title: Hirschsprung disease and use of calretinin in inadequate rectal suction biopsies.
abstract: BACKGROUND
  Up to 17% of all rectal suction biopsies performed in the evaluation of Hirschsprung disease are considered inadequate. In most instances, inadequate biopsies contain too little submucosa or are taken within the anal transition zone.
  
  
  OBJECTIVE
  To examine the utility of calretinin stain in the workup of inadequate biopsies for patients with clinical suspicion of Hirschsprung disease.
  
  
  METHODS
  A retrospective analysis was conducted of all rectal suction biopsies performed in the evaluation of Hirschsprung disease during the previous 12 years that were considered "inadequate." Seventeen cases were identified, and Hirschsprung disease status was determined by clinical or surgical follow-up. Immunohistochemistry for calretinin was performed for all cases containing columnar mucosa, which were evaluated without knowledge of clinical course.
  
  
  RESULTS
  All 12 patients without Hirschsprung disease had calretinin-positive nerve fibers in the lamina propria or muscularis mucosae, and all 5 patients with Hirschsprung disease had no calretinin staining of nerves.
  
  
  CONCLUSIONS
  In this retrospective series, calretinin immunohistochemistry correctly predicted outcome in all instances. Although the gold standard for the diagnosis of Hirschsprung disease in rectal suction biopsies remains the evaluation of ganglion cells in a hematoxylin-eosin staining with sufficient submucosa, calretinin immunohistochemistry is quite helpful in triaging further workup based on clinical suspicion.
authors.affiliation: Department of Pathology and Laboratory Medicine, Cleveland Clinic, Cleveland, Ohio 44195, USA. hernand3@ccf.org
authors.forename: David Hernandez
authors.initials: DH
authors.lastname: Gonzalo
authors.forename: Thomas
authors.initials: T
authors.lastname: Plesec
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000378:metabolism; D001707:Biopsy, Needle; D064032:Calbindin 2; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D006627:Hirschsprung Disease / Q000175:diagnosis* / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D007413:Intestinal Mucosa / Q000294:innervation / Q000378:metabolism / Q000473:pathology; D008297:Male; D009412:Nerve Fibers / Q000378:metabolism / Q000473:pathology; D012007:Rectum / Q000294:innervation / Q000378:metabolism / Q000473:pathology; D012189:Retrospective Studies; D064030:S100 Calcium Binding Protein G / Q000378:metabolism*
chemical_list: D015415:Biomarkers; C576764:CALB2 protein, human; D064032:Calbindin 2; D064030:S100 Calcium Binding Protein G