Idiopathic small bowel diaphragm disease: a case report

Small bowel diaphragm disease is a rare condition usually associated with the prolonged use of non-steroidal anti-inflammatory drugs (NSAID) and that can be mistaken and treated as other pathologies. We describe a case of a 64-year-old man with a prolonged course of pain and subacute bowel obstructions, without any history of NSAID usage, found to have a multiple diaphragmatic stricture in the small bowel.

Diaphragm disease in emergency surgery

Background/Aims Diaphragm disease of the small bowel has been described in the literature over the last three decades. The pathognomonic characteristic of multiple circumferential stenosis is noted on gross examination of the bowel. It is a severe form of non-steroidal anti-inflammatory drug-induced enteropathy, often presenting as acute small bowel obstruction. A systematic review was performed to identify risk factors and patient outcomes in histologically-proven diaphragm disease of the small intestine in patients undergoing emergency operation for small bowel obstruction. Methods A comprehensive search was performed between January 1975 and March 2019 using relevant MeSH terms. Studies were chosen based on predefined inclusion criteria. Diaphragm disease of the small intestine was defined as macroscopically detected thin diaphragm-like mucosal folding inside the lumen of the bowel. The parameters assessed included patient characteristics, duration of use of non-steroidal anti-inflammatory drugs, type of emergency surgery performed, complications, recurrence, presentation and diagnosis of diaphragm disease. Results A total of 21 studies were analysed which included 17 case reports, one case series, and three retrospective comparative studies. Overall 29 patients with diaphragm disease of the small bowel were reported following emergency laparotomy for small bowel obstruction. Use of non-steroidal anti-inflammatory drugs was noted in all cases with an average duration of 3–5 years. All patients presented acutely with features of small bowel obstruction and had emergency laparotomy, except one who underwent laparoscopic resection. In the comparative studies patients were more likely to be female and to have been taking non-steroidal anti-inflammatory drugs for more than 7 years. Conclusions This is a rare disease, difficult to diagnose and often confirmed by the intra-operative macroscopic appearance of circumferential stenosis of the bowel. Risk factors for developing small bowel diaphragm disease include long-term use of non-steroidal anti-inflammatory drugs, and female gender. Patients with this disease are at increased risk of developing acute small bowel obstruction, so early identification is important.

Diaphragm disease of the terminal ileum presenting as acute small bowel obstruction

Diaphragm disease (DD) of the small bowel is a rarely reported complication of non-steroidal anti-inflammatory drug (NSAID) use, characterised by diaphragm-like strictures, most commonly in the ileum, causing varying degrees of obstruction. It typically presents in the elderly, over many years with non-specific symptoms. Diagnosis is challenging, the majority of cases relying on histopathology for confirmation. Treatment involves NSAID cessation and surgery through a combination of stricturoplasties and/or segmental resection. Very rarely DD presents as a surgical emergency. A case presenting as acute small bowel obstruction (SBO) is described, initially diagnosed as adhesions, later confirmed to be DD of the terminal ileum following histopathological examination. Given the widespread use of NSAIDs and an ageing population, it is likely the incidence of DD will increase. It is, therefore, important that surgeons are aware of this disease entity and consider it as a potential diagnosis in patients presenting with acute SBO.

Diaphragm Disease: A Paradox in the Intestine

Diaphragm disease is a paradoxical disease entity related entirely to the bowel and not having involvement of diaphragm muscles. It is the formation of strictures in the bowel secondary to submucosal fibrosis which looks like the diaphragm and hence the term “Diaphragm disease”. It is seen in chronic NSAIDS (Non-steroidal anti-inflammatory drugs) users. It is a rare entity, leading to intestinal obstruction as a severe complication. Diagnosis of diaphragm disease is a diagnosis of exclusion and mostly done intraoperatively in patients presenting with subacute intestinal obstruction because the symptoms are non-specific and no gold standard investigation is available to diagnose it. Grossly diaphragm disease is formation of strictures in the intestinal wall, leading to obstruction due to narrowing of the wall lumen. Serendipitously stasis of food in the stricturous segment leads to formation of an enterolith, thus leading to subacute intestinal obstruction. A chance finding seen in chronic NSAIDS user’s is formation of enterolith secondary to stricture formation due to diaphragm disease leading to subacute intestinal obstruction.