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Singhal M, Krishna S, Lal A, Narayanasamy S, Bal A, Yadav TD, Kochhar R, Sinha SK, Khandelwal N, Sheikh AM. Encapsulating Peritoneal Sclerosis: The Abdominal Cocoon. Radiographics 2018; 39:62-77. [PMID: 30526331 DOI: 10.1148/rg.2019180108] [Citation(s) in RCA: 60] [Impact Index Per Article: 8.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
Encapsulating peritoneal sclerosis (EPS) is a rare but serious condition that results in (a) encapsulation of bowel within a thickened fibrocollagenous peritoneal membrane and (b) recurrent episodes of bowel obstruction. Although described by various names in the literature, the preferred term is encapsulating peritoneal sclerosis because it best describes the morphologic and histologic changes in this disorder. The etiology of EPS is multifactorial, with a wide variety of implicated predisposing factors that disrupt the normal physiologic function of the peritoneal membrane-prime among these factors being long-term peritoneal dialysis and bacterial peritoneal infections, especially tuberculosis. The clinical features of EPS are usually nonspecific, and knowledge of the radiologic features is necessary to make a specific diagnosis. The findings on radiographs are usually normal. Images from small-bowel follow-through studies show the bowel loops conglomerated in a concertina-like fashion with a serpentine arrangement in a fixed U-shaped configuration. US demonstrates a "cauliflower" appearance of bowel with a narrow base, as well as a "trilaminar" appearance depicted especially with use of high-resolution US probes. CT is the imaging modality of choice and allows identification of the thickened contrast material-enhanced abnormal peritoneal membrane and the encapsulated clumped bowel loops. In addition, CT can potentially help identify the cause of EPS (omental granuloma in tuberculosis), as well as the complications of EPS (bowel obstruction). Conservative medical treatment and surgical therapy early in the course of EPS have been used for management of the condition. The purpose of this article is to review the nomenclature and etiopathogenesis of EPS, describe the multimodality imaging appearances of EPS, including differentiating its features from those of other conditions mimicking EPS, and give an overview of management options. Online DICOM image stacks are available for this article. ©RSNA, 2018.
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Affiliation(s)
- Manphool Singhal
- From the Departments of Radiology (M.S., A.L., N.K.), Histopathology (A.B.), General Surgery (T.D.Y.), and Gastroenterology (R.K., S.K.S.), PGIMER, Chandigarh, India; and the Abdominal Imaging Section, Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, Ont, Canada (S.K., S.N., A.M.S.)
| | - Satheesh Krishna
- From the Departments of Radiology (M.S., A.L., N.K.), Histopathology (A.B.), General Surgery (T.D.Y.), and Gastroenterology (R.K., S.K.S.), PGIMER, Chandigarh, India; and the Abdominal Imaging Section, Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, Ont, Canada (S.K., S.N., A.M.S.)
| | - Anupam Lal
- From the Departments of Radiology (M.S., A.L., N.K.), Histopathology (A.B.), General Surgery (T.D.Y.), and Gastroenterology (R.K., S.K.S.), PGIMER, Chandigarh, India; and the Abdominal Imaging Section, Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, Ont, Canada (S.K., S.N., A.M.S.)
| | - Sabarish Narayanasamy
- From the Departments of Radiology (M.S., A.L., N.K.), Histopathology (A.B.), General Surgery (T.D.Y.), and Gastroenterology (R.K., S.K.S.), PGIMER, Chandigarh, India; and the Abdominal Imaging Section, Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, Ont, Canada (S.K., S.N., A.M.S.)
| | - Amanjit Bal
- From the Departments of Radiology (M.S., A.L., N.K.), Histopathology (A.B.), General Surgery (T.D.Y.), and Gastroenterology (R.K., S.K.S.), PGIMER, Chandigarh, India; and the Abdominal Imaging Section, Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, Ont, Canada (S.K., S.N., A.M.S.)
| | - Thakur D Yadav
- From the Departments of Radiology (M.S., A.L., N.K.), Histopathology (A.B.), General Surgery (T.D.Y.), and Gastroenterology (R.K., S.K.S.), PGIMER, Chandigarh, India; and the Abdominal Imaging Section, Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, Ont, Canada (S.K., S.N., A.M.S.)
| | - Rakesh Kochhar
- From the Departments of Radiology (M.S., A.L., N.K.), Histopathology (A.B.), General Surgery (T.D.Y.), and Gastroenterology (R.K., S.K.S.), PGIMER, Chandigarh, India; and the Abdominal Imaging Section, Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, Ont, Canada (S.K., S.N., A.M.S.)
| | - Saroj K Sinha
- From the Departments of Radiology (M.S., A.L., N.K.), Histopathology (A.B.), General Surgery (T.D.Y.), and Gastroenterology (R.K., S.K.S.), PGIMER, Chandigarh, India; and the Abdominal Imaging Section, Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, Ont, Canada (S.K., S.N., A.M.S.)
| | - Niranjan Khandelwal
- From the Departments of Radiology (M.S., A.L., N.K.), Histopathology (A.B.), General Surgery (T.D.Y.), and Gastroenterology (R.K., S.K.S.), PGIMER, Chandigarh, India; and the Abdominal Imaging Section, Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, Ont, Canada (S.K., S.N., A.M.S.)
| | - Adnan M Sheikh
- From the Departments of Radiology (M.S., A.L., N.K.), Histopathology (A.B.), General Surgery (T.D.Y.), and Gastroenterology (R.K., S.K.S.), PGIMER, Chandigarh, India; and the Abdominal Imaging Section, Department of Medical Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, Ont, Canada (S.K., S.N., A.M.S.)
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Danford CJ, Lin SC, Smith MP, Wolf JL. Encapsulating peritoneal sclerosis. World J Gastroenterol 2018; 24:3101-3111. [PMID: 30065556 PMCID: PMC6064970 DOI: 10.3748/wjg.v24.i28.3101] [Citation(s) in RCA: 94] [Impact Index Per Article: 13.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/16/2018] [Revised: 06/07/2018] [Accepted: 06/25/2018] [Indexed: 02/06/2023] Open
Abstract
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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Affiliation(s)
- Christopher J Danford
- Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, United States
| | - Steven C Lin
- Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, United States
| | - Martin P Smith
- Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, United States
| | - Jacqueline L Wolf
- Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, United States
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K A, S V, C G, Khera A. Sclerosing peritonitis occurring in association with juvenile granulosa cell tumour - a cause of concern. J Clin Diagn Res 2014; 8:123-4. [PMID: 24783102 PMCID: PMC4003605 DOI: 10.7860/jcdr/2014/6825.4131] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2013] [Accepted: 01/20/2014] [Indexed: 11/24/2022]
Abstract
Sclerosing Peritonitis (SP) is characterized by fibrous thickening and adhesions of the peritoneum and it is encountered frequently in association with peritoneal dialysis, practolol and anticonvulsant therapy. Rarely does it present as a gynaecological complication, most commonly in conjunction with luteinized thecoma of ovary. Very rarely has sclerosing peritonitis been reported to occur with ovarian cystic teratoma and a luteinized variant of adult granulosa cell tumour, among which only one case has been reported in the English literature. An 18-year-female presented with a history of lower abdominal pain, of one month's duration. At surgery, an ovarian mass was noted, along with adhesion of bowel loops which were enclosed within a diffusely thickened peritoneal membrane, forming a cocoon. Adhesions were seen in bilateral paracolic gutter and infracolic omentum, which were suggestive of metastatic deposits. Histopathology of ovarian lesion was interpreted as a juvenile granulosa cell tumour. The peritoneal process revealed sclerosing peritonitis. We emphasize this rare association of sclerosing peritonitis with juvenile granulosa cell tumour and that the peritoneal process of sclerosing peritonitis in a juvenile granulosa cell tumour should not lead to over staging of an ovarian tumour.
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Affiliation(s)
- Amita K
- Associate Professor, Department of Pathology, Adichunchanagiri Institute of Medical Sciences, Karnataka, India
| | - Vijayshankar S
- Associate Professor, Department of Pathology, Adichunchanagiri Institute of Medical Sciences, Karnataka, India
| | - Gangadhar C
- Consultant Pathologist, Department of Pathology, BGS Global Hospital, Bangalore, India
| | - Abhishek Khera
- Post Graduate, Department of Pathology, Adichunchanagiri Institute of Medical Sciences, Karnataka, India
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Kanthan R, Senger JL, Kanthan S. The multifaceted granulosa cell tumours-myths and realities: a review. ISRN OBSTETRICS AND GYNECOLOGY 2012; 2012:878635. [PMID: 23008780 PMCID: PMC3449135 DOI: 10.5402/2012/878635] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 05/23/2012] [Accepted: 07/05/2012] [Indexed: 12/17/2022]
Abstract
Background. Granulosa cell tumors (GCTs), representing ~2% of ovarian tumours, are poorly understood neoplasms with unpredictable and undetermined biological behaviour. Design. 5 unusual presentations of GCT and a retrospective 14-year (1997–2011) surgical pathology review based on patient sex, age, tumour type and concurrent pathology findings are presented to discuss the “myths and realities” of GCTs in the context of relevant evidence-based literature. Results. The 5 index cases included (1) a 5 month-old boy with a left testicular mass, (2) a 7-day-old neonate with a large complex cystic mass in the abdomen, (3) a 76-year-old woman with an umbilical mass, (4) a 64-year-old woman with a complex solid-cystic pelvic mass, and (5) a 45 year-old woman with an acute abdomen. Pathological analysis confirmed the final diagnosis as (1) juvenile GCT, (2) macrofollicular GCT, (3) recurrent GCT 32 years later, (4) collision tumour: colonic adenocarcinoma and GCT, and (5) ruptured GCT. Conclusion. GCT is best considered as an unusual indolent neoplasm of low malignant potential with late recurrences that can arise in the ovaries and testicles in both the young and the old. Multifaceted clinical presentations coupled with the unpredictable biological behaviour with late relapses are diagnostic pitfalls necessitating a high degree of suspicion for accurate clinical and pathological diagnosis.
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Affiliation(s)
- Rani Kanthan
- Department of Pathology and Laboratory Medicine, University of Saskatchewan, Saskatoon, SK, Canada S7N-0W8 ; Department of Pathology and Laboratory Medicine, Royal University Hospital, 103 Hospital Drive, Room 2868, G-Wing, Saskatoon, SK, Canada S7N-0W8
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