Para-normal-centesis: Percutaneous suction and traction removal of sclerosing peritonitis.

A 41-year-old female with a history of alcohol and alpha-1 antitrypsin deficiency associated cirrhosis, traumatic brain injury, anxiety, panic attacks, depression, and recurrent spontaneous bacterial peritonitis (SBP) presented with failure to drain ascites on repeated paracentesis. The patient had no previous abdominal surgery, autoimmune disease, malignancy, or other known risk factors for SP. The patient had no significant family history. Three months prior to presentation, the patient had been undergoing weekly large volume ultrasound-guided paracentesis with a 5 French needle-catheter for refractory ascites. During the first 2 months, paracentesis drainage averaged approximately 3-4 L weekly. One month before treatment, drainage volume abruptly decreased to 0.75 L despite the patient complaining of abdominal fullness and weight gain. A loculation was visualized on ultrasound ( Fig. 1 A ) . Paracentesis drainage then decreased to 0.2 L the subsequent week. Two additional attempts at ultrasound-guided paracentesis failed to yield fluid despite multiple peritoneal punctures at different sites. Ultrasonography revealed aggregation of echogenic tissue in the peritoneum of unclear etiology ( Fig. 1 B ). The patient’s abdominal fullness and ascites progressively worsened. Physical exam was notable for tense ascites, mild abdominal tenderness, and 2+ edema in the lower extremities, bilaterally. Transjugular intrahepatic portosystemic shunt creation was contraindicated by her Model for End-Stage Liver Disease 3.0 score of 30 (bilirubin: 6.1 g/dL, INR: 2.6, Cr: 1.6 mg/dL, Na: 146 mmol/L, albumin: 3.0 g/dL). Her prior SBP and loculations precluded peritoneovenous shunting, and she was deemed high risk for exploratory laparotomy. The decision was made to re-attempt paracentesis with sheath to disrupt and remove the loculations and debris before pursuing more invasive treatments. Fig. 1 Ultrasound images during paracenteses tracking echogenic, proteinaceous material in the peritoneum over time. (A) Three months prior to the diagnosis of sclerosing mesenteritis showing a small band of material in the peritoneum and (B) Day of diagnosis showing a large aggregate of tissue preventing fluid drainage. Fig 1 – dummy alt text Ultrasound images during paracenteses tracking echogenic, proteinaceous material in the peritoneum over time. (A) Three months prior to the diagnosis of sclerosing mesenteritis showing a small band of material in the peritoneum and (B) Day of diagnosis showing a large aggregate of tissue preventing fluid drainage. Under ultrasound and fluoroscopic guidance, a 6 French, 45 cm sheath was placed in the peritoneum in the vicinity of the visualized debris ( Fig. 2 ) . Syringe suction was applied as the sheath was slowly withdrawn without any fluid drainage. As the sheath was removed from the skin, a 2 cm segment of white, fibrous tissue protruded through the puncture site. Using manual traction, a 40 cm long pale cord was removed and sent to pathology ( Fig. 3 ). Histopathology demonstrated features of SP: fibrous tissue, reactive mesothelial cells, and prominent myofibroblast proliferation ( Figs. 4 A and B). No evidence of malignancy was present on tissue analysis. Paracentesis then removed 3 L of ascites without complication. All subsequent paracenteses drained over 3 L without difficulty and with minimal residual peritoneal debris present. Throughout the time of clinical presentation, the patient was in the process of liver transplantation evaluation. Unfortunately, she passed away after a traumatic fall about 6 months postintervention. Fig. 2 Fluoroscopy imaging from 6 French sheath placement, suction, and retraction to remove sclerosed peritoneal debris. Fig 2 – dummy alt text Fig. 3 Aggregate of thick white opaque rubbery mucinous tissue (3.7 × 3.2 × 0.7 cm) obtained from sheath placement in the peritoneum, followed by suction and manual traction. Pathology indicated features of sclerosing mesenteritis with no evidence of malignancy. Fig 3 – dummy alt text Fig. 4 Histopathological features of the obtained tissue demonstrating peritoneal inflammation, fibrosis, and sclerosis. (A) Cavities lined by reactive mesothelial cells with surrounding fibrous tissue. Prominent myofibroblastic proliferation (black arrow) and rare Langerhans giant cells (white arrow) are visualized (hematoxylin-eosin, original magnification × 100) and (B) Reactive features include pale cytoplasm, prominent nucleoli, and indistinct cell borders. Immunohistochemical staining was positive for calretinin, WT1, and CK7 in these cells, as expected (hematoxylin-eosin, original magnification × 400). Fig 4 – dummy alt text Fluoroscopy imaging from 6 French sheath placement, suction, and retraction to remove sclerosed peritoneal debris. Aggregate of thick white opaque rubbery mucinous tissue (3.7 × 3.2 × 0.7 cm) obtained from sheath placement in the peritoneum, followed by suction and manual traction. Pathology indicated features of sclerosing mesenteritis with no evidence of malignancy. Histopathological features of the obtained tissue demonstrating peritoneal inflammation, fibrosis, and sclerosis. (A) Cavities lined by reactive mesothelial cells with surrounding fibrous tissue. Prominent myofibroblastic proliferation (black arrow) and rare Langerhans giant cells (white arrow) are visualized (hematoxylin-eosin, original magnification × 100) and (B) Reactive features include pale cytoplasm, prominent nucleoli, and indistinct cell borders. Immunohistochemical staining was positive for calretinin, WT1, and CK7 in these cells, as expected (hematoxylin-eosin, original magnification × 400).

All authors have read and contributed to this manuscript. This work has not been previously published and is not under review elsewhere for publication.

A written informed consent was obtained from the patient for the publication of this case report.

Sclerosing peritonitis is uncommonly encountered and may present with a range of abdominal symptoms including impeded paracentesis. Percutaneous removal of the loculations and echogenic peritoneal debris through sheath suction restored the ability to perform successful paracentesis and avoided more invasive treatments.

Sclerosing peritonitis is an uncommon diagnosis, which is asymptomatic but may present with intermittent abdominal pain and even small bowel obstruction from fibrous compression. Other symptoms include nausea, loss of appetite, vomiting, constipation, and weight loss [ 1 , 2 ]. Many patients with SP experience chronic, nonspecific symptoms which evade diagnosis until clinical presentation with acute bowel obstruction, ischemia, or perforation [ 2 ]. Only 206 cases of SP have been reported from 1980 to 2020 [ 3 ]. Nonetheless, it should be considered in the differential for paroxysmal abdominal pain, especially after the exclusion of more common etiologies and particularly if peritoneal abnormalities, small bowel abnormalities, or loculated fluid collections are present. This case report shares a presentation of SP which likely developed from recurrent spontaneous bacterial peritonitis for which the patient was hospitalized twice the previous year. The patient had no previous abdominal surgery, autoimmune disease, malignancy, or other known risk factors for SP. Diagnostic criteria for SP are not well defined and a broad spectrum of associated, yet nonspecific, imaging findings may be present. Abdominal CT with IV contrast is the primary diagnostic modality for SP, showing peritoneal thickening and calcification. The thickened peritoneum can progress to surround the small bowel wall, causing obstruction, lumen narrowing, perforation, or other small bowel pathology. Thickened peritoneal membranes and associated small bowel involvement can lead to loculated fluid collections, especially in the setting of peritoneal dialysis or ascites [ 4 ]. Ultrasound can be a useful adjunctive modality to identify loculations as in the presented case. Although imaging may be helpful in cases of high clinical suspicion, diagnosis of SP is commonly made with tissue analysis confirmation. Treatment for mild SP is medical and depends on the disease progression. SP in the inflammatory stage is managed through steroids and other anti-inflammatory agents. When SP progresses to the fibrotic stage, the addition of tamoxifen or other fibrinolytic agents is beneficial [ 5 ]. Many patients with SP require nutritional support through total parenteral nutrition if SP compromises oral intake. Advanced SP is treated through surgical membrane excision with adhesiolysis and treatment of associated small bowel pathology. While surgery remains the gold standard for SP treatment, it is not recommended for patients with asymptomatic SP or ascites and as in this case, percutaneous image guided therapies could conceivably contribute to treatment [ 4 , 5 ]. In this setting, the echogenic peritoneal debris was visualized and engaged through sheath suction for percutaneous removal. Ultimately, this restored the ability to perform successful paracentesis and avoided more invasive treatments.

Sclerosing peritonitis (SP) is defined by chronic inflammation, fibrotic thickening, and sclerosis of the peritoneum. Although primary SP is idiopathic, secondary SP is more common and attributable to prolonged peritoneal dialysis, endometriosis, prior trauma or surgery, autoimmune or inflammatory diseases, medications, or peritoneal infections [ 1 , 2 ]. SP may be asymptomatic or have varied symptoms including recurrent abdominal pain, acute or chronic intestinal obstruction, anorexia, nausea, vomiting, and ascites. Definitive diagnosis commonly occurs during exploratory laparotomy with histopathologic confirmation. This case shares a unique presentation, diagnosis, and treatment of SP, which arose from recurrent spontaneous bacterial peritonitis and prevented paracentesis in a cirrhotic patient.