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Cystic Liver Disease PBL 12 Tutor: Dr. Aida Ali

Objectives Definition Prevalence Types Etiology Clinical Aspects Diagnosis Prevention and Control

Definition Abnormal sacs filled with fluid in the liver

Prevalence  Estimated 5% of total population  85-90% of them are asymptomatic  Often found incidentally  No patient under the age of 40 years old

2. Types A.Simple B.Polycystic C.Neoplastic D.Hydatid –Unilocular –Multilocular E.Hepatic abscess

A. Simple Cysts Arise from malformation of bile ducts during embryo’s development. Unrelated to Polycystic Liver Disease (PCLD). Occasionally cause dull pain or palpable swelling in upper right quadrant of abdomen. Jaundice associated with bile duct obstruction, is rare.

Treatment No medical therapy proven effective. Percutaneous aspiration or combined with sclerosis and alcohol are successful in some patients but recurrence rate is high Surgical therapy involves “unroofing” cysts by excising the portion that extends to the surface of the liver.

Simple Cysts CT scan appearance of a large hepatic cyst

B. Polycystic Rare genetic disorder Due to mutations of PRKCSH and SEC63 gene. Characterized by many cysts of various sizes that develop throughout the liver. Associated with congenital hepatic fibrosis. May occur with polycystic kidney disease (PKD). Hepatomegaly and abdominal pain may be present. Rupture, hemorrhage and secondary infection, are rare.

Polycystic Liver Dis. CT scan in a PCLD patient presents multiple cysts originating from medium-sized bile ducts

Treatment No medical therapy. Surgical therapy include combination of unroofing and fenestration, or resection of involved portions in selected patients.

C. Neoplastic Cysts Liver tumors with central necrosis often misdiagnosed as liver cysts. Called cystadenoma if benign. Cyst adenocarcinoma as the malignant counterpart. Similar to simple cysts as they are of epithelial origin. Most are asymptomatic or have vague abdominal complaints of bloating, nausea and fullness

Neoplastic Cyst Contrast-enhanced CT scan in an elderly woman. The image demonstrates a large, well-defined, multiloculated, hypoattenuating lesion in the left hepatic lobe. The cystic lesion was lined by columnar epithelium, as observed at histologic evaluation, this finding was consistent with biliary cystadenoma

Treatment No medical therapy (similar to PCLD) Surgical therapy as such:  Enucleation and formal resection  Fenestration and complete fulguration

D. Hydatid Cysts Caused by infection with parasite Echinococcus granulosus larva (common in sheep and cattle farming areas). Sheep, cattle and human as intermediate hosts when they ingest food or drink contaminated by eggs. Unilocular Hydatid cyst

D. Hydatid Cysts cont. Adult tapeworm grow from scolices in the small intestines of carnivores such as dogs or wolves when they ingest the liver of intermediate hosts, thus completing the life cycle of the parasite.. Man act as blind I.H. Unilocular Hydatid cyst

1.The cyst may cause no symptoms. 2.Pressure on bile duct (producing obstructive jaundice). 3.Secondary new cysts (metastases), through seeding the peritoneal cavity with hydatid sand or bits of germinal layer.

4.Rupture into bile ducts → intermittent jaundice, fever and eosinophilia. 5.Allergic manifestations up to anaphylactic shock in case of sudden rupture with entrance of a considerable hydatid material in the blood stream.

Hydatid cyst Typical unilocular hydatid cyst. Unenhanced CT scan shows a large hydatid cyst with a noncalcified, high-attenuation wall in the right hepatic lobe (arrows). This finding can be missed if only contrast-enhanced CT is performed. Because of its elasticity, the cyst accommodates itself to neighboring structures.

Diagnosis Clinically: Presence of a slowly growing cystic tumor and history of close contact with dogs. X-ray: In pulmonary cysts and calcified cysts. CT scan USS: Uncalcified cysts and follow-up after treatment.

Aspiration cytology: Use trichome staining of filtered aspirate show acid fast hooklets –risky and may cause transplantation of the germinal layer and formation of new cysts or anaphylactic shock Find hydatid materials: hydatid fragments from cysts rupture in sputum or urine. Serological tests: IHA, ELISA detect hydatid fluid antigens. Immunoelectrophoresis (IEP) Molecular diagnosis: PCR Intradermal test of Casoni

Treatment 1.Surgical removal. N.B. care must be taken why? 2.Recently percutaneous aspiration for pulmonary, hepatic and accessible cysts. This therapy is known as PAIR i.e. Percutaneous, Aspiration, Injection (of hypertonic saline or other scolicidal fluid e.g. hydrogen peroxide or ethanol) and Reaspiration

1.Proper disposal of infected viscera in slaughter houses. 2.Elimination of stray dogs. 3.Periodic examination of pet dogs and treatment of infected ones by Praziquantil. 4.Man should avoid dogs. 5.Protection of food and drink from being contaminated with dog feces. Prevention and Control

Echinococcus Multicularis and Multilocular or Alveolar Hydatid Disease (MLHD) It is mainly found in North America, Siberia and Eskimos. Hydatid Cysts cont.

The alveolar cyst = MLHC: Adult E. Multilocularis similar to E. Granulosus in morphology but the larva show morphological difference: 1- No fibrous layer around MLHC 2- Laminated layer is absent. 3- Germinal layer highly nucleated. 4- Little or very thick jelly-like H.F. 5- It has an irregular outline which is not defined from the surrounding tissues hence the name (alveolar H.C.).

6- It behaves like a malignant tumor i.e. proliferates & metastasizes in any direction, degenerating and calcified in the center. Its commonest site is the liver (90-100%). In human the cyst is usually sterile. Spread at the periphery. It gives metastasis through the blood or lymph.

E. Hepatic Abscess 3 major forms of liver abscess, classified by etiology, are as follows: Pyogenic abscess, which is most often polymicrobial, accounts for 80% of hepatic abscess cases in the United States. Amebic abscess due to Entamoeba histolytica accounts for 10% of cases. Fungal abscess, most often due to Candida species, accounts for less than 10% of cases.

E. Hepatic Abscess cont. Most frequent symptoms of hepatic abscess include: Fever (continuous or spiking) Chills Rt. upper quadrant pain Anorexia Malaise Cough are due to diaphragmatic irritation.

Hepatic Abscess CT scan of the abdomen with contrast showing large amebic abscess with multiloculated appearance and atypical left liver lobe location. CT scan cannot differentiate amebic liver abscess from pyogenic liver abscess.

Treatment Immediately started on antibiotics/amebicides. Followed by percutaneous drainage for eradication. Surgical drainage may be indicated.

Diagnosis Liver Function Test (LFT) indicates  Mildly abnormal AST and ALT in simple hepatic cysts with normal bilirubin, prothrombin time and activated partial prothrombin time.  Greater abnormalities in PCLD but, liver failure is uncommon. Renal function test shows abnormal blood urea nitrogen and creatinine levels in PCLD Observable eosinophilia in 40% of Hydatid cysts Positive antibody titers in 80% of Hydatid cysts. Leukocytosis generally present in hepatic abscess.

Summary Liver cysts are abnormal sacs filled with fluid in the liver. The cysts are often found incidentally via unrelated abdominal radiological examination. Different types of liver cysts have different causative agents and radiological picture. Treatments through medical and surgical therapy depends on the type.

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Credits Presenters: Akram Diana Faqihah Luqmey Mas Zahid Slide Developers: Basyar Khalid Menna Nasyima Wana Tutor: Dr Aida Ali