| Author |
Age, sex of patient |
Clinical features |
Diagnostic tests and results |
Treatment and outcome |
| Naish, Capper and Brown 1960 [11] |
36, male |
Abdominal pain, abdominal distension, visible and audible peristalsis, steatorrhoea |
Laparotomy and biopsy of jejunal wall showed thickened inner muscular coat. |
A gluten-free diet was given. Further attacks of pseudo-obstruction occurred and were successfully treated with intravenous fluids and intestinal suction. |
| Nahai 1969 [12] |
19, female |
Two-year history of abdominal pain and distension, borborygmi, flatulence and steatorrhoea. The patient also experienced weight loss, ankle swelling and dyspnoea on exertion |
Barium meal and radiographs showed grossly dilated small bowel loops up to 10 cm in diameter and fluid levels. Diagnostic laparotomy revealed distended small bowel loops. Full-thickness biopsy of small bowel showed hypertrophy of the inner circular and outer longitudinal muscular coats. |
Treatment included antibiotics (ampicillin), drip and suck, parenteral feeding, jejunal and ileal enterostomies. The patient was symptomless postoperatively. |
| Pelizzo et al. 2013 [13] |
14, female |
Abdominal distension and severe dehydration |
Abdominal radiograph showed small and large bowel dilatation. Exploratory laparoscopy revealed dilatation of ascending colon and terminal ileum. Full-thickness biopsies of the ileum and colon were performed. Immunohistochemistry revealed decreased expression of α-actin in the circular layer of the small bowel. |
An ileostomy was performed. A diagnosis of Ehlers-Danlos syndrome (classical type) was made following skin biopsy. |
| Küllmer et al. 2016 [14] |
84, male |
Abdominal pain and distension |
CT of the abdomen showed massive dilatation of the small intestine and colon. |
Treatment included laxatives, prokinetic drugs, endoscopic decompression and percutaneous endoscopic caecostomy (PEC). Postoperative death due to pneumonia was reported. |
