Clinical aspects of intestinal neuronal dysplasia

Montedónico, Sandra; Acevedo, Sergio; Fadda, B.

doi:10.1053/jpsu.2002.36720

Cited by 37 publications

(21 citation statements)

References 10 publications

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“…Although patients with Hirschsprung's disease (HD) usually present in the newborn period with delayed passage of meconium, bilious vomiting, obstruction and/or enterocolitis, a subset of patients present later in life with severe chronic constipation [2]. Similarly, patients with intestinal neuronal dysplasia (IND) can either present in the newborn period or later in childhood and the passage of meconium can be delayed or not [3,4].…”

Section: Introductionmentioning

confidence: 99%

Rectal suction biopsy in the workup of childhood chronic constipation: indications and diagnostic value

et al. 2006

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Section: Introductionmentioning

confidence: 99%

Rectal suction biopsy in the workup of childhood chronic constipation: indications and diagnostic value

et al. 2006

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“…Moreover, numerous authors have stressed the lack of correlation between the radiographic transition zone and the real level of aganglionosis, especially in long segment HD and total colonic aganglionosis [21][22][23]. Apart from that, findings in barium enema are non-specific in other forms of intestinal dysganglionosis and are therefore not useful in diagnosing conditions such as IND [7,10].…”

Section: Discussionmentioning

confidence: 99%

“…These children have intractable constipation and grossly slowed intestinal transit time. The incidence of isolated IND varies from 0.3 to 40% of all rectal suction biopsies in different centers [6][7][8][9]. The uncertainty regarding the incidence of IND has resulted from the considerable confusion regarding the essential diagnostic criteria.…”

Section: Introductionmentioning

confidence: 99%

“…However, these have proven to have a low sensitivity and specificity in Hirschsprung's disease, especially if performed during the newborn period and are not useful in the diagnosis of other intestinal dysganglionosis [7,10,12]. A rectal suction biopsy (RSB) is currently the gold standard in the diagnosis of intestinal dysganglionosis especially if they are stained with histochemical stainings [2,13].…”

Section: Introductionmentioning

confidence: 99%

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Histochemical staining of rectal suction biopsies as the first investigation in patients with chronic constipation

Montedónico

Piotrowska²,

Rolle³

et al. 2008

Pediatr Surg Int

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Rectal suction biopsy (RSB) is the gold standard diagnostic procedure for disorders of bowel motility. This study describes our experience with RSB stained with histochemistry as the first diagnostic approach in a large series of patients presenting with chronic constipation. Between 1993 and 2005, 766 children underwent RSB for persistent chronic constipation. The specimens were snap frozen, sectioned and stained with conventional hematoxylin and eosin (H&E) and with nicotinamide adenine dinucleotide phosphate diaphorase (NADPH-d) and acetylcholinesterase (AChE) histochemical stainings. Adequate amount of submucosa was present in 655 (85.5%) out of 766 cases and formed the basis of this study. RSB in 540 (82%) patients were reported as normal. Hirschsprung's disease was found in 47 (7.2%) patients with characteristic features of absence of ganglion cells, increased AChE activity in the lamina propria and muscularis mucosae, thick nerve fibers in the submucosa, and a lack of NADPH-d-positive fibers in muscularis mucosae. RSB in 59 (9%) patients presented features of intestinal neuronal dysplasia such as submucosal hyperganglionosis, giant ganglia, ectopic ganglia and increased AChE activity in lamina propria. Hypoganglionosis was suspected in nine (1.3%) children because of sparse or absent ganglion cells and low AChE and NAPDH-d activity in muscularis mucosae. Three patients (0.4%) developed bleeding following RSB, requiring diathermy of the bleeding point. Thus, we conclude that RSB is a simple and safe method when used as the first diagnostic approach in patients with chronic constipation. The combination of two histochemical stainings techniques provides a high level of accuracy in the diagnosis of intestinal dysganglionosis.

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“…Other morphological features, such as the presence of ectopic ganglion cells, increased acetylcholinesterase activity, ganglion cells with a "button" appearance and hypertrophy of the nerve trunks, are considered diagnostic criteria in some studies [9,10,16,68,79,91] . The criteria described by MeierRuge et al [22] (2004) and slightly altered by MeierRouge et al [5] [5,77,92] .…”

Section: Diagnosismentioning

confidence: 99%

Intestinal neuronal dysplasia type B: A still little known diagnosis for organic causes of intestinal chronic constipation

Lourenção¹,

Terra²,

Ortolan³

et al. 2016

WJGPT

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Clinical aspects of intestinal neuronal dysplasia

Cited by 37 publications

References 10 publications

Rectal suction biopsy in the workup of childhood chronic constipation: indications and diagnostic value

Rectal suction biopsy in the workup of childhood chronic constipation: indications and diagnostic value

Histochemical staining of rectal suction biopsies as the first investigation in patients with chronic constipation

Intestinal neuronal dysplasia type B: A still little known diagnosis for organic causes of intestinal chronic constipation

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