Inflammatory changes secondary to postoperative complications of Hirschsprung’s disease as a cause of histopathologic changes typical of intestinal neuronal dysplasia
Section snippets
Materials and methods
From 1995 through 2002, 36 cases of HD were diagnosed and treated at the Department of Pediatric Surgery at Juntendo University School of Medicine. Twenty-five patients were boys and 11 were girls. Their age at the time of diagnosis ranged from 4 days to 4 years.
Thirty one had rectosigmoid HD, and 5 had long-segment HD. Two had Down’s syndrome. H&E staining of frozen sections obtained at the time of definitive surgery confirmed there was normal distribution of ganglion cells at the proximal
Results
The most proximal margin of all resected specimens was examined, and all but 2 were confirmed to have normal histology on H&E staining and AchE immunohistochemistry. Both had typical findings of IND.
Discussion
Unlike HD, which is a multigenic disorder, the pathogenesis of IND remains unclear. Its etiology is unresolved, with diverse histopathologic mechanisms including developmental disturbances, reactive changes, or inflammatory diseases being discussed.
Data about combined malformations involving IND are limited, and the pathogenesis of IND is not yet understood fully, so an analysis of associated malformations and morbidity may shed light on how and why intestinal dysganglionosis develops. On
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Reoperative surgery for Hirschsprung disease
2012, Seminars in Pediatric SurgeryCitation Excerpt :The specimen must be examined for the presence of hypertrophied nerves at this level, suggesting transition zone pathology, which may not function normally. Moreover, in rare instances, intestinal neuronal dysplasia (IND) may also be present at this site.30,31 As many key decisions regarding a reoperation are dependent on the pathologic interpretation, the authors find reviewing these specimens in person with the pathologist quite useful.
Development of a standardized definition for Hirschsprung's-associated enterocolitis: a Delphi analysis
2009, Journal of Pediatric SurgeryAdvantages of intraoperative semiquantitative evaluation of myenteric nervous plexuses in patients with Hirschsprung disease
2007, Journal of Pediatric SurgeryCitation Excerpt :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 [9-13]. Other studies concluded that abnormalities resembling neuronal dysplasia associated with HD are probably secondary and do not require surgical resection [14-16]. 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.
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2004, Journal of the American College of SurgeonsChallenges in the diagnosis of intestinal neuronal dysplasia type B: A look beyond the number of ganglion cells
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