Long Term Outcomes of Patients with Significant Biliary Obstruction Due to Portal Cavernoma Cholangiopathy and Extra-Hepatic Portal Vein Obstruction (EHPVO) With No Shuntable Veins.

The natural history of portal cavernoma cholangiopathy (PCC) in patients with significant biliary obstruction (SBO) who cannot undergo shunt surgery, is not known. We therefore, analyzed data of patients of extra-hepatic portal venous obstruction (EHPVO) with PCC.Prospectively recorded details of 620 (age 21.2 [11.4] years; 400 [65%] males) patients with primary EHPVO were reviewed. Outcomes (hepatic decompensation/mortality) of patients with PCC and SBO without shuntable veins were noted at follow up of 7 [4-11] years.Ninety-seven of 620 (15.6% [60 men]) EHPVO patients had PCC-SBO. Of these 57 did not have shuntable veins. The median duration from any index symptom to symptomatic PCC was 7 (0-24) years and from index bleed to symptomatic PCC was and 12 (5-24) years, respectively. Thirteen patients underwent endoscopic retrograde cholangiography; nine repeatedly over 7 (4-10) years. Decompensation was seen in 5 patients. Presentation other than variceal bleed was associated with hepatic decompensation (5/19 versus 0/38, P = 0.003).Majority of patients with PCC-SBO do not have shuntable veins, and may have good long-term outcomes. Patients presenting with variceal bleed have low chance of decompensation. Symptomatic PCC appears to be a late event in EHPVO.