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Hirschsprung diseaseHHirschsprung diseaseHirschsprung diseaseEnglishGastrointestinalChild (0-12 years);Teen (13-18 years)Large Intestine/ColonLarge intestineConditions and diseasesCaregivers Adult (19+)NA2014-09-19T04:00:00ZKimberly Colapinto RN (EC), MN;Jacob Langer, MD;Division of General and Thoracic Surgery8.0000000000000064.00000000000001312.00000000000Health (A-Z) - ConditionsHealth A-Z<p>Learn how Hirschprung disease affects the bowel and how it is diagnosed and usually treated.</p><h2>What is Hirschsprung disease?</h2> <p>Hirschsprung disease is a condition in which special nerve cells (ganglion cells) are missing from the bowel or colon. For this reason, the disease is also known as aganglionic megacolon. When these cells are missing, stool (poo) does not move properly. This can cause a blockage and make your child very sick. The condition is named after Dr. Harald Hirschsprung.</p> <p>Hirschsprung disease is a congenital disease. This means that a person is born with it. It occurs in one in every 5,000 births and is more common in boys than girls. Sometimes it is hereditary, meaning that a parent can pass it on to a child.</p><h2>Key points</h2> <ul> <li>Hirschsprung disease is a condition in which nerve cells are missing from part of the bowel. This can cause stool to become blocked and make a child very sick.</li> <li>The usual treatment for Hirschsprung disease is "pull-through" surgery to remove the part of the bowel without nerve cells and allow the rest of the bowel to work normally. Some children might need an <a href="https://akhpub.aboutkidshealth.ca/article?contentid=1027&language=English">ostomy</a> before pull-through surgery.</li> <li>After surgery, apply ointment to ease diaper rash and follow the surgical team's instructions for giving pain medicines and returning for follow-up appointments. </li> <li>Bowel infections can occur after surgery. Contact your surgeon if your child has watery or foul-smelling diarrhea, a swollen belly or fever or is vomiting.</li> </ul><h2>How is Hirschsprung disease diagnosed?</h2> <p>Hirschsprung disease is usually diagnosed in newborn babies, but it can sometimes be diagnosed in an older child who has problems with severe <a href="/Article?contentid=6&language=English">constipation</a>.</p> <p>The diagnosis can be made in several steps.</p> <ol> <li>Your doctor will examine your child and take their medical history.</li> <li>Your child may have a special X-ray called a contrast enema that will help the doctor see what your child's bowel looks like.</li> <li>Your doctor will recommend a rectal biopsy. This involves taking a tiny piece of the inside of the bowel and studying it to see if it has any nerve cells.</li> <li>Sometimes your child will have another test called manometry. The test checks if your child's sphincter muscles (the muscles that help hold in stool) are working normally. To do the test, a small flat balloon is placed into your child's bottom. Once inside, the balloon is filled with air. If the sphincter is not working normally, it could mean that your child has Hirschsprung disease.</li> </ol><h2>How is Hirschsprung disease treated?</h2> <p>Your child will need surgery (an operation) to repair their bowel. The type of surgery they have depends on how much of their bowel is affected by Hirschsprung disease.</p> <p>The most common surgery to correct this is called a "pull-through". This involves removing the part of the bowel that does not have nerve cells and attaching the normal bowel just above the anus.</p> <p>Some children will first need surgery to create an <a href="https://akhpub.aboutkidshealth.ca/article?contentid=1027&language=English">ostomy</a> before the pull-through surgery. This is an opening created from the inside of the body to the outside, on the abdomen (tummy). Stool passes through this opening and is collected in a pouch attached to your child's abdomen.</p> <p>This ostomy is usually only temporary, but, rarely, it can be permanent. How long your child has the ostomy depends on how much of their bowel is affected by Hirschsprung disease.</p> <p>After the ostomy is made, your child's surgeon will discuss which type of surgery to correct Hirschsprung disease will be right for your child and when it should happen.</p> <p>Whichever type of surgery your child has, the goal is always to remove the part of the bowel that does not have nerve cells and allow the remaining bowel to work again.</p> <h2>Can Hirschsprung disease cause any other problems?</h2> <p>All children with Hirschsprung disease are at risk for Hirschsprung enterocolitis before and after surgery. This is a bowel infection that can become life threatening.</p> <p>Contact your surgeon if your child:</p> <ul> <li>has watery, foul smelling or explosive <a href="/Article?contentid=7&language=English">diarrhea</a></li> <li>has abdominal distention (swollen belly)</li> <li>has a <a href="https://akhpub.aboutkidshealth.ca/article?contentid=30&language=English">fever</a><br></li> <li>is <a href="/Article?contentid=746&language=English">vomiting</a>.</li> </ul> <p>Enterocolitis can be different in every child: some children will only have one symptom and others might have all of them. Always talk to your child's surgeon if you feel unsure. If you cannot contact your surgeon within a few hours, bring your child to the emergency department.</p> <p>If you see a paediatrician for an illness that has vomiting and diarrhea as a symptom, remind them that your child has Hirschsprung disease.</p>
Maladie de HirschsprungMMaladie de HirschsprungHirschsprung diseaseFrenchGastrointestinalChild (0-12 years);Teen (13-18 years)Large Intestine/ColonLarge intestineConditions and diseasesAdult (19+) CaregiversNA2014-09-19T04:00:00ZKimberly Colapinto RN (EC), MN;Jacob Langer, MD;Division of General and Thoracic SurgeryHealth (A-Z) - ConditionsHealth A-Z<p>Découvrez comment la maladie de Hirschsprung endommage les intestins, et prenez connaissance de son diagnostic et de son traitement usuel.</p><h2>Qu’est-ce que la maladie de Hirschsprung?</h2> as correctement, ce qui peut entraîner de la constipation chez votre enfant et le rendre très malade. Ce trouble a reçu le nom du Dr Harald Hirschsprung.<p></p> <p>La maladie de Hirschsprung est un trouble congénital. Autrement dit, le patient l’a déjà à la naissance. On la rencontre chez une personne sur 5 000. Elle est plus répandue chez les garçons que chez les filles. Elle peut être héréditaire : les parents peuvent la transmettre à leurs enfants.<br></p> <h2>À retenir</h2><ul><li>La maladie de Hirschsprung est caractérisée par l’absence de neurones dans certaines parties des intestins, ce qui peut causer de la constipation chez l’enfant et le rendre très malade.</li><li>Le traitement habituel consiste à enlever de manière chirurgicale la partie des intestins dépourvue de neurones et à laisser les autres parties fonctionner normalement. Certains enfants peuvent avoir besoin qu’on pratique une <a href="/article?contentid=1027&language=French">stomie</a> avant l’opération.</li><li>Après l’intervention, appliquez un onguent pour soulager l’érythème papulo-érosif (rougeur des fesses) et suivez les instructions de l’équipe chirurgicale quant à l’administration d’analgésiques et aux rendez-vous de suivi.</li><li>Il se peut que les intestins s’infectent après l’opération. Communiquez avec votre chirurgien si votre enfant a la diarrhée et qu’elle est liquide ou nauséabonde, s’il a le ventre enflé, s’il a de la fièvre ou s’il vomit.</li></ul><h2>Comment diagnose-t-on la maladie de Hirschsprung?</h2><p>On diagnostique habituellement la maladie de Hirschsprung chez les nouveau-nés, mais il arrive qu’on le fasse chez des enfants plus âgés souffrant de <a href="/Article?contentid=6&language=French">constipation</a> grave.</p><p>Voici les étapes du diagnostic :</p><ol><li>Votre médecin examine votre enfant et vous interroge sur ses antécédents médicaux.</li><li>Il est possible qu’il passe une radiographie spéciale appelée un lavement baryté qui aidera le médecin à examiner ses intestins.</li><li>Votre médecin recommandera une biopsie rectale. Cette intervention consiste à prélever un petit morceau des intestins et à l’étudier pour vérifier s’il comporte des neurones.</li><li>Il arrive que votre enfant subisse un autre test qui s’appelle la manométrie et qui vérifie si le sphincter (le muscle qui ouvre et ferme l’anus de votre enfant) fonctionne normalement. Pour effectuer ce test, on insère entre ses fesses un petit ballon plat qu’on gonfle par la suite. Si son sphincter ne fonctionne pas normalement, il est possible que votre enfant soit atteint de la maladie de Hirschsprung.</li></ol><h2>Comment traite-t-on la maladie de Hirschsprung?</h2><p>Il faudra opérer votre enfant pour réparer ses intestins. Le type d’opération dépend de la proportion des intestins de l’enfant qui sont touchés.</p><p>L’intervention la plus répandue, nommée « rétablissement de continuité », consiste à enlever la partie des intestins dépourvue de neurones et à fixer la partie normale juste au dessus de l’anus.</p><p>Certains enfants peuvent avoir besoin qu’on pratique une <a href="/article?contentid=1027&language=French">stomie</a> avant l’opération. Il s’agit de la création d’une ouverture abdominale reliant l’intérieur à l’extérieur du corps. Les selles passent par cet orifice et sont recueillies dans un sac fixé à l’abdomen de votre enfant.</p><p>Bien que cette stomie soit temporaire la plupart du temps, il arrive rarement qu’elle soit permanente. Sa durée dépend de la proportion des intestins qui sont touchés par la maladie de Hirschsprung.</p><p>Après la stomie, le chirurgien de votre enfant discutera avec vous du type d’opération recommandé pour votre enfant et de la date de cette intervention.</p><p>Quel que soit le type d’opération, l’objectif consiste toujours à extraire la partie des intestins dépourvue de neurones et à laisser les autres parties fonctionner normalement.</p><h2>La maladie de Hirschsprung peut-elle causer d’autres problèmes?</h2><p>Tous les enfants atteints de la maladie de Hirschsprung courent le risque d’avoir, avant et après l’opération, une entérocolite de Hirschsprung, qui est une infection intestinale qui peut mettre en danger la vie de l’enfant.</p><p>Communiquez avec votre chirurgien dans les cas suivants :</p><ul><li>Votre enfant a la <a href="/Article?contentid=7&language=French">diarrhée</a> et elle est liquide, nauséabonde ou explosive. </li><li>Son abdomen est distendu (enflé).</li><li>Il a de la fièvre.<br></li><li>Il <a href="/Article?contentid=746&language=French">vomit</a>.</li></ul><p>L’entérocolite prend diverses formes selon les enfants : certains ne présentent qu’un seul symptôme tandis que d’autres les ont tous. En cas de doute, n’hésitez jamais à poser des questions au chirurgien de votre enfant. Si vous ne pouvez le joindre au bout de quelques heures, amenez votre enfant au service des urgences.</p><p>Si vous consultez un pédiatre pour une maladie dont les symptômes sont des vomissements et la diarrhée, n’oubliez pas de lui dire que votre enfant est atteint de la maladie de Hirschsprung.</p>

 

 

Hirschsprung disease830.000000000000Hirschsprung diseaseHirschsprung diseaseHEnglishGastrointestinalChild (0-12 years);Teen (13-18 years)Large Intestine/ColonLarge intestineConditions and diseasesCaregivers Adult (19+)NA2014-09-19T04:00:00ZKimberly Colapinto RN (EC), MN;Jacob Langer, MD;Division of General and Thoracic Surgery8.0000000000000064.00000000000001312.00000000000Health (A-Z) - ConditionsHealth A-Z<p>Learn how Hirschprung disease affects the bowel and how it is diagnosed and usually treated.</p><h2>What is Hirschsprung disease?</h2> <p>Hirschsprung disease is a condition in which special nerve cells (ganglion cells) are missing from the bowel or colon. For this reason, the disease is also known as aganglionic megacolon. When these cells are missing, stool (poo) does not move properly. This can cause a blockage and make your child very sick. The condition is named after Dr. Harald Hirschsprung.</p> <p>Hirschsprung disease is a congenital disease. This means that a person is born with it. It occurs in one in every 5,000 births and is more common in boys than girls. Sometimes it is hereditary, meaning that a parent can pass it on to a child.</p><h2>Key points</h2> <ul> <li>Hirschsprung disease is a condition in which nerve cells are missing from part of the bowel. This can cause stool to become blocked and make a child very sick.</li> <li>The usual treatment for Hirschsprung disease is "pull-through" surgery to remove the part of the bowel without nerve cells and allow the rest of the bowel to work normally. Some children might need an <a href="https://akhpub.aboutkidshealth.ca/article?contentid=1027&language=English">ostomy</a> before pull-through surgery.</li> <li>After surgery, apply ointment to ease diaper rash and follow the surgical team's instructions for giving pain medicines and returning for follow-up appointments. </li> <li>Bowel infections can occur after surgery. Contact your surgeon if your child has watery or foul-smelling diarrhea, a swollen belly or fever or is vomiting.</li> </ul><h2>How is Hirschsprung disease diagnosed?</h2> <p>Hirschsprung disease is usually diagnosed in newborn babies, but it can sometimes be diagnosed in an older child who has problems with severe <a href="/Article?contentid=6&language=English">constipation</a>.</p> <p>The diagnosis can be made in several steps.</p> <ol> <li>Your doctor will examine your child and take their medical history.</li> <li>Your child may have a special X-ray called a contrast enema that will help the doctor see what your child's bowel looks like.</li> <li>Your doctor will recommend a rectal biopsy. This involves taking a tiny piece of the inside of the bowel and studying it to see if it has any nerve cells.</li> <li>Sometimes your child will have another test called manometry. The test checks if your child's sphincter muscles (the muscles that help hold in stool) are working normally. To do the test, a small flat balloon is placed into your child's bottom. Once inside, the balloon is filled with air. If the sphincter is not working normally, it could mean that your child has Hirschsprung disease.</li> </ol><h2>How is Hirschsprung disease treated?</h2> <p>Your child will need surgery (an operation) to repair their bowel. The type of surgery they have depends on how much of their bowel is affected by Hirschsprung disease.</p> <p>The most common surgery to correct this is called a "pull-through". This involves removing the part of the bowel that does not have nerve cells and attaching the normal bowel just above the anus.</p> <p>Some children will first need surgery to create an <a href="https://akhpub.aboutkidshealth.ca/article?contentid=1027&language=English">ostomy</a> before the pull-through surgery. This is an opening created from the inside of the body to the outside, on the abdomen (tummy). Stool passes through this opening and is collected in a pouch attached to your child's abdomen.</p> <p>This ostomy is usually only temporary, but, rarely, it can be permanent. How long your child has the ostomy depends on how much of their bowel is affected by Hirschsprung disease.</p> <p>After the ostomy is made, your child's surgeon will discuss which type of surgery to correct Hirschsprung disease will be right for your child and when it should happen.</p> <p>Whichever type of surgery your child has, the goal is always to remove the part of the bowel that does not have nerve cells and allow the remaining bowel to work again.</p> <h2>Can Hirschsprung disease cause any other problems?</h2> <p>All children with Hirschsprung disease are at risk for Hirschsprung enterocolitis before and after surgery. This is a bowel infection that can become life threatening.</p> <p>Contact your surgeon if your child:</p> <ul> <li>has watery, foul smelling or explosive <a href="/Article?contentid=7&language=English">diarrhea</a></li> <li>has abdominal distention (swollen belly)</li> <li>has a <a href="https://akhpub.aboutkidshealth.ca/article?contentid=30&language=English">fever</a><br></li> <li>is <a href="/Article?contentid=746&language=English">vomiting</a>.</li> </ul> <p>Enterocolitis can be different in every child: some children will only have one symptom and others might have all of them. Always talk to your child's surgeon if you feel unsure. If you cannot contact your surgeon within a few hours, bring your child to the emergency department.</p> <p>If you see a paediatrician for an illness that has vomiting and diarrhea as a symptom, remind them that your child has Hirschsprung disease.</p><h2>How do I care for my child after surgery for Hirschsprung disease?</h2> <p>Your child can go home when they:</p> <ul> <li>are eating well</li> <li>are passing stool</li> <li>do not have a <a href="/Article?contentid=30&language=English">fever</a>.</li> </ul> <h3>Medicines, ointments and ostomy care</h3> <p>Your child will need to take pain medicine for the first few days after surgery. Always follow the instructions from the surgical team.</p> <p>It will be common for your child to develop bad <a href="https://akhpub.aboutkidshealth.ca/article?contentid=26&language=English">diaper rash </a>after pull through surgery when their bowel starts working normally again. Before your child leaves hospital, the nurses will teach you how to put special ointments or creams on your child's bottom to protect the skin from the rash.</p> <p>If your child has an ostomy, you will be taught how to care for it.</p> <h3>Follow-ups with the surgeon</h3> <p>If your child has had pull-through surgery, they will have stitches inside their bowel. You will not be able to see these stitches, but they could block the bowel while they are healing.</p> <p>To stop the stitches from blocking the bowel, your child's surgeon will have their first follow-up with your child one to three weeks after surgery. During this appointment, the surgeon will do a rectal dilation (gentle stretching of the anus) with their finger or a special instrument called a Hegar dilator. This tells them how the incision is healing. It is very important that you attend this appointment when it is scheduled.</p> <h3>Further dilations after surgery</h3> <p>The surgeon may bring your child back for more rectal dilatations or will teach you how to do them at home. You may need to buy your own Hegar dilator for this (it is not expensive).</p> <p>Your child may need these dilations for several months after surgery. It is very important for your child's care that you meet with your surgeon and follow their instructions.</p> <p>If you have any questions about your child's care, talk to your doctor.</p> <h2>Taking care of your child at home</h2> <ul> <li>Follow any instructions about bathing your child.</li> <li>To prevent diaper rash, apply ointments or creams to your child's bottom when they first start stooling from their anus.</li> <li>Do not put anything into your child's bottom (for example a temperature probe or medicine) until your child's doctor tells you it is ok to do so.</li> <li>Bring your child for follow-up appointments and follow any special care instructions.</li> <li>It is important for children with Hirschsprung disease to have a good-sized bowel movement every day. Some children will need to take medications that help them with their bowel routine.</li> <li>Most children with Hirschsprung disease respond well to a <a href="/Article?contentid=1205&language=English">high-fibre and low-sugar diet</a>. Some children also notice a difference to their stooling when they take products containing lactose. However, please discuss the best diet for your child with your child's surgeon and health care team.</li> </ul><h2>Long-term effects of Hirschsprung disease</h2> <p>Although most children have excellent results after surgery, some will continue to have problems with bowel movements. Some may have constipation or diarrhea and others may have incontinence (accidents) because they cannot hold their stool. This could be caused by:</p> <ul> <li>a blockage in the bowel</li> <li>poor bowel function (the bowel does not work as it should)</li> <li>the internal sphincter muscle being unable to relax properly</li> <li>stool withholding (your child "holds back" on going to the bathroom).</li> </ul> <p>Continue to follow up with the surgical team until your child is a teenager to make sure your child's problems are managed. Some children will also be followed by the gastroenterology team.</p>Hirschsprung disease

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