Korean J Hepatobiliary Pancreat Surg.  2015 Feb;19(1):40-46. 10.14701/kjhbps.2015.19.1.40.

Fenestration of liver cysts in polycystic liver disease to improve quality of life: a case report and literature review

• Jung DH
• Hwang S
• Ahn CS
• Moon DB
• Song GW
• Kim KH
• Ha TY
• Park GC
• Lee SG

• KMID: 1802239
• DOI: http://doi.org/10.14701/kjhbps.2015.19.1.40

Abstract

Polycystic liver disease (PCLD) is characterized by a large number of liver cysts scattered throughout the liver parenchyma. We herein intend to present the beneficial effect of palliative fenestration treatment on quality of life in a patient with symptomatic PCLD. A 48-year-old female patient had been followed up for 5 years for both polycystic liver and kidney diseases at another institution. During follow-up for last 1 year, we recognized that she had barely maintained her ability of function in daily activities due to progressive worsening of fatigue and dyspnea on exertion. The patient finally underwent surgical fenestration treatment. Multiple cysts in the enlarged liver were opened and the cyst walls were excised with electrocautery. No surgical complication occurred and the patient was discharged 10 days after the open fenestration surgery. The total liver volume was 3,870 ml before surgery and 3,125 ml at 1 week after surgery, showing a volume reduction of 19.3%. After surgery, her performance status improved significantly. In the present case, significant improvement in quality of life and daily activity performance was achieved after open fenestration treatment over 18 months of follow-up without disease recurrence.

Keyword

Fenestration; Polycystic liver disease; Quality of life; Liver transplantation

MeSH Terms

Dyspnea
Electrocoagulation
Fatigue
Female
Follow-Up Studies
Hepatomegaly
Humans
Kidney Diseases
Liver Diseases*
Liver Transplantation
Liver*
Middle Aged
Quality of Life*
Recurrence

Figure

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  Fig. 1 Computed tomography images of the abdomen. Multiple cysts occupied the liver (A) and both kidneys (B), but the hepatic arterial and portal venous flow was well preserved (C) and hepatic veins were extrinsically compressed (D).

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  Fig. 2 Gross morphology of polycystic liver disease according to Gigot's classification. Type I: presence of less than 10 large hepatic cysts measuring more than 10 cm in maximum diameter. Type II: diffuse involvement of liver parenchyma by multiple cysts with remaining large areas of non-cystic liver parenchyma. Type III: presence of diffuse involvement of liver parenchyma by small- and medium-sized liver cysts with only a few areas of normal liver parenchyma.

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  Fig. 3 Magnetic resonance imaging study of the abdomen. Waterfilled multiple liver and kidney cysts were visible (A). The gallbladder was collapsed by the adjacent liver cysts but the bile duct was not dilated (B).

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  Fig. 4 Operative findings of the patient undergoing open fenestration surgery. Enlarged liver was bulged out of the abdomen (A); There were numerous liver cysts, but the majority of liver parenchyma was preserved (B); Fenestration of the liver cyst led to moderate reduction of the whole liver volume (C); Bulging mass in the abdomen disappeared at the time of abdominal wall closure (D).

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  Fig. 5 Computed tomography follow-up of the abdomen. Multiple distended cysts occupied the liver just before surgery (A); Cyst size was significantly reduced 1 week after fenestration surgery (B); No increase in the size of liver cysts was observed in the 6-month (C) and 18-month (D) images. Because of improvement in the nutritional status, subcutaneous fat was thickened in the 18-month image (D).

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  Fig. 6 Operative findings of a 52-year-old female patient undergoing liver transplantation with a MELD score of 18. Markedly enlarged liver was bulged out of the abdomen (A); There were numerous liver cysts that occupied the majority of liver parenchyma (B); Rupture of the liver cysts led to reduction of the liver volume (C), which facilitated handling of the native liver for liver transplantation (D).

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