Neuromuscolar and vascular hamartoma of the duodenum. A clinical case and review of the literature


COD: 195-198 Categorie: ,

Francesco Minni, Alessandra Margiotta, Enrico Guerra, Nicola Marrano, Saverio Selva, Claudio Ricci, Tommaso Grottola, Donatella Santini

Ann. Ital. Chir., 2005; 76: 195-198

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BACKGROUND AND AIMS: The NMVH of the intestine is a rare lesion, typical located in the small bowel. After the first
2 cases, reported in 1982, other 12 cases have been described, ten localized in the jejunual or ileal tract, only 1 in
the cecum.
SUBJECTS AND METHODS: A case of NMVH of the duodenum, simulating a Crohn’s disease, is described. The lesion
extended about 12 cm. and it caused intestinal stenosis. The microscopic analysis on the operative specimen highlighted
the presence of a neuronal hyperplasia at mucous membranae and submucosal levels, associated to muscolaris mucosae
hypertrophy and disorganisation. Expanded and ectasic veins, proliferation of nervous fibres and ganglion cells in the
submucosal were also observed.
RESULTS: NMVH is a non-epithelial hamartomatous, submucosally based proliferation of mature submucosal elements
capable of causing small bowel obstruction. In the controversy about the nature of the NMVH, we consider it a separate
entity, where the final diagnosis depend on the clinicopathologic setting, as well as on the location and number of
the lesions.
CONCLUSIONS: The Authors analyse the main characteristics of this rare pathology with regard to clinical, diagnostic and
therapeutic aspects.