He was underwent laparotomy which showed a mildly distended small bowel, and a remarkable distention and bulking of the terminal ileal loop; however, the ileum was blindly ended and it was bulging into the cecum. Nevertheless, the length of the small bowel was found to be normal; there was no defect in the mesentery (Fig. 3). The intraoperative diagnosis was an isolated ileocecal valve atresia. The dilated bulky part of the terminal ileum was resected along with the atretic segment (Figs. 4 and 5) and an ileocolic anastomosis was performed. The postoperative period was uneventful.
The resected ileal segment was sent to pathology. Gross examination demonstrated a common wall separating the ileum from the cecum indicative of an atresia of the ileocecal valve itself. The ileocecal valve was thickened, completely obstructed. Serial section of the ileoceacal valve showed complete obstruction of the lumen with fibrosis thickening. The microscopic examination showed an ileal mucosa on one side of the obstructed valve and a colonic mucosa on the other side and there was mild submucosal and subserosal congestion.
Intestinal atresia is a common cause of bowel obstruction in the newborn and it can occur at any point in the gastrointestinal tract. Ileocecal valve atresia is a rare entity with only 10 cases having been reported in the English literature 5–12.
Numerous variants of the atresia have been described including failure of development of the entire ileocecal region to a membranous obstruction of the valve with the presence of the appendix 6,7. Our reported case represents the variation of fibrous thickening of the ileocecal valve and complete membranous obstruction with normal looking ileocecal junction, normal mesentery, and presence of the appendix.
The reported cases presented as neonatal intestinal obstruction which include abdominal distension, bilious vomiting, and failure to meconium. The diagnosis of the previously reported cases of illiocecal valve atresia was made intraoperatively because of lack of specific preoperative radiological findings. However, the diagnosis in our case was suspected preoperatively as the contrast study showed intestinal obstruction at the level of the ileoceacal valve. The contrast enema showed microcolon with no reflux of contrast material into the terminal ileum, delineating the obstructed ileocecal valve. The obstructed part of the ilium at the ileocecal valve was bulging in the cecum. This radiological finding was not demonstrated in the previous reported cases.
Different surgical modalities were proposed for the reported ileocecal valve atresia cases depending on the intraoperative findings. Cacciari et al. 7 resected the middle part of the atretic ileocecal valve followed by ileocecal valve repair. In our case, the small bowel had a normal length so we performed a resection of the atretic segment followed by an ileocolonic anastomosis, the same surgical approach was applied by others authors 5,8,9,12. We think that, as in all cases of intestinal atresia, the bulbous dilated preanastomotic bowel should be removed to avoid a longue postoperative ileus due to malfunctioning of this segment.
The reported outcomes of these different cases were good in our case as well.
This paper sheds the light on this rare entity and adds to the very small body of the literature.
It is unlikely to make a preoperative diagnosis of ileocecal valve atresia; however, a proper barium enema may make the diagnosis suspected. Therefore, the intraoperative examination of the intraluminal portion of the ileocecal region is mandatory for the diagnosis of these cases.
There are no conflicts of interest.
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