Pastor et al reported a score for the diagnosis of Hirschsprung-Associated Enterocolitis (HAEC). The authors are from the Hospital for Sick Children in Toronto, University of Toronto, C.S> Mott Children's Hospital in Ann Arbor and Women and Children's Hospital in Buffalo.
Patient selection: pediatric patient with Hirschsprung disease
Parameters:
(1) history (4 questions)
(2) physical examination (5 questions)
(3) radiologic exam of the abdomen (5 questions)
(4) laboratory findings (2 questions)
Parameter
Finding
Points
diarrhea with explosive stool
no
0
yes
2
diarrhea with foul-smelling stool
no
0
yes
2
diarrhea with bloody stool
no
0
yes
1
history of enterocolitis
no
0
yes
1
explosive discharge of gas and stool on rectal exam
no
0
yes
2
distended abdomen
no
0
yes
2
decreased peripheral perfusion
no
0
yes
1
lethargy
no
0
yes
1
fever
no
0
yes
1
multiple air-fluid levels on X-rays
no
0
yes
1
dilated loops of bowel
no
0
yes
1
sawtooth appearance with irregular mucosal lining
no
0
yes
1
cutoff sign in the rectosigmoid colon with an absence of distal air
no
0
yes
1
pneumatosis
no
0
yes
1
leukocytosis
no
0
yes
1
left shift in the differential count
no
0
yes
1
total score =
= SUM(points for all 16 parameters)
Interpretation:
• minimum score: 0
• maximum score: 20
• A score >= 10 is associated with HAEC.
• A mild case of HAEC may have a score less than 10.
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