Pattern of Calretinin Staining in Hirschsprung Disease; Comparison of Aganglionic Segment to the Transition Zone and Ganglionic Colon.
Mojgan Hosseini, Aliya N Husain. University of Chicago, IL
Background: Hirschsprung disease (HD) is a congenital disorder characterized by absence of ganglion cells involving variable lengths of the colon leading to intestinal dysmotility. It is diagnosed by suction rectal biopsy and is definitively treated by resection. Calretinin immunohistochemical stain is recently described to be useful in its diagnosis. The purpose of this study is to describe the pattern of calretinin staining in nerves and ganglion cells in resected specimens for HD.
Design: A search of archives from 2007-2009, revealed 22 cases of biopsy proven HD with subsequent endorectal pull through. Patients were between 11 days to 17 months old at the time of surgery. Length of aganglionic segment ranged from 3 to 95 cm. Sections representing distal margin, transition zone and proximal margin were identified and slides were immunostained with calretinin.
Results: Distal aganglionic zone lacked any immunostaining except for 3 cases which showed very weak, punctate staining of nerve fibers. Within the transition zone, calretinin staining grew stronger in the nerve fibers in a distal to proximal manner. Darkly staining ganglion cells were present in variable numbers in the transition zone. At the proximal resection margin both ganglion cells and nerve fibers were positive for calretinin. 17 cases showed differential staining of the ganglion cell in the proximal ganglionic zone in that darkly staining ganglion cells were present in the same cluster as non- or weakly staining ganglion cells. Available follow up information in 8 cases indicates that resection has been therapeutic. In the normal ganglionic zone, nerve fibers in 20 cases were strongly positive, 2 cases were weakly positive. In addition mast cells stained strongly with calretinin.
Conclusions: Aganglionic segment in HD can have weak positive calretinin staining of nerve fibers (14% of cases). Based on the limited follow up data, it does not appear that the differential staining of ganglion cells at the proximal margin is an indication of dysfunction. However the relationship of differential staining of ganglion cells at the proximal margin to the function of residual colon needs to be further studied. Positive staining of mast cells with calretinin is a potential diagnostic pitfall.
Monday, February 28, 2011 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 85, Monday Morning