Intestinal neuronal dysplasia type A

Rajalakshmi, T; Makhija, Purnima; Babu, M. K.; Kini, Usha

doi:10.1007/bf02723812

Cited by 10 publications

(4 citation statements)

References 7 publications

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“…AChE stain revealed clusters of ganglion cells in the submucosa, showing negatively stained nuclei and deeply stained black cytoplasm along with pattern A staining with increased staining of the fibers in the muscularis mucosae, which were going up for a short distance into the lamina propria. IND shows a false positive pattern with AChE, which is well documented in literature [ 21 ]. This case met with the revised criteria of IND laid by Meier Ruge [ 22 ] in 2006–1) A minimum of 25 submucosal ganglia must be analyzed 2) More than 20 % of submucosal ganglia must be giant ganglia 3) A giant ganglia should contain >8 nerve cells per cross section.…”

Section: Discussionsupporting

confidence: 57%

Acetylcholinesterase histochemistry (AChE) - A helpful technique in the diagnosis and in aiding the operative procedures of Hirschsprung disease

et al. 2015

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BackgroundHirschsprung’s disease (HD) is an anomaly characterized by the absence of myenteric and submucosal ganglion cells (GC) in the distal alimentary tract. Diagnosis of HD is made by the absence of GC and missing out on even a single ganglion cell can be very devastating. Acetylcholinesterase (AChE) histochemistry, done on frozen sections is said to be a very useful ancillary technique in the diagnosis and in aiding the operative procedures of HD.MethodsTo assess this, 73 samples from 42 suspected/known cases of HD were subjected to frozen section analysis with rapid haematoxylin and eosin, toluidin blue stain along with AChE histochemistry. The remnant sample was paraffin embedded for routine haematoxylin and eosin staining.ResultsOn frozen section analysis, 33 samples showed absence of ganglion cells, AChE histochemistry showed a positive staining pattern in 17 samples and paraffin embedded routine, H&E stained sections showed absence of ganglion cells in 19 samples. Sensitivity and specificity of both tests ie frozen section rapid H&E/AChE histochemistry in the diagnosis of HD, were calculated taking paraffin embedded H&E stained sections as the gold standard. Sensitivity of frozen section rapid H&E in the diagnosis of HD is 57.57 % and specificity is 79.10 %. The p-value is <0.0001, which is significant. The sensitivity of AChE histochemistry in the diagnosis of HD is 90.47 % and specificity is 96.36 %. The p-value is <0.0001, which is significant.ConclusionsAcetylcholineesterase (AChE) histochemistry is a very useful ancillary technique in the diagnosis and in aiding the operative procedures of HD. It acts as a double check in the diagnosis of HD.

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Section: Discussionsupporting

confidence: 57%

Acetylcholinesterase histochemistry (AChE) - A helpful technique in the diagnosis and in aiding the operative procedures of Hirschsprung disease

et al. 2015

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“…Thus, prompt surgical treatment is needed [7]. Without timely surgical intervention, the condition may deteriorate rapidly and lead to death [8]. The clinicopathologic fi ndings in IND types A & B are shown in the Table 1, as compared to our case.…”

Section: Discussionmentioning

confidence: 59%

Antenatal sonographic features of intestinal neuronal dysplasia type A associated with polydactyly and micromelia

Son

Jung

et al. 2012

Korean J Obstet Gynecol

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Type A intestinal neuronal dysplasia is a congenital abnormality that is a very rare disease. Here, we report on a patient who had intestinal dilatation with serial changes and polydactyly, as shown on prenatal ultrasound. Bowel obstruction symptoms were shown at 16 days of life. An open abdominal surgery was performed. Malrotation of the bowel and narrowing of the area from 15 cm above the ileocecal valve were noted. Therefore, a right hemicolectomy, including lesions was performed. The patient was diagnosed with type A intestinal neuronal dysplasia by pathology.

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“…Furthermore, A ganglioneuroma of the stomach is extremely unusual, and in this case surgery was done because of the described severe gastric outlet obstruction which was most probably caused by a combination of muscular mural hypertrophy and hyperplasia of the myenteric plexus leading to stricture formation. 6,8…”

Section: Discussionmentioning

confidence: 99%

Sporadic diffuse gastric ganglioneuromatosis causing gastric outlet obstruction in an adult patient: A rare case report and literature review

Banik¹,

B.M²,

Bijjaragi³

et al. 2022

ACHR

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: Gastrointestinal ganglioneuromatosis is a rare benign neural neoplasm, consists of hyperplasia of nerve fibers, ganglion cells, and schwann cells in the wall of stomach and intestine. The disease may affect any part of the gastrointestinal tract but involvement of stomach is extremely unusual. Mainly are of three types: polypoid ganglioneuromas, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. To our knowledge, only two cases were reported as diffuse ganglioneuromatosis located in the stomach.: 27-years male patient, admitted with complaint of dysphagia for both solid and liquid and recurrent non-bilious vomiting since 1 week underwent Billroth - 1 procedure and antral segment of stomach was sent for histopathological examination. A histopathological diagnosis of Diffuse Gastric Ganglioneuromatosis with muscular mural hypertrophy was made. The presence of ganglion cells and positive staining for S-100 and NSE confirms the diagnosis.Obstructive gastrointestinal symptoms with a possibility of diffuse ganglioneuromatosis is mainly a disease of young children and infant. But an adult patient, which is an unusual age group, presenting with obstructive gastrointestinal symptoms, the rare possibility of Diffuse ganglioneuromatosis and intestinal neuronal dysplasia should be considered and investigated in order to avoid unnecessary and extensive surgical intervention.

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Intestinal neuronal dysplasia type A

Cited by 10 publications

References 7 publications

Acetylcholinesterase histochemistry (AChE) - A helpful technique in the diagnosis and in aiding the operative procedures of Hirschsprung disease

Acetylcholinesterase histochemistry (AChE) - A helpful technique in the diagnosis and in aiding the operative procedures of Hirschsprung disease

Antenatal sonographic features of intestinal neuronal dysplasia type A associated with polydactyly and micromelia

Sporadic diffuse gastric ganglioneuromatosis causing gastric outlet obstruction in an adult patient: A rare case report and literature review

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