PURPOSE: To report and correlate the clinical, histopathologic, and imaging findings in 15 patients with liver adenomatosis. MATERIALS AND METHODS: Fifteen adult patients had more than 10 hepatic adenomas each and no history of glycogen storage disease or anabolic steroid use. Ten of them underwent bolus-enhanced dynamic computed tomography (CT) with or without magnetic resonance (MR) imaging, ultrasonography, and/or angiography. RESULTS: Clinical abnormalities included abdominal pain in 11 (73%) and hepatomegaly in 10 (67%) patients, and abnormal liver function in 10 (91%) of 11 patients. The number of adenomas in each patient was 10–50 at imaging, but many more lesions were found in the resected specimens. Hemorrhage was commonly found within adenomas at histopathologic analysis, but only four patients had clinical and imaging evidence of substantial hemorrhage. In all patients, the adenomas increased over time, and two patients developed hepatocellular carcinoma. CT and MR features of the adenomas incl...

Liver adenomatosis: clinical, histopathologic, and imaging findings in 15 patients.

Congenital portosystemic venous shunts are rare. Their gross anatomy has not been well defined. Four different varieties of congenital portosystemic venous shunts are described in six children seen during a 10-year period, focusing on the anatomy of the shunt as determined by imaging studies and surgery. A detailed review of the literature indicates that congenital portosystemic venous shunts are best classified as: extrahepatic or intrahepatic. Extrahepatic shunts may be further subdivided into portocaval shunts (type 1 end-to-side and type 2 side-to-side) and others. Intrahepatic shunts are due to an abnormal intrahepatic connection between the portal vein and hepatic vein/inferior vena cava or a persistent patent ductus venosus. Additional congenital anomalies, particularly cardiac malformations, may be associated with any type. Some congenital intrahepatic portosystemic venous shunts close spontaneously in infancy; all other congenital portosystemic venous shunts tend to remain patent. To a variable extent, depending largely on the volume and duration of the shunt, affected individuals are at risk of developing hepatic encephalopathy and/or an intrahepatic tumor. The key to understanding the pathogenesis of these shunts lies in the normal developmental mechanisms underlying the formation of the portal vein and inferior vena cava in the embryo.

The clinical anatomy of congenital portosystemic venous shunts.

Congenital extrahepatic portosystemic shunt (CEPS) is a rare condition in which the portomesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial shunt. Most often, the diagnosis is made primarily with Doppler ultrasonography. Computed tomographic angiography and magnetic resonance angiography are used for further classification of the shunt and assessment of accompanying anomalies. Conventional angiography is necessary when results of the other tests disagree or are inconclusive. CEPS is classified into two types according to the pattern of anastomoses between the portal vein and systemic vein. In type 1, intrahepatic portal venous supply is absent; in type 2, intrahepatic portal venous supply is preserved. Type 1 usually occurs in girls with associated malformations, such as situs ambiguous with polysplenia and congenital heart defects. Associated anomalies are less frequent in type 2, and symptoms usually develop later without a gender preference. Hepatic encephalopathy and liver dysfunction are possible complications of both types and usually develop during adulthood. Both types are also associated with regenerative hepatic nodules. The clinical setting and imaging appearance of these nodules can help one avoid misdiagnosis. Definitive treatment of CEPS is determined by the type of shunt. Liver transplantation is the only effective treatment for symptomatic type 1 CEPS; surgical closure or embolization of the shunt is the therapeutic approach for type 2.

Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review.

榴状的再生增生(NRH ) 是肝的实质的普遍良性的转变描绘进小再生小瘤的一个稀罕的肝条件。NRH 可以导致非肝脏硬化症的门高血压的发展。NRH 上没有出版系统的人口研究，我们的当前的知识限于案例报告和案例系列。NRH 可以发展经由自体免疫， hematological，传染、肿瘤，或药相关的原因。疾病通常是无征状的，慢慢地或非进步除非门高血压的复杂并发症发展。精确诊断被组织病理学说做，没有含纤维的 septa，它表明弥漫的 micronodular 转变。perinuclear 骨胶原织物的缺乏在肝脏硬化症的肝把 NRH 与典型再生小瘤区分开来。当起始的治疗是探讨内在的疾病时，最终，治疗被指导到门高血压的管理。NRH 的预后两个都取决于内在的病和门高血压的第二等的复杂并发症的预防的严厉。在这评论，我们详细说明 NRH 的传染病学，致病，诊断，管理，和预后。

Nodular regenerative hyperplasia：Evolving concepts on underdiagnosed cause of portal hypertension

Congenital absence of portal vein (CAPV) was a rare event in the past. However, the number of detected CAPV cases has increased in recent years because of advances in imaging techniques. Patients with CAPV present with portal hypertension (PH) or porto-systemic encephalopathy (PSE), but these conditions rarely occur until the patients grow up or become old. The patients usually visit doctors for the complications of venous shunts, hepatic or cardiac abnormalities detected by ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI). The etiology of this disease is not clear, but most investigators consider that it is associated with abnormal embryologic development of the portal vein. Usually, surgical intervention can relieve the symptoms and prevent occurrence of complications in CAPV patients. Moreover, its management should be stressed on a case-by-case basis, depending on the type or anatomy of the disease, as well as the symptoms and clinical conditions of the patient.

Insight into congenital absence of the portal vein: is it rare?

We present a patient with nodular regenerative hyperplasia of the liver (NRH) and portal vein absence studied with CT, MR imaging, and MR angiography. The most striking feature was exuberant hemorrhoids due to a giant hepatofugal inferior mesenteric vein. A relationship between unbalanced portal blood flow and nodular regenerative transformation of the liver is suggested in this patient.

Portal vein absence and nodular regenerative hyperplasia of the liver with giant inferior mesenteric vein

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Portal vein absence and nodular regenerative hyperplasia of the liver with giant inferior mesenteric vein