Presentation
We present a patient with polycystic disease of the liver who developed an aneurysm arising from the right hepatic artery presenting with surgical obstructive jaundice. [ncbi.nlm.nih.gov]
Gastrointestinal
- Abdominal Pain
While mild abdominal pain was observed in a few patients, it was resolved spontaneously under observation. [ncbi.nlm.nih.gov]
Early satiety, malnutrition, and abdominal pain necessitated a liver transplantation. The recipient's weight at transplantation was 59 kg. [nejm.org]
Workup
She refused further workup including liver biopsy. Discussion Polycystic liver disease occurs either in isolation or as part of ADPKD or uncommonly adult dominant polycystic liver disease (ADPLD). [cureus.com]
Other ECG Findings
- Ischemic Changes
Symptoms and Signs Caused By Ischemia- Partial compression of surrounding arterial blood vessels results in ischemic changes of organ. Portal Hypertension- Caused by cyst compressing portal vein. [epainassist.com]
Another theory suggests that ischemic changes caused by granulomatous phlebitis of portal and hepatic veins can lead to cirrhosis and portal hypertension [2]. Both conditions are diagnosed on imaging. [cureus.com]
Treatment
Although various treatments for symptomatic adult polycystic liver disease have been advocated, a definitive treatment remains controversial, especially in diffuse adult polycystic liver disease. [ncbi.nlm.nih.gov]
Prognosis
Prognosis Most patients with PCLD have good prognosis and do not require treatment. The documents contained in this web site are presented for information purposes only. [orpha.net]
To identify predictor factors, beyond MELD criteria that relate to the increased liver volume before development of late symptoms is essential to expeditiously treat patients with the poorest prognosis to improve their outcomes. [ncbi.nlm.nih.gov]
Etiology
Case report peer-reviewed Polycystic Liver Disease and Sarcoidosis: Unusual Coexisting Etiologies of Portal Hypertension Figures etc. [cureus.com]
[…] resemble corpora atretica or fibrosa of ovary TERMINOLOGY Abbreviations • Polycystic liver disease (PLD) • Autosomal dominant polycystic kidney disease (ADPKD) Definitions • Genetic disorder characterized by progressive development of multiple liver cysts ETIOLOGY [basicmedicalkey.com]
Etiology Liver cysts result from overgrowth of biliary epithelium or from dilatation of peribiliary glands. Some cases occur sporadically, but most are inherited as an autosomal dominant trait (ADPCLD). [orpha.net]
Banales J.M. 2017 18 Waitlisted Candidates With Polycystic Liver Disease are More Likely to be Transplanted Than Those With Chronic Liver Failure. ( 28296808 ) Doshi S.D....Goldberg D.S. 2017 19 Polycystic Liver Disease and Sarcoidosis: Unusual Coexisting Etiologies [malacards.org]
Epidemiology
Areas covered: This review begins by looking at the epidemiology of PLD and its natural history. It then describes the pathophysiology and corresponding potential treatment strategies for PLD. [ncbi.nlm.nih.gov]
Summary Epidemiology The prevalence of ADPCLD is 1/100, 000. Clinical description Women are predominantly affected and have a larger number of cysts than affected males. [orpha.net]
The rate of single cysts is about 68–75 % [ 5, 6 ]. 8.2.1 Pathogenesis and Epidemiology Simple cysts contain a clear, bile-like fluid. [abdominalkey.com]
Abu-Wasel, B., et al., Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases. World J Gastroenterol, 2013. 19(35): p. 5775-86. 4. [rarediseases.org]
Pathophysiology
AIM: To provide a systemic review on the pathophysiology and management of PCLD. [ncbi.nlm.nih.gov]
Temmerman, F., et al., Systematic review: the pathophysiology and management of polycystic liver disease. Aliment Pharmacol Ther, 2011. 34(7): p. 702-13. 8. [rarediseases.org]
Prevention
The sclerosing treatment was successful, thus preventing laparotomy. [ncbi.nlm.nih.gov]
The use of postoperative long-acting octreotide has the potential to reduce the growth of remaining cysts and prevent new cysts from developing, and it should be considered in the management of these patients after surgery. [jamanetwork.com]