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A New Meaning to Butterflies in the Stomach

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... Secondary SEP, which is presented more commonly in previous reports, develops when a local or systemic factor triggers inflammatory cascades in the peritoneum (12). SEP is most commonly triggered by long-term peritoneal dialysis (PD) (16,17) but is also reported to be associated with other factors, including medications (18,19), infections (20,21), abdominal tumour surgeries and postoperative chemotherapy (4,5,9), peritoneal shunts (22), recurrent episodes of peritonitis (23) and autoimmune disorders (24,25). However, the pathophysiology of these various factors triggering SEP is not well studied with the exception of PD, which is referred to as the "two-hit" hypothesis (26,27). ...
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Sclerosing encapsulating peritonitis (SEP) is a rare cause of intestinal obstruction that is characterized by a thick greyish-white fibrotic membrane encasing the small bowel. The clinical features are typically nonspecific, and they occasionally present with recurrent episodes of incomplete or complete intestinal obstruction. The etiology of SEP remains unclear, and the diagnosis is often delayed mainly due to the lack of specific symptoms. Here, we first report a patient who suffered from SEP after treatment for malignant mesothelioma (MM) with tumor resection supplemented with hyperthermic intraperitoneal chemotherapy (HIPEC) once and intraperitoneal chemotherapy (IPC) eight times. The patient was discharged ten days after surgery and was free of complications at the 4-month follow-up. In addition, we reviewed the published literature from PubMed, only 7 articles of 16 cases finally met the defined requirements. Nine cases of SEP after IPC and 7 cases of SEP after HIPEC were previously reported. We synthetically review the pathogenesis, treatments, and outcomes. In conclusion, SEP is a rare abdominal disease, which is difficult to diagnose preoperatively. CT scanning is the most helpful imaging method for the diagnosis of SEP. Surgery is the most effective method for diagnosis and treatment, if conservative treatment has no effect or abdominal symptoms are aggravated.
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Encapsulating peritoneal sclerosis (EPS) is a debilitating condition characterized by a fibrocollagenous membrane encasing the small intestine, resulting in recurrent small bowel obstructions. EPS is most commonly associated with long-term peritoneal dialysis, though medications, peritoneal infection, and systemic inflammatory disorders have been implicated. Many cases remain idiopathic. Diagnosis is often delayed given the rarity of the disorder combined with non-specific symptoms and laboratory findings. Although cross-sectional imaging with computed tomography of the abdomen can be suggestive of the disorder, many patients undergo exploratory laparotomy for diagnosis. Mortality approaches 50% one year after diagnosis. Treatment for EPS involves treating the underlying condition or eliminating possible inciting agents (i.e. peritoneal dialysis, medications, infections) and nutritional support, frequently with total parenteral nutrition. EPS-specific treatment depends on the disease stage. In the inflammatory stage, corticosteroids are the treatment of choice, while in the fibrotic stage, tamoxifen may be beneficial. In practice, distinguishing between stages may be difficult and both may be used. Surgical intervention, consisting of peritonectomy and enterolysis, is time-consuming and high-risk and is reserved for situations in which conservative medical therapy fails in institutions with surgical expertise in this area. Herein we review the available literature of the etiology, pathogenesis, diagnosis, and treatment of this rare, but potentially devastating disease.
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Abdominal cocoon, the idiopathic form of sclerosing encapsulating peritonitis, is a rare condition of unknown etiology that results in an intestinal obstruction due to total or partial encapsulation of the small bowel by a fibrocollagenous membrane. Preoperative diagnosis requires a high index of clinical suspicion. The early clinical features are nonspecific, are often not recognized and it is difficult to make a definite pre-operative diagnosis. Clinical suspicion may be generated by the recurrent episodes of small intestinal obstruction combined with relevant imaging findings and lack of other plausible etiologies. The radiological diagnosis of abdominal cocoon may now be confidently made on computed tomography scan. Surgery is important in the management of this disease. Careful dissection and excision of the thick sac with the release of the small intestine leads to complete recovery in the vast majority of cases.