Blue rubber bleb nevus syndrome in a patient on anticoagulation: a management dilemma.

Blue rubber bleb nevus syndrome (BRBNS) is a rare condition characterised by vascular malformations mostly of the skin and gastrointestinal tract and less commonly of the central nervous system, liver, thyroid, spleen and lungs. We report a rare case of BRBNS in a patient on anticoagulation who presented with gastrointestinal bleeding and no cutaneous or other organ involvement. We discuss the difficulty in balancing bleeding and clotting risks in this patient who developed two episodes of venous thromboembolism while off anticoagulation to minimise gastrointestinal bleeding. We also highlight the potential role of somatostatin analogues such as lanreotide in decreasing gastrointestinal bleeding risk in BRBNS, particularly in the setting of anticoagulation. The occurrence of two episodes of venous thromboembolism within a short time frame in this case, in conjunction with known associations between other vascular anomalies and venous thromboembolism, raises the question of whether BRBNS may be associated with a prothrombotic state.

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.

Clinical and pathological predictors of failure of endoscopic therapy for Barrett's related high-grade dysplasia and early esophageal adenocarcinoma.

BACKGROUND AND AIMS: Multimodal endoscopic treatment for Barrett's esophagus (BE) related high-grade dysplasia (HGD) and early esophageal adenocarcinoma (EAC) is safe and effective. However, there is a paucity of data to predict the response to endoscopic treatment. This study aimed to identify predictors of failure to achieve complete eradication of neoplasia (CE-N) and complete eradication of intestinal metaplasia (CE-IM). METHODS: We performed a retrospective analysis of prospectively collected data of all HGD/EAC cases treated endoscopically at a tertiary referral center. Only patients with confirmed HGD/EAC from initial endoscopic mucosal resection (EMR) were included. Potential predictive variables including clinical characteristics, endoscopic features, and index histologic parameters of the EMR specimens were evaluated using multivariate Cox regression. RESULTS: A total of 457 patients were diagnosed with HGD/EAC by initial EMR from January 2008 to January 2019. Of these, 366 patients who underwent subsequent endoscopic treatment with or without RFA were included. Cumulative incidence rates at 3 years for CE-N and CE-IM were 91.4% (95% CI 87.8-94.2%) and 66.8% (95% CI 61.2-72.3%), respectively during a median follow-up period of 35 months. BE segment of 3-10 cm (HR 0.45; 95% CI 0.36-0.57) and > 10 cm (HR 0.25; 95% CI 0.15-0.40) were independent clinical predictors associated with failure to achieve CE-N. With respect to CE-IM, increasing age (HR 0.88; 95% CI 0.78-1.00) was another predictor along with BE segment of 3-10 cm (HR 0.37; 95% CI 0.28-0.49) and > 10 cm (HR 0.15; 95% CI 0.07-0.30). Lymphovascular invasion increased the risk of CE-N and CE-IM failure in EAC cases. CONCLUSION: Failure to achieve CE-N and CE-IM is associated with long-segment BE and other clinical variables. Patients with these predictors should be considered for a more intensive endoscopic treatment approach at expert centers.

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.

Common Symptoms from an Uncommon Infection: Gastrointestinal Anisakiasis.

Clinicians can be forgiven for thinking of anisakiasis as a rare condition low in the differential diagnosis of abdominal pain. Gastrointestinal anisakiasis is a zoonotic parasitic disease caused by consumption of raw or undercooked seafood infected with nematodes of the genus Anisakis. Even though the reported cases indicate that this is a rare disease, the true incidence of the disease could be potentially higher than what is reported in the literature as cases can go undiagnosed. Diagnosis and treatment of gastric anisakiasis are made by a compatible dietary history, direct visualization, and removal of the larvae via gastroscopy. Serologic testing and imaging studies are useful in the diagnosis of intestinal anisakiasis and conservative management should be considered. This disease may mimic other diseases and lead to unnecessary surgery. This emphasizes the importance of suspecting gastrointestinal anisakiasis by history taking and by other diagnostic modalities.

Successful treatment of patients with hepatitis C in rural and remote Western Australia via telehealth.

Rural and remote patients at the Royal Perth Hospital were reviewed and treated for hepatitis C by a hepatologist and nurse practitioner using telehealth (videoconferencing). Over a four-year period, 50 patients were treated with pegylated interferon and ribavirin, and participated in a total of more than 500 telehealth sessions. Sustained virological response rates (SVRs) were compared to those in face-to-face (FTF) clinics to assess treatment outcomes. Treatment through telehealth was found to be non-inferior to FTF clinics. Telehealth patients with genotype 1 infection achieved a higher rate of SVR than those attending FTF clinics (73% versus 54%, respectively), although the difference was not significant. SVR rates for genotype 2 and 3 of 72% were similar in telehealth to FTF rates of 74%. A total of 35 telehealth patients completed a satisfaction questionnaire and most indicated that they were happy with the programme and would participate again in the future. The study confirmed that telehealth is an effective option for the treatment of hepatitis C in rural and remote areas.