Budd-Chiari Syndrome following Pretransplant Mononephrectomy in an Autosomal Dominant Polycystic Kidney Disease Patient with Liver Cysts

Dionisio, P; Sessa, A.; Conte, Ferruccio; Bergia, R; Stramignoni, E; Cravero, Raffaella; Caramello, E; Bajardi, P

doi:10.1159/000189513

Cited by 8 publications

(7 citation statements)

References 6 publications

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“…On the other hand, unilateral nephrectomy seems to be safer with less bleeding and infectious complications [9]. Careful evaluation to select the best surgical approach is therefore necessary because secondary BCS has also been described after left unilateral nephrectomy in AD-PKD patients [10]. Despite being a rare event, secondary BCS should be considered as a differential diagnosis in kidney transplant recipients with edema and acute kidney injury after nephrectomy of polycystic kidneys.…”

Section: Bcs Management Depends On Etiology and Severitymentioning

confidence: 99%

Budd-Chiari Syndrome after Bilateral Nephrectomy for Polycystic Kidney Disease in a Kidney Transplant Recipient

et al. 2020

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We report a rare case of Budd-Chiari syndrome (BCS) as a postoperative complication after bilateral nephrectomy in a kidney transplant recipient with polycystic liver and kidneys. Contrast-enhanced computed tomography of the abdomen showed a narrowed inferior vena cava, compressed by the polycystic liver that moved downwards after nephrectomy. A stenting angioplasty was performed, resulting in remarkable clinical improvement. This case highlights the need for careful evaluation of polycystic kidneys and their anatomic relationship with the liver before nephrectomy, as well as for considering BCS as a differential diagnosis in similar cases.

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Section: Bcs Management Depends On Etiology and Severitymentioning

confidence: 99%

Budd-Chiari Syndrome after Bilateral Nephrectomy for Polycystic Kidney Disease in a Kidney Transplant Recipient

et al. 2020

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“…12 Besides liver volume, another important risk factor for the occurrence of ascites is abdominal surgery such as liver resections, laparoscopic fenestration or nephrectomy. 15,[21][22][23][24] For most patients, post-operative ascites is transient and usually responds to medical management with diuretics, low salt diet and repeat paracenteses. 14 After liver resection, 42% of patients had post-operative ascites.…”

Section: Clinical Symptoms Of Portal Hypertension In Pldmentioning

confidence: 99%

“…1 Case reports have highlighted HVOO and refractory ascites as a complication of nephrectomy in ADPKD patients. 15,21,22 In this respect, it is relevant to weigh the risks and benefits of nephrectomy, as the merits of nephrectomy and patient selection are uncertain. 29 A 2015 guideline suggests that polycystic kidneys should not be routinely removed prior to transplantation, as it is associated with significant morbidity and mortality.…”

Section: Clinical Symptoms Of Portal Hypertension In Pldmentioning

confidence: 99%

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Management of portal hypertension and ascites in polycystic liver disease

Bernts

Drenth

Tjwa

2019

Liver International

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Patients suffering from polycystic liver disease may develop Hepatic Venous Outflow Obstruction, Portal Vein Obstruction and/or Inferior Caval Vein Syndrome because of cystic mass effect. This can cause portal hypertension, leading to ascites, variceal haemorrhage or splenomegaly. For this review, we evaluate the evidence to provide clinical guidance for physicians faced with this complication. Diagnosis is made with imaging such as ultrasound, computed tomography or magnetic resonance imaging. Therapy includes conventional therapy with diuretics and paracentesis, and medical therapy using somatostatin analogues. Based on disease phenotype various (non‐)surgical liver‐volume reducing therapies, hepatic or portal venous stenting, transjugular intrahepatic portosystemic shunts and liver transplantation may be considered. Because of complicated anatomy, use of high‐risk interventions and lack of empirical evidence, patients should be treated in expert centres.

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“…Huge liver cysts may obstruct hepatic venous outfl ow and cause the Budd-Chiari syndrome (Torres et al 1994). Several cases have been reported following bilateral nephrectomy in ADPKD patients (Torres et al 1994;Dioniso et al 1995;Dionisio et al 1997). Sclerotherapy with alcohol, resection or fenestration of the hepatic cyst wall may be helpful with hepatic venous occlusion caused by solitary enlarged cysts.…”

Section: Liver Cystsmentioning

confidence: 99%

Developments in the management of autosomal dominant polycystic kidney disease

Schrier¹

2008

TCRM

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Abstract:Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent life-threatening, hereditary disease. ADPKD is more common than sickle cell anemia, cystic fi brosis, muscular dystrophy, hemophilia, Down's syndrome, and Huntington's disease combined. ADPKD is a multisystemic disorder characterized by the progressive development of renal cysts and marked renal enlargement. Structural and functional renal deterioration occurs in ADPKD patients and is the fourth leading cause of end-stage renal disease (ESRD) in adults. Aside from the renal manifestations, extrarenal structural abnormalities, such as liver cysts, cardiovascular abnormalities, and intracranial aneurysms may lead to morbidity and mortality. Recent studies have identifi ed prognostic factors for progressive renal impairment including gender, race, age, proteinuria, hematuria, hypertension and increased left ventricular mass index (LVMI). Early diagnosis and better understanding of the pathophysiology of the disease provides the opportunity to aggressivly treat hypertension with renin-angiotensin-aldosterone system inhibitors and thereby potentially reduce LVMI, prevent cardiovascular morbidity and mortality and slow progression of the renal disease.

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Budd-Chiari Syndrome following Pretransplant Mononephrectomy in an Autosomal Dominant Polycystic Kidney Disease Patient with Liver Cysts

Cited by 8 publications

References 6 publications

Budd-Chiari Syndrome after Bilateral Nephrectomy for Polycystic Kidney Disease in a Kidney Transplant Recipient

Budd-Chiari Syndrome after Bilateral Nephrectomy for Polycystic Kidney Disease in a Kidney Transplant Recipient

Management of portal hypertension and ascites in polycystic liver disease

Developments in the management of autosomal dominant polycystic kidney disease

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