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 Table of Contents  
ORIGINAL ARTICLE
Year : 2018  |  Volume : 51  |  Issue : 4  |  Page : 153-157

Laparoscopic liver resection for polycystic liver disease


Department of Surgery, Division of General Surgery, Far Eastern Memorial Hospital, New Taipei City, Taiwan

Date of Submission10-May-2017
Date of Decision27-Aug-2017
Date of Acceptance29-Jan-2018
Date of Web Publication22-Aug-2018

Correspondence Address:
Dr. Kuo-Hsin Chen
No. 21, Section 2, Nan-Ya S. Road, Banciao District, New Taipei City 220
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_76_17

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  Abstract 

Introduction: Polycystic liver disease is uncommon and usually asymptomatic. Surgery is indicated for patients with symptoms or complications. Cyst fenestration has been the main surgical option despite the relatively high recurrence rate. Open hepatectomy and fenestration provided sustainable symptomatic relief in selected patients at the cost of higher morbidity. The role of laparoscopic hepatectomy (LH) for this entity remains unclear.
Methods: A retrospective review of patients with polycystic liver disease receiving LH was undertaken. LH in this study referred to the removal of part of normal liver or complex cysts by dividing Glissonean pedicles to the specimen, excluding simple cyst fenestration. Indication for surgery included symptoms related to mass effect with image-confirmed compression on stomach or duodenum and cyst infection.
Results: From December 2004 to September 2016, a total of 14 patients (12 females and 2 males) with a mean age of 55.9 ± 14.0 years had been enrolled. Gigot's classification in this series includes type I in 3, type II in 8, and type III in 3 cases. Extent of liver resection includes partial S2 and S3 in 6, partial S2 and S3 with associated partial S5 and S6 or S7 in 3, partial S5, S6, and S7 in 1, partial S6 and S7 in 1, left hepatectomy in 2, and right hepatectomy in 1. Perioperative outcomes include median operation time of 240 min (60–300, interquartile range [IQR]: 150), estimated blood loss of 50 ml (5–2000, IQR: 98.8), and mean postoperative hospital stay of 5.5 days (2–77, IQR: 7.3). The 90-day postoperative complications developed in three patients, mainly bile leak and pleural effusion. There was no postoperative mortality.
Conclusion: Laparoscopic liver resection and fenestration for polycystic liver disease seem safe and feasible. The perioperative outcomes were acceptable although the bile leakage rate was relatively high. Careful patient selection to choose peripheral lesions is advisable.

Keywords: Hepatectomy, laparoscopic, liver resection, polycystic liver disease


How to cite this article:
Chen KH, Siow TF, Chen YD, Chio UC, Chang YJ, Loi CM, Lin TC, Huang SY, Hsu CH, Wu JM, Jeng KS. Laparoscopic liver resection for polycystic liver disease. Formos J Surg 2018;51:153-7

How to cite this URL:
Chen KH, Siow TF, Chen YD, Chio UC, Chang YJ, Loi CM, Lin TC, Huang SY, Hsu CH, Wu JM, Jeng KS. Laparoscopic liver resection for polycystic liver disease. Formos J Surg [serial online] 2018 [cited 2018 Nov 17];51:153-7. Available from: http://www.e-fjs.org/text.asp?2018/51/4/153/239553


  Introduction Top


Adult polycystic liver disease (APLD) is an uncommon entity which can occur as a frequent extrarenal manifestation of autosomal dominant polycystic kidney disease, or less commonly, in isolation as autosomal dominant polycystic liver disease.[1]

In most patients, polycystic liver disease has a large range of severity, from asymptomatic incidental finding to progressive development of liver failure.[2] The most common symptoms of APLD include abdominal fullness, early satiety, and abdominal pain due to the mass effect of the cysts. Furthermore, complications such as cyst rupture, bleeding, and infection may develop. Cyst fenestration has been used for symptomatic relief for decades.[3],[4],[5] However, its clinical application has been largely restricted by the relative large abdominal incision and associated complications.[6] Since its introduction in the late 1980s, laparoscopic surgery has been used in almost all kinds of abdominal surgery. The inherent advantages include less wound pain, less wound complications, faster recovery, and shorter hospital stay. Laparoscopic fenestration of liver cyst has been proved to be superior to open approach.[7] For patients with symptoms related to clustered small cysts in a localized anatomical region, hepatectomy can offer better symptomatic relief.[8],[9]

Despite wider and wider application of laparoscopic approach for benign and malignant liver diseases,[10] report of laparoscopic hepatectomy (LH) for polycystic liver disease is limited.[11],[12] Reasons for this include limited working space and distorted anatomical landmarks in polycystic liver. All these make laparoscopic liver resection in APLD more unpredictable and challenging than traditional indications.

Herein, to evaluate the feasibility, safety, and efficacy of laparoscopic liver resection for APLD, we reviewed our single-institutional experience based on a prospectively established database of 695 laparoscopic liver procedures.


  Methods Top


From December 2004 to September 2016, a total of 14 patients with symptomatic APLD receiving LH had been enrolled. The primary indications for surgery included early satiety, abdominal fullness caused by mass effect, and infectious complications of the cysts. Definition of hepatectomy in this study referred to the removal of part of normal liver or complex cysts by dividing Glissonean branches to the specimen. Patients receiving simple cyst fenestration were excluded from this study. Combined fenestration of liver cysts in different anatomical regions had also been performed if indicated. Gigot's classification of polycystic liver disease was used in this study. Type I disease refers to limited number (<10) of large cysts (>10 cm), type II means diffuse involvement of liver parenchyma by multiple medium-sized cysts with remaining large areas of noncystic liver parenchyma, and type III refers to massive, diffuse involvement of liver parenchyma by small- and medium-sized liver cysts, with only a few areas of remaining normal liver parenchyma between cysts[4],[13] [Figure 1]. Clinical data had been obtained from the prospectively established database and retrospective chart review. The primary surgical goal of this approach is to remove the cystic mass causing symptoms while preserving as much functioning liver parenchyma as possible. All patients received computed tomography (CT) scan or magnetic resonance imaging before surgery. For patients developing recurrent symptoms, follow-up CT scan had also been performed to determine whether recurrent cysts caused the symptoms or not. The surgical team decided the extent of liver resection primarily according to the size, pattern, and distribution of the cyst as well as the patient's liver reserve. Child–Pugh score and indocyanide green retention test were the primary tools to evaluate the preoperative liver reserve in this study.
Figure 1: Gigot's classification of polycystic liver disease

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Surgical techniques

The patient was placed in supine position with legs split. The procedure started with minilaparotomy to establish CO2 pneumoperitoneum through a 1-cm periumbilical incision. The positions of other ports depend on the intra-abdominal space because it is not uncommon to find hepatomegaly with limited space. The second 10-mm port can be the major working port or camera port. Two more 5-mm ports will be necessary and will be created according to the triangulation principle of laparoscopic surgery. Adhesion around the liver had been freed. Cysts on the liver surface were fenestrated to decrease the volume of liver and enhance further manipulation. Hepatic hilar was approached and inflow control was performed if feasible. Liver parenchyma was opened with electrical cautery or energy devices. Ultrasonic dissector (Harmonic Scalpel, Ethicon EndoSurgery, Cincinnati, Ohio, USA) and LigaSure (Medtronic Inc., Doral, FL, USA) were the two main types of energy devices used in this series. Cysts along the parenchyma transection plane were opened to facilitate liver transection. Glissonean pedicles on the cyst wall could be controlled with endoscopic stapler or clips. Endoscopic stapler had been used in six patients in this series for Glissonean pedicle division or to facilitate parenchymal dissection. Bleeders and bile-leaking sites had been controlled either with hemoclips or suturing. In this cohort, all but one procedure had been completed by pure laparoscopic approach. We used hand-assisted technique in one patient to facilitate liver mobilization. Closed-suction drain placement after surgery was a routine practice in this study to enhance early detection of bleeding or bile leak. Finally, the specimen was placed in a retrieval bag and removed from extended umbilical wound after being fragmented.


  Results Top


The demographic data of this cohort have been summarized in [Table 1]. Gigot's classification of these patients includes type I in 3, type II in 8, and type III in 3. Six patients had combined polycystic kidney disease. Perioperative outcomes included median operation time of 240 min (60–300, interquartile range [IQR]: 150), estimated blood loss of 50 ml (5–2000, IQR: 98.8), and mean postoperative hospital stay of 5.5 days (2–77, IQR: 7.3) [Table 2].
Table 1: Patient Demographics

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Table 2: Perioperative outcomes

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The 90-day postoperative complications included pleural effusion (Clavien–Dindo II)[14] in one patient and bile leakage (Clavien–Dindo III) and pleural effusion in two patients. Endoscopic placement of biliary stent had been done in the two patients with bile leakage, which confirmed central type bile leakage (the leaking point was in communication with the biliary tree) in one patient and peripheral type in the other. (The leaking point was not in communication with the biliary tree.) No patient experienced postoperative hemorrhage needing intervention. There was no postoperative mortality in this cohort. The longest hospital stay happened to a 64-year-old woman with combined liver and kidney polycystic disease. She was intubated for massive pleural effusion, respiratory and liver failure, and sepsis before surgery. Disease severity and postoperative biliary leakage were the main reasons for her long postoperative stay. This patient died of pneumonia 7 years after surgery. Despite progressive deterioration of liver and kidney function, polycystic liver and kidney disease was not the direct cause of mortality in this patient.

Two patients developed recurrent symptoms 9 and 21 months, respectively, after the surgery. No further intervention had been suggested for any image-confirmed cystic mass recurrence.


  Discussion Top


Treatment of polycystic liver disease, including medical and surgical options, depends on the distribution pattern of the cysts and volume of remnant functioning liver.[2],[12],[13],[15],[16] Medical treatment of polycystic liver disease includes cimetidine or somatostatin analogs to decrease cyst fluid secretion from the cystic epithelium. However, there is no controlled trial to support this antisecretin-directed therapy.[17] For patients with few dominant large cysts that cause symptoms, percutaneous aspiration and sclerotherapy is an attractive option for its less invasiveness.[18]

For patients of Gigot's type I disease, treatment options include aspiration and sclerotherapy, fenestration or combined fenestration, and liver resection. For type II and III diseases, surgical choices vary from cyst fenestration with or without combined liver resection to liver transplantation.[19] The incidences of complications were in the range of 28.4% in cyst fenestration, 31.8% in liver resection, and 61.5% in liver transplantation.[20]

The technique of open fenestration of liver cysts, first described by Prof. Lin et al.[3] in 1968, included sequential fenestration of cystic wall from superficial to deep region to decrease liver volume and subsequently control the encountered bleeding and bile leak. This technique is still valid in the modern era and especially useful in the caudal-to-cranial approach of laparoscopic liver resection.[3] Laparoscopic fenestration of liver cysts has been used to relieve symptoms shortly after the development of laparoscopic liver surgery. However, short symptom-free interval and high percentage of reintervention had been reported.[7] Effective cyst fenestration depends on the number and anatomical distribution of cyst-causing symptoms. For large or massive cysts in dome or posterior segments, laparoscopic fenestration can be challenging. Several factors have been proposed in selecting patients for laparoscopic fenestration. These factors include (1) liver volume, (2) diameter of individual cysts, (3) location of cysts, (4) complications by strategically localized cysts, (5) availability of experienced laparoscopists, and (6) possible candidacy for potential liver transplantation.[21],[22],[23]

Liver resection for patients with symptoms related to the mass effect offers better symptomatic relief.[24],[25] Moreover, liver resection makes space for future liver regeneration.[21] Although few studies addressed this issue, this could be an additional benefit of liver resection for polycystic liver disease.

After the 2nd international consensus meeting of laparoscopic liver resection, several advantages of LH had been confirmed.[26] By following all these conceptual changes, minimally invasive liver surgery has been applied in wider and wider indications including tumors in unfavorable locations,[27] donor harvest for living donor liver transplantation,[28] or patients with anatomical variations by traditional laparoscopic or robotic approach.[29]

Parenchymal transection in liver resection for polycystic liver disease can be facilitated by opening the cysts along the transection plane.[3] However, caution should be taken to avoid the vessels and biliary trees which will be more unpredictable due to the anatomical distortion in polycystic liver disease. To facilitate parenchymal transection, endoscopic stapler control of the cystic wall had been found successful and time saving. This technique may also help to avoid delayed bile leak. The importance of careful dissection and wise avoidance of hilar structure cannot be overemphasized. The attempt to control the proximal liver pedicle and then transect the liver parenchyma along the demarcation line may not work well. The demarcation line can be difficult to define in patients with polycystic liver disease.

Bile leak is a possible complication of significant clinical implication and should be handled carefully. There was more bile leak in this cohort than other LH patients in our institute. Two patients in this series experienced bile leak after laparoscopic S23 partial hepatectomy. However, none of the remaining 73 patients receiving laparoscopic S23 resection for other indications in our institute exprienced bile leak after surgery. Careful management of Glissonean pedicles and structures near the hepatic hilum is important to avoid possible injury. Staple closure of cystic septum is effective; however, appropriate choose of cartridge is crucial to avoid bleeding or bile leakage. For patients with infected cysts, dense adhesion between liver and adjacent organs should be taken into consideration and dissected carefully.

Postoperative bile leak can be very troublesome since the risk of bile leaking from disconnected liver parenchyma (peripheral type) is higher due to the distorted liver anatomy. Wise dissection of liver parenchyma to stay away from hilar structure is recommended to avoid biliary complications.[21] For patients with bile leak after surgery, endoscopic placement of biliary stent is indicated in most patients to shorten recovery.[30]

Unlike the specimen retrieval of malignant hepatic tumor, benign specimen could be fragmented before removing from extended umbilical wound. This will further decrease the wound size and ensure the benefits of minimally invasive surgery. However, the possibility of associated cystcarcinoma or cholangiocarcinoma should be kept in mind in patients with suspicious imaging findings or elevated tumor markers.[31]


  Conclusion Top


Despite relatively high postoperative biliary complications, LH for symptomatic polycystic liver disease seems safe with acceptable short-term perioperative outcome and long-term symptom relief. For patients with symptoms due to mass effect of the cysts, long sustainable relief could be expected after combined liver resection and fenestration. In some, at least younger patients, regeneration of normal liver parenchyma had been observed after debulking hepatectomy. Biliary complication remains the “Achilles's heel” in hepatectomy for patients with APLD. Selecting peripheral lesions, avoiding leaving functioning, disconnected liver behind, and avoiding patients with poor liver/kidney reserve are advisable to decrease biliary complications.

Further large-scale, controlled studies are highly expected to clarify the benefits of this approach.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

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