Diaphragm Disease of the Terminal Ileum: The Great Imitator.

Non-steroidal anti-inflammatory drugs (NSAIDs) are associated with an array of intestinal injuries: erosions, ulcers, enteropathy, strictures and diaphragm disease. The diagnosis of diaphragm disease is challenging. Diaphragm disease can cause thin, concentric and stenosing strictures, which can induce intermittent or complete bowel obstruction. NSAID-induced lesions are reversible following discontinuation of the offending agent. Treatment of diaphragm disease can be conservative, endoscopic or surgical through stricturoplasty and/or segmental resection. We report a case of a 59-year-old female presenting with intermittent right lower quadrant pain diagnosed with diaphragm disease upon combined ileo-colonoscopy and histopathological analysis. Her diaphragm disease was successfully treated conservatively through drug cessation, avoiding more invasive procedures like endoscopic and surgical interventions. LEARNING POINTS: The incidence of diaphragm disease has been soaring due to the widespread use of non-steroidal anti-inflammatory drugs (NSAIDs).Diaphragm disease is characterized by diaphragm-like mucosal projections and annular constrictions that induce luminal narrowing and result in bowel obstruction.Physicians should get acquainted with diaphragm disease and include it in their differential diagnosis when approaching a patient with obstruction-like symptoms or non-specific and vague abdominal pain in the setting of chronic NSAIDs usage.

Nonsteroidal anti-inflammatory drug-induced small bowel strictures (diaphragm disease) - an under-recognized cause of small bowel obstruction.

BACKGROUND: Small bowel obstruction is a major source of morbidity and mortality that carries a significant economic burden. Recurrent small bowel obstruction may be secondary to circumferential strictures (small bowel diaphragm disease), an under-recognized entity secondary to long-term nonsteroidal anti-inflammatory drug (NSAID) use. We aimed to describe the sensitivity of preoperative computed tomography (CT) enterography in patients with surgically treated small bowel diaphragm disease. METHODS: We retrospectively reviewed adult patients who underwent elective small bowel resection for small bowel obstruction performed by a single minimally invasive surgeon between 2010 and 2023. Patient history, radiographic, endoscopic, operative, and pathology reports were reviewed for reference to NSAID use, small bowel strictures, diaphragms, and enteropathy. Exclusion criteria were prior radiation, inflammatory bowel disease, malignancy, adhesive disease, and anastomotic strictures. RESULTS: A total of 225 patients were identified, 22 (10%) of whom met the inclusion criteria. The mean age was 60.7 years (range 29-78), with 15 women (68%). All patients underwent minimally invasive small bowel resection for obstruction with histopathologic evidence of stricture without evidence of transmural inflammation, granuloma, or dysplasia and confirmed NSAID use (n = 22, 100%). Anemia was present in 36% (n = 8). Preoperative CT or magnetic resonance (MR) enterography was performed in 18 patients (82%), of which stricturing was reported in 13 (72%). Intraoperatively, palpation identified strictures in all patients. CONCLUSION: NSAID-induced small bowel injury is an under-recognized condition that, in severe cases, can present as small bowel obstruction. Surgeons should consider diaphragm disease in patients with obstruction and NSAID use, in which preoperative CT or MR enterography may be useful but cannot rule out disease.

Non-steroidal anti-inflammatory drug induced intestinal stricturing: diaphragm disease.

Diaphragm disease (DD) is a rare small bowel enteropathy associated with non-steroidal anti-inflammatory drug use. Since the first description there have only been approximately 100 cases of DD reported in the literature. Stricturing webs or 'diaphragms' form in the bowel, causing non-specific abdominal symptoms that can ultimately lead to bleeding and obstruction. Diagnosis is notoriously challenging as there is no single gold standard investigation. We present two cases of DD both of which were ultimately diagnosed by surgical resection. We also propose a novel flow algorithm that can be utilized for working up patients with suspected DD.

A rare presentation of idiopathic small bowel diaphragm disease - A case report.

INTRODUCTION: Diaphragm disease, typically associated with long-term non-steroidal anti-inflammatory drug (NSAID) use, manifests as diaphragm-like small bowel strictures, often resulting in bowel obstruction. CASE DESCRIPTION: A 75-year-old male presented with features of recurrent subacute intestinal obstruction, later diagnosed with multiple small bowel strictures via CT imaging. Surgical intervention, including resection and anastomosis, was performed to alleviate the obstruction. Histopathological examination of the resected specimen confirmed diaphragm disease, challenging its traditional association with NSAID use. DISCUSSION: Diaphragm disease, characterized by mucosal and submucosal diaphragm-like strictures, is typically attributed to NSAID usage. However, this case underscores the possibility of diaphragm disease in the absence of NSAID exposure. Pathological findings supported the presence of diaphragm-like strictures, despite the patient's denial of NSAID use. CONCLUSION: This case emphasizes the importance of considering diaphragm disease as a differential diagnosis in patients with intermittent bowel obstruction, even in the absence of NSAID history.

Idiopathic Small Bowel Diaphragm Disease Without Use of Non-steroidal Anti-inflammatory Drugs: A Case Report and Review of the Literature.

Small bowel diaphragm disease is a rare condition that is characterized by the presence of diaphragm-like strictures that causes intermittent or complete small bowel obstruction. Most cases are asymptomatic until presented with severe abdominal pain due to small bowel obstruction or diagnosed during anemia workup as a cause of occult gastrointestinal bleeding. Small bowel diaphragm disease is usually associated with long-term use of non-steroidal anti-inflammatory drugs (NSAIDs). Here, we present the case of a 50-year-old male with no history of NSAID use who presented with abdominal pain and iron deficiency anemia. He was postoperatively diagnosed with idiopathic small bowel diaphragm disease.

Endoscopy Capsule Retention in a Young Female with Small Bowel Strictures Secondary to Non-Steroidal Anti-Inflammatory Drugs.

Non-steroidal anti-inflammatory drugs (NSAIDs) can cause small bowel damage, which could present in different ways, including abdominal pain and occult gastrointestinal bleeding. NSAID use can also result in small bowel strictures, which can be challenging to diagnose and manage. Here, we describe a case of a 49-year-old female who presented with chronic anaemia and intermittent abdominal pain, with a history of NSAID use. She underwent capsule endoscopy as part of the workup for anaemia and subsequently had capsule retention due to a small bowel stricture.

Diaphragm Disease of the Small Bowel Presenting With Intussusception.

Diaphragm disease of the small bowel is an uncommon condition with nonspecific symptoms, which causes strictures of the small bowel associated with non-steroidal anti-inflammatory drug (NSAID) use. Due to the nature of the disease process and the strictures it can form, patients often present with a clinical picture suggestive of small bowel obstruction, and the true diagnosis is not confirmed until histopathological examination.  In this article, we present the case of a 73-year-old female with chronic NSAID use and gastrointestinal complaints who had undergone multiple endoscopic procedures which failed to identify the cause of her symptoms. Further investigation with video capsule endoscopy and CT enterography led to a diagnosis of intussusception believed to be caused by a small bowel mass. Retention of the video capsule endoscope prompted the decision to undertake diagnostic laparoscopy with push endoscopy and direct visualization of a string of small bowel strictures in the area of intussusception. This characteristic appearance of the bowel was then confirmed by pathology as diaphragm disease lesions. Diagnosis of this disorder is difficult due to its rarity and common symptoms that make other disease processes seem more probable. Early diagnosis can prompt counseling on cessation of NSAID use and interventions to decrease the risk of complications that may require surgical intervention. Physicians should be able to recognize diaphragm disease of the small bowel as a differential in patients presenting with obstructive bowel symptoms and even rare cases of intussusception in the setting of chronic NSAID use.

Diaphragm disease associated with nonsteroidal anti-inflammatory drugs mimicking intestinal tumor: A case report.

INTRODUCTION: Diaphragm disease is rare and caused by intestinal obstruction due to nonsteroidal anti-inflammatory drugs (NSAIDs). Given the availability of video capsule endoscopy (VCE) and balloon enteroscopy (BE) this disease will be diagnosed more often. PRESENTATION OF CASE: A 73-year-old man was presented to our hospital for persistent nausea and vomiting. Abdominal ultrasound and computed tomography revealed small-bowel thickening, stricture in the terminal ileum, and dilation of the proximal small intestine. Differential diagnosis included ileal lymphoma and multiple ileal adenocarcinomas, and a diagnostic laparoscopy was performed. Twenty-centimeter of ileum was resected by primary ileo-ileal anastomosis. On pathological examination, fibrosis of the submucosa was identified, and erosions and numerous inflammatory cells reaching the submucosa were also identified from the specimen. DISCUSSION: The preoperative diagnosis of diaphragm disease is sometimes challenging due to its uncharacteristic symptoms; moreover, radiological findings are usually indefinite and distinctive. Currently, the main treatment for diaphragm disease is surgery. CONCLUSION: We have documented a case of intestinal obstruction by NSAIDs. However, it is desirable to determine the course of treatment based on small bowel endoscopic dilatation cases in the future.

Colonic diaphragm disease secondary to nonsteroidal anti-inflammatory drug use.

Diaphragm disease is a rare condition associated with the long-term use of nonsteroidal anti-inflammatory drugs (NSAIDs) and can lead to severe complications. Most strictures occur in the small bowel, and occurrence in the colon is rare. We report a case of an asymptomatic patient with colonic diaphragm disease secondary to NSAID use.

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.

Histopathologic Spectrum of Neuromuscular and Vascular Hamartoma With Special Reference to the Vasculopathic Phenomenon.

Neuromuscular and vascular hamartoma (NMVH) is an unusual lesion presenting as intestinal obstruction by stricture formation. It is characterized by a hamartomatous mass comprising haphazardly arranged mesenchymal tissue native to the intestinal mucosa and submucosa. We aimed to characterize the clinicohistopathological spectrum of NMVH in adult subjects with a search for an etiological clue. We reviewed 84 resected specimens (adult cases) of intestinal obstruction in our institute and diagnosed 4 cases with NMVH. A panel of special stains (Masson trichrome, Verhoeff-van-Gieson, and periodic acid-Schiff) and immunohistochemistry (smooth muscle actin, S100, Bcl2, CD34, vimentin, desmin, CD117, and CD3) were performed in all cases. All cases of NMVH showed characteristic hamartomatous mounds comprising haphazardly arranged smooth muscle, nerves, ganglia, vessels, and collagen with overlying mucosal ulceration. Adjacent bowel showed submucosal fibrosis, muscularis mucosae thickening, and duplication along with vasculopathy. A typical vasculopathy ("vessel-in-vessel" appearance) was seen in the submucosal and/or subserosal veins. Besides, different other forms of vasculopathic changes like obliterative venopathy and concentric myohypertrophy were also seen. One case had vasculitis and the patient died despite successful surgery. One other case was associated with lymphocytic ganglioneuronitis and granulomatous etiology. We conclude that NMVH can be multifactorial in origin although ischemia resulting from vasculopathy appears to be directly causative. The characteristic vasculopathy in the submucosal location may aid in the diagnosis of NMVH in small biopsy samples.

A Vasculitis-Associated Neuromuscular and Vascular Hamartoma Presenting as a Fatal Form of Abdominal Cocoon.

Neuromuscular and vascular hamartoma (NMVH), also known as neuromesenchymal hamartoma, is a rare hamartomatous condition of the intestine. It usually presents with submucosal humps protruding in the intestinal lumen causing obstructive features. The other clinical manifestations are hematochezia or melena and protein-losing enteropathy. The etiopathogenesis of these lesions is not well known, although an association with small bowel Cröhn's disease and diaphragm disease had been postulated, the latter related to chronic nonsteroidal anti-inflammatory drug intake. Only 24 cases of NMVH are reported in the English literature and all of them could be adequately cured by resection of the affected part of the bowel. Moreover, none of these cases presented with abdominal cocoon or showed any evidence of vasculitis. We describe a peculiar case of NMVH in a 45-year-old male who presented with abdominal cocoon with symptomatic recurrence and fatal outcome within a month of surgery. Histopathology revealed classical histomorphology of NMVH with evidence of vasculitis. This appears to be the first case of a fatal form of NMVH, presenting with abdominal cocoon and associated with vasculitis.

Diaphragm Disease: NSAID-Induced Small Bowel Stricture.

The use of nonsteroidal anti-inflammatory drugs (NSAIDs) is ubiquitous. However, it remains an oftentimes under-recognized risk factor for the development of strictures and small bowel obstruction. Herein we describe the case of a 63-year-old female with a prolonged course of abdominal pain and occult anemia found to have a diaphragmatic stricture in the small bowel related to chronic NSAID use.

Small Bowel Obstruction due to Non-steroidal Anti-inflammatory Drug-induced Diaphragm Disease: A Case Report.

Small bowel obstruction (SBO) is a common illness encountered by general surgeons. However, obstruction caused by diaphragm disease induced by non-steroidal anti-inflammatory drug (NSAID) is exceedingly rare. The diagnosis is challenging as the signs and symptoms are neither sensitive nor specific. We report the case of a 59-year-old male who presented with SBO secondary to this uncommon condition. We hope to raise awareness of this unusual entity.

Review article: a practical approach to the clinical management of NSAID enteropathy.

Co-prescription of acid suppressive therapy, together with advances in small bowel imaging techniques, have shifted the burden of NSAID-related toxicity from gastro-duodenal to more distal small bowel injury. Due to predominantly subclinical disease, NSAID enteropathy remains under-recognised, with an incidence of 53-80% amongst healthy short-term users, and a prevalence of 50-71% following long-term (>3 months) use. Despite their distinct pathogenesis, those at risk of NSAID-related gastro-duodenal and small bowel complications share several risk factors. Clinical complications of NSAID enteropathy such as protein-losing enteropathy, small bowel strictures and diaphragm disease, confer significant morbidity, and are often irreversible. Small bowel prophylaxis has proven of modest efficacy after short-term, high-dose NSAID use in asymptomatic patients. While selective COX-2 inhibitors are associated with fewer gastro-duodenal complications relative to non-selective NSAIDs, their comparative benefit in protecting against small bowel enteropathy remains unclear. Prophylaxis should be considered in those at high risk of small bowel complications, as treatment options for established disease remain limited; however, the optimal agent remains unclear. We propose a clinical algorithm that may help prevent, monitor, investigate, and manage the sequelae of NSAID-induced small bowel toxicity.

Retained enteroscopy capsule in a patient with diaphragm disease.

INTRODUCTION: Diaphragm disease is a rare consequence of small-bowel enteropathy, and usually occurs as a result of longstanding ingestion of non-steroidal anti-inflammatory drugs. DD is characterized by multiple strictures and saccular dilatations leading to symptoms of subacute intestinal obstruction. Often, the diagnosis is made on histological examination after laparotomy and resection of diseased small bowel. CASE HISTORY: Here, we report a case of an elderly female who suffered for many years from chronic, colicky abdominal pain and anaemia due to undiagnosed diaphragm disease. Eventually, she was referred to our surgical team because of a retained enteroscopy capsule. The diagnosis was made after laparotomy and bowel resection. This surgical intervention alleviated chronic symptoms, and the patient remained well at 1-year follow-up. CONCLUSIONS: This case highlights the difficulty of diagnosing diaphragm disease without laparotomy and bowel resection. A high index of suspicion must be exercised in any patient with chronic, colicky abdominal pain and anaemia together with multiple strictures and saccular dilatations on computed tomography even in the absence of longstanding NSAID ingestion. Moreover, capsule enteroscopy should be avoided as a diagnostic modality of small-bowel disease if computed tomography raises the suspicion of strictures.

Diaphragm disease of the small intestine: an interesting case report.

Diaphragm disease of small intestine usually presents with nonspecific clinical features. Radiological investigations often fail to differentiate it from small intestinal tumors and inflammatory bowel disease. It is therefore diagnosed on final histology after surgical resection. We hereby report an interesting case of a suspected small bowel tumor later diagnosed as diaphragm disease on histology.