Treatment of Hirschsprungs

Currently the only form of treatment for Hirschprung’s is by surgical correction. Clinical observations of patients with Hirschsprung’s with Dr. Bo Lovvorn has illustrated the importance of the biopsies needed to determine the margins of the aganglionic abnormal bowel, and how patients manage their symptoms post resection. Below are de-identified photos by Dr. Lovvorn of the surgical resection of the abnormal bowel.

Though there may be an apparent abnormal bowel, it is important to do multiple biopsies to determine the transition zone of the disease. The transition zone (TZ) is where the ganglion cells begin to appear in pathology. Because 10% of patients still have no ganglion cells after this first TZ [J Kessman, 2006], it is important to take additional proximal biopsies to assure resection is before the TZ.

Three confirmation biopsies are taken. One with aganglionosis, one in the TZ, and one proximal to the TZ with normal ganglion present. Once the abnormal bowel has been identified, it needs to be mobilized for anorectal pull-through. The abnormal bowel is then pulled through the anus and reattached at the most proximal biopsy site.

Below is a comprehensive video describing Hirschsprung’s and the anorectal pull-through procedure

https://www.youtube.com/watch?v=nxeEkO1xf7c

This procedure is time-consuming and difficult for a child to handle the amount of anesthesia at just days of life. The procedure also comes with a painful recovery. Because it is not a completely sterile procedure (bowel is considered “dirty”), there is a higher risk for infection.

More research for Hirschsprung’s is needed to better understand development of the enteric nervous system so that other non-invasive treatments are possible to better the lives of the patients born with this disease.