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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 - Korean J Hepatobiliary Pancreat Surg (2015)

Bottom Line: Multiple cysts in the enlarged liver were opened and the cyst walls were excised with electrocautery.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.

View Article: PubMed Central - PubMed

Affiliation: Division of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

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.

No MeSH data available.


Related in: MedlinePlus

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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Figure 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.

Mentions: During the outpatient clinic follow-up for 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. Finally, she complained of shortness of breath even in a resting state. Eastern Cooperative Oncology Group (ECOG) performance status worsened from 2 to 3 during observation for 1 year.7 Gastrointestinal Quality of Life Index (GIQLI) score also deteriorated from 75 to 44 during observation for 1 year.8 Physical examination revealed that the liver was prominently bulged out of the abdomen, but leg edema was absent. Liver and kidney functions were still quite normal. Abdominal computed tomography (CT) showed progressive enlargement of multiple liver cysts with smooth and regular walls (Fig. 1), which was regarded as type III according to Gigot's classification (Fig. 2),9 grade 4 according to Qian's classification (Table 1),10 and type C according to Schnelldorfer's classification (Table 2).11 Magnetic resonance imaging study revealed water-filled multiple liver and kidney cysts (Fig. 3).


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 - Korean J Hepatobiliary Pancreat Surg (2015)

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.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4494095&req=5

Figure 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.
Mentions: During the outpatient clinic follow-up for 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. Finally, she complained of shortness of breath even in a resting state. Eastern Cooperative Oncology Group (ECOG) performance status worsened from 2 to 3 during observation for 1 year.7 Gastrointestinal Quality of Life Index (GIQLI) score also deteriorated from 75 to 44 during observation for 1 year.8 Physical examination revealed that the liver was prominently bulged out of the abdomen, but leg edema was absent. Liver and kidney functions were still quite normal. Abdominal computed tomography (CT) showed progressive enlargement of multiple liver cysts with smooth and regular walls (Fig. 1), which was regarded as type III according to Gigot's classification (Fig. 2),9 grade 4 according to Qian's classification (Table 1),10 and type C according to Schnelldorfer's classification (Table 2).11 Magnetic resonance imaging study revealed water-filled multiple liver and kidney cysts (Fig. 3).

Bottom Line: Multiple cysts in the enlarged liver were opened and the cyst walls were excised with electrocautery.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.

View Article: PubMed Central - PubMed

Affiliation: Division of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

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.

No MeSH data available.


Related in: MedlinePlus