To investigate outcomes in endoscopic management of early oesophageal adenocarcinoma in Barrett oesophagus: experience at three Australian tertiary centres.

BACKGROUND: Barrett oesophagus is a known precursor of oesophageal adenocarcinoma (EAC). Early EAC includes T1a (invasion into mucosa) and T1b (invasion into submucosa but not muscularis propria). Endoscopic mucosal resection (EMR) provides accurate histological staging and definitive treatment for early EAC. Post EMR, the remaining Barrett is eradicated with radiofrequency ablation (RFA). However, there is a paucity of long-term Australian data. AIM: To investigate the efficacy and long-term outcomes of EMR and RFA in the management of early EAC. METHODS: Retrospective analysis of patients early EAC treated endoscopically at three Western Australian tertiary centres, with at least 12-months follow up, over the past 10 years. RESULTS: Sixty-seven patients with early EAC (61 T1a and 6 T1b) were treated with EMR. Complete Barrett eradication was done by EMR in 31 of 67 patients whereas 36/67 patients underwent RFA for residual Barrett. EMR changed pinch biopsy histology from HGD (n = 33), HGD suspicious for IMC (n = 5) and LGD (n = 1) to early EAC in 58.2% (n = 39) patients. During a mean follow up of 37.2 months (interquartile range: 20, 56), complete remission of dysplasia and intestinal metaplasia was seen in 97% (n = 65) and 89.5% (n = 60) patients. One patient with T1b EAC underwent oesophagectomy. No cases developed metachronous EAC, progression to invasive adenocarcinoma or development of nodal/distant metastasis. Complications were endoscopically treated haematemesis (n = 1) and strictures (n = 16) requiring dilatations. Three patients died due to causes unrelated to IMC. CONCLUSION: EMR in conjunction with RFA is an effective and safe management for early EAC. EMR provides accurate staging and has low complication rates.

Utility and limitations of Hepascore and transient elastography to detect advanced hepatic fibrosis in HFE hemochromatosis.

Aspartate aminotransferase-to-platelet ratio index (APRI) and Fibrosis-4 Index (Fib4) have been validated against liver biopsy for detecting advanced hepatic fibrosis in HFE hemochromatosis. We determined the diagnostic utility for advanced hepatic fibrosis of Hepascore and transient elastography compared with APRI and Fib4 in 134 newly diagnosed HFE hemochromatosis subjects with serum ferritin levels > 300 µg/L using area under the receiver operator characteristic curve (AUROC) analysis and APRI- (> 0.44) or Fib4- (> 1.1) cut-offs for AHF, or a combination of both. Compared with APRI, Hepascore demonstrated an AUROC for advanced fibrosis of 0.69 (95% CI 0.56-0.83; sensitivity = 69%, specificity = 65%; P = 0.01) at a cut-off of 0.22. Using a combination of APRI and Fib4, the AUROC for Hepascore for advanced fibrosis was 0.70 (95% CI 0.54-0.86, P = 0.02). Hepascore was not diagnostic for detection of advanced fibrosis using the Fib4 cut-off. Elastography was not diagnostic using either APRI or Fib4 cut-offs. Hepascore and elastography detected significantly fewer true positive or true negative cases of advanced fibrosis compared with APRI and Fib4, except in subjects with serum ferritin levels > 1000 µg/L. In comparison with APRI or Fib4, Hepascore or elastography may underdiagnose advanced fibrosis in HFE Hemochromatosis, except in individuals with serum ferritin levels > 1000 µg/L.

Hepatosplenic T cell lymphoma presenting as multiorgan failure.

A 59-year-oldwoman presented with a 2-month history of malaise, abdominal distention and unintentional weight loss. She was initially managed as community acquired pneumonia with a suspicion of underlying chronic liver disease but she deteriorated rapidly into a multiorgan failure necessitating transfer to intensive care unit of a tertiary hospital. She was investigated with liver and bone marrow biopsy that confirmed the diagnosis of hepatosplenic T cell lymphoma. She was treated with cyclophosphamide, doxorubicin, vincristine, etoposide and prednisolone chemotherapy that was changed to salvage ifosfamide carboplatin etoposide (ICE) chemotherapy due to poor response with first-line chemotherapy and disease progression. Unfortunately, her disease progressed further and she opted for palliative management.

A recurrent, ischaemic ileocolonic anastomosis ulcer refractory to surgery treated with hyperbaric oxygen.

A 54-year-old male had undergone right hemicolectomy and ileo-colonic anastomosis for carcinoma-in-situ found at colonoscopy. Eighteen months later, he presented with a lower gastrointestinal bleed from an anastomotic ulcer, treated with resection of the anastomotic site and ileo-sigmoid anastomosis. In the ensuing 12 months, he had three episodes of haematochezia. Colonoscopy revealed a 12 mm anastomotic ulcer necessitating a further colonic resection and re-anastomosis. Two-years later, he presented with iron deficiency anaemia. He preferred expectant management and received ten iron-infusions over the subsequent four years. Thereafter, he developed painless haematochezia. Colonoscopy showed a 15 mm linear ulcer with mild ooze at the anastomosis. Histology was consistent with an ischaemic ulcer; there was no evidence of recurrence of carcinoma-in-situ. The ulcer remained refractory to endoscopic and medical treatment, as seen at three follow-up colonoscopies. Hyperbaric oxygen treatment (HBOT) was offered and he received 30 sessions over six weeks. Colonoscopy at HBOT completion revealed healing of the ulcer. The patient had no further overt bleeding and serum ferritin has continued to rise spontaneously over 12 months follow-up.

Synchronous oesophageal and gastric sarcomatoid carcinoma: first reported case in Australia.

A 75-year-old retired teacher presents with dysphagia and weight loss for a duration of 6 months. Her gastroscopy showed two synchronous submucosal masses. A 7 cm polypoid mass was seen at the distal oesophagus, arising from a thick stalk and a 4 cm mass seen at the cardia. The biopsies showed high-grade sarcomatoid cancer. Staging CT scan and Positron Emission Tomography scan did not show any distant metastasis except a lesion in the rectum that was subsequently found to be tubulovillous adenoma on transanal excision. The patient was managed with Ivor Lewis oesophagectomy. The biopsies of resection specimen showed spindle cell/sarcomatoid carcinoma with a component of poorly differentiated neuroendocrine carcinoma in oesophageal tumour and a small component of conventional invasive squamous cell carcinoma in tumour at cardia. The patient recovered well after surgery. Since then, she has completed adjuvant chemoradiotherapy. No recurrence has been noted in 10 months follow-up.

Transjugular intrahepatic portosystemic shunt: Indications, complications, survival and its use as a bridging therapy to liver transplant in Western Australia.

INTRODUCTION: Insertion of transjugular intrahepatic portosystemic shunt (TIPS) is an established therapeutic option to treat the complications of portal hypertension. The purpose of this study is to review the experience of a single Australian institute with TIPS and evaluation of result to emphasize the indication, aetiology of portal hypertension, prognostic factors, complications and survival. Use of TIPS as a bridge to liver transplantation was also analysed. METHOD: A retrospective cohort study of patients treated with TIPS at The Western Australian Liver Transplant Unit, Sir Charles Gairdner Hospital, over a period of 12 years. Kaplan-Meier method was used for survival analysis and cox-regression analysis was used to analyse the predictors of survival. RESULTS: Fifty-three patients underwent TIPS between January 2000 and March 2012. The cumulative survival at 1 month, 1 year and 5 years was 90%, 70.9% and 43.9%, respectively. The predictors of survival were indication (variceal bleeding versus ascites, hazard ratio 3.19, CI 95%: 1.164-8.794, P = 0.024) and Model of End Stage Liver Disease score (Hazard ratio 2.513, CI 95%: 1.087-5.810, P = 0.031). Patients who underwent TIPS as a bridge to liver transplant had a 5-year survival of 71% that is comparable to the overall survival of Western Australian liver transplant unit. CONCLUSION: Transjugular intrahepatic portosystemic shunt is a safe and effective method of treatment of complications of portal hypertension. TIPS can be safely used as a bridging therapy to liver transplant. Despite small number of TIPS being performed at our institute, our technical results are comparable to the institutes with bigger number of patients.

High local recurrence of early-stage hepatocellular carcinoma after percutaneous thermal ablation in routine clinical practice.

BACKGROUND AND AIM: The risk of local tumour progression (LTP) and factors predicting LTP following percutaneous thermal ablation (PTA) of early-stage hepatocellular carcinoma (HCC) have not been well studied in non-trial settings and may be underestimated. We aimed to assess these outcomes in a multicentre study. PATIENTS AND METHODS: This was a retrospective review of consecutive patients with early-stage HCC treated with a curative intent across three tertiary Australian centres between 2006 and 2012 with either radiofrequency ablation or microwave ablation. The primary endpoint was LTP and multivariate analysis was carried out to identify the independent predictors of LTP. RESULTS: In total 145 HCC nodules were treated in 126 patients (78% men, mean±SD age 62±10 years) with a mean±SD follow-up of 13.5±13 months. Local recurrence was observed in 23.4% (34/145). Mean±SD LTP-free survival was 46.9±3.6 months. For HCC nodules 2 cm or less, local recurrence rates were lower (15.9%), with a mean±SD LTP-free survival of 48.8±4.2 months. Poorly differentiated HCC [hazard ratio (95% confidence interval)=4.8 (1.1-20.4), P=0.032] and pretreatment α-fetoprotein more than 50 kIU/l [8.2 (1.7-39.0), P=0.008] were independent predictors of LTP. LTP rates were not significantly different between the radiofrequency ablation and the microwave ablation groups (22.8 vs. 25.8%, P=0.7). There were six (4.8%) procedure-related adverse events, but no deaths. CONCLUSION: Local recurrence after PTA for early-stage HCC is high in routine clinical practice. Poorly differentiated HCC and pretreatment α-fetoprotein are important, independent predictors of LTP. Further well-designed randomized controlled trials with larger sample sizes using adjuvant therapies in combination with PTA to decrease LTP rates are warranted.

Hereditary Neuropathy with Liability to Pressure Palsy Presenting as an Acute Brachial Plexopathy: A Lover's Palsy.

It is generally regarded that patients with hereditary neuropathy to pressure palsies, due to a deletion in the PMP22 gene, show recurrent pressure palsy and generalised peripheral neuropathy (pes cavus and hammer toes sometimes develop). Brachial plexopathy is rarely identified as a first presentation of hereditary neuropathy to pressure palsies. We describe a young man who developed a painless flail upper limb with a clinical diagnosis of a brachial plexopathy after his partner slept on his arm - a PMP22 deletion was found. His father, who had a symmetrical polyneuropathy without recurrent mononeuropathies, shared the PMP22 deletion.

Liver abnormalities in drug and substance abusers.

Drug and substance abuse remains a major medical problem. Alcohol use, abuse and dependence are highly prevalent conditions. Alcohol related liver disease can present as simple steatosis, steatohepatitis, alcoholic hepatitis or liver cirrhosis. Paracetamol hepatotoxicity secondary to accidental or deliberate overdose is another common problem. While the adverse cardiovascular, neurological, renal and psychiatric consequences of various illicit substance abuses are widely studied and publicized, less attention has been directed towards possible hepatotoxic effects. Illicit drug abuse can cause a range of liver abnormalities ranging from asymptomatic derangement of liver function tests to fulminant hepatic failure. This article reviews the epidemiology, risk factors, clinical manifestations, pathogenesis, investigations, management and prognostic factors of alcohol related liver disease and paracetamol hepatotoxicity as well as the current knowledge pertaining to hepatotoxicity of the more commonly used illicit substances including cannabis, amphetamine type stimulants, cocaine, khat chewing and complementary and alternate medicine.