Budd-Chiari syndrome associated with congenital afibrinogenaemia reversed after orthotopic liver transplant.

A woman in her mid-20s, a known case of congenital afibrinogenaemia, presented with abdominal pain and distension. She was diagnosed with decompensated liver cirrhosis due to Budd-Chiari syndrome. She underwent deceased donor liver transplantation. Preoperatively, her serum fibrinogen level was undetectable and prothrombin time and international normalised ratio (INR) were unrecordable. Intraoperatively, she was given thromboelastography-guided human fibrinogen concentrate. Postoperatively, her fibrinogen, prothrombin time and INR normalised rapidly. This report summarises the rare occurrence of a complication of hypercoagulability (Budd-Chiari syndrome) in the setting of congenital hypocoagulability (congenital afibrinogenaemia). In this report, we discuss the simultaneous management of these two clinical problems and the curative role of liver transplantation.

Portal biliopathy in patients with non-cirrhotic portal hypertension: does the type of surgery affect outcome?

OBJECTIVES: After portosystemic anastomoses for biliopathy, some patients continue to suffer biliary obstruction. The effects of splenectomy and devascularization of the abdominal oesophagus and upper stomach are unclear. The aim of the current study was to determine the features of portal biliopathy (PB) in patients with non-cirrhotic portal hypertension, and to investigate outcomes in these patients after surgical procedures. METHODS: A retrospective study of 56 patients who underwent surgery for PB during 1996-2010 was conducted. Data on presenting features, treatment received and outcomes were analysed. RESULTS: In total, 41 of these patients had extrahepatic portal venous obstruction and 15 had non-cirrhotic portal fibrosis. Forty patients underwent shunt surgery and 16 underwent splenectomy and devascularization. Median bilirubin levels fell from 1.8 mg/dl (range: 0.4-5.9 mg/dl) to 1.0 mg/dl (range: 0.3-5.4 mg/dl) after shunt surgery and from 1.9 mg/dl (range: 0.6-4.0 mg/dl) to 1.2 mg/dl (range: 0.6-5.2 mg/dl) after splenectomy-devascularization. On follow-up, five of 33 patients had persistent jaundice after successful shunt surgery. These patients had a history of multiple endoscopic stentings and three patients had demonstrated a dominant common bile duct stricture preoperatively. CONCLUSIONS: Portal biliopathy was reversed in 38 of 43 patients by either portosystemic shunting or splenectomy-devascularization. In five patients, direct biliary decompressive procedures were required because of shunt blockage or a non-reversible biliary stricture.

Acute liver failure secondary to yellow phosphorus rodenticide poisoning: Outcomes at a center with dedicated liver intensive care and transplant unit.

Accidental or suicidal poisoning with yellow phosphorus or metal phosphides (YPMP) such as aluminum (AlP) zinc phosphide (Zn3P2) commonly cause acute liver failure (ALF) and cardiotoxicity. These are used as household, agricultural and industrial rodenticides and in production of ammunitions, firecrackers and fertilizers. In absence of a clinically available laboratory test for diagnosis or toxin measurement or an antidote, managing their poisoning is challenging even at a tertiary care center with a dedicated liver intensive care unit (LICU) and liver transplant facility. PATIENTS AND METHODS: Patients with YPMP related ALF were monitored using standardized clinical, hemodynamic, biochemical, metabolic, neurological, electrocardiography (ECG) and SOFA score and managed using uniform intensive care, treatment and transplant protocols in LICU. Socio-demographic characteristics, clinical and biochemical parameters and scores were summarized and compared between 3 groups i.e. spontaneous survivors, transplanted patients and non-survivors. Predictors of spontaneous survival and the need for liver transplant are also evaluated. RESULTS: Nineteen patients with YPMP related ALF were about 32 years old (63.2% females) and presented to us at a median of 3 (0 - 10) days after poisoning. YPMP related cardiotoxicity was rapidly progressive and fatal whereas liver transplant was therapeutic for ALF. Spontaneous survivors had lower dose ingestion (<17.5 grams), absence of cardiotoxicity, < grade 3 HE, lactate < 5.8, SOFA score < 14.5, and increase in SOFA score by < 5.5. Patients with renal failure need for CVVHDF and KCC positivity on account of PT-INR > 6.5 had higher mortality risk. Patients undergoing liver transplant and with spontaneous recovery required longer ICU and hospital stay. At median follow-up of 3.4 (2.6 - 5.5) years, all spontaneous survivors and transplanted patients are well with normal liver function. CONCLUSIONS: Early transfer to a specialized center, pre-emptive close monitoring, and intensive care and organ support with ventilation, CVVHDF, plasmapheresis and others may maximize their chances of spontaneous recovery, allow accurate prognostication and a timely liver transplant.

Estimation of liver volume in the western Indian population.

BACKGROUND: A number of formulae to estimate standard liver volume (SLV) exist. However, studies have shown that only certain formulae are applicable to a particular patient population, whereas the other formulae have not been accurate in estimating the SLV. Aim of this study was to assess which formula is most accurate in estimating SLV in the western Indian population. METHODS: Data for donors of living donor liver transplantation from September 2014 to July 2015 was analyzed. Liver volumes were measured using computed tomography volumetry (CTV). SLV was calculated using formulae by the currently existing formulae. The mean SLV and CTV, percentage error in the SLV, and the correlation between SLV and CTV were calculated. RESULTS: Fifty-nine healthy subjects underwent donor hepatectomy [28 (47.5 %) males]. The mean age, mean body mass index (BMI), and mean body surface area (BSA) were 31.8 ± 8.8 years, 23.8 ± 3.7 kg/m(2), and 1.6 ± 0.4, respectively. Mean CTV was 1178 ± 246.8 mL. Difference between mean SLV and mean CTV ranged from -133.5 (±189) mL to 632.2 (±190.2) mL. Mean SLV was significantly different from CTV by all the formulae except Urata. Percentage of population whose SLV was within 15 % of the mean CTV ranged from 1.7 % to 67.8 %, with the highest percentage obtained by using Fu-Gui's formula. However, there was wide inter-individual variation on scatter plots between SLV and CTV by both these formulae. CONCLUSION: Currently existing formulae were not accurate in estimating SLV in our population.

Surgical management of portal cavernoma cholangiopathy.

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.

Portal biliopathy.

Biliary ductal changes are a common radiological finding in patients with portal hypertension, however only a small percentage of patients (5%-30%) develop symptomatic bile duct obstruction. The exact pathogenesis is not clear, but an involvement of factors such as bile duct compression by venous collaterals, ischemia, and infection is accepted by most authors. Although endoscopic retrograde cholangiopancreatography was used to define and diagnose this condition, magnetic resonance cholangiopancreatography is currently the investigation of choice for diagnosing this condition. Treatment is indicated only for symptomatic cases. Portosystemic shunts are the treatment of choice for symptomatic portal biliopathy. In the majority of patients, the changes caused by biliopathy resolve after shunt surgery, however, 15%-20% patients require a subsequent bilio-enteric bypass or endoscopic management for persistent biliopathy. There is a role for endoscopic therapy in patients with bile duct stones, cholangitis or when portosystemic shunt surgery is not feasible.