Laparoscopic Choledochal Cyst Resection with Simplified Common Bile Duct Reconstruction in an Adult Population: A Case Series

INTRODUCTION

Choledochal cysts are congenital dilatations of the intrahepatic or extrahepatic biliary ducts.^1–5 They are associated with the development of cholangiocarcinoma. Their incidence predominates in eastern population where it is reported to be approximately 0.00769% higher than western population where the incidence is around 0.000666%. These are estimated numbers; the real incidence of CCs in adult population has not been established so far, since in most cases they are incidentally diagnosed on images or during surgery.^1,2,6

The first case of laparoscopic approach for CC resection was published by Farello et al. in 1995, since then, different studies have shown that minimally invasive techniques can reduce surgical time, postoperative morbidity, postoperative pain control, and incidence of surgical site infections (SSI). Better results with laparoscopy are reported on patients who have CC Todani type I, II, and IVb compared with the results with open approaches.^2,6,7

We present our experience with laparoscopic CC resection and simplified bile duct reconstruction in an adult population, analyzing the technical aspects of a novel procedure, postoperative clinical evolution of the patients involved in the study, and the incidence of cholangiocarcinoma in a 2-year postoperative follow-up.

MATERIALS AND METHODS

INDICATIONS

In order to include patients in this study, the following criteria were evaluated. Only patients over 18 years of age, who had a diagnosis of CC (Todani type I, II, and IVb) (Fig. 1) and Table 1 who were taken to laparoscopic CC resection and simplified common bile duct reconstruction were selected. Patients with the following characteristics were excluded from the study: those diagnosed with CC type III, IVa, V, and Child Pugh B (or higher) hepatic cirrhosis, extrinsic bile duct compression (e.g., periampullary tumors), or cholangiocarcinoma, patients who had already been treated surgically for CC at another center, patients who were coursing with cholangitis and patients had a contraindication for laparoscopic surgery.

SURGICAL TECHNIQUE

STATISTICAL ANALYSIS

Being an observational retrospective cohort, a descriptive analysis of the data was performed prospectively in Microsoft Excel databases and analyzed using SPSS1 (Statistical Package for The Social Sciences) V.22.0. Variables were analyzed and median, minimum, maximum values, and percentages were obtained.

RESULTS

DISCUSSION

In 1723, Vater and Ezler described for the first time, the CC; however, it was not until 1977 that Todani described the classification that it is most frequently used nowadays (Fig. 1).^4,5,8,9 The management of this pathology has evolved from a cysto-enterostomy to advanced bilioenteric reconstructions with Roux-en-Y configuration.^3,10

Prior to 1980’s, management for common bile duct consisted on drainage; however, reports of cholangiocarcinoma, recurrent cholangitis, and biliary lithiasis led to a change in the way surgeons managed this disease.^3,4 That is why currently, the management of Todani I, II, and IV CC is similar and involve a complete resection of the defect with posterior bilioenteric reconstruction.^1–3,5,11

Sastry et al. show the incidence of CC according to the Todani classification, being type I the most frequent (69.8%), followed by type IV (23.7%), type V (3.1%), type II (2%) and type III (1.4%).¹² The goal of the surgical management of type IV-A and IV-B CC is to stop or at least slow down the progression of liver damage. The complications of non-operated CC are the result of stasis in which cholangitis, biliary stone formation, recurrent pancreatitis, cirrhosis, and portal hypertension without mentioning the risk for cholangiocarcinoma; for that reason, surgical management in adult population is indicated.¹³

When complete resection of the cyst is not possible (usually in CC type IV-A) complete resection of the extrahepatic biliary tract should be performed in addition to a lobectomy (of the compromised portion of the intrahepatic biliary tree) with posterior biliary-enteric reconstruction. Incomplete resection of the cyst does not seem to be related to perioperative complications although some argue a persistent risk of malignancy.^14,15

The usefulness of laparoscopy in the surgical management of this type of patients has been questioned due to the complexity of the procedures, the need for precise movements, and the long learning curve necessary to obtain adequate results.¹⁶ However, resection with minimally invasive technique with a Roux-en-Y reconstruction has been shown to be safer when compared to open approach.^16,17

Some of the advantages of laparoscopic management in these cases are the better visualization of structures, more precise dissections due to the magnification of structures, less postoperative pain, shorter hospital stay time, better esthetic results, decreased bleeding, lower risk SSI and lower incidence of postoperative ileus; being the only negative aspect a prolonged surgical time.^6,18 However, some authors have described an association between the laparoscopic approach and an augmented risk of malignancy due to incomplete CC resections that lead to chronic inflammatory process; they argue that open approach should not be fully abandoned since it is possible to better identify structures even in cases of severe local inflammatory processes and anatomical distortion.^3,11

Some patients benefit from a two-stage surgical procedure, where during the first surgical time the CC is drained. During the second operative time (after physiological resuscitation in the ICU and resolution of the acute phase), the resection of the cyst is performed.¹ In our series of cases, complete resection of the CC and bilioenteric reconstruction was performed in one surgical time with a 0% conversion rate and no need of reintervention.

Perhaps the most important aspect of the procedure, besides an adequate resection of the CC, is an adequate biliary-enteric reconstruction where the anastomosis made should allow free biliary flow into the intestine, avoiding as biliary reflux, which is the most important cause malignancy in these patients.^1,3 It can be done with a hepatic-duodenostomy, choledochojejunostomy, or with a Roux-en-Y hepaticojejunostomy.^1,3,6 Being the first and third the most frequent; today there is great controversy regarding which of the two most used procedures is the best as it can be evidenced in the experience of Narayanan et al., who in 2013 published a systematic review in which when comparing 679 cases of patients, 60.7% taken to hepatico-duodenostomy and 39.3% to hepaticojejunostomy, respectively, they reported: a hospital stay time of 4.8 days and 6.1 days, the incidence of biliary leakage 2.1% and 2.94%, the incidence of cholangitis 2.47% and 2.42%, the incidence of anastomotic stenosis 1.21% and 1.47%, the incidence of biliary reflux 5.88% and 0%, incidence of intestinal obstruction due to adhesion syndrome 0% and 5.12%, and the need for re-intervention 1.21% and 2.45%.¹⁹ Most studies demonstrate that there are really no clinically significant differences between the two procedures. However, due to the existing evidence with hepaticoduodenostomy and the development of gastric cancer secondary to biliary reflux, in our study, we only performed bilioenteric reconstruction with a Roux-en-Y hepaticojejunostomy.^1,20 In our case series, there was no biliary leakage, no re-intervention were needed; and our hospital stay time was shorter to the one reported in the medical literature.

It is a common factor among the opinion of experts in the world medical literature on this regard that the laparoscopic approach is a challenging for the surgeon but if performed correctly it is effective and appropriate.² The conversion rate to open approach ranges from 0% to 37%, as described by Palanivelu et al. (2008) in a study published in the Journal of the American College of Surgeons that report a conversion rate of 8.5%. However, in our series there were no conversions to open surgery.³ The age of the patient has been shown to be directly related to the need for conversion, is it more frequent in pediatric patients than in adults, which is extrapolated to our results.² On average, the length of hospital stay time in minimally invasive management ranges from 3 days to 4.7 days and in the open approach from 5 days to 20.5 days and has a mortality of up to 3.3%, as evidenced in our series.^2,3,6

Early postoperative complications include pancreatitis, enteric or biliary leakage from the anastomosis, bleeding, SSI, and pancreatic or biliary fistulas. The most frequent delayed complications (after 30 days postoperative) are intrahepatic or extrahepatic bile duct stenosis, lithiasis, malignancy, intestinal obstruction, recurrent pancreatitis, hepatic cirrhosis, and cholangitis.^1,4,8 Postoperative complications in children are rare; however, in adults, they occur between 17% and 40% of cases.¹ the most frequent complication with an incidence of 0% to 20% of cases is an anastomotic leakage; however, in our study, there were no postoperative complications.³

Studies that support the use of robots for the surgical management of CC, such as the one reported by Wang et al. who reported their experience in 2016 with a 26-year-old patient who was diagnosed with a type I CC and decided to take him to a robotic Roux-en-Y hepaticojejunostomy with the alimentary and bilioenteric loops in a retrocolic position with satisfactory results in terms of incidence, early or late complications, esthetic results and SSI, with a surgical time of 480 minutes (longer when compared with our surgical time) and a bleeding of 100 cc (also greater than the one reported in our series).¹⁰ The evidence on robotic surgery in these cases is very limited; however, some authors consider that aspects such as the magnified three-dimensional image, along with more precise movements could represent the difference in the prognosis of the patients.^1,10

As it was said before, some authors have reported that cholangiocarcinoma may be a secondary to unresected portions CC, with an incidence of up to 30% in the adult population.²⁶ The global incidence reported in the word medical literature for cholangiocarcinoma is 0.95 per 100,000, being more frequent in patients with CC.^21,22 The risk of malignancy reported for these patients is 0.7% to 28%, this risk increases over time.^2,5,6,8,23 Sastry et al., in one of their studies with a population of 7,880 patients (1,914 under 18 years of age and 3,866 adults), from which 4.59% had histopathological studies revealing malignancy, 70.4% of these patients had cholangiocarcinoma, 23.5% gallbladder carcinoma, and 6.1% other malignancies. The incidence of cholangiocarcinoma in type I CC is 68%; and in type IV CC it is of 21%.²¹ The risk of malignancy is high, and it is associated with a global survival of 6 to 21 years. Incidence of malignancy referred by Amid et al., in type IV CC was of 9.2%, type I 7.6%, type II 4.3%, type III 4%, and type V 2.5%.¹² For this reason, it is imperative to make a complete resection of CC and to make a strict postoperative follow-up.

There is still some controversy regarding abandoned CC parts and their risk of malignancy; however, some of the studies on this regard seem to indicate that a complete resection is superior to a partial resection in terms of reducing the risk of malignancy as it is shown by Ten Hove et al. in a meta-analysis published in 2018, where they compare a total of 80 studies and 2,904 patients, with an incidence of 10.7% of cholangiocarcinoma, with an increased risk for patients with type I and IV CC with a statistically significant p of 0.016, and with an increased risk for cholangiocarcinoma for patients taken to CC drainage and partial resection with an OR of 3.97, almost four times more than patients taken to complete resection (as it was performed in our series).^1,2,24,25 Mutations in K-ras and p53 genes have been associated with cholangiocarcinoma and a history of common bile duct disease.^1,4,8 Nevertheless, no tumor markers show direct relationship between their serum levels and the presence of cholangiocarcinoma.²⁶

Nicholl et al. revealed a direct correlation between patient age and cancer risk: from 0 year to 30 years (0%), from 31 years to 50 years (19%), and from 51 years to 70 years (50%).²⁷ Surgical management of CC with complete surgical excision during early life tends to decrease the probability of developing cancer cholangiocarcinoma later in life especially in the group of patients with type I CC.^28,29 One study of 56 patients with a previous history of resection three patients progressed to cholangiocarcinoma with a range of 2–19 years after the procedure, showing that a strict follow-up is necessary after the procedure and during a long period of time to ensure an early diagnosis and improve long-term outcomes even in malignant positive patients.³⁰ Other study that supports this shows that 15 patients out of 214 cases reported were associated with malignancy of the biliary tree; their survival was of 6–21 months after being diagnosed, poor prognosis was associated with late ³¹ Lee et al. in a study of 40 patients diagnosed with cholangiocarcinoma during early stages and had a better prognosis; specifically, the 5-year survival rates of patients with stages Ia, Ib, and IIa cholangiocarcinoma reached 90.4%, 40.0%, and 25.1%, respectively.³² The main limitation of this study is that it is an observational retrospective study without randomization and no control group. It is therefore subject to selection bias. In addition, the population size of our study may be too small to draw statistically relevant conclusion, even if it is an infrequent disease in Latin America.

CONCLUSION

Adult CC are a group of rare entities that lead to high mortality and risk of developing cancer, these make their management critical and necessary. Complete resection of the CC and laparoscopic Roux-en-Y bilioenteric reconstruction is an effective and safe method, although complex and challenging for the surgeon. This simplified laparoscopic approach to bile duct reconstruction with Roux-en-Y anastomosis seems to be an effective and safe alternative for the management of this pathology. Additional research is warranted to assess long-term results in a larger series.

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