Diagnosis and Treatment of Biliary Obstruction Caused by Portal Cavernoma

Abstract

While hemorrhagic complications of portal cavernoma are frequent, compression of the bile ducts by portal cavernoma is uncommon and treatment is still a matter for debate. We report here six new cases in order to describe: (a) the clinical, biological, and morphological features of this condition, and (b) the long-term results of a combined endoscopic and surgical treatment. The median age of patients at the time of diagnosis was 36.5 years. The circumstances of diagnosis were acute cholangitis (n=3), asymptomatic biological cholestasis (n=1), pruritus, jaundice and asthenia (n=1) and jaundice alone (n=1). Portal cavernoma and bile duct dilatation were confirmed by abdominal ultrasonography with pulsed color doppler and endoscopic retrograde cholangiography (ERC). Gallstones were found in four patients. Following stenting of the bile duct, there was a good outcome in two patients. In four patients, after failure of prolonged endoscopic treatment, second-line surgical portal-systemic shunting allowed removal of the biliary stent, and no recurrence of disease. In conclusion, biliary involvement in portal cavernoma is now a well-recognized entity, and our results suggest that combined endoscopic and surgical treatment could be required.

Endoscopic Management of Portal Cavernoma Cholangiopathy: Practice, Principles and Strategy

“…Cholangitis was reported in 5 of 10 patients managed with repeated stent exchanges in France, 12 in 4 of 6 patients managed by Dumortier et al 13 and in 3 of 13 patients (3 of 49 procedures; 6%) reported by Oo et al 10 In the series of 13 patients with symptomatic PCC reported by Khare et al, 11 none of the 10 patients who underwent endoscopic management developed cholangitis. However, recent experience from the same center noted that cholangitis was present at the time of 40 of 130 procedures (30%) in 20 patients managed with repeated stent exchanges over a period of 16 years and this was attributed to inability of patients to report for timely stent exchanges.…”

“…The first report of endoscopic extraction of common bile duct stones in PCC 8 was soon followed by other small series 5,[9][10][11][12][13] reporting success without an increase in complications, though some workers reported hemobilia during the procedure. [14][15][16][17] In many centers, endoscopic management has remained confined to establishing drainage of the obstructed biliary system before surgery, in the expectation that either the obstruction would resolve after a period of endoscopic drainage or that portosystemic shunt surgery (PSS), with or without second-stage biliary surgery, would provide definitive management.…”

“…Eleven series published between 1992 and 2011 9,10,12,13,[18][19][20][21][22][23] have reported a mean frequency of 26.3% (16-85%) for biliary calculi among a total of 331 patients, including 143 with symptomatic PCC. Prevalence of both, gallbladder (mean 13.6%, range 0-69%) as well as biliary ductal calculi (mean 17.8%, range 0-77%), is increased and prevalence is much higher in symptomatic PCC, being 60.8% overall in 143 symptomatic patients (35.1% for gallstones, 41.2% for choledocholithiasis).…”

“…Since the first report of biliary stenting for choledochal stenosis in 1993, 6 endoscopic management of symptomatic PCC has been reported in at least 87 patients in 17 case series, 6,[8][9][10][11][12][13]22,[24][25][26][27][28][29][30][31][32] nine of which involved fewer than 4 patients and the largest included 20 patients (Table 2). Results of endoscopic management for biliary stenoses are summarized below.…”

“…[8][9][10][11][12][13] None of the series have reported excessive bleeding during ES, suggesting that it is safe in patients with PCC with no more risk than in patients without PCC. Bhatia et al 8 reported uneventful ES and stone extraction in 3 patients with symptomatic PCC and choledocholithiasis.…”