Chapter 34 Hirschsprung’s Disease
PATHOPHYSIOLOGY
Hirschsprung’s disease, or congenital aganglionic megacolon, is a congenital anomaly characterized by the absence of ganglion cells in the rectum and extending proximally to the colon and, rarely, the small intestines. The absence of ganglion cells causes a lack of enteric nervous system stimulation, resulting in the inability of the internal sphincter to relax and thus increase intestinal tone, which in turn causes decreased peristalsis. Intestinal contents are propelled to the aganglionic segment; because of the lack of innervation, fecal material accumulates, which results in dilation of the bowel (megacolon) proximal to the aganglionic area. Along with the lack of peristalsis, there is loss of the rectosphincteric reflex; namely, the rectal sphincter fails to relax, which prevents the normal passage of stool and thereby contributes to the obstruction. Exact etiology is not known; however, genetics and environmental factors are suspected to play a role. Hirschsprung’s disease generally manifests during the neonatal period; however, it may appear at any age.
SURGICAL MANAGEMENT
Surgical treatment of Hirschsprung’s disease is a two-stage process. Initially, a temporary colostomy is performed (1) to decompress the bowel and divert the fecal contents, and (2) to allow the dilated and hypertrophied portion of the bowel to regain normal tone and size. When the bowel regains normal tone, after approximately 3 to 4 months (infants should be between 6 and 12 months of age or weigh 8 to 10 kg), a rectal pull-through procedure is performed in which all aganglionic bowel is removed and the normal bowel is reconnected to the anus. The colostomy is also closed during the second surgery.
NURSING ASSESSMENT
See the Gastrointestinal Assessment section in Appendix A.
Preoperative
Postoperative
1. Assess child’s postoperative status.
2. Assess for signs of dehydration or fluid overload.
3. Assess fecal contents (ostomy) or return of bowel movements (after pull-through).
4. Assess for signs of infection.
5. Assess child’s level of pain (see Appendix I).
6. Assess child’s and family’s ability to cope with hospital and surgical experience.
7. Assess parents’ ability to manage treatment regimen and ongoing care.
NURSING INTERVENTIONS
Preoperative Care
Postoperative Care
1. Monitor and report child’s postoperative status.
2. Monitor child’s hydration status (depending on child’s status and hospital protocol).
3. Observe and report signs of complications.
4. Promote return of peristalsis.
5. Promote and maintain fluid and electrolyte balance.
6. Alleviate or minimize pain and discomfort (see Appendix I).
7. Promote and maintain respiratory function.
10. Provide emotional support to child and family (see the Preparation for Procedures or Surgery section in Appendix F).
Discharge Planning and Home Care
1. Instruct parents to monitor for signs and symptoms of the following long-term complications:
2. Provide instructions to parents and child about colostomy care:
3. Provide and reinforce instructions about dietary management:
4. Encourage parents’ and child’s expression of concerns related to colostomy (see Appendix F):
5. Provide parents with information regarding enterocolitis:
6. Refer to specific institutional procedures for information to be distributed to parents about home care.
Behrman RE, Kiegman R, Jenson HB. Nelson textbook of pediatrics, ed 17. Philadelphia: WB Saunders; 2004.
De Lorijn F, et al. Diagnosis of Hirschsprung’s disease: A prospective, comparative accuracy study of common tests. J Pediatr. 2005;146(6):787.
Halter JM, et al. Common gastrointestinal problems and emergencies in neonates and children. Clin Fam Pract. 2004;6(3):731.
Hockenberry MJ, et al. Wong’s nursing care of infants and children, ed 7. St. Louis: Mosby; 2004.
James SR, Ashwill JW. Nursing care of children—principles and practice, ed 3. Philadelphia: WB Saunders; 2007.
Swenson O. Hirschsprung’s disease: A review. Pediatrics. 2002;109(5):914.
