Histopathology of Diaphragm Disease of the Small Intestine

Abstract

Diaphragm disease (DD) is an uncommon gastrointestinal abnormality typical of nonsteroidal anti-inflammatory drug (NSAID)-induced injury. DD of the small intestine is well defined in its gross appearance (multiple circumferential stenosing lesions); however, its histologic features have been studied in few cases. To better define and update the histologic features of DD, we describe 10 cases in which patients underwent resection of portions of small intestine for DD. Selected gross features and 12 microscopic features were assessed. The typical gross appearance of DD was associated with focal chronic injury of the mucosa with mild to moderate inflammatory infiltrate. A wide array of additional abnormalities was found: eosinophilic enteritis (3 cases), inflammatory fibroid polyp (1 case), enteritis cystica profunda (1 case), villous atrophy (1 case), and neuromuscular and vascular hamartoma-like changes (9 cases). DD and, therefore, NSAID-related injury, should be considered in the differential diagnosis of several conditions affecting the small intestine.

Subacute small bowel obstruction due to diaphragm disease

“…6 A more recent review of 10 clinical cases observed at a single institution concluded that DD comprises a wide range of histological findings, and these included thickening and chaotic arrangement of muscular bundles in the muscularis mucosae, fibrosis of the lamina propriae, invariable mucosal ulceration at the apex of the diaphragm, numerous vessels, ganglion cells, nerve fibers and eosinophils in the mucosa. 7 An identical histology has also been reported in the condition called neuromuscular and vascular hamartoma, which is much rarer than NSAID-induced DD and commonly presents as small bowel obstruction. 8,9 It is not known currently whether the two described conditions actually belong to the same nosological spectrum.…”

Small bowel diaphragm disease mimicking malignancy

“…This made surgery mandatory, as florid locoregional lymphadenopathy has not been previously described in diaphragm disease 6 11. There has been one previous report of diaphragm disease being misdiagnosed with malignancy, however, in this patient there was ongoing treatment for malignancy and imaging showed only minor mesenteric lymph node enlargement 12.…”

“…The mechanism of small bowel injury by NSAIDs is believed to be a microvascular injury causing a reduction in villous circulation leading to epithelial cell damage. This allows luminal aggressors, enteral bacteria, their products and bile to gain access to the mucosa, causing inflammation 6. Circumferential mucosal ulcers develop.…”

Diaphragm Disease of the Small Intestine

“…Therefore, most of such cases are still diagnosed on histology after surgical resection. [6][7][8] Our case was successfully treated and diagnosis was made on final histology.…”