IgG4 related sclerosing encapsulating peritonitis with cocoon formation: An unusual and undescribed presentation

IgG4-related sclerosing mesenteritis is a rare disease of mesentery of an unknown etiology which shows a constellation of histopathologic findings of lymphoplasmacytic inflammation with IgG4-positive plasma cells and marked fibrosis. This chronic inflammatory condition of mesentery forming an abdominal cocoon has never been described before to the best of our knowledge. Here, we report a patient with a history of subacute small bowel obstruction who was found to have an intra-abdominal encapsulating mass in the right iliac fossa and was finally diagnosed as IgG4-related sclerosing encapsulating peritonitis (abdominal cocoon) based on peroperative findings, histology and immunohistochemistry.

Peritonitis encapsulante esclerosante (síndrome de cocoon): una causa extremadamente rara de obstrucción intestinal. Reporte de un caso y revisión de la literatura / Cocoon syndrome: an extremely rare cause of bowel obstruction. Case report and literature review

La peritonitis esclerosante encapsulante o síndrome de cocoon es una causa rara de obstrucción intestinal, que puede dividirse en pri-maria o secundaria, producida por una membrana fibrocolágena que envuelve el intestino delgado. El diagnóstico es particularmente desafiante, al tratarse de una entidad cuya clínica es inespecífica. El conocimiento de los hallazgos imagenológicos, sumados a una correcta anamnesis, permite una adecuada valoración preoperatoria. Presentamos un caso de interés y de difícil diagnóstico, junto con una revisión de la literatura sobre el tema.

Sclerosing encapsulating peritonitis or cocoon syndrome is a rare cause of intestinal obstruction, that can be divided into primary or secondary. It is produced by a fibro collagenous membrane that surrounds the small bowel. The diagnosis is particularly challenging since it is an entity whose symptoms are nonspecific. Knowledge of the imaging findings, in addition to a well performed anamnesis allows an adequate preoperative assessment. We present a clinical case and a review of the literature.

Idiopathic abdominal cocoon: An enigma

Abdominal cocoon is often described by various terminologies like encapsulating peritoneal sclerosis (EPS) or sclerosing encapsulating peritonitis or peritonitis chronica fibrosa incapsulata is defined as syndromes associated with symptoms due to formation of a fibro-collagenous peritoneal membrane involving commonly the small intestinal loop. Clinical presentation ranges from abdominal pain to features of intestinal obstruction which may be acute or sub-acute. It is believed to be mesenchymal transition of mesothelial cells. This condition is commonly associated with tuberculosis, peritoneal dialysis and previous abdominal surgeries but may also be idiopathic. Though a wide range of medical management has been tried for conservative management of the patient, surgery is the preferred choice of treatment to alleviate the persisting symptoms. This is one condition where on table intra op diagnosis supersedes the imaging and histological diagnosis. Here, we discuss the case report of 32 year old male, known diabetic for 4 years, who presented with complaints of abdominal pain, intermittent for over 5 years, with no evidence of intestinal obstruction. Imaging showed abdominal cocoon of small bowel loops and mid gut rotation anomaly with internal hernia. Laparoscopically the cocoon sac was removed and adhesiolysis was done. This case report is to add richness to limited amount literary resources available about abdominal cocoon syndrome.

Obstrucción intestinal por peritonitis esclerosante encapsulada en un adulto con derivación ventriculoperitoneal / Bowel obstruction caused by sclerosing encapsulating peritonitis in adult with ventriculoperitoneal shunt

Resumen Introducción La peritonitis esclerosante encapsulada es una inflamación crónica del peritoneo caracterizada por la formación progresiva de colágeno que produce un engrosamiento peritoneal que encapsula las asas del intestino delgado, produciendo una obstrucción intestinal. Caso clínico Varón de 83 años portador de catéter de derivación ventriculoperitoneal de baja presión desde hacía 8 años. Acude a Urgencias por clínica de obstrucción intestinal, con hallazgos radiológicos en relación con obstrucción de intestino delgado en probable relación con catéter de derivación ventriculoperitoneal, por lo que se decide realizar intervención quirúrgica urgente. Se confirmó la obstrucción intestinal y un cambio de calibre del íleon terminal. El intestino delgado estaba dilatado formando un ovillo, englobado por una pequeña cápsula fibrosa. Se realizó adhesiólisis y hemicolectomía derecha con resección del segmento intestinal afectado. La anatomía patológica fue compatible con peritonitis esclerosante encapsulada. Conclusiones La peritonitis esclerosante encapsulada es una causa poco frecuente y potencialmente grave de obstrucción intestinal y su forma secundaria está habitualmente asociada a la diálisis peritoneal crónica. Su diagnóstico radiológico es difícil y el tratamiento de elección es quirúrgico. Debemos considerarla en casos de obstrucción intestinal en pacientes portadores de derivación ventriculoperitoneal.

Introduction Sclerosing encapsulating peritonitis is a chronic inflammation of the peritoneum characterized by the progressive accumulation of collagen. This leads to a thickening of the peritoneum, encapsulating loops of small bowel and causing intestinal obstruction. Case report 83 year old male, carrying a ventriculoperitoneal low pressure shunt for 8 years. The patient was admitted for emergency surgery after presentation for possible intestinal obstruction with radiological findings consistent with small bowel obstruction, probably related to the catheter of the ventriculoperitoneal shunt. Surgery confirmed bowel obstruction and a change of gauge of the terminal ileum. The small bowel was dilated into a ball, encased by a small fibrous capsule. Adhesiolysis and right hemicolectomy was performed with resection of the affected bowel segment. The pathology was consistent with sclerosing encapsulating peritonitis. Conclusions Sclerosing encapsulating peritonitis is a rare and potentially serious cause of bowel obstruction and its secondary form is usually associated with chronic peritoneal dialysis. The radiological diagnosis is difficult and the treatment of choice is surgical. We must consider it in cases of intestinal obstruction in patients with ventriculoperitoneal shunt.

Idiopathic Sclerosing Encapsulating Peritonitis Revealed by Intestinal Obstruction.

Sclerosing encapsulating peritonitis (SEP) is a very rare entity characterized by encasement of small intestine by a fi­brocollagenous membrane. It is divided into primary (idiopathic) which is named as abdominal cocoon and secondary forms. The preoperative diagnosis is difficult and most cases are diagnosed intraoperatively. A conservative treatment approach is the most suitable management strategy in asymptomatic idiopathic SEP. In this paper, we aimed to present a case of idiopathic SEP revealed by intestinal obstruction.

Sclerosing encapsulating peritonitis: a case report / Peritonite esclerosante encapsulante: relato de caso

Sclerosing encapsulating peritonitis, a rare cause of bowel obstruction, was described as a complication associated with peritoneal dialysis which is much feared because of its severity. The authors report a case where radiological findings in association with clinical symptoms have allowed for a noninvasive diagnosis of sclerosing encapsulating peritonitis, emphasizing the high sensitivity and specificity of computed tomography to demonstrate the characteristic findings of such a condition.

Peritonite esclerosante encapsulante, causa rara de obstrução intestinal, foi descrita como uma complicação associada à diálise peritoneal, muito temida por sua gravidade. Relata-se um caso em que os achados radiológicos associados aos sintomas clínicos permitiram o diagnóstico não invasivo de peritonite esclerosante encapsulante, destacando-se a alta sensibilidade e especificidade da tomografia computadorizada na demonstração dos achados característicos.

Peritonite encapsulante: tomografia computadorizada e correlação cirúrgica / Encapsulating peritonitis: computed tomography and surgical correlation

A peritonite esclerosante encapsulante é uma entidade rara, muitas vezes grave, caracterizada pelo envolvimento total ou parcial de alças do intestino delgado por uma membrana de tecido fibroso. Apresenta quadro clínico inespecífico de obstrução intestinal, exigindo diagnóstico por imagem preciso para orientação do tratamento. O presente relato enfatiza a importância da tomografia computadorizada no diagnóstico desta doença, com confirmação por correlação cirúrgica.

Sclerosing encapsulating peritonitis is a rare and frequently severe entity characterized by total or partial involvement of small bowel loops by a membrane of fibrous tissue. The disease presents with nonspecific clinical features of intestinal obstruction, requiring precise imaging diagnosis to guide the treatment. The present report emphasizes the importance of computed tomography in the diagnosis of this condition and its confirmation by surgical correlation.

A Case of Sclerosing Encapsulating Peritonitis Presented with Systemic Lupus Erythematosus

Sclerosing encapsulating peritonitis (SEP) is characterized by peritoneal fibrosis and adhesion of the peritoneum with the loops of the small intestine. Although the prevalence is low, most cases are caused by peritoneal dialysis, infection, medication, systemic lupus erythematosus (SLE), and intra-abdominal neoplasm. We describe a 22-year old man who was presented with abdominal pain and distension, which were attributed to SLE with peritonitis. He had no specific history of previous medical illness and peritoneal dialysis. He was treated with intravenous high dose methylprednisolone 1 g/day for 3 days, followed by intravenous methylprednisolone 1 mg/kg daily and immunoglobulin. However, his symptoms did not improve. Eventually, a laparoscopic biopsy was performed for an accurate diagnosis. The histopathologic findings were presented in accordance to the typical characteristics of SEP. In spite of medical treatment, he did not show an improvement of clinical symptoms and radiologic findings. As a result, he died from nutritional deficiency, upper gastrointestinal bleeding, and congestive heart failure.

Sclerosing Encapsulating Peritonitis (Abdominal Cocoon) after Abdominal Hysterectomy

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.

Clinical Characteristics of Dialysis Related Sclerosing Encapsulating Peritonitis: Multi-center Experience in Korea

Sclerosing encapsulating peritonitis (SEP) is a rare but serious complication in patients with continuous ambulatory peritoneal dialysis (CAPD), and is characterized by a progressive, intra-abdominal, inflammatory process resulting in the formation of sheets of new fibrous tissue, which cover, bind, and constrict the viscera, thereby compromising the motility of the bowel. No satisfactory estimate is available on the comparative incidence of dialysis related SEP and the pathogenesis of SEP still remains uncertain. Although recent therapeutic approaches have reported varying degrees of success, an efficient measure to detect, at an early stage, patients at risk for SEP would be beneficial and a standardized treatment regimen to prevent the illness is urgently needed. This study aimed to evaluate the clinical features of SEP and to identify the possible risk factors for the development of SEP in CAPD patients. We retrospectively reviewed by questionnaire SEP cases among CAPD patients from 7 university hospital dialysis centers in Korea, including Yonsei University, Ajou University, Catholic University, Inha University, Kyungpook University, Seoul National University and Soonchunhyang University, from January 1981 to December 2002. Out of a total of 4, 290 CAPD patients in these centers, 34 cases developed SEP with an overall prevalence of 0.79%. The male to female ratio was 17: 17. The median age of these patients was 44.5 years (range 19 - 66). The median duration of CAPD before SEP was 64 months (9 - 144) and 68% of patients (23/34) had been on CAPD for more than 4 years. Peritonitis (including two fungal cases) was the main cause of catheter removal in SEP (27 cases, 79%). Seventy-five percent of the cases (15/ 20) were administered beta-blocker for a mean duration of 85 months (26 - 130). Among 10 cases with available peritoneal equilibration test (PET) data, 8 showed high transporter characteristics, and the remaining 2 were high average. Eighteen cases were diagnosed by clinical and radiologic methods, and 16 were surgically diagnosed. Eleven cases were surgically treated and the others were treated conservatively with intermittent total parenteral nutrition (TPN). The overall mortality rate was 24%. SEP is a serious, life threatening complication of CAPD. Most cases had a PD duration of more than 4 years, a history of severe peritonitis, and high transporter characteristics in PET. Therefore, to reduce the incidence of SEP, careful monitoring and treatment, including early catheter removal in patients with severe peritonitis, should be considered for long-term CAPD patients with the above characteristics.

A Case of Small Bowel Perforation Associated with Sclerosing Encapsulating Peritonitis in Patient with Intermittent Peritoneal Dialysis (IPD) / 대한신장학회잡지

Sclerosing ecapsulating peritonitis (SEP) first described by Gandhi and Humyn at 1980 is generally recognized, but uncommon complication of continous ambulatory peritoneal dialysis (CAPD) and the prognosis is very poor. A 62-year old female was admitted to our hospital with chief complaint of abdominal pain, nausea, vomiting. On physical examination, abdominal pain was not detected. Abdominal CT demonstrated paralytic ileus and adhesion in proximal ileum. She underwent adhesilolysis of ileum and repair of perforated ileum. We experienced one case of SEP presenting small bowel perforation and peritonitis in patient with IPD. We report this case with review of the literature.

A case of surgical treatment after steroid therapy for early onset sclerosing encapsulating peritonitis in patient undergoing continuous ambulatory peritoneal dialysis (CAPD) / 대한내과학회지

Sclerosing encapsulating peritonitis (SEP) is the peritoneal thickness including the state of peritoneal fibrosis resulting from peritoneal inflammatory process of multifactorial etiologies. The symptoms and signs related to SEP include anorexia, nausea, vomiting, diarrhea, constipation, abdominal distension, fever, weight loss, abdominal pain and partial or intermittent bowel obstruction resulting from disturbance of the intestinal motility by the peritoneal sclerosis. The choice of management is considered as conservative treatment. However, if surgical intervention is necessary, the prognosis after surgery is usually poor. Recently, a few reports have suggested that immunosuppression (cyclophosphamide, azathioprine (100~125 mg), colchicine) and/or corticosteroid (30~50 mg) therapy might be effective in the treatment of SEP. We report a case of SEP that developed earlier than previous reported cases in patient undergoing continuous ambulatory peritoneal dialysis (CAPD) and report the surgical treatment of SEP which responded to steroid therapy thereafter symptoms by mechanical ileus were recurred.

Two Cases of Resection of Localized Sclerosing Encapsulating Peritonitis

Sclerosing encapsulating peritonitis (SEP), or abdominal cocoon, is generally recognized as a rare complication of continuous ambulatory peritoneal dialysis (CAPD), and the prognosis is very poor. The causes of SEP are multifactorial, including acetate in dialysate, recurrent peritonitis and dialysate con tamination with antiseptics containing chlorhexidine. Patients experience the characteristic symptoms and signs of nausea, vomiting, abdominal pain, and partial or intermittent bowel obstruction, and weight loss and malnutrition develop in severe case. We performed bowel resection on two patients with SEP, one idiopathic, and the other from CAPD. We briefly review the literature, and discuss the pathophysiology and the management of SEP.

A case of sclerosing encapsulating peritonitis presenting a huge pseudocyst formation in end stage renal failure on the maintenance hemodialysis / 대한내과학회지

Sclerosing encapsulating peritonitis(SEP) is now a well-recognised, but uncommon complication of chronic ambulatory peritoneal dialysis(CAPD). The etiology is unclear, but SEP is believed to be multifactorial. The most important factors seem to be the length of time on peritoneal dialysis and previous episodes of peritonitis. Anorexia, nausea, vomiting, weight loss, abdominal pain, and a reduction of the ability of the peritoneum to act as a dialyzing membrane are the usual presenting problems. We experienced one case of SEP presenting a huge pseudocyst in the patient of end stage renal failure on the maintenance hemodialysis for 5 years, even though he had a history of CAPD for 5 years. So we report this case with review of the literature.