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Bright B, Salam R, Moorthy S. A Case Series and Brief Review of Literature on Encapsulating Peritoneal Sclerosis: Unveiling the Cocoon. Cureus 2024; 16:e73802. [PMID: 39687804 PMCID: PMC11648041 DOI: 10.7759/cureus.73802] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2024] [Accepted: 11/15/2024] [Indexed: 12/18/2024] Open
Abstract
This case series explores four distinct instances of encapsulating peritoneal sclerosis (EPS), a rare but serious condition characterized by the encapsulation of abdominal viscera, commonly referred to as abdominal cocoon. EPS is associated with severe complications, including bowel obstruction and sepsis, which can significantly impact patient outcomes. The first case involves a 41-year-old male patient who had undergone a liver transplant and ultimately succumbed to extensively drug-resistant (XDR) sepsis. The second case features a 31-year-old male patient diagnosed with abdominal tuberculosis, who successfully recovered following comprehensive antitubercular therapy (ATT). The third case presents a 26-day-old neonate with CHARGE syndrome (coloboma of the eye, heart defects, atresia of the choanae, retardation of growth and development, genital abnormalities, and ear anomalies, including deafness), who tragically succumbed to septic shock. The last case is of a 41-year-old male patient with disseminated tuberculosis who showed marked improvement with appropriate treatment. These cases illustrate the diverse clinical backgrounds and grave outcomes associated with EPS, highlighting the urgent need for early diagnosis and intervention. Despite EPS being a leading cause of small bowel obstruction in many inpatient settings, its diagnosis is frequently overlooked due to insufficient awareness among healthcare professionals. This series aims to enhance understanding of the causes, imaging characteristics, and management strategies for EPS. By disseminating this knowledge, we hope to facilitate earlier identification of the condition, particularly through primary imaging techniques such as ultrasound (USG). Ultimately, increasing awareness and understanding of EPS is crucial to improving patient outcomes and reducing the associated morbidity and mortality.
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Affiliation(s)
- Bribin Bright
- Radiodiagnosis, Amrita Institute of Medical Sciences, Kochi, IND
| | - Roshna Salam
- Radiodiagnosis, Amrita Institute of Medical Sciences, Kochi, IND
| | - Srikanth Moorthy
- Radiodiagnosis, Amrita Institute of Medical Sciences, Kochi, IND
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2
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Loughrey MB. Inflammatory disorders of the peritoneum. MORSON AND DAWSON'S GASTROINTESTINAL PATHOLOGY 2024:1057-1071. [DOI: 10.1002/9781119423195.ch47] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Watanabe-Kusunoki K, Kusunoki Y, Goto J, Kukita K. Liver cirrhosis with encapsulating peritoneal sclerosis after 4 years of peritoneal dialysis: A case report. Medicine (Baltimore) 2021; 100:e28350. [PMID: 34941145 PMCID: PMC8702031 DOI: 10.1097/md.0000000000028350] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/18/2021] [Accepted: 12/01/2021] [Indexed: 01/05/2023] Open
Abstract
RATIONALE Encapsulating peritoneal sclerosis (EPS), or abdominal cocoon, is a rare but fatal syndrome characterized by intestinal obstruction owing to adhesions in a diffusely thickened peritoneum. Long-term peritoneal dialysis (PD) for more than 5 years is commonly associated with EPS, while liver cirrhosis also carries a risk of EPS. However, there have been only a few reports that describe a case of EPS complicated with both cirrhosis and PD. We herein describe a case of advanced liver cirrhosis with end-stage renal disease (ESRD) who developed EPS after 4 years of PD and who was successfully recovered by surgery. PATIENT CONCERNS A 58-year-old man with alcoholic liver cirrhosis suffered abdominal pain. The patient had a 4-year history of continuous cycling PD to manage ESRD as well as cirrhotic complications of refractory ascites and hypotension. Laboratory test results showed increased levels of inflammation, and contrast-enhanced computed tomography scan showed dilated loops of small bowel proximal to the site of intestinal obstruction. The patient was suspected to have developed intestinal obstruction owing to EPS. The patient discontinued continuous cycling peritoneal dialysis and switched to hemodiafiltration. DIAGNOSES Laparoscopy revealed a whitish membranous material wrapped around the bowel, especially at the terminal ileum with a narrowed portion, consistent with EPS. INTERVENTIONS Repeated decortication of fibrous peritoneal membranes successfully released the intestinal obstruction. OUTCOMES The postoperative course went well and abdominal pain remained in remission. Because abdominal distension owing to ascites got intolerable in a few days after surgery, a PD catheter was re-inserted and ascitic fluid drainage was resumed with peritoneal lavage. The patient continued hemodiafiltration using vasopressor agents. LESSONS The Cirrhotic patient with ESRD undergoing PD could develop EPS after a short duration of PD.
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Affiliation(s)
| | - Yoshihiro Kusunoki
- Department of Internal Medicine, Kushiro Red Cross Hospital, Kushiro, Japan
| | - Junichi Goto
- Department of Surgery, Sapporo Hokuyu Hospital, Sapporo, Japan
| | - Kazutaka Kukita
- Department of Surgery, Sapporo Hokuyu Hospital, Sapporo, Japan
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Yin MY, Qian LJ, Xi LT, Yu YX, Shi YQ, Liu L, Xu CF. Encapsulating peritoneal sclerosis in an AMA-M2 positive patient: A case report. World J Clin Cases 2021; 9:6138-6144. [PMID: 34368336 PMCID: PMC8316956 DOI: 10.12998/wjcc.v9.i21.6138] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/26/2021] [Revised: 05/07/2021] [Accepted: 05/24/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Encapsulating peritoneal sclerosis (EPS) is hard to diagnose because of nonspecific symptoms and signs. It is a general consensus that EPS is classified as primary and secondary. There have been several studies discovering some high-risk factors such as liver cirrhosis, of which AMA-M2 is a biomarker, and intra-abdominal surgery such as laparoscopic surgery. Imaging studies help to diagnose EPS and exploratory laparotomy might be an alternative if imaging fails. Nowadays, laparotomy plays a key role in treating EPS, especially when medical treatments do not work and medical therapy fails to ease patients’ symptoms.
CASE SUMMARY A 58-year-old man complained of unexplained vomiting and abdominal distension 2 mo after laparoscopic cholecystectomy. Increased alkaline phosphatase and liver enzymes were discovered. An autoimmune liver disease test showed that AMA-M2 was positive. A gastroscopy revealed bile reflux gastritis. A magnetic resonance imaging scan showed a slight dilatation of the intrahepatic bile duct. A colonoscopy showed that there was a mucosal eminence lesion in the sigmoid colon (24 cm away from the anus), with a size of 3 cm × 3 cm and erosive surface. At last, the small intestine and the stomach were found to be encased in a cocoon-like membrane during the surgery. The membrane was dissected and adhesiolysis was done to release the trapped organs. The patient recovered and was discharged 44 d after the operation, and there was no recurrence during a follow-up period of 3 mo.
CONCLUSION AMA-M2 is a marker of primary biliary sclerosis and may help to make a preoperative diagnosis of EPS.
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Affiliation(s)
- Min-Yue Yin
- Department of Gastroenterology, The First Affiliated Hospital of Soochow University, Suzhou 215006, Jiangsu Province, China
| | - Li-Juan Qian
- Department of Gastroenterology, The First Affiliated Hospital of Soochow University, Suzhou 215006, Jiangsu Province, China
| | - Li-Ting Xi
- Department of Gastroenterology, The First Affiliated Hospital of Soochow University, Suzhou 215006, Jiangsu Province, China
| | - Yi-Xing Yu
- Department of Gastroenterology, The First Affiliated Hospital of Soochow University, Suzhou 215006, Jiangsu Province, China
| | - Yu-Qi Shi
- Department of Gastroenterology, The First Affiliated Hospital of Soochow University, Suzhou 215006, Jiangsu Province, China
| | - Lu Liu
- Department of Gastroenterology, The First Affiliated Hospital of Soochow University, Suzhou 215006, Jiangsu Province, China
| | - Chun-Fang Xu
- Department of Gastroenterology, The First Affiliated Hospital of Soochow University, Suzhou 215006, Jiangsu Province, China
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Kang JH. A rare case of intestinal obstruction: Sclerosing encapsulating peritonitis of unknown cause. Turk J Emerg Med 2020; 20:152-155. [PMID: 32832736 PMCID: PMC7416848 DOI: 10.4103/2452-2473.290068] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/14/2020] [Accepted: 04/23/2020] [Indexed: 12/15/2022] Open
Abstract
Sclerosing encapsulating peritonitis (SEP) is characterized by the partial or complete enclosing of the small intestines by a thick fibro-collagenous membrane, which can cause recurrent intestinal obstruction. SEP is a clinically rare disease, and the major risk factor is peritoneal dialysis (PD). Early diagnosis of SEP is an important factor in the patient's prognosis, but it is clinically difficult. A 52-year-old woman visited the emergency department (ED) with a 2-day history of abdominal pain and vomiting. She had a history of liver cirrhosis with chronic hepatitis B, but no history of PD, and she underwent a biopsy of the peritoneum by laparoscopy a month ago. On physical examination, there were peritoneal irritation signs on the right lower quadrant (RLQ). Abdominal computed tomography (CT) showed dilated small intestinal loops clustered in the RLQ, which were surrounded by a sac-like, thick fibrous membrane. Based on CT findings, small intestinal obstruction due to SEP was early diagnosed in the ED. Emergency physicians should include SEP in the differential diagnosis of the cause of intestinal obstruction. Abdominal CT is a useful modality for the early diagnosis of SEP in the ED.
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Affiliation(s)
- Jeong Ho Kang
- Department of Emergency Medicine, Jeju National University School of Medicine, Jeju-do, Republic of Korea
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Iwai M, Kunishi Y, Suriawinata AA. Peritoneal Diseases. DIAGNOSIS OF LIVER DISEASE 2019:287-297. [DOI: 10.1007/978-981-13-6806-6_20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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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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Aliyev V, Yagi S, Hammad A, Badawy A, Sasaki Y, Masano Y, Yamamoto G, Kamo N, Taura K, Okajima H, Kaido T, Uemoto S. Sclerosing encapsulating peritonitis after living-donor liver transplantation: A case series, Kyoto experience. Ann Hepatobiliary Pancreat Surg 2018; 22:144-149. [PMID: 29896575 PMCID: PMC5981144 DOI: 10.14701/ahbps.2018.22.2.144] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/05/2017] [Revised: 01/29/2018] [Accepted: 01/30/2018] [Indexed: 11/17/2022] Open
Abstract
Sclerosing encapsulating peritonitis (SEP), or abdominal cocoon is a rare cause of intestinal obstruction, and still etiology remains unknown. We report a series of 4 patients with abdominal cocoon, and all the 4 patients had previously undergone living-donor liver transplantation (LDLT). There was no evidence of SEP before and during LDLT. At the time of diagnosis of SEP, 3 out of 4 patients had ascites. First and fourth patients had multiple episodes or attacks of cholangitis, which were managed by percutaneous transhepatic biliary drainage and hepaticojejunostomy, respectively. All 4 patients presented with intestinal obstruction and 3 of them underwent a successful operation. The fourth patient died due to liver failure and complications of the SEP. The first 3 patients are doing well without SEP recurrence. Our experience suggest that the prognosis of SEP is poor in patients with poor graft liver functions after LDLT.
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Affiliation(s)
- Vusal Aliyev
- Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Shintaro Yagi
- Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Ahmed Hammad
- Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan.,Department of General Surgery, Mansoura University, Mansoura, Egypt
| | - Amr Badawy
- Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan.,Department of Surgery, Alexandria University, Alexandria, Egypt
| | - Yudai Sasaki
- Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Yuki Masano
- Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Gen Yamamoto
- Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Naoko Kamo
- Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Kojiro Taura
- Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Hideaki Okajima
- Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Toshimi Kaido
- Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Shinji Uemoto
- Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
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Noh SH, Ye BD, So H, Kim YS, Suh DJ, Yoon SN. Sclerosing encapsulating peritonitis in a long-term propranolol user. Intest Res 2016; 14:375-378. [PMID: 27799890 PMCID: PMC5083268 DOI: 10.5217/ir.2016.14.4.375] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/17/2015] [Revised: 08/08/2015] [Accepted: 08/24/2015] [Indexed: 12/11/2022] Open
Abstract
Sclerosing encapsulating peritonitis (SEP) is a rare cause of bowel obstruction that is characterized by the encasement of the small bowel by a thick, whitish, and fibrous membrane. The pathophysiology of SEP is poorly understood and preoperative diagnosis is difficult. Previous reports suggest that SEP may be linked to the chronic use of β-adrenergic blockers. A 46-year-old man with liver cirrhosis and end-stage renal disease on hemodialysis presented with recurrent abdominal pain and borborygmi. He had been taking propranolol to prevent bleeding from gastroesophageal varices for the past 15 years. Abdominal computed tomography showed ileal loops encapsulated by soft tissue with dilatation of the proximal small bowel on the right side of the abdomen. Barium follow-through showed conglomerated distal ileal loops with a cauliflower-like appearance. Explorative laparotomy revealed a thick, fibrous, whitish capsule encapsulating the ileal loops. The covering membrane was dissected and excised, resulting in an improvement in symptoms after surgery. Accordingly, a final diagnosis of SEP was made. Due to the lack of other apparent causes for SEP, we conclude that in this case, the long-term use of propranolol may be associated with the development of SEP.
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Affiliation(s)
- Se Hui Noh
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Byong Duk Ye
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.; Inflammatory Bowel Disease Center, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Hoonsub So
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Yu Seok Kim
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Dong Jin Suh
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Sang Nam Yoon
- Department of Colon & Rectal Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Ikeda Y, Sato Y, Kamihara Y, Hirakawa M, Onuma H, Takada K, Hayashi T, Sato T, Miyanishi K, Takimoto R, Kohune M, Nobuoka T, Noguchi H, Oi M, Honma H, Hirata K, Hasegawa T, Kato J. A case of idiopathic encapsulating peritoneal sclerosis with intractable ileus successfully treated by surgery and steroid therapy. Clin J Gastroenterol 2013; 6:295-8. [PMID: 26181733 DOI: 10.1007/s12328-013-0395-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2012] [Accepted: 06/04/2013] [Indexed: 11/28/2022]
Abstract
Encapsulating peritoneal sclerosis (EPS) occurring without a history of peritoneal dialysis is rare. We report on a patient with idiopathic EPS following intractable ileus who was successfully treated by surgery and postoperative steroid therapy without any sign of recurrence. A 67-year-old woman was referred to our department for further treatment of intractable ileus. Abdominal CT scanning revealed wall thickening of the proximal jejunum. Double-balloon enteroscopy disclosed stenosis of the jejunum at 20 cm anally from the Treitz ligament, although the intestinal mucosa appeared normal without specific biopsy findings. In addition, FDG-PET showed no abnormal accumulation, thus discounting a malignant lesion. Since conservative therapy failed to improve the ileus, we performed an operation on her in order to release the ileus and make a histological diagnosis. Surgical findings included a whitish thickening of the serosa extending to the intestine and the whole mesentery. Accordingly, we made a diagnosis of idiopathic encapsulating peritoneal sclerosis because of her negative history of peritoneal dialysis, laparotomy or peritonitis, in addition to the above-noted findings. Postoperative oral administration of steroid has suppressed EPS recurrence. In patients with intractable ileus, EPS should be added to the list of differential diagnoses, even if they have not undergone peritoneal dialysis.
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Affiliation(s)
- Yuuki Ikeda
- Fourth Department of Internal Medicine, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Yasushi Sato
- Fourth Department of Internal Medicine, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Yuusuke Kamihara
- Fourth Department of Internal Medicine, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Masahiro Hirakawa
- Fourth Department of Internal Medicine, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Hiroyuki Onuma
- Fourth Department of Internal Medicine, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Kohichi Takada
- Fourth Department of Internal Medicine, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Tsuyoshi Hayashi
- Fourth Department of Internal Medicine, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Tsutomu Sato
- Fourth Department of Internal Medicine, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Koji Miyanishi
- Fourth Department of Internal Medicine, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Rishu Takimoto
- Fourth Department of Internal Medicine, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Masayoshi Kohune
- Fourth Department of Internal Medicine, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Takayuki Nobuoka
- First Department of Surgery, Sapporo Medical University, Sapporo, Japan
| | - Hiroko Noguchi
- Department of Surgical Pathology, Sapporo Medical University, Sapporo, Japan
| | - Motoo Oi
- Department of Gastroenterology, Kyoritsu Gorinbashi Hospital, Sapporo, Japan
| | - Hisato Honma
- Department of Gastroenterology, Kyoritsu Gorinbashi Hospital, Sapporo, Japan
| | - Kohichi Hirata
- First Department of Surgery, Sapporo Medical University, Sapporo, Japan
| | - Tadashi Hasegawa
- Department of Surgical Pathology, Sapporo Medical University, Sapporo, Japan
| | - Junji Kato
- Fourth Department of Internal Medicine, Sapporo Medical University, South 1 West 16, Chuo-ku, Sapporo, 060-8543, Japan.
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Sclerosing encapsulating peritonitis after living donor liver transplantation: a case successfully treated with tamoxifen: report of a case. Surg Today 2012; 43:1326-9. [PMID: 23099621 DOI: 10.1007/s00595-012-0368-5] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/21/2012] [Accepted: 05/17/2012] [Indexed: 01/29/2023]
Abstract
Sclerosing encapsulating peritonitis (SEP) is a rare cause of bowel obstruction. It is difficult to diagnose and the prognosis is poor. This report describes a case of SEP after living donor liver transplantation that was successfully treated with tamoxifen. A 56-year-old male, that had received a liver transplant for hepatitis C virus-related hepatocellular carcinoma 5 years earlier, was admitted with continuous abdominal pain and nausea. He had increased C-reactive protein levels and white blood cell count, and underwent laparotomy 5 days after hospitalization. The surgical findings showed ascites and SEP of the small bowel. An attempt to peel off the adhesions was stopped because there was a strong risk of intestinal tract damage. Tamoxifen treatment was initiated for SEP after surgery. The patient's symptoms gradually improved and he was able to resume feeding. He had been symptom-free for over 3 years at the last follow-up.
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Da Luz MMP, Barral SM, Barral CM, Bechara CDS, Lacerda-Filho A. Idiopathic encapsulating peritonitis: report of two cases. Surg Today 2011; 41:1644-8. [PMID: 21969199 DOI: 10.1007/s00595-010-4493-8] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2010] [Accepted: 12/26/2010] [Indexed: 12/18/2022]
Abstract
This report presents two cases of young males who developed the rare idiopathic form of sclerosing encapsulating peritonitis (SEP) presented as partial bowel obstruction, both diagnosed during surgical treatment, with satisfactory outcomes. Sclerosing encapsulating peritonitis is a rare and enigmatic condition, characterized by intraperitoneal fibrosclerosis, which causes intestinal obstruction. It is a chronic entity with a poorly elucidated pathophysiology, leading to the constitution of a thick white nacreous fibrosis membrane that wraps the bowel in a concertina-like fashion with some adhesions configuring an intra-abdominal cocoon. Sclerosing encapsulating peritonitis is reported in a wide variety of patients, including those who have undergone peritoneal dialysis, young adolescent girls, cirrhotic patients after peritoneal-venous shunting, and patients treated with β-blockers. Nevertheless, the etiology of SEP remains obscure. This entity presents many difficulties in preoperative diagnosis because of its peculiar characteristics. Recognition of the SEP results in proper management and prevents unnecessary bowel resection. Regardless of cause, the treatment of the obstruction is surgical, with dissection of the encasing membrane from the intestine and separation of adherent loops of small bowel until they are laid free and returned to their normal configuration. The prognosis after appropriate surgical therapy is good, but depends on coexisting diseases.
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Affiliation(s)
- Magda Maria Profeta Da Luz
- Division of Coloproctology and Small Bowel, Alfa Institute of Gastroenterology, Federal University of Minas Gerais Hospital, Av. Alfredo Balena 110 - 2° andar, Belo Horizonte, MG 30130-100, Brazil
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13
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IZAWA T, MURAI F, AKIYOSHI H, OHASHI F, YAMATE J, KUWAMURA M. Encapsulating Peritoneal Sclerosis Associated with Abnormal Liver Development in a Young Dog. J Vet Med Sci 2011; 73:697-700. [DOI: 10.1292/jvms.10-0503] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Affiliation(s)
- Takeshi IZAWA
- Laboratory of Veterinary Pathology, Osaka Prefecture University
| | - Fumi MURAI
- Laboratory of Veterinary Pathology, Osaka Prefecture University
| | - Hideo AKIYOSHI
- Laboratory of Veterinary Surgery, Osaka Prefecture University
| | - Fumihito OHASHI
- Laboratory of Veterinary Surgery, Osaka Prefecture University
| | - Jyoji YAMATE
- Laboratory of Veterinary Pathology, Osaka Prefecture University
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14
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Tagnaouti M, Branger B, Ied C, Reboul P, Zabadani B, Vecina F, Deschodt G, Carolfi J, Prudhomme M, Godlevski G. La sclérose péritonéale encapsulante : aspects actuels. Nephrol Ther 2009; 5:122-33. [DOI: 10.1016/j.nephro.2008.08.014] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/20/2008] [Revised: 07/08/2008] [Accepted: 08/28/2008] [Indexed: 10/21/2022]
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15
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Mekeel K, Moss A, Reddy KS, Douglas D, Mulligan D. Sclerosing peritonitis and mortality after liver transplantation. Liver Transpl 2009; 15:435-9. [PMID: 19326414 DOI: 10.1002/lt.21702] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Sclerosing peritonitis describes the development of a peel or rind of fibrosis that spreads over the peritoneal surface and can lead to recalcitrant ascites, bowel obstruction, and sepsis. It is well described as a complication of peritoneal dialysis, especially with episodes of bacterial peritonitis. It is also a complication of end-stage liver disease with ascites and liver transplantation. This article describes 3 cases of sclerosing peritonitis present at the time of liver transplantation or soon after. All 3 patients had massive refractory ascites with episodes of spontaneous bacterial peritonitis prior to transplantation. Two patients had evidence of a fibrous peel at the time of transplantation. Postoperatively, all 3 patients continued to have refractory ascites and episodes of peritonitis, along with partial small bowel obstructions, abdominal pain, and malnutrition. Two patients also had constriction of the graft, including biliary obstruction and inferior vena cava and outflow obstruction, which has not been previously described. All 3 patients eventually died from complications related to the sclerosing peritonitis.
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Affiliation(s)
- Kristin Mekeel
- Division of Transplant Surgery, Mayo Clinic Hospital, Phoenix, AZ 85254, USA.
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Suh WN, Lee SK, Chang H, Hwang HJ, Hyung WJ, Park YN, Kim TI. Sclerosing encapsulating peritonitis (abdominal cocoon) after abdominal hysterectomy. Korean J Intern Med 2007; 22:125-9. [PMID: 17616031 PMCID: PMC2687622 DOI: 10.3904/kjim.2007.22.2.125] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/27/2022] Open
Abstract
Sclerosing encapsulating peritonitis (SEP) is a poorly understood and rarely documented cause of small bowel obstruction. Although recurrent peritonitis has been reported as the main contributory factor leading to secondary SEP, the pathogenesis of primary (idiopathic) SEP is still uncertain. A 40-year-old woman with a history of total abdominal hysterectomy due to gestational trophoblastic disease presented with progressive lower abdominal pain and abdominal distension. Ultrasonography and contrast-enhanced abdomen-pelvis computed tomography of the abdomen revealed encapsulation of the entire small bowel with a sclerotic capsule. At laparotomy, a fibrous thick capsule encasing small bowel loops was revealed. Extensive adhesiolysis and removal of the capsule from the bowel loops were performed. The patient recovered uneventfully; she was discharged without complications. SEP is a rare cause of small bowel obstruction. We treated a case of abdominal cocoon with intestinal partial obstruction in a woman with a history of abdominal hysterectomy due to gestational trophoblastic disease. Surgical treatment was effective and the patient recovered without complication.
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Affiliation(s)
- Won Na Suh
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
| | - Sang Kil Lee
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
| | - Hyun Chang
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
| | - Hye Jin Hwang
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
| | - Woo Jin Hyung
- Institute of Gastroenterology surgery, Yonsei University College of Medicine, Seoul, Korea
| | - Young Nyun Park
- Department of Pathology, Yonsei University College of Medicine, Seoul, Korea
| | - Tae Il Kim
- Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
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17
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Chronisch fibrosierende (sklerosierende) Peritonitis als Ursache des „plötzlichen“ Todes. Rechtsmedizin (Berl) 2006. [DOI: 10.1007/s00194-006-0369-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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18
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Lin CH, Yu JC, Chen TW, Chan DC, Chen CJ, Hsieh CB. Sclerosing encapsulating peritonitis in a liver transplant patient: A case report. World J Gastroenterol 2005; 11:5412-3. [PMID: 16149160 PMCID: PMC4622823 DOI: 10.3748/wjg.v11.i34.5412] [Citation(s) in RCA: 18] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
We report a patient with HBV-related hepatocellular carcinoma (HCC) and refractory ascites who had received a peritoneal-venous shunt (PVS) 1 year before liver transplantation. Urgent surgical intervention following bowel obstruction and failure of immunosuppression therapy. No intestinal obstruction was found during an initial PVS. However, intestinal obstruction developed 2 wk after liver transplantation; and a cocoon abdomen was found upon exploration. This is the first reported case of cocoon abdomen caused by PVS and exacerbated by liver transplantation.
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Affiliation(s)
- Chien-Hua Lin
- Division of General Surgery, Department of Surgery, Tri-Service General Hospital, National Defense Medical Center, 325, Sec. 2, Cheng-Kung Road, Neihu 114, Taipei, Taiwan, China
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