{"id":118,"date":"2020-09-14T12:36:16","date_gmt":"2020-09-14T12:36:16","guid":{"rendered":"http:\/\/www.paedsurgeryindelhi.com\/blog\/?p=118"},"modified":"2020-09-15T04:15:39","modified_gmt":"2020-09-15T04:15:39","slug":"hirschsprungs-disease","status":"publish","type":"post","link":"https:\/\/www.paedsurgeryindelhi.com\/blog\/hirschsprungs-disease\/","title":{"rendered":"Hirschsprung\u2019s disease"},"content":{"rendered":"\n<p><strong>Hirschsprung\u2019s disease<\/strong> is a disease affecting the nerve cells (Ganglion cells) of the large intestine (It sometimes also affects the nerve cells of the small intestine). These nerve cells control the muscles that help in the movement of the intestines (peristalsis) topush thefood\/waste (or stool)from the small intestines through the large intestine to be finally excreted through the anus. During development, these nerve cells are formedin the intestines in forward fashion i.e. from the mouth to the anus. In babies with Hirschsprung\u2019s disease, these nerve cells don\u2019t grow past a certain part of the large intestine.And so,the muscles of that part can\u2019t move food and stool forwardand become partly or fully blocked. The waste gets collected in the intestinebehind the blockage so it begins to grow larger than normal, while the abnormal intestine which is present after the blockage remains thin. This then leads toabdominal swelling and infection.<\/p>\n<p>Most babies with Hirschsprung\u2019s disease have symptoms in the first few weeks of life. However, in some cases only a short part of the intestine may be affected, so they may present later in life. Therefore each child\u2019s symptoms and severity varies depending on the length of the involved intestinal segment. <\/p>\n<p><strong>Symptoms in new-borns may include:<\/strong><\/p>\n<ul class=\"list01\">\n  <li>Not having a bowel movement in the first 48 hours of  life<\/li>\n  <li>Slow distension of the abdomen <\/li>\n  <li>Vomiting green or brown fluid<\/li>\n  <li>Infection of the intestines (Enterocolitis)<\/li>\n<\/ul>\n<p><strong>Older Children who don\u2019t show early symptoms may have:<\/strong><\/p>\n<ul class=\"list01\">\n  <li>Severe Constipation that does not respond to  medication<\/li>\n  <li>Loss of appetite<\/li>\n  <li>Slow or delayed growth<\/li>\n  <li>Infection of the intestines (Enterocolitis)<\/li>\n<\/ul>\n<p>A numberof tests are done to find out if the child has Hirschsprung\u2019s disease or not. These tests include:<\/p>\n<ul class=\"list01\">\n  <li><strong>Abdominal X-ray.&nbsp;<\/strong>This test may show a  lack of stool in the large intestine or near the anus. It can also show if part  of the large intestine is dilated or not. <\/li>\n  <li><strong>Barium enema<\/strong><strong> (Dye Test)<\/strong><strong>.&nbsp;<\/strong>This  is a special X-ray which checks the large intestine for any problems. The child  is given a contrast called barium which is put into  a tube and inserted into the child\u2019s anus as an enema. Serial X-rays are taken  as the dye is being injected. The X-rays are looked for any narrowed areas or  any blockages. <\/li>\n  <li><strong>Biopsy of the rectum or large intestine.&nbsp;<\/strong>It is a short procedure, done under sedation of  General anaesthesia. A tiny piece of the Rectum\/large intestine is taken, either with an  Endoscope or by open technique and checked to see whether nerve  cells are missing.<strong>This&nbsp; confirms  the diagnosis of Hirschsprung\u2019s disease.<\/strong><strong> <\/strong><\/li>\n<\/ul>\n<p>The treatment depends on the child\u2019s symptoms, age and general health.Hirschsprung\u2019s disease is treated with a surgery called as pull-through procedure which can be performed by open as well as laparoscopic technique.In this procedure the surgeon removes the abnormal part of the large intestine that lacks nerve cells and the normal proximal part (which has the nerve cells) is connected to the anal opening. Although, It can be done as a single stage procedure, insome cases it may require two stage procedure. A child who is very sick because ofHirschsprung\u2019s disease may first need ostomy surgery (Stoma &#8211; opening of the intestine in the abdomen). This helps the child\u2019s intestine to heal before the final pull-through surgery. <\/p>\n<p>It is a major surgical procedure and complications caninclude loss of bowel control (leading to leaking of stools), constipation and infections.<\/p>\n<h4><strong>Interesting case <\/strong><\/h4>\n<p><img decoding=\"async\" src=\"https:\/\/www.paedsurgeryindelhi.com\/blog\/wp-content\/uploads\/2020\/09\/hirschsprungs1.jpg\" class=\"imgleft\" \/> A 6 month old male baby presented with complaints of abdominal distension, diarrhoea and vomiting. The baby had history of delayed passage of meconium since birth. The parents also complained that he had abdominal distension of an on and was not gaining weight. The baby was evaluated and was found to have enterocolitis (infection of the intestine). He was first treated with intravenous fluids and antibiotics, but had persistent abdominal distension. He was taken up for surgery and was found to have dilated large intestine and distal part of the large intestine (beyond the blockage) was thin. He was suspected to have Hirschsprung\u2019s disease, so biopsy of the abnormal intestine was taken and a stoma surgery was done for the healing of the intestine. The biopsy confirmed the diagnosis and so the child underwent pull through procedure after 2 months.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Hirschsprung\u2019s disease is a disease affecting the nerve cells (Ganglion cells) of the large intestine (It sometimes also affects the nerve cells of the small intestine).<\/p>\n","protected":false},"author":1,"featured_media":123,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":[],"categories":[49],"tags":[48],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v20.10 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Hirschsprung\u2019s disease<\/title>\n<meta name=\"description\" content=\"Hirschsprung\u2019s disease is a disease affecting the nerve cells (Ganglion cells) of the large intestine.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" 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