بسم الله الرحمن الرحيم

1. بسم الله الرحمن الرحيم

2. LIVER CYSTS By Prof. Dr. AmrAlyAbdelMoety

3. Liver cysts can occur in solitary or in multiple form. They may be congenital or acquired. The reported frequency varies between 1% and 20%. The cysts are lined with a layer of cuboid cells and mostly filled with fluid. They are usually detected by chance. When cysts compress bile ducts or vessels, they become symptomatic. • In sonography, the cyst appears as an anechoic (black) space with well-defined margins and distal sound amplification. Haemorrhage into the cyst cavity and debris can mimic a cystadenoma or even cystadenocarcinoma. The presence of septa suggests a neoplastic cyst.

4. In compute tomography, their content can generally be determined at +0 to +15 houndsfield units. The i.v. administration of a contrast medium shows no evidence of enhancement. • Cysts are easily detected by magnetic resonance imaging (T1 image = dark; T2 image = light with high signal intensity). Laparoscopy provides a very impressive view of cysts.

5. CT with contrast medium: solitary liver cyst

6. Multiple cysts with several chambers in the area of the right liver lobe • Giant (fist-sized) solitary cyst in the right liver lobe

7. Four types of cysts can be differentiated: • Dysontogeneticcysts. • Parasitic (or infectious) cysts. • Neoplastic cysts. • Post-traumatic cysts. • The cause of cystic neoplasms is unknown. • Traumatic cysts occur from an injured intrahepatic bile duct after blunt abdominal trauma.

8. TREATMENT • Asymptomatic cysts do not require treatment. • large cysts which might rupture are an exception. • Symptomatic cysts (usually > 5 cm) are generally sclerosed. Reliable sclerotherapeutic agents include aethoxysclerol, (1%) alcohol (95%), minocycline hydrochloride, tetracycline hydrochloride (1 g), and sodium chloride solution (10%). The sclerosing procedure is successful and low in side effects.

9. Percutaneous aspiration of the cysts has a high relapse rate. Sclerotherapy does not prevent relapse, since a sclerotherapeutic agent destroys the cells, but not the cyst walls. • Parasitic cysts require specific therapeutic measures. • Under laparoscopy, cysts which prove to be problematic may be treated reliably by fenestration . • Surgical procedures (e.g. cyst excision, resection, cystjejunostomy, cystenterostomy) are rarely indicated and involve a much higher risk.

10. POLYCYSTIC LIVER DISEASE • The incidence is; 1:500 to 1:5,000 births/100,000/per year. • The gene responsible is located on chromosome 16; often another gene located on chromosome 4 is also involved. • This autosomal dominant polycystic disease of the liver and kidneys can occur in (1.) children and (2.) adults (♂:♀ 1:5).

11. The intrahepatic bile ducts are widely dilated and lined with bile-duct epithelium. The cysts are surrounded by a fibrous capsule. Occasionally, the cysts cover the liver in a balloon-like manner. Rupture of large cysts may give rise to the clinical picture of acute abdomen. Compression leads to portal hypertension, jaundice, cholestasis and leg oedema. • Renal insufficiency is also a dangerous complication.

12. Polycystic liver with spheroid, balloon-like cysts in the right liver lobe

13. Treatment may be by laparoscopic or surgical fenestration, injection of alcohol (40%), or minocycline hydrochloride, as well as surgical management. Liver transplantation is a rare indication.

14. PARASITIC CYSTS • The cysts of Echinococcusgranulosus (cysticus) and Echinococcusmultilocularis (alveolaris)

15. Imaging study depicting 2 complex cystic liver masses with curvilinear wall calcification (more prominent in the smaller lesion) compatible with echinococcal (hydatid) cysts.

16. CAROLI'S DISEASE/ SYNDROME • This congenital clinical picture was first described in the form of intrahepatic stones by H.R. Vachell et al. in 1906. Later on, in 1958, J. Caroli et al. were able to establish an association between characteristic congenital bile-duct alterations, cholangitis, cholangiolithiasis and renal cyst formation. In 1964 J. Caroli et al. differentiated a particular form with simultaneous congenital liver fibrosis (which had already been described by D.V.S. Kerr et al. in 1961). Both forms are autosomal recessive.

17. Caroli's disease is characterized by congenital, segmental dilatation of the intrahepatic bile ducts, causing bile sludge formation and the development of gallstones. Clinical findings include hepatomegaly, cholestasis and subicterus as well as cholangitis with upper abdominal pain and biochemical signs of inflammation. Diagnosis is by CT, Doppler sonography and ERC.

18. ERC findings in Caroli’s disease: mainly segmental, sack-like dilatations of the intrahepatic bile ducts with some small diameter connections to the efferent bile ducts

19. Caroli’s syndrome is additionally characterized by congenital liver fibrosis. There may be fibroangiomatosis of the bile ducts and microcysts in the kidneys. The condition can lead to portal (presinusoidal) hypertension. Choledochal cysts, polycystic degeneration of the liver and kidneys, Laurence-Moon-Biedl syndrome and ectasia of the renal tubules may all appear. • Apart from cholangitis and hepatolithiasis, potential complications include liver abscess, pancreatitis, amyloidosis, malignant tumours and liver failure. Treatment consists of biliary drainage, antibiotics, surgical procedures and liver transplantation. Ursodeoxycholic acid may be used as an adjuvant.

20. HEPATOBILIARY CYSTADENOMA • This rare benign tumour probably develops from congenital bile-duct malformations. • It is found mostly in women (> 90%), mainly after the age of 45-50 years. • The tumour grows very slowly, yet can reach a considerable size (5-25 cm).

21. TYPES • The mucous type consists of a mucous/gelatinous, bile-coloured fluid, often containing old blood. • Serous type of cystadenoma without mesenchymalstroma is found. • There is evidence of ovary-like stroma together with unilaminar bile epithelium, which is folded in a polyploid or papillary manner in places. The collagenic capsule is rich in vessels. Surrounding the tumour, numerous abnormal bile ducts and arterial vascular clusters are evident. Cholestasis or even obstructive jaundice develops.

22. Contrast-enhanced computed tomography scan in a 37-year-old woman with a history of non-Hodgkin lymphoma. The image demonstrates a large, multiloculated, cystic lesion in the left hepatic lobe with fine peripheral calcifications. Aspiration revealed a cloudy grayish-brown fluid. Histologic analysis demonstrated the presence of small glands lined by columnar epithelium and dense cellular stroma of uniform spindle cells; these findings were consistent with hepatobiliarycystadenoma

23. Hepatobiliarycystadenoma with ovary-like stroma (HE)

24. Characteristic feature of this cystic lesion (mainly in the right lobe) is their cystic apperance which is the formation of cyst clusters divided by thin septa and fine calcification in it wall by imaging techniques. • Due to the tendency of cystadenoma towards malignant degeneration,suspected by:( thickening of the wall,presence of solid masses or septa) resection should be carried out as soon as possible.

25. BILE-DUCT ADENOMAS • Bile-duct adenomas are rare. Usually, they are solitary and <2 cm in size. • This type of adenoma is more often detected in men than in women (3:1), and it occurs in patients mainly over the age of 50. It is nearly always localized beneath the liver capsule. • Sonography shows roundish, hypoechoic foci of different sizes with relatively unclear contours. They are visible in MRT as hypotense (T1-weighted) lesions. Imaging procedures do not reveal any characteristic features.

26. With the help of laparoscopical examination, roundish, greyish-white, well-defined foci are visible. The lesions cause slight bulging on the liver surface. The surrounding tissue is unchanged and without vascularization. The biopsy material taken from the foci turned out to be crumbly. This adenoma has the form of a firm, whitish or whitish-grey node and consists of bile-duct proliferations. It contains biliary acini and tubules which are lined with a layer of cuboid epithelium within loose fibrous, partially hyaline stroma.

27. Several roundish, hypoechoic foci of different sizes with relatively blurred contours

28. Axial T1-weighted MRT showing at least three hypointense roundish foci with slightly hazy edges in the right liver lobe. One focal lesions is subcapsular, causing minimal bulging on the surface. There are no signs of any radiological features which might facilitate a diagnosis • Diffusely disseminated greyish-white foci of different sizes in both liver lobes with relatively sharp contours. The liver surface shows slight bulging (about 1_2 mm), but no central dipping; no vascularisation is visible. The perifocal tissue is unchanged.

29. There is evidence of mononuclear inflammatory cells and, occasionally, lymph follicles. It is discussed that such biliary adenomas are peribiliary gland hamartomas. There are no clinical or biochemical abnormalities, nor is there any tendency towards malignant degeneration.

30. Bile-duct adenoma in subcapsular liver tissue (HE) • Bile-duct adenoma composed of small tubules, which are lined by a single layer of slightly irregular epithelial cells. The ducts are embedded in a dense collagenous stroma (HE)

31. BILE-DUCT PAPILLOMATOSIS • Bile-duct papillomatosis is a very rare finding (up to now about 50 cases have been reported) which occurs mainly in elderly women. There are papillomatous proliferations in both intrahepatic and extrahepatic bile ducts. Clinically, it is characterized by recurrent episodes of jaundice and cholestasis as well as ascending cholangitis and haemobilia. • The bile ducts can be dilated; their wall surface is irregular. The course is progredient; the prognosis is poor. Recently, encouraging results have been reported following intraluminal iridium therapy and liver transplantation. Malignant degeneration into cholangiocarcinoma is possible.

32. BILIARY HAMARTOMA • Benign biliary microhamartomas (Meyenburg’s complex) were described by H. von Meyenburg in 1918. • They consist of small cysts developing from dilatations of the small (interlobular) bile ducts and are surrounded by fibrous stroma. The cysts are remnants of ductal plate malformations. Sometimes typical ductal plates are also detected. The hamartomas can reach a diameter of up to 0.5 (1.0) cm.

33. Macroscopically, they appear as firm, greyish-white nodules which are clearly delineated from the liver parenchyma. Subcapsular nodules may also be detected by laparoscopy. They are sometimes misinterpreted as small metastases. Here, MRI is of great diagnostic value. Microhamartomasare often associated with malformations of the small branches of the portal veins. They may represent a transition to the autosomal dominant form of polycystic degeneration. These tumours have a tendency to develop into cholangiocarcinoma.

34. Case Presentation • Hassan abd el salam,47 y old male pt from elamerya married with 4 offspring admitted since 4 weeks with an attack of hematemesis and rthypochondrial pain. • Clinically;Pallor,rthypochondrial dull aching pain. • Liver not enlarged ,spleen sligthly enlarged • Lab data:Bil 0.5,ALT 25,AST 30,Albumin:2.7,Alkaline phosphatase (119) Renal function normal,AFP :2.5 ,CEA:1.3 CA 19-9:8 • HCV Ab:+ve. • Ultrasound:liver cirrhosis /numerous small cyst and splenomegally. • Endoscopy:Sliding hiatal hernia/No varices

35. Thank you