Hirschsprung disease

Diagnosis and grading of Hirschsprung-associated enterocolitis (HAEC)

The following criteria have been proposed as a standardized, clinically relevant approach to the diagnosis of HAEC to help guide appropriate treatment.[11]Gosain A, Frykman PK, Cowles RA, et al. Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int. 2017 May 33(5):517-21. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5395325 http://www.ncbi.nlm.nih.gov/pubmed/28154902?tool=bestpractice.com

Possible HAEC (Grade I)

• Clinical history: anorexia, diarrhea

• Physical exam: mild abdominal distension

• Radiographic findings: normal or mild ileus gas pattern

Definite HAEC (Grade II)

• Clinical history: previous HAEC, explosive diarrhea, fevers, lethargy

• Physical exam: fever, tachycardia, abdominal distension, abdominal tenderness, explosive gas/stool on rectal exam

• Radiographic findings: ileus gas pattern, air/fluid levels, dilated loops of bowel, rectosigmoid cutoff

Severe HAEC (Grade III)

• Clinical history: obstipation, obtunded

• Physical exam: decreased peripheral perfusion, hypotension, altered mentation, marked abdominal distension, peritonitis

• Radiographic findings: pneumatosis, pneumoperitoneum