Definition & FactsHirschsprung disease is a birth defect in which some nerve cells are missing in the large intestine, so a child’s intestine can’t move stool and becomes blocked. About 1 in 5,000 newborns has Hirschsprung disease. Show
 Symptoms & CausesSome infants with Hirschsprung disease have signs and symptoms of intestinal obstruction shortly after they are born. Common symptoms in older infants and children include chronic constipation and swelling of the abdomen. Certain genes increase the chance that a child will have Hirschsprung disease. DiagnosisDoctors diagnose Hirschsprung disease by taking a medical and family history, performing a physical exam, and ordering tests. Tests may include imaging tests, anorectal manometry, and rectal biopsies. TreatmentDoctors typically treat Hirschsprung disease with a type of surgery called a pull-through procedure. In some cases, doctors recommend ostomy surgery followed by a pull-through procedure. Children with Hirschsprung disease most often feel better after surgery. Eating, Diet, & NutritionBefore Hirschsprung disease is diagnosed and treated, a child may develop problems with feeding, have a failure to thrive, or malnutrition. Talk with a doctor about what your child should eat and drink after surgery for Hirschsprung disease. Once they recover, children usually don’t need to follow a special diet. Clinical TrialsThe National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) conducts and supports clinical trials in many diseases and conditions, including digestive diseases. The trials look to find new ways to prevent, detect, or treat disease and improve quality of life.
Your Digestive System & How It WorksThe digestive system is made up of the gastrointestinal (GI) tract—also called the digestive tract—and the liver, pancreas, and the gallbladder. The GI tract is a series of hollow organs joined in a long, twisting tube from the mouth to the anus. Related Research
This content is provided as a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), part of the National Institutes of Health. The NIDDK translates and disseminates research findings to increase knowledge and understanding about health and disease among patients, health professionals, and the public. Content produced by the NIDDK is carefully reviewed by NIDDK scientists and other experts. The
NIDDK would like to thank: OverviewHirschsprung's (HIRSH-sproongz) disease is a condition that affects the large intestine (colon) and causes problems with passing stool. The condition is present at birth (congenital) as a result of missing nerve cells in the muscles of the baby's colon. Without these nerve cells stimulating gut muscles to help move contents through the colon, the contents can back up and cause blockages in the bowel. A newborn who has Hirschsprung's disease usually can't have a bowel movement in the days after birth. In mild cases, the condition might not be detected until later in childhood. Uncommonly, Hirschsprung's disease is first diagnosed in adults. Surgery to bypass or remove the diseased part of the colon is the treatment. SymptomsSigns and symptoms of Hirschsprung's disease vary with the severity of the condition. Usually signs and symptoms appear shortly after birth, but sometimes they're not apparent until later in life. Typically, the most obvious sign is a newborn's failure to have a bowel movement within 48 hours after birth. Other signs and symptoms in newborns may include:
In older children, signs and symptoms can include:
CausesIt's not clear what causes Hirschsprung's disease. It sometimes occurs in families and might, in some cases, be associated with a genetic mutation. Hirschsprung's disease occurs when nerve cells in the colon don't form completely. Nerves in the colon control the muscle contractions that move food through the bowels. Without the contractions, stool stays in the large intestine. Risk factorsFactors that may increase the risk of Hirschsprung's disease include:
ComplicationsChildren who have Hirschsprung's disease are prone to a serious intestinal infection called enterocolitis. Enterocolitis can be life-threatening and requires immediate treatment. Aug. 21, 2021
RelatedAssociated ProceduresProducts & ServicesWhat occurs in the gastrointestinal system of the child with Hirschsprung disease?In children with Hirschsprung's disease, nerves fail to form in all or part of the large intestine (colon). Waste from digestion cannot pass through the part of the colon lacking nerve tissue. The normal colon swells with blocked stool.
Which procedure would the nurse anticipate to confirm the diagnosis of Hirschsprung disease congenital Aganglionic Megacolon in a 1 month old infant?Diagnosis is confirmed through rectal biopsy.
Which of the following is the nurse's priority in the plan of care for a child admitted with pyloric stenosis?The major nursing care planning goals for a child with pyloric stenosis are: Improving nutrition and hydration.
Which type of nutrition would the nurse expect to administer to a preterm infant who was born at 34 weeks gestation and has developed necrotizing enterocolitis NEC?Breast milk is the best nutrition for all babies, especially preemies. It has proteins that help fight infection. Most preemies can't feed straight from the breast or bottle at first. Mothers pump their milk and it's given to babies through a tube that goes through the nose or mouth and into the stomach.
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Quizlet what occurs in the gastrointestinal system of the child with hirschsprung disease?
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