Cruvelier-Baumgarten Syndrome Type II in Combination with Arteriovenous Splenic Artery Fistula: Literature Review and Clinical Case
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Abstract
Cruvelier-Baumgarten syndrome is a rare disease that causes portal hypertension in association with unclogged umbilical or paraumbilical veins, hypoplasia of the intrahepatic portal system, and liver atrophy with minimal or no fibrosis. Patients with this condition experience the classic signs of portal hypertension such as gastrointestinal bleeding, ascites, and encephalopathy. Frequent clinical manifestations are the symptom of "jellyfish head" and venous murmur over the umbilical region due to the presence of large umbilical and paraumbilical veins.
In the presented clinical case of Cruvelier-Baumgarten syndrome type II in combination with splenic artery arteriovenous fistula, pathological changes were not pronounced. There was no "jellyfish head" symptom, but a venous murmur was heard in the umbilical region. The patient complained of moderate pain in the epigastrium, right hypochondrium. Examination of the patient, which revealed, in addition to the typical manifestations of this disease, also multiple SAVFs, as well as angiography data regarding pressure in the umbilical vein - 5 mm Hg, determined the tactics of treatment in the volume of endovascular occlusion of the splenic artery branches, which was successfully completed. As a result, there was a decrease in pressure in the portal system and a sharp decrease in discharge through the recanalized umbilical vein into the basin of the inferior vena cava, which made it possible to level out complaints. At the control examination two months after treatment, the patient had no complaints.
A review of the literature on the Cruveilhier-Baumgarten syndrome and disease is presented.
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References
I. Pegot N. Tumeur variqueuse avec anomalie du systeme veineux et persistance de la veine ombilicale. developpement des veins souscutanees abdominales. Bull Soc Anat (Paris) 1833; 8: 57.
II. Cruveilhier J. Maladie des veines. In: Anatomie pathologique du corps humain. Paris, Bailliére. 1829-1835, volume I, page 16.
III. Von Baumgarten P. Uebervoll standiges Offenbleiben der Vena umbilicalis; zugleich ein Beitrag zur Frage des Morbus Bantii. Arb Geb Pathol Anat Int Tubingen. 1907; 6: 93-110.
IV. Macpherson A., Morton E. Continuous venous hum in a case of portal cirrhosis. British Heart Journal. 1955; 17: 105-108. doi: 10.1136/hrt.17.1.105
V. Armstrong EL, Adams WL, Tragerman LI, Townsend EW. The Cruveilhier-Baumgarten syndrome. Ann Intern Med 1942; 16: 113-51.
VI. Steinberg J.S., Galambos J.T. Cruveilhier-Baumgarten (C-B) disease. An experiment of nature. Am J Med. 1967 Aug; 43(2): 284-288. doi: 10.1016/0002-9343(67)90170-2.
VII. Jahnke E.H. Jr, Palmer E.D., Brick I.B. The Cruveilhier-Baumgarten syndrome: a review and report of four cases; three treated by direct portacaval shunt. Ann Surg. 1954 Jul; 140(1):44-55. doi: 10.1097/00000658-195407000-00004.
VIII. Patsiora M.D., Ershov Iu.A., Danil'iants E.V., Kharazov F.V. Cruveilhier-Baumgarten syndrome. Klin Med (Mosk). 1975 Nov; 53(11): 83-78.
IX. Isogai J., Sakamoto H., Asahi J.P., Tsukuba J.P. Cruveilhier-Baumgarten syndrome: anatomical and pathologic imaging of periumbilical venous network. Poster: ECR 2014/C-0442. DOI: 10.1594/ecr2014/C-0442
X. Stepanova Yu.A., Karmazanovsky G.G., Kokov L.S., Tsygankov V.N. True aneurysms of the visceral arteries: radiological methods of diagnosis and treatment. Doctor.Ru. 2015; 1(11):46–52.
XI. Elamurugan T.P., Kumar S.S., Muthukumarassamy R., Kate V. Splenic artery aneurysm presenting as extrahepatic portal vein obstruction: a case report. Case Rep Gastrointest Med. 2011; 2011: 908529. doi: 10.1155/2011/908529.
XII. Khan A., Ayub M., Haider I., Humayun M., Shah Z., Ajmal F. Coexisting giant splenic artery and portal vein aneurysms leading to non-cirrhotic portal hypertension: a case report. J Med Case Rep. 2016 Sep 29; 10(1): 270. doi: 10.1186/s13256-016-1059-4.
XIII. Beksac K., Karakoc D. Multiple Giant Splenic Artery Aneurysms Causing Sinistral (Left-Sided) Portal Hypertension. Case Rep Gastrointest Med. 2016; 2016: 6278452. doi: 10.1155/2016/6278452.
XIV. Rehman Z.U. Multiple Giant Splenic Artery Aneurysms with Hypersplenism and Portal Hypertension: A Case Report. Ann Vasc Dis. 2019 Jun 25; 12(2): 250-252. doi: 10.3400/avd.cr.19-00021.
XV. Satchithanantham V., Bandara G., Gooneratne T., Prasath S., Ubayasiri R. Multiple splenic artery aneurysms causing sinistral portal hypertension. Sri Lanka Journal of Surgery. April 2020; 38(1): 62.
XVI. Cecil D.M., Chaturvedi A., Kapoor D., Shekhar A. Cruveilhier-Baumgarten syndrome with multiple splenic artery aneurysms: A case report. Medical Journal of Dr. D.Y. Patil University. Jan-Feb 2016; 9(1): doi: 10.4103/0975-2870.167983
XVII. Weigert C. In die milzvene geborstenes aneurysma einer milzarterie. Arch Pathol Anat. 1886; 104: 26-30.
XVIII. Hung C.F., Tseng J.H., Lui K.W., Wan Y.L., Tsai C.C., Shem C.H., Wu C.S. Intractable oesophageal variceal bleeding caused by splenic arteriovenous fistula: treatment by transcatheter arterial embolization. Postgrad J Med 1999; 6: 75: 355.
XIX. Woźniak W., Mlosek R.K., Miłek T., Myrcha P., Ciostek P. Splenic arteriovenous fistula - late complications of splenectomy. Acta Gastroenterol Belg. 2011 Sep; 74(3): 465-467.
XX. Gartside R., Gamelli R.L. Splenic Arteriovenous fistula. J Trauma. 1987; 27: 6: 671.
XXI. Yadav R., Tiwari M.K., Mathur R.M., Verma A.K. Unusually giant splenic artery and vein aneurysm with arteriovenous fistula with hypersplenism in a nulliparous woman. Interactive cardiovascular and thoracic surgery. 2009; 8: 384-386.
XXII. De Perrot M., Buhler L., Deleaval J., Borisch B., Mentha G., Morel P. Management of true aneurysms of the splenic artery. Am J Surg 1998; 175: 466-468.
XXIII. Pulli R., Innocenti A., Barbanti E., Dorigo W., Turini F., Gatti M., Pratesi C. Early and long-term results of surgical treatment of splenic artery aneurysms. Am J Surg 2001; 182: 520-523.
XXIV. Hogendoorn W., Lavida A., Hunink M.G., Moll F.L., Geroulakos G., Muhs B.E., Sumpio B.E. Open repair, endovascular repair, and conservative management of true splenic artery aneurysms. J Vasc Surg. 2014 Dec; 60(6): 1667-76.e1. doi: 10.1016/j.jvs.2014.08.067.
XXV. Siablis D., Papathanassiou Z.G., Karnabatidis D., Christeas N., Katsanos K. Splenic arteriovenous fistula and sudden onset of portal hypertension as complications of a ruptured splenic artery aneurysm: Successful treatment with transcatheter arterial embolization. A case study and review of the literature. World Journal of Gastroenterology 2006; 7: 12: 26: 4264-4266.
XXVI. Lam K.C., Juttner H.U., Reynolds T.B. Spontaneous portosystemic shunt: Relationship to spontaneous encephalopathy and gastrointestinal hemorrhage. Dig Dis Sci 1981; 26: 346-352.
XXVII. Cho K.C., Patel Y.D., Wachsberg R.H., Seeff J. Varices in portal hypertension: Evaluation with CT. Radiographics 1995; 15: 609-622.
XXVIII. Singla V., Galwa R.P., Saxena A.K., Khandelwal N. Cruveilhier-Baumgarten syndrome with giant paraumbilical vein. J Postgrad Med 2008; 54: 328-329.
XXIX. Sodhi J.S., Zarger S.A., Khan M.A., Javid G., Khan B.A., Shah A.H., Gulzar G.M., Manjeet. Cruveilheir-Baumgarten syndrome revisited. Indian J Gastroenterol 2007; 26: 173.
XXX. Henseler K.P., Pozniak M.A., Lee F.T. Jr, Winter T.C. 3rd. Threedimensional CT angiography of spontaneous portosystemic shunts. Radiographics. 2001; 21: 691-704.
XXXI. Khati N.J., Enquist E.G., Javitt M.C. Imaging of the umbilicus and periumbilical region. Radiographics. 1998 Mar-Apr; 18(2): 413-431. doi: 10.1148/radiographics.18.2.9536487.
XXXII. Moubarak E., Bouvier A., Boursier J., Lebigot J., Ridereau-Zins C., Thouveny F., Willoteaux S., Aubé C. Portosystemic collateral vessels in liver cirrhosis: a three-dimensional MDCT pictorial review. Abdom Imaging. 2012 Oct; 37(5): 746-766. doi: 10.1007/s00261-011-9811-0.
XXXIII. Nogueira Junqueira J.C., Cavalanti A., Chammas M.C., Durante H., Ando S.M. Patent paraumbilical vein on ultrasound: not always cirrhosis/ Poster: ECR 2018 / C-2303 doi: 10.1594/ecr2018/C-2303
XXXIV. Sodhi K.S., Saxena A.K., Khandelwal N., Dhiman R.K. Giant paraumblical veins in Cruveilhier-Baumgarten syndrome. Gastrointest Endosc. 2010; 72: 435-436.
XXXV. Bisseru B., Patel J.S. Cruveilhier-Baumgarten (C-B) disease. Gut. 1989 Jan; 30(1): 136–137. doi: 10.1136/gut.30.1.136
XXXVI. Yakubovitch D., Halak M., Khaikin M., Silverberg D. Multiple splenic artery aneurysms in a patient with portal hypertension. Isr Med Assoc J. 2013; 15: 55-56.