abdominal effusion , and incision infection . Thirty-one patients underwent intraoperative or postoperative blood transfusion due to a large amount of postoperative exudation, anemia, or insufficient blood volume. One case of anastomotic leakage after operation was cured after conservative treatment .

    Table 2. Surgical method and complications.

    What Can I Expect After My Child Has Surgery For Hirschsprung Disease


    Children with Hirschsprung disease most often feel better after surgery.

    About half of children may have ongoing problems after surgery.11,12 Problems may include

    If your child develops one or more of these problems after surgery, his or her doctor may recommend additional tests and treatments. In many cases, these problems improve over time with guidance from your childs doctor.

    If your child has ostomy surgery to treat Hirschsprung disease, your childs doctor and ostomy nurse will provide information and advice about recovering from surgery and caring for a stoma.

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    What Is The Life Expectancy For Someone With Hirschsprung Disease

    Because of scientific advances, its become less likely that Hirschsprung disease is a fatal condition. When diagnosed early, most infants and babies can go on to live full lives.

    But its important to know that even after surgery, its not uncommon for some people to still experience complications such as digestive upset, constipation, or even enterocolitis.

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