Hirschsprung disease

Its a pediatric disease which is very severe, this content will be useful for the students who pursuing nursing course

1. HIRSCHSPRUNG’S DISEASE:

2. HIRSCHSPRUNG’S DISEASE: Definition: Hirschsprung’s disease is a disorder of the gut caused due to congenital absence of ganglion cells in the submucosal and myentric plexus of intestine. It is also known as Megacolon or Congenital Aganglionic Megacolon .

3. INCIDENCE & ETIOLOGY: 1 in 5000 live births , it is more common in males than females. Through research it is suggested that it is caused by interaction between two proteins encoded by two genes: RET Proto- oncogene (chromosome 10) EDNRB gene (chromosome 13)

4. PATHOPHYSIOLOGY:

5. CLINICAL FEATURES: In neonates and infants: Failure to pass meconium Abdominal distension Bile- stained vomiting Shock Episodes of diarrhea & constipation In older children: Constipation with abdominal distension When stool passed foul smelling, and liquid in consistency Malnourished and anemic

6. DIAGNOSTIC EVALUATION: Hirschsprung’s disease is suspected in a baby who has not passed meconium within 48 hours of birth . Rectal examination Palpation Anorectal manometry Barium enema & Rectal biopsy

7. MANAGEMENT: Medical management: Administration of Isotonic enema Administration of stool softeners Low residue diet B. Surgical management: The surgery involves two steps: TEMPORARY COLOSTOMY DEFINITIVE SURGERY, after about a year of these surgeries, the colostomy is closed.

8. A): Swenson Procedure B): Duhamel Procedure C): Soave’s Procedure

9. Swenson & Duhamel Procedure:

10. C): Soave’s Procedure:

11. NURSING MANAGEMENT: Pre- operative care: Assessment, complete history of new born. After diagnosis, nurse must help parents. Nurse taught about giving isotonic enema, suppositories and stool softeners. Low residue diet must be given to the child. Monitor vital signs & abdominal girth of child. Keep the child in semi-fowlers postion . Withhold oral feeds & Nasogastric aspiration done on the night before surgery.

12. Post-operative care: Monitor vital signs, observe abdominal bleeding. Place the child in comfortable position according to the physician order. Child is NPO, so administer IV fluids as ordered. Monitor bowel sound. Colostomy care is to be done which includes following: Observe stoma for its colour (reddish- pink colour ) Observe for bleeding, purulent drainage, edema. Provide bland diet. apply zinc oxide ointment on skin around stoma.

13. Frequently empty the collecting bag. Keep the colostomy clean and dry. Educate parents about colostomy care. vi. Encouraging and supporting the family during this stressful time is the key nursing intervention.

14. THANK YOU..!