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Table.  
Patient Characteristics and Extent of Operation
Patient Characteristics and Extent of Operation
1.
Vauthey  JN, Maddern  GJ, Blumgart  LH.  Adult polycystic disease of the liver. Br J Surg. 1991;78(5):524-527.
PubMedArticle
2.
Chauveau  D, Fakhouri  F, Grünfeld  JP.  Liver involvement in autosomal-dominant polycystic kidney disease: therapeutic dilemma. J Am Soc Nephrol. 2000;11(9):1767-1775.
PubMed
3.
Schnelldorfer  T, Torres  VE, Zakaria  S, Rosen  CB, Nagorney  DM.  Polycystic liver disease: a critical appraisal of hepatic resection, cyst fenestration, and liver transplantation. Ann Surg. 2009;250(1):112-118.
PubMedArticle
4.
van Keimpema  L, Nevens  F, Vanslembrouck  R,  et al.  Lanreotide reduces the volume of polycystic liver: a randomized, double-blind, placebo-controlled trial. Gastroenterology. 2009;137(5):1661-1668.
PubMedArticle
5.
Chrispijn  M, Nevens  F, Gevers  TJ,  et al.  The long-term outcome of patients with polycystic liver disease treated with lanreotide. Aliment Pharmacol Ther. 2012;35(2):266-274.
PubMedArticle
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Research Letter
Pacific Coast Surgical Association
January 2015

Management of Symptomatic Polycystic Liver Disease With Hepatic Resection

Author Affiliations
  • 1Department of General Surgery, Oregon Health and Science University, Portland
  • 2Division of Abdominal Organ Transplantation/Hepatobiliary Surgery, Oregon Health and Science University, Portland
JAMA Surg. 2015;150(1):81-82. doi:10.1001/jamasurg.2014.307

Polycystic liver disease causes devastating symptoms owing to hepatomegaly. Aspiration or fenestration fails to definitively remove the cyst wall and results in the reaccumulation of cyst fluid. Preserved hepatic function generally precludes consideration of a liver transplant, leaving hepatic resection the best available treatment option for patients with impaired quality of life. This case series outlines a successful outcome using this operative strategy.

Methods

A retrospective, single-center medical record review approved by the Oregon Health and Science University institutional reivew board was performed for patients with polycystic liver disease who underwent formal liver resection (2-4 segments) by 1 surgeon during the period from June 2001 to June 2013. All patients with polycystic liver disease who had severe symptoms and normal liver and kidney function were offered this treatment. Our operative technique included formal resection of the segment or lobe with the largest cysts. The remaining unresected cysts were drained and partially resected using an endogastrointestinal vascular stapler, with marsupialization via a 3-layer staple line, oversewing any internal communication with the biliary tract (5-0 absorbable sutures) to avoid sclerosing the biliary tree, and sclerosis of the remaining cyst wall by 15 to 20 minutes of exposure to laparotomy sponges soaked in alcohol, 70%, or betadine.

Results

Eleven patients were treated with a median duration of follow-up of 32 months (range, 6-146 months). All patients described significant improvement in their symptoms. No patients required a reoperation for symptoms. Complications included incisional hernias, postoperative ascites that resolved with medical therapy, secondary bacterial peritonitis from infected ascites, and bile leakage (Table). Four of the patients received long-acting octreotide acetate postoperatively for treatment of remaining large cysts.

Discussion

The primary aims of hepatic resection are to reduce liver volume by more than 50% and to remove or drain any infected or bleeding cysts.1,2 As the initial step in the operation, drainage and partial resection of the larger cysts provide better exposure to the porta hepatis and facilitate liver mobilization. Our series had excellent results with the use of the endogastrointestinal staple device instead of suture ligatures.3 Whereas fenestration entails creating holes in liver cysts, this marsupialization technique opened up the cyst completely, with partial resection via an endogastrointestinal anastomosis stapler and sclerosis of any residual cyst wall to prevent regrowth. Because the extent of liver resection is determined at the time of operation, and owing to the necessity of adequate liver mobilization, an open approach is the preferred surgical technique for hepatic resection. Perihepatic drain placement addresses the accumulation of intra-abdominal fluid, and with its placement, we can monitor the development of biliary leaks.

Octreotide therapy has been shown to decrease the volume of liver cysts, as well as prevent the growth of new cysts; however, 15% of patients do not respond to this type of therapy.4 Our patients failed to develop the most common adverse effects of octreotide, mainly abdominal cramps or diarrhea. Discontinuation of octreotide therapy has resulted in the immediate recurrence in the growth of liver cysts, which suggests that continuous treatment is important for long-lasting effect.5

In conclusion, the preferred approach for patients with severely symptomatic polycystic liver disease with preserved liver and renal function is hepatic resection with drainage, partial liver resection, sclerosis, and marsupialization of the remaining cysts. This safe and feasible strategy helps patients avoid a liver transplant, experience resolution of their symptoms, and have an improved quality of life. Our series of patients experienced long-term success in eradiating symptoms after the majority of them had failed previous, less aggressive surgical options. There were no major complications, no need for reoperation, and no deaths. 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.

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Article Information

Corresponding Author: Jennifer Tseng, MD, Department of General Surgery, Oregon Health and Science University, 3181 SW Sam Jackson Park Rd, L223, Portland, OR 97239 (tseng@ohsu.edu).

Published Online: November 26, 2014. doi:10.1001/jamasurg.2014.307.

Author Contributions: Drs Tseng and Orloff had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

Study concept and design: Tseng, Orloff.

Acquisition, analysis, or interpretation of data: Tseng, Orloff.

Drafting of the manuscript: Tseng.

Critical revision of the manuscript for important intellectual content: Tseng, Orloff.

Statistical analysis: Tseng.

Administrative, technical, or material support: Orloff.

Study supervision: Orloff.

Conflict of Interest Disclosures: None reported.

Previous Presentation: This paper was presented at the 85th Annual Meeting of the Pacific Coast Surgical Association; February 15, 2014; Dana Point, California.

References
1.
Vauthey  JN, Maddern  GJ, Blumgart  LH.  Adult polycystic disease of the liver. Br J Surg. 1991;78(5):524-527.
PubMedArticle
2.
Chauveau  D, Fakhouri  F, Grünfeld  JP.  Liver involvement in autosomal-dominant polycystic kidney disease: therapeutic dilemma. J Am Soc Nephrol. 2000;11(9):1767-1775.
PubMed
3.
Schnelldorfer  T, Torres  VE, Zakaria  S, Rosen  CB, Nagorney  DM.  Polycystic liver disease: a critical appraisal of hepatic resection, cyst fenestration, and liver transplantation. Ann Surg. 2009;250(1):112-118.
PubMedArticle
4.
van Keimpema  L, Nevens  F, Vanslembrouck  R,  et al.  Lanreotide reduces the volume of polycystic liver: a randomized, double-blind, placebo-controlled trial. Gastroenterology. 2009;137(5):1661-1668.
PubMedArticle
5.
Chrispijn  M, Nevens  F, Gevers  TJ,  et al.  The long-term outcome of patients with polycystic liver disease treated with lanreotide. Aliment Pharmacol Ther. 2012;35(2):266-274.
PubMedArticle
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