Long-term follow-up of patients with intestinal neuronal dysplasia type B

Lourenção, Pedro Luiz Toledo de Arruda; Ortolan, Érika Veruska Paiva; Rosa, Laura Luiza Minelli; Angelini, Marcos Curcio; Terra, Simone Antunes; Rodrigues, Maria Aparecida Marchesan

doi:10.1097/md.0000000000007485

Cited by 4 publications

(4 citation statements)

References 30 publications

(41 reference statements)

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“…The mean maximum number of days without bowel movements was 9.07 ± 6.24, with a median of 7 (1–25) days. Except for patients who had an intestinal obstruction in the neonatal period and one patient who had incomplete information in the medical record, all the other 23 patients met the Rome IV criteria for the diagnosis of constipation [ 17 , 18 ]. The pre-treatment ISI had a mean of 3.47 ± 1.34 and a median of 3.0 (1.0/6.0) [ 19 , 20 ].…”

Section: Resultsmentioning

confidence: 99%

“…Factor 1, responsible for most of the described variations, was composed of histopathological variables and some clinical variables that can be considered severe ones, such as the need for bowel lavage and rectal bleeding. Factor 2, on the other hand, was composed of the main clinical symptoms presented by patients with IND-B, including the intestinal symptom index (ISI), an index proposed to summarize the most common signs and symptoms in the clinical picture of IND-B [ 18 , 19 ].…”

Section: Discussionmentioning

confidence: 99%

“…In addition, we obtained specific information on bowel habits present at the time of diagnosis of IND-B, mainly related to symptoms of constipation, including bowel movement frequency, stool consistency, painful or straining bowel movements, bowel bleeding, abdominal pain, the presence of fecalomas, need for bowel washing, and fecal incontinence. These data allowed us to assess the presence of each of the six Rome IV criteria (RIV C) [ 33 , 34 ] for the diagnosis of constipation in children (RIV C1: ≤2 bowel movements per week; RIV C2: >1 episode of fecal incontinence per week if toiled trained; RIV C3: retentive posturing or excessive volitional stool retention; RIV C4: painful or hard bowel movements; RIV C5: large fecal mass present in the rectum; RIV C6: history of large diameter stools that can obstruct the toilet) and also to apply the intestinal symptom index (ISI), which is an index proposed to summarize the most common signs and symptoms in the clinical course of IND-B [ 18 , 19 ].…”

Section: Methodsmentioning

confidence: 99%

“…Two therapeutic modalities have been used in patients with IND-B: conservative clinical therapy and surgical treatment [ 7 , 18 , 19 ]. Conservative therapy is composed of changes in diet, laxatives, and enemas in cases of fecal retention [ 4 , 19 , 20 ].…”

Section: Introductionmentioning

confidence: 99%

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Association between Clinical and Histopathological Findings in Intestinal Neuronal Dysplasia Type B: An Advance towards Its Definition as a Disease

Gonçalves

Oliveira

Filho

et al. 2023

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Introduction: Intestinal neuronal dysplasia type B (IND-B) is a controversial entity that affects the submucosal nerve plexus of the distal intestine. The lack of definition of the causal relationship between histological findings and clinical symptoms has been identified as the primary point to be elucidated in the scientific investigation related to IND-B, which is essential for it to be considered a disease. Objective: To investigate the relationship between histopathological findings and symptoms in a series of patients with IND-B. Methods: Twenty-seven patients with histopathological diagnosis of IND-B, according to the Frankfurt Consensus (1990), who underwent surgical treatment through colorectal resections were included. Data from medical records regarding the clinical picture of the patients at the time of diagnosis, including the intestinal symptom index (ISI) and a detailed histopathological analysis of the rectal specimens, were retrieved. Exploratory factor analysis was performed, applying the principal components method for clusters with Varimax rotation. Results: Two factors were determined: the first, determined by histopathological and clinical variables, and the second, composed of the main symptoms presented in patients with IND-B, including ISI. Factorial rotation showed the association between the two factors and, through a graph, demonstrated the proximity between ISI values and histopathological alterations. Conclusion: There was evidence of an association between the clinical features presented by patients with IND-B and the histopathological findings of the rectal samples. These results support the understanding of IND-B as a disease.

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