Rare cause of small bowel obstruction: diaphragm disease
- Peter Foulser ,
- Alhamza Al-Khatib ,
- Simon Moodie and
- Philippa Youd
- Department of Gastroenterology, Epsom and Saint Helier University Hospitals NHS Trust Epsom Hospital, Epsom, UK
- Correspondence to Dr Peter Foulser; pfgfoulser@hotmail.com
Abstract
Diaphragm disease is a rare cause of small bowel obstruction, caused by circular fibrotic membranes resulting in a narrowed intestinal lumen. It is associated with prolonged non-steroidal anti-inflammatory drug use, and often requires surgical resection. We report the case of a man in his 50s presenting with recurrent anaemia and intermittent small bowel obstruction. Exploratory laparoscopy identified three distinct areas of congested, thickened and narrowed ileum. Symptoms resolved following small bowel resection. Histological examination found elongated mucosal folds with ulceration and submucosal fibrosis consistent with diaphragm disease. Common radiological findings include small bowel strictures and thickening, mucosal hyperenhancement, and small bowel dilatation. In this case, on retrospective review of the initial CT scan, it is possible to appreciate circumferential mural thickening correlating with the histological findings. This case highlights the importance of rigorous examination of CT imaging and the utility of exploratory laparoscopy in diagnosing diaphragm disease.
Background
Diaphragm disease is a rare cause of small bowel obstruction. It is characterised by the development of discrete circumferential small bowel strictures, which can cause small bowel obstruction due to a stenosis of the lumen.1–4 The clinical presentation is most often with abdominal pain, weight loss, vomiting, diarrhoea and anaemia.5 It was originally and is most commonly associated with prior use of non-steroidal anti-inflammatory drugs (NSAID), however, has also been linked with coeliac disease, oral potassium supplements, radiotherapy and eosinophilic gastroenteritis.1
Case presentation
A man in his 50s attended the general practitioner with intermittent abdominal pain and dyspepsia. He had a background of gastro-oesophageal reflux disease for which he took regular cimetidine, lansoprazole and amitriptyline. He had no known drug allergies, and was otherwise fit and well. There was no reported weight loss. Blood tests demonstrated a microcytic hypochromic iron deficiency anaemia (haemoglobin (Hb) 94 g/L, MCV 81 fL, ferritin 9 μg/L). Baseline blood tests were otherwise normal. Examination was unremarkable.
The patient was referred to the gastroenterology department via the cancer referral pathway. He reported fluctuating abdominal pain requiring the use of regular codeine and tramadol, with intermittent episodes of vomiting. Gastroduodenoscopy and colonoscopy were normal other than a small hiatus hernia. CT of the abdomen demonstrated mildly dilated loops of small bowel involving the proximal ileum. There was no obstructing small bowel lesion. The findings suggested an incomplete small bowel obstruction. He settled spontaneously.
When seen in the general surgical clinic 4 weeks later, his abdominal pain had improved, the vomiting had subsided and he was opening his bowels regularly. MRI of the small bowel showed normal thickness of the small intestine with no lesions and no evidence of obstruction. Laparoscopy was put on hold. Instead he was referred for a video capsule endoscopy to assess for small bowel ulceration and lesions, and planned initially for a patency capsule due to the risk of capsule obstruction.
On arrival for the patency capsule, he reported fatigue and breathlessness. Blood tests demonstrated a worsening anaemia (Hb 70 g/L, MCV 69 fL). He was transfused two units of blood and commenced oral iron supplementation. The patency capsule remained in the small bowel at 30 hours, so he was unable to have a video capsule endoscopy due to risk of capsule obstruction. He was not clinically obstructed at that point.
Repeat CT scan 4 months after the first demonstrated normal small bowel. Fluorodeoxyglucose-positron emission tomography showed no significant uptake in the small bowel, no enlarged lymph nodes and no signs of malignancy.
He remained under a watchful-wait programme. He continued to have intermittent and self-resolving central abdominal pain, occasionally associated with vomiting and intermittent diarrhoea. Over 2 months, he again became more anaemic, with Hb dropping from 94 g/L to 76 g/L, necessitating an iron infusion.
He was referred back to the surgical team who performed a laparoscopy. Three distinct areas of congested, thickened and narrowed ileum were identified. A 29 cm small bowel resection was performed with primary anastomosis, removing all three narrowed segments. One month postoperativel,y he was well, with no abdominal pain or vomiting and a normal bowel pattern. He remains well and asymptomatic with a normal Hb 18 months postoperatively.
Histological examination demonstrated multiple elongated mucosal folds with ulceration and submucosal fibrosis, with minimal chronic inflammatory cell infiltrate. There were no features of Crohn’s disease, eosinophilia, dysplasia or malignancy. These findings were in keeping with a diagnosis of diaphragm disease of the small bowel.
Discussion
This case highlights the diagnostic challenge when faced with a patient with intermittent small bowel obstruction. In our case, the underlying diagnosis of diaphragm disease was only found following laparoscopy and small bowel resection. Diagnosing diaphragm disease using non-invasive imaging techniques can be challenging, as the lesions may often be small and therefore appear normal, or the mucosal diaphragms may appear like valvulae conniventes.6 Even during laparotomy, the external appearance of the bowel may be normal, necessitating palpation for thickened segments or intraoperative enteroscopy.7
The pathophysiology of diaphragm disease is incompletely understood, and is most commonly seen in patients with a prior history of NSAID use, which are known to cause both upper and lower gastrointestinal tract strictures.7 It can affect both the small and large bowel, and has been reported in 2% of patients taking long-term NSAIDs or COX-2 inhibitors.8
A review of CT findings in this disorder demonstrated that the most common features were strictures, small bowel thickening, mucosal hyperenhancement, prominent mesenteric lymph nodes and small bowel dilatation.5 The same study found that use of video capsule endoscopy was associated with a high rate of capsule retention (67%).5
Intraluminally, the diaphragms appear as short concentric fibrotic bands, interspersed with normal bowel. Mucosal diaphragms have a wide array of histological features, typically with a focal chronic injury to the mucosa with inflammatory cell infiltrate.1 Further features include eosinophilic enteritis, inflammatory fibroid polyps, villous atrophy and neuromuscular and vascular hamartoma-like changes.
A retrospective review of seven cases of diaphragm disease found that all patients had subacute obstructive symptoms or gastrointestinal blood loss, and all had prior use of NSAIDs.7 Oesophagogastroduodenoscopy, colonoscopy and radiological investigations were normal in six patients, with the diagnosis made during videocapsule endoscopy during which the capsule was retained in the obstructed bowel, necessitating operative extraction. Surgical resection was performed in all seven patients, with resolution of symptoms.
In our case, on retrospective review of the initial CT scan, it is possible to appreciate the circumferential mural thickening relating to the operative and histological findings (figure 1A–C). The use of a video capsule endoscopy would likely have led to capsule obstruction necessitating emergency surgery.
(A, B) Coronal CT images demonstrating dilated small bowel loops, with two areas of circumferential mural thickening in the ileum (arrows) consistent with mucosal diaphragms seen intra-operatively. (C) Axial CT image demonstrating the same area of circumferentially thickened ileum.
Our patient had none of the known risk factors for diaphragm disease. He denied significant prior use of NSAIDs and coeliac screen was negative.
This case highlights the importance of rigorous examination of CT imaging, and the value of direct visualisation of the bowel with exploratory laparoscopy in the diagnosis and management of diaphragm disease.
Learning points
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Diaphragm disease is a rare cause of small bowel obstruction and anaemia, requiring a high index of suspicion for diagnosis.
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It is most commonly associated with a prior history of non-steroidal anti-inflammatory drug use.
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The most common symptoms and signs are abdominal pain, weight loss, vomiting, diarrhoea and anaemia.
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It can be successfully treated with small bowel resection.
Ethics statements
Patient consent for publication
Footnotes
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Contributors PF is first author. AA-K edited the manuscript, and provided images and radiology analysis. SM edited the manuscript. PY edited and oversaw the writing of the manuscript and analysis.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.
References
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