Articles
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20 results found
Article
Reye syndrome
Reye syndrome is a rare pediatric condition characterized by acute onset encephalopathy, severe vomiting and fatty liver failure. It typically presents in children recovering from a viral illness, most commonly influenza or chickenpox 1.
Epidemiology
Reye syndrome is usually seen between the ...
Article
Glycogen storage disease
Glycogen storage disease (GSD) refers to a number of syndromes which are characterized by a defect in synthesis, metabolism or storage of glycogen.
Pathology
There are many types of GSD:
type I: von Gierke disease
type II: Pompe disease
type III: Cori or Forbes disease
type IV: Andersen di...
Article
Mirizzi syndrome
Mirizzi syndrome refers to an uncommon phenomenon that results in extrinsic compression of an extrahepatic biliary duct from one or more calculi within the cystic duct or gallbladder. It is a functional hepatic syndrome but can often present with biliary duct dilatation and can mimic other hepat...
Article
Abdominal compartment syndrome
Abdominal compartment syndrome is a disease defined by the presence of new end-organ dysfunction secondary to elevated intra-abdominal pressure. Radiological diagnosis is difficult and usually suggested when a collection of imaging findings are present in the appropriate clinical setting or if t...
Article
Budd-Chiari syndrome
Budd-Chiari syndrome, also known as hepatic venous outflow obstruction (HVOO), refers to the clinical picture that occurs when there is partial or complete obstruction of the hepatic veins.
There is no clear consensus regarding the number of occluded veins, some authors claim that there should...
Article
Lemmel syndrome
Lemmel syndrome is defined as obstructive jaundice caused by a periampullary duodenal diverticulum compressing the intrapancreatic common bile duct with resultant bile duct dilatation.
Clinical presentation
Patients may present with recurrent episodes of jaundice, pancreatitis and/or cholangit...
Article
Alagille syndrome
Alagille syndrome (also known as arteriohepatic dysplasia) is a congenital genetic multisystem disorder.
Clinical presentation
Infants typically present with symptoms relating to the liver where it is one of the most common causes of hereditary cholestasis.
Genetics
Alagille syndrome is inhe...
Article
HELLP syndrome
HELLP syndrome is a pregnancy-related condition and is an abbreviation for:
haemolysis
elevated liver enzymes and
low platelets
It is considered a severe and life-threatening form of pre-eclampsia although it can occur without co-existing pre-eclampsia.
Epidemiology
The estimated inciden...
Article
Hepatopulmonary syndrome
Hepatopulmonary syndrome (HPS) is a clinical syndrome defined by the presence of the following:
liver disease
dilation of pulmonary vasculature
may involve pulmonary capillaries, pulmonary arteriovenous malformations (AVMs), or pleural arteriovenous malformations
abnormalities in oxygenation...
Article
Bile plug syndrome
Bile plug syndrome, also known as inspissated bile syndrome, is an uncommon cause of jaundice in neonates. Refers to a rare extrahepatic mechanical obstruction of the major bile duct in the perinatal period caused by viscous bile (sludge) within its lumen 3.
Epidemiology
Risk factors
Risk fa...
Article
Low phospholipid-associated cholelithiasis syndrome
Low phospholipid-associated cholelithiasis (LPAC) syndrome is one of the syndromes associated with ABCB4/MDR3 mutation. Characteristics of this syndrome include 1,2:
intrahepatic microlithiasis/sludge
symptomatic cholesterol stones with early onset (<40 years)
recurrent symptoms po...
Article
COACH syndrome
The acronym COACH describes a syndrome associated with:
C: cerebellar vermis defect (Joubert syndrome)
O: oligophrenia
A: ataxia
C: coloboma
H: hepatic fibrosis
Epidemiology
COACH syndrome is considered extremely rare.
Associations
The association with infantile polycystic kidney diseas...
Article
Whipple triad
Whipple triad is the clinical presentation of pancreatic insulinoma and consists of:
fasting hypoglycemia (<50 mg/dL or <2.7 mmol/L)
symptoms of hypoglycemia
immediate relief of symptoms after the administration of intravenous glucose
History and etymology
The triad and also the Whipple pro...
Article
Fitz-Hugh-Curtis syndrome
Fitz-Hugh-Curtis syndrome (FHCS), or perihepatitis, is the inflammation of the liver capsule and overlying peritoneum associated with adhesion formation, without the involvement of the hepatic parenchyma. It is a chronic complication of pelvic inflammatory disease (PID).
Epidemiology
The preva...
Article
Hereditary hemorrhagic telangiectasia
Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a rare inherited disorder characterized by abnormal blood vessel formation in the skin, mucous membranes, and organs including the lungs, liver, and central nervous system.
Epidemiology
Worldwide prevalen...
Article
Pepper syndrome
Pepper syndrome is a term not readily used in day-to-day practice and usually refers to primary adrenal neuroblastoma with extensive liver metastases 1. In essence, it refers to stage 4S neuroblastoma (see staging of neuroblastoma). Cohen syndrome, a genetic disorder, is sometimes referred to a...
Article
Gullo syndrome
Gullo syndrome, also known as benign pancreatic hyperenzymemia, is characterized by the abnormal elevation of the serum levels of most or all of the pancreatic enzymes without any evidence of underlying pancreatic pathology. It is a diagnosis of exclusion made when all other laboratory assays an...
Article
Familial atypical multiple mole melanoma syndrome
Familial atypical multiple mole melanoma (FAMMM) syndrome is an autosomal dominant genodermatosis characterized by multiple melanocytic nevi (often more than 50) and a family history of melanoma.
Pathology
Genetics
It is associated with mutations in the CDKN2A gene and shows reduced penetranc...
Article
Stauffer syndrome
Stauffer syndrome is paraneoplastic nephrogenic hepatomegaly. It most commonly occurs in the setting of renal cell carcinoma, and is the enlargement of the liver without hepatic metastases. It is a cause of cholestasis and cholestatic jaundice.
History and etymology
It is named after Maurice ...
Article
Bile sump syndrome
Bile sump syndrome refers to a complication following a side-to-side choledochoduodenostomy.
Epidemiology
This complication occurs in ~1% of patients following a side-to-side choledochoduodenostomy.
Clinical presentation
Recurrent abdominal pain, cholangitis, pancreatitis, or biliary obstr...
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