Portal cavernoma cholangiopathy (PCC) is defined as abnormalities in the extrahepatic biliary system including the cystic duct and gallbladder with or without abnormalities in the 1st and 2nd generation biliary ducts in a patient with portal cavernoma. Presence of a portal cavernoma, typical cholangiographic changes on endoscopic or magnetic resonance cholangiography and the absence of other causes of these biliary changes like bile duct injury, primary sclerosing cholangitis, cholangiocarcinoma etc are mandatory to arrive a diagnosis. Compression by porto-portal collateral veins involving the paracholedochal and epicholedochal venous plexuses and cholecystic veins and ischemic insult due to deficient portal blood supply or prolonged compression by collaterals bring about biliary changes. While the former are reversible after porto-systemic shunt surgery, the latter are not. Majority of the patients with PCC are asymptomatic and approximately 21% are symptomatic. Symptoms in PCC could be in the form of long standing jaundice due to chronic cholestasis, or biliary pain with or without cholangitis due to biliary stones. Endoscopic retrograde cholangiography has no diagnostic role because it is invasive and is associated with risk of complications, hence it is reserved for therapeutic procedures. Magnetic resonance cholangiography and portovenography is a noninvasive and comprehensive imaging technique, and is the modality of choice for mapping of the biliary and vascular abnormalities in these patients. PCC is a progressive condition and symptoms develop late in the course of portal hypertension only in patients with severe or advanced changes of cholangiopathy. Asymptomatic patients with PCC do not require any treatment. Treatment of symptomatic PCC can be approached in a phased manner, coping first with biliary clearance by nasobiliary or biliary stent placement for acute cholangitis and endoscopic biliary sphincterotomy for biliary stone removal; second, with portal decompression by creating portosystemic shunt; and third, with persistent biliary obstruction by performing second-stage biliary drainage surgery such as hepaticojejunostomy or choledochoduodenostomy. Patients with symptomatic PCC have good prognosis after successful endoscopic biliary drainage and after successful shunt surgery.

https://www.inasl.org.in/images/portal-%20pavernoma%20-pholangiopathy.pdf

Portal Cavernoma Cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver

The majority of patients with portal cavernoma cholangiopathy (PCC) are asymptomatic, however some (5–38%) present with obstructive jaundice, cholangitis, or even biliary pain due to bile duct stones which form as a result of stasis. Most patients with extrahepatic portal venous obstruction (EHPVO) present with variceal bleeding and hypersplenism and these are the usual indications for surgery. Those who present with PCC may also need decompression of their portosystemic system to reverse the biliary obstruction. It is important to realize that though endoscopic drainage has been proposed as a non-surgical approach to the management of PCC it is successful in only certain specific situations like those with bile duct calculi, cholangitis, etc. A small proportion of such patients will continue to have biliary obstruction and these patients are thought to have a mechanical ischemic stricture. These patients will require a second stage procedure in the form of a bilioenteric bypass to reverse the symptoms related to PCC. In the absence of a shuntable vein splenectomy and devascularization may resolve the PCC in a subset of patients by decreasing the portal pressure.

Surgical Management of Portal Cavernoma Cholangiopathy

Portal cavernoma cholangiopathy (PCC) is a difficult clinical problem, where the portal cavernoma is both the cause of biliary obstruction and the obstacle to its safe surgical treatment. The available endoscopic and surgical treatment is successful in majority and further intervention is seldom required since the native liver is normal. PCC is not an accepted indication for liver transplantation as only a small proportion of patients will fail both endoscopic and surgical treatment and progressive liver failure is rarely seen. Secondary biliary cirrhosis as a result of long standing biliary obstruction is an accepted indication however establishing a portal inflow in these patients is often difficult and challenging. The deceased donor liver transplantation would always be preferable over living donor liver transplantation as PCC is usually a non-emergency transplant and the graft can have portal blood inflow through a conduit to even a small segment of patent portal venous system or even to a cavernoma vessel.

Liver Transplantation for Portal Cavernoma Cholangiopathy

High-quality evidence is lacking to support one treatment strategy over another in patients with foot drop due to peroneal nerve entrapment. This leads to strong variation in daily practice.The FOOTDROP (Follow-up and Outcome of Operative Treatment with Decompressive Release Of The Peroneal nerve) trial is a randomized, multi-centre study in which patients with peroneal nerve entrapment and persistent foot drop, despite initial conservative treatment, will be randomized 10 (± 4) weeks after onset between non-invasive treatment and surgical decompression. The primary endpoint is the difference in distance covered during the 6-min walk test between randomization and 9 months later. Time to recovery is the key secondary endpoint. Other secondary outcome measures encompass ankle dorsiflexion strength (MRC score and isometric dynamometry), gait assessment (10-m walk test, functional ambulation categories, Stanmore questionnaire), patient-reported outcome measures (EQ5D-5L), surgical complications, neurological deficits (sensory changes, motor scores for ankle eversion and hallux extension), health economic assessment (WPAI) and electrodiagnostic assessment.The results of this randomized trial may elucidate the role of surgical decompression of the peroneal nerve and aid in clinical decision-making.ClinicalTrials.gov NCT04695834. Registered on 4 January 2021. 

/pdf/conservative-versus-surgical-treatment-of-foot-drop-in-3uw5v7b4.pdf

Conservative versus surgical treatment of foot drop in peroneal nerve entrapment: rationale and design of a prospective, multi-centre, randomized parallel-group controlled trial

The term portal biliopathy (PB) is used to describe the biliary abnormalities associated with portal hypertension. Between 5% and 30% of patients with PB develop biliary obstruction. We report on a patient with extrahepatic biliary obstruction caused by PB that was successfully managed with an intrahepatic segment 3 bypass. The traditional surgical approach for a patient with extrahepatic biliary obstruction caused by PB would be a portosystemic shunt followed by a hepaticojejenostomy if the jaundice persited. An intrahepatic segment 3 bypass provides definitive treatment ensuring biliary decompression and stone removal in a single procedure in appropriately selected patients.

/pdf/single-stage-definitive-surgical-treatment-for-portal-br544rprnq.pdf

Single-stage definitive surgical treatment for portal biliopathy

INTRODUCTION Perioperative peroneal neuropathy is an uncommon complication following operations remote from the leg or in supine position including liver transplant. MATERIALS AND METHODS We retrospectively reviewed the medical records of 132 living-donor liver transplant recipients done at our center between September 2006 and December 2008. Various potential preoperative, intraoperative, and postoperative factors were studied in the cases that developed perioperative peroneal neuropathy. RESULTS Peroneal neuropathy was reported in 7 recipients (5.3%) following liver transplant. Apart from intraoperative positioning, other identifiable predisposing factors appear to be poor nutritional status, tall and slender body shape, alcoholic liver disease, and higher pretransplant model for endstage liver disease score. All patients were treated conservatively, including nutritionally balanced diet and vitamin supplements combined with physical rehabilitation therapy. The motor power returned to normal within 6 months in all 7 patients. CONCLUSIONS Perioperative peroneal neuropathy may be contributed by various preoperative factors apart from intraoperative nerve compression. It can be effectively prevented by being aware of the predisposing factors and implicating adequate precautions perioperatively.

Peroneal neuropathy following liver transplantation: possible predisposing factors and outcome.

Portal biliopathy refers to abnormalities of the entire biliary tract in patients with extrahepatic portal vein obstruction Most of the patients are asymptomatic However, more than 80% of patients show characteristic morphologic features on endoscopic retrograde cholangiopancreatography Symptomatic cases usually require therapy The proposed therapies include endoscopic dilatation and stenting of the common bile duct, portosystemic shunt with splenectomy, meso Rex bypass, and occasionally, bilioenteric bypass In patients with failed stenting and nonshuntable anatomy, liver transplant may be the only option, provided an adequate inflow can be achieved to the graft To the best of our knowledge, only 2 cases have been reported, and these have undergone deceased-donor liver transplant for portal biliopathy We report an index case treated successfully with living-donor liver transplant

Portal biliopathy treated with living-donor liver transplant: index case.

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Peroneal neuropathy following liver transplantation: possible predisposing factors and outcome.

Portal biliopathy treated with living-donor liver transplant: index case.