Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention

Abstract

Hirschsprung-associated enterocolitis (HAEC) is a common and sometimes life-threatening complication of Hirschsprung disease (HD). Presenting either before or after definitive surgery for HD, HAEC may manifest clinically as abdominal distension and explosive diarrhea, along with emesis, fever, lethargy, and even shock. The pathogenesis of HAEC, the subject of ongoing research, likely involves a complex interplay between a dysfunctional enteric nervous system, abnormal mucin production, insufficient immunoglobulin secretion, and unbalanced intestinal microflora. Early recognition of HAEC and preventative practices, such as rectal washouts following a pull-through, can lead to improved outcomes. Treatment strategies for acute HAEC include timely resuscitation, colonic decompression, and antibiotics. Recurrent or persistent HAEC requires evaluation for mechanical obstruction or residual aganglionosis, and may require surgical treatment with posterior myotomy/myectomy or redo pull-through. This chapter describes the incidence, pathogenesis, treatment, and preventative strategies in management of HAEC.

Established and emerging concepts in Hirschsprung’s-associated enterocolitis

“…These measures include routine use of rectal irrigations in the post-operative period, long-term administration of oral metronidazole, use of pro-biotic therapy, and diverting enterostomy. Unfortunately, no definitive evidence to support routine use of these “preventive” measures exists[19]. …”

Bowel Function and Quality of Life After Transanal Endorectal Pull-through for Hirschsprung Disease

“…1 The surgical treatment involves removal of the aganglionic segment and anastomosis of the innervated proximal bowel to the anus. 2 Bowel dysfunction, particularly varying degrees of fecal incontinence, are observed after all operative techniques for HD, [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] although direct comparison of outcomes between series is challenging due to substantial variations in the methods used for the assessment of bowel function, length of follow-up, and inclusion criteria. During the past decades, 1-stage transanal endorectal pull-through (TEPT) has become one of the most commonly performed operations for HD.…”

Long none coding RNA HOTTIP/HOXA13 act as synergistic role by decreasing cell migration and proliferation in Hirschsprung disease

“…The absence of enteric neurons from variable lengths of the bowel in HSCR results from a failure of neural crest-derived cells to colonize the affected gut regions [25]. HSCR are regarded as a multigenic disorder and a lot of genes, as well as none coding genes, have been reported to be involved in the pathogenesis of HSCR.…”