Materiały źródłowe

• #1 Hirschsprung’s disease – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hirschsprungs-disease/diagnosis-treatment/drc-20351561

  For most people, Hirschsprung’s disease is treated with surgery to bypass or remove the part of the colon that’s lacking nerve cells. There are two ways this can be done: a pull-through surgery or an ostomy surgery. […] In this procedure, the lining of the diseased part of the colon is stripped away. Then, the normal section is pulled through the colon from the inside and attached to the anus. This is usually done using minimally invasive (laparoscopic) methods, operating through the anus. […] In children who are very ill, surgery might be done in two steps. […] First, the abnormal portion of the colon is removed and the top, healthy portion of the colon is connected to an opening the surgeon creates in the child’s abdomen. Stool then leaves the body through the opening into a bag that attaches to the end of the intestine that protrudes through the hole in the abdomen (stoma). This allows time for the lower part of the colon to heal.

• #1 Hirschsprung’s disease – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hirschsprungs-disease/diagnosis-treatment/drc-20351561

  Once the colon has had time to heal, a second procedure is done to close the stoma and connect the healthy portion of the intestine to the rectum or anus. […] After surgery, most children are able to pass stool through the anus. […] Possible complications that may improve with time include: […] Children also continue to be at risk of developing a bowel infection (enterocolitis) after surgery, especially in the first year. Call the doctor immediately if any of the signs and symptoms of enterocolitis occur, such as:

• #2 Hirschsprung’s disease – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hirschsprungs-disease/symptoms-causes/syc-20351556

  Surgery to bypass or remove the diseased part of the colon is the treatment. […] Children who have Hirschsprung’s disease are prone to a serious intestinal infection called enterocolitis. Enterocolitis can be life-threatening and requires immediate treatment.

• #2 Hirschsprung Disease: What It Is and How It’s Treated

  https://www.healthline.com/health/hirschsprungs-disease

  For most people diagnosed with Hirschsprung disease, surgery is the standard treatment. Depending on the type of Hirschsprung disease, different procedures might be used. […] There are two main types of Hirschsprung disease surgeries: pull-through procedure (corrective surgery) or ostomy surgery. […] During a pull-through procedure, a surgeon removes the part of the large intestines that’s missing nerve cells. Then the remaining intestines are surgically connected to the rectum. This surgery is done either laparoscopically or via open surgery. […] This surgery is usually recommended for people who have more serious complications like perforations, megacolon, or Hirschsprung-associated enterocolitis. An ostomy essentially allows the intestines time to heal. The procedure may sometimes be recommended before performing a pull-through procedure. […] Currently, surgery is the only cure for Hirschsprung disease. This can include a pull-through surgery to remove the affected portions of the intestines or an ostomy to create a stoma to redirect waste out of the body.

• #2 Treatment for Hirschsprung Disease – NIDDK

  https://www.niddk.nih.gov/health-information/digestive-diseases/hirschsprung-disease/treatment

  Doctors typically treat Hirschsprung disease with a type of surgery called a pull-through procedure. […] In some cases, doctors recommend ostomy surgery of the bowel followed by a pull-through procedure. […] During a pull-through procedure, a surgeon removes the part of the large intestine that is missing nerve cells. […] Doctors may recommend that children have ostomy surgery before having a pull-through procedure, in some cases. […] Ostomy surgery allows a child’s health to improve and intestine to heal before pull-through surgery. […] After ostomy surgery, waste will leave the child’s body through the stoma. […] In most cases, surgeons can later close the stoma and connect the healthy part of the intestine to the anus. […] If your child has ostomy surgery to treat Hirschsprung disease, your child’s doctor and ostomy nurse will provide information and advice about recovering from surgery and caring for a stoma.

• #2 Treatment for Hirschsprung Disease – NIDDK

  https://www.niddk.nih.gov/health-information/digestive-diseases/hirschsprung-disease/treatment

  Doctors may recommend treating mild Hirschsprung-associated enterocolitis at home with oral antibiotics and oral rehydration solutions, such as Pedialyte, Naturalyte, Infalyte, and CeraLyte. […] Doctors typically treat more severe Hirschsprung-associated enterocolitis in the hospital. […] If enterocolitis doesn’t improve with other treatments, doctors may recommend ostomy surgery. […] If a child with Hirschsprung disease has other complications such as megacolon or perforation, doctors may recommend ostomy surgery.

• #3 Hirschsprung’s Disease: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/9844-hirschsprung-disease

  Theres no cure for Hirschsprungs disease, but surgical treatment often results in a positive outcome. There are two types of surgeries to treat Hirschsprungs disease: a pull-through procedure and an ostomy. […] A surgeon removes the section of your babys large intestine thats missing nerve cells. Then they connect the healthy part of your babys large intestine to their anus. The surgeon may use laparoscopic or traditional surgery to perform the pull-through procedure. The pull-through procedure is the most common surgery for Hirschsprungs disease and has the best outcome for recovery. […] Your baby may need a colostomy (large intestine) or ileostomy (small intestine) before, or at the same time as, a pull-through procedure. […] Some nonsurgical treatments work well in addition to surgery. They include: Bowel management: A routine involving medicines and/or enemas to make sure your childs pooping habits are healthy. Sacral nerve stimulation: A surgeon inserts a tiny device near your lower spine to control when you pee and poop. Biofeedback: Therapy that involves learning strategies to have more control over involuntary bodily functions in this case, pooping.

• #3 Hirschsprung’s Disease: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/9844-hirschsprung-disease

  If everything goes well, your baby will feel much better a few days after surgery. […] After surgery, some babies with Hirschsprungs disease may still have constipation, pooping accidents and colon infections. But with long-term follow-up care, most children can manage pooping without becoming severely constipated or developing fecal incontinence. […] Treatments that can help your child manage Hirschsprungs disease after surgery include: Nutritional guidance. Regular care from a gastroenterologist and dedicated nurse. Biofeedback / pelvic floor rehabilitation. Therapy from a psychologist or social worker.

• #4 Hirschsprung Disease (HD): Symptoms, Diagnosis and Treatment Options | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/hirschsprung-disease

  The goals of surgery are to remove the abnormal part of the intestine, and to reconnect a healthy part of the intestine to the anus so that the body can pass waste normally. In most cases, the rectum and the sigmoid colon (the last parts of the large intestine) will need to be removed. […] Surgery is typically performed on a healthy infant that has responded well to bowel irrigations. The operation performed while your child is under anesthesia involves both laparoscopic and transanal surgery. This allows the surgeon to reach the bowel through three or four tiny (3-5 mm) incisions on the abdomen (stomach area). This approach is minimally invasive and helps reduce recovery time. […] In rare cases, if a child is very ill from enterocolitis (intestinal infection), and irrigations are not working, an ileostomy or colostomy is performed. These ostomy procedures collect waste from the small or large intestines and channel it into a bag attached to the child’s side. This is a temporary measure (lasting from a few months to a few years) which allows the child’s intestines to heal until surgery can be performed.

• #4 Hirschsprung Disease (HD): Symptoms, Diagnosis and Treatment Options | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/hirschsprung-disease

  Children with Hirschsprung disease will always need surgery to remove the non-functional segment of the intestine, and to restore the ability to push stool through the digestive tract and out of the body. With proper surgical intervention, the long-term outcomes for children with Hirschsprung disease are excellent, and most will go on to recover normal bowel control and function. […] Treatment for Hirschsprung disease depends on the severity of the disease and your child’s overall health. All children with the condition undergo procedures that help eliminate static stool, and ultimately, surgery to remove the portion of the intestine that is malfunctioning. […] Most children who have treatment and surgery do very well and go on to have normal, healthy bowel function. […] As a first step, doctors will irrigate the bowel to remove trapped waste and gas. The irrigation is done by inserting a tube through the anus to flush out the colon. In most cases, this successfully clears the intestines and provides immediate relief to the child. These irrigations are continued until surgery can be done.

• #4 Hirschsprung Disease (HD): Symptoms, Diagnosis and Treatment Options | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/hirschsprung-disease

  While surgical outcomes are generally excellent, intestinal infections can occur even after surgery. This type of infection is called enterocolitis. […] Constipation is another potential complication but can be managed with diet and laxatives. […] 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. […] 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.

• #5 Hirschsprung’s Disease: Diagnosis and Management | AAFP

  https://www.aafp.org/pubs/afp/issues/2006/1015/p1319.html

  Hirschsprungs disease should be confirmed using rectal suction biopsy. Surgery is the recommended treatment for patients with Hirschsprungs disease. […] After Hirschsprungs disease is diagnosed, surgery usually is needed. Before surgery, serial rectal irrigation helps decompress the bowel and prevent enterocolitis. In otherwise healthy newborns with undistended colons and short-segment Hirschsprungs disease, the definitive ileoanal pull-through anastomosis can be performed. If the child has Hirschsprungs-associated enterocolitis or a significantly dilated colon, a colostomy can be placed for several months while the child recovers; the pull-through procedure usually is performed four to six months after colostomy placement. […] There are several pull-through techniques, with complication rates ranging from 4 to 16 percent. Swensons operation involves removing the rectum, pulling the healthy ganglionated colon through, and connecting it to the anus. Newer techniques (e.g., Duhamel operation, Soave operation) help preserve the intricate nerve supply to the rectum and bladder. All of these procedures have high success rates, and morbidity is minimal. […] Some surgeons perform a one-stage transanal Soave operation in newborns with short-segment disease, eliminating the need for an abdominal incision and colostomy.

• #6 Hirschsprung Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/178493-treatment

  In 2019, the APSA Hirschsprung Disease Interest Group released guidelines for managing postoperative soiling in children with Hirschsprung disease. […] Management includes a combination of bowel management, dietary changes, and laxatives. If indicated, a redo operation may be considered. […] The Swenson procedure was the original pull-through procedure used to treat Hirschsprung disease. […] The Duhamel procedure was first described in 1956 as a modification to the Swenson procedure. […] The Soave procedure was introduced in the 1960s. […] For patients with extremely short-segment Hirschsprung disease, anorectal myomectomy is an alternative surgical option. […] Patients with total colonic involvement require modified procedures to exclude the aganglionic colon while preserving maximal absorptive epithelium.

• #6 Hirschsprung Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/178493-treatment

  A laparoscopic approach to the surgical treatment of Hirschsprung disease was first described in 1999 by Georgeson. […] Transanal pull-through procedures have been described in which no intra-abdominal dissection is performed. […] Several other creative approaches have been described, including a modification of the transanal approach with transabdominal open or laparoscopic assistance, single-incision laparoscopic endorectal pull-through (SILEP), and natural orifice transluminal endoscopic surgery (NOTES). […] Regenerative strategies are under investigation to restore function in the aganglionic intestine.

• #6 Hirschsprung Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/178493-treatment

  If Hirschsprung disease is suspected, neonates and children should be assigned to a center where pediatric specialists are available to make the diagnosis and to provide definitive care. […] Consult with pediatric surgeons and pediatric gastroenterologists. Genetic consultation may be indicated (if a heritable or chromosomal anomaly is suspected). […] The general goals of medical care are three-fold: (1) to treat the manifestations and complications of untreated Hirschsprung disease, (2) to institute temporizing measures until definitive reconstructive surgery, and (3) to manage the postoperative bowel function. […] The objectives of medical care are to maintain normal fluid and electrolyte balance, to minimize bowel distention and prevent perforation, and to manage complications. […] Postoperatively, routine colonic irrigation and prophylactic antibiotic therapy may decrease the risk of developing enterocolitis.

• #6 Hirschsprung Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/178493-treatment

  For patients who do develop enterocolitis, nasogastric decompression, intravenous fluids, antibiotics, and colonic lavage may be necessary. […] Sodium cromoglycate, a mast cell stabilizer, has also been reported to benefit these patients. […] Botulinum toxin injections within the contracted internal sphincter mechanism have been reported to induce more normal patterns of bowel movements in postoperative patients with obstruction or enterocolitis. […] In 2017, the American Pediatric Surgical Association (APSA) Hirschsprung Disease Interest Group released guidelines for managing Hirschsprung-associated enterocolitis. […] In general, treatment of enterocolitis consists of intravenous antibiotics and aggressive colonic irrigations. […] Some experts advocate decompression of the bowel, especially in patients with long-segment disease, by placing an enterostomy proximal to the transition zone.

• #6 Hirschsprung Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/178493-treatment

  A number of definitive procedures have demonstrated excellent results when performed by experienced surgeons. […] The most commonly performed repairs are the Swenson, Duhamel, and Soave procedures. […] In any elective operation for Hirschsprung disease, a robust preoperative colon cleansing must be performed. […] Intraoperatively, histological examination of a frozen-section biopsy must confirm the presence of ganglion cells at the proximal margin of bowel intended for anastomosis. […] However, long-term complications of pull-through procedures may include intermittent enterocolitis, severe stool retention, as well as intestinal obstruction. […] In 2017, the American Pediatric Surgical Association (APSA) Hirschsprung Disease Interest Group released guidelines for managing postoperative obstructive symptoms that include an algorithm to aid in the diagnosis and treatment of such symptoms following pull-through procedures.

• #7 Hirschsprung Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562142/

  The diagnosis of Hirschsprung disease (HD) almost exclusively demands surgical intervention. […] Surgical planning is profoundly affected by the presence of comorbidities, while short-segment HD without any comorbidities can be subjected to a single-stage pull-through procedure. […] In contrast, in the presence of HAEC or a remarkably dilated colon, a staged reconstruction, starting with a temporary decompressive colostomy, should be preferred. […] The recommended timing for a definite pull-through procedure varies from four to six months following colostomy placement. […] A variety of pull-through surgeries have been identified. The traditional Swenson’s technique involves proctectomy, pulling the healthy ganglionated colon through, and anastomosing it to the anus. […] Novel surgical procedures (e.g., Duhamel’s and Soave’s procedures) have the advantage of preserving the intricate innervation of the rectum and bladder.

• #8 Hirschsprung’s disease – Wikipedia

  https://en.wikipedia.org/wiki/Hirschsprung%27s_disease

  Treatment is generally by surgery to remove the affected section of bowel. […] The surgical procedure most often carried out is known as a „pull through”. […] Currently, several different surgical approaches are used, which include the Swenson, Soave, Duhamel, and Boley procedures. […] The pull-through procedure repairs the colon by connecting the functioning portion of the bowel to the anus. […] For the 15% of children who do not obtain full bowel control, other treatments are available. […] A lack of bowel control may be addressed by an ileostomy similar to a colostomy, but uses the end of the small intestine rather than the colon. […] The Malone antegrade colonic enema (ACE) is also an option. […] If the affected portion of the lower intestine is restricted to the lower portion of the rectum, other surgical procedures may be performed, such as a posterior rectal myectomy.

• #9 Hirschsprung Disease (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/hirschsprung.html

  Treatment always requires surgery. Fortunately, most children who have surgery are able to pass bowel movements (BMs) normally. […] Surgery is thought to be the most effective treatment for Hirschsprung disease. This is done in one step or two, depending on how severe it is. Children who are very sick at the time of surgery (from an inflamed intestine or poor nutrition) may need to undergo surgery in two steps. […] The most common surgery to correct Hirschsprung disease involves removing the section of the intestine without nerves and reattaching the remaining section to the rectum. Often, this can be done in one step through minimally invasive (laparoscopic) surgery right after the condition is diagnosed. […] In some cases, the doctor may do the surgery in two steps. […] In the first surgery: The doctor will find the piece of intestine that has healthy nerves by doing biopsies of the intestine in surgery. Then, in a procedure called an ostomy, the doctor creates a small hole, or stoma, in the child’s abdomen and attaches the upper, healthy section of the intestine to the hole.

• #9 Hirschsprung Disease (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/hirschsprung.html

  The child’s stool passes through the stoma into a bag that is connected to it, which needs to be emptied several times a day. This allows the child to recuperate from enterocolitis or improve their nutrition before the second surgery. […] In the second surgery: The doctor closes the hole and attaches the normal section of the colon to the rectum. […] Most children treated surgically for Hirschsprung disease have an excellent outcome. Most can pass stool normally and have no lasting problems. A few kids might continue to have symptoms, including constipation and bowel control problems.

• #10 Hirschsprung’s Disease Treatment | Bladder & Bowel Community

  https://www.bladderandbowel.org/bowel/bowel-treatments/hirschsprungs-disease-treatment/

  Some children will undergo a staged repair to allow the bowel to heal before it is joined up to the anus. In this case, the first operation will consist of a stoma being formed on the abdomen (either a colostomy or ileostomy), followed by a second operation a few month later to perform the pull-through operation described above. […] In instances of severe constipation, children can undergo a procedure called ACE (Antegrade Colonic Enema). […] This procedure involves creating a continent stoma from usually the appendix but can also be from the small bowel. An small artificial opening in created low down in the abdomen or through the belly button. A catheter can be inserted into the stoma and a washout solution injected to carry out a controlled bowel movement.

• #11 Hirschsprung Disease- Treatment, Symptoms, Diagnosis, & Causes

  https://www.impactguru.com/info/hirschsprung-disease/

  Ostomy surgery: This is sometimes done before or instead of a pull-through procedure, especially if the patient has complications such as severe infection, megacolon, or perforation. […] Bowel resection with colostomy: This is another option for patients with a large portion of their colon affected by Hirschsprung disease. […] After surgery, most patients can pass stool through their anus and have a normal quality of life. However, some patients may have complications or ongoing problems, such as: […] Hirschsprung disease is a severe but treatable condition that affects the colon. Most patients can have normal bowel function quality of life with proper diagnosis and treatment. […] Treatment options for Hirschsprung disease typically involve surgery to extract the affected part of the colon and reconnect the healthy segments. Postoperative care and management may include dietary adjustments and medications to address residual issues.

• #12 Hirschsprung’s Disease – About Constipation

  https://aboutconstipation.org/living-with-constipation/hirschsprung-s-disease/

  The treatment of Hirschsprungs disease is primarily surgical. The goal is to remove the abnormal bowel and attach the normal bowel to the anus just above the sphincter. The three commonly performed operations, known as pullthrough procedures, are called the Swenson, Duhamel, and Soave operations. The long-term results appear to be similar whichever operation is used. […] In the past 10-15 years, more surgeons have been performing a single-stage repair. Recently, less invasive operations using laparoscopy or a transanal incisionless approach have been introduced, with shorter hospital stays, less pain, and less scarring. […] Although most children have excellent results following surgery for Hirschsprungs disease, approximately 10-20% experience continued defecatory problems in the form of enterocolitis, incontinence, and persistent constipation.

• #13 Hirschsprung’s Disease | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/hirschsprung-s-disease

  Definitive treatment for Hirschsprungs disease is the surgical removal of the portion of the bowel without ganglion cells. Surgical correction may take up to three stages. […] Treatment for Hirschsprungs disease varies based on each child’s symptoms and overall health. Your child’s treatment plan may include one or several of the following steps. […] Rectal irrigation is used to help your child defecate. A soft catheter (tube) is inserted through the anus into the rectum and the doctor flushes the rectum and intestine with saline solution in order to remove stool. This can be performed at the bedside. This may be used until surgery is performed and sometimes as a treatment afterward. […] Depending on the overall health of your child, a surgeon may decide they need a stoma a surgically created opening in the bowel, also called an ostomy. The surgeon creates a small hole in the abdomen and brings a small portion of the intestine up to the skin. This allows stool to come out easily into a small bag.

• #13 Hirschsprung’s Disease | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/hirschsprung-s-disease

  In a pull-through procedure, a surgeon removes the abnormal section of the intestine and connects (pulls through) the healthy intestine with ganglion cells to replace it and connect to the rectum just above the anal sphincter. […] After the pull-through procedure, your child will be able to go home when they are eating well, are passing stool and have no fever. Initially, your child will poop frequently. It is important to protect their skin with barrier creams. […] After your child undergoes a procedure, they will return for a follow-up appointment with their surgeon. […] The Pediatric Colorectal Program will continue to follow your child’s progress closely after surgery. Your initial appointment following surgery will occur about two weeks after discharge and then monthly as directed by your surgeon. […] If your child continues to experience problems with bowel function or fecal incontinence, we will work with them on a long-term bowel management program and supplemental treatment that may include medications or a therapeutic diet.

• #14 Hirschsprung Disease: What You Need to Know

  https://www.massgeneral.org/children/hirschsprung-disease

  Treatment for Hirschsprungs disease starts with medical treatment that leads up to surgery (called pull-through surgery) to remove the aganglionic bowel. Early diagnosis and starting medical treatment as soon as possible are important to keep your child healthy and prevent complications. […] Once doctors confirm that your child has Hirschsprungs disease, they will begin treatment with anal dilations and/or rectal irrigations. These are important ways of decompressing (emptying) the colon and helping your child pass stool. The pediatric surgeon will teach you how to do this at home with your child. […] Your child’s pediatric surgeon will help you decide when they can perform pull-through surgery. In this surgery, the aganglionic part of your child’s intestine is removed and the healthy section of the intestine (the part with normal nerve cells) is attached to the rectum. Most babies with Hirschsprungs disease have surgery within the first several months of age.

• #14 Hirschsprung Disease: What You Need to Know

  https://www.massgeneral.org/children/hirschsprung-disease

  After surgery, your child’s care team can help with pain control and monitor for stool output (how much stool your child produces and passes). They can also help with feeding once your child’s digestive tract starts to work again. […] Enterocolitis must be treated promptly. If the enterocolitis is mild, treatment includes antibiotics and rectal irrigation. More severe cases require admission to the hospital for rectal irrigations, IV antibiotics and IV fluids. […] Development of stem cell therapy for Hirschsprungs disease. The idea is that the adult stem cells or a patient’s 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.

• #15 Adult Hirschsprung’s disease presenting as chronic constipation: a case report | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-023-03986-y

  Hirschsprungs disease in adults is usually a short or ultra-short aganglionic segment because it shows relatively mild symptoms. […] Surgical removal of the aganglionic segment of the gut is the definitive treatment for Hirschsprungs disease. […] Initial treatment to decompress the abdomen was necessary to alleviate the symptoms, using nasogastric tube and rectal washout using laxative agents. However, the definitive treatment of surgery using one of the techniques stated above has to be done. […] The surgical approach for Hirschsprungs disease is determined by the length of the achalasic zone, the length and reversibility of colonic dilation, the nutritional status of the patient, and the experience of the operator. […] Surgical treatment was done, and histological findings showed aganglionic part of sigmoid, thus confirming the diagnosis of Hirschsprungs disease. […] Intravenous fluid resuscitation, decompression method with nasogastric tube insertion and rectal washouts, along with antibiotic treatment when indicated, for example when perforation of abdominal organs is suspected, are the initial treatments for adult Hirschsprungs disease.

• #16

• #17 Hirschsprung Disease in Children | Children’s Mercy Kansas City

  https://www.childrensmercy.org/departments-and-clinics/colorectal-center/hirschsprung-disease/

  Hirschsprung disease can cause your child to have a backup of stool and gas in their colon. This can make them ill and struggle to have a bowel movement. However, there are a few steps you can take that will help keep your child’s colon clean. […] Rectal irrigation can get rid of waste and keep the child’s colon healthy until surgery can be performed. Irrigation is done by inserting a tube into the child’s anal opening and flushing the colon out slowly. This can get rid of the waste and gas that are making the child sick and provides immediate relief. […] While rectal irrigation may provide temporary relief, the primary treatment for Hirschsprung disease is surgery to remove the portion of the colon without any nerve cells. This is called a pull-through procedure, or the Swenson procedure.

• #18 Hirschsprung’s disease | Altru Health System

  https://www.altru.org/health-library/conditions/hirschsprungs-disease

  Once the colon has had time to heal, a second procedure is done to close the stoma and connect the healthy portion of the intestine to the rectum or anus. […] After surgery, most children are able to pass stool through the anus. […] Possible complications that may improve with time include: Diarrhea, Constipation, Leaking stool (fecal incontinence), Delays in toilet training. […] Children also continue to be at risk of developing a bowel infection (enterocolitis) after surgery, especially in the first year. Call the doctor immediately if any of the signs and symptoms of enterocolitis occur, such as: Bleeding from the rectum, Diarrhea, Fever, Swollen abdomen, Vomiting.

• #19 Hirschsprung’s Disease | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/h/hirschsprung

  Children with Hirschsprungs disease will need rectal irrigations when they are first diagnosed to help prevent enterocolitis. Irrigations will remove trapped stool (poop) and gas. Parents and caregivers will be taught to do rectal irrigations, as they may be needed after surgery to treat enterocolitis. […] After surgery, children are still at risk for enterocolitis. But, with successful surgery and long-term specialized follow-up care, most children can achieve normal bowel habits and an excellent quality of life.

• #20 Hirschsprung’s Disease Treatment & Care | Dayton Children’s Hospital

  https://www.childrensdayton.org/patients-visitors/services/pediatric-surgery/conditions-we-treat/hirschsprung-disease

  Some children continue to experience constipation after their surgery and may need to make modifications to their lifestyle and diet to encourage more comfortable and regular bowel movements. If your child continues to experience constipation after surgery, discuss with your pediatric gastroenterologist or pediatric surgeon whether to try any of the following: Serve high-fiber foods, Increase fluids, Encourage physical activity, Laxatives (only as directed by your child’s doctor).

• #21 Hirschsprung’s Disease | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/hirschsprungs-disease

  Our team of doctors will then collaborate on a treatment plan that may include: […] Medications to either slow the transit of the stool in the colon or to make it faster […] Injections of Botulinum toxin (Botox) into the anal sphincter to relax this muscle […] Other rectal interventions or surgical procedures that may include redoing previous operations […] Further removal of abnormal intestine […] Appendicostomy, a surgical procedure to flush out and empty the colon by creating an opening outside the belly […] Bowel management program. […] After surgery, your child will be followed by a gastroenterologist to help address chronic constipation and other possible consequences of Hirschsprung’s disease.

• #22 Hirschsprung Disease Treatment in Delhi, India | Symptoms & Causes | Max Hospital

  https://www.maxhealthcare.in/our-specialities/paediatric/conditions-treatments/hirschsprung-s-disease

  Certain non-surgical treatments can support recovery and improve bowel function after surgery. These include: Bowel management A structured plan using medications and/or enemas to regulate bowel movements and prevent complications like constipation or soiling. Sacral nerve stimulation A small device is implanted near the lower spine to help control bowel and bladder function by stimulating the nerves. Biofeedback therapy A training technique that helps children develop better control over bowel movements by improving coordination of the pelvic muscles. These treatments can be beneficial in managing symptoms and improving quality of life, especially for children experiencing ongoing digestive issues after surgery.

• #23 Understanding Hirschsprung’s Disease: A Guide For Pelvic Floor Therapists – Kids Bowel Bladder

  https://kidsbowelbladder.com/hirschsprungs-disease-management-and-treatment-in-pediatric-therapy/

  Bowel retraining programs help your children establish regular bowel habits. You can create individualized bowel schedules to train the child in effective muscle coordination during defecation. This will help reduce straining and minimize constipation while promoting healthy bowel routines. […] Pelvic floor training is important in children dealing with hypotonic or hypertonic muscles. Strengthening weak pelvic floor muscles can improve continence, while relaxation training can help children with hypertonic muscles learn to release tension. Biofeedback is particularly useful for pelvic floor training because it helps kids to visualize the functional contraction and relaxation of their muscles. […] Children with Hirschsprung’s disease often benefit from sensory integration techniques. These techniques help improve their awareness of signals in their stomach and bowels, teaching kids to recognize the sensation of stool in the rectum and appropriate times to initiate defecation.

• #23 Understanding Hirschsprung’s Disease: A Guide For Pelvic Floor Therapists – Kids Bowel Bladder

  https://kidsbowelbladder.com/hirschsprungs-disease-management-and-treatment-in-pediatric-therapy/

  You can support bowel health through recommendations for high-fiber diets, proper hydration, and using stool softeners or laxatives when indicated. Collaboration with pediatric gastroenterologists, dieticians and functional medicine doctors can also be beneficial when addressing diet and restoring a health microbiome. […] As a pediatric pelvic floor therapist, you can provide invaluable guidance on managing expectations and uncertainty. By teaching parents and caregivers to be proactive, you help empower them to support their child through the confusing, frustrating, and scary process of managing Hirschsprung’s disease.

• #23 Understanding Hirschsprung’s Disease: A Guide For Pelvic Floor Therapists – Kids Bowel Bladder

  https://kidsbowelbladder.com/hirschsprungs-disease-management-and-treatment-in-pediatric-therapy/

  Fecal incontinence and soiling as the repaired bowel may struggle with coordinated muscle function. Children with more complex anorectal malformations or spinal cord disorders will have further issues. […] Pelvic floor dysfunction, including difficulty coordinating relaxation and contraction of the pelvic floor muscles. […] Enterocolitis before and/or after surgery, presenting as pain, fever, nausea/vomiting. […] Colostomy or additional surgery for further bowel management. […] These complications present many opportunities for you to contribute to both short-term recovery and long-term bowel management in these children. […] For children with Hirschsprung’s disease, pelvic floor dysfunction can exacerbate symptoms of constipation and fecal incontinence. […] As a pediatric pelvic floor therapist, your interventions play a vital role in managing Hirschsprung’s disease and improving the quality of life for these children.

• #24 Stem cell-based therapy for hirschsprung disease, do we have the guts to treat? | Gene Therapy

  https://www.nature.com/articles/s41434-021-00268-4

  Progress in regenerative medicine offers cell-based solutions to the problem of ENS dysfunction, which extend beyond HSCR into other enteric neuropathies such as esophageal achalasia and gastroparesis. […] The principle of the long-sought HSCR cell-based therapy is the reestablishment of a functional ENS in the aganglionic segment by harnessing the proliferative potential of ENCC. […] The envisaged therapeutic approaches could be categorized according to the source of ENCC: induced pluripotent stem cell (iPSC), embryonic stem cell (ESC), or native ENCC. […] With regards to ESC-derived ENCC, because the cell source would be the inner mass of a healthy human preimplantation embryo (non autologous), the treatment steps would only include differentiation and direct transplantation, bypassing the need for mutation correction.

• #24 Stem cell-based therapy for hirschsprung disease, do we have the guts to treat? | Gene Therapy

  https://www.nature.com/articles/s41434-021-00268-4

  Hirschsprung disease (HSCR) is a congenital anomaly of the colon that results from failure of enteric nervous system formation, leading to a constricted dysfunctional segment of the colon with variable lengths, and necessitating surgical intervention. […] Despite the high success rate of the curative surgeries, they are associated with significant adverse outcomes such as enterocolitis, fecal soiling, and chronic constipation. […] The mainstay of HSCR treatment is resection of the aganglionic segment and anastomosis of the healthy end of the colon with the anus though pull-through procedures. […] The management of HSCR depends on the clinical presentation and the extent of aganglionosis. […] Although the overall treatment success rate is high (mortality rate 5%), HSCR is associated with significant morbidity.

• #25 Hirschsprung’s Disease—Recent Understanding of Embryonic Aspects, Etiopathogenesis and Future Treatment Avenues

  https://www.mdpi.com/1648-9144/56/11/611

  Hirschsprung’s disease is a neurocristopathy, caused by defective migration, proliferation, differentiation and survival of neural crest cells, leading to gut aganglionosis. […] The treatment of Hirschsprung’s disease is usually surgical resection of the aganglionic part of the intestine, however, as many as 30–50% of patients experience persisting symptoms. […] The surgical management of HSCR has improved considerably over recent decades. […] The development of modern approaches such as single-stage trans-anal pull through has ensured milder scarring after surgery, superior pain alleviation, reduced hospitalisation time, as well as better safety and effectiveness. […] Therefore, highly promising future prospects are focused on stem cell research and tissue engineering. […] All three scenarios proved to be viable.

• #26 Hirschsprung Disease | Rady Children’s Hospital

  https://www.rchsd.org/health-article/hirschsprung-disease

  The two types of ostomy are: Ileostomy: removing the entire large intestine and connecting the small intestine to the stoma; Colostomy: removing just a section of the colon. […] After surgery, kids often get constipated. Laxatives can offer some relief, but check with your doctor about which would be best for your child. […] Most children treated surgically for Hirschsprung disease have an excellent outcome. Most can pass stool normally and have no lasting problems. A few kids might continue to have symptoms, including constipation and bowel control problems.

• #27 Hirschsprung Disease | Conditions | Penn State Health

  https://www.pennstatehealth.org/childrens/services-treatments/hirschsprung-disease

  Surgical treatment improves or eliminates symptoms of Hirschsprung disease for most children. […] A small number may have constipation issues or experience fecal incontinence. […] Children who are treated early or who have a shorter segment of bowel involved tend to have the best outcomes.

• #28 Frequently Asked Questions about Hirschsprung’s Disease – About Kids GI

  https://aboutkidsgi.org/lower-gi/hirschsprungs-disease/frequently-asked-questions-about-hirschsprungs-disease/

  Follow-up visits with your surgeon are important so that progress may be monitored and possible complications identified and dealt with. […] Overall, about 90% of children with Hirschsprungs have no major complications or difficulties. Of the 10% who do have problems, most eventually get better with help from their doctor and other health professionals and go on to live a perfectly normal life.

• #29 Hirschsprung’s Disease: Causes, Symptoms, Diagnosis, Treatment

  https://www.webmd.com/children/what-is-hirschsprungs-disease

  After surgery, most children with Hirschsprung’s disease can poop normally and regain control over their bowels. Others continue to have bowel issues such as incontinence and GI symptoms as they get older, and they may need more surgeries. Hirschsprung’s is a lifelong condition that needs regular monitoring. […] The main treatment is surgery to remove the part of the intestine with the missing nerve cells. After surgery, many children with Hirschsprung’s are able to have normal bowel movements.

Zobacz więcej