Hirschsprung Disease

Protocols

Clinical Features:

  • 1)Chronic constipation not respond medical treatment. 
  • 2)Stool- small,pillet,Ribon like. 
  • 3)Enterocolitis 
  • 4)H/O delayed passage meconium 
  • 5)Abdominal distension, billious vomiting 


O/E:

  • P/R- easily pass figure but feel strong grip.& Rectum empty when out finger small fees & gas discharge. 

Investigation: 

  • 1) plan X-ray abdomen : distended guts
  • 2) Unprepared bowel with contrast enema: 
    • proximal dilated segment 
    • Narrow lower colon 
    • Rectal diameter less then sigmoid colon -HPD
  • 3)Rectal Suction biposy: Absence of ganglionic cell 
  • 4)Rectal manometry 

Rx:

In Acute conditions 
  • NPO 
  • NG Tube- to decompression stomach
  • Antibiotic (inj.Ciprofloxacin / ceftriaxone) 
  • Rectal irregation with normal saline 
  • KT inj. If need 

Definative treatment : surgery

1st step: 

  • Colostomy

2nd step: 

  • Resection of aganglionic part & end to end anastomosis

3rd step: 

  • Colostomy closure 

Now a days Single stage procedure most popular. 

Prognosis: good if treated early