Neuromuscular and Vascular Hamartoma of the Small Bowel: A Rare Cause of Intestinal Obstruction

Abstract

Neuromuscular and vascular hamartoma (NMVH) is a rare lesion arising chiefly in the small intestine. As it shares many of the histological features with other commonly occurring stricturous conditions of the small bowel, there is an ongoing debate whether it is truly hamartomatous or represents just a reactive condition. We are reporting a case of NMVH in the terminal ileum in a 32-year-old male who presented with symptoms of intestinal obstruction.

A Vasculitis-Associated Neuromuscular and Vascular Hamartoma Presenting as a Fatal Form of Abdominal Cocoon

“…17 In total, there are fewer than 25 sporadic cases of NMVH and neuromesenchymal hamartoma reported in the world literature involving predominantly the small intestine in adult patients, although large bowel involvement, occurrence within a Meckel’s diverticulum, and childhood presentation are also described in isolated case reports. 7,8,11,12,18-32 Our case has many unique features in contrast to these reports.…”

Neuromuscular and vascular hamartoma as an unusual cause of small bowel obstruction

“…These lesions demonstrate haphazard, aberrant fascicles of smooth muscle from the muscularis mucosae, bundles of non-myelinated nerve fibres with scattered abnormal ganglion cells, and hemangiomatous vascular channels [1] , [2] . Debate exists regarding the hamartomatous nature of this disorder, given that many of the features of NMVH are seen in a range of reactive conditions, including Crohn’s disease, ischaemic enteritis, radiation enteritis and NSAID-induced small bowel strictures (so-called ‘diaphragm disease’) [2] , [6] , [7] .…”

Neuromuscular and vascular hamartoma: A rare entity or special phase of Crohn's disease

“…Macroscopically, most of the cases show single or multiple strictures in the jejunum and ileum, including one case in the cecum . Characteristic histological findings include a haphazard distribution of fascicles of smooth muscle, vascular channels with thickened wall, thickened unmyelinated nerve bundles and S100‐positive, irregularly scattered ganglion cells . These findings are similar to those found in our patient.…”

“…Neuromuscular and vascular hamartoma (NMVH) of the small bowel is a very rare entity that causes intestinal strictures, resulted in recurrent small bowel obstruction or chronic gastrointestinal (GI) bleeding . It was first described by Fernando and McGovern in 1982 as a hamartomatous lesion, consisting of fascicles of smooth muscle derived from muscularis mucosa, bundles of unmyelinated nerve fibers with scattered ganglion cells and hemangiomatous vessels, that occurs focally within a segment of the small intestine.…”