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P86 Taking the pressure off complex portal hypertension

gutjnl · 2025-10-06 · canonical JSON source

15 visible annotations · policy: published · automated confidence ≥ 75.00%

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Introduction The majority of portal hypertension (PHTN) cases are related to underlying cirrhosis. However, there are rare causes of PHTN which require specific management. Increasing awareness of these cases and referral pathways to high volume centres may help reduce delays in management.Method Outcomes of three patients referred for PHTN related complications were gathered from patient medical records at a single tertiary liver unit.Results Case 1: A 65 year old (yo) woman was referred for further management following an upper oesophageal variceal bleed. She had a previous history of thoracotomy without surgical intervention aged 18 for an oesophageal bleed and has stable sarcoidosis. Imaging did not show other features of PHTN but low flow vascular malformation around the mid thoracic oesophagus feeding into the downhill varices ( figure 1). There were no features of superior vena cava obstruction. After review in the Vascular multidisciplinary meeting (MDM), she was initiated on sirolimus with good response at 2 months follow up. Case 2: A 67 yo woman with cirrhosis and PHTN was referred following a life threatening oesophageal bleed. Endoscopy identified upper oesophageal (downhill) varices. She had a historic lumbar and cervical sympathectomy aged 19 years for Raynaud’s. Endoscopic band ligation led to further bleeding and she was requiring regular blood transfusions. Imaging showed innumerable vessels surrounding the upper third of the oesophagus in keeping with high flow oesophageal arteriovenous malformations (AVMs) as the cause. She underwent 2 embolosclerotherapy at 6 months interval followed by Thalidomide after Vascular MDM. She achieved stability and reduction in blood product support for 1 year with Thalidomide before requiring further embolosclerotherapy. Case 3: A 36 yo man was referred for refractory oesophageal variceal bleed. Index presentation was 6 months prior with a variceal bleed. He had a previous history intravenous drug use and no longer had risk taking behaviour related to the liver. Non-invasive liver screen was negative, and ultrasound was in keeping with PHTN (splenomegaly and ascites). CT scan showed multiple intrapancreatic AVM with rapid shunting from splenic artery into the portal system. He underwent 2 successive embolization with good temporisation. Subsequent imaging showed residual shunts. Liver biopsy did not show cirrhosis. He had a semi-elective distal pancreatectomy and splenectomy as definitive management. Abstract P86 Figure 1Conclusion Uncommon causes of PHTN require diagnostic vigilance and an MDM approach to individualised management. Sharing outcomes of these cases highlights the importance of having a robust referral pathway to vascular hubs.