Intestinal Neuronal Dysplasia Type B in Adults: A Controversial Entity
A 71-year-old male patient presented to the surgical emergency department of our hospital complaining of abdominal pain and constipation. Basic laboratory tests on admission were within normal range. Clinical examination revealed abdominal distention, dehydration and no evidence of bowel sounds. Systemic and neurologic diseases as well as infections and malabsorption syndromes were excluded by medical history, physical and blood examination. From the patient's history, chronic constipation had achieved 20 points in the Wexner Constipation Scale (WCS) [] since his infancy. He had occasionally received treatment with laxatives and rectal enemas. His last surgery had been 10 years before and included sigmoidectomy. Histopathology analysis of the resected sigmoid colon showed a dilated bowel with no evidence of malignancy, and no other immunohistological analysis was done. The patient stated that the symptoms of constipation had never resolved, while during the last 6 months he had been admitted to hospital twice due to bowel obstruction and treated without surgery.\nDuring the last admission to our hospital, an abdominal X-ray showed a massively distended large bowel (fig. ), and an abdominal computed tomography scan did not reveal any mechanical obstruction (fig. ). Due to excess abdominal tenderness and radiological findings, it was decided that an exploratory laparotomy would be appropriate. During laparotomy (fig. ) no obvious mechanical cause was found and a subtotal colectomy and Hartmann's procedure was performed. The patient had an uneventful postoperative course and was discharged on the 12th postoperative day with a temporary ileostomy.\nIn the pathology department, gross examination showed that the colon and especially the cecum was distended and its mucosal surface appeared flattened, with a polypoid lesion measuring 2.5 cm found in the cecum. Microscopic examination and immunohistochemical study of the polypoid lesion proved that it was a villoglandular adenoma with low-grade dysplasia, and full-thickness analysis of the resected colon revealed enlarged myenteric and submucosal neurons as well as an increased number of giant cells. The nerve bundles appeared hypertrophic, containing increased numbers of thickened disorganized axons. A small number of isolated ganglion cells and giant submucosal ganglia (containing 7–15 ganglion cells) (fig. ) were also detected in the submucosa. Immunohistochemistry was applied by using antibodies against protein S-100, neuron-specific enolase, glial fibrillary acidic protein and synaptophysin. Protein S-100 and synaptophysin revealed an increase in ganglion cells, and glial fibrillary acidic protein staining showed increased nerve plexus around blood vessels. The diagnosis of IND was established.\nThree months later, during his second admission for restoration of bowel continuity, preoperative rectoscopy and rectal manometry showed no significant findings. During surgery for bowel continuity restoration, the upper third of the rectum was removed and an ileorectal (side to end) low anterior anastomosis to the posterior wall of the rectum 6 cm above the dentate line was performed. The patient was discharged on postoperative day 10 and during a 2-year follow-up has remained asymptomatic with normal bowel motility. The WCS score is 8 points, showing a significant difference compared to 20 points preoperatively.