Total Colonic Hirschsprung's Disease: The Hypermotility and Skin Rash Protocol

Alejandra Vilanova-Sanchez; Monica Ivanov; Devin R. Halleran; Andrea Wagner; Carlos Albert Reck-Burneo; Brenda Ruth; Meghan FIsher; Hira Ahmad; Laura Weaver; Onnalisa Nash; Danielle Buker; Rebecca Maria Rentea; Erin Hoover; Tassiana Maloof; Richard J. Wood; Marc A. Levitt

doi:10.1055/s-0039-1694744

European Journal of Pediatric Surgery, Table of Contents

Eur J Pediatr Surg 2020; 30(04): 309-316
DOI: 10.1055/s-0039-1694744

Review Article

Georg Thieme Verlag KG Stuttgart · New York

Total Colonic Hirschsprung's Disease: The Hypermotility and Skin Rash Protocol

Alejandra Vilanova-Sanchez

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

²Department of Pediatric Surgery, University Hospital La Paz, Madrid, Spain

,

Monica Ivanov

³Department of Pediatric Surgery, Emergency Clinical Hospital for children “MS Curie,” Bucharest, Romania

,

Devin R. Halleran

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

,

Andrea Wagner

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

,

Carlos Albert Reck-Burneo

⁴Department of Pediatric Surgery, Medical University of Vienna, Wien, Austria

,

Brenda Ruth

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

,

Meghan FIsher

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

,

Hira Ahmad

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

,

Laura Weaver

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

,

Onnalisa Nash

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

,

Danielle Buker

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

,

Rebecca Maria Rentea

⁵Department of Pediatric Surgery, Children's Mercy Hospital, Kansas City, Missouri, United States

,

Erin Hoover

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

,

Tassiana Maloof

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

,

Richard J. Wood

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

,

Marc A. Levitt

¹Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States

› Author Affiliations

Abstract

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.

Keywords

total colonic Hirschsprung's disease - hypermotility - perineal rash - medical management

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References

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