Recurrent Variceal Hemorrhage in Non-Cirrhotic Portal Hypertension with Thymoma-Related Myasthenia Gravis : A Case Report
Keywords:
Extrahepatic Portal Vein Obstruction, Esophageal Varices, Myasthenia Gravis, Thymoma, Plasmapheresis, Portal Hypertension, Case ReportAbstract
Background: Extrahepatic portal vein obstruction (EHPVO) in adults is uncommon and usually is accompanied by portal hypertension but preserved liver function. Its association with thymoma-associated myasthenia gravis (MG) presents with distinct therapeutic challenges.
Case Presentation: We present a 33-year-old man with EHPVO and high-risk esophageal varices that needed to be treated with repeated endoscopic variceal ligation (EVL). He also had invasive thymoma complicated by MG with high titers of acetylcholine receptor antibodies. The management was done via a multidisciplinary team including hepatology, neurology, and surgery. Beta-blockers and plasmapheresis stabilized the patient before thymectomy.
Conclusion: The simultaneous management of risk of variceal bleeding and MG-associated thymoma underscores the importance of individualized, multidisciplinary care in multisystem disorders.
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Garcia-Tsao G, Abraldes JG, Berzigotti A, Bosch J. Portal hypertensive bleeding in cirrhosis: Risk stratification, diagnosis, and management: 2022 Practice Guidance by the AASLD. Hepatology. 2022;75(1):154–174.
Sanders DB, Wolfe GI, Benatar M, et al. International consensus guidance for management of myasthenia gravis: 2021 update. Neurology. 2021;96(3):114–122.
Gilhus NE. Myasthenia gravis. N Engl J Med. 2021;385(6):573–584.
Dhiman RK, Chawla YK, Vasishta RK, et al. Non-cirrhotic portal fibrosis and extrahepatic portal venous obstruction. Trop Gastroenterol. 2020;41(1):1–10.
Sharma M, Rameshbabu CS. Collateral pathways in portal hypertension. J Clin Exp Hepatol. 2019;9(4):469–477.
Garcia-Pagán JC, Caca K, Bureau C, et al. Early use of TIPS in patients with cirrhosis and variceal bleeding. N Engl J Med. 2019;362(25):2370–2379.
Wolfe GI, Kaminski HJ, Aban IB, et al. Randomized trial of thymectomy in myasthenia gravis. N Engl J Med. 2016;375(6):511–522.
de Franchis R. Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop. J Hepatol. 2015;63(3):743–752.
Weiler T, Ahmed SS, Smith AG, et al. Autoimmune MG and anesthesia: Perioperative care. Can J Anaesth. 2015;62(8):842–851.
Karasu T, Ozcan T, Aygun C, et al. Evaluation of patients with cavernomatous transformation of the portal vein. World J Gastroenterol. 2014;20(21):6694–6700.
Romi F. Thymoma in myasthenia gravis: From diagnosis to treatment. Autoimmune Dis. 2014;2014:474084.
Lin MW, Chang YL, Huang PM, et al. Thymoma management and long-term survival. J Thorac Oncol. 2010;5(2):199–204.
Nayyar D, Kumar A, Chawla YK, et al. Bleeding risk in non-cirrhotic portal hypertension. Hepatol Int. 2012;6(4):746–752.
Kumar A, Sharma P, Sarin SK. Hepatic venous pressure gradient in non-cirrhotic portal hypertension. World J Gastroenterol. 2012;18(41):5925–5930.
Sussman J, Farrugia ME, Maddison P, et al. Myasthenia gravis: Association with thymoma. J Neurol Neurosurg Psychiatry. 2012;83(5):492–498.
Poddar U. Approach to extrahepatic portal vein obstruction. Indian Pediatr. 2013;50(2):195–202.
Khandelwal N, Bhattacharya A, Chawla Y, et al. Imaging in portal hypertension. Indian J Radiol Imaging. 2008;18(3):203–214.
Sarin SK, Agarwal SR. Extrahepatic portal vein obstruction. Semin Liver Dis. 2002;22(1):43–58.
Bhatia V, Sarin SK. Recurrent variceal bleeding in EHPVO: Long-term management. Trop Gastroenterol. 2003;24(3):116–120.
Gauthier F, Claude A, Delalande O, et al. Preoperative plasmapheresis in thymomatous MG. Ann Neurol. 2003;53(4):426–430.
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