Rare Case of Budd-Chiari Syndrome in a Young Child: A Diagnostic Conundrum

Timsaal, Yumna; Ali, Syed; Malik, Farheen; Chawla, Ashok; Ahmed, Jawad

doi:10.7759/cureus.16407

Cited by 2 publications

(4 citation statements)

References 16 publications

(22 reference statements)

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“…Medical Science 27, e181ms2838 (2023) 6 of 9 The causes of Budd-Chiari syndrome are inherited and acquired state of hypercoagulable. Inherited causes such as protein C, S deficiency, factor V Leiden mutation and anti-thrombin III deficiency are the common causes of hepatic vein thrombosis which results in BCS (Timsaal et al, 2021). Acquired causes such as polycythaemia Vera, paroxysmal nocturnal haemoglobinuria and myelofibrosis, accounts for >50% of BCS cases.…”

Section: Case Report | Open Accessmentioning

confidence: 99%

Rare case of Budd-Chiari Syndrome with recurrent thrombosis requiring stenting

Myadam¹,

Meshram²,

Taksande³

2023

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Budd-Chiari syndrome is a very rare disorder in paediatric age group, which is characterised by narrowing or obstruction of hepatic venous outflow. The obstruction might be thrombotic or non-thrombotic along the course of hepatic venules to junction of inferior vena cava to the right atrium, which leads to abdominal pain in right hypochondrium, hepatomegaly, ascites and portal hypertension. The cause of venous obstruction in children is mainly due to hypercoagulable state. The mean age of Budd-Chiari syndrome is 20-40 years, but here we can see that it occurs in paediatric age group, so good clinical suspicion is required along with investigation to confirm the diagnosis. Traditional approach to the treatment of Budd-Chiari syndrome is systemic thrombolysis or surgical Porto systemic shunt. Recently, successful treatment of Budd-Chiari syndrome is done by endovascular techniques, including angioplasty and stent placement. Here, in this case it shows a successful percutaneous recanalization of complete hepatic vein occlusion by angioplasty and stent placement.

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Section: Case Report | Open Accessmentioning

confidence: 99%

Rare case of Budd-Chiari Syndrome with recurrent thrombosis requiring stenting

Myadam¹,

Meshram²,

Taksande³

2023

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“…

Budd-Chiari syndrome (BCS) is an infrequent occurrence, predominantly found in south-eastern Asia with an incidence of one per one million across the world annually. 1,2 Primary BCS is associated with hepatic venous outflow tract obstruction due to thrombosis along the course of hepatic veins to the junction of inferior vena cava (IVC) with right atrium, clinically presenting acutely with ascites, hepatomegaly and right upper quadrant pain, and chronically with symptoms of portal hypertension and liver failure, which is more common. Studies have reported genetic predispositions causing thrombophilia in such patients.

…”

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confidence: 99%

“…Studies have reported genetic predispositions causing thrombophilia in such patients. 1,2 Early diagnosis can be done by a Doppler study and liver biopsy to prevent chronic complications of portal hypertension, and progression to hepatic fibrosis and cirrhosis.Management is spearheaded by using non-invasive methods like oral anticoagulants, followed by radiological interventions (stenting/transjugular intrahepatic…”

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confidence: 99%

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A rare presentation of chronic Budd Chiari syndrome in a 13 years old female

Bharmal,

Mital,

Sharma

et al. 2023

Int J Res Med Sci

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These days pathologies related to liver are increasing due to one or other factors. Here, we review a case of a girl with Budd Chiari syndrome (BCS), which is due to obstruction of hepatic veins, in turn causing portal hypertension. The pathology of the syndrome includes hepatic venous outflow tract obstruction due to thrombosis along the vein. This in turn leads to portal hypertension and increased pressure in the inferior vena cava (IVC). Clinically, it affects the heart, liver, esophagus, rectum, superficial skin. Early diagnosis can be done by Doppler and liver biopsy to prevent chronic complications of hepatic fibrosis and cirrhosis. The patient was a 13-year-old girl who had abdominal distension, pedal edema, discoloured and decreased urine and fever. On examining the patient, we found out stunted linear and intellectual growth as per her age, low IQ and communication deficits. On ultrasonography, a nodular liver surface with rounded edges and hypoechoic nodules within the parenchyma were seen. Biochemical investigations showed increase in liver enzymes. Also, there was free fluid in abdomen, few anechoic channels in periumbilical and perisplenic regions showing color flow on Doppler. Ascitic tap showed a high total cell count with a lymphocytic predominance. Based on history by the patient, radiological findings, Ascitic tap and biochemical investigations we have come to the conclusion that this is a case of BCS. After being admitted, we gave antibiotics, diuretic, folic acid, albumin, lactulose. Patient was discharged on propanolol as prophylaxis for portal hypertension.

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Rare Case of Budd-Chiari Syndrome in a Young Child: A Diagnostic Conundrum

Cited by 2 publications

References 16 publications

Rare case of Budd-Chiari Syndrome with recurrent thrombosis requiring stenting

Rare case of Budd-Chiari Syndrome with recurrent thrombosis requiring stenting

A rare presentation of chronic Budd Chiari syndrome in a 13 years old female

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