**5. Treatment**

**Figure 14.** CT showing coexistence of two hepatic lesions: central simple hepatic cyst, in close proximity with gallblad‐ der, and hepatic hemangioma in segment VI. (A) Axial view, native; (B) axial view, after i.v. contrast, arterial phase; (C) sagittal view, after i.v. contrast, arterial phase. Patient was submitted to laparoscopic fenestration of the hepatic

**Figure 13.** CT, axial view: voluminous hydatid cyst in the right hepatic lobe and small cyst mass in segment II-III. (A)

Nonparasitic cysts can also coexist with peripheral cholangiocarcinoma. The association was found in patients with PLD after kidney transplantation. The presence of liver metastasis from

**Figure 15.** CT, axial view, with (A) and without (B) i.v. contrast showing coexistence of a bulk solid liver metastasis

cyst. The operation was successful in abolishing the right quadrant pain.

Native; (B) after administration of i.v. contrast.

264 Recent Advances in Liver Diseases and Surgery

from colonic cancer in a patient with previously known PLD.

colorectal cancer was also found in patients with PLD (Figure 15).

Asymptomatic single liver cysts do not require treatment or surveillance. Symptomatic and complicated hepatic cysts require therapeutic intervention (Algorithm 2). It is considered that surgery is necessary for cyst of more than 5 cm in diameter. The primary indication for surgery is troublesome pain.

#### **5.1. Percutaneous treatment**

Percutaneous aspiration under ultrasound guidance of the cyst fluid is a mini-invasive procedure with a high recurrence rate if it is not associated with injection of a sclerosing agent in the remnant cavity.

Percutaneous treatment is performed on an inpatient basis under local anesthesia with lidocaine or sedation. For cyst puncture, an 18-gauge aspiration needle can be used. The puncture line must be chosen through normal hepatic parenchyma to avoid fluid leakage into peritoneal cavity. To prevent intracystic bleeding during evacuation caused by the sharp contact of the needle with the cyst wall, a 6- to 7-F catheter can be used instead. After complete evacuation of the cyst, the fluid is sent to cytological and bacteriological examination. Even in the absence of obvious signs of communication of the cyst with the biliary tree, this possibility must also be ruled out before injecting the sclerosant. Otherwise, an irreversible sclerosing cholangitis may supervene. There are available some imagistic methods to check the commu‐ nication between liver cyst and biliary ducts. The most feasible and reliable method is the injection of a diluted US contrast agent (e.g., 2–4 drops of SonoVue, Bracco International B.V. in 40–200 ml 0.9% saline) in the evacuated cyst. The volume of contrast agent injected is the same or lower than the aspirated one [33]. Other alternative methods in ruling out cyst–biliary tree communication are cystography, ERCP, and bile duct scintigraphy [23].

Only after the absence of biliary communication is certified, the instillation of the sclerosant is allowed. One has to choose from a list of sclerosing agents that include ethanol [34–36], minocycline hydrochloride [37], tetracycline chloride [38], hypertonic saline solution [39], polidocanol [40], and ethanolamine oleate [41].

Ethanol has remained the most used sclerosing agent. It destroys the cell lining of the cystic cavity and discontinues the cystic fluid secretion. Different concentrations of ethanol (95–99%) and different volumes (10–50% of the cystic volume) have been reported. The exposure time varies from 10 min to 4 h. In order to minimize ethanol side effects, time exposure to ethanol should be less than 60 min. When using ethanol instillation, the cyst must be completely evacuated, a condition that is not necessary when using other sclerosing agents. After complete cyst evacuation, its walls collapse hampering the thorough distribution of sclerosant, with the risk of its subsequent lobulation. The other sclerosants are active in small concentration and do not need complete evacuation of the cystic fluid. When the cyst is only partially evacuated, the misplacement of needle tip is also avoided. The residual fluid is progressively resorbed through microscopic communication between the cyst and the surrounding liver parenchyma. Polidocanol needs only one application. Its application is painless and hence no intracystic anesthesia or sedation is required [33].

The percutaneous treatment can be repeated when necessary. Percutaneous drainage must be done under antibiotic prophylaxis. The recommended antimicrobial is ciprofloxacin because cephalosporins (e.g., cefazolin) were not found totally successful in preventing cyst infection after drainage [42]. Even if infection of the remnant cavity occurs, it can be remitted by oral Ciprofloxacin (500 mg twice daily) associated or not with clindamycin (300 mg three times daily) [42]. Oral ciprofloxacin is also indicated as the first line treatment in infected cysts [43].

#### **5.2. Radiofrequency ablation**

Radiofrequency ablation (RFA) for the treatment of hepatic cysts with the largest diameter up to 10 cm was reported to be efficient, safe, and free from complications [44].

#### **5.3. Surgical treatment**

High success rates in the treatment of hepatic cysts were reported being associated with laparoscopic or open deroofing [24].

#### *5.3.1. Laparoscopic approach*

Laparoscopic fenestration was first reported by Z'graggen in 1991 [45]. Being associated with low recurrence rate, reduced morbidity, and short hospital stay, laparoscopic fenestration tends to become the standard treatment for the simple hepatic cysts.

The indications for laparoscopic approach are determined not by the size but the location of the cysts, being limited to those located in the segments II, III, IV, V, and VI (Figure 16) [29]. However, some authors advocate that the use of a flexible laparoscope facilitates the laparo‐ scopic approach of lesions located in the postero-superior segments of the liver (segments I, VII, and VIII) [46]. Hepatic cysts that cannot be entirely explored laparoscopy are not candi‐ dates for laparoscopic approach.

minocycline hydrochloride [37], tetracycline chloride [38], hypertonic saline solution [39],

Ethanol has remained the most used sclerosing agent. It destroys the cell lining of the cystic cavity and discontinues the cystic fluid secretion. Different concentrations of ethanol (95–99%) and different volumes (10–50% of the cystic volume) have been reported. The exposure time varies from 10 min to 4 h. In order to minimize ethanol side effects, time exposure to ethanol should be less than 60 min. When using ethanol instillation, the cyst must be completely evacuated, a condition that is not necessary when using other sclerosing agents. After complete cyst evacuation, its walls collapse hampering the thorough distribution of sclerosant, with the risk of its subsequent lobulation. The other sclerosants are active in small concentration and do not need complete evacuation of the cystic fluid. When the cyst is only partially evacuated, the misplacement of needle tip is also avoided. The residual fluid is progressively resorbed through microscopic communication between the cyst and the surrounding liver parenchyma. Polidocanol needs only one application. Its application is painless and hence no intracystic

The percutaneous treatment can be repeated when necessary. Percutaneous drainage must be done under antibiotic prophylaxis. The recommended antimicrobial is ciprofloxacin because cephalosporins (e.g., cefazolin) were not found totally successful in preventing cyst infection after drainage [42]. Even if infection of the remnant cavity occurs, it can be remitted by oral Ciprofloxacin (500 mg twice daily) associated or not with clindamycin (300 mg three times daily) [42]. Oral ciprofloxacin is also indicated as the first line treatment in infected cysts [43].

Radiofrequency ablation (RFA) for the treatment of hepatic cysts with the largest diameter up

High success rates in the treatment of hepatic cysts were reported being associated with

Laparoscopic fenestration was first reported by Z'graggen in 1991 [45]. Being associated with low recurrence rate, reduced morbidity, and short hospital stay, laparoscopic fenestration

The indications for laparoscopic approach are determined not by the size but the location of the cysts, being limited to those located in the segments II, III, IV, V, and VI (Figure 16) [29]. However, some authors advocate that the use of a flexible laparoscope facilitates the laparo‐ scopic approach of lesions located in the postero-superior segments of the liver (segments I, VII, and VIII) [46]. Hepatic cysts that cannot be entirely explored laparoscopy are not candi‐

to 10 cm was reported to be efficient, safe, and free from complications [44].

tends to become the standard treatment for the simple hepatic cysts.

polidocanol [40], and ethanolamine oleate [41].

266 Recent Advances in Liver Diseases and Surgery

anesthesia or sedation is required [33].

**5.2. Radiofrequency ablation**

laparoscopic or open deroofing [24].

dates for laparoscopic approach.

**5.3. Surgical treatment**

*5.3.1. Laparoscopic approach*

**Figure 16.** CT showing serous hepatic cyst in segment VI–VII. (A) Axial view, (B) coronal view, and (C) sagittal view. Laparoscopic approach is not indicated for such localization of the hepatic cyst.

Laparoscopy is not suitable for fenestration in case of close proximity of the cyst wall to the hepatic veins and inferior vena cava (Figure 17) [47].

**Figure 17.** CT, axial view, with contrast (A) and without contrast (B): large simple hepatic cyst in contact with the infe‐ rior vena cava and hepatic veins and other two small cysts in proximity. Patient was submitted to open surgery.

Previous laparotomies or laparoscopies are not considered contraindications to the laparo‐ scopic approach.

A 30° laparoscope is used. A medium CO2 insufflation and an intra-abdominal pressure less than 12 mm Hg should be used to avoid gas embolism. Liver veins are little prone to col‐ lapse in the supine position. The effect of venous gas embolism depends on the rate of CO2 infusion and its volume. For adults, the potentially lethal volume is estimated at 200–300 ml or 3–5 ml/kg [48]. Surgeons must routinely purge laparoscopic tubing systems with CO2 gas. If the system is not adequately purged with CO2 gas before, substantial amounts of air (con‐ taining 79% of the insoluble N2) may be insufflated into the peritoneal cavity from the tub‐ ing and may cause air embolism [48].Generally, the patient lies in the supine position with/ without abducted legs or lithotomy position on the operating table. A left side-lying posi‐ tion of the patient can help approaching liver cysts located in the segments V-VI.

applied [46].

The operative procedure in the standard operation of fenestration is performed using three or four ports. One 10-mm trocar is inserted into the abdominal cavity through the umbilicus as an observation port. The main operating hole is located under the xiphoid and is made to accommodate a 10- or 12-mm trocar. Another hole is placed in the right upper quadrant of the abdomen at the medioclavicular line for insertion of a 5-mm trocar. An additional port can be placed for exposure, depending on the cyst localization. quadrant of the abdomen at the medioclavicular line for insertion of a 5‐mm trocar. An additional port can be placed for

On video inspection, the liver mass appears exteriorized on the hepatic surface with a translucent wall. If the serous hepatic cyst has no complication, there are no tight adhesions to the surrounding tissues. Instead, if the cyst is complicated by inflammation, infection, or hemorrhage, the surface of the corticalized cyst may develop tight adhesions to the surround‐ ing organs and the cyst wall appears thick and thus indistinguishable from other hepatic cysts (e.g., hydatid cyst, pyogenic abscess, and tuberculoma) (Figure 18). exposure, depending on the cyst localization. On video inspection, the liver mass appears exteriorized on the hepatic surface with a translucent wall. If the serous hepatic cyst has no complication, there are no tight adhesions to the surrounding tissues. Instead, if the cyst is complicated by inflammation, infection, or hemorrhage, the surface of the corticalized cyst may develop tight adhesions to the surrounding organs and the cyst wall appears thick and thus indistinguishable from other hepatic cysts (e.g., hydatid cyst, pyogenic abscess, and tuberculoma) (Figure 18).

cyst with adhesions to the diaphragm and with thick wall that make it undistinguishable from hydatid cyst. (B) Intraoperative aspect of the aspirated liquid. The brownish color is likely the result of an unrecognized intracystic hemorrhage. **Figure 18.** Serous cyst in the segments IV, V, and VIII. (A) Intraoperative aspect of the complicated hepatic cyst with adhesions to the diaphragm and with thick wall that make it undistinguishable from hydatid cyst. (B) Intraoperative aspect of the aspirated liquid. The brownish color is likely the result of an unrecognized intracystic hemorrhage.

**Figure 18.** Serous cyst in segment IV, V, and VIII. (A) Intraoperative aspect of complicated hepatic

After cyst evacuation, a large access to the cavity of the cyst should be obtained. The walls of the cyst are carefully inspected to identify possible indentation, vegetation, or thickness. Any suspicious lesion of the cyst wall must be biopsied and send to frozen examination to rule out malignancy. If neoplastic changes are found, the laparoscopy is converted to open operation, and liver resection is performed. If mural nodules, intracystic septum, or honeycomb appearance is visualized, but there is no microscopic evidence of cystadenoma or cystadenocarcinoma on frozen sections, hepatectomy can be performed laparoscopically to clear all the affected segments. Any suspected communication with the bile duct should be carefully investigated intraoperatively. Some authors consider the closure of the open bile duct under laparotomy to be a more rational choice [49]. Other authors advocate for After cyst evacuation, a large access to the cavity of the cyst should be obtained. The walls of the cyst are carefully inspected to identify possible indentation, vegetation, or thickness. Any suspicious lesion of the cyst wall must be biopsied and sent to frozen examination to rule out malignancy. If neoplastic changes are found, the laparoscopy is converted to open operation, and liver resection is performed. If mural nodules, intracystic septum, or honeycomb appear‐ ance are visualized, but there is no microscopic evidence of cystadenoma or cystadenocarci‐ noma on frozen sections, hepatectomy can be performed laparoscopically to clear all the affected segments.

laparoscopic closure of the communication with the bile duct [50]. Recurrence is low if wide cyst wall resection is accomplished. Fenestration can be carried out by electrocautery hook, bipolar scissors, high frequency bipolar electrocoagulation LigaSureTM (Valleylab, Tyco International Healthcare, Boulder, CO), Ethicon Ultracision Harmonic Scalpel LCS‐5 (Ethicon, Cincinnati, Ohio), or cutting stapler. Argon beam coagulation may complete hemostasis. The cystic dome is resected up to 3–5 mm from the hepatic parenchyma to avoid Any suspected communication with the bile duct should be carefully investigated intraoper‐ atively. Some authors consider the closure of the open bile duct under laparotomy to be a more rational choice [49]. Other authors advocate for laparoscopic closure of the communication with the bile duct [50].

hemorrhage and bile leakage from the cut edge of the liver parenchyma. The resected cyst is sent for permanent histopathologic evaluation. If the cyst is not well corticalized on the liver capsule and the standard unroofing of the cyst is considered of inadequate size that can predispose to recurrence, then the cyst wall should be excised including a 3‐ to 4‐mm rim of hepatic parenchyma. If the bile is spotted along the cystic edge, a hemostatic clip or a tie suture can be Recurrence is low if wide cyst wall resection is accomplished. Fenestration can be carried out by electrocautery hook, bipolar scissors, high frequency bipolar electrocoagulation Liga‐ SureTM (Valleylab, Tyco International Healthcare, Boulder, CO), Ethicon Ultracision Harmonic Scalpel LCS-5 (Ethicon, Cincinnati, Ohio), or cutting stapler. Argon beam coagulation may

In case of aspiration of a straw‐colored liquid and in the absence of bile staining of the cystic wall, intraoperative cholangiography is not indicated. However, in case of bile contamination of the cyst or compression of the biliary tree, intraoperative cholangiography is required to evaluate the biliary tree [51]. Intraoperatively missed biliary

Sclerosant or alcohol application on the remnant wall of the cyst is recommended to prevent possible further fluid secretion by cyst epithelium and, hence, hepatic cyst recurrence. The great omentum should be packed into larger cavities to decrease dead space and prevent fluid collection. An omental flap transposition is recognized as an important

The drainage of the remnant cavity must meet the declivity principle, especially for those cysts situated in the upper liver segments. The main reason for reconstitution of the cyst after operation is the coverage of the remnant cavity by the diaphragm. To establish a dry residual cavity after fenestration of liver cysts located in the segments VII and VIII, the vacuum effect of the respiratory movements of the diaphragm should be counteracted by an efficient external drainage. The drainage tube should be left in place until complete cessation of the secretion certified by ultrasound evaluation. The use of pigtail catheters for drainage is preferred to the usual drainage tubes for upper sited liver cysts. The realization of

communication could lead to prolonged biliary fistula and even biliary peritonitis.

aid in reducing the risk of cyst recurrence, but it is contraindicated in infected cysts.

15

an efficient external drainage might be accomplished by transparenchymatous placement of the tube.

complete hemostasis. The cystic dome is resected up to 3–5 mm from the hepatic parenchyma to avoid hemorrhage and bile leakage from the cut edge of the liver parenchyma. The resected cyst is sent for frozen section and permanent histopathologic evaluation. If the cyst is not well corticalized on the liver capsule and the standard unroofing of the cyst is considered of inadequate size that can predispose to recurrence, then the cyst wall should be excised including a 3- to 4-mm rim of hepatic parenchyma. If the bile is spotted along the cystic edge, a hemostatic clip or a tie suture can be applied [46].

In case of aspiration of a straw-colored liquid and in the absence of bile staining of the cystic wall, intraoperative cholangiography is not indicated. However, in case of bile contamination of the cyst or compression of the biliary tree, intraoperative cholangiography is required to evaluate the biliary tree [51]. Intraoperatively missed biliary communication could lead to prolonged biliary fistula and even biliary peritonitis.

Sclerosant or alcohol application on the remnant wall of the cyst is recommended to prevent possible further fluid secretion by cyst epithelium and, hence, hepatic cyst recurrence. The great omentum should be packed into larger cavities to decrease dead space and prevent fluid collection. An omental flap transposition is recognized as an important aid in reducing the risk of cyst recurrence, but it is contraindicated in infected cysts.

The drainage of the remnant cavity must meet the declivity principle, especially for those cysts situated in the upper liver segments. The main reason for reconstitution of the cyst after operation is the coverage of the remnant cavity by the diaphragm. To establish a dry residual cavity after fenestration of liver cysts located in the segments VII and VIII, the vacuum effect of the respiratory movements of the diaphragm should be counteracted by an efficient external drainage. The drainage tube should be left in place until complete cessation of the secretion certified by ultrasound evaluation. The use of pigtail catheters for drainage is preferred to the usual drainage tubes for upper sited liver cysts. The realization of an efficient external drainage might be accomplished by transparenchymatous placement of the tube.

The atypical hepatic resections for large and/or multiple hepatic cysts that occupy more segments are laparoscopically feasible if performed by surgeons with advanced training in this technique. Most of these atypical hepatectomies involve the left lobe (Figure 19). Generally, the cut surface of the liver remains covered by the cyst wall.

#### *5.3.2. Single-incision laparoscopy*

The operative procedure in the standard operation of fenestration is performed using three or four ports. One 10-mm trocar is inserted into the abdominal cavity through the umbilicus as an observation port. The main operating hole is located under the xiphoid and is made to accommodate a 10- or 12-mm trocar. Another hole is placed in the right upper quadrant of the abdomen at the medioclavicular line for insertion of a 5-mm trocar. An additional port can be

On video inspection, the liver mass appears exteriorized on the hepatic surface with a translucent wall. If the serous hepatic cyst has no complication, there are no tight adhesions to the surrounding tissues. Instead, if the cyst is complicated by inflammation, infection, or hemorrhage, the surface of the corticalized cyst may develop tight adhesions to the surround‐ ing organs and the cyst wall appears thick and thus indistinguishable from other hepatic cysts

On video inspection, the liver mass appears exteriorized on the hepatic surface with a translucent wall. If the serous hepatic cyst has no complication, there are no tight adhesions to the surrounding tissues. Instead, if the cyst is complicated by inflammation, infection, or hemorrhage, the surface of the corticalized cyst may develop tight adhesions to the surrounding organs and the cyst wall appears thick and thus indistinguishable from other hepatic cysts (e.g.,

> **Figure 18.** Serous cyst in segment IV, V, and VIII. (A) Intraoperative aspect of complicated hepatic cyst with adhesions to the diaphragm and with thick wall that make it undistinguishable from hydatid cyst. (B) Intraoperative aspect of the aspirated liquid. The brownish color is likely the

**Figure 18.** Serous cyst in the segments IV, V, and VIII. (A) Intraoperative aspect of the complicated hepatic cyst with adhesions to the diaphragm and with thick wall that make it undistinguishable from hydatid cyst. (B) Intraoperative aspect of the aspirated liquid. The brownish color is likely the result of an unrecognized intracystic hemorrhage.

After cyst evacuation, a large access to the cavity of the cyst should be obtained. The walls of the cyst are carefully inspected to identify possible indentation, vegetation, or thickness. Any suspicious lesion of the cyst wall must be biopsied and send to frozen examination to rule out malignancy. If neoplastic changes are found, the laparoscopy is converted to open operation, and liver resection is performed. If mural nodules, intracystic septum, or honeycomb appearance is visualized, but there is no microscopic evidence of cystadenoma or cystadenocarcinoma on frozen

After cyst evacuation, a large access to the cavity of the cyst should be obtained. The walls of the cyst are carefully inspected to identify possible indentation, vegetation, or thickness. Any suspicious lesion of the cyst wall must be biopsied and sent to frozen examination to rule out malignancy. If neoplastic changes are found, the laparoscopy is converted to open operation, and liver resection is performed. If mural nodules, intracystic septum, or honeycomb appear‐ ance are visualized, but there is no microscopic evidence of cystadenoma or cystadenocarci‐ noma on frozen sections, hepatectomy can be performed laparoscopically to clear all the

Any suspected communication with the bile duct should be carefully investigated intraoperatively. Some authors consider the closure of the open bile duct under laparotomy to be a more rational choice [49]. Other authors advocate for

Recurrence is low if wide cyst wall resection is accomplished. Fenestration can be carried out by electrocautery hook, bipolar scissors, high frequency bipolar electrocoagulation LigaSureTM (Valleylab, Tyco International Healthcare, Boulder, CO), Ethicon Ultracision Harmonic Scalpel LCS‐5 (Ethicon, Cincinnati, Ohio), or cutting stapler. Argon beam coagulation may complete hemostasis. The cystic dome is resected up to 3–5 mm from the hepatic parenchyma to avoid hemorrhage and bile leakage from the cut edge of the liver parenchyma. The resected cyst is sent for permanent histopathologic evaluation. If the cyst is not well corticalized on the liver capsule and the standard unroofing of the cyst is considered of inadequate size that can predispose to recurrence, then the cyst wall should be excised including a 3‐ to 4‐mm rim of hepatic parenchyma. If the bile is spotted along the cystic edge, a hemostatic clip or a tie suture can be

Recurrence is low if wide cyst wall resection is accomplished. Fenestration can be carried out by electrocautery hook, bipolar scissors, high frequency bipolar electrocoagulation Liga‐ SureTM (Valleylab, Tyco International Healthcare, Boulder, CO), Ethicon Ultracision Harmonic Scalpel LCS-5 (Ethicon, Cincinnati, Ohio), or cutting stapler. Argon beam coagulation may

Any suspected communication with the bile duct should be carefully investigated intraoper‐ atively. Some authors consider the closure of the open bile duct under laparotomy to be a more rational choice [49]. Other authors advocate for laparoscopic closure of the communication

In case of aspiration of a straw‐colored liquid and in the absence of bile staining of the cystic wall, intraoperative cholangiography is not indicated. However, in case of bile contamination of the cyst or compression of the biliary tree, intraoperative cholangiography is required to evaluate the biliary tree [51]. Intraoperatively missed biliary

Sclerosant or alcohol application on the remnant wall of the cyst is recommended to prevent possible further fluid secretion by cyst epithelium and, hence, hepatic cyst recurrence. The great omentum should be packed into larger cavities to decrease dead space and prevent fluid collection. An omental flap transposition is recognized as an important

The drainage of the remnant cavity must meet the declivity principle, especially for those cysts situated in the upper liver segments. The main reason for reconstitution of the cyst after operation is the coverage of the remnant cavity by the diaphragm. To establish a dry residual cavity after fenestration of liver cysts located in the segments VII and VIII, the vacuum effect of the respiratory movements of the diaphragm should be counteracted by an efficient external drainage. The drainage tube should be left in place until complete cessation of the secretion certified by ultrasound evaluation. The use of pigtail catheters for drainage is preferred to the usual drainage tubes for upper sited liver cysts. The realization of

sections, hepatectomy can be performed laparoscopically to clear all the affected segments.

quadrant of the abdomen at the medioclavicular line for insertion of a 5‐mm trocar. An additional port can be placed for

15

an efficient external drainage might be accomplished by transparenchymatous placement of the tube.

placed for exposure, depending on the cyst localization.

result of an unrecognized intracystic hemorrhage.

laparoscopic closure of the communication with the bile duct [50].

affected segments.

with the bile duct [50].

communication could lead to prolonged biliary fistula and even biliary peritonitis.

aid in reducing the risk of cyst recurrence, but it is contraindicated in infected cysts.

applied [46].

exposure, depending on the cyst localization.

268 Recent Advances in Liver Diseases and Surgery

hydatid cyst, pyogenic abscess, and tuberculoma) (Figure 18).

(e.g., hydatid cyst, pyogenic abscess, and tuberculoma) (Figure 18).

Single-incision laparoscopic surgery can reach the effect of "no scar" and can be safely and effectively carried out in these patients. The operation is performed using a dedicated port such SILSTM Port (Covidien, MA, USA), GelPort® Laparoscopic System (Applied Medical, CA, USA), QuadPort+ (Olympus, PA, USA), or X-cone (Karl Storz, Germany). However, three trocars can also be used by their insertion into abdominal cavity through one 2.5-cm incision designed along the ventral midline on the upper edge of the umbilicus. The incision is sufficient to accommodate one 10-mm trocar and two 5-mm trocars [19]. The abdominal drainage tube can be appropriately placed through the same incision at the end of cyst fenestration. If the telescope cannot directly observe the liver cyst, the patient is not suited for single-incision laparoscopic surgery.

**Figure 19.** Patient with PLD and ADPKD operated by laparoscopic approach for hepatic cysts. CT in axial view shows small serous cysts grouped in segments II and III (A) and a large serous cyst in segment IV (B). Laparoscopic interven‐ tion was successfully performed.

Minilaparoscopic-assisted transvaginal approach is also mentioned [52].

#### *5.3.3. Robotic approach*

Robotic surgery is another possible approach, and it has been used in our clinic for nonparasitic hepatic cyst fenestration. The use of the Da Vinci robotic surgical system has certain technical advantages over the standard laparoscopic technique in case of the posterior location of the liver cysts [53]. However, conversion to laparoscopic or open surgery in case of difficulty is laborious and time consuming and could be life-threatening in a dire emergency.

#### *5.3.4. Classic approach*

The communication between cystic cavity and bile duct encountered during operation can be managed in different ways. The suture of the biliary communication is enough, but the means of doing that depend on different surgeons. Cystojejunostomy is mentioned by some authors [54], but it became history in our clinic. Biliary-enteric anastomosis is necessary if there is a concern of postoperative leak or intrahepatic biliary obstruction after suture control [55].

If cystadenoma is suspected, due to its malignant potential, the recommended therapy is laparoscopic or open cyst enucleation or liver resection. But if any suspicion of cystadenocar‐ cinoma exists or especially if malignancy is proved by cytology of aspirated cyst fluid or frozen sections, the surgical decision should be open liver resection with tumor-free margins.

Enucleation is generally feasible due to the existence of a well-defined plane between the cyst wall and the normal liver parenchyma that is generally avascular (Figure 20). For cysts centrally situated, with an increased risk of intraoperative hemorrhage, Pringle maneuver Diagnostic and Therapeutic Challenges in Nonparasitic Liver Cysts http://dx.doi.org/10.5772/61057 271

Minilaparoscopic-assisted transvaginal approach is also mentioned [52].

Robotic surgery is another possible approach, and it has been used in our clinic for nonparasitic hepatic cyst fenestration. The use of the Da Vinci robotic surgical system has certain technical advantages over the standard laparoscopic technique in case of the posterior location of the liver cysts [53]. However, conversion to laparoscopic or open surgery in case of difficulty is

**Figure 19.** Patient with PLD and ADPKD operated by laparoscopic approach for hepatic cysts. CT in axial view shows small serous cysts grouped in segments II and III (A) and a large serous cyst in segment IV (B). Laparoscopic interven‐

The communication between cystic cavity and bile duct encountered during operation can be managed in different ways. The suture of the biliary communication is enough, but the means of doing that depend on different surgeons. Cystojejunostomy is mentioned by some authors [54], but it became history in our clinic. Biliary-enteric anastomosis is necessary if there is a concern of postoperative leak or intrahepatic biliary obstruction after suture control [55].

If cystadenoma is suspected, due to its malignant potential, the recommended therapy is laparoscopic or open cyst enucleation or liver resection. But if any suspicion of cystadenocar‐ cinoma exists or especially if malignancy is proved by cytology of aspirated cyst fluid or frozen sections, the surgical decision should be open liver resection with tumor-free margins.

Enucleation is generally feasible due to the existence of a well-defined plane between the cyst wall and the normal liver parenchyma that is generally avascular (Figure 20). For cysts centrally situated, with an increased risk of intraoperative hemorrhage, Pringle maneuver

laborious and time consuming and could be life-threatening in a dire emergency.

*5.3.3. Robotic approach*

tion was successfully performed.

270 Recent Advances in Liver Diseases and Surgery

*5.3.4. Classic approach*

brings clear benefits. If there is a direct contact of the cyst wall with the portal pedicle, hepatic vein, or inferior vena cava, that portion of the wall can be abandoned if no malignancy is identified on frozen sections (Figure 21). However, there still remains the risk of abandoning malignant areas or malignant degeneration of the remnant cyst wall over time. For this reason,

enucleation (D).

it is recommended to destroy the epithelium lining of the remnant cyst wall by electrofulgu‐ ration or argon beam coagulation. Enucleation is generally feasible due to the existence of a well‐defined plane between the cyst wall and the normal liver parenchyma that is generally avascular (Figure 20). For cysts centrally situated, with an increased risk of intraoperative hemorrhage, Pringle maneuver brings clear benefits. If there is a direct contact of the cyst wall with the portal pedicle,

In case of malignant cytology and/or histopathology, the only potentially curative treat‐ ment is complete removal of the hepatic cyst, usually by a major liver resection with 1 cm free margin [15]. hepatic vein, or inferior vena cava, that portion of the wall can be abandoned if no malignancy is identified on frozen sections (Figure 21). However, there still remains the risk of abandoning malignant areas or malignant degeneration of the remnant cyst wall over time. For this reason, it is recommended to destroy the epithelium lining of the remnant cyst

The elective treatment for the infected hepatic cysts is antibiotherapy combined with percu‐ taneous drainage. It is thought that infection itself kills the inner epithelium, so the regular use of sclerosant injection into the cyst is not generally adopted by some authors. A shortening of time for sclerosant instillation is suggested by others [56]. In case of failure of the mentioned treatment, there is a need for surgery. wall by electrofulguration or argon beam coagulation. In case of malignant cytology and/or histopathology, the only potentially curative treatment is complete removal of the hepatic cyst, usually by a major liver resection with 1‐cm free margin [15]. The elective treatment for the infected hepatic cysts is antibiotherapy combined with percutaneous drainage. It is thought that infection itself kills the inner epithelium, so the regular use of sclerosant injection into the cyst is not

When intracystic or intraparenchymatous hemorrhage is certain, the active bleeding with negative hemodynamic impact imposes emergency operation. Otherwise, the percutaneous treatment can be the first option. generally adopted by authors. A shortening of time for sclerosant instillation is suggested by others [56]. In case of failure of the mentioned treatment, there is a need for surgery. When intracystic or intraparenchymatous hemorrhage is certain, the active bleeding with negative hemodynamic impact

In dealing with cyst rupture, there is no standard therapy. In the presence of peritoneal irritation or internal hemorrhage signs, emergency operation is the rule. In the absence of such signs, the management may vary from conservatory treatment with close observation to surgical intervention [57]. impose emergency operation. Otherwise, the percutaneous treatment may be the first option. In dealing with cyst rupture, there is no standard therapy. In the presence of peritoneal irritation or internal hemorrhage signs, emergency operation is the rule. In the absence of such signs, the management may vary from conservatory treatment with close observation to surgical intervention [57].

portal pedicle (A) and gallbladder (B) on CT, coronal views. Intraoperative aspect of a thick cyst wall (C) and of hepatic parenchyma after cyst **Figure 20.** Recurrent cyst in segments IV–V with close proximity to the right portal pedicle (A) and gallbladder (B) on CT, coronal views. Intraoperative aspect of a thick cyst wall (C) and of hepatic parenchyma after cyst enucleation (D).

**Figure 20.** Recurrent cyst in segments IV–V with close proximity to the right

18

continuous suture.

**Figure 21.** Intraoperative feature of the abandoned wall of a huge serous cyst that occupied segments IV and VIII and contained a chocolate‐like fluid. The hemostasis on the hepatic rim was achieved by

**Figure 21.** Intraoperative feature of the abandoned wall of a huge serous cyst that occupied segments IV and VIII and contained a chocolate-like fluid. The hemostasis on the hepatic rim was achieved by continuous suture.

#### *5.3.5. Treatment of PLD*

it is recommended to destroy the epithelium lining of the remnant cyst wall by electrofulgu‐

Enucleation is generally feasible due to the existence of a well‐defined plane between the cyst wall and the normal liver parenchyma that is generally avascular (Figure 20). For cysts centrally situated, with an increased risk of intraoperative hemorrhage, Pringle maneuver brings clear benefits. If there is a direct contact of the cyst wall with the portal pedicle, hepatic vein, or inferior vena cava, that portion of the wall can be abandoned if no malignancy is identified on frozen sections (Figure 21). However, there still remains the risk of abandoning malignant areas or malignant degeneration of the remnant cyst wall over time. For this reason, it is recommended to destroy the epithelium lining of the remnant cyst

In case of malignant cytology and/or histopathology, the only potentially curative treat‐ ment is complete removal of the hepatic cyst, usually by a major liver resection with 1-

The elective treatment for the infected hepatic cysts is antibiotherapy combined with percu‐ taneous drainage. It is thought that infection itself kills the inner epithelium, so the regular use of sclerosant injection into the cyst is not generally adopted by some authors. A shortening of time for sclerosant instillation is suggested by others [56]. In case of failure of the mentioned

In case of malignant cytology and/or histopathology, the only potentially curative treatment is complete removal of the

The elective treatment for the infected hepatic cysts is antibiotherapy combined with percutaneous drainage. It is thought that infection itself kills the inner epithelium, so the regular use of sclerosant injection into the cyst is not generally adopted by authors. A shortening of time for sclerosant instillation is suggested by others [56]. In case of

When intracystic or intraparenchymatous hemorrhage is certain, the active bleeding with negative hemodynamic impact imposes emergency operation. Otherwise, the percutaneous

In dealing with cyst rupture, there is no standard therapy. In the presence of peritoneal irritation or internal hemorrhage signs, emergency operation is the rule. In the absence of such signs, the management may vary from conservatory treatment with close observation to

In dealing with cyst rupture, there is no standard therapy. In the presence of peritoneal irritation or internal hemorrhage signs, emergency operation is the rule. In the absence of such signs, the management may vary from conservatory

> **Figure 20.** Recurrent cyst in segments IV–V with close proximity to the right portal pedicle (A) and gallbladder (B) on CT, coronal views. Intraoperative

**Figure 20.** Recurrent cyst in segments IV–V with close proximity to the right portal pedicle (A) and gallbladder (B) on CT, coronal views. Intraoperative aspect of a thick cyst wall (C) and of hepatic parenchyma after cyst enucleation (D).

aspect of a thick cyst wall (C) and of hepatic parenchyma after cyst

When intracystic or intraparenchymatous hemorrhage is certain, the active bleeding with negative hemodynamic impact

18

continuous suture.

**Figure 21.** Intraoperative feature of the abandoned wall of a huge serous cyst that occupied segments IV and VIII and contained a chocolate‐like fluid. The hemostasis on the hepatic rim was achieved by

ration or argon beam coagulation.

272 Recent Advances in Liver Diseases and Surgery

treatment, there is a need for surgery.

impose emergency operation. Otherwise, the percutaneous treatment may be the first option.

hepatic cyst, usually by a major liver resection with 1‐cm free margin [15].

treatment can be the first option.

surgical intervention [57].

enucleation (D).

treatment with close observation to surgical intervention [57].

failure of the mentioned treatment, there is a need for surgery.

cm free margin [15].

wall by electrofulguration or argon beam coagulation.

The appropriate treatment of PLD is based on Gigot's classification on CT findings. Most current therapies are invasive. However, the conservative management with lanreotide, a long-acting somatostatin analogue, is promising, being associated with a reduction of liver volume in PLD [58, 59]. Sclerotherapy is considered ineffective in the management of PLD. Laparoscopic fenestration is feasible only for PLD type I in Gigot's classification (Figure 22). For type II, open fenestration is indicated. For type III, only liver resection or liver transplan‐ tation is permitted in symptomatic patients. If liver transplantation is anticipated, fenestration or resection should be avoided to decrease postoperative morbidity and mortality.

#### *5.3.6. Liver transplantation for PLD*

Most of the patients with PLD have combined liver and kidney cystic disease. There are questions regarding whether kidney and liver transplantation must be performed and whether these transplants should be performed simultaneously or consecutively. The patients with advanced renal failure (dialysis or predialysis stage) need combined liver and kidney replace‐ ment. However, those patients who have normal renal function do not need a combined transplantation. It was reported that maximum 33% of the patients who first received a liver transplant alone needed a kidney transplant later [60]. In many patients, the renal function improves after orthotopic liver transplantation (OLT), possibly due to the release of compart‐ ment syndrome. Massive hepatomegaly causes an abdominal compartment syndrome that negatively affects renal function which can be reversed after OLT. Other authors argue that a combined liver and kidney transplantation should be performed even in patients with limited

fenestration of the largest cysts situated in hepatic segments IV–V and VII–VIII. (A) Axial view of the upper liver. (B) Axial view of the lower liver. (C) Sagittal view of the liver. D. Coronal view of the liver. **Figure 22.** CT for a female patient with PLD submitted to laparoscopic fenestration of the largest cysts situated in the hepatic segments IV–V and VII–VIII. (A) Axial view of the upper liver. (B) Axial view of the lower liver. (C) Sagittal view of the liver. D. Coronal view of the liver.

**Figure 22.** CT for a female patient with PLD submitted to laparoscopic

renal dysfunction or even normal renal function. This strategy is justified by the progression of cystic renal disease after OLT and the immune advantage in transplanting both organs harvested from the same donor, rather than having to perform a third-party kidney trans‐ plantation later [61]. **5.3.6. Liver transplantation for PLD** Most of the patients with PLD have combined liver and kidney cystic disease. There are questions regarding whether kidney and liver transplantation must be performed and whether these transplants should be performed simultaneously

OLT is a rare procedure for PLD. It represents approximately 1% of all indications [62]. Total hepatectomy followed by OLT offers the chance of definitive treatment. The indication of OLT may be too drastic considering the absence of immediately life-threatening liver failure, the potential hazards associated with OLT procedure and postoperative immunosuppression, and the organ shortage. Indications for OLT in PLD are massive hepatomegaly, compartment syndrome, and clinically advanced malnutrition that produce severe physical and social handicap. Generally, these patients have preserved liver function and normal model for end stage liver disease (MELD) score if they do not have renal involvement [63]. or consecutively. The patients with advanced renal failure (dialysis or predialysis stage) need combined liver and kidney replacement. However, those patients who have normal renal function do not need a combined transplantation. It was reported that maximum 33% of the patients who first received a liver transplant alone needed a kidney transplant later [60]. In many patients, the renal function improves after orthotopic liver transplantation (OLT), possibly due to the release of compartment syndrome. Massive hepatomegaly causes an abdominal compartment syndrome that negatively affects renal function which can be reversed after OLT. Other authors argue that a combined liver and kidney transplantation should be performed even in patients with limited renal dysfunction or even normal renal function. This strategy is justified by the progression of cystic renal disease after OLT and the immune advantage in transplanting both organs harvested from the same donor, rather than having to perform a third‐party kidney transplantation later [61].

Two drawbacks to OLT for patients with PLD are the susceptibility to infection caused by severe malnutrition and overimmunosuppression, and further degradation of renal function caused by immunosuppression. Therefore, the maintenance of immunosuppression in such patients must be lower than usual and the steroids should be discontinued after 3 months. It is also important not to delay OLT in these patients; otherwise, complications such portal vein thrombosis, portal hypertension, Budd–Chiari syndrome, peripheral cholangiocarcinoma, and liver failure may result in poorer tolerance of the patient to procedure, increased graft loss, and increased costs [62]. OLT is a rare procedure for PLD. It represents approximately 1% of all indications [62]. Total hepatectomy followed by OLT offers the chance of definitive treatment. The indication of OLT may be too drastic considering the absence of immediately life‐threatening liver failure, the potential hazards associated with OLT procedure and postoperative immunosuppression, and the organ shortage. Indications for OLT in PLD are massive hepatomegaly, compartment syndrome, and clinically advanced malnutrition that produce severe physical and social handicap. Generally, these patients have preserved liver function and normal model for end stage liver disease (MELD) score if they do not have renal involvement [63]. Two drawbacks to OLT for patients with PLD are the susceptibility to infection caused by severe malnutrition and overimmunosuppression and further degradation of renal function caused by immunosuppression. Therefore, the

maintenance of immunosuppression in such patients must be lower than usual and the steroids should be discontinued after 3 months. It is also important not to delay OLT in these patients; otherwise, complications such portal vein thrombosis, portal hypertension, Budd–Chiari syndrome, peripheral cholangiocarcinoma, and liver failure may result in

The first report of OLT for PLD was by Kwork and Lewin in 1988, but the patient died intraoperatively of intractable

OLT in PLD is technically challenging because of the massive organomegaly (Figure 23). If OLT is anticipated on a patient with PLD, then any other surgical interventions should be withheld in order to avoid massive intraoperative

poorer tolerance of the patient to procedure, increased graft loss, and increased costs [62].

bleeding. In 1990, Starzl successfully performed OLT on 4 patients with PLD.

bleeding and thus transfusion requirements.

19

The first report of OLT for PLD was by Kwork and Lewin in 1988, but the patient died intraoperatively of intractable bleeding. In 1990, Starzl successfully performed OLT on 4 patients with PLD.

OLT in PLD is technically challenging because of the massive organomegaly (Figure 23). If OLT is anticipated on a patient with PLD, then any other surgical interventions should be withheld in order to avoid massive intraoperative bleeding and thus transfusion requirements.

The anatomic position of the vessels is not respected in patients with PLD. In total hepatectomy, dissection starts in the liver hilum because it is the only structure that lies at a near-normal anatomic position. Hepatic artery, portal vein, and principal biliary duct are identified. No attempt is made to control the suprahepatic vena cava at the beginning of dissection. Early attempts at controlling it may result in its laceration, fatal bleeding, and pulmonary air embolism. Patients with PLD have little portal hypertension and spontaneous portocaval shunts and thus poor tolerance of portal clamping [62]. The surgeons must be prepared for portal bypass if hypotension develops when portal vein is clamped. Other surgeons system‐ atically use portal bypass. By dissecting portal vein for cannulation, a broad exposure of the intrahepatic vena cava is obtained. The decision of inferior vena cava resection is made based on the intraoperative conditions determined during the dissection phase [64]. Some surgeons choose to continue the dissection of the liver en bloc with the native vena cava and eventually safely control the suprahepatic vena cava. The en bloc resection of the liver with the native vena cava is justified by the fact that usually the cava vein is embedded within an extremely voluminous caudate lobe [62]. There are other surgeons who performed dissection of liver parenchyma from IVC. Cysts were aspirated to facilitate final access to the suprahepatic inferior vena cava. However, one should be prepared for vein control through transdiaphrag‐ matic or transsternal approach.

Transcatheter embolization has been proposed to decrease arterial supply of the cysts and thus reduce the liver size and may be beneficial prior to liver transplantation [65].

renal dysfunction or even normal renal function. This strategy is justified by the progression of cystic renal disease after OLT and the immune advantage in transplanting both organs harvested from the same donor, rather than having to perform a third-party kidney trans‐

Most of the patients with PLD have combined liver and kidney cystic disease. There are questions regarding whether kidney and liver transplantation must be performed and whether these transplants should be performed simultaneously or consecutively. The patients with advanced renal failure (dialysis or predialysis stage) need combined liver and kidney replacement. However, those patients who have normal renal function do not need a combined transplantation. It was reported that maximum 33% of the patients who first received a liver transplant alone needed a kidney transplant later [60]. In many patients, the renal function improves after orthotopic liver transplantation (OLT), possibly due to the release of compartment syndrome. Massive hepatomegaly causes an abdominal compartment syndrome that negatively affects renal function which can be reversed after OLT. Other authors argue that a combined liver and kidney transplantation should be performed even in patients with limited renal dysfunction or even normal renal function. This strategy is justified by the progression of cystic renal disease after OLT and the immune advantage in transplanting both organs harvested from the same donor, rather than having to perform a third‐party kidney transplantation later [61]. OLT is a rare procedure for PLD. It represents approximately 1% of all indications [62]. Total hepatectomy followed by OLT offers the chance of definitive treatment. The indication of OLT may be too drastic considering the absence of immediately life‐threatening liver failure, the potential hazards associated with OLT procedure and postoperative immunosuppression, and the organ shortage. Indications for OLT in PLD are massive hepatomegaly, compartment syndrome, and clinically advanced malnutrition that produce severe physical and social handicap. Generally, these patients have preserved liver function and normal model for end stage liver disease (MELD) score if they do not have

**Figure 22.** CT for a female patient with PLD submitted to laparoscopic fenestration of the largest cysts situated in hepatic segments IV–V and VII–VIII. (A) Axial view of the upper liver. (B) Axial view of the lower liver. (C) Sagittal

**Figure 22.** CT for a female patient with PLD submitted to laparoscopic fenestration of the largest cysts situated in the hepatic segments IV–V and VII–VIII. (A) Axial view of the upper liver. (B) Axial view of the lower liver. (C) Sagittal

OLT is a rare procedure for PLD. It represents approximately 1% of all indications [62]. Total hepatectomy followed by OLT offers the chance of definitive treatment. The indication of OLT may be too drastic considering the absence of immediately life-threatening liver failure, the potential hazards associated with OLT procedure and postoperative immunosuppression, and the organ shortage. Indications for OLT in PLD are massive hepatomegaly, compartment syndrome, and clinically advanced malnutrition that produce severe physical and social handicap. Generally, these patients have preserved liver function and normal model for end

Two drawbacks to OLT for patients with PLD are the susceptibility to infection caused by severe malnutrition and overimmunosuppression, and further degradation of renal function caused by immunosuppression. Therefore, the maintenance of immunosuppression in such patients must be lower than usual and the steroids should be discontinued after 3 months. It is also important not to delay OLT in these patients; otherwise, complications such portal vein thrombosis, portal hypertension, Budd–Chiari syndrome, peripheral cholangiocarcinoma, and liver failure may result in poorer tolerance of the patient to procedure, increased graft loss,

Two drawbacks to OLT for patients with PLD are the susceptibility to infection caused by severe malnutrition and overimmunosuppression and further degradation of renal function caused by immunosuppression. Therefore, the maintenance of immunosuppression in such patients must be lower than usual and the steroids should be discontinued after 3 months. It is also important not to delay OLT in these patients; otherwise, complications such portal vein thrombosis, portal hypertension, Budd–Chiari syndrome, peripheral cholangiocarcinoma, and liver failure may result in

The first report of OLT for PLD was by Kwork and Lewin in 1988, but the patient died intraoperatively of intractable

OLT in PLD is technically challenging because of the massive organomegaly (Figure 23). If OLT is anticipated on a patient with PLD, then any other surgical interventions should be withheld in order to avoid massive intraoperative

19

stage liver disease (MELD) score if they do not have renal involvement [63].

poorer tolerance of the patient to procedure, increased graft loss, and increased costs [62].

bleeding. In 1990, Starzl successfully performed OLT on 4 patients with PLD.

view of the liver. D. Coronal view of the liver.

plantation later [61].

view of the liver. D. Coronal view of the liver.

274 Recent Advances in Liver Diseases and Surgery

**5.3.6. Liver transplantation for PLD**

and increased costs [62].

bleeding and thus transfusion requirements.

renal involvement [63].

**Figure 23.** Female patient with PLD and ADPKD with indication for liver transplantation. (A) Body topography with huge abdomen volume. (B) CT, axial view: extensive hepatomegaly with multiple diffuse hepatic cysts entirely occu‐ pying the liver parenchyma. (C) CT, axial view: extensive hepatomegaly with the inferior border of the liver reaching the pelvis; multiple bilateral kidney cysts with both kidney displaced in the pelvis.
