HIRSCHSPRUNG'S + ENTEROCOLITIS

Question 1

Approach to the Critically Ill Hirschsrung’s Enterocolitis

Answer 1

20 ml/kg bolus crystalloid followed by 1.5x maintenance fluid for shock / dehydration

Maintenance Fluid D5 0.45% NS

4 cc/kg/hr for 1st 10 kg
2 cc/kg/hr for 10-20 kg
1 cc/kg/hr for every kg > 20 kg

KCl @ 0.5 mEq/kg/hr IV

Serum potassium <3mEq/L give total of 1 mEq/kg (up to adult dose)
Serum potassium 3-3.5 mEq/L give 0.2 mEq/kg and recheck

Obtain i-STAT labs, including glucose.

Analgesia:
morphine 0.05-0.1 mg/kg IV
OR
fentanyl 1 μg/kg IV

Ondansetron:
2 mg < 15 kg
4 mg > 15 kg
8 mg > 30 kg

Early Surgical Consult

Diagnostic Imaging

Ceftriaxone:
50 mg/kg intravenous or intramuscular every 12 hours (max 2000 mg)
PLUS
Metronidazole:
10 mg/kg intravenous every 8 hours (max 500 mg)

Pip-Tazo:
Age 2 months to 9 months: 80 mg/kg/dose (based on piperacillin component) intravenous every 8 hours (maximum dose: 3000 mg piperacillin)
Age >9 months, children, and adolescents weighing <40 kg: 100 mg/kg/dose (based on piperacillin component) intravenous every 8 hours (maximum dose: 3000 mg piperacillin)
Children and adolescents weighing >40 kg: 3000 mg piperacillin intravenous every 6 hours

Question 2

Pathophys / Key concepts of Hirschsprung’s

Answer 2

Congenital aganglionic segment of the colon -> neurogenic dysfunction
-> impeded peristaltic function -> obstruction

Lengthier colonic involvement presents in infancy BUT shorter segments can present in childhood

Question 3

Complications of Hirschsprung’s

Answer 3

Enterocolitis

Anyone with Hirschsprung’s and abdominal pain need to be worked up for enterocolitis

Toxic Megacolon

Question 4

Risk Factors for Enterocolitis

Answer 4

Hirschprung’s

Enterocolitis is the leading cause of morbidity and mortality for Hirschsprung’s

Can present even post surgery (usually within a year)

Down’s

Question 5

History and Physical

Answer 5

History of constipation since birth

Failure to pass meconium in the first 48 hrs

Minimal pain, distended abdomen, empty rectal vault

Foul Smelling Stool

+/- Vomiting, Explosive Diarrhea, Lethargy, Rectal Bleeding, Spetic shock

Question 6

Investigations

Answer 6

CBC
Lytes
CRP
Blood Cultures

2 View Abdominal XRAY: assess for obstruction, fecal impaction, perforations

Question 7

Disposition: Uncomplicated Hirschsprung’s Disease

Answer 7

Discharge Home

Follow up with Gastroenterology

Question 8

Disposition: Hirschsprung’s Enterocolitis

Answer 8

Management as above

Early surgical consult

Admit to monitored bed

Question 9

Disposition: Uncomplicated Hirschsprung’s Disease

Answer 9

Discharge Home

Follow up with Gastroenterology