Introduction
Budd–Chiari Syndrome (BCS) is a rare disorder typically acquired rather
than congenital. It is characterized by an obstruction within the
hepatic venous outflow tract, occurring anywhere from the terminal
hepatic veins to the confluence of the inferior vena cava (IVC) and the
right atrium. This obstruction leads to hepatic dysfunction. The
diagnosis of primary BCS is established when obstruction primarily
results from a vascular pathology, including thrombosis or phlebitis.
Conversely, secondary BCS is identified when external compression or
invasion of the hepatic veins and/or inferior vena cava (IVC) is induced
by a lesion, such as malignancy 1,2.
George Budd is credited with the initial delineation of a clinical
syndrome characterized by the occlusion of hepatic veins, BCS, which was
documented in 1846 3. Primary myeloproliferative
diseases stand out as the predominant causative factor for the onset of
BCS. Clinically discernible disease manifestation requires the occlusion
of at least two hepatic veins 4.
The majority of BCS patients present with the classic triad of abdominal
pain in the upper right quadrant presenting in about 61% of cases,
hepatomegaly presenting in 67% of cases and ascites presenting in 83%
of cases 5,6. Advanced BCS leads to the development of
portal hypertension and liver failure, accompanied by complications such
as encephalopathy and hematemesis 7. BCS should be
considered in both symptomatic and asymptomatic individuals with acute
or chronic liver disease. Recent guidelines emphasize the crucial role
of non-invasive imaging for early BCS identification and assessment,
which include Doppler ultrasound, Magnetic resonance imaging (MRI), and
computed tomography (CT) 1,8.
Survival rates in patients with Budd–Chiari syndrome have significantly
improved due to advancements in endoscopic technology, Transjugular
Intrahepatic Portosystemic Shunt (TIPSS), and liver transplantation,
with reported survival rates reaching 96%9. However,
a large proportion of cases go undiagnosed due to nonspecific symptoms
and delayed diagnosis.
Adrenal insufficiency poses a life-threatening risk arising from either
primary adrenal failure or secondary adrenal disease, often attributed
to dysfunction in the hypothalamic-pituitary axis. This condition
manifests clinically as the inadequate production or effectiveness of
glucocorticoids, sometimes accompanied by a deficiency in
mineralocorticoids and adrenal androgens. Hydrocortisone serves as the
cornerstone in the therapeutic approach for addressing adrenal
insufficiency. It is also documented to be associated with a
procoagulant state leading to an increased risk of venous thrombosis and
thrombo-embolism 10,11.
Herein, we present a unique instance of Budd-Chiari syndrome occurring
in a long-term hydrocortisone user with Addison’s insufficiency, marking
the first documented case of its kind.
This case report is in line with the CARE guidelines for case reports12. The patient provided all the data used for the
case report and consent to the study.