**9. Management**

The treatment time of antenatal diagnosed of CCs has been still a matter of debate. Some reports say that they can be operated within 2–6 weeks, even if they are asymptomatic, due to a potential complication risk of cysts, whereas, the others suggest that they can be followed-up for a time of period with US and regular monitoring of liver functions [108, 109].

Surgical treatment of CCs should be performed electively except complicated ones such as cyst perforation. Children, who have acute problems related to liver and pancreas, should be initially received appropriate medical treatment to remove inflammation and associated obstruction at the pancreaticobiliary system before surgery [110].

In the first half of this century, treatment methods such as cyst aspiration, marsupialization, and external drainage (cholecystostomy, tube drainage) had been used more extensively in the surgical treatment of CCs. And as expected, high mortality and morbidity rates had been detected in patient's follow-up period. Surgical methods such as partial cyst excision and cystoduodenostomy were defined between 1920 and 1930 [111, 112]. Especially, cystoduodenostomy has been the preferred method by many surgeons until the early 1970s [113, 114] when the long-term morbidity was detected as higher (30–50%) [7]. Therefore, in those years, Roux-en-Y cystojejunostomy had identified with the idea of preventing the reflux of the duodenal contents into the bile ducts [7, 30, 115]. Indeed, the cholangitis had been significantly reduced with this method, but not completely eliminated [28]. After soon, it had been demonstrated that anastomosis with a large stoma, as possible as (at least 4 cm) is more important for protecting reflux-related cholangitis attacks than which intestinal segment it is performed (duodenum or jejunum) [116]. The recognition of the cancer development from the left cystic wall and Babbitt's APBDU theory made the idea of cyst excision popular soon after. It had been reported that carcinoma develops after the internal drainage procedures at a frequency of 2.5–17.5% several years later as a consequence of chronic inflammation of the cyst wall. Therefore, cyst excision had gain popularity in a very short time [116].

Contemporarily, the definitive treatment for CCs are total excision that has become preferred management strategy over the internal drainage procedures (choledochocystoduodenostomy or choledochocystojejunostomy), which have an only historical value today despite they had been used as a treatment method in the past although caused high morbidity (probably because of not relieving biliary stasis sufficiently) [117, 118]. Furthermore, only complete resection can fully decrease the risk of malignant degeneration: a critical point in the pediatric population with a large number of expectant life years. The general aim is to remove the cyst completely and restore biliary enteric drainage either into the duodenum by hepaticoduodenostomy (HD) or jejunum by Roux-en-Y hepaticojejunostomy (RYHJ), although specific approaches for types vary minimally. Surgical intervention should be elective and patients should be medically optimized prior to operative intervention. İf patient has a cholangitis or pancreatitis attack preoperatively, the infection should be adequately treated with broad-spectrum intravenous antibiotics or biliary decompression if needed [118].

Surgery for CC disease can be performed open or laparoscopically based on patient characteristics and surgeon preference.

HD and RYHJ are the two most commonly utilized techniques of reconstruction [119], although other replacement conduits such as jejunal interposition HD, valved jejunal interposition HD, nonrefluxing biliary appendicoduodenostomy, hepaticoenterostomy, and wide hilar hepaticojejunostomy have been reported [120–125]. HD has been favored by some groups [126, 127] but most series suggests significantly more bile reflux compared with RYHJ [121], which is currently the most commonly utilized reconstruction.

#### **9.1 Open surgery**

In all cases, cholangiography should be performed initially to obtain detailed anatomical information about the intra- and extrahepatic bile ducts, irrespective of

**97**

**Figure 5.**

*Pediatric Choledochal Cysts: Unknowns are Decreasing DOI: http://dx.doi.org/10.5772/intechopen.84301*

seventh postoperative day [124].

*Intraoperative pictures and drawn cartoon showing of the procedure.*

preoperative examinations. Dissection of extrahepatic bile ducts starts from the gallbladder. The terminal end of the cyst opening to the duodenum should be isolated, clamped, cut, and transfixed, firstly. Some surgeons suggest that dissection should be continued until the appearance of pancreatic ducts, while the others not suggest. Additionally, some surgeons taking into account that dissection toward the lower end of the cyst may cause inevitable unplanned pancreatic duct injury that pancreaticoduodenectomy requirement should be in your mind, although very rarely [75]. After the distal portion of the cyst is ligated and cut, the posterior wall is dissected from the surface of the portal vein. In cases of marked inflammation, the cyst may be excised by leaving the posterior wall on the portal vein. The dissection should go on till the hepatic hilus. The best strategy to obtain a wide anastomosis stoma is to make a hepatic dissection more proximally until the left hepatic duct is seen. Although all parts of CCs need to be removed, sometimes residual proximal cyst walls can be left to facilitate biliary anastomosis [75]. Dilated bile ducts should be irrigated with heparinized saline to clear the gallstones before anastomosis. After the cyst is excised, one of the hepaticoenterostomy methods, such as hepaticojejunostomy, HD, jejunal interposition HD, valved jejunal interposition HD, nonrefluxing biliary appendicoduodenostomy, hepaticoenterostomy, and wide hilar hepaticojejunostomy [120–125] is performed for biliary reconstruction. In RYHJ, 40-cm jejunal loop replaces to the hepatic hilus. In RYHJ surgery, to avoid the elongation of a blind pouch as the child grows, an end-to-end anastomosis of the jejunum to the CBD is recommended if technically possible [128]. If an end-to-side anastomosis is required (in some cases, the bile duct is too small), it should be as close as possible to the closed end of the jejunal limb. Additionally, although it is not possible to predetermine the length of the Roux limb, it should be appropriate to the child's overall bowel length considering future growth. In HD, anastomosis is performed between the duodenum second part and the bile duct. The duodenum was mobilized to a limit. The duodenum is anchored to the liver at porta to avoid tension on the anastomosis (**Figure 5**). The intraabdominal drain, kept in Morison's pouch, may be removed on the

## *Pediatric Choledochal Cysts: Unknowns are Decreasing DOI: http://dx.doi.org/10.5772/intechopen.84301*

*Pediatric Surgery, Flowcharts and Clinical Algorithms*

excision had gain popularity in a very short time [116].

decompression if needed [118].

patient characteristics and surgeon preference.

most commonly utilized reconstruction.

surgery [110].

Surgical treatment of CCs should be performed electively except complicated ones such as cyst perforation. Children, who have acute problems related to liver and pancreas, should be initially received appropriate medical treatment to remove inflammation and associated obstruction at the pancreaticobiliary system before

In the first half of this century, treatment methods such as cyst aspiration, marsupialization, and external drainage (cholecystostomy, tube drainage) had been used more extensively in the surgical treatment of CCs. And as expected, high mortality and morbidity rates had been detected in patient's follow-up period. Surgical methods such as partial cyst excision and cystoduodenostomy were defined between 1920 and 1930 [111, 112]. Especially, cystoduodenostomy has been the preferred method by many surgeons until the early 1970s [113, 114] when the long-term morbidity was detected as higher (30–50%) [7]. Therefore, in those years, Roux-en-Y cystojejunostomy had identified with the idea of preventing the reflux of the duodenal contents into the bile ducts [7, 30, 115]. Indeed, the cholangitis had been significantly reduced with this method, but not completely eliminated [28]. After soon, it had been demonstrated that anastomosis with a large stoma, as possible as (at least 4 cm) is more important for protecting reflux-related cholangitis attacks than which intestinal segment it is performed (duodenum or jejunum) [116]. The recognition of the cancer development from the left cystic wall and Babbitt's APBDU theory made the idea of cyst excision popular soon after. It had been reported that carcinoma develops after the internal drainage procedures at a frequency of 2.5–17.5% several years later as a consequence of chronic inflammation of the cyst wall. Therefore, cyst

Contemporarily, the definitive treatment for CCs are total excision that has become preferred management strategy over the internal drainage procedures (choledochocystoduodenostomy or choledochocystojejunostomy), which have an only historical value today despite they had been used as a treatment method in the past although caused high morbidity (probably because of not relieving biliary stasis sufficiently) [117, 118]. Furthermore, only complete resection can fully decrease the risk of malignant degeneration: a critical point in the pediatric population with a large number of expectant life years. The general aim is to remove the cyst completely and restore biliary enteric drainage either into the duodenum by hepaticoduodenostomy (HD) or jejunum by Roux-en-Y hepaticojejunostomy (RYHJ), although specific approaches for types vary minimally. Surgical intervention should be elective and patients should be medically optimized prior to operative intervention. İf patient has a cholangitis or pancreatitis attack preoperatively, the infection should be adequately treated with broad-spectrum intravenous antibiotics or biliary

Surgery for CC disease can be performed open or laparoscopically based on

HD and RYHJ are the two most commonly utilized techniques of reconstruction [119], although other replacement conduits such as jejunal interposition HD, valved jejunal interposition HD, nonrefluxing biliary appendicoduodenostomy, hepaticoenterostomy, and wide hilar hepaticojejunostomy have been reported [120–125]. HD has been favored by some groups [126, 127] but most series suggests significantly more bile reflux compared with RYHJ [121], which is currently the

In all cases, cholangiography should be performed initially to obtain detailed anatomical information about the intra- and extrahepatic bile ducts, irrespective of

**96**

**9.1 Open surgery**

preoperative examinations. Dissection of extrahepatic bile ducts starts from the gallbladder. The terminal end of the cyst opening to the duodenum should be isolated, clamped, cut, and transfixed, firstly. Some surgeons suggest that dissection should be continued until the appearance of pancreatic ducts, while the others not suggest. Additionally, some surgeons taking into account that dissection toward the lower end of the cyst may cause inevitable unplanned pancreatic duct injury that pancreaticoduodenectomy requirement should be in your mind, although very rarely [75]. After the distal portion of the cyst is ligated and cut, the posterior wall is dissected from the surface of the portal vein. In cases of marked inflammation, the cyst may be excised by leaving the posterior wall on the portal vein. The dissection should go on till the hepatic hilus. The best strategy to obtain a wide anastomosis stoma is to make a hepatic dissection more proximally until the left hepatic duct is seen. Although all parts of CCs need to be removed, sometimes residual proximal cyst walls can be left to facilitate biliary anastomosis [75]. Dilated bile ducts should be irrigated with heparinized saline to clear the gallstones before anastomosis. After the cyst is excised, one of the hepaticoenterostomy methods, such as hepaticojejunostomy, HD, jejunal interposition HD, valved jejunal interposition HD, nonrefluxing biliary appendicoduodenostomy, hepaticoenterostomy, and wide hilar hepaticojejunostomy [120–125] is performed for biliary reconstruction. In RYHJ, 40-cm jejunal loop replaces to the hepatic hilus. In RYHJ surgery, to avoid the elongation of a blind pouch as the child grows, an end-to-end anastomosis of the jejunum to the CBD is recommended if technically possible [128]. If an end-to-side anastomosis is required (in some cases, the bile duct is too small), it should be as close as possible to the closed end of the jejunal limb. Additionally, although it is not possible to predetermine the length of the Roux limb, it should be appropriate to the child's overall bowel length considering future growth. In HD, anastomosis is performed between the duodenum second part and the bile duct. The duodenum was mobilized to a limit. The duodenum is anchored to the liver at porta to avoid tension on the anastomosis (**Figure 5**).

The intraabdominal drain, kept in Morison's pouch, may be removed on the seventh postoperative day [124].

**Figure 5.** *Intraoperative pictures and drawn cartoon showing of the procedure.*

### **9.2 Laparoscopic surgery**

Laparoscopic treatment of choledochal cysts was first described in 1995 [129] and demonstrated that it could be performed in children as young as 3 months [130] and as small as 6 kg [131]. As with most surgical diseases, longer operating time and shorter hospital stay [132] were comparable with open surgical approaches, and in the absence of cholangitis or pancreatitis, it becomes more suitable treatment [132]. While four or five ports are typically used in the traditional laparoscopic approach [130, 133], the use of single-port laparoscopy [134] and robotic surgical system [135] has also been reported. In a prospective randomized study of 121 children undergoing laparoscopic cyst excision with RYHJ, routine postoperative drainage has been shown to be unnecessary [136].

### **9.3 The optimal technique for biliary reconstruction**

The most commonly performed operations for biliary reconstruction after complete surgical resection of CCs are RYHJ or HD [124]. There is a debate regarding the optimal technique for biliary reconstruction [124]. RYHJ is considered as an ideal technique for the repair of CC, but HD has gained wide acceptance and favored by many surgeons open as well as laparoscopically because of its advantages over hepaticojejunostomy. HD is more physiologic, but theoretically, the closeness of hepaticoenterostomy to stomach makes HD to have greater chance of cholangitis and bile gastritis, but in a meta-analyzed study [137], it has been shown that while the incidence of bile gastritis after HD is even higher when examined endoscopically, interestingly, there is no difference of cholangitis between HD and RYHJ. Additionally, HD is simpler to perform and associated with fewer complications such as adhesive bowel obstruction, anastomotic leakage, and peptic ulcer as compared to RYHJ [138]. HD requires less operative time, allows faster recovery of bowel function, and produces fewer complications requiring reoperation [139]. If there is an anastomotic stricture following HD, it can be easily managed by endoscopy as against hepaticojejunostomy [124]. But, when the diameter of the common hepatic duct more than 10 mm that lets duodenal contents more likely to reflux easily into the intrahepatic bile ducts through the HD anastomosis or when the intrahepatic biliary dilatation is present that lets refluxed duodenal contents remain longer in the intrahepatic bile ducts, HD is not recommended because of higher risk of cholangitis or anastomotic stricture formation [124]. Some studies have demonstrated high incidence of secondary bile reflux proven by endoscopy after HD [121]. Recently, a patient with hilary bile duct carcinoma, who was performed HD for the biliary reconstruction at the age of 13 months, has been reported in the 19 years follow-up after the primary cyst excision. Reflux of duodenal contents (including activated pancreatic enzymes) into the intrahepatic bile ducts through the HD anastomosis is thought to be hazardous to the bile duct mucosa in this patient [124]. Adhesive bowel obstruction is seen with a higher incidence in RYHJ that comprises a Roux-en-Y jejuna limb and two anastomoses, compared with HD. Cholangitis, peptic ulcer, fat malabsorption, diarrhea, and malnutrition are the other complications [126]. A significant incidence of long-term complications requiring reoperation such as anastomosis stenosis has been observed with the follow-up studies of patients who underwent hepaticojejunostomy after cyst excision [138, 140], and a wide hilar hepaticojejunostomy extending into the left hepatic duct is advocated for the way to prevent it [125].

#### **9.4 Treatment specifically for types**

Treatment of type I CC includes excision of the extrahepatic biliary tract, cholecystectomy, and reconstruction of the biliary system. If the duct is dilated at the

**99**

unresectable intrahepatic cyst [18].

*Pediatric Choledochal Cysts: Unknowns are Decreasing DOI: http://dx.doi.org/10.5772/intechopen.84301*

should be considered [18].

alization of the cavity [18].

distal margin, the mucosa may be left behind to prevent damage to the pancreaticobiliary system and can be striped. Infrequently, because of recurrent episodes of the cholangitis, the cyst wall may densely adherent to the portal vein, precluding safe resection [141]. In such cases, resection of the anterior wall with careful fulguration of the mucosa of the posterior wall can be performed [141]. Hepatic bifurcation is carefully evaluated for stricture and inflammation before performing anastomosis during proximal transection. If one of them is seen, more proximal transection

For type II CCs, mostly, diverticulectomy or simple cyst excision is enough for the treatment. Primary or over a T-tube closure can be performed, and reconstruc-

Type IV CC is approached differently based on the presence or absence and location of intrahepatic disease [15]. Type IVb cysts are treated in the same fashion as type I. Management for IVa disease differs due to the presence of intra- as well as extrahepatic involvement, as well as the presence of functional liver disease. Of foremost importance is the characterization of actual type IVa as opposed to type I with upstream ductal dilatation due to stasis and functional obstruction [50]. If the dilatation is anatomic and isolated (limited; i.e., left hemiliver), partial hepatectomy with reconstruction to the remaining hepatic ducts may be warranted due to the ongoing risk of malignant transformation in the intrahepatic biliary system [85]. However, not all patients are appropriate candidates for partial hepatectomy [144]. Those patients with obvious dilatations and stenosis of intrahepatic ducts, intrahepatic duct stones, or parenchymal atrophy may benefit from hepatectomy [144]. If hepatectomy is planned concomitantly with extrahepatic duct excision, the distribution should allow removal of all disease (of the vast majority of the severe disease) with adequate future liver remnant [15]. If the pattern is more diffuse or imaging is inconclusive, treatment in a type I paradigm with close postoperative surveillance to follow intrahepatic ducts has been utilized [145]. This approach is justified by studies demonstrating that patients who progress to malignancy most commonly develop extrahepatic cholangiocarcinoma or gallbladder cancer (approximately 85% of malignancy), whereas intrahepatic cholangiocarcinoma rarely occurs [144]. It is reported that the intrahepatic component has actually resolved in 3–6 months with adequate drainage [146]. To differentiate type IVa from type I, while in adults, preoperative percutaneous biliary drainage to decompress the intrahepatic biliary ductal system has been advocated [147], in children, this practice has not been reported probability due to the difficulty in maintaining the external tube. Although long-term results are not known, intrahepatic cystojejunostomy, in addition to hepaticojejunostomy, has been described as a way of preventing liver resection in type IVa cysts [148]. Complete extrahepatic excision with hepaticoenterostomy and drainage of the remaining cyst externally or internally should adequately ameliorate biliary stasis in the presence of bilobar

Management of patients with Caroli's disease can be particularly difficult given the location of the cysts and frequent necessity for surgery (considerable potential for cholangitis, liver complications, and biliary cirrhosis; moderate potential for neoplasia (7%)) [15]. In Caroli disease, intrahepatic cysts can be seen as limited

tion is occasionally required if there is significant luminal narrowing [18]. One of the methods such as endoscopic sphincterotomy, sphincteroplasty, sphincteroplasty with cyst excision, or pancreaticoduodenectomy may be used to manage pediatric patients with type III CCs (choledochoceles) [10, 25]. Various reports denote adequate symptom control with this approach [142, 143]; however, long-term follow-up is lacking. Cysts not amenable to endoscopic intervention may benefit from lateral duodenotomy with sphincteroplasty and unroofing or marsupi*Pediatric Choledochal Cysts: Unknowns are Decreasing DOI: http://dx.doi.org/10.5772/intechopen.84301*

*Pediatric Surgery, Flowcharts and Clinical Algorithms*

has been shown to be unnecessary [136].

**9.3 The optimal technique for biliary reconstruction**

Laparoscopic treatment of choledochal cysts was first described in 1995 [129] and demonstrated that it could be performed in children as young as 3 months [130] and as small as 6 kg [131]. As with most surgical diseases, longer operating time and shorter hospital stay [132] were comparable with open surgical approaches, and in the absence of cholangitis or pancreatitis, it becomes more suitable treatment [132]. While four or five ports are typically used in the traditional laparoscopic approach [130, 133], the use of single-port laparoscopy [134] and robotic surgical system [135] has also been reported. In a prospective randomized study of 121 children undergoing laparoscopic cyst excision with RYHJ, routine postoperative drainage

The most commonly performed operations for biliary reconstruction after complete surgical resection of CCs are RYHJ or HD [124]. There is a debate regarding the optimal technique for biliary reconstruction [124]. RYHJ is considered as an ideal technique for the repair of CC, but HD has gained wide acceptance and favored by many surgeons open as well as laparoscopically because of its advantages over hepaticojejunostomy. HD is more physiologic, but theoretically, the closeness of hepaticoenterostomy to stomach makes HD to have greater chance of cholangitis and bile gastritis, but in a meta-analyzed study [137], it has been shown that while the incidence of bile gastritis after HD is even higher when examined endoscopically, interestingly, there is no difference of cholangitis between HD and RYHJ. Additionally, HD is simpler to perform and associated with fewer complications such as adhesive bowel obstruction, anastomotic leakage, and peptic ulcer as compared to RYHJ [138]. HD requires less operative time, allows faster recovery of bowel function, and produces fewer complications requiring reoperation [139]. If there is an anastomotic stricture following HD, it can be easily managed by endoscopy as against hepaticojejunostomy [124]. But, when the diameter of the common hepatic duct more than 10 mm that lets duodenal contents more likely to reflux easily into the intrahepatic bile ducts through the HD anastomosis or when the intrahepatic biliary dilatation is present that lets refluxed duodenal contents remain longer in the intrahepatic bile ducts, HD is not recommended because of higher risk of cholangitis or anastomotic stricture formation [124]. Some studies have demonstrated high incidence of secondary bile reflux proven by endoscopy after HD [121]. Recently, a patient with hilary bile duct carcinoma, who was performed HD for the biliary reconstruction at the age of 13 months, has been reported in the 19 years follow-up after the primary cyst excision. Reflux of duodenal contents (including activated pancreatic enzymes) into the intrahepatic bile ducts through the HD anastomosis is thought to be hazardous to the bile duct mucosa in this patient [124]. Adhesive bowel obstruction is seen with a higher incidence in RYHJ that comprises a Roux-en-Y jejuna limb and two anastomoses, compared with HD. Cholangitis, peptic ulcer, fat malabsorption, diarrhea, and malnutrition are the other complications [126]. A significant incidence of long-term complications requiring reoperation such as anastomosis stenosis has been observed with the follow-up studies of patients who underwent hepaticojejunostomy after cyst excision [138, 140], and a wide hilar hepaticojejunostomy extending into the left hepatic duct is advocated for the way to prevent it [125].

Treatment of type I CC includes excision of the extrahepatic biliary tract, cholecystectomy, and reconstruction of the biliary system. If the duct is dilated at the

**9.2 Laparoscopic surgery**

**98**

**9.4 Treatment specifically for types**

distal margin, the mucosa may be left behind to prevent damage to the pancreaticobiliary system and can be striped. Infrequently, because of recurrent episodes of the cholangitis, the cyst wall may densely adherent to the portal vein, precluding safe resection [141]. In such cases, resection of the anterior wall with careful fulguration of the mucosa of the posterior wall can be performed [141]. Hepatic bifurcation is carefully evaluated for stricture and inflammation before performing anastomosis during proximal transection. If one of them is seen, more proximal transection should be considered [18].

For type II CCs, mostly, diverticulectomy or simple cyst excision is enough for the treatment. Primary or over a T-tube closure can be performed, and reconstruction is occasionally required if there is significant luminal narrowing [18].

One of the methods such as endoscopic sphincterotomy, sphincteroplasty, sphincteroplasty with cyst excision, or pancreaticoduodenectomy may be used to manage pediatric patients with type III CCs (choledochoceles) [10, 25]. Various reports denote adequate symptom control with this approach [142, 143]; however, long-term follow-up is lacking. Cysts not amenable to endoscopic intervention may benefit from lateral duodenotomy with sphincteroplasty and unroofing or marsupialization of the cavity [18].

Type IV CC is approached differently based on the presence or absence and location of intrahepatic disease [15]. Type IVb cysts are treated in the same fashion as type I. Management for IVa disease differs due to the presence of intra- as well as extrahepatic involvement, as well as the presence of functional liver disease. Of foremost importance is the characterization of actual type IVa as opposed to type I with upstream ductal dilatation due to stasis and functional obstruction [50]. If the dilatation is anatomic and isolated (limited; i.e., left hemiliver), partial hepatectomy with reconstruction to the remaining hepatic ducts may be warranted due to the ongoing risk of malignant transformation in the intrahepatic biliary system [85]. However, not all patients are appropriate candidates for partial hepatectomy [144]. Those patients with obvious dilatations and stenosis of intrahepatic ducts, intrahepatic duct stones, or parenchymal atrophy may benefit from hepatectomy [144]. If hepatectomy is planned concomitantly with extrahepatic duct excision, the distribution should allow removal of all disease (of the vast majority of the severe disease) with adequate future liver remnant [15]. If the pattern is more diffuse or imaging is inconclusive, treatment in a type I paradigm with close postoperative surveillance to follow intrahepatic ducts has been utilized [145]. This approach is justified by studies demonstrating that patients who progress to malignancy most commonly develop extrahepatic cholangiocarcinoma or gallbladder cancer (approximately 85% of malignancy), whereas intrahepatic cholangiocarcinoma rarely occurs [144]. It is reported that the intrahepatic component has actually resolved in 3–6 months with adequate drainage [146]. To differentiate type IVa from type I, while in adults, preoperative percutaneous biliary drainage to decompress the intrahepatic biliary ductal system has been advocated [147], in children, this practice has not been reported probability due to the difficulty in maintaining the external tube. Although long-term results are not known, intrahepatic cystojejunostomy, in addition to hepaticojejunostomy, has been described as a way of preventing liver resection in type IVa cysts [148]. Complete extrahepatic excision with hepaticoenterostomy and drainage of the remaining cyst externally or internally should adequately ameliorate biliary stasis in the presence of bilobar unresectable intrahepatic cyst [18].

Management of patients with Caroli's disease can be particularly difficult given the location of the cysts and frequent necessity for surgery (considerable potential for cholangitis, liver complications, and biliary cirrhosis; moderate potential for neoplasia (7%)) [15]. In Caroli disease, intrahepatic cysts can be seen as limited

disease restricted to a single segment/lobe or diffuse disease involving the entire intrahepatic biliary tree. If the patient has not developed cirrhosis and portal hypertension, the unilobar cystic disease should be treated with anatomic hepatectomy and biliary enteric bypass. However, bilobar disease should be treated with symptom-directed nonoperative treatment methods as including litholytic agents such as ursodiol, antibiotics, and percutaneous drainage if possible. Close follow-up is required for malignant transformation. Although there is no identification for prophylactically orthotopic liver transplantation in the treatment of the disease, it should be kept in mind for the choice of the treatment in patients who have diffuse symptomatic disease with cirrhosis or portal hypertension [149].

#### **9.5 Follow-up**

Patients should be monitored every 6 months during the postoperative 3 years and then annually. On initial follow-up, while all patients should be evaluated with complete blood count, liver function tests and abdominal US, on subsequent followup, investigations are done only in symptomatic ones. Long-term follow-up can be made by visits, telephonic conversations, and postal inquiry [124].
