Inflammatory changes secondary to postoperative complications of Hirschsprung’s disease as a cause of histopathologic changes typical of intestinal neuronal dysplasia

Development of a standardized definition for Hirschsprung's-associated enterocolitis: a Delphi analysis

“…Hirschsprung's-associated enterocolitis seems to be more common in children with longer segment disease [3,[13][14][15]. There is also evidence that children who develop HAEC are more likely to have recurrent episodes [5,16,17] and that intestinal neuronal dysplasia is a risk factor for the development of HAEC [16,18]. Postoperative complications and presence of anastomotic stricture and development of intestinal obstruction may also be associated with enterocolitis [19,20].…”

“…Such nonspecific symptoms can easily be overlooked and misdiagnosed as gastroenteritis, leading to either a missed diagnosis or delay in diagnosis. Several authors suggested HAEC is limited to the ganglionic segment [26], whereas other authors demonstrated that the histologic features of HAEC affect both ganglionic and aganglionic segments [16]. Lack of a clear definition for HAEC has made it extremely difficult to evaluate the literature and compare surgical interventions and management strategies in which HAEC is a significant outcome measure.…”

Advantages of intraoperative semiquantitative evaluation of myenteric nervous plexuses in patients with Hirschsprung disease

“…Abnormalities resembling neuronal dysplasia have been observed above the segment affected by HD and are thought to be responsible for the persistence of occlusive symptoms after resection [9][10][11][12][13]. Other studies concluded that abnormalities resembling neuronal dysplasia associated with HD are probably secondary and do not require surgical resection [14][15][16]. Immunohistochemically detected abnormalities of neurofilaments [17], synapses [18], smooth muscle actin [19], or Cajal cells [20] have been blamed for the postoperative persistence of occlusive symptoms.…”

Challenges in the diagnosis of intestinal neuronal dysplasia type B: A look beyond the number of ganglion cells

“…Since then, the association between IND-B and HD has been described with rates ranging from 6% to 75% of HD cases[ 38 , 51 , 52 ]. In these cases, the presence of colonic segments with IND-B can be explained both by primary embryological alteration of the ENS, which gives rise to neuropathies, and by secondary adaptation to distal intestinal obstruction, caused by the spastic aganglionic intestinal segment[ 22 , 53 ].…”