Cystic Diseases of Liver

Dr Anang PangeniJR

Introduction

• Frequency : not known

• Estimated to occur in 5% of the population.

• Usually found as an incidental finding on imaging or

at the time of laparotomy

• Most series in the literature are relatively small,

reporting fewer than 50 patients each.

Introduction A number of different cystic lesions may affect the liver,

InfectiousPyogenic Liver Abscess

Amebic Liver Abscess

Hydatid Liver Cysts

CongenitalSimple hepatic cysts

Polycystic liver disease

NeoplasticCystadenoma

Cystadenocarcinoma

Traumatic

Pyogenic Liver Abscess• Hippocrates 400BC , and we still debating on what’s the best

form of treatment.

• Open surgical drainage – recommended treatment for many years.

• 1953, McFadzean and associates• advocated closed aspiration and antibiotics for solitary abscess; • however, did not gain widespread acceptance • 1980s, greater acceptance : widespread advancements in Imaging allowing

for precise localization and a percutaneous approach to treatment.

• Current treatment includes antibiotics, usually with a percutaneous drainage procedure.

“Appendicitis” as a cause, overwhelmingly described in literature, has now been shifting to other etiologies in modern era.

1. Bile ducts, causing ascending cholangitis;2. Portal vein, causing pylephlebitis from appendicitis or

diverticulitis; 3. Direct extension from a contiguous disease; 4. Trauma due to blunt or penetrating injuries; 5. Hepatic artery, due to septicemia; and 6. Cryptogenic## 40% of pyogenic liver abscesses of biliary origin are related to

an underlying malignancy

Pyogenic Liver Abscess

Pyogenic Liver Abscess

35-40%

20%

Predisposing factors Pyogenic Liver Abscess

PathologyPyogenic Liver Abscess

• Number and Size– solitary and large,

• portal,• traumatic, and • cryptogenic hepatic abscesses

– multiple and small (and these are usually bilobar!!! Also remember Fungal abscesses)• biliary origin • arterial abscesses

• Site – Right lobe : 2/3rd (63%) – Left lobe : 14%– Both lobes : 22%

• Organisms – Most common are Gram negative aerobes (50-70%), positive aerobes (55%) and

anaerobes.• Biliary : E. cooli , Klebsiella• GIT : klebsiella , E. coli, enterococcus and anerobes • Cryptogenic : anaerobes• AIDS related : Mycobacterium

PresentationPyogenic Liver Abscess

– Subacute and nonspecific • Fever • Malaise • Weight loss , anorexia , nausea vomiting • Chest symptoms when diaphragm is involved • Right upper Quadrant tenderness • Hepatomegaly • Jaundice • Pleural effusion

– Acute • Rupture resulting in peritonitis • Septic shock

Imaging

• Ultrasonography and computed tomography (CT) scans - modalities of choice

• USG:– Sensitivity : 85% and 90%– Specificity : far less• Aspiration for diagnosis and microbiologic testing

usually done.

– Troublesome in • morbidly obese• Inhomogenous liver

Pyogenic Liver Abscess

Imaging

• CT scan– Sensitivity : 95% -100%– better than USG (not limited by air / ribs )– Hypoattenuated than liver – Wall enhaces on contrast

Pyogenic Liver Abscess

Imaging

• Plain films– Right lung (33%)• basilar atelectasis or• pleural effusion.

– Right diaphragm elevated and less mobile than the left.

– Abdomen• usually normal or show only hepatomegaly.• Air-fluid level

Pyogenic Liver Abscess

MRI Pyogenic Liver Abscess

Management Pyogenic Liver Abscess

• Goals – Treat the abscess – Treat the source

• Steps IV antibiotics •Multiple small abscesses

Radiological confirmation

Drainage• Aspiration and Percutaneous

• Surgical

Management Pyogenic Liver Abscess

• Antibiotics Alone – Multiple abscesses <1.5cm (Biliary source?)– No concurrent surgical disease

• Antibiotics (preferably 6 wk; 2wk may suffice)– Empirical (multidrug)

• Aminoglycosides( or Fluoroquinolones) , clindamycin (or Metronidazole)and Ampicillin or Vancomycin

– Empirical (single agent)• Ticarcillin-clavulanate • Imipenem-cilastin• Pipercillin – tazobactam

– Selective • As per culture reports

Non –surgical Management Pyogenic Liver Abscess

• Aspiration alone Vs percutaneous drainage – Higher recurrence (more with biliary source!) – Higher rates of surgical drainage later– Less invasive – Less expensive – Mortality rates similar

• Percutaneous drainage (those tails !)not indicated – Large multiple abscesses– Known intrabdominal source – AUO– Ascites – Abscess requiring approach Transpleurally

Thus

SURGERY

Surgical Drainage Pyogenic Liver Abscess

• After Oschner (the man of conservative appendicitis regime !)

– Extraperitoneal (see fig.)• Now-a-days indicated in – Failed non operative methods – Requires surgery for underlying source – Multiple macroscopic abscesses– Steroids use – Ascites

Complications Pyogenic Liver Abscess

• generalized sepsis (most common)• pleural effusions, empyema, and pneumonia• Intraperitoneal rupture (frequently fatal)• perihepatic abscess.• hemobilia and hepatic vein thrombosis

Risk Factors for poor Outcome

• Mischinger Et al– High white blood cell count,– Hyperbilirubinemia,– Anemia, and

• Chou et al.(Multivariate analysis, series 352 patients)– Advanced age,– Hypoalbuminemia, – Altered renal function, and– Hyperbilirubinemia

• John Hopkins Hospital (univariate analysis)– Hyperbilirubinemia,– Hypoalbuminemia,– Multiple abscesses, – Associated malignancy, – Significant complications, and– Septic shock.– Others: Diabetes, cirrhosis, and gas in the abscess

Pyogenic Liver Abscess

Maingot’s Compilation

Amebic Liver Abscess

Hydatid Cystsechinococcus , Greek, means hedgehog berry

hydatid ,Latin hydatis, meansdrop of water

hudatid, hudatis, Greek, means a drop of water

A recent MEDLINE search showed that 86% of articles published on the subject were written by surgeons or in association with surgeons, and yet the surgical treatment of hydatid disease remains controversial!!

E. Granulosus (black shade)E. Multilocularis (cross mark)

Meet the Parasite!

Distribution

50-75%

25%

Characteristics

80%20%

•Slow growing• 1cm in first 6 months • 2-3 cm /year •75% are solitary•50% are multilocular , containing daughter cysts

Cyst anatomy

Pericyst

Ectocyst

Endocyst

Clinical picture

• Most commons

Asymptomatic (>70%)• Pain in the RUQ or epigastrium • Hepatomegaly and a palpable mass

•Non-specific • Dyspepsia • Fever /chills • Jaundice

•Signs • RUQ mass • RUQ tenderness

Imaging

Treatment Complicated

uncomplicated

Medical

Percutaneous

Surgical

Open

Laparoscopic

Rare but Described in LiteratureSalmonella typhi abscess as a late complication of simple cyst of the liver: A case reportIsmail GOMCEL‹, Ahmet GURER, Mehmet OZDO⁄AN, Nurayd›n OZLEM, Raci AYDIN 1st General Surgery Clinic, Ankara Atatürk Education and Training Hospital, Ankara

Abdominal CT showing an abscess cavity (10x5 cm) at the localization of the previous simple liver cyst.

This case report emphasizes that simple liver cyst could be infected with Salmonella and progress to a complicated liver abscess, which should respond well to percutaneous catheter drainage and antibiotherapy.

Summary of presentation

• Simple cysts– no symptoms but may produce dull right upper quadrant pain if large in size. abdominal bloating and early satiety. – Occasionally, a cyst is large enough to produce a palpable abdominal mass. – jaundice caused by bile duct obstruction is rare, as is cyst rupture and acute torsion of a mobile cyst. – Patients with cyst torsion may present with an acute abdomen. – When simple cysts rupture, patients may develop secondary infection, leading to a presentation similar to a hepatic abscess

with abdominal pain, fever, and leukocytosis.

• Polycystic liver disease– rarely arises in childhood. – puberty and increase in adulthood. – with PKD. – Women are more commonly affected, and an increase in cyst size and number is correlated with estrogen level. In PCLD, – hepatomegaly may be prominent, and, occasionally, patients progress to hepatic fibrosis, portal hypertension, and liver

failure. – Complications, such as rupture, hemorrhage, and infection, are rare. However, patients do present with abdominal pain as

the cysts enlarge.

Presentation

• Neoplastic cysts– Cystadenoma in middle-aged women; cystadenocarcinoma equally affects both men and women.– Most patients are asymptomatic or have vague abdominal complaints of bloating, nausea, and fullness. – These patients, like all those with hepatic cysts, eventually present with abdominal pain.– Rarely, they present with evidence of biliary obstruction.

• Hydatid cysts– most often asymptomatic, but pain may develop as the cyst grows.– Larger lesions typically cause pain and are more likely to develop complications than simple cysts.– a palpable mass in the right upper quadrant.– Cyst rupture is the most serious complication of hydatid cysts. Cysts may rupture into the biliary tree,

through the diaphragm into the chest, or freely into the peritoneal cavity. Rupture into the biliary tree may result in jaundice or cholangitis. Free rupture into the peritoneal cavity may cause anaphylactic shock.

– secondary infection and subsequent hepatic abscesses.

• Hepatic abscesses– present with abdominal pain, fever, and leukocytosis– Those patients with amebiasis can have history of diarrhea and weight loss, – some may be asymptomatic.– Pyogenic abscesses often present with cholangitis, abdominal infections, or sepsis. Rarely, abscesses will

rupture, and patients present with peritonitis.

labs• history • a physical examination• imaging study, such as an abdominal CT scan, to define the anatomy of the cyst.

– simple hepatic cysts• require little preoperative laboratory workup.• Liver function test results, such as transaminases or alkaline phosphatase, may be mildly abnormal, but bilirubin, prothrombin time,

and activated partial thromboplastin times are usually within the reference range.

– PCLD, • greater abnormalities in liver function test results are found, but liver failure is uncommon.• Renal function test results, including blood urea nitrogen and creatinine levels, are often abnormal and should be performed on

initial evaluation.

– hydatid cysts,• eosinophilia is noted in approximately 40% of patients, and echinococcal antibody titers are positive in nearly 80% of patients.

– cystic tumors• As with simple cysts, liver function test results are normal• There may be mild abnormalities in some patients.• Carbohydrate antigen (CA) 19-9 levels are elevated in some patients.• Cyst fluid can be sent for CA 19-9 testing at the time of surgery as a marker for cystadenoma and cystadenocarcinoma.

– hepatic abscesses • can usually be easily identified by the clinical presentation.• Leukocytosis is generally present.• The enzyme immunoassay (EIA) test detects specific antibodies to E histolytica.

imaging• Before the widespread availability of abdominal imaging techniques, including ultrasonography and CT scans, liver cysts were diagnosed only

when they grew to an enormous size and became apparent as an abdominal mass or as an incidental finding during laparotomy. Today, imaging studies often reveal asymptomatic lesions incidentally.

• The clinician has a number of options for imaging the liver in patients with hepatic cysts. Ultrasonography is readily available, noninvasive, and highly sensitive. Computed tomography scan (see image below) is also highly sensitive and is easier for most clinicians to interpret, particularly for treatment planning. MRI, nuclear medicine scanning, and hepatic angiography have a limited role in the evaluation of hepatic cysts.Computed tomography (CT) scan appearance of a large hepatic cyst.

• Simple cysts have a typical radiographic appearance. They are thin walled with a homogenous low-density interior.• PCLD is confirmed by ultrasound or CT scan with multiple liver cysts identified at the time initial of evaluation, as depicted in the image

below.Computed tomography (CT) scan of polycystic liver disease curiously limited to the right lobe.• Hydatid cysts can be identified by the presence of daughter cysts within a thick-walled main cavity, which are clear in the MRI below.Hepatic cysts.

Sagittal magnetic resonance imaging (MRI) reconstruction in a patient with a large echinococcal cyst; note daughter cysts in interior.• In patients who are jaundiced with hydatid disease, endoscopic retrograde cholangiopancreatography (ERCP) should be performed to determine if

the cyst has ruptured into the bile duct.• Central necrosis of large solid neoplasms can mimic cystic hepatic tumors, as this area of necrosis appears cystic.• Cystadenoma and cystadenocarcinoma usually appear multiloculated with internal septations, heterogeneous density, and irregularities in the cyst

wall. The image below is a CT scan of biliary cystadenoma.Computed tomography (CT) scan appearance of biliary cystadenoma.• Unlike many tumors, calcifications are rare in cystadenoma and cystadenocarcinoma.• A practical problem in the evaluation of a patient with a cystic hepatic lesion is differentiating cystic neoplasms from simple cysts.Cystic neoplasms

tend to have thicker, irregular, hypervascular walls, whereas simple cysts tend to be thin walled and uniform.• Simple cysts tend to have homogenous low-density interiors, whereas neoplastic cysts usually have heterogeneous interiors with septa and

papillary extrusions.• Abscesses of the liver appear cystic on imaging studies, as shown in the image below, but can usually be diagnosed from the overall clinical

presentation.Ultrasonographic appearance of a patient with a large simple hepatic cyst.• Previous