Incomplete intestinal obstruction as the possible main complaint in Behcet’s disease after surgery for recurrent abdominal aortic pseudoaneurysms: a case report and literature review
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Behcet’s disease (BD) is a systemic vasculitis characterized by oral and genital aphthosis, and ocular and skin lesions. The disease is involved in vascular, gastrointestinal, and central nervous systems. Vasculitis may exacerbate fatal problems, such as anastomotic pseudoaneurysms. If the mesenteric vessels are involved, severe abdominal symptoms such as intestinal obstruction may occur.
This case report describes a young female patient who suffered from BD with recurrent abdominal aortic pseudoaneurysms, as well as deep venous thrombosis and subsequent complications of incomplete intestinal obstruction. This patient first underwent stent grafting, which was followed by rupture of two newly formed anastomotic pseudoaneurysms within six months. Emergency open surgical repair (OSR) was then performed on the ruptured pseudoaneurysms. Thrombosis and incomplete ileus occurred five months after surgery. This case was unique due to the presence of incomplete intestinal obstruction being the possible main complaint for a patient with Behcet’s disease, and it is the first ever case to be reported.
Intestinal obstruction may present as the possible main complaint in BD. Careful and attentive strategy should be carried out to prevent fatal outcomes.
KeywordsBehcet’s disease Recurrent abdominal aortic pseudoaneurysm Incomplete intestinal obstruction
Computed tomographic angiogram
Intensive care unit
Open surgical repair
Behcet’s disease (BD) is a disease that involves complex multisystem disorders. However, the exact physiopathologic mechanism of BD remains unclear. Evidence shows that arterial and venous systems are involved in BD. Involvement of the venous systems results in the occurrence of thrombosis and superficial phlebitis. Involvement of the arterial systems result in aneurysm or pseudoaneurysm, stenosis, and occlusion . The pseudoaneurysm, which evolves quickly, is prone to rupture and can become life-threatening. Therefore, it is crucial to diagnose and manipulate this fatal condition early. If the mesenteric vessels are involved, severe abdominal symptoms such as intestinal obstruction may occur . However, such symptoms, especially those presenting as the main complaint, are easily misdiagnosed. Here, we report a rare case of a patient who presented with incomplete intestinal obstruction as the possible main complaint after a second surgery. We believe that an examination of this case will help the field make progress toward developing a common procedural strategy for treating such rare conditions.
A 28-year-old female Chinese patient presented with the onset of acute continuous right abdomen pain, nausea and vomiting at the emergency department. On admission, abdominal dynamic computed tomography (CT) with a multislice detector row CT scanner showed several air-fluid levels in the enteric cavity, and the diagnosis was considered to be ileus.
The patient was diagnosed with BD four years ago. She had received medications regularly, including immunosuppressive therapy with oral prednisone (60 mg/day) and cyclophosphamide (100 mg/day).
Discussion and conclusions
Here we demonstrated a case of a patient with a diagnosis of BD who presented with incomplete intestinal obstruction as the possible main complaint. To the best of our knowledge, this is the first report of the presence of incomplete intestinal obstruction after a second surgery for BD.
Recent studies have reported that HLA-B51 is associated with a more severe disease and is related to familial BD . Other studies have shown that polymorphism in the IL-21, IL-10, and IL-8 genes and in the tumour necrosis factor-(TNF-)alpha-1031C allele are correlated with the pathogenesis of BD [6, 7, 8, 9, 10, 11]. The role of the IL-23/IL-17 axis has been found to be associated with the onset of BD [12, 13]. Meanwhile, delayed-type hypersensitivity to infections such as streptococcus species (especially sanguis) has also been proposed to be an important factor in inducing BD . Hematological diseases such as coagulation abnormalities and platelet overactivity may play additional roles in the development of BD [15, 16, 17]. Nitric oxide (NO) accumulation after induction by interferon-gamma is also related to the activation of BD .
BD can be divided into two categories according to the pathological mechanism: occlusive and lesion. Although venous involvement is the most common vasculo-BD complication, Ketari et al. has been reported that arterial involvement is more common than venous involvement . The most severe complication is aneurysm formation and rupture. Approximately 60% of reported arterial lesions associated with BD are aneurysms . The aorta is the most commonly affected artery followed by the pulmonary artery. Aneurysms occur more frequently in the abdominal aorta than in the thoracic aorta [21, 22, 23].
Surgical repair is the standard treatment for aneurysms with dilatation, stenosis, or obstruction, while alternative treatments involve minimally invasive endovascular therapy for aneurysm repair [22, 24, 25]. Shen et al. reported that both open surgery and endovascular repair were safe and effective for treating aortic pseudoaneurysm in BD . Furthermore, Naganuma et al. reported that ulcer lesions in the ileum and colon, which could lead to intestinal obstruction, were found in surgical patients with intestinal BD .
Characteristics of lesion, treatment and outcome for patients complicated with intestinal obstruction from literature review
Treatment (graft size: mm & type)
38 yrs. male
Interposition (Polytetrafluoroethylene) 16 × 8, bifurcated
Incomplete Intestinal Obstruction
Prednisolone + Colchicines
31 yrs. male
Incomplete Intestinal Obstruction
Colchicines + Azathioprine
50 yrs. male
Complete Intestinal Obstruction
Considering the paucity of data available, the management of patients with intestinal obstruction should be individualized, and a multidisciplinary approach should be taken. Differential diagnosis and management are essential when addressing this fatal problem.
Technologies such as abdominal computed tomography and angiography are recommended resources when determining differential diagnosis. Finally, if intestinal obstruction is confirmed with the diagnosis, appropriate measures (surgical or nonsurgical treatment) to treat this disease should be taken.
We would like to express our sincere appreciation to Dr. Jinping Zhao and Dr. Ming Xu of the Department of Cardiothoracic Surgery for their valuable comments.
This study was supported by the National Natural Science Foundation of China (Z.P. 81560131) and Hubei Province Key Projects (Z.P. WJ2017Z008).
Availability of data and materials
All relevant data supporting the conclusions are contained within the article.
ZP designed this study and revised the manuscript. FJ & HX analyzed and interpreted the patient data regarding Behcet’s disease. FJ & ZP conducted the manuscript writing. ZP, FJ & HX treated the patient in the ICU. All authors read and approved the final manuscript.
Ethics approval and consent to participate
Consent for publication
Written consent was obtained from the patient’s father for the publication of this case report, because the patient was critically ill and her father was authorized to act as her guardian.
The authors declare that they have no competing interests.
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