Jung Dong-Hwan, Hwang Shin, Ahn Chul-Soo, Moon Deok-Bog, Song Gi-Won, Kim Ki-Hun, Ha Tae-Yong, Park Gil-Chun, Lee Sung-Gyu
Division of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Korean J Hepatobiliary Pancreat Surg. 2015 Feb;19(1):40-6. doi: 10.14701/kjhbps.2015.19.1.40. Epub 2015 Feb 28.
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.
多囊肝病(PCLD)的特征是大量肝囊肿散布于整个肝实质。我们在此旨在阐述姑息性开窗治疗对有症状的PCLD患者生活质量的有益影响。一名48岁女性患者在另一机构对多囊肝和多囊肾病进行了5年的随访。在过去1年的随访期间,我们认识到由于疲劳和劳力性呼吸困难的进行性加重,她几乎无法维持日常活动功能。该患者最终接受了手术开窗治疗。切开肿大肝脏中的多个囊肿,用电灼切除囊肿壁。未发生手术并发症,患者在开放性开窗手术后10天出院。术前肝脏总体积为3870 ml,术后1周为3125 ml,体积减少了19.3%。术后,她的体能状态显著改善。在本病例中,经过18个月的随访,开放性开窗治疗后生活质量和日常活动表现得到显著改善,且无疾病复发。