Abdominal cocoon syndrome (sclerosing encapsulated peritonitis) causing small bowel occlusion in a patient with gastric bypass.

A rare condition, sclerosing encapsulating peritonitis, is characterized by a fibrotic membrane forming over the bowels, leading to intestinal obstruction. In this case of a 56-year-old male patient with a history of laparoscopic gastric bypass, a computed tomography scan showed findings indicative of the condition. Extensive adhesiolysis was performed, and biopsies confirmed the presence of fusiform cells (D2-40 positive on immunochemistry) resembling fibroblasts, within dense collagenous peritoneal tissue sheets, typical of sclerosing encapsulating peritonitis. The prevalence of this condition is uncertain, and diagnosis typically requires a peritoneal biopsy due to the nonspecific clinical presentation.

La peritonitis esclerosante encapsulada es una condición rara caracterizada por una membrana fibrótica que se genera sobre las asas intestinales causando cuadros de oclusión intestinal. Se presenta el caso de un paciente varón de 56 años con antecedente de derivación gastroyeyunal por laparoscopia que presenta oclusión intestinal. Se realizó tomografía computada que evidenció sitio de transición previo al sitio de anastomosis. Se realizó de anastomosis extensa y toma de biopsias. Histológicamente se observó engrosamiento de la membrana peritoneal, células fusiformes (D2-40 positivo en inmunohistoquímica) similares a fibroblastos con láminas de colágeno peritoneal denso. La peritonitis esclerosante encapsulada es una patología de prevalencia desconocida. El cuadro clínico es inespecífico y el diagnóstico definitivo es por patología con biopsia peritoneal.

Surgical Treatment of Medically Refractory Encapsulating Peritoneal Sclerosis in a Patient After Kidney Transplant.

Encapsulating peritoneal sclerosis is a rare but highly morbid disease process in patients with end-stage kidney disease on peritoneal dialysis. Surgical management has been described in patients with encapsulation of bowel causing obstruction. Here, we describe a case of surgical management in a patient following kidney transplant with medically refractory ascites and lower extremity edema.

Encapsulating Peritoneal Sclerosis 43 Years after Distal Gastrectomy for Early Gastric Cancer.

We herein report a case of encapsulating peritoneal sclerosis (EPS) in a patient without chronic kidney disease after gastrectomy. A 69-year-old man underwent distal gastrectomy for early gastric cancer at 25 years old. After 43 years, he developed bowel obstruction and underwent enterolysis of the encapsulated small intestine. A pathological examination of the capsular membranes revealed inflammation, foam, and giant cells that destroyed foreign substances. The patient was discharged 1.5 months later. Foreign body reactions to surgical instruments used in gastrectomy are considered a cause of EPS. EPS due to foreign body reactions to surgical instruments should also be considered in such cases.

Prediction of surgical outcomes in severe encapsulating peritoneal sclerosis using a computed tomography scoring system.

BACKGROUND/PURPOSE: Encapsulating peritoneal sclerosis (EPS) is a rare and potential lethal complication of peritoneal dialysis characterized by bowel obstruction. Surgical enterolysis is the only curative therapy. Currently, there are no tools for predicting postsurgical prognosis. This study aimed to identify a computed tomography (CT) scoring system that could predict mortality after surgery in patients with severe EPS. METHODS: This retrospective study enrolled patients with severe EPS who underwent surgical enterolysis in a tertiary referral medical center. The association of CT score with surgical outcomes including mortality, blood loss, and bowel perforation was analyzed. RESULTS: Thirty-four patients who underwent 37 procedures were recruited and divided into a survivor and non-survivor group. The survivor group had higher body mass indices (BMIs, 18.1 vs. 16.7 kg/m2, p = 0.035) and lower CT scores (11 vs. 17, p < 0.001) than the non-survivor group. The receiver operating characteristic curve revealed that a CT score of ≥15 could be considered a cutoff point to predict surgical mortality, with an area under the curve of 0.93, sensitivity of 88.9%, and specificity of 82.1%. Compared with the group with CT scores of <15, the group with CT scores of ≥15 had a lower BMI (19.7 vs. 16.2 kg/m2, p = 0.004), higher mortality (4.2% vs. 61.5%, p < 0.001), greater blood loss (50 vs. 400 mL, p = 0.007), and higher incidence of bowel perforation (12.5% vs. 61.5%, p = 0.006). CONCLUSION: The CT scoring system could be useful in predicting surgical risk in patients with severe EPS receiving enterolysis.

A case report of encapsulating peritoneal sclerosis followed by cesarean section: Clinical diagnosis and treatment experience.

RATIONALE: Encapsulating peritoneal sclerosis (EPS), also known as abdominal cocoon syndrome, is an uncommon condition that typically presents with features of bowel obstruction. EPS followed by cesarean section is extremely rare. Intestinal obstruction caused by EPS lacks specificity and poses clinical difficulties for diagnosis and treatment. We present the case of a patient with recurrent intestinal obstruction followed by cesarean section, and the diagnosis of EPS was confirmed intraoperatively. The patient recovered well postoperatively, and achieved satisfactory therapeutic results. Management of this condition tests the surgeon's knowledge and experience of the disease. PATIENT CONCERNS: A 27-year-old woman with recurrent abdominal pain and distention accompanied by reduced anal discharge and defecation there months. The patient had a history of cesarean section 4 months earlier and recovered well after operation. She had no other history of abdominal surgery or diseases. On examination, a 10-cm long transverse incision was made in the lower abdomen, and marking on the intestinal movements were observed in the left mid-abdomen. A long, soft lump with good mobility was touched in the left lower abdomen. The abdominal computed tomography and small bowel barium meal examination revealed incomplete intestinal obstruction. DIAGNOSIS: Incomplete small bowel obstruction due to abdominal adhesions after the cesarean section was initially considered. INTERVENTIONS: After conservative treatment, the symptom of intestinal obstruction still recurred. Thus, we decided to perform a surgery of repeated decortication of fibrous peritoneal membranes. OUTCOMES: The operation successfully released the intestinal obstruction and abdominal pain, postoperative course recovered smoothly. LESSONS: After cesarean section could develop EPS. Intestinal obstruction caused by EPS lacks specificity and poses clinical difficulty in diagnosis and treatment. The management of this condition tests the surgeon's knowledge and experience, and surgery is an effective treatment measure.

Surgical management of Encapsulating Peritoneal Sclerosis (EPS) in children: international case series and literature review.

BACKGROUND: Encapsulating Peritoneal Sclerosis (EPS) is a rare phenomenon in paediatric patients with kidney failure treated with peritoneal dialysis (PD). This study highlights clinical challenges in the management of EPS, with particular emphasis on peri-operative considerations and surgical technique. METHODS: Retrospective analysis of all paediatric patients with EPS treated at the Manchester Centre for Transplantation. RESULTS: Four patients were included with a median duration of 78 months on PD. All patients had recurrent peritonitis (> 3 episodes), and all had symptoms within three months of a change of dialysis modality from PD to haemodialysis or transplant. In Manchester, care was delivered by a multi-disciplinary team, including surgeons delivering the adult EPS surgical service with a particular focus on nutritional optimisation, sepsis control, and wound management. The surgery involved laparotomy, lavage, and enterolysis of the small bowel + / - stoma formation, depending on intra-abdominal contamination. Two patients had a formal stoma, which were reversed at three and six months, respectively. Two patients underwent primary closure of the abdomen, whereas two patients had re-look procedures at 48 h with secondary closure. One patient had a post-operative wound infection, which was managed medically. One patient's stoma became detached, leading to an intra-abdominal collection requiring re-laparotomy. The median length of stay was 25 days, and patients were discharged once enteral feeding was established. All patients remained free of recurrence with normal gut function and currently two out of four have functioning transplants. CONCLUSIONS: This series demonstrates 100% survival and parenteral feed independence following EPS surgery. Post-operative morbidity was common; however, with individualised experience-based decision-making and relevant additional interventions, patients made full recoveries. Health and development post-surgery continued, allowing the potential for transplantation. A higher resolution version of the Graphical abstract is available as Supplementary information.

Liver cirrhosis with encapsulating peritoneal sclerosis after 4 years of peritoneal dialysis: A case report.

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.

[Encapsulant peritonitis]. / Une péritonite encapsulante.

INTRODUCTION: Encapsulating peritonitis is a rare but severe chronic fibrotic condition related to the development of a white fibrous membrane surrounding the digestive tract. Idiopathic forms have been described, however the disease is most often secondary to peritoneal dialysis or more rarely to surgery. Treatment is difficult and not codified. CASE REPORT: We report here the observation of a 36-year-old patient whose diagnosis of encapsulating peritonitis was made after a long sub-occlusive history, eight years after a gastric ulcer perforation. DISCUSSION: We discuss the possible etiologies and we present a focus on this rare and little-known entity.

Massive recurrent hemoperitoneum with encapsulating peritonitis: another enigmatic clinical feature of endometriosis.

OBJECTIVE: To describe the clinical characteristics and laparoscopic findings of a very uncommon presentation of a patient with endometriosis. DESIGN: Video presentation of case report (Canadian Task Force classification III). (The institutional review board of the Hospital Naval Pedro Mallo, Buenos Aires, Argentina, has ruled that approval was not required for the publication of this case report.) SETTING: Hospital. PATIENT(S): Thirty-two-year-old woman with endometriosis presenting with hemorrhagic ascites. INTERVENTION(S): We demonstrate the laparoscopic appearance of the peritoneal organs in the presence of massive hemoperitoneum and encapsulating peritonitis and also describe the diagnosis and management options of an uncommon clinical presentation of endometriosis. The patient is a 32-year-old woman, gravida 0, who presented with abdominal pain and ascites. Initially, she underwent exploratory laparotomy with drainage of 5 liters of ascites and excision of endometrial peritoneal implants. She then presented 4 months later with sudden worsening abdominal pain and distention, weight gain, bloating, and shortness of breath. A diagnostic laparoscopy was performed with the findings of over 10 liters of dark hemoperitoneum and diffuse pelviperitonitis with loose necrotic, easy to remove, dense peritoneal tissue. Patient was started on triptorelin acetate with great response. MAIN OUTCOME MEASURE(S): Resolution of the symptomatology secondary to hemorrhagic peritonitis. RESULT(S): Clinical improvement of symptomatology of a patient with endometriosis and hemorrhagic ascites. CONCLUSION(S): Endometriosis can have different clinical presentations. Endometriosis should be a differential diagnosis in women of reproductive age presenting with massive hemorrhagic ascites. Hemorrhagic ascites, considered an exceedingly rare clinical course of endometriosis, represents a challenge to the surgeon who is unfamiliar with this condition. Bilateral oophorectomy is the definitive treatment, but conservative therapy is indicated for women of childbearing age. Diagnostic laparoscopy with drainage of hemoperitoneum is a feasible option to obtain a pathology-confirmed diagnosis in patients presenting with hemoperitoneum secondary to pelvic endometriosis. Awareness of this condition will prevent unnecessary aggressive resection, as is commonly performed when the condition is confused with ovarian cancer.

Abdominal cocoon syndrome: A rare cause of acute abdomen syndrome.

BACKGROUND: A rare cause of acute abdomen or intestinal obstruction, the abdominal cocoon syndrome is also described in the literature as sclerosing peritonitis or sclerosing encapsulating peritonitis. Abdominal cocoon is characterized by the total or partial wrapping of the abdominal organs by a fibrous membrane. Although it is usually observed in young women, the etiology is unknown. The diagnosis is usually made during laparotomy. In this case series, we aimed to present seven patients diagnosed with abdominal cocoon syndrome during operation. METHODS: The records of patients who underwent laparotomy for abdominal pain and/or intestinal obstruction in our hospital and diagnosed as abdominal cocoon during operation between January 2012 and November 2018 were retrospectively reviewed. The demographic characteristics of the patients, etiologic factors, surgical procedures, operative findings and follow-up of the patients were recorded. RESULTS: Four out of seven patients who were operated for abdominal cocoon were male and 3 of them were female. The median age of patients was 61 (57-63) years in male and 39.6 (28-49) years in female. Six of the patients were operated in emergency conditions with the diagnosis of an acute abdomen or ileus. One of the patients was operated with the diagnosis of an intra-abdominal mass in elective conditions. In five out of seven patients, all of the small intestines were wrapped with a fibrous collagen capsule, while two of the patient intestines were partially wrapped with a fibrous collagen capsule. Four of the patients had no underlying disease, while one of the patients had Familial Mediterranean Fever (FMF), one had Endometriosis and one had beta-blocker medication. One patient who had small bowel necrosis and septic peritonitis were observed during the operation and died post operative 6th days. Postoperative complications were not observed in the follow-up of other patients and reoperation was not required due to recurrence. CONCLUSION: Abdominal cocoon is a condition that is usually diagnosed during operation in patients that were operated for reasons, such as the acute abdomen or intestinal obstruction. When the diagnose delayed, death can be seen due to small bowel necrosis and septic complications. High clinical suspicion and radiological imaging are important in the preoperative diagnosis. Treatment is required adhesiolysis and excision of the fibrous membranes.

Congenital peritoneal encapsulation: A review and novel classification system.

Congenital peritoneal encapsulation (CPE) is a very rare, congenital condition characterised by the presence of an accessory peritoneal membrane which encases a variable extent of the small bowel. It is unclear how CPE develops, however it is currently understood to be a result of an aberrant adhesion in the peritoneal lining of the physiological hernia in foetal mid-gut development. The condition was first described in 1868, and subsequently there have been only 45 case reports of the phenomenon. No formal, systematised review of CPE has yet been performed, meaning the condition remains poorly understood, underdiagnosed and mismanaged. Diagnosis of CPE remains clinical with important adjuncts provided by imaging and diagnostic laparoscopy. Two thirds of patients present with abdominal pain, likely secondary to sub-acute bowel obstruction. A fixed, asymmetrical distension of the abdomen and differential consistency on abdominal palpation are more specific clinical features present in approximately 10% of cases. CPE is virtually undetectable on plain imaging, and is only detected on 40% of patients with computed tomography scan. Most patients will undergo diagnostic laparotomy to confirm the diagnosis. Management of CPE includes both medical management of the critically-unstable patient and surgical laparotomy, partial peritonectomy and adhesiolysis. Prognosis following prompt surgical treatment is excellent, with a majority of patients being symptom free at follow up. This review summarises the current literature on the aetiology, diagnosis and treatment of this rare disease. We also introduce a novel classification system for encapsulating bowel diseases, which may distinguish CPE from the commoner, more morbid conditions of abdominal cocoon and encapsulating peritoneal sclerosis.

Surgical Outcomes in Patients with Abdominal Cocoon: Series of 15 Patients.

BACKGROUND: Abdominal cocoon (AC) or sclerosing encapsulating peritonitis is an uncommon cause of intestinal obstruction. Surgical intervention is warranted in patients with persistent pain or intestinal obstruction. METHODOLOGY: A retrospective analysis of patients operated for AC was performed. Clinical presentation, radiological data, postoperative outcomes (Ryles tube (RT) removal, duration of hospital stay, enterocutaneous fistula, requirement for re-exploration and mortality) were retrieved and analyzed. RESULTS: Fifteen patients of abdominal cocoon required surgical intervention for various indications. The mean age was 34.46 years (13-60), and 11 (73.3%) were males. Intermittent abdominal pain was present in 14 (93.3%) followed by recurrent subacute intestinal obstruction (SAIO) in 11 (73.3%). Three patients presented with intestinal perforation. Of the 14 patients with preoperative computed tomography, radiological diagnosis was possible in five patients. The mean duration for surgery was 159 min (60-360 min). Membrane encasement was complete in 9/15 and partial in 6/15 patients. Adhesiolysis was done in all patients (complete-10/15 and partial-5/15). Mean duration for RT removal and hospital stay was 4.3 and 12.3 days, respectively. Recurrence of SAIO was observed in three patients, and one patient needed re-exploration for the same. One patient developed postoperative enterocutaneous fistula requiring surgical intervention. Overall mortality in the study was 13.3% (2/15). Four patients had underlying tuberculosis, and the rest were idiopathic. CONCLUSION: Etiology of AC is not known in majority of patients. Persistent pain and recurrent SAIO are the most common indications for surgery. This morbidity associated with surgery can be reduced by meticulous dissection techniques and appropriate peri-operative care.

Surgical treatment of post-transplant encapsulating peritoneal sclerosis: A single-center experience.

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) related to peritoneal dialysis (PD) has a vague etiology and high mortality. In this study, our aim was to determine treatment options for EPS cases. METHODS: A total of 169 patients underwent kidney transplantation from January 2008 to January 2018 and 119 patients from a cadaveric and 50 patients from a living donor. Twenty-one patients were undergoing PD before the transplantation. The mean PD time was 6.9 (IQR 3-14) years. Four patients received surgical treatment for EPS that occurred after the transplant. After the surgical treatment, 2 patients died because of sepsis. Two patients were discharged without complications, but 1 had late-term EPS recurrence. RESULTS: EPS is a rare but serious complication of long-term PD. It has a high mortality and morbidity rate. Long-term PD is the most significant factor for triggering EPS. Nutritional support and surgical intervention is the next step if medical treatment fails. Resistant cases should be treated surgically without much delay before the condition deteriorates. CONCLUSION: It can be especially devastating for patients with a long-term PD history to have EPS after a successful transplant. Because EPS is a challenging condition, its management should be done in experienced clinics to decrease its mortality and morbidity rates.

Surgical Treatment for Encapsulating Peritoneal Sclerosis: 24 Years' Experience.

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is a serious complication of long-term peritoneal dialysis. The mortality rate for EPS is high, primarily due to complications related to bowel obstruction. Surgery was previously contraindicated; however, surgical enterolysis is performed for patients in whom bowel obstruction fails to improve. METHODS: This was a retrospective observational study of patients with EPS who received surgical intervention at a single center between November 1993 and October 2017. The severity of intestine damage was characterized by grade-3 peritoneal calcification on abdominal computed tomography (CT) scan and degeneration of the small intestinal wall in surgery. RESULTS: Two-hundred and forty-three patients with EPS opted for surgery. Among them, 58 had recurrence and required re-surgery; a total of 318 EPS surgeries were performed. Death was related to EPS in 61 patients (25.1%), of whom 15 died postoperatively. Sixty-seven patients (27.6%) died from other causes. The actuarial survival rates at 1, 2, 3, 5, and 8 years after EPS diagnosis were 91%, 83%, 77%, 66%, and 53% respectively. The 50% actuarial survival points after EPS diagnosis and surgery were 104 months and 85 months, respectively. Peritoneal calcification and small intestinal wall degeneration grading showed significant association with the mortality curve for EPS-related death. CONCLUSION: Excellent outcomes for EPS are achieved with surgery. The degree of peritoneal deterioration affected the clinical outcomes. Currently, EPS is no longer recognized as a fatal complication.

Should End-of-Life Preferences Be Discussed Routinely before High-Risk Surgery?

Encapsulating peritoneal sclerosis (EPS) is a rare but devastating complication of peritoneal dialysis. It is characterized by peritoneal neovascularization, fibrosis, and calcification ultimately leading to intestinal obstruction and eventual failure. Surgery for EPS has a mortality approaching 50% and most patients require some form of postoperative life-sustaining therapy (LST) during their admission. A 43-year-old gentleman with progressive EPS and significant comorbidities was assessed for enterolysis after a failed first attempt at another center. Because of his comorbidities, postoperative mortality was quoted above 50%. The patient favored surgery to improve his survival and quality of life, but was reluctant to receive prolonged LST in the event of failure of surgical therapy. The surgical team, in conjunction with a palliative care physician, therefore held extensive discussions with the patient and his partner regarding LST and its limitations. Clinical parameters to trigger a transition to palliative care were identified and agreed. Limitations on LST that are directly expressed by patients can represent a contraindication to surgery for many surgeons. Surgical Buy-In is a concept described as a perceived contract, or covenant, between the patient and clinician regarding implied consent for postoperative LST. Currently, preoperative discussions regarding limitations of LST are infrequent, and there can be reticence among patients and surgeons to have these conversations, leading to dissatisfaction on behalf of the patient and their family. After the Montgomery legal ruling, the provision and perception of informed consent are particularly pertinent. The palliative care physician is uniquely placed to contribute to such discussions as part of the surgical multidisciplinary team.

Encapsulating sclerosing peritonitis : two rare observations and literature review.

Sclerosing encapsulating peritonitis (SEP) is a rare and little known pathological entity. It is a chronic fibro-inflammatory disease of the peritoneum, resulting in the formation of a thick fibrous membrane, which engages partially or totally the abdominal organs. Clinical and radiological signs make the diagnosis difficult to establish preoperatively. We present two original observations of SEP illustrating different clinical presentations, diagnostic and therapeutic means. His diagnosis requires a peritoneal biopsy. The treatment is not completely established. Surgical treatment is difficult, involving viscerolysis and multiple incisions of the fibrous membrane. Despite the current progress in therapeutic management, the prognosis remains pejorative, with significant mortality.