University of South Carolina Columbia, SC, United States
Shayan Noorani, DO1, Melissa Whitman, DO2, Stuart M. Smith, MD2 1Prisma Health-University of South Carolina School of Medicine, Columbia, SC; 2University of South Carolina, Columbia, SC
Introduction: Encapsulating peritoneal sclerosis (EPS) is a rare disease that presents with recurrent small bowel obstructions. Most commonly EPS is associated with peritoneal dialysis, although it is divided into primary idiopathic onset versus secondary onset with many etiologies implicated for the latter. Early recognition is essential because of the high morbidity and mortality associated with this disease if untreated. Additionally, management oftentimes depends on the underlying etiology making workup of secondary EPS essential.
Case Description/Methods: Here we present a fifty-eight-year-old female with a past medical history of multiple prior abdominal surgeries, diabetes mellitus and hypertension presenting with three days of vomiting and constipation. On physical exam, she was afebrile with a heart rate of 100 bpm and a blood pressure of 106 / 60 mm Hg. She was uncomfortable appearing, and abdominal exam was remarkable for absent bowel sounds, diffuse tenderness, and slight distention. A computed tomography with IV contrast revealed multiple small bowel loops surrounded by moderate ascites contained within irregular calcifications and stranded densities about the anterior margin of the fluid collection, suggesting encapsulating peritoneal sclerosis. The visualized fluid was sampled, and cytology revealed atypical samples suspicious for malignancy. Subsequent laparoscopy with biopsy confirmed low grade serous ovarian carcinoma. She underwent treatment for her ovarian cancer with chemotherapy and immunotherapy and has not had a recurrence of her bowel obstruction.
Discussion: EPS is often a delayed diagnosis because it requires such a high degree of suspicion to find. Some helpful signs on CT imaging include peritoneal thickening greater than 2 mm, calcifications, interbowel ascites and loculated fluid collections. Here, EPS was found in the setting of an undiagnosed gynecologic neoplasm. The mechanism has been described that a gynecologic neoplasm in a predisposing genetic background is an inciting factor that stimulated proinflammatory proangiogenic cytokines to upregulate profibrotic gene expression leading to peritoneal thickening and fibrosis. Current medical therapies focus on immunosuppression, although new data has found some anti-fibrotic benefits of Tamoxifen. Unfortunately, there is little literature on medical therapy specifically in the setting of malignancy, and thus more research is needed to explore how to best manage EPS when it presents as a cancer related diagnosis.
Figure: CT abdomen that shows many of the small bowel loops that are clustered in the center of the abdomen with ascites present about the bowel loops. This thick collagenous membrane represents the "cocooning" of bowel loops typically seen in EPS. Calcifications and strandy densities present along the anterior margin as well.
A. Axial view; B. Coronal view; C. Sagittal view
Disclosures: Shayan Noorani indicated no relevant financial relationships. Melissa Whitman indicated no relevant financial relationships. Stuart Smith indicated no relevant financial relationships.
Shayan Noorani, DO1, Melissa Whitman, DO2, Stuart M. Smith, MD2. P0934 - An Entangled Gut- Unraveling an Unusual Etiology of Encapsulating Peritoneal Sclerosis, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.