Laparoscopic Splenectomy for a Patient with Splenomegaly and Hypersplenism due to Idiopathic Portal Hypertension

Idiopathic portal hypertension (IPH) is a rare disorder which is clinically characterized by portal hypertension, splenomegaly, hypersplenism and the absence of liver cirrhosis. Patients with IPH have massive splenomegaly leading to increased portal venous flow and subsequent portal hypertension. In selected IPH patients with splenomegaly and hypersplenism, splenectomy can be regarded as an effective treatment protocol for decreasing portal hypertension. We report a case of a 44-year-old woman who was diagnosed with IPH accompanied by splenomegaly and hypersplenism. She underwent laparoscopic splenectomy and clinical symptoms and hypersplenism resolved. Our study shows that laparoscopic splenectomy can be considered as a procedure for treating patients with splenomegaly and hypersplenism due to IPH.

Research advances in non-cirrhotic portal hypertension / 临床肝胆病杂志

Although liver cirrhosis is the most common cause of portal hypertension (PH), about 20% of PH cases are caused by non-cirrhotic reasons, which are referred to as non-cirrhotic portal hypertension (NCPH), with a high incidence rate in developing countries. NCPH is a group of heterogeneous hepatic vascular diseases, including idiopathic portal hypertension (IPH) and extrahepatic portal vein obstruction (EHPVO), as well as the rare diseases in clinical practice such as Budd-Chiari syndrome, congenital hepatic fibrosis, and nodular regenerative hyperplasia. The patients with NCPH usually have the symptoms of portal hypertension, such as recurrent variceal bleeding and splenomegaly, but liver function is well preserved in these patients. At present, the diagnosis of NCPH lacks a universally accepted standard and remains a challenge. In clinical practice, the method of exclusion is usually applied for the diagnosis of HCPH, and liver biopsy is performed when necessary to make a confirmed diagnosis. This paper introduces the pathogenesis and pathological manifestations of IPH and EHPVO, as well as the selection of diagnostic methods and therapeutic strategies. If upper gastrointestinal bleeding can be effectively controlled, NCPH is considered to have a relatively good prognosis.

Recurrent portal and superior mesenteric vein thrombosis in a patient with idiopathic portal hypertension / 대한내과학회지

Idiopathic portal hypertension (IPH) is characterized by portal hypertension and splenomegaly without portal vein obstruction or significant liver disease. Although IPH may occasionally be accompanied by portal vein thrombosis (PVT) and extrahepatic portal vein thrombosis (EHPVT), recurrent PVT and EHPVT are very rare in IPH. Herein, we report the case of a 30-year-old male who developed IPH with recurrent PVT and EHPVT. Eleven years earlier, the patient had undergone splenectomy and endoscopic sclerotherapy due to hypersplenism and esophageal variceal bleeding, respectively. Ten years earlier, the patient had suffered recurrent esophageal variceal bleeding, which was treated via band ligation, and was diagnosed with IPH via portography and liver biopsy. Then, 8 years prior to presentation, the patient complained of acute abdominal pain and was diagnosed with PVT and EHPVT. After a 6-month course of anticoagulation therapy, the PVT and EHPVT resolved completely. However, 8 years later, he complained again of abdominal pain and was diagnosed with recurrent PVT and EHPVT.

Clinicopathological Analysis of Eight Cases of Idiopathic Portal Hypertension

BACKGROUND: Idiopathic portal hypertension (IPH) is a rare clinicopathologic entity that shows clinical evidences of portal hypertension with no pathologic features of cirrhosis. METHODS: The clinical and pathologic features of 8 cases with IPH were analyzed via the medical records along with the biopsy or resected liver specimens. RESULTS: Six patients were male and two were female. The chief complaints were sudden variceal bleeding in seven patients and abdominal pain in one patient. Six patients were treated with varix ligation and one was treated with splenectomy after the failure of bleeding control. One patient underwent a liver transplantation due to severe symptoms of portal hypertension. The prognosis of all the patients was excellent. Microscopically, the portal tracts were variably fibrotic, and the portal veins in them were sclerotic, obliterated or dilated in 7 cases; pathologic abnormalities were absent in 1 case. Cirrhosis was absent in all cases, while septal fibrosis was present in one resected liver. CONCLUSIONS: IPH is a minor cause of portal hypertension. However, a liver biopsy to show the subtle portal vascular changes and fibrosis in patients who have the clinical symptoms of portal hypertension is important for making the diagnosis of IPH.

Diagnosis and treatment of on-cirrhotic portal hypertension / 中国实用内科杂志

Non-cirrhotic portal hypertension(NCPH)is a group of diseases that show evidences of portal hypertension but no cirrhosis is present.Common causes of NCPH include pre-sinusoidal portal lesions such as portal vein thrombosis,congenital liver fibrosis and idiopathic portal hypertension,and post-sinusoidal portal lesions.The major feature of this group of diseases is well preserved liver function in spite of prominent portal hypertensive manifestations such as esophageal varices/gastrointestinal bleeding and splenomegaly/hypersplenism.Careful differentiation from cirrhosis requires thorough clinical,radiological and pathological investigation.Preventing and control of variceal bleeding and hypersplenism through medical,endoscopic and interventional procedures yield good prognosis in most of the patients with NCPH.

A Case of Bleeding Duodenal Varices in a Patient with Idiopathic Portal Hypertension / 대한소화기내시경학회지

Bleeding duodenal varices are a rare complication in patients with portal hypertension. Cirrhosis followed by portal vein obstruction and splenic vein obstruction are the most common causes. Although the prognosis of bleeding duodenal varices is usually poor, an awareness of its characteristic presentation may enable diagnostic and therapeutic proce- dures to be performed rapidly with an increased likelihood of a reaching successful out- come. In this study, we report a case of bleeding duodenal varices in a 23-year-old woman with idiopathic portal hypertension who was also suffering with recurrent melena. Panendoscopy identified prominant tortuous varices with central erosion in the 3rd portion of the duodenum and no esophageal and gastric varices. The varices were successfully treated by distal splenorenal shunt.