HIRCHSPRUNG DISEASE

HIRSCHSPRUNG DISEASEBaylon, Jaya Neca F.BSN3A2HIRSCHSPRUNG DISEASEAlso known as AGANGLIONIC MEGACOLONAbsence of ganglionic innervation of a section of the bowel.Aganglionic disease is higher in the siblings of a child with the disorder than in other childrenIt occurs more often in male than in female.The incidence is approximately 1 in 5000 live births.

CLINICAL MANIFESTATIONThe newborn presents with failure to pass meconium within 24-48 hours, refusal to eatoran alteration in feeding patterns,and episodes of bilious vomiting(vomiting bile)Presentation in the infant: failure to thrive, jaundice, chronic constipation, abdominal distention, bowel obstruction, episodes of vomiting and diarrheaPresentation in the child: constipation and fecal impaction, abdominal distention, visible peristalsis, stool that appears ribbon-like, palpable fecal mass, malnourished appearance

ASSESSMENTSymptoms become apparent until 6-12 mos.History of constipation or intermittent constipation and diarrheaEmpty rectum after digital rectal exam (DRE) DIAGNOSTIC PROCEDUREAbdominal radiographsAnorectal manometryContrast enema radiographs (barium enema)Suction rectal biopsy procedureTHERAPEUTIC MANAGEMENTRemoval of affected portion with anastomosis of the intestine.TWO STAGE OPERATION:Temporary colostomyBowel repair: 12-18 mons.Permanent colostomy: no nerve endings on anus.NURSING INTERVENTIONInfection: monitor the stoma or wound site for signs of infectionGrowth: monitor weight gain and height closelyDietary: implement the dietary regimen to provide for the childs nutritional needsSurgical care: teach the parent or caregiver how to provide care for the child before and after thecolostomyProvide for education on the surgical process, including anticipated length of the visit, preparatory, and postoperative care