What is Hirschsprung disease (HD)?
Hirschsprung* disease (HD) is a disease of the large intestine that causes severe constipation or intestinal obstruction. Constipation means stool moves through the intestines slower than usual. Bowel movements occur less often than normal and stools are difficult to pass. Some children with HD can’t pass stool at all, which can result in the complete blockage of the intestines, a condition called intestinal obstruction. People with HD are born with it and are usually diagnosed when they are infants. Less severe cases are sometimes diagnosed when a child is older. An HD diagnosis in an adult is rare.
*See Pronunciation Guide for tips on how to say the words in bold type.
HD is a disease of the large intestine that causes severe constipation or intestinal obstruction.
What are the large intestine, colon, rectum, and anus?
The large intestine, which includes the colon and rectum, is the last part of the digestive tract. The large intestine’s main job is to absorb water and hold stool. The rectum connects the colon to the anus. Stool passes out of the body through the anus. At birth, the large intestine is about 2 feet long. An adult’s large intestine is about 5 feet long.
Why does HD cause constipation?
People with HD have constipation because they lack nerve cells in a part or all of the large intestine. The nerve cells signal muscles in the large intestine to push stool toward the anus. Without a signal to push stool along, stool will remain in the large intestine.
How severe HD is depends on how much of the large intestine is affected. Short-segment HD means only the last part of the large intestine lacks nerve cells. Long-segment HD means most or all of the large intestine, and sometimes the last part of the small intestine, lacks nerve cells.
In a person with HD, stool moves through the large intestine until it reaches the part lacking nerve cells. At that point, the stool moves slowly or stops, causing an intestinal obstruction.
What causes HD?
Before birth, a child’s nerve cells normally grow along the intestines in the direction of the anus. With HD, the nerve cells stop growing too soon. Why the nerve cells stop growing is unclear. Some HD is inherited, meaning it is passed from parent to child through genes. HD is not caused by anything a mother did while pregnant.
What are the symptoms of HD?
The main symptoms of HD are constipation or intestinal obstruction, usually appearing shortly after birth. Constipation in infants and children is common and usually comes and goes, but if your child has had ongoing constipation since birth, HD may be the problem.
Symptoms in Newborns
Newborns with HD almost always fail to have their first bowel movement within 48 hours after birth. Other symptoms include
•green or brown vomit
•explosive stools after a doctor inserts a finger into the rectum
•swelling of the belly, also known as the abdomen
•lots of gas
Symptoms in Toddlers and Older Children
Symptoms of HD in toddlers and older children include
•not being able to pass stools without laxatives or enemas. A laxative is medicine that loosens stool and increases bowel movements. An enema is performed by flushing water, or sometimes a mild soap solution, into the anus using a special wash bottle.
•swelling of the abdomen.
•lots of gas.
•slow growth or development.
•lack of energy because of a shortage of red blood cells, called anemia.
People with anemia tire easily because of a shortage of red blood cells.
How is HD diagnosed?
HD is diagnosed based on symptoms and test results.
A doctor will perform a physical exam and ask questions about your child’s bowel movements. HD is much less likely if parents can identify a time when their child’s bowel habits were normal.
If HD is suspected, the doctor will do one or more tests.
An x ray is a black-and-white picture of the inside of the body. To make the large intestine show up better, the doctor may fill it with barium liquid. Barium liquid is inserted into the large intestine through the anus.
If HD is the problem, the last segment of the large intestine will look narrower than normal. Just before this narrow segment, the intestine will look bulged. The bulging is caused by blocked stool stretching the intestine.
During manometry, the doctor inflates a small balloon inside the rectum. Normally, the rectal muscles will relax. If the muscles don’t relax, HD may be the problem. This test is most often done in older children and adults.
Biopsy is the most accurate test for HD. The doctor removes a tiny piece of the large intestine and looks at it with a microscope. If nerve cells are missing, HD is the problem.
How is HD treated?
HD is treated with surgery called a pull-through procedure. A surgeon removes the segment of the large intestine lacking nerve cells and connects the healthy segment to the anus. The pull-through procedure is usually done soon after diagnosis.
An ostomy allows stool to leave the body through an opening in the abdomen. Although most children with HD do not need an ostomy, a child who has been very sick from HD may need an ostomy to get better before the pull-through procedure.
For ostomy surgery, the surgeon first takes out the diseased segment of the large intestine. The end of the healthy intestine is moved to an opening in the abdomen where a stoma is created. A stoma is created by rolling the intestine’s end back on itself, like a shirt cuff, and stitching it to the abdominal wall. An ostomy pouch is attached to the stoma and worn outside the body to collect stool. The pouch will need to be emptied several times each day.
If the surgeon removes the entire large intestine and connects the small intestine to the stoma, the surgery is called an ileostomy. If the surgeon leaves part of the large intestine and connects it to the stoma, the surgery is called a colostomy.
Later, during the pull-through procedure, the surgeon removes the stoma and closes the abdomen with stitches.
Step 1: The diseased segment is removed.
Step 2: The healthy intestine is moved to an opening in the abdomen where a stoma is created.
What will my child's life be like after surgery?
After Ostomy Surgery
Infants will feel better after ostomy surgery because they will be able to easily pass gas and stool.
Older children will feel better, too, but they must adjust to living with an ostomy. They will need to learn how to take care of the stoma and how to change the ostomy pouch. With a few changes, children with ostomies can lead normal lives. However, they may worry about being different from their friends. A special nurse called an ostomy nurse can answer questions and show how to care for an ostomy.
An ostomy nurse can answer questions and show how to care for an ostomy.
After the Pull-through Procedure
Most children pass stool normally after the pull-through procedure. Children may have diarrhea for awhile, and infants and toddlers may develop diaper rash, which is treatable with diaper creams. Over time, stool will become more solid and the child will go to the bathroom less often. Toilet training may take longer. Children often must learn how to use the muscles of the anus after surgery. Some children may leak stool for awhile, but most will learn to have better bowel control as they get older.
Diet and Nutrition
After the pull-through procedure, children with long-segment HD need to drink more fluids. Now that the large intestine is shorter, or entirely gone, it is less able to absorb fluids the body needs. Drinking more helps make up for the loss.
Some infants may need tube feedings for awhile. A feeding tube allows infant formula or milk to be pumped directly into the stomach or small intestine. The feeding tube is passed through the nose or through an incision in the abdomen.
Eating high-fiber foods can help reduce constipation and diarrhea. Fiber helps form stool, making bowel movements easier. High-fiber foods include whole-grain breads, vegetables, and fruits. Some children may need laxatives to treat ongoing constipation. Consult a doctor before giving a laxative to your child.
People with HD can suffer from an infection of the intestines, called enterocolitis, before or after surgery.
•bleeding from the rectum
•lack of energy
Call the doctor right away if your child shows any of these signs.
Children with enterocolitis need to go to the hospital. An intravenous (IV) tube is inserted into a vein to give fluids and antibiotics. The large intestine is rinsed regularly with a mild saltwater solution until all stool has been removed. The solution may also contain antibiotics to kill bacteria. A temporary ostomy may be needed to help the intestine heal.
Sometimes infection is a sign of a problem with the pull-through procedure. More surgery may be needed to correct the problem and prevent future infections.
If I have more children, will they also have HD?
If you have a child with HD, your chance of having more children with HD is greater. Talk with your doctor about the risk.
Points to Remember
•Hirschsprung disease (HD) is a disease of the large intestine that causes severe constipation or intestinal obstruction. People with HD are born with it.
•The large intestine, which includes the colon and rectum, is the last part of the digestive tract.
•The cause of HD is unclear. HD is not caused by anything a mother did while pregnant.
•The main symptoms of HD are constipation or intestinal obstruction, usually appearing shortly after birth.
•Newborns with HD almost always fail to have their first bowel movement within 48 hours after birth.
•HD is diagnosed based on symptoms and test results.
•HD is treated with surgery called a pull-through procedure.
•A child who has been very sick from HD may need an ostomy to get better before the pull-through procedure.
•Most children pass stool normally after the pull-through procedure.
•People with HD can suffer from an infection of the intestines, called enterocolitis, before or after surgery.
•If you have a child with HD, your chance of having more children with HD is greater.
Hope through Research
The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) conducts and supports basic and clinical research into many digestive disorders, including HD. Scientists are studying the genetics involved in HD to improve diagnosis, treatment, and genetic counseling. They are also looking at therapies for treating enterocolitis, a major complication of HD.
Participants in clinical trials can play a more active role in their own health care, gain access to new research treatments before they are widely available, and help others by contributing to medical research. For information about current studies, visit www.ClinicalTrials.gov.
For More Information
International Foundation for Functional Gastrointestinal Disorders
P.O. Box 170864
Milwaukee, WI 53217–8076
Phone: 1–888–964–2001 or 414–964–1799
United Ostomy Associations of America, Inc.
P.O. Box 66
Fairview, TN 37062–0066
Phone: 1–800–826–0826 or 615–799–2990
You may also find additional information about this topic by visiting MedlinePlus at www.medlineplus.gov .
This publication may contain information about medications. When prepared, this publication included the most current information available. For updates or for questions about any medications, contact the U.S. Food and Drug Administration toll-free at 1–888–INFO–FDA (1–888–463–6332) or visit www.fda.govExternal Link Disclaimer. Consult your doctor for more information.
Publications produced by the Clearinghouse are carefully reviewed by both NIDDK scientists and outside experts. The National Digestive Diseases Information Clearinghouse would like to thank the following individuals for providing scientific and editorial review or facilitating field-testing of the original version of this publication:
Paul Hyman, M.D.
University of Kansas Medical Center
Kansas City, KS
Jacob C. Langer, M.D.
The Hospital for Sick Children
Toronto, Ontario, Canada
Andrea M. Anastas
International Foundation for Functional Gastrointestinal Disorders, Inc.
North Andover, MA
Hirschsprungs & Motility Disorders Support Network, The Guardian Society
Land O' Lakes, FL
Thank you also to the parents of children with Hirschsprung disease and the nurse practitioners who helped shape this booklet.
National Digestive Diseases Information Clearinghouse
2 Information Way
Bethesda, MD 20892–3570
The National Digestive Diseases Information Clearinghouse (NDDIC) is a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). The NIDDK is part of the National Institutes of Health of the U.S. Department of Health and Human Services. Established in 1980, the Clearinghouse provides information about digestive diseases to people with digestive disorders and to their families, health care professionals, and the public. The NDDIC answers inquiries, develops and distributes publications, and works closely with professional and patient organizations and Government agencies to coordinate resources about digestive diseases.
This publication is not copyrighted. The Clearinghouse encourages users of this publication to duplicate and distribute as many copies as desired.
NIH Publication No. 10–4384
Hirschsprung Disease Treatment & Management
Author: Justin P Wagner, MD; Chief Editor: BS Anand, MD
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).
Hirschsprung disease cannot be prevented; however, perceptive clinical acumen may prevent delays in diagnosis.
The general goals of medical care are 3-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 postoperative bowel function.
The goals of medical care are to maintain normal fluid and electrolyte balance, to minimize bowel distension and prevent perforation, and to manage complications. Intravenous fluid resuscitation and maintenance, nasogastric decompression, and administration of intravenous antibiotics (as indicated) remain the cornerstones of initial medical management.
Colonic lavage, consisting of mechanical irrigation with a large-bore rectal tube and large volumes of irrigant, may be required.
Intravenous administration of balanced salt solutions may help prevent electrolyte imbalances.
Postoperative medical management
Postoperatively, routine colonic irrigation and prophylactic antibiotic therapy may decrease the risk of developing enterocolitis.[35, 36] 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 enterocolitis.
Diet and activity
The patient should have nothing by mouth for 6-8 hours prior to operation.
Postoperatively, the patient will receive intravenous fluids and antibiotics; however, nothing may be administered by mouth until passage of flatus or stool signifies return of bowel function. If a newborn undergoes creation of a diverting colostomy, the passage of flatus or stool from the stoma is necessary prior to institution of oral feeding.
Upon resumption of bowel function, tube feeding or formula/breast milk may resume. Clear liquids are delivered by mouth, and the diet may be advanced until feeding goals are met. Feedings are usually initiated 24-48 hours after the creation of a colostomy. The patient may be discharged from the hospital upon attaining full feedings.
Diets consisting of fresh fruits, vegetables, and high-fiber articles may improve postoperative bowel function.
With regard to activity, limit physical activity for about 6 weeks to allow incisions to heal properly (applies more to older children).
Also see Pediatric Hirschsprung Disease and Hirschsprung Disease Imaging.
Surgical management of Hirschsprung disease begins with the initial diagnosis, which often requires a full-thickness rectal biopsy. Traditionally, a diverting colostomy was created at the time of diagnosis, and definitive repair was delayed until the child grew to a weight of 10 kilograms.
This standard of treatment was developed in the 1950s after Swenson reported relatively high leak and stricture rates with a single-stage operation. Advancements in anesthesia administration and hemodynamic monitoring have led many surgeons to advocate a single-stage pull-through procedure without initial diversion. Contraindications to a single-stage procedure include severely dilated proximal bowel, severe enterocolitis, perforation, malnutrition, and inability to accurately determine the zone of transition between healthy and aganglionic bowel, intraoperatively.
For neonates undergoing creation of a diverting colostomy, the transition zone is identified and the colostomy is placed proximal to this area. The presence of ganglion cells at the colostomy site must be unequivocally confirmed by histological evaluation of a frozen-section biopsy. Either a loop- or end-colostomy is created at the surgeon’s discretion.
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 cleanse 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. A meta-analysis performed by Friedmacher and Puri in 2011 reported that residual aganglionosis and transition-zone tissue account for persistent bowel symptoms in one third of patients undergoing a second, corrective pull-through procedure.
Also see Pediatric Hirschsprung Disease and Hirschsprung Disease Imaging.
The Swenson procedure was the original pull-through procedure used to treat Hirschsprung disease. The aganglionic segment is resected down to the sigmoid colon and rectum, and an oblique anastomosis is performed between the normal colon and the low rectum.
The Duhamel procedure was first described in 1956 as a modification to the Swenson procedure. A retrorectal approach is used, and a significant segment of aganglionic rectum is retained.
The aganglionic bowel is resected down to the rectum, and the rectum is oversewn. The proximal bowel is then brought through the retrorectal space (between rectum and sacrum), and an end-to-side anastomosis is performed with the remaining rectum.
Soave (endorectal) procedure
The Soave procedure was introduced in the 1960s. The mucosa and submucosa of the rectum are resected, and the ganglionic bowel is pulled through the aganglionic muscular cuff of the rectum.
The original operation did not include a formal anastomosis, relying on scar tissue formation between the pull-through segment and the surrounding aganglionic bowel. The procedure has since been modified by Boley to include a primary anastomosis at the anus.
For patients with extremely short-segment Hirschsprung disease, anorectal myomectomy is an alternative surgical option.
The surgeon removes a 1-cm-wide strip of extramucosal rectal wall, beginning immediately proximal to the dentate line and extending to the normal ganglionic rectum. The mucosa and submucosa are preserved and closed.
Procedures for long-segment Hirschsprung disease
Patients with total colonic involvement require modified procedures to exclude the aganglionic colon while preserving maximal absorptive epithelium. The goal of these procedures is to bypass dysfunctional bowel while maximizing the chance of postoperative nutritional function and growth.
Most procedures include a side-to-side anastomosis of healthy small bowel with a short segment of the aganglionic/absorptive colon. Either a short right colonic patch or the small bowel is anastomosed to the rectal wall, similar to a Duhamel procedure. Importantly, a short patch (< 10 cm) is maintained.
Long-segment anastomoses, such as the Martin procedure, are no longer advocated.
A laparoscopic approach to the surgical treatment of Hirschsprung disease was first described in 1999 by Georgeson. The transition zone is first identified laparoscopically, after which the rectum is mobilized below the peritoneal reflection. A transanal mucosal dissection is performed, and the rectum and aganglionic bowel is prolapsed through the anus. The healthy proximal bowel is anastomosed to the rectal cuff. Functional outcomes of this laparoscopic approach appear to be equivalent to open techniques based on short-term results.[41, 42, 43]
Transanal pull-through procedures
Transanal pull-through procedures have been described in which no intra-abdominal dissection is performed.[21, 44] The entire procedure is performed transanally in a manner similar to perineal rectosigmoidectomy.
The mucosa is incised circumferentially above the dentate line, and a submucosal dissection is directed proximally. The muscularis is incised circumferentially, and the remainder of the dissection is carried external to the rectal wall until the transition zone is identified. Upon confirmation of ganglion cells on frozen section, the aganglionic bowel is resected and an anastomosis is performed.
Outcomes of the transanal pull-through procedure have been similar to open single-stage approaches, and analgesia requirements and hospital stays are decreased.[21, 45, 46] Recent studies also report lower rates of postoperative incontinence and shorter operating times in transanal pull-through procedures.[47, 48]
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).[49, 50, 51, 52]
Regenerative strategies are under investigation to restore function in aganglionic intestine. Stem cell transplantation to regenerate the enteric nervous system is the subject of many recent experimental series. Stem cells derived from the neural crest persist into adulthood, and several are capable of proliferation and differentiation within the intestine. Hotta and colleagues recently reported successful generation of functional enteric neurons from precursor cells transplanted into recipient colon. Though auspicious, these discoveries warrant further study to translate cell-based therapies into clinical practice.
After a definitive pull-through procedure is performed, the patient should achieve normal growth and development.
Patients should be monitored for their bowel habit. Patients with no other underlying disorders and no postoperative complications often develop improved bowel function; however, normal bowel habit may take years to develop.
After definitive surgical repair, patients may experience persistent abnormal gastrointestinal motility. Postoperative hypomotility is relatively common, and many patients require a prolonged course of laxative treatment. Patients who retain stool despite laxative therapy may require enemas.
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