Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder, characterized by the progressive formation of renal cysts. Although ADPKD is strongly associated with cerebral and cardiovascular complications, cerebral ischemia caused by dissection of thoracic and carotid arteries has rarely been reported. We report the case of a 71-year-old Japanese woman who complained of hemiparesis. She required maintenance hemodialysis therapy with a background of ADPKD. Cerebral infarction was initially diagnosed by excluding intracranial hemorrhage and aneurysm rupture that are recognized as common complications of ADPKD and thereby anticoagulation therapy was initiated. However, the patient was suspected as having painless aortic dissection because a chest X-ray examination showed expanded upper mediastinum. Sequential vascular imagings revealed dissection of the aorta, originating from brachiocephalic trunk to the right common carotid artery with mediastinal hematoma. The patient died from progression of dissection. Herein, we described a case of the ADPKD patient that an acute aortic dissection without any pain induced the occlusion of supplying vessels to the brain, resulting in cerebral ischemic symptoms. A high level of clinical vigilance for an acute aortic dissection should be maintained in the ADPKD population with sudden onset of neurological symptoms even in the absence of pain. Furthermore, the initiation of anticoagulation treatment for cerebral ischemia which may aggravate the risk of further dissection requires careful consideration.
Similar content being viewed by others
References
Persu A, Duyme M, Pirson Y, Lens XM, Messiaen T, Breuning MH, et al. Comparison between siblings and twins supports a role for modifier genes in ADPKD. Kidney Int. 2004;66(6):2132–6. https://doi.org/10.1111/j.1523-1755.2004.66003.x.
Perrone RD, Malek AM, Watnick T. Vascular complications in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 2015;11(10):589–98. https://doi.org/10.1038/nrneph.2015.128.
Igarashi P, Somlo S. Genetics and pathogenesis of polycystic kidney disease. J Am Soc Nephrol. 2002;13(9):2384–98.
Saadi-Kheddouci S, Berrebi D, Romagnolo B, Cluzeaud F, Peuchmaur M, Kahn A, et al. Early development of polycystic kidney disease in transgenic mice expressing an activated mutant of the beta-catenin gene. Oncogene. 2001;20(42):5972–81. https://doi.org/10.1038/sj.onc.1204825.
Qian F, Germino FJ, Cai Y, Zhang X, Somlo S, Germino GG. PKD1 interacts with PKD2 through a probable coiled-coil domain. Nat Genet. 1997;16(2):179–83. https://doi.org/10.1038/ng0697-179.
Tsiokas L, Kim E, Arnould T, Sukhatme VP, Walz G. Homo- and heterodimeric interactions between the gene products of PKD1 and PKD2. Proc Natl Acad Sci USA. 1997;94(13):6965–70.
Peters DJ, Breuning MH. Autosomal dominant polycystic kidney disease: modification of disease progression. Lancet (London, England). 2001;358(9291):1439–44. https://doi.org/10.1016/s0140-6736(01)06531-x.
Ecder T, Schrier RW. Cardiovascular abnormalities in autosomal-dominant polycystic kidney disease. Nat Rev Nephrol. 2009;5(4):221–8. https://doi.org/10.1038/nrneph.2009.13.
Leier CV, Baker PB, Kilman JW, Wooley CF. Cardiovascular abnormalities associated with adult polycystic kidney disease. Ann Intern Med. 1984;100(5):683–8.
Grunfeld JP. Hereditary renal diseases. Saudi J Kidney Dis Transpl. 1997;8(3):227–34.
Sung PH, Yang YH, Chiang HJ, Chiang JY, Chen CJ, Liu CT, et al. Risk of aortic aneurysm and dissection in patients with autosomal-dominant polycystic kidney disease: a nationwide population-based cohort study. Oncotarget. 2017;8(34):57594–604. https://doi.org/10.18632/oncotarget.16338.
Stanley I, Sharma VK, Tsivgoulis G, Lao AY, Alexandrov AV. Painless aortic dissection with unusual extension into intracranial internal carotid arteries. Cerebrovasc Dis (Basel, Switzerland). 2007;24(2–3):314–5. https://doi.org/10.1159/000106517.
Rivera M, Gonzalo A, Gobernado JM, Orte L, Quereda C, Ortuno J. Stroke in adult polycystic kidney disease. Postgrad Med J. 1992;68(803):735–8.
Graf S, Schischma A, Eberhardt KE, Istel R, Stiasny B, Schulze BD. Intracranial aneurysms and dolichoectasia in autosomal dominant polycystic kidney disease. Nephrol Dialysis Transpl. 2002;17(5):819–23.
Chapman AB, Rubinstein D, Hughes R, Stears JC, Earnest MP, Johnson AM, et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. New England J Med. 1992;327(13):916–20. https://doi.org/10.1056/nejm199209243271303.
Huston J 3rd, Torres VE, Sulivan PP, Offord KP, Wiebers DO. Value of magnetic resonance angiography for the detection of intracranial aneurysms in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1993;3(12):1871–7.
Fick GM, Johnson AM, Hammond WS, Gabow PA. Causes of death in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1995;5(12):2048–56.
Yoo DJ, Agodoa L, Yuan CM, Abbott KC, Nee R. Risk of intracranial hemorrhage associated with autosomal dominant polycystic kidney disease in patients with end stage renal disease. BMC Nephrol. 2014;15:39. https://doi.org/10.1186/1471-2369-15-39.
Pirson Y, Chauveau D. ADPKD-associated intracranial aneurysm: new insights and unanswered questions. Contrib Nephrol. 1995;115:53–8.
Pirson Y, Chauveau D, van Gijn J. Subarachnoid haemorrhage in ADPKD patients: how to recognize and how to manage? Nephrol Dialysis Transpl. 1996;11(7):1236–8.
Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL, et al. The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. JAMA. 2000;283(7):897–903. https://doi.org/10.1001/jama.283.7.897.
Elefteriades JA, Sang A, Kuzmik G, Hornick M. Guilt by association: paradigm for detecting a silent killer (thoracic aortic aneurysm). Open Heart. 2015;2(1):e000169. https://doi.org/10.1136/openhrt-2014-000169.
Aoyagi S, Oda T, Kanamoto R, Nakamura E, Yasunaga H. Aortic dissection associated with autosomal dominant polycystic kidney disease. Heart Surgery Forum. 2019;22(1):E032–E34. https://doi.org/10.1532/hsf.2027.
Haruta S, Gunji K, Kawamura T, Hiroshima K. Generalized vascular dissection on pathological examination in a patient with polycystic kidney disease and acute aortic dissection. J Natl Med Assoc. 2019;111(5):563–8. https://doi.org/10.1016/j.jnma.2019.04.008.
Park SW, Hutchison S, Mehta RH, Isselbacher EM, Cooper JV, Fang J, et al. Association of painless acute aortic dissection with increased mortality. Mayo Clin Proc. 2004;79(10):1252–7. https://doi.org/10.4065/79.10.1252.
Spittell PC, Spittell JA Jr, Joyce JW, Tajik AJ, Edwards WD, Schaff HV, et al. Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). Mayo Clin Proc. 1993;68(7):642–51. https://doi.org/10.1016/s0025-6196(12)60599-0.
Gaul C, Dietrich W, Friedrich I, Sirch J, Erbguth FJ. Neurological symptoms in type A aortic dissections. Stroke. 2007;38(2):292–7. https://doi.org/10.1161/01.STR.0000254594.33408.b1.
Cohen S, Littmann D. Painless dissecting aneurysm of the aorta. New England J Med. 1964;271:143–5. https://doi.org/10.1056/nejm196407162710308.
Wooley CF, Sparks EH, Boudoulas H. Aortic pain. Prog Cardiovasc Dis. 1998;40(6):563–89. https://doi.org/10.1016/s0033-0620(98)80004-2.
Rahmatullah SI, Khan IA, Nair VM, Caccavo ND, Vasavada BC, Sacchi TJ. Painless limited dissection of the ascending aorta presenting with aortic valve regurgitation. Am J Emerg Med. 1999;17(7):700–1. https://doi.org/10.1016/s0735-6757(99)90165-1.
Marroush TS, Boshara AR, Parvataneni KC, Takla R, Mesiha NA. Painless aortic dissection. Am J Med Sci. 2017;354(5):513–20. https://doi.org/10.1016/j.amjms.2016.11.005.
Imamura H, Sekiguchi Y, Iwashita T, Dohgomori H, Mochizuki K, Aizawa K, et al. Painless acute aortic dissection—diagnostic, prognostic and clinical implications. Circ J. 2011;75(1):59–66. https://doi.org/10.1253/circj.cj-10-0183.
Moon J, Lee SH, Yang WI, Ko YG, Han WK, Jang Y. Fatal renal bleeding in a patient treated with aggressive antithrombotic therapy after recurrent coronary stent thrombosis. Korean Circ J. 2010;40(7):348–51. https://doi.org/10.4070/kcj.2010.40.7.348.
Faggion Vinholo T, Zafar MA, Ziganshin BA, Elefteriades JA. Nonsyndromic thoracic aortic aneurysms and dissections—is screening possible? Semin Thorac Cardiovasc Surg. 2019;31(4):628–34. https://doi.org/10.1053/j.semtcvs.2019.05.035.
Author information
Authors and Affiliations
Corresponding author
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
About this article
Cite this article
Yamaguchi, S., Wakino, S., Tokuyama, H. et al. A case of cerebral infarction caused by painless acute aortic dissection in autosomal dominant polycystic kidney disease. CEN Case Rep 9, 177–181 (2020). https://doi.org/10.1007/s13730-020-00450-4
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s13730-020-00450-4