Polycystic liver disease

Cholangiodysplasia or congenital liver fibrosis (microcystic liver), congenital polycystic disease... 

A wide variety of focal liver lesions can be diagnosed by ultrasound, notably cysts, for which it is the most specific and sensitive test (Gaines and Sampson 1989). They are seen as echo-free spherical spaces with thin, smooth walls and a characteristic band of brighter liver distally, caused by the lower attenuation of ultrasound by their fluid compared to the liver (Fig. 1.4) (Bryant et al. 2004). The same appearance characterises the individual cysts of dominant polycystic disease except that they may be very numerous (Kuni et al. 1978). The lesions themselves and the heterogeneous liver texture that results from the numerous bands of increased sound transmission make the detection of co-existent liver disease difficult or impossible. Similarly, haemorrhage into a cyst or superinfection are not usually detectable with ultrasound. 

Hepatic cysts can also be part of polycystic liver disease, an autosomal dominant disorder often found in association with renal polycystic disease (van Sonnenberg et al. 1994). It is due to a ductal plate malformation of the small intrahepatic bile ducts, which loses communication with the biliary tree. It is characterized by the presence of multiple, sometimes innumerable cysts ranging in size from less than 1 cm to more than 12 cm spontaneous intracystic hemorrhage, infection, and rupture may occur. Usually, patients with autosomal dominant polycystic liver disease are asymptomatic and liver dysfunction occurs rarely... 

Adult polycystic kidney disease (APKD) is one of the most common autosomal dominant diseases, affecting about 1/1,000 whites. The key feature of this disease is the progressive accumulation of renal cysts, which ultimately culminate in kidney failure. APKD is responsible for approximately 10% of end-stage renal disease in North America. Patients may also have hypertension, cerebral aneurysms, liver cysts, and cardiac valvular defects. 

This condition is probably a variant of polycystic liver disease. Aberrant bile ducts and blood vessels are noticeable in the fibrous cyst capsule. The cyst fluid is clear or brownish due to altered blood. Usually, the cyst develops in the inferior-anterior segment of the right lobe. The symptoms, which depend on the size of the cyst, include pressure effects on adjacent organs, cholestasis and obstructive jaundice. 

Kabbey, M., Sanvanet, A., Chanvean, D., Farges, O., Belghiti, J. Laparoscopic fenestration in polycystic liver disease. Brit. J. Surg. 1996 83 1697-1701... 

Levine, E., Cook, L.T., Grantham, J.J. Liver cysts in autosomal-dominant polycystic kidney disease clinical and computed tomographic study. Amer. J. Roentgenol. 1985 145 229-233... 

Pirson, Y., Lannoy, N., Peters, D., Geubel, A., Gigot, J.-F., Brennlng, M., Verellen-Dumonlln, C. Isolated polycystic liver disease as a distinct... 

Que, F., Nagorney, D.M., Gross, J.B., Torres, V.E. Liver resection and cyst fenestration in the treatment of severe polycystic liver disease. Gastroenterology 1995 108 487 -494... 

Starzl, T.E., Reyes, J., Tzakls, A., Mieles, L., Todo, S., Gordon, R. Liver transplantation for polycystic hver disease. Arch. Surg. 1990 125 575-577... 

Swenson, K., Sen, E, Klnkhabwaia, M., Maggard, M., Martin, R, Goss, J., Bnssntii, R. Liver transplantation for adult polycystic hver disease. Hepatology 1998 28 412-415... 

Abdominal trauma Laparoscopic cholecystectomy Membranous obstruction (73) Polycystic liver disease (35) Retroperitoneal neurilemmoma (65) Tumour in the right atrium (42)... 

Koyama, I., Fuchinoue, S., Urashima, Y., Kato, Y., Tsuji, K., Kawase, T., Murakami, T., Tojimbara, T., Nakajima, I., Teraoka, S. Living related liver transplantation for polycystic liver disease. Transplant Internat. 2002 15 578-580... 

Indications Liver blood and kidney yin and yang vacuity, loss of harmony of the chong and ren. Menopausal complaints, amenorrhea, hypertension, nephritis, chronic pyelonephritis, chronic glomerulonephritis, polycystic kidneys, renal vascular disease, urinary tract infection, and hypofunction of the anterior pituitary... 

Polycystic Liver Disease 139 Neoplasms 140 Benign Neoplasms 140 Malignant Neoplasms 145... 

Two types of polycystic liver disease (PLD) have been identified one is related to the autosomal dominant polycystic kidney disease (ADPKD) linked to PKD 1 or PKD 2, while the second form is an isolated form totally unlinked to PKD 1 or PKD 2. Patients with PLD will present liver involvement in approximately 45% of cases (Suchy 2003 Pirson et al. 1996). While ADPKD is usually a disease of adults, about l%-2% of patients display an early manifesting clinical course and may die perinatally. 

Arrive L, Tubiana JM (2003) Serial needle aspiration in polycystic liver disease. AJR Am J Roentgenol 181 1717... 

Pirson Y, Lannoy N, Peters D et al (1996) Isolated polycystic liver disease as a distinct genetic disease, unlinked to polycystic kidney disease 1 and polycystic kidney disease... 

There are several contraindications for TIPS absolute contraindications consist of right-sided heart failure with elevated central venous pressure, polycystic liver disease, and severe hepatic failure (Shiffman et al. 1995). The latter contraindication is based on the fact that the TIPS shunts blood away from the liver, thus further compromising liver function. Relative contraindications consist of active or systemic infection, as TIPS makes use of a foreign device that could act as a colonization site for bacteria, severe hepatic encephalopathy poorly controlled by medical therapy and portal vein thrombosis. 

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