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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 4  |  Issue : 3  |  Page : 222-224

Polycystic liver disease with huge infected cyst displacing the pancreas, inferior vena cava and right kidney


1 Department of Medicine, King Fahd Armed Forces Hospital, Jeddah, Saudi Arabia
2 Department of Radiology, King Fahd Armed Forces Hospital, Jeddah, Saudi Arabia

Date of Web Publication11-Aug-2016

Correspondence Address:
Adil H Al-Harthi
King Fahd Armed Forces Hospital, P. O. Box 9862, Jeddah 21159
Saudi Arabia
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DOI: 10.4103/1658-631X.188259

PMID: 30787735

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  Abstract 

Multiple liver cysts can be an isolated disease (isolated polycystic liver disease [PLD]) or they can be part of multi-organ involvement in other diseases, such as adult autosomal dominant polycystic disease (APD), which is the most frequently inherited polycystic disease affecting 1 in 1,000 of the population. About 33% of APD patients are expected to develop PLD. Cysts in the liver, as opposed to cysts in the kidney, grow dramatically in number and size. They are usually asymptomatic but may present with signs and symptoms of compression of adjacent organs as the cysts grow. We present a case of a 73 year-old female patient with PLD and a large (25 × 19 × 18 cm) infected cyst which caused the displacement of the inferior vena cava, right kidney, and pancreas. We also discuss the management of this patient along with a review of the literature.

  Abstract in Arabic 

ملخص البحث:
قد تظهر الأكياس الكبدية كمرض مستقل أو تكون جزءاً من مرض يشمل تكيسات في أعضاء أخرى مثل مرض التكيس المتعدد ذي الموروث الساند للبالغين (D PA) وهو المرض الوراثي الأكثر شيوعا من بين أمراض التكيس المتعدد. يعرض الباحثون حالة لمريضة تبلغ 73 عاماً تعاني من مرض التكيس المتعدد مع وجود كيس كبدي كبيـــــــر 18×19×25 سم مما أدى إلى إزاحة الأعضاء المجاورة عن مواضعها الطبيعية.

Keywords: Adult autosomal dominant polycystic kidney disease, liver cyst, polycystic liver disease


How to cite this article:
Al-Harthi AH, Khan PM, Al Marwani M. Polycystic liver disease with huge infected cyst displacing the pancreas, inferior vena cava and right kidney. Saudi J Med Med Sci 2016;4:222-4

How to cite this URL:
Al-Harthi AH, Khan PM, Al Marwani M. Polycystic liver disease with huge infected cyst displacing the pancreas, inferior vena cava and right kidney. Saudi J Med Med Sci [serial online] 2016 [cited 2020 Aug 11];4:222-4. Available from: http://www.sjmms.net/text.asp?2016/4/3/222/188259


  Introduction Top


Adult autosomal dominant polycystic disease (APD) is the most commonly inherited polycystic disease affecting 1 in 1,000 of the population. The majority of extra renal cysts in APKD are mainly located in the liver (33%), but the disease may also involve other organs.[1] Isolated polycystic liver disease (PLD) is rare, with an estimated incidence below 0.01%.[2] Both APKD and isolated PLD have an autosomal dominant inheritance pattern.[3] PLD usually has more severe liver phenotypes, especially in patients with a history of multiple pregnancies or prolonged exogenous estrogen exposure, which suggests a hormonal component.[4] PLD usually remains asymptomatic. Symptoms, if present, are usually nonspecific and are caused by increasing liver volume and adjacent visceral compression. Several acute complications may occur due to the sheer size of these cysts, which grow steadily and attain huge dimensions, due to displacement and compression of adjacent organs. A number of cases have been reported in which the cysts have attained enormous proportions (22 × 19 cm), resulting in acute complications such as pancreatitis, deep vein thrombosis, and acute renal failure. The present case describes a 73-year-old female patient with APD with PLD, with a hepatic cyst (25 × 19 × 18 cm), which caused the displacement of the right kidney, pancreas, and inferior vena cava (IVC).

This patient was known to have hypothyroidism, hypertension, and APD with chronic renal impairment. She presented with abdominal discomfort and supra pubic abdominal pain of one week's duration. She also complained of nausea and vomiting of one day's duration.

On examination her blood pressure was 117/76 mm/hg, pulse rate was 117/min, and a temperature of 36.8°C; there was no icterus. Cardiovascular, respiratory, and neurological system examinations were unremarkable. Her abdomen was distended, non-tender hepatomegaly. A computed tomography scan of the abdomen and pelvis without contrast revealed that both kidneys were enlarged with multiple variable sized cysts and hepatomegaly with multiple cysts, the largest of which was on the right side and measured approximately 25 × 19 × 18 cm, exerting a significant mass effect on the adjacent organs [Figure 1] and [Figure 2]. The laboratory results are shown in [Table 1].
Figure 1: Computed tomography abdomen shows a large liver cyst displacing the inferior vena cava to left anteriolateral position in relation to the abdominal aorta.

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Figure 2: Computed tomography abdomen shows a large liver cyst displacing the head of the pancreas to a midline position and the right kidney displaced inferiorly.

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Table 1: Lab investigations

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The cystic fluid was aspirated two days after starting antibiotics and the analysis of the fluid revealed white blood cells. However, a culture did not result in the growth of bacteria. As a result of the infected cysts, she was diagnosed with PLD. The patient was started on intravenous fluids. The following day, an ultrasound-guided pigtail was inserted to drain the fluid from the cyst; a total of and 3 liters of fluid was drained. After a period of one week, the patient improved clinically and was discharged.


  Discussion Top


APD is the most frequently inherited polycystic disease affecting 1 in 1,000 of the population. Approximately 33% of extrarenal cysts in APD are located in the liver, but various other organs may also be involved. Isolated PLD is rare, with an estimated incidence below 0.01%. Both APD and isolated PLD have an autosomal dominant inheritance pattern. Cysts in the liver grow to huge dimensions rapidly as opposed to renal cysts. PLD usually remains asymptomatic. Symptoms, if present, are usually nonspecific and are caused by increasing liver volume and adjacent visceral compression. Less than 5% of patients with PLD have acute medical complications. These complications include hemorrhage into the cyst, infection, rupture, portal hypertension, ascites, Budd–Chiari syndrome, and deep vein thrombosis.[5] The cysts in PLD may multiply in number and grow continuously to attain huge dimensions. Campos Franco et al. reported a case of PLD with a cyst measuring 22 × 19 cm, which displaced the right kidney.[6] A literature review has revealed very few cases of large cysts in PLD with acute complications due to pressure effects.

A previous report described a 70-year-old female who had a partial intestinal obstruction secondary to the multiple renal and hepatic cysts compressing the bowel that was relieved after unilateral right nephrectomy.[7] In another report, a 66-year-old man who presented with progressive lower limp edema and high jugular venous pressure was found to have compression of the right atrium and IVC due to a hepatic cyst which was revealed through an echo.[8] Massive deep vein thrombosis in leg veins reaching the distal part of the IVC due to huge liver cyst has also been reported.[9] A rare case of chronic obstructive pancreatitis due to a pancreatic cyst in a patient with APD has also been reported.[10] Our patient, who was a 73-year-old female patient known to have APD with chronic renal failure and PLD, presented with abdominal pain and on investigation was found to have a giant (25 × 19 × 18 cm) infected liver cyst causing displacement of the adjacent structures. To the best of our knowledge, this is the largest liver cyst reported in PLD. Even though significant pressure effects were seen on right kidney, pancreas and IVC, no acute complications with these organs were seen. The management of compressive large liver cysts is by different modalities, depending on the cause and urgency of the situation. Aspiration of cyst fluid, ethanol sclerosis, and laparoscopic fenestration are possible treatment options. A liver transplant is another option if the cysts are multiple and compressing on adjacent organs. Surgical intervention may be needed in patients presenting with massive hepatomegaly or other complications such as cyst rupture, infection, hemorrhage, or obstructive jaundice.[11]


  Conclusion Top


In conclusion, cysts in PLD may grow to enormous sizes and compress and compromise the function of adjacent organs and present with acute complications such as pancreatitis, renal failure, intestinal obstruction, and deep venous thrombosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Thomsen HS, Thaysen JH. Frequency of hepatic cysts in adult polycystic kidney disease. Acta Med Scand 1988;224:381-4.  Back to cited text no. 1
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2.
Russell RT, Pinson CW. Surgical management of polycystic liver disease. World J Gastroenterol 2007;13:5052-9.  Back to cited text no. 2
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3.
Pei Y. Diagnostic approach in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol 2006;1:1108-14.  Back to cited text no. 3
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4.
Qian Q, Li A, King BF, Kamath PS, Lager DJ, Huston J 3rd, et al. Clinical profile of autosomal dominant polycystic liver disease. Hepatology 2003;37:164-71.  Back to cited text no. 4
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5.
Arnold HL, Harrison SA. New advances in evaluation and management of patients with polycystic liver disease. Am J Gastroenterol 2005;100:2569-82.  Back to cited text no. 5
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6.
Campos Franco J, Otero Anton E, Gomborino Carames E, Martinez Castro J, Varo Perez E. Polycystic liver disease. Patol Dig 2009;101:283-4.  Back to cited text no. 6
    
7.
Goncalves J, Filipe R, Santo C, Mantalban J, Ramalheiro A, Rocha E. Partial intestinal obstruction: A rare complication of autosomal dominant polycystic kidney disease. Case report and review of the literature. Port J Nephrol Hypertens 2012;26:207-12.  Back to cited text no. 7
    
8.
Chalisey A, Karim M. Mass effect from hepatomegaly in polycystic kidney disease. Published online 2014 Aug 16. doi: 10.1007/s11606-014-2963-1.  Back to cited text no. 8
    
9.
Clausen JH, Bonde-Petersen J. Large liver cyst with compression of the inferior vena cava causing massive deep vein thrombosis. Ugeskr Laeger 2014;176. pii: V04140251.  Back to cited text no. 9
    
10.
Malka D, Hammel P, Vilgrain V, Fléjou JF, Belghiti J, Bernades P. Chronic obstructive pancreatitis due to a pancreatic cyst in a patient with autosomal dominant polycystic kidney disease. Gut 1998;42:131-4.  Back to cited text no. 10
    
11.
Henne-Bruns D, Klomp HJ, Kremer B. Non-parasitic liver cysts and polycystic liver disease: Results of surgical treatment. Hepatogastroenterology 1993;40:1-5.  Back to cited text no. 11
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