Disorders of the Biliary Tract

Definition

• results from interruption of biliary cholesterol homeostasis

  • increase in hepatic cholesterol secretion

    • increased estrogen leads to increased hepatic synthesis and secretion 

    • obesity, HLD

  • hypomotility of the gallbladder

    • increased progesterone leads to gallbladder hypomotility and a longer time in which the supersaturated bile will remain in the gallbladder, promoting crystallization

    • gallbladder motility is stimulated by fat content in ingested meals,  vagal (cholingergic) nerve stimulation, cholecystokinin (CCK) and motilin

  • decreased bile salt secretion

    • increased estrogen leads to decreased bile salt synthesis

    • Crohn's disease and short gut syndrome impair enterohepatic circulation of bile salts leading to a decrease in bile salt secretion 

    • ileal Crohn's disease and ileal resection - reduced hepatic bile acid secretion and supersaturation of bile with cholesterol

  • increased cholesterol absorption 

Risk Factors

• Increased age

• Female sex

• Pregnancy

• Dyslipidemia

• Diabetes

• Obesity

• Rapid weight loss

• TPN use

  • biliary stasis due to a decrease in enteral stimulation

• Medications

  • oral contraceptives

  • hormone replacement therapy

  • ceftriaxone

  • fibrates

    • clofibrate

      • reduces activity of cholesterol 7α-hydroxylase (rate limiting enzyme in bile salt synthesis in the classical pathway) 

      • promotes cholesterol supersaturation in the bile

      • decreases bile salt concentrations

  • calcineurin inhibitors

  • octreotide

• Crohn's disease

• Sickle cell

  • often pigmented stone secondary to hemolysis leading to excess systemic bilirubin

  • unconjugated bilirubin is not water soluble and combines with calcium to form calcium bilirubinate within the mucin glycoproteins that are secreted by the gallbladder mucosa

Lab Findings

• Cholesterol stones

  • contain more than 50% cholesterol by weight

  • associated with obesity, female gender, HLD

• Pigmented stones

  • less common in the US than cholesterol stones

  • contain less than 30% cholesterol by weight

  • Both types of pigmented stones...

    • contain excess unconjugated bilirubin 

    • are composed of calcium hydrogen bilirubinate

      • oxidized and polymerized in hard black stones

      • unpolymerized in softer brown stones

  • Black

    • frequently radiopaque

    • form in sterile bile

    • seen in chronic hemolysis (ex. sickle cell, hereditary spherocytosis), cirrhosis, cystic fibrosis, diseases of the ileum (ex. Crohn's)

  • Brown

    • radiolucent

    • more likely to occur in bile ducts

    • form secondary to biliary stasis (ex. biliary stricture) and infection (ex. Clornorchis sinensis, reucrrent pyogenic cholangitis)

Treatment

• potential benefit of prophylactic cholecystectomy during Roux-en-Y gastric bypass given risk of rapid weight loss following surgery

  • use of UDCA following surgery may also help reduce risk fo gallstone formation in this group

Definition

• entry of a gallstone into the bowel via a biliary-entric fistula

Definition

• Strong predictor of choledocholithiasis

  • bilirubin >1.8-4 mg/dL

  • ascending cholangitis

  • CBD stone visualized on abdominal ultrasound

  • CBD dilation >6mm with intact gallbladder

• Moderate predictors of CBD stone

  • abnormal liver tests other than bilirubin

  • age 55 yo

  • gallstone pancreatitis

Lab Findings

• Bilirubin 1.8-4 mg/dL

Imaging Findings

• Right Upper quadrant ultrasound

  • sensitivity for detection of choledocholithiasis is ~50%

  • sensitivity for detection of cholelithiasis, bile duct dilation, and GB wall thickness is high

• MRCP

  • sensitivity for choledocholithiasis is ~92%

  • Risk stratify with MRCP if diagnosis is not certain unless...

    • there is clear evidence of acute bacterial cholangitis

    • CBD dilated to 10mm or more AND total bilirubin is 4 g/dL or more

• EUS

  • sensitivity for choledocholithiasis is ~95%

Treatment

• ERCP prior to cholecystectomy if strong predictors are present

• Intermediate risk for CBD stone - proceed with one of the following...

  • preoperative MRCP

  • preoperative EUS

  • intraoperative cholangiogram

• surgical consultation for consideration of cholecystectomy during the hospital admission

  • recurrent biliary events (biliary colic, recurrent gallstone pancreatitis, choledocholithiasis) observed to occur in 18-40% if cholecystectomy is delayed beyond initial hospitalization

• If no strong or moderate predictors for cholelithiasis and imaging findings consistent with cholecystitis, should undergo cholecystectomy directly

Signs and Symptoms

• Charcot's triad

  • fever

  • jaundice

  • RUQ pain

• Reynold's pentad

  • fever

  • jaundice

  • abdominal pain

  • altered mental status

  • hypotension

• may be associated with pancreatitis

Imaging Findings

• Abdominal Ultrasound

  • dilated common bile duct

  • gallstones in the gallbladder

  • gallstones in the bile duct

  • There is no need for MRCP if US findings strongly suggest bile duct stones

Treatment

• patients presenting with acute pancreatitis and concurrent cholangitis should undergo ERCP within the first 24 hr of admission

  • delayed ERCP associated with increased risk of death, organ failure, and length of ICU stay

  • ERCP can safely be performed on anticoagulants

    • sphincterotomy should be avoided given the high risk for bleeding

    • stent or balloon dilation are alternatives for decompression and stone extraction

  • if pre-test probability for choledocholithiasis is high enough additional imaging with EUS or MRCP is not warranted 

  • Consider percutaneous drain placement if ERCP fails

• also treat with antibiotics and IVF

• surgical consultation for consideration of cholecystectomy during the hospital admission

  • recurrent biliary events (biliary colic, recurrent gallstone pancreatitis, choledocholithiasis) observed to occur in 18-40% if cholecystectomy is delayed beyond initial hospitalization

Definition

• often in East Asia and Southeast Asia

• recurrent episodes of cholangitis with intra and extrahepatic ductal dilation and choledocholithiasis

  • left hepatic ducts are more often involved than those on the right

• biliary pigmented stones

  • often with brown pigmented stones

    • form from stasis and infection

    • radiolucent

    • often in the left biliary system

    • colonies of bacteria can be found growing on the stones

    • versus black pigmented or cholesterol stones which are formed in sterile bile within the gallbladder

• can lead to liver abscesses

• risk for cholangiocarcionma

• exact etiology unknown though suspected secondary to biliary parasitic infection

  • Chlonorchis sinensis

  • Opisthorchis species

  • Fasciola hepatica

  • Ascaris Lumbricoides

Signs and Symptoms

• hepatolithiasis

• Jaundice

• RUQ tenderness

• hyperbilirubinemia

Treatment

• ERCP

• left hepatectomy 

  • for recurrent disease for which frequent repeat ERCP is not feasible

Versus

• Entamoeba histolyticum - liver abscess

• Echinococcus - multiseptated, cystic lesions of the liver

• Cryptosporidium parvum - AIDS cholangiopathy

• Schistosomiasis - portal fibrosis and granulomas of the liver with portal hypertension and eosinophilia

Definition

• first degree relatives are ~100x more likely to develop PBC than the general population

• autoantigens of AMA identified as E2 subunits of the 2-oxo-acid dehydrogenase complexes, including the...

  • E2 subunits of the pyruvate dehydrogenase complex (PDC-E2)

    • AMA directed to PDC-E2 in 95% of PBC patients

  • branched chain 2-oxo acid dehydrogenase complex

  • 2-oxo glutarate dehydrogenase complex

  • E3 bending protein of dihydrolipoamide dehydrogenase

• AMA target antigens are all localized within the inner mitochondrial matrix

• Total bilirubin level is the best predictor of survival in PBC

• more slowly progressive chronic liver disease compared to other etiologies

• AMA negative PBC have similar natural history fo AMA positive PBC

• PBC much more common in women than men

Diagnosis

• positive antimitochondrial antibody (AMA) in the setting of elevated alk phos

• if AMA is negative with elevated alk phos, liver biopsy is required for diagnosis

  • <5% of patients with PBC are AMA negative

  • same prognosis and response to UDCA as those who are AMA positive

Risk Factors

• female gender

Signs and Symptoms

• varices in the absence of cirrhosis

  • PBC can be associated with pre-sinusoidal portal hypertension in the absence of cirrhosis

Lab Findings

• elevated alkaline phosphatase

• positive AMA

• elevated serum IgM

Pathology Findings

• florid duct lesions

• mononuclear inflammatory infiltrate surrounding bile ducts

Treatment

• ursodeoxycholic acid (UDCA)

  • biochemical response to UDCA predicts prognosis in PBC

    • ~30% have suboptimal response and are at risk for more rapid progression of disease

  • 13-15 mg/kg/day

  • reduces the risk of variceal bleeding

  • reduces LDL levels

  • slows progression and reduces risk of needing liver transplant

  • has NOT been shown to improve fatigue or reduce risk of osteoporosis

• Fenofibrate 

  • if inadequate response to UDCA

    • persistent elevation in alkaline phosphatase >1.67x the upper limit of normal after one year of UDCA therapy

  • 145 mg daily

  • available in the US and approved for treatment of hyperlipidemia

  • reduced risk of liver decompensation, mortality, and liver transplantation when used in combination with UDCA in those with inadequate biochemical response to UDCA alone

• Benzafibrate

  • agonist of peroxisome proliferator activated receptor 

  • improves biochemical response 

  • not available in the US

• obeticholic acid

  • if inadequate response to UDCA

  • farsenoid X receptor agonist

    • decreases alkaline phosphatase and total bilirubin levels when used alone or in combination with UDCA

  • Side effect - pruritus, a dose related effect more common at 10 mg than 5 mg

    • start at 5mg in those with advanced liver disease

• Methotrexate

  • not routinely recommended, mixed results

• Liver transplantation

  • for advanced liver disease secondary to PBC

Definition

• may present with isolated extrahepatic duct stricturing 

  • also consider HIV cholangiopathy and PSC cholangiopathy (though less commonly presents with just isolated stricturing) in a young patient 

• one manifestation of IgG4 related disease (IgG4-RD), a systemic disease characterized by extensive tissue infiltration by IgG4 positive plasma cells and T cells, which cause fibrotic lesions associated with obliterative phlebitis

• occurs more often in older men

• clinically distinct from PSC

  • robust response to corticosteroids 

  • versus: PSC where there is no proven medical treatment 

Signs and Symptoms

• jaundice

• prior episodes of idiopathic pancreatitis

• lymphadenopathy

• Can affect the pancreas, bile duct, gallbladder, salivary glands (Mikulicz disease), kidney (tubulointerstitial nephritis), lung, prostate, and retroperitoneum

• more commonly affects older men

Lab Findings

• characterized by elevated IgG4 levels in upt to 66%

Imaging Findings

• RUQ US - extra and intrahepatic ductal dilation

• MR/MRCP

  • abrupt tapering of the CBD within the pancreas head 

  • prominent pancreas

  • delayed pancreatic enhancement with a thin rim of peripheral enhancement

    • "sausage shaped" pancreas with capsular enhancement = autoimmune pancreatitis

  • no pancreas head mass seen

  • imaging features may be similar to PSC

Treatment

• Corticosteroids

• Rituximab in the case of steroid failure or intolerance

Definition

• ~80% of cases are in patients with advanced disease (CD4<100)

• most commonly presents with isolated papillary stenosis

• sclerosing cholangitis can be seen alone or in conjunction with papillary stenosis

• Associated pathogens

  • Cryptosporidium parvum (most commonly) 

  • CMV

  • Microsporidium

  • Cyclospora

Signs and Symptoms

• risk factors for HIV/AIDS

• Kaposi sarcoma

Endoscopic Findings

• ERCP - papillary stenosis, no irregularity or stricture within the remainder of the bile duct or intrahepatic ducts

Pathology Findings

• ERCP brushings - unremarkable

Lab Findings

• markedly increased ALP

• mildly increased AST, ALT, bilirubin

• negative CEA and CA 19-9

  • may have low level nonspecific elevation of CA 19-9

Imaging Findings

• RUQ US - intra and extrahepatic ductal dilation

• CTAP - mild dilation of bile duct without pancreas head mass

Treatment

• antimicrobial therapy directed at the underlying pathogen does not improve the disease course or cholangiographic abnormalities 

• HAART

• endoscopic measures to relieve biliary obstruction if present

• mild improvement with UDCA in serum biochemical parameters and symptoms

Definition

• Rare cause of GI bleeding

• Triad of bleeding, jaundice, and abdominal pain

  • only 1/3 will have all through features

• recent history of biliary tract or hepatic parenchymal instrumentation

• trauma

• may occur secondary to cholelithiasis, choledocholithiasis, or ruptured hepatic artery aneurysm, but these are less common

Treatment

• angiography can identify the source of bleeding and achieve hemostasis through embolization

• ERCP can be done to clear the biliary tree in those with ascending cholangitis secondary to thrombus

  • typically not used for hemostasis

Definition

• previously known as Sphincter of Oddi dysfunction type I (SOD 1)

• objective findings of biliary or pancreatic duct obstruction

Definition

• biliary type pain with elevated liver enzymes or biliary duct dilation coincident with episodes

• diagnose with ERCP with biliary manometry 

Signs and Symptoms

• intermittent RUQ pain  occurring every 2-3 months, lasting 30 min to 1 hr

• feels well between episodes

• not improved with acid suppression or bowel movements

Lab Findings

• elevated AST and ALT

Imaging Findings

• structural etiology for biliary pain excluded

Treatment

• ERCP with sphincterotomy

  • perform only if manometry shows basal biliary sphincter pressure >40 mmHg

  • ERCP in this condition carries a very high risk of post ERCP pancreatitis, ~10-30%

  • Sphincterotomy carries addition risks of bleeding and perforation 

  • long term improvement has been shown in up to 70-90% of patients

• Fiber supplements, dicyclomine, and narcotic pain medications are not effective

Definition

• biliary type pain

• no significant benefit from sphincter ablation

Defintion

• occurs in 10-20% of adults who undergo cholecystectomy 

Signs and Symptoms

• biliary type pain

• pain not improved with defecation

Imaging Findings

• Absence of structural etiology for pain

• No CBD dilation, pericholecystic fluid, gallbladder wall thickening, cholelithiasis, sludge

• CCK-stimulated Cholescintigraphy - low gallbladder ejection fraction

Lab Findings

• Unremarkable LFTs and lipase

Versus

• role of biliary and/or pancreatic sphincter dysfunction in those with intact gallbladder is controversial as intact gallbladder is felt to serve as a pressure offset

  • prior cholecystectomy is required to diagnosis function biliary or pancreatic sphincter disorder

Definition

• these adenomas can progress to adenocarcinoma

Treatment

• upfront resection favored over surveillance

  • adenoma could harbor underlying carcinoma not diagnosed by pinch biopsies

• non-invasive ampullary adenomas (<4cm without ductal involvement)

  • endoscopic removal is preferred

  • if lesion does not lift at time of endoscopy, consider surgical resection

• pancreaticoduodenectomy or trans duodenal ampullectomy

  • indicated if biopsies positive for carcinoma in situ

  • associated with significant morbidity and mortality

Risk Factors

• post cholecystectomy

• post liver transplant

  • occurs due to postoperative ischemia related to poor hepatic artery perfusion

  • biliary strictures commonly associated with hepatic artery stenosis

Diagnosis

• hepatobiliary scintigraphy scan

  • can confirm diagnosis but do not delay care if clinical suspicion is high

• percutaneous drainage to confirm if fluid analysis is positive for bile

Treatment

• Symptomatic bile leak post cholecystectomy

  • percutaneous drainage

    • particularly if concerned for infection

  • ERCP with sphincterotomy alone can be effective for low-grade bile leaks

  • ERCP with sphincterotomy or stent placement is effective for treatment of bile leaks, but it will do little for an already formed symptomatic fluid collection 

  • surgical revision if...

    • percutaneous drainage and ERCP fail to resolve the leak

    • transection injury occurs (unlikely to be amenable to endoscopic therapy)

Definition

• gastric outlet obstruction secondary to a gallstone that has formed a cholecystoduodenal fistula

• consider in all cases of GOO and unexplained pneumobilia 

• rare cause of obstruction, ~2% of all gallstone ileus cases

Imaging Findings

• CTAP

  • markedly dilated stomach without small bowel dilation

  • gallbladder heterogeneous in appearance with several calcified stones

  • gallbladder wall thickened and appears inseparable from the adjacent duodenum

  • air seen within the gallbladder lumen

  • CBD not dilated

Treatment

• Start with endoscopy - only successful in ~9%

• Surgery often required for definitive management

  • carries ~12% mortality rate (likely related to advanced age and comorbidities)

• neither EUS or ERCP will be of benefit as stone has eroded through the duodenal bulb

• NGT allows for decompression of the stomach but does not resolve obstruction 

Definition

• occurs in 3-10% of patients following metal stent placement

  • associated with tumor involvement of the cystic orifice

  • no clear association between type f metal stent and cholecystitis 

  • this is higher than the rate of post ERCP pancreatitis (~1-2%)

Imaging Findings

• RUQ US - pericholecystic fluid and thickened gallbladder wall

  • may consider CT as alternative

• AXR - stent in position, air cholangiogram

Lab Findings

• no evidence of stent obstruction

Definition

• among the most common post-transplant complications

  • occurs in 5-15% following deceased-donor transplant

  • occurs in up to 32% following living-donor transplant

• post-transplant biliary strictures can be classified as...

  • anastomotic 

  • non-anastomotic

    • typically develop at the hilum and/or the intrahepatic ducts

    • can be secondary to...

      • ischemic injury

        • hepatic artery thrombosis or stenosis (most common)

        • prolonged ischemia time

      • immune-mediated processes

        • ABO-incompatible graft

        • chronic ductopenic rejection

• recurrent PSC

  • cholangiographic or histologic evidence of PSC at least 3 mo following transplant with exclusion of other secondary causes of biliary strictures

Treatment

• Strictures < 4 wks after transplant

  • stent alone

• Strictures > 4 wks after transplant

  • balloon dilation with stent placement (better than stent alone)

Definition

• rate of major biliary injury is 2-7x higher with laparoscopic compared to open approach

• right posterior duct stricture may result in...

  • cholestasis

  • cholangitis

  • atrophy of the right hepatic lobe over time

• subvesical bile duct (duct of Luschka) leak

  • one of the most common complications of cholecystectomy

  • ducts pass through the gallbladder fossa and empty into the right hepatic or common hepatic duct

  • they are small and difficult to detect, making them prone to injury

• post-cholecystectomy syndrome

  • group of symptoms which recur or do not remit following cholecystectomy

    • abdominal pain

    • dyspepsia

Diagnosis

• right posterior duct stricture

  • cholangiogram with lack of filling of the right posterior duct

Treatment

• right posterior duct stricture

  • managed surgically

• subvesical bile duct (duct of Luschka) leak

  • ERCP with temporary stent placement or sphincterotomy

    • enables the site to heal by decreasing the pressures in the proximal biliary tree

    • allows bile to preferentially flow across the papilla rather than the site of the leak

Definition

• diverticulum of the extrahepatic bile duct

• low malignant potential (~2%)

Treatment

• Diverticulotomy followed by primary common bile duct closure

Definition

• multiple dilations of the intra and extrahepatic biliary tree

• Type I, IV, and V carry a high risk for biliary tract cancers

Treatment

• surgical cyst resection of complete extrahepatic bile duct cyst

  • Roux-en-Y hepaticojejunostomy 

Definition

• multiple dilations of the extrahepatic bile duct

• Type I, IV, and V carry a high risk for biliary tract cancers

Treatment

• surgical cyst resection of complete extrahepatic bile duct cyst

  • Roux-en-Y hepaticojejunostomy 

• if intrahepatic ducts are involved, hepatic lobe resection is also recommended 

Definition

• congenital disorder

  • thought to be due to genetic mutation in ciliary proteins

• multifocal, segmental dilation of large intrahepatic bile ducts associated with congenital hepatic fibrosis

• normal extrahepatic duct

• Type I, IV, and V carry a high risk for biliary tract cancers

Diagnosis

• Imaging with characteristic findings

Signs and Symptoms

• Renal manifestations occur in up to 60% of patients with Caroli's syndrome

  • medullary sponge kidney

  • cortical cysts

  • autosomal recessive polycystic kidney disease

  • autosomal dominant polycystic kidney disease (rare)

• Increased risk for cholangiocarcionma 

  • though to be due to bile stasis and high concentrations of unconjugated secondary bile salts

• predisposed to cholangitis

Imaging Findings

• MRCP

  • diffuse dilation of the biliary tree

  • extensive fusiform and saccular dilation of the intrahepatic bile ducts

• CT 

  • central dot sign - intraluminal portal vein radicals appear as foci of contrast enhancement within dilated bile ducts

  • biliary cysts - irregularly shaped and communicate with bile ducts

Treatment

• Hepatic resection or liver transplantation (when indicated)

Versus

• Polycystic liver disease - cysts are more regularly shaped and do NOT communicate with bile ducts

Definition

• autosomal dominant

• associated with JAG1 gene mutation

  • encodes the NOTCH signaling pathway ligand Jagged-1

  • found in 95% of cases

• defects in the NOTCH2 gene can be found in the small number of patients without JAG1 mutations

Signs and Symptoms

• neonatal jaundice 

• cholestasis

• cirrhosis, complications secondary to portal hypertension

• liver failure

• dysmorphic facies

  • triangular face

  • deep set eyes

  • prominent forehead

• cardiac abnormalities 

  • present in 90% of patients

  • peripheral pulmonary stenosis

• skeletal abnormalities

  • abnormal fusion of the spine resulting in butterfly shaped thoracic vertebrae

• renal abnormalities 

• pruritus

• ocular abnormalities

  • posterior embryotoxon

    • a corneal abnormality notable on slit-lamp exam

    • can be seen in 90% of Alagille syndrome patients

    • can also be seen in 15% of the normal population

Lab Findings

• conjugated hyperbilirubinemia

Pathology Findings

• Ductopenia on liver biops

Definition 

• group of autosomal recessive disorder

• impaired bile formation leading to generalized cholestasis 

Diagnosis

• PFIC type III

  • as subset presents as young adults

  • elevated GGT

  • ductular proliferation on liver biopsy 

  • associated with ABCB4 gene mutation

    • affects a protein involved in biliary phospholipid excretion (MDR3)

      • phosphatidycholine translocation in the bile canaliculus

    • diminished biliary phospholipid excretion leads to an increase in bile lithogenicity 

• PFIC type I 

  • present within the first few months of life

  • normal GGT

  • absent ductal proliferation in liver biopsy

  • associated with ATP8B1 gene mutation

    • encodes the canalicular membrane protein FIC1

• PFIC type II

  • present within the first few months of life

  • normal GGT

  • absent ductal proliferation in liver biopsy

  • associated with ACBC11 gene mutation

    • encodes an ATP-dependent canalicular bile salt export pump (BSEP)

  • associated with increased risk for HCC

Signs and Symptoms

• symptomatic cholestasis

• cirrhosis and complications from portal hypertension can develop early in life

Treatment

• UDCA - initial treatment for all PFIC subtypes

Screening

• PFIC type II warrants HCC screening beginning at a young age

Definition

• a group of disorders, congenital and acquired

• results in cholestasis due to progressive destruction of intrahepatic bile ducts

Definition

• a fibro-obliterative disease of the extrahepatic bile ducts

• presents with biliary obstruction in the neonatal period

Signs and Symptoms

• usually presents in isolation but can occur with laterality malformation or congenital malformations

  • intestinal atresia

  • cardiac abnormalities 

Definition

• irregular cystic lesions

• slow-growing tumors originating from the intrahepatic biliary tree

  • rarely the extraheptic biliary tree

• found in middle-age women (90%)

• up to 20% undergo malignant transformation into biliary cystadenocarcinomas

Lab Findings

• elevated CA 19-9

  • can be seen in biliary cystadenoma or cystadenocarcinomas

Imaging Findings

• multiloculated cyst with internal septations and a thickened irregular wall

• calcifications and papillary wall nodules may also be seen

Pathology Findings

• layer of cuboidal cells with underlying ovarian-type stroma the expresses receptors for estrogen and progesterone

Treatment

• complete surgical resection 

• rate of recurrence after surgery is low

Surveillance

• < 6mm, without concern for malignancy

  • no follow-up

• 6-9mm, without concern for malignancy

  • repeat abd US in 6 mo

  • If similar size/morphology, repeat US in 12 mo

  • If similar size/morphology, discontinue surveillance

• ≥ 10 mm 

  • follow up with CT or EUS

    • also pursue further imaging for age ≥ 60 and sessile polyps

  • if no evidence of malignancy, repeat imaging with same modality in 6 mo

  • If similar size/morphology, repeat imaging in 12 mo

Treatment

• cholecystectomy if theres is concern for malignancy

Definition

• Complete intramural calcification

  • continuous band of calcium infiltrates in the gallbladder wall

• Selective mucosal calcifications

  • segmental deposits of calcium

• Associated with an increased risk of gallbladder adenocarcinoma

  • risk appears to be higher in those with selective mucosal calcification

• often found incidentally on imaging

Signs and Symptoms

• often asymptomatic

Imaging Findings

• confirm incidental findings with CT abd/pelvis with IV contrast

Treatment

• controversial but generally considered an indication for cholecystectomy given malignancy risk

Definition

• Type 1 IPNB

  • similar to IPMN

  • superficial spreading growth pattern

  • mucin production

  • incremental dysplastic changes progressing to invasive biliary carcinoma

  • can be intra- or extrahepatic

Pathology Findings

• Cholangioscopic biopsies

  • adenocarcinoma

  • papillary proiferation

  • fibrovascular core

Signs and Symptoms

• Quincke's triad

  • RUQ pain

  • jaundice

  • evidence of GI bleed

Risk Factors

• blunt-force trauma

• biliary tract malignancy

• recent instrumentation

  • liver biopsy

  • transhepatic biliary drainage

• portal biliopathy

• cholangitis

  • parasitic

• prolonged ductal obstruction

Treatment

• consult interventional radiology for embolization

Signs and Symptoms

• rose-color spots

• fever

• abdominal pain

Treatment

• Asymptomatic carrier

  • cholecystectomy to remove persistent Salmonella

• Unexposed patient

  • Typhoid vaccination

Definiton

• Infection >3 wks

  • retained dead organisms within the biliary tree

Risk Factors

• Southeast US

  • Appalachia

• Pig farmers

Signs and Symptoms

• pyogenic cholangitis

Diagnosis

• Stool ova and parasite

  • eggs present in stool for ~40 days after onset of pulmonary symptoms

Treatment

• Albendazole

• mebendazole

• ivrmectin

Definiton

• Typhoid fever

  • high fever

  • abdominal pain

  • headache

  • rose-colored spots

• Chronic carrier

  • increased risk in the presence of cholelithiasis 

Risk Factors

• Sick contacts

Signs and Symptoms

• Carriers may be asymptomatic

• Colonization results in increased risk of gallbladder carcinoma

Diagnosis

• Suggestive history

Treatment

• Chronic carrier with cholelithiasis

  • Cholecystectomy 

  • antibiotics alone will be ineffective due to biofilm formation

Definiton

• Etiologies

  • Extrinsic compression of biliary tract by dilated veins (paracholedochal, epicholedochal, cholecystic)

  • Portal vein thrombosis

  • Cavernous transformation

Risk Factors

• Portal hypertension

Signs and Symptoms

• Jaundice

Diagnosis

• Imaging

Treatment

• TIPS