Primary Budd-Chiari syndrome (BCS) is a spontaneously fatal disease characterized by an obstruction of the hepatic venous outflow tract due to thrombosis or a primary disease of the venous wall. The primary form of BCS is extremely rare. This is a disease mainly affecting young adults of both sexes. Clinical manifestations are variable; they can be asymptomatic, acute, or subacute but mostly chronic. Several causes have been identified, such as myeloproliferative syndrome, antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, and inherited thrombotic disorders. Data on primary BCS in Sub-Saharan Africa is rare as most publications available are case reports. In these reports, the causes are unknown with poor prognosis in most cases often leading to patient death. We herein present a case report of a male patient diagnosed with a primary BCS at Yaoundé General Hospital (Cameroon) caused by a Protein C deficiency who presented with ascites decompensating liver cirrhosis. Treatment was based on anticoagulants, diuretics and laxatives administration. Two years after the diagnosis, the patient is alive with clinical and paraclinical improvement.
References
[1]
Garcia-Pagán, J.C. and Valla, D.C. (2023) Primary Budd-Chiari Syndrome. The New England Journal of Medicine, 388, 1307-1316. https://doi.org/10.1056/NEJMra2207738
[2]
Kahindo, K.C. and Tsongo, K.Z. (2019) Syndrome de Budd-Chiari : A propos d’un cas. Kisangani Médical, 9, 356-361.
[3]
Service, G., Deby, G., Atipo, B.I., Maganga Moussavou, I.F., Ngoma Nkandoulou, P.H., Ibara, J.R., Itoua Ngaporo, A. and Okouo, M. (2014) Syndrome de Budd-Chiari. A propos d’un cas au CHU de Brazzaville et revue de la literature. Médecine D’Afrique Noire, 61, 263-269.
Grus, T., Lambert, L., Grusová, G., Banerjee, R. and Burgetová, A. (2017) Budd-Chiari Syndrome. Prague Medical Report, 118, 69-80. https://doi.org/10.14712/23362936.2017.6
[6]
Ouhadi, L., Creemers, E., Honoré, P., Delwaide, J., Marchetta, S. and Defraigne, J.O. (2015) Budd-Chiari Syndrome: A Case Report and Review of the Literature. Revue Médicale De Liège, 70, 378-383.
[7]
Plessier, A. (2016) Syndrome de Budd-Chiari (Budd-Chiari Syndrome). Société nationale française de gastroentérologie.
[8]
Benali, F. and Aqodad, N. (2020) Budd-Chiari primitif: Défi diagnostique et thérapeutique. The Pan African Medical Journal, 37, Article 142. https://doi.org/10.11604/pamj.2020.37.142.25109
[9]
Aydinli, M. and Bayraktar, Y. (2007) Budd-Chiari Syndrome: Etiology, Pathogenesis and Diagnosis. World Journal of Gastroenterology, 13, 2693-2696. https://doi.org/10.3748/wjg.v13.i19.2693
[10]
Plessier, A. and Valla, D.C. (2008) Budd-Chiari Syndrome. Seminars in Liver Disease, 28, 259-269. https://doi.org/10.1055/s-0028-1085094
[11]
Singh, V., Sinha, S.K., Nain, C.K., Bambery, P., Kaur, U., Verma, S., et al. (2000) Budd-Chiari Syndrome: Our Experience of 71 Patients. Journal of Gastroenterology and Hepatology, 15, 550-554. https://doi.org/10.1046/j.1440-1746.2000.02157.x
[12]
Cazals-Hatem, D., Vilgrain, V., Genin, P., Denninger, M.H., Durand, F., Belghiti, J., et al. (2003) Arterial and Portal Circulation and Parenchymal Changes in Budd-Chiari Syndrome: A Study in 17 Explanted Livers. Hepatology, 37, 510-519. https://doi.org/10.1053/jhep.2003.50076
[13]
Sempoux, C., Paradis, V., Komuta, M., Wee, A., Calderaro, J., Balabaud, C., et al. (2015) Hepatocellular Nodules Expressing Markers of Hepatocellular Adenomas in Budd-Chiari Syndrome and Other Rare Hepatic Vascular Disorders. Journal of Hepatology, 63, 1173-1180. https://doi.org/10.1016/j.jhep.2015.06.017
[14]
Van Wettere, M., Paulatto, L., Raynaud, L., Bruno, O., Payancé, A., Plessier, A., et al. (2020) Hepatobiliary MR Contrast Agents Are Useful to Diagnose Hepatocellular Carcinoma in Patients with Budd-Chiari Syndrome. JHEP Reports, 2, Article ID: 100097. https://doi.org/10.1016/j.jhepr.2020.100097
[15]
Garcia-Pagán, J.C., Heydtmann, M., Raffa, S., Plessier, A., Murad, S., Fabris, F., et al. (2008) TIPS for Budd-Chiari Syndrome: Long-Term Results and Prognostics Factors in 124 Patients. Gastroenterology, 135, 808-815. https://doi.org/10.1053/j.gastro.2008.05.051
[16]
Hamulyák, E.N., Daams, J.G., Leebeek, F.W.G., Biemond, B.J., Te Boekhorst, P.A.W., Middeldorp, S., et al. (2021) A Systematic Review of Antithrombotic Treatment of Venous Thromboembolism in Patients with Myeloproliferative Neoplasms. Blood Advances, 5, 113-121. https://doi.org/10.1182/bloodadvances.2020003628
[17]
Semmler, G., Lindorfer, A., Schäfer, B., Bartl, S., Hametner-Schreil, S., Gensluckner, S., et al. (2023) Outcome of Budd-Chiari Syndrome Patients Treated with Direct Oral Anticoagulants: An Austrian Multicenter Study. Clinical Gastroenterology and Hepatology, 21, 978-887.E2. https://doi.org/10.1016/j.cgh.2022.04.024
[18]
Tavill, A.S., Wood, E.J., Kreel, L., Jones, E.A., Gregory, M. and Sherlock, S. (1975) The Budd-Chiari Syndrome: Correlation between Hepatic Scintigraphy and the Clinical, Radiological, and Pathological Findings in Nineteen Cases of Hepatic Venous Outflow Obstruction. Gastroenterology, 68, 509-518. https://doi.org/10.1016/S0016-5085(75)80090-4
[19]
Seijo, S., Plessier, A., Hoekstra, J., Dell’era, A., Mandair, D., Rifai, K., et al. (2013) Good Long-Term Outcome of Budd-Chiari Syndrome with a Step-Wise Management. Hepatology, 57, 1962-1968. https://doi.org/10.1002/hep.26306
[20]
Zeitoun, G., Escolano, S., Hadengue, A., Azar, N., El Younsi, M., Mallet, A., et al. (1999) Outcome of Budd-Chiari Syndrome: A Multivariate Analysis of Factors Related to Survival Including Surgical Portosystemic Shunting. Hepatology, 30, 84-89. https://doi.org/10.1002/hep.510300125
[21]
Langlet, P., Escolano, S., Valla, D., Coste-Zeitoun, D., Denie, C., Mallet, A., et al. (2003) Clinicopathological Forms and Prognostic Index in Budd-Chiari Syndrome. Journal of Hepatology, 39, 496-501. https://doi.org/10.1016/S0168-8278(03)00323-4
[22]
Darwish Murad, S., Valla, D.C., de Groen, P.C., Zeitoun, G., Hopmans, J.A.M., Haagsma, E.B., et al. (2004) Determinants of Survival and the Effect of Portosystemic Shunting in Patients with Budd-Chiari Syndrome. Hepatology, 39, 500-508. https://doi.org/10.1002/hep.20064
[23]
Rautou, P.E., Moucari, R., Escolano, S., Cazals-Hatem, D., Denié, C., Chagneau-Derrode, C., et al. (2009) Prognostic Indices for Budd-Chiari Syndrome: Valid for Clinical Studies But Insufficient for Individual Management. American Journal of Gastroenterology, 104, 1140-1146. https://doi.org/10.1038/ajg.2009.63
