Objectives

To discuss diagnostic radiologic imaging modalities and findings of low-phospholipid associated cholelithiasis (LPAC) syndrome.

Background

LPAC syndrome is a genetic disorder associated with dysfunction of biliary carrier protein (MDR3), which consequently may lead to cholelithiasis due to excessive bile accumulation.

LPAC syndrome is defined by the appearance of at least two of the three diagnostic criteria: onset of biliary lithiasis symptoms in a young adult (less than 40 years of age), recurrent biliary pain subsequent to cholecystectomy and typical radiological signs of intrahepatic lithiasis.

Although the majority of patients with LPAC syndrome have been cholecystectomized prior to the diagnosis of the syndrome, the standard treatment of choice is ursodeoxycholic acid.

Imaging findings OR Procedure findings

Imaging findings by means of ultrasound, magnetic resonance cholangiopancreatography and computed tomography are not specific and may include intrahepatic stones, choledocholithiasis and biliary duct dilatation. Among these intrahepatic stones are the most common finding, tending to involve one liver segment or lobe, occurring more commonly on the left side.

Besides initial assessment of LPAC syndrome diagnostic criteria, cross-sectional imaging may serve as a reasonable imaging tool in detection of possible LPAC syndrome complications (i.e., acute and/or chronic cholangitis, intrahepatic abscess and intrahepatic cholangiocarcinoma).

Conclusion

Radiologist should be informed about LPAC syndrome, its diagnostic criteria and possible imaging findings in order to provide patients with efficient medical treatment and to avoid unnecessary surgery.

Objectives

To discuss diagnostic criteria of splenic artery steal syndrome (SASS).

To present the role of interventional radiology in the management of this post-liver transplant complication.

Background

SASS is probably under-recognized vascular complication after liver transplantation (LT). It is defined by the reduction of the arterial inflow in the hepatic artery caused by increased flow in some other branch of the celiac trunk, most commonly in the splenic artery, although cases of gastroduodenal and left gastric artery steal syndrome have also been reported.

SASS causes hepatic hypoperfusion with clinical manifestations of graft dysfunction, as evidenced by abnormal liver function tests and cholestasis, possibly requiring re-LT.

Imaging findings OR Procedure findings

Currently, there are no specific clinical or diagnostic criteria for SASS. In proper clinical settings and based on CDUS, MDCT and MRI imaging findings only suspicion of SASS could be made.

Confirming the diagnosis requires conventional angiography, where the following diagnostic criteria must be met:

- splenic artery to hepatic artery diameter ratio >1.5 (or increased splenic artery diameter > 4 mm,

- early filling of the splenic artery with delayed hepatic artery filling,

- late or lack of filling of the intrahepatic branches of the hepatic artery.

Although these findings are highly suspicious of SASS, diagnosis is definitely confirmed only after successful endovascular treatment and normalization of enzyme levels.

Conclusion

In adequate clinical settings, a high degree of suspicion for diagnosing SASS should be made by the radiologists, since knowing its diagnostic criteria and possible treatment options may prevent graft failure and unnecessary re-LT.

Purpose

The role of this prospective preliminary study was to assess whether secretin-enhanced MR cholangiopancreatography (S-MRCP) can detect early pancreatic exocrine insufficiency (PEI).

Material and methods

17 patients underwent S-MRCP. Images were evaluated for grade of duodenal filling (PEI if ≤ grade 2) and indirect signs of main pancreatic duct (MPD) compliance (degree of dilatation; return to normal after secretin application). Patients were divided into three groups according to fecal elastase 1 (FE-1) levels: group 1 (>200 µg/g, expected normal exocrine function), group 2 (15-200 µg/g, exocrine disfunction) and group 3 (<15 µg/g, chronic pancreatitis on therapy). Correlations beetween imaging parameters and different FE-1 groups were calculated with Spearman's correlation test.

Results

There were 9 patients in FE-1 group 1 (median, 323µg/g; range 270 – 593µg/g), 3 patients in group 2 (178 µg/g; 170 – 198µg/g) and 1 patient in group 3 (14µg/g). Grade 3 duodenal filling was detected in 12 patients (71.6%). 4 of 5 patients with signs of PEI on S-MRCP (4 patients, grade 2; 1 patient, grade 1 filling, respectively) and expected normal exocrine function according to FE-1 levels (275 µg/g; 205-325 µg/g) also showed signs of decreased MPD compliance (no dilatation, 3 patients; delayed return to normal; 1 patient). A borderline negative significant correlation was found between the degree of MPD dilatation and FE-1 groups (p=0.079, r=-0.533) while grade of duodenal filling (p=0.696) and return to normal (p=0.110) showed no significant correlation.

Conclusion

S-MRCP may potentionally indicate patients with early PEI. Larger prospective trials are needed to validate these data.

Purpose

The role of this prospective preliminary study was to assess whether secretin-enhanced MR cholangiopancreatography (S-MRCP) can detect early pancreatic exocrine insufficiency (PEI).

Material and methods

17 patients underwent S-MRCP. Images were evaluated for grade of duodenal filling (PEI if ≤ grade 2) and indirect signs of main pancreatic duct (MPD) compliance (degree of dilatation; return to normal after secretin application). Patients were divided into three groups according to fecal elastase 1 (FE-1) levels: group 1 (>200 µg/g, expected normal exocrine function), group 2 (15-200 µg/g, exocrine disfunction) and group 3 (<15 µg/g, chronic pancreatitis on therapy). Correlations beetween imaging parameters and different FE-1 groups were calculated with Spearman's correlation test.

Results

There were 9 patients in FE-1 group 1 (median, 323µg/g; range 270 – 593µg/g), 3 patients in group 2 (178 µg/g; 170 – 198µg/g) and 1 patient in group 3 (14µg/g). Grade 3 duodenal filling was detected in 12 patients (71.6%). 4 of 5 patients with signs of PEI on S-MRCP (4 patients, grade 2; 1 patient, grade 1 filling, respectively) and expected normal exocrine function according to FE-1 levels (275 µg/g; 205-325 µg/g) also showed signs of decreased MPD compliance (no dilatation, 3 patients; delayed return to normal; 1 patient). A borderline negative significant correlation was found between the degree of MPD dilatation and FE-1 groups (p=0.079, r=-0.533) while grade of duodenal filling (p=0.696) and return to normal (p=0.110) showed no significant correlation.

Conclusion

S-MRCP may potentionally indicate patients with early PEI. Larger prospective trials are needed to validate these data.