Mohammad Salih

Reversal of acquired hepatocerebral degeneration with living donor liver transplantation

Many patients with cirrhosis eventually develop complications of end-stage liver disease (ESLD). Deposition of toxins, eg, ammonia in the brain, results in hepatic encephalopathy (HE). In a subset of patients, there is a chronic form of non-Wilsonian encephalopathy called acquired hepatocerebral degeneration (AHD). The pathogenesis of AHD is thought to be due to an accumulation of manganese (Mn) in the brain, which can be seen as hyperintensities of globus pallidus (GP), lenticular nucleus, and substantia nigra on T1-weighted sequences of brain magnetic resonance imaging (MRI) scans. Clinically, AHD is characterized by extrapyramidal and neuropsychiatric symptoms, including delirium, apathy, lethargy, somnolence, emotional instability, and movement disorders, such as tremor, parkinsonism, akinesia, choreoathetosis, myoclonus, ataxia, asterixis, dystonia, pyramidal signs, and myelopathy. No medical treatment has been reported to reverse the progression of AHD. Before the advent of liver transplant, HE was considered reversible with the only exception of the AHD cases, which were labeled refractory to optimal available treatment. Recent studies suggest that liver transplant may be an effective therapy for AHD. In Pakistan, only 1 case report has been published on AHD. Shifa International Hospital, being the only liver transplantation center in the country, offers a needed platform to test this hypothesis. Herein, we describe findings of a retrospective study done to determine the radiological and clinical reversibility of AHD in chronic liver disease (CLD) patients treated with living donor liver transplantation (LDLT) or optimal medical treatment. The study was approved by the hospital ethics committee and funded by the Neurology Department Education and Research Fund. We reviewed a database of 531 patients diagnosed with CLD in the inpatient records of the gastroenterology and hepatology department of Shifa International Hospital. Data were extracted via an electronic timeline search from January 1, 2013 to December 31, 2013 using keywords “CLD,” “chronic liver disease,” “endstage liver disease,” and/or “cirrhosis.” A similar search was run in the radiology department of the hospital to screen patients who underwent a brain MRI. Sixty-three out of the 531 patients underwent MRI brain scans. We searched for patients with confirmed AHD in their diagnosis. Patients were filtered and selected on the basis of our set of inclusion and exclusion criteria: inclusion criteria—patients with CLD and AHD who underwent brain MRI from January 1, 2013 to December 31, 2013; exclusion criteria—patients with CLD but with other central nervous system findings on MRIs, ie, cerebrovascular accident (CVA), infectious diseases, space-occupying lesions (SOLs), or Wilson’s disease. Decompensated liver disease was documented by reviewing results from patients’ medical records. The severity of the liver disease was assessed by ChildTurcotte-Pugh (CTP) and Model for End-Stage Liver Disease (MELD) scores. Pretransplant and posttransplant signs and symptoms were separately listed. The motor impairment severity (MIS) index was used to assess the presence and severity (0, absent; 1, mild; 2, moderate; 3, severe; 4, extremely severe) of the following motor signs: bradykinesia, tremor, dystonia, dyskinesia, dysarthria, and ataxia. The graded

Reply to 16‐076

We thank Drs. Salehi and Shawcross for their comments on our publication “Reversal of acquired hepatocerebral degeneration with living donor liver transplantation.” Our study includes a review of retrospective data of patients with acquired hepatocerebral degeneration (AHD) who were seen in 2013. Our case series gives us an insight that patients with AHD who are otherwise transplant candidates may have radiological and clinical reversal after liver transplant. Drs. Salehi and Shawcross quote a series published as correspondence in Hepatology. We would like to point out that our patients who underwent living donor liver transplantation were primarily liver transplant candidates and that neurological evaluation for Parkinsonism/movement disorders was performed during pretransplant workup and AHD was not a primary indication for liver transplant. Patients who are admitted with hepatic encephalopathy are routinely given rifaximin in our department because it became the standard of care, and our data have also been published. However, we believe that larger blinded studies over a longer period of time are needed before establishing that rifaximin used without liver transplantation may provide long-lasting benefits in AHD due to chronic liver disease. Ahmed Hassaan Qavi, M.B.B.S.

Prognostication of Learning Curve on Surgical Management of Vasculobiliary Injuries after Cholecystectomy

Background. Concomitant vascular injury might adversely impact outcomes after iatrogenic bile duct injury (IBDI). Whether a new HPB center should embark upon repair of complex biliary injuries with associated vascular injuries during learning curve is unknown. The objective of this study was to determine outcome of surgical management of IBDI with and without vascular injuries in a new HPB center during its learning curve. Methods. We retrospectively reviewed patients who underwent surgical management of IBDI at our center. A total of 39 patients were included. Patients without (Group 1) and with vascular injuries (Group 2) were compared. Outcome was defined as 90-day morbidity and mortality. Results. Median age was 39 (20–80) years. There were 10 (25.6%) vascular injuries. E2 injuries were associated significantly with high frequency of vascular injuries (66% versus 15.1%) (P = 0.01). Right hepatectomy was performed in three patients. Out of these, two had a right hepatic duct stricture and one patient had combined right arterial and portal venous injury. The number of patients who developed postoperative complications was not significantly different between the two groups (11.1% versus 23.4%) (P = 0.6). Conclusion. Learning curve is not a negative prognostic variable in the surgical management of iatrogenic vasculobiliary injuries after cholecystectomy.

Assigning Treatment to HCC Patients for Transplantation: Utility of a New Decision-Making Tool

BACKGROUND The Barcelona clinic liver cancer (BCLC) staging system is considered the standard of care for hepatocellular carcinoma (HCC) management. It has various limitations, including lack of second-line treatment options and combination therapy. We prospectively collected data on our HCC patients based on a new decision-making tool (NDT). The objective of this study was to determine the applicability of this tool and compare it with BCLC for treatment allocation, in particular with respect to liver transplantation. MATERIAL AND METHODS We retrospectively reviewed HCC patients who were managed based on an NDT that was developed in 2012. All patients whose treatment decision was based on this tool between 2012 and 2015 were included. Comparison was made with BCLC. Survival was compared for patients who underwent liver transplantation. RESULTS Based on the NDT, 406 (40.6%) patients were eligible for curative treatment versus only 22 (2.2%) patients based on BCLC. A total of 58 (5.8%) patients underwent liver transplant based on the NDT, while only 2 (0.2%) were transplantable based on BCLC. Estimated 3-year survival for transplanted patients based on the NDT was 73%. There were 41 (4.1%) stage C and 15 (1.5%) stage D BCLC patients who received transplant based on the NDT. Estimated 3-year survival for stage A, C, and D BCLC patients who received transplantation was 100%,72%, and 67%, respectively (P=0.6). CONCLUSIONS The NDT correctly identified a group of HCC patients for liver transplantation who would otherwise have received palliative treatment based on the BCLC algorithm.