**10. Outcomes and results**

Resection of pediatric CC is generally well tolerated [18]. Despite recent advances in surgical techniques and perioperative management, short- and long-term complications are not rare in children, while they are more common in adults [6]. Complications such as recurrent cholangitis attacks, malignant transformation, intracystic or intrahepatic gallstone formation, cirrhosis development, and pancreatitis are common in patients who are not operated on. Complications such as anastomotic leakage, gastrointestinal or intraabdominal bleeding, acute pancreatitis, pancreatic leakage, wound infection, wound dehiscence, intraabdominal infection/abscess, intussusception can be seen in early postoperative period defined as short-term complications [150]. Most early complications can be treated conservatively [151].

However, most series are without early mortality and report rates of acute complications including wound infections from 0 to 17%, without significant difference between infants and children [46, 132].

Surgical inexperience and severe inflammation are often implicated in the development of anastomotic bile leakage [151]. The diagnosis of bile leakage is difficult and delayed in some cases due to nonspecific symptoms [151]. It may not always be possible to differentiate with the imaging findings of US and CT because not all of the intraabdominal fluid collection after surgery is associated with bile leakage, and so, this late diagnosis may result in mortality due to septicemia and septic shock [151]. However, it is reported that MRCP can be used to diagnose and accurately localize the site of bile leakage noninvasively [7, 151]. Bile leaks in the hepaticooenterostomy line can self-limited within a few weeks if they can be drained externally. If the bowel movement is sufficient during this period, the child can be fed by enterally. If the extracted bile is given back to the stomach with NG catheter, electrolyte losses can also be prevented. The bilirubin level of the child may remain high due to edema in the anastomosis line within the first 2 weeks, even if the operation has been successfully performed. If this takes longer, biliary tree, even anastomosis, can be evaluated with PTC [110]. Reoperation is considered only after the failure of conservative treatment [151]. The leakage can be repaired by a circumferential buried suture around the anastomotic site, peritoneal lavage, and effective drainage [151].

**101**

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

duct stone formation [117].

bowel obstruction developing postoperatively [156].

Gastrointestinal bleeding may be due to hepaticojejunostomy or stress ulcer. Acute pancreatitis occurs in patients with CC, both preoperatively and due to injury of the pancreatic tissue during distal dissection of the cyst or to edema in the distal part of the pancreatic duct related postoperatively (4.2%) [151]. Therefore, it has been recovered with a conservative treatment for a short time period. Some reports say that CC excision without ligation of the distal stenotic stump decreases the incidence of pancreatic duct injury [75]. A probe inserted into the pancreatic duct through a duodenotomy may help to prevent pancreatic duct injury in difficult cases [75]. Additionally, Urushihara et al. [152] consider that using bipolar electro cautery to scrape pancreatic tissue away from the bile duct wall during the dissection of the intrapancreatic part of the bile duct causes minimal bleeding and enables clear identification of the narrow part of the CBD [151]. Eventually, complete resection of the distal portion of the cyst, removal of debris and protein plugs in the long common channel and pancreatic duct, and correction of anomalous arrangement of the pancreaticobiliary duct junction are essential to minimize pancreatic complications after the operations [151]. The pancreatic fistula occurs because of not closing the distal choledoch well after the cyst excision or injured pancreatic duct during the dissection. An external drainage of 3–4 weeks allows the fistulas closing. Late/long-term complications (5–15%) [151] include anastomotic stricture, cholangitis, hepatolithiasis, ileus, cirrhosis, and malignancy. Benign anastomotic stricture with recurrent cholangitis is less common than in adults but is still seen in many as 10–25% of patients and can be associated with both intrahepatic and bile

After intraabdominal surgery, small bowel obstructions, mostly due to adhesions, are common [154]. Patients should be closely monitored for any possible clinical deterioration [154]. If there is no improvement after 48 h of follow-up, there is a high risk of bowel resection due to bowel necrosis despite it has not been clearly defined [156]. Furthermore, it has been defined that during biliary reconstruction, the length and placement of the Roux loop is very important in adhesive

In terms of anastomotic stricture, improvement of surgical skills, preservation of blood supply, no or mild inflammation cyst wall, and construction of wide (larger than 1 cm) and tension-free stoma are key factors to reduce anastomosisrelated complication [138]. There should be no delay in surgical or endoscopic intervention once biliary obstruction develops postoperatively, but a great deal of planning and a thoughtful workup are required [151]. Kim et al. [155] reported that PTC with stone removal and balloon dilatation was useful in patients with anastomotic stricture. However, some investigators consider that recurrent anastomotic strictures may occur due to fibrosis even after balloon dilatation with PTCS, and repeated cholangitis may cause multiple intrahepatic biliary strictures, recurrent hepatic stones, and development of biliary carcinoma [152, 153]. Hence, especially in young patients, it is recommended that revision of the hepaticojejunostomy followed by ductoplasty, to create a wide stoma for sufficient bile drainage [152, 153]. Rigorous long-term follow-up after pediatric CC resection is limited, but the risk of biliary carcinoma (cholangiocarcinoma, squamous cell carcinoma, sarcoma, gallbladder cancer [12, 157], most often cholangiocarcinoma) clearly remains elevated even after CC excision compared to the general population [18]. The malignancy risk is considered to increase with age at surgery, and the cumulative biliary malignancy risk 25 years after primary surgery has been reported to be as high as 11% [75]. Malignant disease has been noted in up to 14% 0f patients after CC resection as a child [46]. In fact, cancer is the most frequent cause of late mortality in pediatric CC series [18]. Even after complete excision, patients are at higher risk for malignancy than general population [158]. Continued surveillance is, therefore,

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

*Pediatric Surgery, Flowcharts and Clinical Algorithms*

**9.5 Follow-up**

**10. Outcomes and results**

between infants and children [46, 132].

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

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

Resection of pediatric CC is generally well tolerated [18]. Despite recent advances

in surgical techniques and perioperative management, short- and long-term complications are not rare in children, while they are more common in adults [6]. Complications such as recurrent cholangitis attacks, malignant transformation, intracystic or intrahepatic gallstone formation, cirrhosis development, and pancreatitis are common in patients who are not operated on. Complications such as anastomotic leakage, gastrointestinal or intraabdominal bleeding, acute pancreatitis, pancreatic leakage, wound infection, wound dehiscence, intraabdominal infection/abscess, intussusception can be seen in early postoperative period defined as short-term complications [150]. Most early complications can be treated conservatively [151]. However, most series are without early mortality and report rates of acute complications including wound infections from 0 to 17%, without significant difference

Surgical inexperience and severe inflammation are often implicated in the development of anastomotic bile leakage [151]. The diagnosis of bile leakage is difficult and delayed in some cases due to nonspecific symptoms [151]. It may not always be possible to differentiate with the imaging findings of US and CT because not all of the intraabdominal fluid collection after surgery is associated with bile leakage, and so, this late diagnosis may result in mortality due to septicemia and septic shock [151]. However, it is reported that MRCP can be used to diagnose and accurately localize the site of bile leakage noninvasively [7, 151]. Bile leaks in the hepaticooenterostomy line can self-limited within a few weeks if they can be drained externally. If the bowel movement is sufficient during this period, the child can be fed by enterally. If the extracted bile is given back to the stomach with NG catheter, electrolyte losses can also be prevented. The bilirubin level of the child may remain high due to edema in the anastomosis line within the first 2 weeks, even if the operation has been successfully performed. If this takes longer, biliary tree, even anastomosis, can be evaluated with PTC [110]. Reoperation is considered only after the failure of conservative treatment [151]. The leakage can be repaired by a circumferential buried suture around the anastomotic site, peritoneal lavage, and

symptomatic disease with cirrhosis or portal hypertension [149].

made by visits, telephonic conversations, and postal inquiry [124].

**100**

effective drainage [151].

Gastrointestinal bleeding may be due to hepaticojejunostomy or stress ulcer.

Acute pancreatitis occurs in patients with CC, both preoperatively and due to injury of the pancreatic tissue during distal dissection of the cyst or to edema in the distal part of the pancreatic duct related postoperatively (4.2%) [151]. Therefore, it has been recovered with a conservative treatment for a short time period. Some reports say that CC excision without ligation of the distal stenotic stump decreases the incidence of pancreatic duct injury [75]. A probe inserted into the pancreatic duct through a duodenotomy may help to prevent pancreatic duct injury in difficult cases [75]. Additionally, Urushihara et al. [152] consider that using bipolar electro cautery to scrape pancreatic tissue away from the bile duct wall during the dissection of the intrapancreatic part of the bile duct causes minimal bleeding and enables clear identification of the narrow part of the CBD [151]. Eventually, complete resection of the distal portion of the cyst, removal of debris and protein plugs in the long common channel and pancreatic duct, and correction of anomalous arrangement of the pancreaticobiliary duct junction are essential to minimize pancreatic complications after the operations [151]. The pancreatic fistula occurs because of not closing the distal choledoch well after the cyst excision or injured pancreatic duct during the dissection. An external drainage of 3–4 weeks allows the fistulas closing.

Late/long-term complications (5–15%) [151] include anastomotic stricture, cholangitis, hepatolithiasis, ileus, cirrhosis, and malignancy. Benign anastomotic stricture with recurrent cholangitis is less common than in adults but is still seen in many as 10–25% of patients and can be associated with both intrahepatic and bile duct stone formation [117].

After intraabdominal surgery, small bowel obstructions, mostly due to adhesions, are common [154]. Patients should be closely monitored for any possible clinical deterioration [154]. If there is no improvement after 48 h of follow-up, there is a high risk of bowel resection due to bowel necrosis despite it has not been clearly defined [156]. Furthermore, it has been defined that during biliary reconstruction, the length and placement of the Roux loop is very important in adhesive bowel obstruction developing postoperatively [156].

In terms of anastomotic stricture, improvement of surgical skills, preservation of blood supply, no or mild inflammation cyst wall, and construction of wide (larger than 1 cm) and tension-free stoma are key factors to reduce anastomosisrelated complication [138]. There should be no delay in surgical or endoscopic intervention once biliary obstruction develops postoperatively, but a great deal of planning and a thoughtful workup are required [151]. Kim et al. [155] reported that PTC with stone removal and balloon dilatation was useful in patients with anastomotic stricture. However, some investigators consider that recurrent anastomotic strictures may occur due to fibrosis even after balloon dilatation with PTCS, and repeated cholangitis may cause multiple intrahepatic biliary strictures, recurrent hepatic stones, and development of biliary carcinoma [152, 153]. Hence, especially in young patients, it is recommended that revision of the hepaticojejunostomy followed by ductoplasty, to create a wide stoma for sufficient bile drainage [152, 153].

Rigorous long-term follow-up after pediatric CC resection is limited, but the risk of biliary carcinoma (cholangiocarcinoma, squamous cell carcinoma, sarcoma, gallbladder cancer [12, 157], most often cholangiocarcinoma) clearly remains elevated even after CC excision compared to the general population [18]. The malignancy risk is considered to increase with age at surgery, and the cumulative biliary malignancy risk 25 years after primary surgery has been reported to be as high as 11% [75]. Malignant disease has been noted in up to 14% 0f patients after CC resection as a child [46]. In fact, cancer is the most frequent cause of late mortality in pediatric CC series [18]. Even after complete excision, patients are at higher risk for malignancy than general population [158]. Continued surveillance is, therefore,

strongly recommended, though it is not known whether there are risk factors such as retained portion of cyst or not [18]. In those with known malignancy, oncologic principles should apply; patients who can undergo safe resection with negative margins are appropriate for operation [15]. Resection may include hepatectomy with regional lymphadenectomy, extirpation of extrahepatic bile ducts with regional lymphadenectomy (and cholecystectomy), or pancreaticoduodenectomy [15]. However, the 5-year survival rate for patients with CCs complicated by malignancy is high, up to 55% in patients with cholangiocarcinoma [159].
