Pregnancy Outcome in Patients with Budd Chiari Syndrome: A Single Centre Experience

Abstract Background Budd-Chiari syndrome (BCS) is a rare disease caused by hepatic venous outflow obstruction and results in hepatic failure due to sinusoidal congestion, decrease in the blood supply to the to the liver and increase the pressure in portal vein . Objectives The aim beyond this study is assessment of the maternal and foetal events in a group of women who became pregnant while having BCS. Patients and methods It is a prospective and retrospective study which was conducted on 32 patients with Budd- Chiari syndrome admitted to Ain Shams University Hospitals, Tropical Medicine department or attended the outpatient clinics during the period from May 2006 to March 2019 who became pregnant after diagnosis by BCS . Results Number of cases that underwent tips revision 3 cases and one case underwent liver transplantation after delivery and 2 cases had died during follow up.Using . of enoxaparin in 1st trimester in our cases as a significant protective factor against abortion. Conclusion BCS isn’t a contraindication to pregnancy in patients with a well-controlled disease. Fetal outcome remains relatively poor, which is at least partly due to the underlying prothrombotic disorders. so patients should be accurately informed about the risk of this pregnancies .

Download Full-text

TIPS for acute and chronic Budd–Chiari syndrome: a single-centre experience

Journal of Hepatology ◽

10.1016/s0168-8278(03)00118-1 ◽

2003 ◽

Vol 38 (6) ◽

pp. 751-754 ◽

Cited By ~ 100

Author(s):

Andrea Mancuso ◽

Konrad Fung ◽

Maria Mela ◽

John Tibballs ◽

Anthony Watkinson ◽

...

Keyword(s):

Budd Chiari Syndrome ◽

Single Centre ◽

Single Centre Experience ◽

Chiari Syndrome

Download Full-text

The Spectrum of Histologic Findings in Hepatic Outflow Obstruction

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2015-0388-oa ◽

2016 ◽

Vol 141 (1) ◽

pp. 98-103 ◽

Cited By ~ 7

Author(s):

Raul S. Gonzalez ◽

Michael A. Gilger ◽

Won Jae Huh ◽

Mary Kay Washington

Keyword(s):

Portal Tract ◽

Central Vein ◽

Budd Chiari Syndrome ◽

Outflow Obstruction ◽

Venous Outflow ◽

Ductular Reaction ◽

Chiari Syndrome ◽

Hepatic Venous Outflow ◽

Venous Outflow Obstruction ◽

Hematoxylin And Eosin

Context.—Cardiac hepatopathy and Budd-Chiari syndrome are 2 forms of hepatic venous outflow obstruction with different pathophysiology but overlapping histologic findings, including sinusoidal dilation and centrilobular necrosis. Objective.—To determine whether a constellation of morphologic findings could help distinguish between the 2 and could suggest the diagnoses in previously undiagnosed patients. Design.—We identified 26 specimens with a diagnosis of cardiac hepatopathy and 23 with a diagnosis of Budd-Chiari syndrome. Slides stained with hematoxylin and eosin and with trichrome were evaluated for several distinctive histologic findings. Results.—Features common to both forms of hepatic outflow obstruction included sinusoidal dilation and portal tract changes of fibrosis, chronic inflammation, and bile ductular reaction. Histologic findings significantly more common in cardiac hepatopathy included pericellular/sinusoidal fibrosis and fibrosis around the central vein. Only centrilobular hepatocyte dropout/necrosis was significantly more common in Budd-Chiari, regardless of duration. Conclusions.—The finding of pericellular/sinusoidal fibrosis in cardiac hepatopathy compared with Budd-Chiari is not unexpected, given the chronic nature of most cardiac hepatopathy. Portal tract changes are common in both forms of hepatic outflow obstruction and should not deter one from making the diagnosis of hepatic outflow obstruction. Fibrosis along sinusoids and around the central vein may be suggestive of cardiac hepatopathy in biopsies from patients without a prior diagnosis.

Download Full-text

Hepatic venous outflow obstruction in piggyback liver transplantation: single centre experience

ANZ Journal of Surgery ◽

10.1111/ans.13344 ◽

2015 ◽

Vol 87 (3) ◽

pp. 182-185 ◽

Cited By ~ 1

Author(s):

Joel Arudchelvam ◽

Adam Bartlett ◽

John McCall ◽

Peter Johnston ◽

Edward Gane ◽

...

Keyword(s):

Liver Transplantation ◽

Single Centre ◽

Outflow Obstruction ◽

Venous Outflow ◽

Hepatic Venous Outflow Obstruction ◽

Single Centre Experience ◽

Hepatic Venous Outflow ◽

Venous Outflow Obstruction

Download Full-text

Polycythemia vera-geïnduceerd syndroom van Budd-Chiari bij een oudere patiënte

Tijdschrift voor Geneeskunde ◽

10.47671/tvg.77.21.007 ◽

2021 ◽

Author(s):

S. VAN DESSEL ◽

W. LALEMAN ◽

E. GIELEN

Keyword(s):

Polycythemia Vera ◽

Myeloproliferative Neoplasms ◽

Laboratory Data ◽

Jak2 V617f ◽

Budd Chiari Syndrome ◽

Older Patient ◽

Aged Patients ◽

Chiari Syndrome ◽

Hepatic Venous Outflow ◽

Venous Outflow Obstruction

Polycythemia vera-induced Budd-Chiari syndrome in an older patient The case of a 94-year-old patient with subacute Budd-Chiari syndrome (BCS) caused by a novel diagnosis of polycythemia vera (PV) is reported. BCS is mostly seen in young or middle-aged patients. The presentation in a nonagenarian is rare, making this case exceptional. BCS is defined by a hepatic venous outflow obstruction. Its clinical presentation is variable from fulminant liver failure to an insidious form with symptoms of cirrhosis at the time of the diagnosis. In western countries, primary BCS is mainly seen, which is caused by an endoluminal lesion. A hypercoagulable state provoked by myeloproliferative neoplasms (MPN) is mostly responsible. The patient presented with abdominal distention and anorexia since two months. Physical examination revealed hepatomegaly and ascites. Laboratory data indicated polycythemia and cholestasis. The CT scan of the abdomen was diagnostic for subacute BCS. A JAK2-V617F mutation was found. The therapy consisted of anticoagulation, low-dose acetylsalicylic acid, phlebotomies and supportive care with diuretics and paracentesis.

Download Full-text

Classification of Hepatic Venous Outflow Obstruction: Ambiguous Terminology of the Budd-Chiari Syndrome

Mayo Clinic Proceedings ◽

10.1016/s0025-6196(12)62109-0 ◽

1990 ◽

Vol 65 (1) ◽

pp. 51-55 ◽

Cited By ~ 144

Author(s):

JURGEN LUDWIG ◽

ETSUKO HASHIMOTO ◽

DOUGLAS B. McGILL ◽

JON A. van HEERDEN

Keyword(s):

Budd Chiari Syndrome ◽

Outflow Obstruction ◽

Venous Outflow ◽

Hepatic Venous Outflow Obstruction ◽

Chiari Syndrome ◽

Hepatic Venous Outflow ◽

Venous Outflow Obstruction

Download Full-text

Management of hepatic venous outflow obstruction (Budd Chiari Syndrome): Balloon angioplasty with or without the use of a stent

Catheterization and Cardiovascular Diagnosis ◽

10.1002/(sici)1097-0304(199710)42:2<240::aid-ccd39>3.0.co;2-p ◽

1997 ◽

Vol 42 (2) ◽

pp. 240-240 ◽

Cited By ~ 1

Author(s):

Upendra Kaul ◽

Rajiv Agarwal

Keyword(s):

Balloon Angioplasty ◽

Budd Chiari Syndrome ◽

Outflow Obstruction ◽

Venous Outflow ◽

Hepatic Venous Outflow Obstruction ◽

Chiari Syndrome ◽

Hepatic Venous Outflow ◽

Venous Outflow Obstruction

Download Full-text

Budd-Chiari Syndrome – A review of the diagnosis and management

Acute Medicine Journal ◽

10.52964/amja.0439 ◽

2011 ◽

Vol 10 (1) ◽

pp. 5-9

Author(s):

MA Fox ◽

◽

JA Fox ◽

MH Davies ◽

◽

...

Keyword(s):

Liver Disease ◽

Abdominal Discomfort ◽

Budd Chiari Syndrome ◽

Outflow Obstruction ◽

Venous Outflow ◽

Hepatic Venous Outflow Obstruction ◽

Key Points ◽

Chiari Syndrome ◽

Hepatic Venous Outflow ◽

Venous Outflow Obstruction

Budd-Chiari syndrome (BCS) is the liver disease resulting from hepatic venous outflow obstruction comprising a triad of abdominal discomfort, hepatomegaly and ascites. Advances in the management of this disorder over the last three decades have dramatically improved survival. We present a review of the management of BCS followed by a case which illustrates some key points in the diagnosis and treatment of this condition.

Download Full-text