Biliarycystadenoma or cystadenocarcinomahighe levels of serum CA19-9 are observed
Simple hepatic cysts are benign developmental lesions that do not communicate with the biliary tree. The current theory regarding the origin of true hepatic cysts is that they originate from hamartomatous tissue. Hepatic cysts are commonand are presumed to be present in 2.5% of the population. They are more often discovered in women and are almost always asymptomatic. Simple hepatic cysts can be solitary or multiple, with the latter being the more typical scenario. At histopathologic analysis, true hepatic cysts contain serous fluid and are lined by a nearly imperceptible wall consisting of cuboidal epithelium, identical to that of bile ducts, and a thin underlying rim of fibrous stroma.
With the frequent use of ultrasonography and CT scan, simple hepatic cyst are now detected in 2.5-5% of the population.Only 15-16% of such cysts are symptomatic. Symptomatic cysts are found more commonly in women who are over 50 years of age.
Three categories: active, transitional, and inactive.Five types: CE1 to CE5.
Water lily: زنبق الماء - النيلوفرThere are more than 15 classification schemes for liver hydatid cysts.The liver is the most common site of hydatid disease involvement, and most cysts are located in the right lobe.a CL (cystic lesion) type has been added in the WHO classification that was not included in Gharbi’s classification, for those cysts whose parasitic nature cannot be determined based on solely on the results of the US examination.There are 3 types of cyst rupture: contained, communicating, or direct.
Water lily: زنبق الماء - النيلوفر
Studies on the postoperative appearance of the cyst are limited in number.Recurrence rate 8 – 10 %.Postoperative collection of fluid and blood in the lesion area may be misinterpreted as a pseudocyst.The parasite residing in the liver grows up to 1 cm in the first 6 months and 2–3 cm annually depending on the immune status of the host and the pathogenity of the organism.
Studies on the postoperative appearance of the cyst are limited in numberLate ultrasonographic findings in cases operated for hydatid cyst of the liverEuropean Journal of Radiology 56 (2005) 91–96. Purpose: The aim of this study was to evaluate and present the images due to surgical intervention and to recurrences in patients who had been operated for hydatid cyst of the liver at least 12 months prior to the imaging process.Material and methods: A total of 77 patients (46 females, 31 males) with a mean age of 38 years (10–60 years) who had undergone surgical intervention for hydatid cyst of the liver were included in this study. The type and the number of operations were determined by reviewing previous medical records of the patients. Recurrence findings and postoperative images were examined by ultrasonography in all patients.Results: Of the 77 patients, 68 had undergone surgical operation for hydatid cyst of the liver for once, six cases for twice, one patient for three and another patient for four times. Ultrasonographic examination was considered normal in 9 (11.6%) patients. The most frequent finding in the remaining patients was hypoechoic (n = 6) and anechoic (n = 14) images with a hyperechoic periphery within the operation area. While a coarse heterogenous area was visualized in 12 cases (15.5%), a sole hypoechoic image was present in 10 patients. Recurrence was detected in 9 (11.6%) patients of whom 7 were asymptomatic. While daughter cysts were detected in two recurrent cases; the remaining were unilocular cysts. An omentum image extending to the operation area was detected in 11 patients. Calcification was present in 14 patients, whereas four cases had less common findings of anechoic tubular structures adjacent to the operation area.Conclusion: While the liver may seem normal by ultrasonography in the late postoperative period in patients, who had been operated forhydatid cyst of the liver, various images may also be present. These images may be misinterpreted as recurrence or other pathologies. Thus, the radiologist should be familiar with the postoperative ultrasonographic findings of hydatid cyst and should not misinterpret the image of anechoic fluid as recurrence. When in doubt, ultrasonographic follow-up is essential. An early postoperative ultrasonographic examination may be the key point in precluding a misdiagnosis.
Motile and sensory functions expressed on almost all vertebrate cells and show remarkable conservation from protozoa to humans.In the liver, cholangiocyte cilia are positioned to detect changes in bile flow, composition,and osmolality and have been shown to possess mechanosensory, chemosensory, and osmosensory functions.
Congenital bile duct malformations due to the failure of embryonic involution
The prevalence of biliaryhamartomas has been estimated up to 5–6 % in autopsy series, and 0.6% in needle biopsy series.Cystic dilated bile ducts smaller than 10 mm in diameter & it is probably the result of ductal plate malformation.VMC are often confused with metastatic cancer and reports describe single, multiple, or-most often innumerable well-defined solid nodules usually less than 1 cm in diameter. Nodules are usually uniformly hypoechoic and less commonly hyperechoic on sonographyand hypodense on contrast-enhancedCT scan. Bright echogenic foci in the liver with distal "ringdown" artifact without obvious mass effect are also documented on sonograms on patients with VMC. We believe that these echogenic foci could be related to the presence of tiny cysts beyond the resolution of the ultrasound equipment. VMC are usually isolated and insignificant observations. VMC may occur with other congenital disorders such as congenital hepatic fibrosis or polycystic kidney or liver disease. Association of VMC with cholangiocarcinoma has been suggested.
The best imaging modality probably is MRI as biliaryhamartomas usually appear hypointense on T1-weighted images with corresponding bright white lesions high signal intensity on T2-weighted images.Small (<1 cm) – Often multiple – Easily mistaken for metastasesVon Meyenburg complexes (also known as biliaryhamartomas) are composed of a cluster of proliferated bile ducts embedded in a fibrous stroma. These small (usually <1 cm) benign lesions are detected incidentally in 1% to 5% of reported autopsies and are often multiple. They are of no clinical significance except that they can be easily mistaken for metastases on CT and ultrasonography. Von Meyenburg complexes are usually uniformly hypoechoic on ultrasonography but may contain echogenic foci with ringdown artifact related tothe presence of cholesterol crystals in the dilated tubules.Von Meyenburg complexes usually can be differentiated from metastases at MRI with very high signal on T2-weighted images. No enhancement or only a rim of enhancement is visible on post-gadolinium images. This rim enhancement is thought to be caused by compressed liver parenchyma surrounding the biliaryhamartoma.
Peribiliary cysts are multiple small cystic dilatations of the intrahepatic extramural peribiliary glands. They are located exclusively in the hepatic hilum and within the larger portal tract. To date, fewer than 60 cases of multiple hepatic peribiliary cysts have been reported in the literature, but the prevalence appears to be higher and has been estimated at 0.26% in all autopsy series. The pathogenesis is incompletely understood and it is currently unclear whether this condition has a significant genetic component or not. Peribiliary cysts can be found in cases with cirrhosis, specifically in those patients with portal hypertension. It has been suggested that circulatory disturbances may be involved in the pathogenesis of these cysts.Peribiliary cysts are adjacent to, but did not communicate with the biliary tree and are lined with columnar or cuboidal epithelium. Peribiliary cysts are usually a clinically benign condition; however, focal biliary obstruction can arise as a result of larger cysts. Long term follow-up has demonstrated that they can increase in size and number over time.On ultrasound, rounded or tubular anechoic structures can be observed along the portal tracts, findings that may be confounded with dilated bile ducts. Likewise cystic structures central in the liver can be seen along the portal tracts of the liver at CT and MRI.It is key to make an accurate diagnosis of peribiliary cyst because complications secondary to peribiliary cysts, such as obstructive jaundice, can arise, and may avoid unnecessary workup and therapy.
Type IV (15%)Type IVa Multiple dilatations of the intrahepatic and extrahepaticbiliary tree.Type IVb Multiple dilatations restricted to the extrahepatic bile duct.Reported rates range from one case per two million live births to one case per 100,000–150,000 live births in Western countries. The etiology of choledochal cysts has still to be elucidated. Consistent with other fibrocystic disorders it can be hypothesised that it results from ductal plate malformation of the extrahepatic bile ducts. An alternative possibility might be a persistent reflux of pancreatic enzymes into the common bile duct secondary to an anomalous pancreaticobiliary junction and obstruction of the distal common bile duct.
A very large proportion of (and possibly all) patients with choledochal cyst have an unusually long common channel between the junction of the common bile duct and the main pancreatic duct and their joint outflow into the duodenum. The increased intraductal pressure associated with this anomaly predisposes to reflux and may also be associated with ectasia of the common channel or indeed with pancreatitis.
Prevalence rates for cholangiocarcinoma vary widely among different case series (0–28%) depending on size of the series and length of follow-up. A larger series of 48 patients reported an incidence of 12.5%.
Given their rarity, diverticular cysts are the most difficult to classify and for which to propose an underlying etiology. They are characterized by localized pathological dilation in an otherwise normal bile duct. They are connected to the normal bile duct by a well-defined neck. Diverticula have been reported to extend off of the extrahepatic bile ducts as well as the right and left hepatic ducts. They may occur at any age and no gender predilection as been reported. They may be single or multiple.
Very small risk of carcinomain association with choledochoceleTo our knowledge, there have been four cases reported in the literature to date.
The “dot” is a portal or arterial branch at the periphery or center of a cyst or within a pseudo-septum running through the cyst.
Central dot sign not specific for Caroli’s disease.It has been described in some cysts without communication with the biliary tract. Its existence is correlated with the presence of ductal plate malformation.Reference:Luciani A. GastroenterolClinBiol 2005 ; 29 : 870 – 874.
Parfrey PS et al. N Engl J Med 1990 ; 323 : 1085 – 90.US, because of high sensitivity and low cost, has become the primary method of diagnosing ADPKD and following the cysts.US screening for ADPKD typically begins between ages 10 & 15 years, (false negatives in 14% of patients younger than 30 years). Bear and colleagues developed criteria that are widely used to diagnose ADPKD. In adults who have a family history of ADPKD, the presence of at least three cysts in both kidneys, with at least one cyst in each kidney, is a positive finding.
Renal US & DNA analysis for ADPKD were performed in 319 patients who were at risk. PKD1: Encodes polycystin-1 – Short arm of chromosome 16 – Account for 85 – 90 % of population with ADPKD.PKD2: encodes polycystin-2 – Long arm of chromosome 4 – Account for 10 – 15% of population with ADPKD.In some other families, no linkage to either PKD1 or PKD2 has been reported.
Patients with two truncating mutations generally display a severe phenotype with perior neonatal death, whereas patients surviving the neonatal period usually carry at least one missense mutation.
Mutations in the polycystic kidney and hepatic disease 1 gene (PKHD1) on chromosome 6p21.1-p12 were recently identified as the genetic cause of autosomal recessive polycystic kidney disease/CHF. This gene is responsible primarily for the expression of the protein named polyductin or fibrocystin and the defect of this protein leads to cyst formations in different organ systems including liver, pancreas and kidney.
Unlike in autosomal dominant polycystic kidney disease:1- Cysts are rarely complicated by hemorrhage or infection2- Kidneys markedly enlarged before birth & continue to grow in the first 2–3 years of life Thereafter, kidney growth plateaus or even decreases. Kidneys associated with ADPKD demonstrate progressive growth throughout life as the size & number of macrocysts increase.
It is a disease primarily of ectatic tubules and fibrosis.It is a hereditary form of cystic kidney disease that has an estimated incidence of one in 20,000 live birthsOrigins of the foci are uncertain: 1- Lucaya et al. suggested that they may represent urine stagnation within dilated tubules including calcium precipitation secondary to diminished citrate excretion 2- An alternative explanation is that the multiple sharp interfaces between dilated collecting ducts causes artefactualechogenic foci. However, the precise nature of the echogenic foci remains to be determined.
With advancing clinical course and development of larger renal cysts accompanied by interstitial fibrosis, the kidney structure of ARPKD may increasingly resemble that of ADPKD.
Congenital hepatic fibrosis is a dynamic disorder that is characterized histologically by a variable degree of periportal fibrosis.The term autosomal recessive polycystic disease is used for those cases in which renal involvement is the predominant feature.It can occur alone but almost always occurs in association with autosomal recessive polycystic-kidney disease.Congenital hepatic fibrosis associated with Caroli disease (dilatation of intrahepatic bile ducts) is termed Caroli syndrome.Four clinical forms of congenital hepatic fibrosis have been described: portal hypertensive, cholangitic, mixed portal hypertensive-cholangitic, and latent forms.There is a progression in the extent of liver fibrosis over time, with occasional evolution into true cirrhosis of the liver. In most patients, the first manifestations of the disease are signs or symptoms related to portal hypertension, especially splenomegaly and varices, often with spontaneous gastrointestinal bleeding.The timing of the onset of signs and symptoms is variable, ranging from early childhood to the 5th or 6th decade of life, although most cases are diagnosed in adolescents or young adults.Liver function test results may remain normal or be only modestly elevated.In patients with congenital hepatic fibrosis, there are some distinctive hepatic morphologic findings (along with associated abnormalities).Atrophy of the right lobe and hypertrophy of the left lateral segment and caudate lobe are common both in patients with congenital hepatic fibrosis and in those with advanced viral or alcoholic cirrhosis.
The gallbladder separates the right lobe from the left medial segment.
The gallbladder separates the right lobe from the left medial segment.
these cavernous transformation-like periportal vessels probably represent a developmental duplication of hepatic vascular branching that is part of CHF and is not an acquired pathology since it is usually present before the onset of PH and is not associated with occlusion of theportal vein.
Fenestration is a technique that combines aspiration and surgical deroofing of the cyst in asingle procedure. Surgical access has the advantage that multiple cysts can be treated at once during the procedure.
Fenestration is a technique that combines aspiration and surgical deroofing of the cyst in asingle procedure. Surgical access has the advantage that multiple cysts can be treated at once during the procedure.
Its precise pathogenesis is unknown, and many possible pathologic processes have been proposed, including detached outpouching of hepatic diverticulum or adjacent enteric foregut. Thus, histologic findings of CHFC are basically similar to those of a brochogenic or esophageal cyst. Ciliated foregut cysts are relatively rare and usually seen at the level of the mediastinum and esophagus; hepatic foregut cysts are very rare. Reports of US findings of CHFC have been relatively scarce and divergent, ranging from completely echo free to echo rich (pseudosolid pattern).The chief differential diagnosis in CHFCs that present with complex cysts or containing echogenic material in the lumen is either cystadenoma or cystadenocarcinoma. The essential differentiating feature in the latter includes the multilocular nature and sometimes the presence of intramural nodules.Further confirmation of the diagnosis may be possible by FNAC, which has been tried in a total of 17 CHFC cases and achieves a positive predictive value of 76%. The characteristic feature on FNAC is the presence of the ciliated pseudostratified tall columnar epithelial cells suspended in a mucoid background.Surgical removal of CHFC should be avoided because it is benign and has no malignant potential.The lesion appears as a lined, ciliated, pseudostratified, mucin-secreting, columnar epithelium.
Schoenlank first described peliosishepatis in 1916. It is a benign disorder defined by the presence of irregularly shaped cystic blood filled cavities in the liver. Although the term was originally applied to a macroscopic appearance (from Greek pelios, meaning dusky/purple), it is now defined microscopically. Peliosis can affect organs other than the liver, especially the spleen, lymph nodes and, less commonly, bone marrow, lungs, pleura, kidneys, adrenals, stomach and ileum.The aetiology of peliosishepatis is unknown, however a number of causes have been postulated. These include outflow obstruction of blood flow at sinusoidal/centrilobular level, hepatocellular necrosis, a direct lesion of the sinusoidal barrier or a toxic effect on the sinusoidal endothelium.Peliosishepatis is associated with drugs (anabolic, oestrogenic or adrenocortical steroids, azathioprine and tamoxifen), wasting syndromes (neoplasm, especially hepatocellular carcinoma and tuberculosis), toxins (polyvinyl chloride, arsenic and thorium oxide) and infection in acquired immunodeficiency syndrome patients. In 20–50% of cases no associated condition is identified.The clinical presentation is variable. The disease may be asymptomatic and focal liver lesions discovered by chance on hepatic imaging or there may be hepatomegaly, abnormal liver function tests, and rarely, complications such as liver failure, portal hypertension or intra-abdominal haemorrhage. Imaging findings in peliosishepatis are variable depending on differing pathological patterns of disease and the presence of concomitant hepatic steatosis. Conventional grey-scale ultrasound shows homogeneous, hypoechoic lesions in patients with fatty liver, hyperechoic lesions in patients with a normal liver and heterogeneous lesions if complicated by haemorrhage. After enhancement with Levovist (a galactose and palmitic acid compound) transient “fast surge” echo enhancement was seen centrally in some lesions, with no centripetal enhancement, a potential differentiating feature from hepatic haemangiomata.The differential diagnoses for transient central enhancement includes high-flow haemangioma, focal nodular hyperplasia and malignant tumours with arteriovenous shunts as described by Wermke.
In the largest series, tumor size averaged greater than 10 cm.Most HBCAs are multiloculated with internal septations. Other common features are focal calcification, nodularity, and septal thickening.
Cystadenomas are uncommon tumors that are often incorrectly diagnosed as simple cysts.Less than 200 cases of cystadenomas and a little more than half as many cystadenocarcinomas have been reported in the literature. More than 85% of cystadenomas are reported in women.Preoperative imaging that demonstrates the presence of internal septations highly suggests the diagnosis of cystadenoma.
Most hepatic metastases are solid, but some have a complete or partially cystic appearance. In general, two different pathologic mechanisms can explain the cystlike appearance of hepatic metastases. First, hypervascular metastatic tumors with rapid growth may lead to necrosis and cystic degeneration. This mechanism is frequently demonstrated in metastases from neuroendocrine tumors, sarcoma, melanoma, and certain subtypes of lung and breast carcinoma. Second, cystic metastases may also be seen with mucinousadenocarcinomas, such as colorectal or ovarian carcinoma.
Fever, sepsis Presence of air, double target sign, enhancing wall
Presence of air within a lesion, although uncommon, is diagnostic of a gas-forming organism if there is no history of instrumentationor rupture into a hollow viscus. The shape and location (air fluid level) should enable correct diagnosis.
Increased peripheral rim enhancement, which is secondary to increased capillary permeability in the surrounding liver parenchyma (the “double target” sign). Because of hemorrhage into the cavities, the abscesses are sometimes filled with a chocolate-colored, pasty material known as “anchovy paste” . Secondary bacterial infection may make these abscesses purulent. In many patients, it is difficult to differentiate amebic abscesses from pyogenic abscesses, but epidemiologic & clinical information, in conjunction with positive amebic titers, may suggest the diagnosis. Available serologic tests include an ELISA and indirect hemagglutination, cellulose acetate precipitin, counterimmunoelectrophoresis, immunofluorescent antibody, and rapid latex agglutination tests. Serologic test results must be interpreted in the clinical context because serum antibody levels may remain elevated for years after recovery or cure. The sensitivity of these tests is approximately 95%, and the specificity is more than 95%. False-negative results may occur within the first 10 days of infection. PCR-based tests to detect amebic DNA and an ELISA to detect amebic antigens in serum are available in the research setting.
Tunis Med. 2009 Dec;87(12):801-4.Pancreatic pseudocysts located in the liver: a systematic review of the literature.Guesmi F, Zoghlami A, Saidi Y, Najeh N, Dziri C.Service de ChirurgieGénérale, Centre de Traumnatologie et des GrandsBrûles, Ben Arous.AbstractBACKGROUND: Pancreatic pseudocysts (PC) are a common complication of both acute and chronic pancreatitis. Most pancreatic pseudocysts are located within the head and the body of the pancreas, but 20% of them are extrapancreatic (pleura, mediastinum, pelvis and spleen). The location of a pseudocyst in the liver is an exceptional event, only thirty three cases are reported in the literature.AIM: This article aimed to report a new case of PC located in the liver combined with a systematic review of reported cases published in peer-reviewed journals.METHODS: A new case of PC located in the liver was reported. An extensive electronic search of the relevant literature since 1990 was carried out using Medline. We retained only the articles reporting one or several cases. When the article was unavailable, we considered the relevant abstracts which should report clinical patterns and therapeutic modalities. Reviews of the literature, systematic reviews, letters to editors and incomplete abstracts were excluded. A descriptive analysis of the collected sample including our case was performed. Morphological, therapeutic and outcome variables were reported.RESULTS: The analysis of 22 cases reported in the literature and our observation provided the following data: 17 men and 6 women with a mean age of 51 +/- 3.2 years. Seventeen patients presented an acute pancreatitis, complicating a chronic pancreatitis in seven cases, alcoholic in six cases, biliary in three cases and traumatic in one case. Six patients presented a chronic pancreatitis. The PC was located in the left lobe of the liver in 12 cases, in the right lobe in 6 cases and interested the two lobes in 5 cases. The lesion was unique in 8 patients and multiple in 15 patients. Fifteen patients were treated by Ultrasound or CT guided percutaneous drainage. Four patients were managed surgically. Three patients had no specific treatment. One patient was successfully treated by endoscopic transpapillary drainage. The evolution was favourable for all patients except in three patients who died.CONCLUSIONS: Pancreatic pseudocyst located in the liver is an exceptional event, commonly following acute pancreatitis, rising in older male, involving the left lobe of the liver and treated by percutaneous drainage.
MRI imaging more suitable than CT for detection & characterization of hemorrhage.Magnetic: مغناطيسي
Cystic liver lesions - An ultrasound perspective
Diagnosis of cystic hepatic lesionsSamir Haffar M.D.Associate Professor of Gastroenterology
Cystic lesions of the liver• Simple hepatic cyst• Hydatid cyst of the liver• Congenital fibrocystic liver diseases• Miscellaneous cystic lesions of the liverMurray KF & Larson AM. Fibrocystic diseases of the Liver.Springer Science + Business Media, Springer, 2010.
Simple hepatic cystCongenital origin• Incidence 2.5 – 10% in general population by USIncreases dramatically in > 60 years• Symptoms Rare – cyst > 5 cm especially in womenObstructive effect: pain, jaundice, PHT• Complications Hemorrhage – rupture – infection• Diagnosis US highly sensitive & specificHemorrhagic cyst: CEUS – CECT• CA19-9 Usually normal or slightly elevated• Treatment Conservative, sclerotherapy, fenestrationShaked O et al. Clin Gastroenterol Hepatol 2011 ; 9 : 547 – 562.
Typical hepatic cystLesions such as this require no further imaging evaluationTchelepi H et al. Ultrasound Quarterly 2004 ; 20 : 155 – 169.Anechoic lesionThin wallWell-defined borderLateral refraction shadowPosterior acoustic enhancement
Mobile echoes in large liver cystsNaganuma H et al. J Clin Ultrasound 2010 ; 38 : 475 – 479.No intra-cystic artifactPRF: 4 kHzPRF: 6 kHzDeep locationPRF: 5.5 kHzSuperficial locationIntracystic range-ambiguity artifactsErroneous display of returning echoes from heartDepth according to PRF – Disappear at a low PR
Atypical liver cystDietrich CF et al. Ultrasound of the liver, European course book, EFSUMB.http://www.efsumb.org/ecb/Conventional ultrasoundPseudo-solid patternSmall diameter - Slice thickness artefactContrast enhanced ultrasoundCEUS proves the cyst
Simple hepatic cystMurray KF & Larson AM. Fibrocystic diseases of the Liver.Springer Science + Business Media, Springer, 2010.Non CECTNon- enhancement ofcyst wall or contentsCECTUltrasoundTypical features
Simple liver cyst complicated by hemorrhageRumack CM et al. Diagnostic ultrasound.Elsevier Mosby, St. Louis, Missouri, 3rd edition, 2005.Well-defined mass with uniformlow-level internal echoesmisinterpreted as solid massUltrasoundNonenhancing low-densitymass consistent with cystCECT scan
Diagrammatic representation of metacestodeof Echinococcus granulosusManual on Echinococcosis in humans & animals: a public health problem of global concern.Eds: J. Eckert, M.A.Gemmell, F.-X. Meslin & Z.S. Pawłowski. Paris, France 2001.Adventitial layerHost tissueLaminated layerGerminal layer Daughter cystCapsule withprotoscolex
WHO-IWGE classificationSonographic findings relating to viability of parasiteActive InactiveTransitionalWHO-IWGE: World Health Organization Informal Working Group on EchinococcosisWHO, IWGE. Acta Trop 2003 ; 85 (2) : 253 – 61.CL (cystic lesion) added:Parasitic nature not determined on US
WHO-IWGE classificationInternational consensus classification5 types on the basis of US appearancesCE I Unilocular simple cystsCE3CE2CE4CE 5WHO-IWGE: World Health Organization Informal Working Group on EchinococcosisWHO Informal Working Group. Acta Trop 2003 ; 85 : 253 – 261.Floating membrane (water lily sign)Multivesicular multiseptated cystHeterogeneous degenerative contentsThick calcified wallActiveTransitionalActiveInactiveInactive
Hydatid cyst of the liverCE 1Turgut AT et al. Ultrasound Quarterly 2008 ; 24 : 17 – 29.Hydatid sandPathognomonicDouble-line signPathognomonicSimple hepatic cystor hydatid cystCystic lesion (CL)Snow flake sign
Sonographic scoring system with routine serology*to differentiate hepatic from hydatid cysts• No wall sign• Irregular borders• Irregular shape• No wall calcifications• Females• Stable US appearance at follow-up• Septa: one or two septa – irregular shape* Serology: IHA (indirect hemagglutination assays) – ELISA IgGGrisolia A et al. J Ultrasound 2009 ; 12 : 75 – 79.
Septate hepatic cystGrisolia A et al. J Ultrasound 2009 ; 12 : 75 – 79.Slightly irregular septum
Hydatid cyst of the liverCE 2 – Daughter cysts“honeycomb-like” or „„rosette-like‟‟ structurePathognomonicCzermak BV et al. Abdom Imaging 2008 ; 33 : 133 – 143.
Liver hydatid cystCE 3 – Water lily signTurgut AT et al. Ultrasound Quarterly 2008 ; 24 : 17 – 29.Lutz HT et al. Manual of diagnostic ultrasound in infectious tropical diseases. Springer, 2006.Focal splitting of cyst wall Extended splitting of cyst wall„„floating membrane‟‟
Czermak BV et al. Abdom Imaging 2008 ; 33 : 133 – 143.„„ball of wool sign‟‟Hypoechoic, hyperechoic, or intermediate patternLiver hydatid cystCE 4
Liver hydatid cystCE 5Well-defined hyperechoic wallCalcified dead hydatid cystTurgut AT et al. Ultrasound Quarterly 2008 ; 24 :17 – 29.
US in operated cases for hydatid cystRecurrence or pseudocyst?Follow-up US• Partial involusion in follow-up images• Follow-up should be performed every 6 months12 months to distinguish recurrence from pseudocystPseudocyst might persist for many yearsOzturk A et al. Eur J Radiol 2005 : 56 : 91 – 96.Pseudocyst• Cavities smaller than original cyst• Irregular & thick borders
US findings in operated cases for hydatid cyst77 patients – Mean age 38 years – Recurrence 11%Ozturk A et al. Eur J Radiol 2005 : 56 : 91 – 96.HypoechoicThick & irregular wallPseudocystAnechoicThick & irregular wallPseudocyst RecurrenceAnechoicUnilocular hydatid cyst
Normal ductal plate developmentSingle-layered ductal plateDouble-layered ductal platesPrimary resorptionSlit-like primitive BDExtensive resorptionFine BD around PVInsufficient resorptionDilated BD & abnormal branching“central dot sign”Abnormal developmentTurkbey B et al. Pediatr Radiol 2009 ; 39 : 100 – 111.
Structure & function of primary ciliumHair-like projection from plasma membrane into extracellular spaceMurray KF & Larson AM. Fibrocystic diseases of the Liver. Springer, NY, 2010.Intraflagellar transport (IFT) proteins bi-directionallyMechanosensory, chemosensory, & osmosensory functions
Association of fibrocystic diseases of the liverSeries of 51 patientsCombination of CHF & Caroli’s disease most strikingBiliary hamartomas reported in 10 patientsSummerfield JA et al. J Hepatol 1986 ; 2 : 141.
• Cystic dilated bile ducts < 10 mm in diameter• Usually distributed equally throughout the liver• 5 % in autopsy series, 0.5% in needle biopsy• Liver biopsy: dilated or branching BD lined by singlelayer of cuboidal cells embedded in collagenous stroma• Misdiagnosed as multiple liver metastases on imaging• MRI is the best imaging modalityBiliary hamartomasVon Meyenburg complexesDrenth JPH et al. Best Prac Res Clin Gastroenterol 2010 ; 24 : 573 – 584.
Biliary hamartomasVon Meyenburg complexesCystic space with fibrous stroma lined by layer of biliary epitheliumBile plugs within dilated bile ductsBrancatelli G et al. RadioGraphics 2005 ; 25 : 659 – 670.
Rumack CM et al. Diagnostic ultrasound.Elsevier Mosby, St. Louis, Missouri, 3rd edition, 2005.Bright echogenic foci with"ringdown" artifactSingle small hypoechoicliver massBiliary hamartomasVon Meyenburg complexes
Biliary hamartomasVon Meyenburg complexesSmaller than 10 mmDisconnected from biliary treeWhite onT2-weighted imagesGadolinium-enhanced T2-weighted MRIDrenth JPH et al. Best Prac Res Clin Gastroenterol 2010 ; 24 : 573 – 584.
Peribiliary cystsChronic liver disease specifically with PHT• Dilated peribiliary glands along portal veins & bile ductsLocated in hepatic hilum & larger portal tractNo communicate with biliary treeFew millimeters to 10 mm in size – larger cysts reported• Usually clinical benign conditionFocal biliary obstruction as result of larger cysts• US, CT & MRI: rounded anechoic structures along portal tractMay be confounded with dilated bile ductsDrenth JPH et al. Best Prac Res Clin Gastroenterol 2010 ; 24 : 573 – 584.
Peribiliary cystsFewer than 60 reported casesBrancatelli G et al. Eur J Radiol 2007 ; 61 : 57 – 69.T2-weighted MRIHigh intensity cystslying along left PV“string of beads”T1-weighted MRILow intensity cystslying along left PVPeribiliary cysts at hilumHigh intensity areas in RLMRCP
Choledochal cystsTodani’s classificationType IICBD diverticulumType IIICholedococeleType IVIH & EHTodani T et al. Am J Surg 1977 ; 134 : 263 – 9.Savader SJ et al. Am J Roentgenol 1991 ; 156 : 327.Type IMost frequent78%Type VCaroli‟s disease1 %
Choledochal cyst type ILarge cystic mass in RUQ connected to biliary tractLevy AD et al. Curr Probl Diagn Radiol 2003 ; 32 : 233 – 263.UltrasoundClassic triad: abdominal pain, jaundice, palpable mass (rare)
Choledochal cyst type IBrancatelli G et al. RadioGraphics 2005 ; 25 : 659 – 670.Cystic mass due todilatation of distal portion of CBDContrast-enhanced CTFusiform dilatation of CBDDilatation of IHBTReformatted image
Mild fusiform dilatation of common hepatic ductMarked fusiform dilatation of common bile ductLevy AD et al. Curr Probl Diagn Radiol 2003 ; 32 : 233 – 263.Choledochal cyst type IMRCP
Fusiform dilatation of extra-hepatic bile ductsAberrant entry of CBD at side of pancreatic ductBrancatelli G et al. RadioGraphics 2005 ; 25 : 659 – 670.PTCCholedochal cyst type I
Normal pancreatico-biliary junctionLevy AD et al. Curr Probl Diagn Radiol 2003 ; 32 : 233 – 263.Common channel forpancreatic & common bile ductSeparate channels forpancreatic & common bile duct
Common channel of pancreatic & biliary ductsForbes A & Murray-Lyon IM. Gut Supplement 1991, S 116 – S 122.Long common channel ofpancreatic & biliary ductsNormal common channel ofpancreatic & biliary ducts
Anomalous pancreatico-biliary junctionWith choledochal cyst65 – 95% by ERCPWithout choledochal cystCarcinoma of gallbladder & biliary tractGallbladder adenomyomatosisCholelithiasisPancreatitisLevy AD et al. Curr Probl Diagn Radiol 2003 ; 32 : 233 – 263.No further evaluation“forme fruste choledochal cyst”
Choledochal cyst with cholangiocarcinoma50-year-old woman with 1 month history of abdominal painLevy AD et al. Curr Probl Diagn Radiol 2003 ; 32 : 233 – 263.2 cm enhancing polypoid massfrom wall of choledochal cystDominant filling-defect: adenocaOther filling defects: sludgeA series of 48 patients reported incidence of 12.5%
Levy AD et al. Curr Probl Diagn Radiol 2003 ; 32 : 233 – 263.Diverticulum connected to CBD by thin neckCholedochal cyst type IIBiliary diverticulaERCP
Choledochal cyst type III / CholedochoceleCoined by Wheeler in 1940 – Similarity to ureteroceleSaccular dilation of intramural segment of distal CBDVery small risk of carcinoma (4 reported cases)Levy AD et al. Curr Probl Diagn Radiol 2003 ; 32 : 233 – 263.
CholedochoceleCBDWSingh M & Coyle W. Gastroenterology 2010 ; 138 : e3 – e4.EUS
Scholz classification of choledochoceleScholz FJ et al. Radiology 1976 ; 118 : 25 – 8.Levy AD et al. Curr Probl Diagn Radiol 2003 ; 32 : 233 – 263.Type ACystic dilation of ampullaType BDiverticulum extending from ampulla
Caroli J et al. Semin Hop Paris 1958 ; 34 : 128 – 35.http://emedicine.medscape.comCholedochal cyst type VCaroli’s diseaseDescribed by Caroli & coworkers in 1958
Caroli’s disease & Caroli’s syndromeCaroli’s diseaseCaroli’s syndrome (more common)Segmental dilatation of large intra-hepatic biliary ductsSegmental dilatation of small intra-hepatic biliary ducts& congenital hepatic fibrosisDrenth JPH et al. Best Prac Res Clin Gastroenterol 2010 ; 24 : 573 – 584.
Caroli’s disease / UltrasoundLefere M et al. Eur J Gastroenterol Hepatol 2011 ; 23 : 578 – 585.Cystic dilation of intra-hepatic bile ductscommunicating with normal bile duct
Caroli’s disease / Central dot signAkhan O et al. Eur J Radiol 2007 ; 61 : 18 – 24.PV branch protruding into lumen of dilated bile ductCystic ductal dilatationCharacteristic central dot signT1-weighted MRICystic ductal dilatationCharacteristic central dot signCECT scan
Caroli’s disease / Central dot signTurkbey B et al. Pediatr Radiol 2009 ; 39 : 100 – 111.Dilated IHBD with central dot signCoarsened liver consistent with fibrosisFlow within central dot signrepresenting portal venous flow7-year-old boy with Caroli syndromeHigh-resolution US image with 6-MHz probe
Caroli’s disease / PTCMultiple saccular dilatations of intra-hepatic bile ductsMostly at periphery of the liverBrancatelli G et al. RadioGraphics 2005 ; 25 : 659 – 670.
Complications of Caroli’s disease• Cholangitis Frequency vary greatly• Liver abscess• Lithiasis Intra & extra-hepatic lithiasis• Cholangiocarcinoma 2.5 – 15%• AmyoidosisYonem O & Bayraktar Y. World J Gastroenterol 2007 ; 13 : 1930 – 1933.
Biliary stones in Caroli’s diseaseLefere M et al. Eur J Gastroenterol Hepatol 2011 ; 23 : 578 – 585.Brancatelli G et al. RadioGraphics 2005 ; 25 : 659 – 670.UltrasoundSaccular IHBD dilatationcontaining stoneDilated bile ductswith multiple stonesUnenhanced CT scan
Adenocarcinoma in Caroli diseaseERCPLevy AD et al. Curr Probl Diagn Radiol 2003 ; 32 : 233 – 263.Irregular narrowing of left hepatic duct at site of adenocarcinomaDilated ducts peripheral to neoplasm containing lithiasis
Autosomal Dominant Polycystic Kidney DiseaseSystemic disorder• Polycystic liver disease Most common• Other cysts Splenic cystsPancreatic cystsSeminal vesicle cystsArachnoid cysts• Intra-cerebral aneurysms 8%• Cardiac valve disease Mitral valve prolapse: 25%Drenth JPH et al. Best Prac Res Clin Gastroenterol 2010 ; 24 : 573 – 584.
Screening of ADPKDRenal US & DNA analysis in 319 patients at riskRavine D et all. Lancet 1994 ; 343 : 824 – 827.Nicolau C et al. Radiology 1999 ; 213 : 273 – 276.Person at risk & younger than 30 yearsTwo cysts in one kidney or one cyst in each kidneySen: 95% (ADPKD-1) – 65% (ADPKD-2)Person at risk & aged 30 – 59 yearsTwo cysts in each kidney (Sen: 100%)Person at risk & aged 60 years or olderFour cysts in each kidney (Sen: 100%)
Autosomal Recessive Polycystic Kidney DiseasePediatric disease – 1/20 000 live births• Late pregnancy or at birth OligohydramniosMassively enlarged kidneysPulmonary hypoplasiaPotter faciesContracted limbs & clubfeet• Congenital hepatic fibrosis Almost all patients – PHT• Other ductal plate malformationsDrenth JPH et al. Best Prac Res Clin Gastroenterol 2010 ; 24 : 573 – 584.
Fibrocystin in cilia of bile ducts of liver& collecting ducts of kidneyTurkbey B et al. Pediatr Radiol 2009 ; 39 : 100 – 111.Defect of this protein leads to cyst formation in liver & kidney
Autosomal Recessive Polycystic Kidney DiseaseMultiple small cystsSalt & pepper pattern27 years old womanTuma J et al. Genitourinary ultrasound, European course book, EFSUMB.http://www.efsumb.org/ecb/
Congenital hepatic fibrosisAffecting hepatobiliary &renal systems• Congenital inherited in autosomal recessive fashion• ARPKD with less renal involvement & longer survival• Onset usually in adolescence or young adulthoodEarly childhood to 5th or 6th decade• Liver biopsy: High specificity – Low sensibilityPeriportal fibrosis & irregular proliferating bile ductsAkhan O et al. Eur J Radiol 2007 ; 61 : 18 – 24.Difficulty in clinical & pathologic diagnosis
Congenital hepatic fibrosisUnknown incidence & prevalencePortal tract expanded by fibrosisSeveral BD some with curvilinear profiles (ductal plate malformation)Murray KF & Larson AM. Fibrocystic diseases of the Liver.Springer Science + Business Media, Springer, 2010.
Imaging of congenital hepatic fibrosis• Distinctive morphologic findingsAtrophic right lobe Cirrhosis/CHFHypertrophic caudate lobe Cirrhosis/CHFHypertrophic left lateral segment Cirrhosis/CHFNormal or hypertrophic left medial segment CHF• Enlarged hepatic artery• Large regenerative nodules• Alterations of echotexture & homogeneityBrancatelli G et al. RadioGraphics 2005 ; 25 : 659 – 670.
Hypertrophic left medial segmentHypertrophic left lateral segmentSplenomegalyCECT scan – Portal phaseCongenital hepatic fibrosisZeitoun D et al. Radiology 2004 ; 231 : 109 – 116.
Turkbey B et al. Pediatr Radiol 2009 ; 39 : 100 – 111.Congenital hepatic fibrosis35-year-old man with CHF/ARPKDMultiple vessels around PV resembling cavernomaPatent portal vein“Cavernoma-like periportal vessels”
Akhan O et al. Eur J Radiol 2007 ; 61 : 18 – 24.Congenital hepatic fibrosisRegenerative nodulesDilatation of intra-hepatic bile ductsUS image ultrasound
Congenital hepatic fibrosisTurkbey B et al. Pediatr Radiol 2009 ; 39 : 100 – 111.Echogenic liver compared with RKSecondary to fibrosisSignificantly coarsenedparenchymal echotextureHigh-resolution US (6-MHz)Conventional US (4-MHz)10-year-old boy with CHF/ARPKD
Congenital hepatic fibrosisTurkbey B et al. Pediatr Radiol 2009 ; 39 : 100 – 111.10-year-old boy with CHF/ARPKDModerately echogenic liver parenchymaThickened periportal space consistent with fibrosis“Periportal cuffing”
Autosomal Dominant Polycystic Liver DiseaseADPLDPRKCSH: Protein kinase substrate 80K-HOnori P et al. Dig Liver Dis 2010 ; 42 : 261 – 271.
Types of PCLDDrenth JPH et al. Hepatology 2010 ; 52 : 223 – 2230.One or few dominant cystsType IMultiple cysts limited to one part of liverType IICysts through several segments of liverSome segments relatively free from cystsType IIIExtensive polycystic liverHardly recognizable liver parenchymaType IV
Polycystic Liver Disease (PCLD)Drenth JPH et al. Best Prac Res Clin Gastroenterol 2010 ; 24 : 573 – 584.Diagnosis made when > 20 liver cysts are presentOr > 4 liver cysts in member of family with known PCLD
Polycystic Liver Disease (PCLD)Murray KF & Larson AM. Fibrocystic diseases of the Liver.Springer Science + Business Media, Springer, 2010.Numerous cysts, many of them calcifiedMost calcifications not completely circular
Turkbey B et al. Pediatr Radiol 2009 ; 39 : 100 – 111.Numerous anechoic lesionsConsistent with hepatic cysts39-year-old woman with CHFAutosomal Recessive Polycystic Liver DiseaseARPLD
Miscellaneous cystic lesions of the liver• Ciliated hepatic foregut cyst• Peliosis hepatis• Primary neoplasm: biliary cystadenoma – hemangioma – HCC• Cystic metastasis• Hepatic abscess: pyogenic – amebic• Intrahepatic pancreatic pseudocyst• Endometriosis• Hematoma & bilomaMurray KF & Larson AM. Fibrocystic diseases of the Liver.Springer Science + Business Media, Springer, 2010.
Ciliated hepatic foregut cystSharma S et al. Dig Dis Sci 2008 ; 53 : 2818 – 2821.T2 MRI: hyperintense cystHypodense non-enhanced lesionHypoechoic cystic massColumnar epithelium with cilia
Conditions associated with peliosis hepatisAzathioprine6-ThioguanineAIDS/Bartonella infectionTuberculosisMyeloproliferative diseasesLeukemiaLymphomaMultiple myelomaMacroglobulinemiaAnabolic steroidsOral contraceptivesArsenicThoratrastVinyl chlorideYamada’s textbook of gastroenterology, Blackwell Publishing, 5th Edition, 2009.
Peliosis hepatisFrom Greek pelios (purple)Kleinig P et al. Clinical Radiology 2003 ; 58 : 995 – 998.US: hypoechoic lesion inbackground of liver steatosisCEUS: transient “fast surge”central echo-enhancement
Biliary cystadenomaOften incorrectly diagnosed as simple cystsTchelepi H et al. Ultrasound Quarterly 2004 ; 20 : 155 – 169.Irregular cystic mass with thick septationsUseful cystic fluid analysis for CA 19-9 & CEAExcision to prevent recurrence & malignant transformation (25%)
Biliary cystadenoma< 200 reported cases – women in 85%56-year-old asymptomatic woman – CECT scanMultilocular cyst in porta hepatisEnhancing septaeMultilocular cyst in porta hepatisMural calcificationLevy AD et al. Curr Probl Diagn Radiol 2003 ; 32 : 233 – 263.
Biliary cystadenomaDietrich CF et al. Ultrasound of the liver, European course book, EFSUMB.http://www.efsumb.org/ecb/Gray-scale ultrasoundHepatic cystCEUSIntra-cystic thick septation
Large heterogeneous hemangioma (rare)Partially or entirely cystic massNonenhanced CT scanHypoattenuating lesion withmarkedly hypoattenuating centerContrast-enhanced CT scanTypical enhancement patternof hemangiomaVilgrain V et al. RadioGraphics 2000 ; 20 : 379 – 397.
Cystic hepato-cellular carcinoma (rare)Partially or entirely cystic massTeoh AYB et al. World J Surg 2006 ; 30 : 1560 – 1566.Internal necrosis: Rapid growth, stemic & locoregional treatmentSigns or complications of liver cirrhosis, ↑α fetoprotein
Cyst-like appearance of hepatic metastases• Hypervascular metastasis with rapid growth & necrosisNeuroendocrine tumorsSarcomaMelanomaCertain subtypes of lung & breast carcinoma• Mucinous adenocarcinomaColorectal carcinomaOvarian carcinomaMortelé KJ et al. RadioGraphics 2001; 21 : 895 – 910.
Cystic metastases from breast cancerLencioni R, Cioni D & Bartolozzi C. Focal Liver Lesions. Springer-Verlag, Berlin, 2005.Cyst-like hypoattenuating lesionwithout peripheral enhancementPortal phase CECT scan Arterial phase CECT scanPeripheral rim-enhancementTypical of metastatic lesionKnown primary tumor, multiple lesions, rim enhancement
Cystic metastasis from ovarian cancerPortal venous phase CECT scanMortele KJ et al. RadioGraphics 2001; 21 : 895 – 910.Ovarian metastases spread by peritoneal seedingElliptical cystic lesion on surface of liver
Pyogenic hepatic abscessThick-walled cystic lesionPeripheral enhancement “double target” signLencioni R, Cioni D & Bartolozzi C. Focal Liver Lesions.Springer-Verlag, Berlin Heidelberg , 2005.Portal venous-phase CECT scan
Pyogenic liver abscessGas within lesionRumack CM et al. Diagnostic ultrasound.Elsevier Mosby, St. Louis, Missouri, 3rd edition, 2005.Poorly defined hypoechoic masswith multiple gas bubblesLiver mass with extensivegas content
Amebic hepatic abscessFeldman M et al. Sleisenger and Fordtrans Gastrointestinal & Liver Disease.Saunders Elsevier, 9th edition, 2010.“Double target” signSerologic tests (ELISA & IHA): Sen & Sp 95%
Intra-hepatic pancreatic pseudocystSystematic review – 23 reported casesGuesmi F et al. Tunis Med. 2009 ; 87 : 801 – 4.Acute severe pancreatitis 3 weeks earlierCystic lesion of left lobe – Thin capsulePancreatitis , left liver lobe, thin capsule, signs of pancreatitis
Endometrial cyst of liverExtremely rare – 7 reported casesCystic lesion in right lobeUltrasound T2-weighted MRIHigh intensity signal in right lobeRao M et al. Gut 2006 ; 55 : 742.Cyst aspirated under CT guidanceEndometrial glandular & stromal elements
Hepatic hematomaSubcapsular hemorrhage after US-guided liver biopsyT2-weighted MRIHigh signal intensity& internal debrisMortelé KJ et al. RadioGraphics 2001 ; 21 : 895 – 910.T1-weighted MRIHigh signal intensityParamagnetic effect of methemoglobinLiver trauma, known HCC or adenoma
Hepatic bilomasThree intra-hepatic collections compatible with bilomas21-year-old man with biliary leakage after severe accidentPortal phase CECT scanMortele KJ et al. RadioGraphics 2001; 21 : 895 – 910.Trauma or biliary surgery – No septa capsules or calcifications
Conclusion• Clinical context is of utmost importance• Laboratory tests could be very beneficial in some cases• Imaging modalities (US, CEUS, EUS, CT, MRI) alonecan made the correct diagnosis in the majority of cases• Liver biopsy when diagnosis remains elusive