HIRCHSPRUNG’S DISEAS

prepared by; Maryam Yousaf

 DEFINITION
• It is a disorder of gut caused due to
congental absence of ganglion cells in
the submicosal and myentric plexus of
intestine.it is also known as Megacolon
or Aganglionic Megacolon.
 EPIDEMOLOGY
• INCIDENCE:- 1/5000 live birth
newborn
• 70-80% is boys (M/F 4:1)
• less common in blacks
PATHOLOGY
due to absence of ganglionic cells

there is lack of peristlasis in affected portion

functional obstruction in colon

accumulation of gas & faeces proximal to defect

enlargment of colon occur and called megacolon

 CLINICAL FEATURS
• IN NEONATE • IN OLDER CHILD
• abdominal distension • constipation with
• failure to pass abdominal distension
meconium • foul smell of stool
• bile stain • liquid consistency of
• vomiting stool
• eppisodes of dirrhea • malnurished
& constipation • anemia
 MANAGEMENT

• MEDICAL MANAGEMENT

• SURGICAL MANAGMENT
A ( Medical Management)
• administration of isotonic enema

• adminstration of softners

• low residue dite

 B( Surgical Managment)
• surgery involve two steps
• Temporay colostomy
• definitive surgeries
• (1) Swenson procedure
• (2) Duhamel procedure
• (3) Soave’s procedure
 NURSING MANAGEMENT
• PRE OP CARE
• assessment
• complete history of new born
• after diagnosis nurse must help parents
• nurse give isotonic anema & stool softners
• low residue dite given to child
• check vital signs & abdominal grith of child
• keep the child semi- fowlers position

• withhold oral feed

• nasogastric aspiration done on the night
before surgery
 POST OP CARE
• moniter vital signs
• observe abdominal bleeding
• place the child in comfertable position
according to physician order
• child is NPO,so adminster IV fluid as order
• colostomy care done which include fillowing
• observe stoma of its color (red - pink color)
• provide balance dite
• apply zinc oxide ointment on skin