Enteric neuropathology of congenital intestinal obstruction: A case report

Abstract

Experimental evidence indicates that chronic mechanical sub-occlusion of the intestine may damage the enteric nervous system (ENS), although data in humans are lacking. We here describe the first case of enteric degenerative neuropathy related to a congenital obstruction of the gut. A 3-year and 9-mo old girl began to complain of vomiting, abdominal distension, constipation with air-fluid levels at plane abdominal radiology. Her subsequent medical history was characterized by 3 operations: the first showed dilated duodeno-jejunal loops in the absence of occlusive lesions; the second (2 years later) was performed to obtain full-thickness biopsies of the dilated intestinal loops and revealed hyperganglionosis at histopathology; the third (9 years after the hyperganglionosis was identified) disclosed a Ladd's band which was removed and the associated gut malrotation was corrected. Repeated intraoperative full-thickness biopsies showed enteric degenerative neuropathy along with reduced interstitial cells of Cajal network in dilated loops above the obstruction and a normal neuromuscular layer below the Ladd's band. One year after the latest surgery the patient tolerated oral feeding and did well, suggesting that congenital (partial) mechanical obstruction of the small bowel in humans can evoke progressive adaptive changes of the ENS which are similar to those found in animal models of intestinal mechanical occlusion. Such ENS changes mimic neuronal abnormalities observed in intestinal pseudo-obstruction.

Figure 1

Representative pictures showing clinical (A and B) and laparotomic views (C and D) observed in this case. Note (A) the hugely distended abdomen and (C and D) abnormally dilated loops located proximally to the removed Ladd’s band (not visible in these examples) at laparotomy. B illustrates the considerable deflation of the abdomen following surgery.

Figure 2

Representative examples illustrating the general neuronal marker NSE (A, B, C) and BCL-2 (D, E, F) immunoreactivities in the neuromuscular layer of a control subject (A, D), in the non dilated loop distal to the congenital obstruction of the patient (B, E) and in the dilated loop proximal to the congenital obstruction of the patient (C, F). Note the marked reduction of NSE and BCL-2 immunoreactivities in myenteric ganglion cells and nerve fibers targeting the muscular layer observed only in the dilated loop. Streptavidin biotin immunoperoxidase technique. Calibration bar (A-F): 20 μm. Compared to controls (G) and the non-dilated segment (H), myenteric neurons of the present case (representative example in I) showed chromatin clumping, cell body shrinkage and cytoplasmic vacuolization. Uranyl acetate and lead citrate staining, transmission electron microscopy. Calibration bar (G, H, I): 2 μm.