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Hirschsprung 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.
Some 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.
Doctors 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.
Doctors 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.
Before 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.
The 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.
The 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.
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(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:
Congenital aganglionic megacolon, also called Hirschsprung disease, is characterized by persistent constipation resulting from partial or complete intestinal obstruction of mechanical origin. What is Hirschsprung Disease?In some cases, aganglionic megacolon or Hirschsprung disease may be severe enough to be recognized. Hirschsprung’s disease: Enlargement of the colon, caused by bowel obstruction resulting from an aganglionic section of bowel (the normal enteric nerves are absent).
PathophysiologyThe pathophysiology of aganglionic megacolon is as follows:
Statistics and IncidencesCases of aganglionic megacolon is seen in approximately 1 per 5000 live births.
CausesThe causes of aganglionic megacolon are:
Clinical ManifestationsExamination of infants affected with Hirschsprung disease reveals:
Assessment and Diagnostic FindingsThe diagnosis of aganglionic megacolon is made through the following data:
Medical ManagementTreatment involves:
Pharmacologic ManagementDrug therapy currently is not a component of the standard of care for this disease itself; however, some medications may be used to treat complications of Hirschsprung disease.
Surgical ManagementThe surgical options vary according to the patient’s age, mental status, ability to perform activities of daily living, length of the aganglionic segment, degree of colonic dilation, and presence of enterocolitis.
Nursing ManagementNursing care management for a child with aganglionic megacolon include: Nursing AssessmentAssessment involves:
Nursing DiagnosisBased on the assessment data, the major nursing diagnoses for Hirschsprung disease are:
Nursing Care Planning and GoalsThe major nursing care planning goals for patients with Hirschsprung Disease are:
Nursing InterventionsNursing interventions for a child with aganglionic megacolon include:
EvaluationGoals are met as evidenced by:
DocumentationDocumentation in a patient with aganglionic megacolon include:
Practice Quiz: Aganglionic MegacolonHere’s a 5-item quiz for Aganglionic Megacolon study guide. Please visit our nursing test bank page for more NCLEX practice questions. 1. The nurse is assessing an infant with Hirschsprung’s disease. The nurse can expect the infant to: A. Weigh less than expected for height and age. B. Have a scaphoid-shaped abdomen. C. Exhibit clubbing of the fingers and toes. D. Have hyperactive deep tendon reflexes. 1. Answer: B. Have a scaphoid-shaped abdomen.
2. Dustin who was diagnosed with Hirschsprung’s disease has a fever and watery explosive diarrhea. Which of the following would Nurse Joyce do first? A. Administer an antidiarrheal. B. Notify the physician immediately. C. Monitor the child every 30 minutes. D. Nothing. (These findings are common in Hirschsprung’s disease.) 2. Answer: B. Notify the physician immediately.
3. Parents bring their infant to the clinic, seeking treatment for vomiting and diarrhea that has lasted for 2 days. On assessment, the nurse in charge detects dry mucous membranes and lethargy. What other findings suggest a fluid volume deficit? A. A sunken fontanel 3. Answer: A. A sunken fontanel.
4. A newborn’s failure to pass meconium within the first 24 hours after birth may indicate which of the following? A. Hirschsprung disease B. Celiac disease C. Intussusception D. Abdominal wall defect 4. Answer: A. Hirschsprung disease.
5. Which of the following electrolytes are lost as a result of vomiting? A. bicarbonate and calcium B. sodium and hydrogen C. sodium and potassium D. hydrogen and potassium 5. Answer: D. hydrogen and potassium.
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