Left-sided portal hypertension caused by idiopathic splenic vein stenosis improved by splenectomy: a case report

BACKGROUND: Splenic vein stenosis and occlusion, which are known causes of left-sided portal hypertension, often occur secondary to trauma, pancreatitis, or invasion or compression by pancreatic tumors. However, few reports have described idiopathic splenic vein stenosis. CASE PRESENTATION: A 70-year-old man was referred to our hospital for examination of isolated gastric varices. He had no history of liver disease, pancreatitis, or abdominal trauma. Computed tomography revealed stenosis of almost the entire length of the splenic vein, and development of gastric fundal and short gastric varices. No inflammatory changes or neoplastic lesions of the pancreas were observed in any imaging study. The patient was diagnosed with left-sided portal hypertension caused by idiopathic splenic vein stenosis, and splenectomy was performed. The postoperative course was smooth, and improvement of the gastric varices was shown by upper gastrointestinal endoscopy at 3 months after the operation. CONCLUSIONS: Idiopathic splenic vein stenosis is an extremely rare cause of left-sided portal hypertension. Splenectomy is one of the most effective treatments for left-sided portal hypertension caused by idiopathic splenic vein stenosis.