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Total Colonic Hirschsprung's Disease: The Hypermotility and Skin Rash Protocol
Alejandra Vilanova-Sanchez1,2, Monica Ivanov3, Devin R Halleran1
1Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States.
Insights
A new protocol for total colonic Hirschsprung's disease (TCHD) effectively manages hypermotility and perineal rash in young children. This approach minimizes symptoms and improves outcomes for patients, even those not toilet trained.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Dermatology
Background:
- Total colonic Hirschsprung's disease (TCHD) poses significant postoperative challenges, including frequent stools and severe perineal rash.
- These issues are particularly problematic in younger, non-toilet-trained children.
Purpose of the Study:
- To introduce and evaluate a protocol developed by pediatric surgeons and ostomy nurses.
- The protocol aims to prevent and treat hypermotility and perineal rash in TCHD patients.
Main Methods:
- A retrospective review of 25 TCHD patients' charts from 2014 to 2017.
- All patients received a standardized protocol for managing hypermotility and perineal rash.
- Outcomes assessed included bowel movement frequency, perineal skin status, and growth.
Main Results:
- Nineteen of 25 patients were not toilet trained; the mean bowel movement frequency was 4 per day.
- All patients experienced resolution of perineal rash and liquid stools within 3 months.
- Eleven patients presented with failure to thrive, and two older patients had severe proctalgia.
Conclusions:
- The implemented bowel management and perineal skin protocol effectively minimized common TCHD symptoms like multiple stools and rash.
- The study supports performing pull-through procedures for TCHD between 6 and 18 months of age.
- This protocol improves outcomes for TCHD patients, including those not toilet trained.
Introduction:
Total colonic Hirschsprung's disease (TCHD) presents a postoperative challenge due to multiple stools and perineal rash. We propose a protocol developed by pediatric surgeons and ostomy nurses to help prevent and treat hypermotility and severe perineal rash, especially in younger children who are not toilet trained.
Materials And Methods:
We retrospectively reviewed our TCHD patients' charts from 2014 to 2017. All patients received a prescribed protocol for the treatment of hypermotility and perineal rash. We describe patients who underwent their pull through before and after the age of urine toilet training, and assessed the number of bowel movements, the perineal skin status, and growth.
Results:
We treated 25 patients. Out of 25, 9 patients received a straight ileoanal pull through before the age of 18 months. Nine of 25 patients presented for a second opinion and had redo pull through. The remaining seven presented for bowel management after having a pull through at another institution. All these were treated following the hypermotility protocol. In total, 19 of 25 patients were not toilet trained. The mean number of bowel movements in all groups was 4 (3-5). All had a resolution of perineal rash and liquid stools after 3 months. Eleven of the 25 patients presented with failure to thrive. Two older patients experienced severe proctalgia requiring replacement of the ileostomy.
Conclusion:
TCHD patients who underwent definitive pull through had nine high incidence of multiple stool, perineal rash, and low growth. With the implementation of bowel management care to slow the stools and a perineal skin protocol to treat the skin, we believe that these symptoms can be minimized even in patients who are not toilet trained. Since the implementation of this protocol, we have changed our practice to perform the pull through in such patients between the age of 6 and 18 months.
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