Spontaneous rupture of a mucinous cystic neoplasm of the liver resulting in a huge biloma in a pregnant woman: A case report.

BACKGROUND: Mucinous cystic neoplasm of the liver (MCN-L) and intraductal papillary neoplasm of the bile duct (IPN-B) are two different types of mucin-producing bile duct tumour that may complicate the course of pregnancy. To the best of our knowledge, we describe herein the first case of MCN-L with spontaneous rupture during pregnancy necessitating complex surgical treatment. CASE SUMMARY: A 24-year-old woman was initially admitted to another hospital in October 2018 with signs of jaundice (serum bilirubin level 12 mg/dL) and upper abdominal pain radiating to the left shoulder. Initial magnetic resonance imaging (MRI) of the abdominal cavity revealed a multilocular cystic tumour of the liver hilum (37 mm × 40 mm in diameter) located between segments 3 and 4 of the left liver lobe. Six weeks later (December 2018), the patient was found to be 12 wk pregnant and was referred to our institution for further diagnostics and treatment. At admission, a soft, palpable, and tender mass in the left upper abdomen was found. It was determined via MRI (with no intravenous contrast in view of the first-trimester pregnancy) to be a large collection of fluid (19 cm × 17 cm × 10 cm) located close to the liver hilum and below the left liver lobe. The patient did not undergo any diagnostic or therapeutic procedures nor did they have any abdominal trauma in the preceding weeks. The fluid collection proved to be of biliary origin following percutaneous drainage. Therefore, we concluded this was a spontaneous rupture of an MCN-L with the formation of a biloma. The MRI study also revealed the previously found cystic tumour of the liver hilum communicating with the left hepatic duct, which, together with left hepatic duct dilatation, suggested the diagnosis of IPN-B. The follow-up MRI with intravenous gadolinium contrast performed in the second trimester of pregnancy (week 14) showed, in turn, some features of MCN-L, including enhancement of the internal septations within the cystic liver mass. A precise preoperative differential diagnosis between IPN-B and MCN-L was therefore not possible. The patient was submitted to surgery in the second trimester of pregnancy (week 18). Surgery included a cholecystectomy, left hepatectomy, and concomitant resection of the extrahepatic bile ducts followed by anastomosis of the right hepatic duct with the Roux limb of the jejunum. The post-operative period was uneventful and the patient was discharged 8 days after surgery. The histopathological examination of the resected specimen revealed a final diagnosis of MCN-L with low-grade dysplasia and epithelium surrounded by ovarian-type stromal tissue. The patient delivered a healthy baby girl and both remain well at present, after 2 years of follow-up since surgery. CONCLUSION: The differential diagnosis and management of MCN-L and IPN-B may be very challenging, particularly in the setting of pregnancy. When indications for surgery are obvious, the final diagnosis is based on histopathological examination, with ovarian-type stroma being pathognomonic for MCN-L. We believe that the growth of this subepithelial stroma secondary to the high levels of sex hormones produced during pregnancy might have been the main causative factor leading to the tumour rupture with the formation of a biloma in our patient.

Chronic Fatigue Persists in a Significant Proportion of Female Patients After Transplantation for Primary Sclerosing Cholangitis.

Chronic fatigue and an impairment of general health-related quality of life (HRQoL) are frequently reported by patients with primary sclerosing cholangitis (PSC). Studies on patients with primary biliary cholangitis (PBC) suggest that, unlike pruritus, fatigue may not be ameliorated by liver transplantation (LT). However, there are few data regarding the assessment of fatigue before and after transplantation in PSC. To investigate the effect of LT on fatigue and HRQoL in patients with PSC, 81 patients with PSC (median age 33 years; 69% men) were prospectively enrolled in this study. The PBC-40 and Short Form 36 (SF-36) questionnaires were used for assessment before and twice after LT. A total of 26 patients who received a transplant for PBC were included as controls. The potential impact of the clinical and laboratory parameters was evaluated by univariate and multivariate analyses. Although in addition to other well-being indexes the median fatigue score improved after LT (P < 0.001), a detailed analysis demonstrated that fatigue persists in one-third of patients. A significant fatigue reduction was seen in men (P < 0.001) but not women (P = 0.25). Posttransplant fatigue did not depend on concomitant inflammatory bowel disease, laboratory indexes of cholestasis, or disease recurrence. In the multivariate regression model, female sex was the only independent covariate associated with persistent fatigue. In terms of other measures of HRQoL, LT caused a substantial improvement in the majority of SF-36 and PBC-40 domains. Recurrent PSC and unemployment negatively affected the well-being of patients. Patients who received a transplant for PSC had significantly better HRQoL than those patients with PBC. LT improves various measures of HRQoL, but it does not ameliorate fatigue in female patients with PSC.

Difficult iatrogenic bile duct injuries following different types of upper abdominal surgery: report of three cases and review of literature.

BACKGROUND: Iatrogenic bile duct injuries (BDIs) are mostly associated with laparoscopic cholecystectomy but may also occur following gastroduodenal surgery or liver resection. Delayed diagnosis of type of injury with an ongoing biliary leak as well as the management in a non-specialized general surgical units are still the main factors affecting the outcome. CASE PRESENTATION: Herein we present three types of BDIs (Bismuth type I, IV and V) following three different types of upper abdominal surgery, ie. Billroth II gastric resection, laparoscopic cholecystectomy and left hepatectomy. All of them were complex injuries with complete bile duct transections necessitating surgical treatment. All were also very difficult to treat mainly because of a delayed diagnosis of type of injury, associated biliary leak and as a consequence severe inflammatory changes within the liver hilum. The treatment was carried out in our specialist hepatobiliary unit and first focused on infection and inflammation control with adequate biliary drainage. This was followed by a delayed surgical repair with the technique which had to be tailored to the type of injury in each case. CONCLUSION: We emphasize that staged and individualized treatment strategy is often necessary in case of a delayed diagnosis of complex BDIs presenting with a biliary leak, inflammatory intraabdominal changes and infection. Referral of such patients to expert hepatobiliary centres is crucial for the outcome.

Cerebral amyloid angiopathy-related inflammation - A case report presenting diagnostic difficulties.

We describe an 86-year-old woman with a history of hypertension who presented sudden disturbances of consciousness and left hemiparesis. Brain magnetic resonance imaging (MRI) revealed diffused hyperintensive changes on T2-weighted images localized subcortically in the white matter of both cerebral hemispheres, corresponding to acute vasogenic edema, causing moderate mass effect. Posterior reversible encephalopathy syndrome was initially diagnosed. After implementation of anti-edema intravenous steroid treatment and hypotensive therapy the symptoms began to retire, till the total regression. The successive hospitalizations took place two and eight months later due to the occurrence of seizures, motor deficits and the development of mild cognitive impairment. Brain MRI revealed progression of the white matter changes and diffused subcortical microhemorrhages. Each time pulse steroid therapy was implemented and the symptoms improved significantly after several days. Chronic oral steroid treatment resulted in the stabilization of neurological status. The long-term observation of clinical symptoms, remission after immunosuppressive therapy and white matter changes with subcortical microhemorrhages in brain MRI leaded to the diagnosis of cerebral amyloid angiopathy-related inflammation.

Detection of active bile leak with Gd-EOB-DTPA enhanced MR cholangiography: comparison of 20-25 min delayed and 60-180 min delayed images.

OBJECTIVES: The purpose of this study was to assess the value of contrast-enhanced magnetic resonance cholangiography (MRC) performed in different time delays after injection of gadoxetic acid disodium (Gd-EOB-DTPA) for the diagnosis of active bile leak. METHODS: This retrospective analysis included Gd-EOB-DTPA enhanced MR images of 34 patients suspected of bile leak. Images were acquired 20-25 min after Gd-EOB-DTPA injection. If there was inadequate contrast in the bile ducts then delayed images after 60-90 min and 150-180 min were obtained. Results were correlated with intraoperative findings, ERCP results, clinical data, laboratory tests, and follow-up examinations. RESULTS: Gd-EOB-DTPA enhanced MRC yielded an overall sensitivity of 96.4%, specificity of 100% and accuracy of 97.1% for the diagnosis of an active bile leak. The sensitivity of 20-25 min delayed MR images was 42.9%, of combined 20-25 min and 60-90 min delayed images was 92.9% and of combined 20-25 min, 60-90 min and 150-180 min delayed images was 96.4%. CONCLUSIONS: Gd-EOB-DTPA enhanced MRC utilizing delayed phase images was effective for detecting the presence and location of active bile leaks. The images acquired 60-180 min post-injection enabled identification of bile leaks even in patients with a dilated biliary system or moderate liver dysfunction.

Biliary complications after liver transplantation: The role of MR imaging using different hydrographic sequences in patients with biliary-enteric and duct-to-duct biliary anastomosis.

BACKGROUND: To aim of this study was to assess the diagnostic performance of the state-of-the-art magnetic resonance cholangiography (MRC) comprising several 2D and 3D hydrographic sequences in patients after liver transplantation with biliary-enteric and duct-to-duct biliary anastomosis. MATERIAL AND METHODS: Retrospective analysis included MRC examinations of 42 patients (21 men, 21 women) performed from 18 days to 86 months (average, 18.9 months) after liver transplantation. Studies were carried out on 1.5 T units, using 4 hydrographic (turbo spin echo, TSE) sequences. The analysis included accuracy of MRC in detecting biliary complications, as well as frequency of specific complications in patients with biliary-enteric anastomosis (n=15) and duct-to-duct biliary anastomosis (n=27). In 34 patients the results were correlated with endoscopic retrograde cholangiopancreatography ERCP (n=9), ERCP and CT (n=5), ERCP and percutaneous cholangiography (n=2), ERCP and T-tube cholangiography (n=1), ERCP, PTC, and histopathology (n=1), PTC (n=1), PTC and CT (n=1), PTC and histopathology (n=1), T-tube cholangiography (n=4), T-tube cholangiography and CT (n=1), fistulography (n=1), CT (n=4), and histopathology (n=3). In the remaining 8 patients other imaging studies (US, CT, follow-up MRC), laboratory liver function tests, and clinical status were the standard of reference. Results MRC yielded 94.6% sensitivity in detecting biliary complications in patients after liver transplantation. In patients with biliary-enteric anastomosis, disseminated biliary strictures were more frequent than in patients with duct-to-duct biliary anastomosis (20% vs. 7%). Less frequently, the biliary-enteric anastomosis was accompanied by anastomotic strictures (40% vs. 56%) and the presence of stones/sludge (20% vs. 37%), but the differences did not show statistical significance. CONCLUSIONS: MRC allowed accurate diagnosis of biliary complications in patients after liver transplantation and therefore can be used as a safe alternative to invasive diagnostic procedures such as ERCP and PTC, especially in patients with biliary-enteric anastomosis, in which invasive diagnostic procedures are technically challenging. The main limitation of MR hydrography is its low accuracy in distinguishing bilomas from other fluid collections.

Pancreatic lipoma: An incydentaloma which can resemble cancer - analysis of 13 cases studied with CT and MRI.

BACKGROUND: The purpose of this article is to present computed tomography (CT) and magnetic resonance imaging (MRI) findings of rare pancreatic lipomas. MATERIAL/METHODS: The analysis covered 13 patients (7 men and 6 women, aged 47-88, average: 65.6 years), with 13 pancreatic lipomas, whose cases constituted the basis for 10 contrast-enhanced CT and 5 MRI studies. RESULTS: Lipomas measured from 6 mm to 32 mm (average 12.8 mm) and were located in the pancreatic head (n=7), body (n=2), tail (n=3) and uncinate process (n=1). Most lesions (n=11) were homogenous, well-circumscribed. On contrast-enhanced CT scans, macroscopic fat (<-30 HU) was present in 9 lipomas. In one case (10 mm lesion) the density was -20 HU and the lesion was poorly circumscribed with septations, which altogether made it difficult to precisely characterize its contents. On MR scans fat was demonstrated in all studied cases (n=5). CONCLUSIONS: Lipomas are rare, small, homogenous and well-circumscribed pancreatic tumours. The most important feature, decisive for the diagnosis and distinguishing them from pancreatic carcinoma, is detection of fatty tissue on CT and MR scans. In these cases differential diagnosis includes other rare fatty tumours of the pancreas (focal fatty infiltration, teratoma, liposarcoma).

Characterization of focal liver lesions using quantitative techniques: comparison of apparent diffusion coefficient values and T2 relaxation times.

OBJECTIVES: To compare the efficacy of two quantitative methods for discrimination between benign and malignant focal liver lesions (FLLs): apparent diffusion coefficient (ADC) values and T2 relaxation times. METHODS: Seventy-three patients with 215 confirmed FLLs (115 benign, 100 malignant) underwent 1.5-T MRI with respiratory-triggered single-shot SE DWI (b = 50, 400, 800) and dual-echo T2TSE (TR = 3,000 ms; TE1 = 84 ms; TE2 = 228 ms). ADC values and T2 relaxation times of FLLs were calculated. Sensitivity, specificity and accuracy of both techniques in diagnosing malignancy were assessed. RESULTS: The mean ADC value of malignant tumours (1.07 × 10(-3) mm(2)/s) was significantly lower (P < 0.05) than that of benign lesions (1.86 × 10(-3) mm(2)/s ); however, with the use of the optimal cut-off value of 1.25 × 10(-3) mm(2)/s, 20 false positive (FP) and 20 false negative (FN) diagnoses of malignancy were noted, generating 79 % sensitivity, 82.6 % specificity and 80.9 % accuracy. The mean T2 relaxation time of malignant tumours (64.4 ms) was significantly lower (P < 0.05) than that of benign lesions (476.1 ms). At the threshold of 107 ms 22 FP and 1 FN diagnoses were noted; the sensitivity was 99 %, specificity 80.9 % and accuracy 89.3 %. CONCLUSIONS: Quantitative analysis of T2 relaxation times yielded significantly higher sensitivity and accuracy in diagnosing malignant liver tumour than ADC values. KEY POINTS: • Diffusion-weighted magnetic resonance imaging is increasingly used for liver lesions. • But ADC values demonstrated only moderate accuracy for differentiation of liver lesions. • T2 relaxation times yielded higher accuracy in diagnosing malignant liver tumours. • Both ADC and T2 values overlapped between focal nodular hyperplasia and malignant lesions. • Nevertheless T2 liver mapping could be valuable for evaluating focal liver lesions.