Budd-Chiari syndrome associated with nodular regenerative hyperplasia of the liver

The Budd-Chiari syndrome due to membranous obstruction of the hepatic blood outflow tract is a rare condition in western countries, and its association with nodular regenerative hyperplasia of the liver has never been described. We present the case of a 34-year-old woman with membranous obstruction of hepatic veins and nodular regenerative hyperplasia of the liver. Although webs have been difficult to demonstrate by sonography, we were able to image a structure in the hepatic vein near the junction with the inferior vena cava, suggesting a membranous nature.     

Epithelioid hemangioendothelioma associated with nodular regenerative hyperplasia

Epithelioid hemangioendothelioma of the liver is a rare neoplasm of vascular origin. We report a case of epithelioid hemangioendothelioma occurring in a patient with nodular regenerative hyperplasia. This association suggests that some hepatic vascular changes might promote the growth of epithelioid hemangioendothelioma.     

The pathogenesis of focal nodular hyperplasia of the liver

Abstract   The pathogenesis of focal nodular hyperplasia is poorly understood. The lesion has been reported adjacent to many other focal hepatic lesions suggesting this is a nonspecific reaction to injury. Recent evidence suggests that arterio-venous shunt formation may trigger a positive feedback loop that includes hepatocellular hyperplasia.     

Oesophageal variceal bleeding in Felty''s syndrome associated with nodular regenerative hyperplasia.

Four patients with Felty's syndrome developed massive upper gastrointestinal bleeding due to oesophageal varices. The underlying hepatic pathology in all 4 was nodular regenerative hyperplasia. This appears to be a difficult histological diagnosis to make, having been initially reported as normal on percutaneous biopsy or as fibrosis or cirrhosis on wedge biopsy. This series brings the total number of cases reported in the English literature of this association to 12, suggesting a definite symptom complex. The portal hypertension seems to be due to a combination of increased splenic blood flow and postsinusoidal resistance. The clinical importance of this syndrome is that the appropriate therapy for bleeding oesophageal varices appears to be shunt procedure such as a splenorenal shunt with splenectomy, which should be well tolerated.     

Nodular regenerative hyperplasia and focal nodular hyperplasia of the liver associated with severe cholestasis. Report of a case with autopsy findings

Nodular regenerative hyperplasia of the liver was found at liver biopsy and at autopsy in a 61-year-old woman with severe cholestasis. Increased bilirubin levels persisted over a follow-up period of 4 years. Microscopic findings included hyperplastic hepatocytic nodules, interlobular bile duct lesions, fibrous medial and intimal thickening of small arterial branches and portal veins, and recanalization of a thrombus in a major portal branch related to an area of focal nodular hyperplasia of the liver. Bile duct and small vessel changes were prominent in the present case. Their role as contributing factors to the development of nodular regenerative hyperplasia of the liver is discussed.     

Nodular regenerative hyperplasia of the liver associated with macroglobulinemia: A clue to the pathogenesis

Nodular regenerative hyperplasia of the liver is an infrequent condition characterized by transformation of the hepatic parenchyma into nodules with only mild fibrosis. Little is known about the etiology except that there is usually an underlying chronic disease, such as Felly's syndrome, which antedates the development of clinical liver disease. It is poorly understood how the associated diseases contribute to the pathogenesis of nodular regenerative hyperplasia. Presented are four cases of nodular regenerative hyperplasia in which macroglobulinemia was also present. This new association suggests to us a hypothesis for the pathogenesis of nodular regenerative hyperplasia.

Histologic examination of the livers in these and other cases of nodular regenerative hyperplasia reveals widespread obliteration of the small portal veins. Postmortem angiography of one liver in the present series demonstrated that the nodules were well perfused and that the atrophic areas were poorly perfused with portal blood. This supports the view that atrophy of lobules results from a lack of portal blood supply and that nodules develop from lobules well supplied with portal blood. In each of the clinical conditions associated with nodular regenerative hyperplasia, including macroglobulinemia, inflammatory or thrombotic vascular lesions are found in many organs. Therefore, nodular regenerative hyperplasia may be the hepatic expression of a more widespread vascular disease.     

对肝结节状再生性增生的再认识—附18例临床分析

目的通过对18例肝结节状再生性增生(nodular regenerative hyperplasia of liver,NRH)病例进行总结分析,以提高临床医师对此类疾病的认识。方法对北京协和医院1983年1月~2008年12月住院的18例NRH患者的临床表现、实验室检查、影像学检查及病理学检查等多项资料进行总结和分析。结果NRH临床上可表现为肝脏弥漫性病变和占位性病变,但以前者多见。门脉高压症是NRH常见的临床表现,以脾大、脾亢较为突出,肝功能仅轻度异常。NRH常合并自身免疫或胶原血管性疾病,此类患者常伴有多种自身抗体、ESR增快、Ig增高、γ%增高等异常。病理学检查肝脏内弥漫分布小的无纤维分隔的再生结节,其门静脉分支有不同程度的狭窄或闭塞。结论对临床上难以解释或不明原因的门脉高压症患者,尤其是合并自身免疫性疾病的患者,要警惕NRH的可能性。     

Systemic lupus erythematosus and nodular regenerative hyperplasia of the liver

A patient with systemic lupus erythematosus and nodular regenerative hyperplasia of the liver is presented.     

Focal nodular hyperplasia of the liver associated with high-dosage estrogens.

A patient developed benign nodular hyperplasia of the liver after long-term, high-dose estrogen therapy. A 10.5-cm benign liver nodule appeared after three years of high-dose estrogen therapy and was resected. Fourteen months later, while still receiving estrogen, a 4.5-cm nodule and multiple small nodules were seen on angiography. Four months after estrogen was discontinued, the larger liver nodule disappeared and the smaller ones decreased.     

Nodular regenerative hyperplasia of the liver in a patient with rheumatoid vasculitis: a morphometric study suggesting a role for hepatic arteritis in the pathogenesis

Nodular regenerative hyperplasia (NRH) of the liver commonly occurs as a complication of Felty's syndrome but the pathogenesis of the hepatic lesion is not certain. We present a patient with rheumatoid arthritis, leukopenia, glomerulonephritis, NRH and portal hypertension who had histologic evidence of widespread intrahepatic arteritis and paucity of arteries less than 40 micron diameter. We postulate that in this patient the arterial lesions caused secondary portal venous obliteration, portal hypertension, and nodular transformation.     

Imaging of nodular regenerative hyperplasia of the liver. Study of 9 cases

Radiologic patterns of the liver in nodular regenerative hyperplasia are poorly known. The authors describe the features of the liver observed during ultrasonography, angiography, computed tomography and magnetic resonance imaging in 9 patients with nodular regenerative hyperplasia. Generally the liver appeared normal, occasionally with signs of portal hypertension. However, a pseudotumoral pattern was observed in 2 patients: they had well limited hypoechoic lesions on ultrasonography, and in one patient angiography showed hypervascularity.     

Encephalopathy following portal shunting in nodular regenerative hyperplasia of the liver

Two patients with nodular hyperplasia of the liver developed a chronic disabling encephalopathy after an interposition mesocaval shunt. Both had a low total hepatic blood flow-rate postoperatively. Encephalopathy disappeared following surgical occlusion of the shunt. These observations emphasize the risk of postshunt encephalopathy in patients with non-cirrhotic intrahepatic portal hypertension.     

Focal nodular hyperplasia of the liver associated with intracranial vascular malformations.

Focal nodular hyperplasia of the liver, neoplasia of the brain, and intracranial vascular malformations have recently been described as a new syndrome. We report three cases of focal nodular hyperplasia of the liver with intracranial vascular anomalies. Two patients died from ruptured berry aneurysm. It is important to consider intracranial pathology in patients with focal nodular hyperplasia of the liver.     

On the pathogenesis of focal nodular hyperplasia of the liver

Fifty-one focal nodular hyperplasia lesions from 36 patients were examined histologically. Serial sections and three-dimensional models were studied in selected cases. Lesions were multiple in 19% of patients. Thirty-four patients were female. One case had 11 focal nodular hyperplasia lesions and 7 hemangiomata in the liver. Three had astrocytoma and one had anomalous pulmonary venous drainage. Morphometric analysis revealed that the lesions were supplied by an anomalous artery larger than expected for the locale in the liver. This artery branched to form a spider-like structure and was usually not accompanied by a portal vein or duct. Each terminal arterial branch supplied a separate nodule 1 mm in diameter; adjacent nodules coalesced to form the focal nodular hyperplasia lesion. The arterial blood appeared to drain directly into the sinusoids of the nodule. We propose that focal nodular hyperplasia is an hyperplastic response of the hepatic parenchyma to a preexisting arterial spider-like malformation. The frequent coexistence of focal nodular hyperplasia with other vascular and neuroendocrine anomalies suggest that the malformations are developmental in origin. The basic requirement for development of hepatic hyperplasia may be greater blood flow to a region compared to the adjacent parenchyma. This requirement appears to be met in the other forms of nodular transformation of the liver, i.e., nodular regenerative hyperplasia and partial nodular transformation.