Malignant retroperitoneal fibrosis: MRI characteristics in 50 patients

ABSTRACT: We analyzed magnetic resonance imaging (MRI) morphologic patterns of retroperitoneal fibrosis (RF) to identify those able to distinguish malignant RF (mRF) from idiopathic RF (iRF). This retrospective study concerned 50 consecutive patients with MRI-based RF diagnoses, 35 of whom also had histologically proven RF. Previous radiotherapy, abdominal or pelvic surgery or infection during the preceding 6 months, vascular aneurysm (aorta or iliac artery), presence of retroperitoneal multiple nodular masses, or enlarged lymph nodes with a diameter >15 mm constituted exclusion criteria.Patients with mRF differed from those with iRF by age, smoking habits, and follow-up duration but not by clinical manifestations, inflammatory syndrome, or renal insufficiency. MRI-documented mRF extension along the aorta, from above the renal arteries to below the aortic bifurcation, was more frequent than iRF (47% vs. 0%; p = 0.001) but less frequent between the renal arteries and the aortic bifurcation (18% vs. 50%; p = 0.04); mRF extension behind the aorta was wider than iRF (5.0 vs. 2.5 mm; p = 0.03). Neither urinary tract nor vessel involvement differed. Medial ureteral attraction was significantly less frequent in mRF than iRF (24% vs. 83%; p < 0.001), according to univariate and multivariate analyses. An algorithm based on the most discriminant criteria (RF extending from above the renal arteries to below the aortic bifurcation and the absence of medial ureteral attraction) for mRF diagnosis had 82% sensitivity and 83% specificity. When applied to the 15 iRF patients without histologic data, specificity was 73%.This mRF decision tree, consisting of the 2 most discriminant MRI criteria, could be used as a supplementary argument to support RF biopsy.     

Occult diverticulitis,a cause of retroperitoneal fibrosis

A case is reported in which extensive mesocolonic and retroperitoneal granulomatous and fibrotic disease were apparently due to occult diverticulitis. The patient later had a good result from aggressive but limited operation for obstructions of the ureter and colon.     

Spontaneous bacterial peritonitis in a patient with congenital hepatic fibrosis and Caroli's disease

The authors describe by the first time in the literature a patient with congenital hepatic fibrosis and Caroli's disease complicated by spontaneous bacterial peritonitis. The possible routes of ascitic infection are discussed.     

Caroli's disease in congenital hepatic fibrosis and infantile polycystic disease

ABSTRACT— Two of three patients with infantile polycystic disease and all three patients with congenital hepatic fibrosis revealed multiple gross cystic dilatation of the intrahepatic biliary tree, referred to as Caroli's disease. All three patients with congenital hepatic fibrosis showed recurrent cholangitis related to coexisting Caroli's disease, and two of them died of sepsis following cholangitis. There were several common morphologic findings in the intrahepatic biliary tree of macroscopic and microscopic levels in infantile polycystic disease and congenital hepatic fibrosis with Caroli's disease: 1. irregular, non-obstructive dilatation of the duct lumen; 2. bulbar protrusion of the duct wall into the lumen; and 3. bridge formation of the duct wall across the lumen. These ductal changes might be caused by a combination of uneven and disproportionate overgrowth of biliary epithelia and their supporting connective tissue. This pathogenetic mechanism might be operative along the entire intrahepatic biliary system in this disease group, and involvement of the smaller levels in early life might result in infantile polycystic disease and congenital hepatic fibrosis and simultaneous or possibly later involvement of the larger levels in Caroli's disease.     

Follow up in a case of congenital hepatic fibrosis (author's transl)

A case of congenital hepatic fibrosis (CHF) is described. CHF is characterized by hepatomegaly, portal hypertension, extensive portal fibrosis, ectatic bile ducts, and hypoplasia of terminal portal vein branches. In contrast to the severe portal hypertension liver function tests are largely normal. In our case the disease was first detected when the patient was 7 years old. During the following 9 1/2 years three sequential liver biopsies were performed. Each of them showed the same picture and no progression occurred. The characteristic histological picture of CHF includes mature bile ducts without epithelial proliferation, absence of significant intraportal or interlobular inflammatory infiltrates, and small or hypoplastic distal portal vein branches. On the basis of these features the disease can easily be separated from other forms of liver cirrhosis.     

先天性肝纤维化14例临床病理特点

目的总结先天性肝纤维化的临床特点,描述先天性肝纤维化时产生门静脉高压的病理形态学特点。方法回顾性分析2000年～2009年9月,北京友谊医院门诊及住院诊断为先天性肝纤维化患者的临床特点,对活检肝组织标本进行特殊染色及免疫组织化学染色。结果先天性肝纤维化14例,男女比例1.33：1,平均年龄（14.78±8.52）岁,临床主要表现为门脉高压症型占57.1%,胆管炎型占14.29%,混合型占7.14%。肝脾肿大为最主要的体征占85.7%。常见的合并症为多囊肾和肝内胆管扩张,即Caroli’s病各占14.28%。病理形态学观察提示先天性肝纤维化导致门脉高压的机制包括：门静脉发育不良,门脉数量减少,纤维间隔挤压门静脉。结论先天性肝纤维化是导致非肝硬化性门脉高压症的常见原因之一,掌握其临床及病理特点,有助于提高诊治水平。     

Volvulus in pregnancy: a diagnostic dilemma

In this case report, we describe a pregnant female patient at 27 weeks' gestation with a rare complication of volvulus. Her initial symptom was acute cramping pain of the lower right abdomen. Surgical intervention was performed under the impression of peritonitis. Pathologic diagnosis revealed volvulus of the mid ileum. The remainder of her pregnancy was uneventful. The physiologic changes of pregnancy may predispose the parturient to bowel obstruction due to compression of the gravid uterus against the intestine. If volvulus is suspected, then emergent surgery should be performed. Delays in treatment may result in septic shock and even death. We present this case to remind obstetricians of such rare causes of acute abdomen during pregnancy.     

Grading and staging of hepatic fibrosis, and its relationship with noninvasive diagnostic parameters

AIM: To explore the grade and stage of pathology and the relationship between grading and staging of hepatic fibrosis and noninvasive diagnostic parameters.METHODS: Inflammatory activity and fibrosis of consecutive liver biopsies from 200 patients with chronic liver disease were determined according to the Diagnostic Criteria of Chronic Hepatitis in China, 1995. A comparative analysis was made in these patients comparing serum markers,Doppler ultrasonography, CT and/or MR imaging with the findings of liver biopsy.RESULTS: With increase of inflammatory activity, the degree of fibrosis also rose. There was a close correlation between liver fibrosis and inflammatory activity. AST, GGT, albumin,albumin/globulin, ALP, AFP, hyaluronic acid, N-terminal procollagen Ⅲ(P Ⅲ NP), collagen type Ⅳ(Col Ⅳ), tissue inhibitors of metalloproteinases-1(TIMP-1), alpha-2-macroglobulin, natural killer cells(NK), some parameters of Doppler ultrasonography, CT and/or MR imaging were all related to the degree of inflammatory activity. GGT, albumin,albumin/globulin, ALP, AFP, hyaluronic acid, Col Ⅳ, TIMP1, alpha-2- macroglobulin, transforming growth factor-beta 1(TGFβ1), NK, some parameters of Doppler ultrasonography,CT and/or MR imaging were all related to the staging of fibrosis. By regression analysis, the parameters used in combination to differentiate the presence or absence of fibrosis were age, GGT, the parameter of blood flow of portal vein per minute, the maximum oblique diameter of right liver by B ultrasound, the wavy hepatic surface contour by CT and/or MR. The sensitivity, specificity and accuracy of the above parameters were 80.36 %, 86.67 %, and 81.10 %,respectively.CONCLUSION: There is close correlation between liver fibrosis and inflammatory activity. The grading and staging of liver fibrosis are related to serum markers, Doppler ultrasonography, CT and/or MR imaging. The combinationof the above mentioned noninvasive parameters are quite sensitive and specific in the diagnosis of hepatic fibrosis.     

Riedel's thyroiditis in a patient with retroperitoneal fibrosis

A 56-yr-old man was known with retroperitoneal fibrosis. Seven years later he developed a painful swelling of the left thyroid lobe, which turned out to be Riedel's thyroiditis. The associations between Riedel's thyroiditis, retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis and fibrosis in other organ systems are thought to be manifestations of multifocal idiopathic fibrosclerosis.     

高龄患者特发性腹膜后纤维化1例

腹膜后纤维化(RPF)是一种少见的纤维素性炎性疾病,其沿腹主动脉及髂动脉发展,并扩展到相邻腹膜后腔,常引起尿路梗阻及肾功能衰竭,好发于中年患者,男女比约为3:1~2:1。诊断依赖于腹部B超、CT和MRI等影像学检查,确诊需行腹膜后肿块穿刺活检,临床常采取对症治疗,糖皮质激素被推荐为一线治疗药物。本文对1例83岁特发性RPF患者进行了讨论。     

Two cases of congenital hepatic fibrosis in children of consanguineous marriage

An association of congenital hepatic fibrosis (CHF), Caroli's disease, medullary sponge kidney, type II interventricular defect with right transposition of the aorta, multiple cervical vertebra malformations, and first sacral vertebra schisis in a 16 year old son of consanguineous parents (consanguinity factor = 1/32), is described. The patient's sister presented asymptomatic CHF and medullary sponge kidney only. Parental consanguinity in this case lends support to the view that CHF, medullary sponge kidney and cardiac malformations are more likely to be manifestation of a single recessive gene, with a varying phenotypic expression, than of different mutant genes.