Histologic Criteria for the Diagnosis of Allied Diseases of Hirschsprung's Disease in Adults

Abstract

A few reports in the literature have discussed the histologic criteria for the diagnosis of allied diseases of Hirschsprung's disease in adults, and studies report that intestinal neuronal dysplasia (IND) in adults may develop from IND in infants. The aim of this study was to examine the differences between the histological findings of IND in infants and those in adults, and to assess whether allied diseases of Hirschsprung's disease (HD) in adults should be considered as congenital or acquired diseases. For these purposes, we studied nine adult patients with severe constipation, and an adult patient with acute intestinal obstruction. We routinely examined the patients using barium enema, anorectal manometry and rectal mucosal biopsy. However, in patients suspected of allied diseases, we carried out full-thickness rectal biopsies. In seven operated cases, we also examined resected intestines. The tissue samples were examined using AChE-staining, NADPH-diaphorase staining, HE-staining, and silver impregnation. Histologically, we diagnosed two males as having HD, two males as having IND, five patients (two males and three females) as having hypoganglionosis, and one female as having a degeneration of the intramural plexus. The following conclusions were drawn: 1) Inflammations such as ulcerative colitis or ischemic colitis may cause IND TYPE B in adults whose histological findings are similar to those generally seen in infants; 2) It is suggested that IND is closely related to hypoganglionosis; 3) In hypoganglionosis, a patient with findings of elevated AChE-positive nerve fibers in the mucosa and AChE-positive nerve fibers in an arterial wall may belong to a subtype of IND; 4) Most of the allied diseases of HD in adults may occur as an acquired disease, not as a congenital disease.

Surgery for right-sided colonic diverticulitis: results of a 10-year-observation period

“…Recent studies from Japan show an increased incidence of complicated right-sided diverticulitis, so that the dimension of resection needs to be discussed again [12]. Our own experiences in surgical therapy for right-sided diverticulitis are also supported by other authors [1,2,4,16,23].…”

“…The clinical picture of IND contains signs of intestinal obstruction of the low type. With the description of cases of IND in adult patients, the onset of IND symptoms has expanded from the first year of life to adulthood [16]. An isolated form of IND was described in nearly 60% of adult patients suffering from chronic constipation, and it is supposed that these patients may develop inflammatory bowel disease [17].…”

Novel Classification and Pathogenetic Analysis of Hypoganglionosis and Adult-Onset Hirschsprung’s Disease

“…Therefore, hypoganglionosis is usually defined by presence of small, sparse, myenteric ganglion cells compared with controls [19,20]. Diagnosis by this criterion might depend on subjective insight by pathologists because of lack of objective criteria.…”

A critical appraisal of the morphological criteria for diagnosing intestinal neuronal dysplasia type B

“…This last finding, characteristic of Hirschsprung's disease, is rarely discussed in intestinal neuronal dysplasia type B and can be considered as one of the criteria for its diagnosis. 8,14,36,[40][41][42][43] When comparing different age groups, based on the cutoff age of 1 year, no statistically significant differences were observed in the qualitative analysis of the morphological changes verified in the (Table 2). Despite evidence that the histopathological changes suggestive of immaturity could change with age, through maturation and apoptosis, 21,36,[44][45][46] our results showed that these changes were present in both age groups and call into question the need to establish an age cutoff for the diagnosis of intestinal neuronal dysplasia type B.…”