Indian Journal of Pathology and Microbiology
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Year : 2022  |  Volume : 65  |  Issue : 3  |  Page : 673-675

Isolated intestinal neuronal dysplasia- type B of ileum: A rare occurrence

1 Department of Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Pathology, Homi Bhabha Cancer Hospital/Mahamana Pandit Madan Mohan Malaviya Cancer Centre, Varanasi, Uttar Pradesh, India

Correspondence Address:
Pratishtha Sengar
Department of Pathology, Homi Bhabha Cancer Hospital/Mahamana Pandit Madan Mohan Malaviya Cancer Centre, Varanasi - 221 005, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_636_21

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Intestinal neuronal dysplasia type B in the gastrointestinal tract is a rare occurrence and may occur alone or in combination with Hirschsprung disease. Distal colon seems to be a frequent site for isolated IND-B cases; however, small bowel involvement is scarcely reported. We report a case of 9 years old boy presenting with features of intestinal pseudo-obstruction for 5 years. Exploratory laparotomy revealed narrowed distal ileum with huge proximal dilatation. Histopathology of the resected terminal ileum revealed giant submucosal ganglion, hyperplastic submucosal nerves, and ectopic ganglion cells in the lamina propria suggestive of IND-B. Although IND-B involving ileum in isolation is a rare occurrence, suspicion should be kept in cases of intestinal obstruction with minimal response to conventional treatment.

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