Infected Polycystic Liver Disease- An Unusual Presentation of - PDF document

Journal of Rawalpindi Medical College (JRMC); 2017;21(3): 308-310 Case Report Infected Polycystic Liver Disease- An Unusual Presentation of Extra-renal Autosomal Dominant Polycystic Kidney Disease ( ADPKD) Mohammad Hourani and Daniyal Nagi Department of Internal Medicine, Tawam Hospital, Al-Ain, UAE Introduction from some peripheral cysts, the intrahepatic biliary ducts were non-dilated. Differential diagnoses Autosomal dominant polycystic kidney disease included complex hepatic cysts (with haemorrhage (ADPKD) is characterized by the progressive and possibility of infection-abscess), biliary expansion of multiple bilateral renal cysts leading to cystadenoma/cystadenocarcinoma and less likely type enlargement and distortion of the kidney and V choledochal cyst (Caroli disease). Multidisciplinary ultimately, end-stage renal disease . ADPKD is the team decided medical treatment with antibiotics most common inherited cause of kidney disease without percutaneous intervention. Subsequently, associated with both PKD1 and non-PKD1 genotypes, patient started improving clinically, liver function can occur as extra-renal manifestation such as Adult tests, complete blood count and Inflammatory markers Polycystic Liver Disease (APLD) or distinct disease in returned to the baseline and patient made a absence of renal cysts. Typically these are satisfactory recovery asymptomatic but may be associated with infection or haemorrhage, liver function is typically preserved . 1-3 Case Report A 91 year old female with no past medical history presented with fever and mild abdominal pain. Blood culture showed bacteraemia (E. coli), ultrasound abdomen showed multiloculate septated intra- abdominal collection involving the epigastric and right upper quadrant. CT scan of the abdomen showed multiple large cystic lesions scattered throughout the liver, of uncertain clinical significance, the largest Figure 1: CT of the abdomen showing multiple measuring 7.2 x 6.9 cm (Figure 1), with multiple large cyst lesions . scattered tiny cystic lesions throughout both kidneys. On the presentation at hospital, the examination Discussion revealed a febrile patient, vitally stable with non- Adult liver cystic lesions are classified as hereditary, tender abdomen. There was no family history of developmental, neoplastic, inflammatory, or mixed kidney disease. Laboratory investigations showed leucocytosis with high inflammatory markers and lesions. The hereditary forms of polycystic liver disease (PLD) are associated with autosomal dominant deranged liver function test with pattern suggestive of polycystic kidney disease (ADPKD) and autosomal biliary obstruction. Tumour markers (CA 19-9, AFP & recessive polycystic kidney disease (ARPKD), or occur CEA) were negative. Echinococcosis and Entamoeba histolytica antibodies were negative. MRCP results as a distinct genetic disease in the absence of renal cysts. 5 Autosomal dominant polycystic kidney disease showed numerous cysts of variable sizes, noted (ADPKD) is an inherited, progressive multisystem throughout the liver with predominance for the left disease characterized by the formation and lobe. Some of the large cysts showed heterogenous signal intensity. These cysts in the caudate and in enlargement of cysts in the kidney and other organs (e.g., liver, pancreas, spleen). The incidence of hepatic segment 6 of the liver showed hyperintense signal on cysts in ADPKD increases with age from T1-weighted images with fluid level likely approximately 10% below the age of 30 to greater than representing haemorrhagic components. Although 50% percent over the age of 60. 4 Two mutations have some radicals of the biliary tree appeared inseparable 308

Journal of Rawalpindi Medical College (JRMC); 2017;21(3): 308-310 been found to cause this disorder: a mutation in the needs to consider liver transplantation. 9 Somatostatin PRKCSH gene that encodes a protein called analogues have been tested to help reduce cyst volume hepatocystin, and a mutation in the SEC63 gene that and provide symptomatic relief in cases of PLD. A encodes for a component of the protein translocation recent pooled analysis of 2 randomized, double-blind, machinery in the endoplasmic reticulum. 6 placebo-controlled trial examined 96 patients with Typically, PLD is asymptomatic, but the symptoms PLD treated between 6 and 12 months showed become more frequent with age, and so increase improvement in life quality related to symptoms and because of increased life expectancy, especially in hepatomegaly. 10 Although a 2008 pilot trial of 16 patients with ADPKD due to dialysis and patient with ADPKD demonstrate that Sirolimus, an transplantation. Hepatic cysts are more prevalent and mTOR inhibitor, reduced PLD volume by 26%. 9 hepatic cyst volume is larger in women than in men, In a systemic review of 54 patients with hepatic cyst and multiple bile cystic lesions vary from 20 to 30 cm infection (Table 1), it is more common in female with to small microscopic nodules. 3 The clinical course of mean age of 63. ADPKD was the most prevalent PLD is relatively benign compared with ADPKD. The underlying cyst disease. Initial management was symptoms that are typically caused by mass effects are through antimicrobial treatment (56%), the rest of the hepatomegaly and portal hypertension, ascites, case, initial management involved percutaneous/ jaundice, haemorrhage, dyspnoea, early satiety and surgical intervention. Results showed that first line weight loss, gastro-oesophageal reflux, and pain in the treatment with antimicrobial alone is associated with lower back region. 5 Symptomatic cyst complications high rate of treatment failure (70%). Despite multiple include cyst haemorrhage, infection, and rarely switches in antimicrobials, most of them ultimately torsion, or rupture . Other complications of mass effect required percutaneous drainage or surgery (64%). are vena cava compression and lower portal vein and Nonetheless, recurrent cyst infection was common and bile duct compression that present itself as obstructive developed in 20% of patients. jaundice. 7 Table 1: Comparison between 54 patients with Despite advances in the management of autosomal hepatic cyst infection regarding Gender, mean age, dominant polycystic kidney disease over the past two underlying cyst disease, management, and decades, infection of liver and kidney cysts remains a serious and potentially threatening complication. It is recurrence. 12 Gender Male 39%, Female 57% differentiated from haemorrhage by the clinical presentation (mainly the severity and duration of Mean Age 63 ±12 fever), C-reactive protein (CRP) and white blood cells Underlying ADPKD: 46%, PCLD: 4%, levels, and the density of the suspected cyst on disease multiple liver cyst: 15%, multiple computed tomography. Liver cyst infection occurs kidney cyst: 2%, more frequently in patients with large cysts volumes. Management Antimicrobial 56% with It can be life threatening and tends to recur. The best Quinolones and Cephalosporin, radiological imaging technique is positron emission failed in 70%. tomography after intravenous injection of [18F]- Surgical treatment in 31% fluorodeoxyglucose combined with computed Recurrence 20% tomography. 8 Management is based on symptomatic presentation. Counselling should be done prior to testing among Often no specific therapy is warranted as therapy is asymptomatic patients with a family history of directed toward symptoms and the patient may be ADPKD. In children (age less than 18 years), there is asymptomatic. In patient with infected cyst; we no need for screening since the adverse effects from a recommend initial treatment with a fluoroquinolone pre-symptomatic diagnosis outweigh the current guided by culture and sensitivity for the duration of 6 benefits. In adults (age greater than 18 years), we weeks. However, due to the growing rate of recommend screening potential living, related kidney Ciprofloxacin resistance organisms, the choice of the donors, in other adults, the decision to screen for the empirical antimicrobial agent will depend upon the disease should be based upon patient's preferences centre-specific resistance rate. Cyst drainage should and values, after the benefits and adverse be considered when cysts are very large and or when consequences of certainty concerning the diagnosis are infection is resistant to antibiotic treatment. Patient fully understood. If screening is performed among with recurrent liver cyst infection or angiocholitis asymptomatic patients with a family history of 309