Hirschsprung Disease

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In order to identify ganglion cells, one of two types of biopsies will be received:

  1. Full Thickness Colorectal Wall Biopsies: In definitive pull through and/or re-sectioned cases in the operating room, the surgeon may send full thickness colorectal wall biopsies to establish the level of aganglionosis. The initial specimens are usually for frozen section diagnosis. (see Hirschsprung for Frozen)
  2. Rectal Mucosal Biopsies: All biopsies should be fixed in formalin and submitted for STAT/same day processing (FNB protocol) if received before 2:00 p.m.  After that time submit for rush processing for the next morning (including Saturdays – notify the Saturday attending that a RUSH biopsy will need to be read).
  3. For all biopsy cases, request RUSH processing Stamp requisition with “Priority” stamp, enter RUSH request into Vantage, and notify front desk.

Resection cases should be reviewed either with Dr. Husain, one of the GI attendings, or the frozen section attending to make certain that you understand where the aganglionic segment is supposed to be located and how to accordingly demonstrate this.

  1. For a resection specimen, measure the length of the segment and external diameter / inner circumference at aganglionic segment (narrow) and at normal segment (dilated).
  2. Fix in formalin overnight. The specimen can be fixed intact or opened and pinned on wax.


  1. It is very important to know the length of the aganglionic segment (aka where the ganglion cells start/stop) – so sections must be taken at precise locations along the length of the colon.
  2. For short lengths of colon, transversely section the specimen and submit entirely, in order. You can either note the distance of the section from one end of the colon or you can divide the number of sections by total length in order to get an estimate.
  3. For longer lengths of colon, submit a transverse section (entire circumference) every 5 mm, noting the distance of the section from one end of the colon.
  4. You may put 2 pieces per cassette, but be sure to ink one to designate which is more distal to the other.
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