A challenging case of IVC compression in an adult polycystic patient. A case report

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Abstract Adult polycystic kidney disease (ADPKD) is a multi system genetic disorder characterized by the development and progressive enlargement of fluid-filled cysts in both kidneys, along with other organs. As one of the main causes of kidney failure, ADPKD can progress to end-stage renal disease (ESRD), with over 50% of affected individuals progressing to ESRD by age 50. The symptoms in ADPKD are variable, with some patients experiencing nonspecific signs, while others present with symptoms related to the mass effect of enlarged kidneys on surrounding structures. This case report highlights an unusual presentation of ADPKD in a patient who developed symptoms of inferior vena cava (IVC) compression. Remarkably, these symptoms improved after bilateral nephrectomies, suggesting that bilateral nephrectomy would provide help in these situations.
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A challenging case of IVC compression in an adult polycystic patient. A case report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report A challenging case of IVC compression in an adult polycystic patient. A case report Ahmad Matarneh, Abdelraouf Akkari, Sundus Sardar, Ronald Miller, and 3 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-5405666/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Adult polycystic kidney disease (ADPKD) is a multi system genetic disorder characterized by the development and progressive enlargement of fluid-filled cysts in both kidneys, along with other organs. As one of the main causes of kidney failure, ADPKD can progress to end-stage renal disease (ESRD), with over 50% of affected individuals progressing to ESRD by age 50. The symptoms in ADPKD are variable, with some patients experiencing nonspecific signs, while others present with symptoms related to the mass effect of enlarged kidneys on surrounding structures. This case report highlights an unusual presentation of ADPKD in a patient who developed symptoms of inferior vena cava (IVC) compression. Remarkably, these symptoms improved after bilateral nephrectomies, suggesting that bilateral nephrectomy would provide help in these situations. ADPKD IVC compression nephrectomy Figures Figure 1 Figure 2 Figure 3 Figure 4 Introduction ADPKD is an autosomal dominant inherited condition caused by mutations in the PKD1 gene. It is characterized by the formation of cysts within the kidneys, as well as other organs ( 1 ). It may be asymptomatic initially, but as cysts enlarge, it can cause a wide range of symptoms related to the disease’s mass effect. Abdominal discomfort, hematuria, renal colic due to renal calculi are among the most common symptoms ( 2 ). Massive kidney enlargement can lead to compression of nearby vascular structures, notably the IVC. IVC compression can result in impaired venous return, leading to venous stasis, congestion, and edema in the lower limbs ( 3 ). Diagnosis of IVC compression typically requires imaging studies, such as abdominal CT scans with contrast, to assess the extent of kidney enlargement and vascular involvement ( 4 ). This report presents the case of a 51-year-old female with ESRD due to ADPKD who experienced persistent lower limb edema with cramping during hemodialysis. Imaging revealed significant IVC compression due to the mass effect of her kidneys.She improved after bilateral nephrectomies Case Presentation The patient is a 51-year-old female with a history of ESRD secondary to ADPKD, for which she has been receiving maintenance hemodialysis (HD) for several years. She presented to the HD clinic with complaints of persistent bilateral lower limb edema, which did not respond to adjustments in her estimated dry weight (EDW) and increasing ultrafiltration during dialysis sessions. Despite her chest examination being clear with no evidence of fluid overload in the lungs, her edema persisted. Attempts to modify her dialysis prescription to achieve better fluid removal were unsuccessful, and she continued to experience lower limb edema as well as cramping during HD. On physical examination, the patient had a distended abdomen, though bowel sounds were normal. There was no tenderness on palpation, but the kidneys appeared to be markedly enlarged. Given her ongoing symptoms and the lack of response to fluid management changes, there was a suspicion that her symptoms could be related to IVC compression caused by enlarged kidneys. A bedside ultrasound was performed, which revealed large, cyst-filled kidneys. Consequently, a contrast-enhanced CT scan of the abdomen was ordered, confirming the presence of massively enlarged kidneys, with measurements of 22x22x12 cm on the left side and 29x19x12 cm on the right. The CT scan further revealed compression of the IVC, likely resulting in venous congestion and contributing to her lower limb edema and dialysis-associated cramping. (Figs. 1–3) Following this diagnosis, the patient was referred to the urology team, who recommended bilateral nephrectomies to relieve the IVC compression. The patient subsequently underwent the surgical procedure, which was successful, and her symptoms improved significantly postoperatively. She continued to undergo HD without further episodes of cramping or persistent edema. At follow-up, the patient reported a substantial improvement in her quality of life and an absence of her previous symptoms. CT scan was done for follow up (Fig. 4) Discussion ADPKD is among the most common genetic kidney diseases and a leading cause of ESRD, affecting approximately 1 in 400 to 1 in 1,000 individuals worldwide ( 5 ). The disease is characterized by gradual kidney enlargement due to cyst growth, which often results in progressive renal failure. As cysts become more enlarged, they compress surrounding renal parenchyma, ultimately compromising renal function ( 6 ). In advanced cases, ADPKD can lead to ESRD, necessitating renal replacement therapies such as dialysis or transplantation. In addition to renal involvement, ADPKD patients may experience complications involving the liver, pancreas, and vascular system, with a significant number developing cardiovascular issues such as hypertension and vascular aneurysms ( 7 ). When kidney enlargement is substantial, the cystic masses can compress nearby structures, including the gastrointestinal tract and vascular structures such as the IVC and, in rare instances, the aorta ( 8 ). IVC compression due to ADPKD-related kidney enlargement is uncommon but clinically important, as it can mimic other conditions, including volume overload, by presenting with edema and circulatory congestion in the lower extremities. Symptoms of IVC compression may include edema, venous stasis, and discomfort due to the increased pressure in the lower extremity veins, which impairs normal venous return to the heart ( 9 ). This can create diagnostic challenges in ESRD patients on HD, where fluid management is critical and symptoms are often attributed to volume overload or intradialytic cramping. The case presented here describes an unusual presentation of ADPKD with IVC compression that mimicked symptoms of volume overload. Diagnostic imaging confirmed the mass effect from the enlarged kidneys on the IVC, a finding that guided the decision to pursue bilateral nephrectomies as a therapeutic intervention. Following nephrectomy, the patient’s symptoms resolved, indicating that the mass effect was indeed the primary cause of her lower limb edema and HD-related cramping. This case sheds light on the importance of considering mass effect and vascular compression as potential contributors to symptoms in ADPKD patients, particularly those with ESRD where typical volume management strategies may not alleviate symptoms. While the exact incidence of IVC compression in ADPKD remains unclear, reports suggest that it is a rare but potentially underdiagnosed complication in patients with extensive kidney enlargement ( 10 ). This case reinforces the role of imaging in assessing symptomatic ADPKD patients with atypical presentations and provides support for considering surgical interventions, such as nephrectomy, when vascular compression leads to significant clinical symptoms. Conclusion Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a prevalent condition that can lead to End-Stage Renal Disease (ESRD), presenting a range of symptoms from asymptomatic cases to those with significant mass effects. When IVC (inferior vena cava) compression occurs, it may produce nonspecific symptoms that complicate diagnosis. Therefore, timely imaging is crucial for accurate diagnosis and management. Surgical intervention leads to alleviation of symptoms. Declarations Ethical Considerations: NA Consent to Participate: NA Consent for Publication: Verbal informed consent was obtained from the patient to publish this report in accordance with the journal’s patient consent policy Funding: The funding process is solely done from the writing authors Declaration of Conflicting Interest No COI to declare by authors Data Availability: Data included in this manuscript will be available upon a reasonable request. Trial Registration: NA Clinical trial number: not applicable. Guarantor: U.F Contributorship: Ahmad Matarneh: Manuscript writing, literature review, Clinical care Abdelraouf Akkari: Manuscript writing, literature review, clinical care Sundus sardar: Manuscript writing, literature review, Clinical care Navin verma: Manuscript writing, literature review, Clinical care Nasrollah Ghahramani: Clinical care, literature review, manuscript write up, Mentor Umar Farooq: Clinical care, literature review, manuscript write up, Mentor Acknowledgements: We thank the nephrology department at Pennsylvania State Milton S. Hershey for allowing us to participate in this publication. References Chapman AB, Devuyst O, Eckardt KU, Gansevoort RT, Harris T, Horie S, Kasiske BL, Odland D, Pei Y, Perrone RD, Pirson Y. Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney international. 2015 Jul 1;88(1):17-27. Bergmann C. ARPKD and early manifestations of ADPKD: the original polycystic kidney disease and phenocopies. Pediatric Nephrology. 2015 Jan;30(1):15-30. Yin X, Blumenfeld JD, Riyahi S, Luo X, Rennert H, Barash I, Prince MR. Prevalence of inferior vena cava compression in ADPKD. Kidney International Reports. 2021 Jan 1;6(1):168-78. Iguchi S, Kasai A, Kishimoto H, Suzuki K, Ito S, Ogawa Y, Nishi S, Gejyo F, Ohno Y. Thrombosis in inferior vena cava (IVC) due to intra-cystic hemorrhage into a hepatic local cyst with autosomal dominant polycystic kidney disease (ADPKD). Internal medicine. 2004;43(3):209-12. Willey C, Kamat S, Stellhorn R, Blais J. Analysis of nationwide data to determine the incidence and diagnosed prevalence of autosomal dominant polycystic kidney disease in the USA: 2013–2015. Kidney Diseases. 2019 Jan 9;5(2):107-17. Sutter M, Germino GG. Autosomal dominant polycystic kidney disease: molecular genetics and pathophysiology. Journal of Laboratory and Clinical Medicine. 2003 Feb 1;141(2):91-101. Bergmann C, Guay-Woodford LM, Harris PC, Horie S, Peters DJ, Torres VE. Polycystic kidney disease. Nature reviews Disease primers. 2018 Dec 6;4(1):50. Fujimaru T, Mori T, Sekine A, Mandai S, Chiga M, Kikuchi H, Ando F, Mori Y, Nomura N, Iimori S, Naito S. Kidney enlargement and multiple liver cyst formation implicate mutations in PKD1/2 in adult sporadic polycystic kidney disease. Clinical genetics. 2018 Jul;94(1):125-31. Yugueros X, Alvarez B, Fernández E, Boqué M, Matas M. Compressive symptoms due to thrombosed or hypertrophic collateral circulation in infrarenal inferior vena cava agenesis. Annals of Vascular Surgery. 2013 Feb 1;27(2):238-e9. Fuller TF, Brennan TV, Feng S, Kang SM, Stock PG, Freise CE. End stage polycystic kidney disease: indications and timing of native nephrectomy relative to kidney transplantation. The Journal of urology. 2005 Dec 1;174(6):2284-8. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-5405666","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":379776019,"identity":"5f4fbd05-90a2-4116-a023-95582848edc1","order_by":0,"name":"Ahmad 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A case report","fulltext":[{"header":"Introduction","content":"\u003cp\u003eADPKD is an autosomal dominant inherited condition caused by mutations in the PKD1 gene. It is characterized by the formation of cysts within the kidneys, as well as other organs (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). It may be asymptomatic initially, but as cysts enlarge, it can cause a wide range of symptoms related to the disease’s mass effect. Abdominal discomfort, hematuria, renal colic due to renal calculi are among the most common symptoms (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). Massive kidney enlargement can lead to compression of nearby vascular structures, notably the IVC. IVC compression can result in impaired venous return, leading to venous stasis, congestion, and edema in the lower limbs (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). Diagnosis of IVC compression typically requires imaging studies, such as abdominal CT scans with contrast, to assess the extent of kidney enlargement and vascular involvement (\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e). This report presents the case of a 51-year-old female with ESRD due to ADPKD who experienced persistent lower limb edema with cramping during hemodialysis. Imaging revealed significant IVC compression due to the mass effect of her kidneys.She improved after bilateral nephrectomies\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eThe patient is a 51-year-old female with a history of ESRD secondary to ADPKD, for which she has been receiving maintenance hemodialysis (HD) for several years. She presented to the HD clinic with complaints of persistent bilateral lower limb edema, which did not respond to adjustments in her estimated dry weight (EDW) and increasing ultrafiltration during dialysis sessions. Despite her chest examination being clear with no evidence of fluid overload in the lungs, her edema persisted. Attempts to modify her dialysis prescription to achieve better fluid removal were unsuccessful, and she continued to experience lower limb edema as well as cramping during HD.\u003c/p\u003e\u003cp\u003eOn physical examination, the patient had a distended abdomen, though bowel sounds were normal. There was no tenderness on palpation, but the kidneys appeared to be markedly enlarged. Given her ongoing symptoms and the lack of response to fluid management changes, there was a suspicion that her symptoms could be related to IVC compression caused by enlarged kidneys. A bedside ultrasound was performed, which revealed large, cyst-filled kidneys. Consequently, a contrast-enhanced CT scan of the abdomen was ordered, confirming the presence of massively enlarged kidneys, with measurements of 22x22x12 cm on the left side and 29x19x12 cm on the right. The CT scan further revealed compression of the IVC, likely resulting in venous congestion and contributing to her lower limb edema and dialysis-associated cramping. (Figs.\u0026nbsp;1–3)\u003c/p\u003e\u003cp\u003eFollowing this diagnosis, the patient was referred to the urology team, who recommended bilateral nephrectomies to relieve the IVC compression. The patient subsequently underwent the surgical procedure, which was successful, and her symptoms improved significantly postoperatively. She continued to undergo HD without further episodes of cramping or persistent edema. At follow-up, the patient reported a substantial improvement in her quality of life and an absence of her previous symptoms. CT scan was done for follow up (Fig.\u0026nbsp;4)\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eADPKD is among the most common genetic kidney diseases and a leading cause of ESRD, affecting approximately 1 in 400 to 1 in 1,000 individuals worldwide (\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e). The disease is characterized by gradual kidney enlargement due to cyst growth, which often results in progressive renal failure. As cysts become more enlarged, they compress surrounding renal parenchyma, ultimately compromising renal function (\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e). In advanced cases, ADPKD can lead to ESRD, necessitating renal replacement therapies such as dialysis or transplantation. In addition to renal involvement, ADPKD patients may experience complications involving the liver, pancreas, and vascular system, with a significant number developing cardiovascular issues such as hypertension and vascular aneurysms (\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eWhen kidney enlargement is substantial, the cystic masses can compress nearby structures, including the gastrointestinal tract and vascular structures such as the IVC and, in rare instances, the aorta (\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e). IVC compression due to ADPKD-related kidney enlargement is uncommon but clinically important, as it can mimic other conditions, including volume overload, by presenting with edema and circulatory congestion in the lower extremities. Symptoms of IVC compression may include edema, venous stasis, and discomfort due to the increased pressure in the lower extremity veins, which impairs normal venous return to the heart (\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e). This can create diagnostic challenges in ESRD patients on HD, where fluid management is critical and symptoms are often attributed to volume overload or intradialytic cramping.\u003c/p\u003e \u003cp\u003eThe case presented here describes an unusual presentation of ADPKD with IVC compression that mimicked symptoms of volume overload. Diagnostic imaging confirmed the mass effect from the enlarged kidneys on the IVC, a finding that guided the decision to pursue bilateral nephrectomies as a therapeutic intervention. Following nephrectomy, the patient\u0026rsquo;s symptoms resolved, indicating that the mass effect was indeed the primary cause of her lower limb edema and HD-related cramping. This case sheds light on the importance of considering mass effect and vascular compression as potential contributors to symptoms in ADPKD patients, particularly those with ESRD where typical volume management strategies may not alleviate symptoms.\u003c/p\u003e \u003cp\u003eWhile the exact incidence of IVC compression in ADPKD remains unclear, reports suggest that it is a rare but potentially underdiagnosed complication in patients with extensive kidney enlargement (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e). This case reinforces the role of imaging in assessing symptomatic ADPKD patients with atypical presentations and provides support for considering surgical interventions, such as nephrectomy, when vascular compression leads to significant clinical symptoms.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eAutosomal Dominant Polycystic Kidney Disease (ADPKD) is a prevalent condition that can lead to End-Stage Renal Disease (ESRD), presenting a range of symptoms from asymptomatic cases to those with significant mass effects. When IVC (inferior vena cava) compression occurs, it may produce nonspecific symptoms that complicate diagnosis. Therefore, timely imaging is crucial for accurate diagnosis and management. Surgical intervention leads to alleviation of symptoms.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003eEthical Considerations:\u003c/p\u003e\n\u003cp\u003eNA\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eConsent to Participate:\u003c/p\u003e\n\u003cp\u003eNA\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eConsent for Publication:\u003c/p\u003e\n\u003cp\u003eVerbal informed consent was obtained from the patient to publish this report in accordance with the journal’s patient consent policy\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eFunding:\u003c/p\u003e\n\u003cp\u003eThe funding process is solely done from the writing authors\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eDeclaration of Conflicting Interest\u003cbr\u003e\u0026nbsp;No COI to declare by authors\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eData Availability:\u003c/p\u003e\n\u003cp\u003eData included in this manuscript will be available upon a reasonable request.\u003c/p\u003e\n\u003cp\u003eTrial Registration:\u003c/p\u003e\n\u003cp\u003eNA\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eClinical trial number: not applicable.\u003c/p\u003e\n\u003cp\u003eGuarantor: U.F\u003c/p\u003e\n\u003cp\u003eContributorship:\u003c/p\u003e\n\u003cp\u003eAhmad Matarneh: Manuscript writing, literature review, Clinical care\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eAbdelraouf Akkari: Manuscript writing, literature review, clinical care\u003c/p\u003e\n\u003cp\u003eSundus sardar: Manuscript writing, literature review, Clinical care\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eNavin verma: Manuscript writing, literature review, Clinical care\u003c/p\u003e\n\u003cp\u003eNasrollah Ghahramani: Clinical care, literature review, manuscript write up, Mentor\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eUmar Farooq: Clinical care, literature review, manuscript write up, Mentor\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eAcknowledgements:\u003c/p\u003e\n\u003cp\u003eWe thank the nephrology department at Pennsylvania State Milton S. Hershey for allowing us to participate in this publication.\u003c/p\u003e"},{"header":"References","content":"\u003col start=\"1\" type=\"1\"\u003e\n \u003cli\u003eChapman AB, Devuyst O, Eckardt KU, Gansevoort RT, Harris T, Horie S, Kasiske BL, Odland D, Pei Y, Perrone RD, Pirson Y. Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney international. 2015 Jul 1;88(1):17-27.\u003c/li\u003e\n \u003cli\u003eBergmann C. ARPKD and early manifestations of ADPKD: the original polycystic kidney disease and phenocopies. Pediatric Nephrology. 2015 Jan;30(1):15-30.\u003c/li\u003e\n \u003cli\u003eYin X, Blumenfeld JD, Riyahi S, Luo X, Rennert H, Barash I, Prince MR. Prevalence of inferior vena cava compression in ADPKD. Kidney International Reports. 2021 Jan 1;6(1):168-78.\u003c/li\u003e\n \u003cli\u003eIguchi S, Kasai A, Kishimoto H, Suzuki K, Ito S, Ogawa Y, Nishi S, Gejyo F, Ohno Y. Thrombosis in inferior vena cava (IVC) due to intra-cystic hemorrhage into a hepatic local cyst with autosomal dominant polycystic kidney disease (ADPKD). Internal medicine. 2004;43(3):209-12.\u003c/li\u003e\n \u003cli\u003eWilley C, Kamat S, Stellhorn R, Blais J. Analysis of nationwide data to determine the incidence and diagnosed prevalence of autosomal dominant polycystic kidney disease in the USA: 2013\u0026ndash;2015. Kidney Diseases. 2019 Jan 9;5(2):107-17.\u003c/li\u003e\n \u003cli\u003eSutter M, Germino GG. Autosomal dominant polycystic kidney disease: molecular genetics and pathophysiology. Journal of Laboratory and Clinical Medicine. 2003 Feb 1;141(2):91-101.\u003c/li\u003e\n \u003cli\u003eBergmann C, Guay-Woodford LM, Harris PC, Horie S, Peters DJ, Torres VE. Polycystic kidney disease. Nature reviews Disease primers. 2018 Dec 6;4(1):50.\u003c/li\u003e\n \u003cli\u003eFujimaru T, Mori T, Sekine A, Mandai S, Chiga M, Kikuchi H, Ando F, Mori Y, Nomura N, Iimori S, Naito S. Kidney enlargement and multiple liver cyst formation implicate mutations in PKD1/2 in adult sporadic polycystic kidney disease. Clinical genetics. 2018 Jul;94(1):125-31.\u003c/li\u003e\n \u003cli\u003eYugueros X, Alvarez B, Fern\u0026aacute;ndez E, Boqu\u0026eacute; M, Matas M. Compressive symptoms due to thrombosed or hypertrophic collateral circulation in infrarenal inferior vena cava agenesis. Annals of Vascular Surgery. 2013 Feb 1;27(2):238-e9.\u003c/li\u003e\n \u003cli\u003eFuller TF, Brennan TV, Feng S, Kang SM, Stock PG, Freise CE. End stage polycystic kidney disease: indications and timing of native nephrectomy relative to kidney transplantation. The Journal of urology. 2005 Dec 1;174(6):2284-8.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"ADPKD, IVC compression, nephrectomy","lastPublishedDoi":"10.21203/rs.3.rs-5405666/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-5405666/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eAdult polycystic kidney disease (ADPKD) is a multi system genetic disorder characterized by the development and progressive enlargement of fluid-filled cysts in both kidneys, along with other organs. As one of the main causes of kidney failure, ADPKD can progress to end-stage renal disease (ESRD), with over 50% of affected individuals progressing to ESRD by age 50. The symptoms in ADPKD are variable, with some patients experiencing nonspecific signs, while others present with symptoms related to the mass effect of enlarged kidneys on surrounding structures. This case report highlights an unusual presentation of ADPKD in a patient who developed symptoms of inferior vena cava (IVC) compression. Remarkably, these symptoms improved after bilateral nephrectomies, suggesting that bilateral nephrectomy would provide help in these situations.\u003c/p\u003e","manuscriptTitle":"A challenging case of IVC compression in an adult polycystic patient. A case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-12-03 14:53:38","doi":"10.21203/rs.3.rs-5405666/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"636194d0-e667-422d-9290-f36c83ed9338","owner":[],"postedDate":"December 3rd, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-12-03T14:53:38+00:00","versionOfRecord":[],"versionCreatedAt":"2024-12-03 14:53:38","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-5405666","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-5405666","identity":"rs-5405666","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}

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