Abstract
Approximately 40% of children with Hirschsprung's disease (HD) suffer from Hirschsprung's associated enterocolitis (HAEC) despite correct surgery. Disturbances of the intestinal microbiome may play a role. Treatment with probiotics based on individual analyses of the fecal microbiome has not been published for HD patients with recurrent HAEC yet. A boy with trisomy 21 received transanal pull-through at the age of 6 months for rectosigmoid HD. With four years, he suffered from recurrent episodes of HAEC. The fecal microbiome was measured during three healthy and three HAEC episodes by next-generation sequencing. The patient was started on daily probiotics for 3 months; the fecal microbiome was measured weekly. The fecal microbiome differed significantly between healthy and HAEC episodes. HAEC episodes were associated with significant decreases of Actinobacteria and significant increases of Bacteroidetes and Proteobacteria. Probiotic treatment led to a significant increase of alpha diversity and a significant increase of Bifidobacterium and Streptococcus as well as decreases of Rikenellaceae, Pseudobutyrivibrio, Blautia, and Lachnospiraceae. A longitudinal observation of the microbiome has never been performed following correction of Hirschsprung's disease. Probiotic treatment significantly changed the fecal microbiome; the alterations were not limited to strains contained in the administered probiotics.
Highlights
Hirschsprung’s disease (HD) is represented by a congenital segmental absence of the enteral nervous system in both the myenteric and submucosal plexus with variable proximal expression due to a failure of migration of neural crest cells during embryonic development [1]. e resulting intestinal obstruction is usually treated by surgical removal of the aganglionic bowel and a pull-through of unaffected ganglionic bowel.Despite correct endorectal pull-through for HD, up to 40% of the patients continue to suffer from Hirschsprung’s associated enterocolitis (HAEC) defined as a clinical condition with diarrhea, abdominal discomfort, fever, and eventually subsequent septic shock [2]
With reference to Hirschsprung’s disease, there are a limited number of reports describing disruptions of the intestinal microbiome in patients suffering from HAEC when compared to healthy Hirschsprung’s disease patients [3,4,5]
In a corrected HD patient, HAEC led to significant alterations of the fecal microbiome when compared to healthy episodes
Summary
Hirschsprung’s disease (HD) is represented by a congenital segmental absence of the enteral nervous system in both the myenteric and submucosal plexus with variable proximal expression due to a failure of migration of neural crest cells during embryonic development [1]. e resulting intestinal obstruction is usually treated by surgical removal of the aganglionic bowel and a pull-through of unaffected ganglionic bowel. Despite correct endorectal pull-through for HD, up to 40% of the patients continue to suffer from Hirschsprung’s associated enterocolitis (HAEC) defined as a clinical condition with diarrhea, abdominal discomfort, fever, and eventually subsequent septic shock [2]. With reference to Hirschsprung’s disease, there are a limited number of reports describing disruptions of the intestinal microbiome in patients suffering from HAEC when compared to healthy Hirschsprung’s disease patients [3,4,5]. Case Reports in Pediatrics intestinal microbiome may be associated with the development of HAEC. Neither has been studied whether or not treatment of Hirschsprung’s disease patients with probiotics alters the intestinal microbiome. Erefore, the aim of the present report was to describe the intestinal microbiome of a patient su ering from Hirschsprung’s disease during episodes with and without enterocolitis and during treatment with probiotics applying 16S rRNA gene next-generation sequencing
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