Introduction

Walled-off necrosis (WON) may develop in the long-term follow-up after acute pancreatitis due to acute inflammation of the pancreas. It can be managed through endoscopic, percutaneous, or surgical drainage, depending on its clinical condition. The methodology is a topic of debate due to the scarcity of data regarding the progression of asymptomatic WON in patients (1).

Aims & Methods

The aim of this study is to determine the course of asymptomatic individuals who have developed walled-off necrosis (WON) following to an episode of acute pancreatitis over a long time of observation. A retrospective evaluation was conducted on 46 (3.9%) out of 1173 patients diagnosed with acute pancreatitis between June 2016 and December 2019, who developed WON during the follow-up period. Individuals who exhibited symptoms either at the point of diagnosis or during subsequent monitoring had been determined. An analysis was made between the clinical and WON characteristics at baseline of patients who required drainage during follow-up and those who did not.

Results

Out of the total of 46 patients who developed WON, 4 individuals were excluded due to inadequate follow-up data, 9 patients underwent drainage at the time of diagnosis, and 2 patients lost without receiving any form of drainage intervention. During the course of the study, a total of 31 patients were followed up for a mean duration of 25.20±17.40 months. Out of these patients, 10 (32.3%) required interventional intervention within a mean duration of 122.5±152.5 days (Group 1), while the remaining 21 patients (67.7%) were monitored without any intervention. Embolization was executed on a single patient owing to the presence of a splenic artery aneurysm identified in WON, however, drainage was not carried out. Endoscopic drainage was employed in five patients, percutaneous drainage in three patients, and surgical drainage in one patient. The prevalence of biliary etiology was found to be significantly higher in Group 1 compared to Group 2 (90.0% vs 47.6%, p<0.050). The study revealed that the Intra-WON necrosis rate and initial WON size were significantly higher in patients who required drainage at follow-up. The Intra-WON necrosis rate was 68.50±28.50% compared to 38.10±26.90% in the non-drainage group (p<0.05). Similarly, the initial WON size was 125.90±46.60 mm in the drainage group compared to 89.30±45.00 mm in the non-drainage group (p<0.05).

Conclusion

Asymptomatic patients with walled-off necrosis (WON) ought to be managed conservatively through careful ongoing monitoring. It is recommended that the results obtained be validated through extensive longitudinal research.

References

1)Freeman MF, Werner J, van Santvoort HC, et al. International Multidisciplinary Panel of Speakers and Moderators. Interventions for necrotizing pancreatitis: summary of a multi-disciplinary consensus conference. Pancreas. 2012;41(8):1176-1194.

Clinical Case Summary

Familial intrahepatic cholestasis is a heterogeneous group of genetic diseases with autosomal recessive transmission. BSEP (Bile-Salt Export Pump) is the main bile acid export pump in hepatocytes. Mutations in the gene encoding BSEP, ABCB11, are associated with heterogeneous clinical phenotypes ranging from diseases with a benign course to severe forms requiring liver transplantation. Here we report the case of a 17-year-old girl admitted to our Clinic because of severe jaundice and itching. She reported a similar episode 2 years earlier with spontaneous recovery. Family history is positive for cholelithiasis. On admission, laboratory tests showed: total and direct bilirubin values of 24.5 mg/dl and 18.9 mg/dl, AST 307 IU/L and ALT 238 IU/L, respectively. GammaGT was normal. Viral, autoimmune, metabolic causes, and biliary obstruction were excluded. Liver biopsy showed a picture of hepatocellular and intracanalicular cholestasis with cholestatic rosettes, inflammatory activity, and mild portal and pericellular fibrosis. Immunohistochemical staining for BSEP showed only weak diffuse expression. Genetic testing documented compound heterozygosity of the ABCB11 gene with the variants c.1460G>A - p(Arg487His) of paternal origin and c2599C>T -(Gln867*) of maternal origin. Genetic testing of relatives is also ongoing. The patient was treated with ursodesoxycholic acid, cholestyramine and cetirizine for 4 months with disappearance of jaundice and pruritus and normalization of bilirubin levels. Follow-up at 1 year was free of events without therapy. This is the first time that the c2599C>T -(Gln867*) variant has been described in a patient with familial intrahepatic cholestasis. Application of the "temporal test" will allow this new variant to be attributed to the BRIC2 form rather than PFIC2.