Boudhina–Yedes–Khiari syndrome

Boudhina–Yedes–Khiari syndrome
Synonyms
Pronounce	N/A
Specialty	Gastroenterology, Hepatology
Symptoms	Portal hypertension, Esophageal varices, Splenomegaly
Complications	Liver cirrhosis, Hepatic encephalopathy
Onset
Duration
Types	N/A
Causes	Portal vein thrombosis
Risks
Diagnosis	Ultrasound, CT scan, MRI
Differential diagnosis	N/A
Prevention	N/A
Treatment	Anticoagulation, Surgical shunt
Medication	N/A
Prognosis
Frequency	Rare
Deaths	N/A

Boudhina–Yedes–Khiari syndrome is a rare medical condition characterized by portal hypertension due to portal vein thrombosis. This syndrome is named after the researchers who first described it. It is a significant cause of non-cirrhotic portal hypertension, particularly in regions where Budd–Chiari syndrome is less prevalent.

Pathophysiology

The primary feature of Boudhina–Yedes–Khiari syndrome is the obstruction of the portal vein, which leads to increased pressure in the portal venous system. This increased pressure can cause the development of esophageal varices, splenomegaly, and other complications associated with portal hypertension. The obstruction is typically due to thrombosis, which may be related to underlying prothrombotic conditions.

Clinical Presentation

Patients with Boudhina–Yedes–Khiari syndrome often present with symptoms related to portal hypertension. These may include:

• Abdominal pain
• Ascites
• Gastrointestinal bleeding from esophageal varices
• Splenomegaly

Diagnosis

The diagnosis of Boudhina–Yedes–Khiari syndrome is primarily based on imaging studies. Ultrasound with Doppler can reveal the absence of flow in the portal vein, while CT scan and MRI can provide detailed images of the portal venous system and identify any thrombotic occlusions.

Treatment

Management of Boudhina–Yedes–Khiari syndrome focuses on addressing the underlying thrombosis and managing complications of portal hypertension. Treatment options include:

• Anticoagulation therapy to prevent further thrombus formation
• Surgical shunt procedures to decompress the portal venous system
• Endoscopic treatment of esophageal varices to prevent bleeding

Prognosis

The prognosis of Boudhina–Yedes–Khiari syndrome depends on the severity of portal hypertension and the success of treatment interventions. Early diagnosis and management are crucial to prevent complications such as liver cirrhosis and hepatic encephalopathy.

Also see

• Portal hypertension
• Budd–Chiari syndrome
• Esophageal varices
• Splenomegaly