Abstract

 Diffuse intestinal ganglioneuromatosis is hamartomatous polyposis distinguished by a scattered, intramural or transmural proliferation of neural elements involving the enteric plexuses. This condition may involve any segment of the gastrointestinal tract, but the ileum, colon, and appendix are most frequently affected. It is a rare, benign neoplastic condition that has a well known association with multiple endocrine neoplasia type 2b, Cowden syndrome and a rare but documented association with neurofibromatosis type 1. We reported the case of a female patient's history of a neurofibromatosis who presented with chronic diarrhea. On the CT scan of the abdomen, thickening of the wall up to 2 cm, air-fluid leveling and dilatation reaching up to 6 cm at its maximum in the ileal segments and mesentery lymphadenopathies and intraperitoneal effusion were reported. There was not any peculiarity except ileal oedema and erythema on the colonoscopic examination. Endoscopic biopsy specimens obtained from the terminal ileum showed acute inflammation without any definitive findings of Crohn’s disease. Due to intestinal obstruction, the patient underwent surgical resection. Diffuse ganglioneuromatosis was observed in the resected specimen.

Keywords: Intestinal Ganglioneuromatosis; Neurofibromatosis; Diarrhea; Crohn's Disease

References

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Citation

Citation: Erkan Çağlar., et al. “A Study on the Challenges of Diagnosis and Interpretation in the Subject with Intestinal Ganglioneuromatosis". Acta Scientific Gastrointestinal Disorders 3.10 (2020): 14-16.

Copyright

Copyright: © 2020 Erkan Çağlar., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.