Neuronal intestinal dysplasia

Pistor, G.; Kap-herr, S Hofmann-von; Grüssner, R; Munakata, Keimei; Müntefering, Horst

doi:10.1007/bf00175648

Cited by 24 publications

(12 citation statements)

References 12 publications

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“…The quota of dysganglionosis diagnosed as cranial of aganglionosis lies between the value presented by Meier-Ruge (17) and the 52-65% of some other reports (8,21,26). The unspecific sex ratio in dysganglionosis confirmed former impressions that the male preponderance of aganglionosis is not shown in dysganglionosis.…”

Section: Discussionsupporting

confidence: 72%

“…No statistically significant difference was found between IND and agiND. included some follow-up information pointing out complications like long-term defecation disturbance, dysmotility and prolonged need for enterostomy (21,22). De Duonni presented the first 14 years foIlow-up investigation of 20 aglND and 14 isolated IND children on the c1inical situation of these children treated in the Children's Hospital of Zürich/Switzerland (8).…”

Section: Aganglionosis With Ind (Agind) 28mentioning

confidence: 99%

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Clinical Long-Term Follow-Up Results in Intestinal Neuronal Dysplasia (IND)

Schmittenbecher

Gluck²,

Wiebecke³

et al. 2000

Eur J Pediatr Surg

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Commonly available information on intestinal neuronal dysplasia (IND) is sparse. Especially well documented long-term courses are lacking. The aim of this study was to correlate defecation as a clinical parameter of the long-term course in malformations of the enteric nervous system with the morphological diagnosis. 57 children with intestinal neuronal dysplasia (IND) or aganglionosis with cranial intestinal dysganglionosis (agIND), diagnosed between 1983 and 1992, were analysed including histomorphological classification, collection of clinical data and evaluation of the defecation mode by questionnaire as a parameter of the long-term course. Of 29 dysganglionic (IND) patients, 9/29 cases (31 %) had been treated conservatively, 18/29 cases surgically (62.1%), in two children (6.9%) no therapy had been necessary. All 28 patients with Hirschsprung's disease and cranial IND (agIND) underwent resection. 46 of the children could be followed up 3.64 years after the end of the main therapeutic period and with a mean age of 6.7 years at the time of follow-up; 43.5% of the analysed children still showed severe constipation. 23.9% only were really cured; 15.2% had normal defecation still using conservative treatment and 17.4% had diarrhea. No significant difference was found between both groups, IND and agIND, and the results were independent of treatment modality. The results were much worse than in idiopathic constipation as reported in the literature and even worse in comparison to unselected Hirschsprung collectives. It has to be concluded that in IND with chronic constipation intensive long-term care is necessary and it is crucial that treatment algorithms should be outlined urgently together by pediatric gastroenterologists and pediatric surgeons. AgIND seems to need more extended resection following an exact histomorphological mapping by biopsies taken during enterostomy procedure.

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Section: Discussionsupporting

confidence: 72%

Section: Aganglionosis With Ind (Agind) 28mentioning

confidence: 99%

Clinical Long-Term Follow-Up Results in Intestinal Neuronal Dysplasia (IND)

Schmittenbecher

Gluck²,

Wiebecke³

et al. 2000

Eur J Pediatr Surg

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“…The AChE reaction was used in 83 patients and one or two additional dehydrogenase reactions in 60. Dierent supplemental investigations were performed, i.e., the S 100 reaction (20), NADPH (13), neuron-speci®c enolase (6), and others (4). In 10 cases AChE was used exclusively.…”

Section: Resultsmentioning

confidence: 99%

“…Nevertheless, these colostomies all functioned well. The adequate function of these colostomies may represent passive emptying of the colon, and does not prove sucient contractility of the remaining colon to pass stools through the aganglionic sphincter and remaining rectum after pull-through, however [19,20].…”

Section: Discussionmentioning

confidence: 99%

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Hirschsprung's disease and intestinal neuronal dysplasia - a frequent association with implications for the postoperative course

Schmittenbecher

Sacher

Cholewa

et al. 1999

Pediatric Surgery International

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Between 1991 and 1993, 106 newly diagnosed cases of Hirschsprung's disease (HD) were prospectively analyzed for the association of HD and intestinal neuronal dysplasia (IND) at ten pediatric surgical departments in central Europe. Hirschsprung-associated IND (HaIND) was found in 40% of cases. IND was disseminated in one-third and localized in two-thirds of the patients. Initial clinical symptoms were related to the length of the aganglionic segment, but not to the presence of HaIND. An enterostomy performed in 72 cases (67.9%) was located in a segment of pathologically innervated bowel in 50% of all cases, but in 72% of cases of HaIND. The proximal margin of the resected bowel showed pathological innervation in 44% of cases. Supplemental biopsies from the intestine (apart from diagnostic suction biopsies and biopsies at the enterostomy site) led to the first identification or definition of length of associated IND in 17.9% of cases. Postoperatively, the presence of long-segment aganglionosis or associated IND implied a delay in the restoration or normal defecation. Persistent constipation was found in 40% of patients with associated disseminated IND at follow-up at 6 months, compared to 20.6% in patients with isolated HD. These children needed secondary interventions more often than patients with associated localized IND or isolated HD. HaIND thus has clinical implications for the postoperative course if IND is disseminated.

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Intestinal Neuronal Dysplasia

Schofield,

Yunis

1991

J. pediatr. gastroenterol. nutr.

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SummaryFour hundred ninety‐eight acetylcholinesterase‐stained suction rectal biopsies from 456 children were examined to assess the occurrence of “isolated” or “primary” intestinal neuronal dysplasia at Children's Hospital of Pittsburgh. Cases of proven Hirschsprung's disease were excluded. In 38 biopsies from 38 patients, we found a mild to moderate increase in mucosal acetyl‐cholinesterase staining and abundant submucosal ganglion cells (large, irregular ganglia or at least five ganglia per HPF). This group was clinically heterogeneous with a frequent history of prematurity and small left colon/meconium plug syndrome, protein/formula intolerance, or obstructive anatomic gastrointestinal abnormalities. We feel that isolated “intestinal neuronal dysplasia,” as diagnosed by suction rectal biopsy and the above criteria, is a descriptive biopsy appearance. Rather than characterizing a unique disease entity, it is encountered in a variety of clinical situations. Based on our observations and review of the literature, we therefore conclude that “intestinal neuronal dysplasia” not associated with Hirschsprung's disease, neurofibromatosis, or multiple endocrine neoplasia syndrome has yet to be well defined in clinical and histopathologic terms. The histologic diagnosis, at this time, should be reserved for those rare, florid cases of parasympathetic hyperganglionosis that are documented by adequate tissue sampling.

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Neuronal intestinal dysplasia

Cited by 24 publications

References 12 publications

Clinical Long-Term Follow-Up Results in Intestinal Neuronal Dysplasia (IND)

Clinical Long-Term Follow-Up Results in Intestinal Neuronal Dysplasia (IND)

Hirschsprung's disease and intestinal neuronal dysplasia - a frequent association with implications for the postoperative course

Intestinal Neuronal Dysplasia

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