Emergent orthotopic liver transplantation for hemorrhage from a giant cavernous hepatic hemangioma: case report and review.

INTRODUCTION: Cavernous hemangiomas represent the most common benign primary hepatic neoplasm, often being incidentally detected. Although the majority of hepatic hemangiomas remain asymptomatic, symptomatic hepatic hemangiomas can present with abdominal pain, hemorrhage, biliary compression, or a consumptive coagulopathy. The optimal surgical management of symptomatic hepatic hemangiomas remains controversial, with resection, enucleation, and both deceased donor and living donor liver transplantation having been reported. CASE REPORT: We report the case of a patient found to have a unique syndrome of multiorgan cavernous hemangiomatosis involving the liver, lung, omentum, and spleen without cutaneous involvement. Sixteen years following her initial diagnosis, the patient suffered from intra-abdominal hemorrhage due to her giant cavernous hepatic hemangioma. Evidence of continued bleeding, in the setting of Kasabach-Merritt Syndrome and worsening abdominal compartment syndrome, prompted MELD exemption listing. The patient subsequently underwent emergent liver transplantation without complication. CONCLUSION: Although cavernous hemangiomas represent the most common benign primary hepatic neoplasm, hepatic hemangioma rupture remains a rare presentation in these patients. Management at a center with expertise in liver transplantation is warranted for those patients presenting with worsening DIC or hemorrhage, given the potential for rapid clinical decompensation.

Diagnosis of primary sclerosing cholangitis: a blinded comparative study using magnetic resonance cholangiography and endoscopic retrograde cholangiography.

BACKGROUND: We hypothesized that magnetic resonance cholangiography (MRC) may have less accuracy for the diagnosis and the assessment of the severity of primary sclerosing cholangitis (PSC) than endoscopic retrograde cholangiography (ERC). OBJECTIVE: The aim of this study was to determine the diagnostic accuracy and interobserver agreement of both ERC and MRC in PSC. DESIGN: A case-control study. SETTING: University Hospital. PATIENTS: ERCs and MRCs of 36 patients with PSC and 51 controls (normal/other biliary tract diseases) were read in an independent, blinded, and random fashion by 2 magnetic resonance radiologists and 2 interventional endoscopists by using a previously validated classification system. Readers had no access to clinical history, laboratory results, or patient mix. RESULTS: Extrahepatic ductal (EHD) and intrahepatic ductal (IHD) visualization was excellent in 64% of 66% of MRCs and 86% of 74% of ERCs. Sensitivity and specificity for diagnosis of PSC for readers 1 to 4 were 91% and 85%, 88% and 90%, 81% and 96%, and 83% and 96%. respectively. Receiver operating curve values were excellent for all readers (all >0.9). Interobserver agreement (kappa statistics) for the diagnosis of PSC (MRC, 0.83; ERC, 0.73) and for identifying the presence of IHD strictures (MRC, 0.64; ERC, 0.86) was good for both modalities, but only ERC (ERC, 0.55; MRC, 0.36) was good for the presence and the severity of EHD strictures. When assessment of disease severity was limited to the 36 patients with PSC, interobserver agreement was very poor for both MRC (0.23 and 0.07 for EHD and IHD, respectively) and ERC (0.24 and 0.34 for EHD and IHD, respectively). LIMITATIONS: The retrospective case-control study made it difficult to assess the impact of the diagnosis on patient management. CONCLUSIONS: ERC and MRC were comparable for diagnosing PSC, with very good interobserver agreement for the diagnosis of PSC and IHD strictures. Only ERC had good agreement for EHD strictures. Interobserver agreement was very poor for both MRC and ERC when disease severity of PSC was assessed.

Utility of bile duct brushings for the early detection of cholangiocarcinoma in patients with primary sclerosing cholangitis.

BACKGROUND: In patients with primary sclerosing cholangitis, there are no reliable markers or imaging modalities to detect malignant changes or early cholangiocarcinoma when curative interventions may still be possible. METHODS: Clinical features and outcomes were evaluated for 47 patients who underwent 101 endoscopic retrograde cholangiopancreatography (ERCP) procedures with bile duct brushings and cytopathologic examination for the detection of malignant changes between January 2001 and 2004. Bile duct cytology was characterized as unsatisfactory, benign, atypical, or malignant. RESULTS: Fifteen patients had at least one sample that was abnormal. Patients with abnormal findings were older (P = 0.03); otherwise the groups were similar. Three of six patients with marked atypia have undergone transplantation for these abnormal findings, and of these, two had cholangiocarcinoma and one had no cancer detected in the explant. Of the nine patients with focal atypia, seven are doing well, one underwent transplantation for marked atypia seen on brushings at another hospital, and the other underwent transplantation for decompensated cirrhosis. Two patients with benign findings developed cholangiocarcinoma, whereas 29 others have not. CONCLUSION: Cytopathologic examination of bile duct brushings taken at ERCP may be useful for the early detection of malignant changes in patients with primary sclerosing cholangitis. However, long-term prospective studies are needed to confirm the utility of surveillance ERCP and brushings.