Long-term Outcome for Children Undergoing Open Hepatico-jejunostomy for Choledochal Malformations: a 43-year Single-center Experience

Long-term Outcome for Children Undergoing Open Hepatico-jejunostomy for Choledochal Malformations: a 43-year Single-center Experience | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Method Article Long-term Outcome for Children Undergoing Open Hepatico-jejunostomy for Choledochal Malformations: a 43-year Single-center Experience Maria Vittoria Stern, Giovanni Boroni Boroni, Filippo Parolini, and 3 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-3615808/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 19 Jan, 2024 Read the published version in Pediatric Surgery International → Version 1 posted 7 You are reading this latest preprint version Abstract Purpose To report on our 43-year single-center experience with children operated on for Choledochal Malformations (CMs), focusing on long term results and quality of life (QoL). Materials and Methods All consecutive pediatric patients with CMs who underwent surgical treatment at our center between October 1980 and December 2022 were enrolled in this retrospective study. We focused on long-term postoperative complications (POCs), considered to be complications arising at least 5 years after surgery. We analyzed QoL status once patients reached adulthood, comparing the results with a control group of the same age and sex. Results 113 patients underwent open excision of CMs with a Roux-en-Y hepaticojejunostomy (HJ). The median follow-up was 8.95 years (IQR: 3.74–24.41). Major long-term POCs occurred in six patients (8.9%), with a median presentation of 11 years after surgery. The oldest patient is currently 51. No cases of biliary malignancy were detected. The QoL of our patients was comparable with the control group. Conclusion Our experience suggests that open complete excision of CMs with HJ achieves excellent results in terms of long-term postoperative outcomes. However, since the most severe complications can occur many years after surgery, international cooperation is advisable to define a precise transitional care follow-up protocol. choledochal malformation long term follow up Roux-en-Y hepaticojejunostomy quality of life Figures Figure 1 Figure 2 Introduction Formerly called choledochal cyst, Choledochal Malformations (CMs) have been more recently defined as congenital morphological abnormalities of the biliary tract characterized by abnormal dilatation (cystic or fusiform) of the choledochus in the absence of any mechanical obstruction [ 1 ] [ 2 ] [ 3 ]. Most CM cases are associated with a Pancreatico-Biliary Maljunction (PBM) and a Long Bilio-Pancreatic Common Channel (LBPCC); cases with concomitant intrahepatic bile duct (IHBD) dilatation are included in this entity. [ 3 ] [ 4 ] The incidence of CMs shows significant geographical variation. In Western populations they are increasingly diagnosed in childhood probably due to a higher index of suspicion and improved imaging techniques. [ 5 ] [ 6 ] [ 7 ] Regardless the time of presentation and diagnosis, early surgery is essential to mitigate potential damage to the liver and prevent severe complications and cancer. [ 4 ] [ 3 ] [ 8 ] Open complete excision of the Dilated Part of the Extrahepatic Biliary Tree and restoration of intestinal bile drainage with a Roux-en-Y hepaticojejunal anastomosis (RHJ) is still the gold standard treatment [ 9 ], even if minimally invasive approaches such as laparoscopic and robotic RHJ or hepatico-duodenostomy (HD) have gained popularity.[ 10 ][ 11 ][ 12 ] . In this study, we present our 43-year single-center experience with a large cohort of children with CM managed using an open surgery approach with follow-up into adulthood. To the best of our knowledge, this is one of the largest pediatric single-center series and the first Italian study of CM pediatric patients with very long-term follow-up. Material and Methods Data Source and Study Population All consecutive pediatric patients (<18 years) with CM who underwent surgical treatment in our Tertiary care Pediatric Surgery Department between October 1980 and December 2022 were enrolled in this retrospective study. Institutional Review Board approval was obtained (NP4246/2020). Written informed consent was obtained before all stages of the study. For each patient, we collected demographic, clinical data, imaging results, information on surgical procedures, POCs and clinical outcomes. Data were retrieved from patient medical records, outpatient clinic check-ups, telephone, and email interviews. For patients reaching adulthood, follow-up data were supplemented by information from the records held by their general practitioners. Even though diagnostic assessment and postoperative therapy varied through the period of the study, protocols were implemented to improve the standard of care. Preoperative assessment included laboratory tests, abdominal ultrasound (US), abdominal Computed Tomography Scan (CT) and – in the last 15 years – Magnetic Resonance Cholangio-Pancreatography (MRCP) and Endoscopic Retrograde Cholangio-Pancreatography (ERCP). Scheduled postoperative follow-up included blood tests and abdominal US initially at 1, 3, 6 months, and then at 12-month intervals for the first 5 years, every 2 years up to 10 years and subsequently every 5 years. In the last 4 years, we routinely added CA 19-9 to the laboratory exams. CMs were classified according to Todani classification. [13] Cholangitis was defined as presence of fever, abdominal pain with or without jaundice, increased levels of total and conjugated bilirubin and gamma glutamyl transferase. [14] Pancreatitis was defined as presence of abdominal pain, plasma amylase levels over three times the upper limit of normal and a positive US. [15] Pseudopancreatitis was defined as plasma amylase levels over three times the upper limit of normal but with normal US. Surgical complications were referred according to Clavien-Dindo Classification; Grades III and IV were major complications. [16] Long term POCs were those occurring at least 5 years after surgery. Quality of life (QoL) - evaluated only for patients once they came into adulthood - was assessed with a 26-item self-reported Word Health Organization Quality of life questionnaire (WHOQOL Bref), evaluating the subjective perception of individual health in four areas: physical, psychological health, social relationships, and environment. [17] The results were compared with a control group (CG) of healthy people, composed by medical students and other survey volunteers, of the same age and sex, to whom the questionnaire was also administered. Twenty-one people were enrolled in the CG. The questionnaire was sent by email and participants were asked to select from multiple choice answers. Informed consent was obtained from all participants. Statistical Analysis Quantitave data were described as mean (standard deviation), median (IQR). Median follow-up was estimated using Kaplan-Meier reverse estimator. Association between categorical data were estimated using χ2 test with Monte Carlo p-values computations or Fisher exact test, while quantitave variable were compared among groups using a Welch t-test for independent group. All tests were two sided and assumed a significance level of 5%. Surgical procedure Open complete excision of the Dilated Part of the Extrahepatic Biliary Tree and RHJ is our surgical procedure of choice. During the first 10 years, the procedure involved a right subcostal incision, extended to the left rectus abdominis muscle. Later, we used a self-retaining retractor (Thompson, Thompson Surgical Instrument, MI), that enabled us to perform smaller incisions. A bile sample was taken from the gallbladder or from the CM for amylase dosage. Surgical dissection went from the gallbladder fundus down to the common bile duct (CBD); the common hepatic duct (CHD) was then dissected and encircled with an umbilical tape or a vessel loop, observing its position in relation to the right hepatic artery, because it can cross the duct both above and below it. Since the 2000s, we have performed an intraoperative endoscopic evaluation (IEE) of the CM in all cases using a 7 or 7.5 Fr pediatric operating cystourethroscopy (Karl Storz GmbH & Co-KG, Tuttlingen, Germany). The use of IEE improved the detection and cleaning of debris, stones, or biliary sludge from the IHBD, the distal choledochus and the LPBCC; any bile duct strictures at the hepatic hilum are detected. Eventually IEE enabled us to visualize the outlet of the main pancreatic duct into the choledochus and explore the LBPCC. ( Video 1 ). We resected the CHD before beginning the CBD dissection, to ensure a safer dissection of its posterior wall from the portal vein and hepatic artery ( Fig. 1 ). The aim was to remove the distal part of CBD next to the junction with the main pancreatic duct, avoiding leaving an excessively long choledochal stump ( Fig. 2 ). The Lilly technique for CM was adopted in case of bleeding during this step of surgery. [18] Open complete excision of the Dilated Part of the Extrahepatic Biliary Tree was followed by an end-to-side or end-to-end RHJ with a jejunal loop of 35-50 cm in length according to the patient age, fashioned with 4/0 or 5/0 Polyglycol in the first 10 years and later with 5/0 or 6/0 Polydioxanone suture. Interrupted stitches, tied outside, were adopted when the CHD diameter was less than 1 cm, while two continuous hemisutures were used in all other cases. In case of end-to-side anastomosis, the ascending limb of the Roux-en-Y was opened as close as possible to the end of the blind jejunal pouch. When biliary stenosis was detected at hepatic hilum, biliary ductoplasty was performed with 7/0 polydioxanone interrupted stitches. Since 2000, we have added a 5-7 cm long jejunal “spur valve” approximating the native jejunum to the Roux-en-Y limb, to prevent the reflux of air and GI secretion into the biliary tree. Results Population One-hundred-thirteen patients with CM were managed at our department. CM was suspected during prenatal US assessment in 9 patients (8%), at a median of 28 gestational weeks (range 27–34). In the remaining 104 patients, the median age at diagnosis was 29 months (0.5–190 months). Eighty-nine patients (78.7%) were symptomatic at time of the diagnosis: 73 (64.6%) presented jaundice and/or increased values of serum markers of obstructive jaundice; 28 patients (24.7%) were diagnosed with true pancreatitis or pseudopancreatitis. Fifteen patients (13.3%) presented non-specific symptoms (e.g., recurrent abdominal pain, nausea, vomiting). In 24 patients (21%), CM was an incidental US finding. Epidemiological and Clinical data are summarized in Table 1 . Table 1 Demographic and clinical data Patients, n 113 Sex (F) 89 female (78.7%), Age at diagnosis, median (range) 29 months (0-190) Antenatal diagnosis, n (%) 9 (8%) Gestational week, median (range) 28 gestational week (27–34) Incidental diagnosis (asymptomatic), n (%) 24 (21%) Weight (at surgery) 13.8 kg (3.4–49) Todani Classification · Todani I a :53 · Todani I b: 5 · Todani I c: 35 · Todani II: 1 · Todani IV a: 16 · Todani IV b: 3 Surgical details All patients underwent surgery at a median age of 39 months (0.5–193), with a median weight of 13.8 kg (4–49). Patients with antenatal diagnosis were operated, at a median age of 3 months (0.5–32) ( p = 0.002). Ten patients (8.8%) had undergone previous treatments at other hospitals: 2 had undergone a cholecysto-jejunostomy; 2 a cysto-jejunostomy; 2 a cholecystectomy; 1 an hepaticojejunostomy and 3 an ERCP with papillo-sphincterotomy. All the patients in our series underwent open complete excision of the Dilated Part of the Extrahepatic Biliary Tree followed by an RHJ. LPBCC was detected in all patients. No patient had biliary carcinoma at the time of the operation. Complications and QoL Sixty-eight patients (60%) completed a follow-up longer than 5 years, 45 (39.8%) completed a 10-year follow up and 28 (24.7%) a follow-up longer than 20 years and up to 42 years. The overall survival rate of our series was 100%. In the study period, major surgical complications (Clavien Dindo grade III-IV) occurred in 8 patients (7%). Seven of these 8 occurred in patients that underwent surgery before 2000. Since then, only 1 case of adhesive small bowel obstruction (ASBO) was encountered. (Table 2 ). Table 2 Long-term complications, treatment, outcomes and follow up Major complications N (%) Todani Age at surgery Long-term POC Treatment Age at Follow-up & Outcome HJ stenosis with IHBD stones and cholangitis 2 (2.9%) 1 A 4 months 23rd POY Redo HJ 27.4 yrs, alive and well, NLE 1 A 107 months 24th POY PTHC and HJ stenting 30.8 yrs, alive and well, NLE Pancreatitis 1 (1.5%) 1 A 16 months 14th POY ERCP with papillo- sphincterotomy and stone removal 21.5 yrs, alive and well, NLE ASBO 2 (2.9%) 1 C 95 months 6th POY Open adhesiolysis 15.6 yrs, alive and well, NLE 1 A 7 months 8th POY Open adhesiolysis 26 yrs, alive and well, NLE Intussusception 1 (1.5%) 1 A 50 months 5th POY Operative reduction 21 yrs, alive and well, NLE Total 6 (8.8%) Legend : POY: postoperative year; HJ: hepaticojejunostomy; IHBD: intrahepatic bile ducts; ASBO: adhesive small bowel obstruction; JJ: jejunojejunostomy; NLE: normal liver enzymes. Focusing on patients with a follow up of at least 5 years, we observed 6 major complications (8.9%). Two patients (2.9%) developed stricture of the hepaticojejunal anastomosis (HJ) with cholangitis 23 and 24 years after the initial operation. One underwent successful open redo of the HJ and stone removal and the other underwent percutaneous transhepatic stone removal with lithotripsy and HJ stenting. One patient (1.5%) operated on when he was 3 years old, complained of recurrent pancreatitis with a pseudocyst formation 14 years after surgery. A stone was detected in the CBD stump. He underwent ERCP with papillo-sphincterotomy and stone removal followed by the insertion of a “pigtail” drain in the pancreatic pseudocyst. So far, 8 years after the endoscopic procedure, he is doing well with no recurrence of pancreatitis or stones. He was operated on before we routinely used IEE. Two patients (2.9%) with ASBO, and patient (1.5%) with an ileo-ileal intussusception underwent surgery respectively 6, 8 and 5 years after the CM treatment. One patient underwent an open redo of HJ anastomosis because of a major abdominal trauma with liver and bile duct injury caused by a car accident at the age of 45, 30 years after the first surgery. At present, at 35 years of follow-up, she is doing well with normal liver function tests and US. We did not find any relationship between type of CM, coded as I and IV, and HJ anastomotic stricture (Fisher exact test, p = 1), and age at surgery (Welch t-test, IV vs I, difference − 6.39, CI 95% -16.17; 28.95, p = 0.57). One patient developed a non-Hodgkin Lymphoma 20 years after surgery. He’s currently disease free with normal liver function tests and liver US at follow-ups, 16 years after his lymphoma diagnosis and 36 years after surgery. No cases of hepatic cirrhosis, portal hypertension and malignant transformation were recorded. At the last follow-up, 61 patients (90%) are symptom free, 6 (9%) reported dyspepsia and/or 4 (6%) recurrent abdominal pain. Given that we decided to administer the questionnaire only to patients aged over 18 years, currently 82 patients (72%) fall into this subset; 60 of these did not answer and 1 was excluded because of severe mental retardation. The remaining 21 patients (25.6%) completed the questionnaire. The median follow-up for this subset of patients after surgery was 29.28 years (IQR, 24.34–33.67, range = 8-38.5). Twenty-one people were enrolled in the CG. The mean age of the CG was 31.1 years (SD = 6.78), median age 30 years (IQR = 4, range = 18–52). No statistically significant differences were found between groups in term of sex (proportion of males 19.0% Cases in vs 23.8% CG, Fisher exact test, p = 1) and age (CG vs Cases, Welch t-test, difference − 0.95, CI 95% -5.81; 3.90, p = 0.69). Patients with CM achieved total scores comparable to the CG (Table 3 ). Table 3 Comparison of Quality of Life among CM patients and the Control Group CM patients (n = 21) Control Group (n = 21) p value Males, n (%) 4 (19.0%) 5 (23.8%) 1 Age Mean (sd) 31.6 (8.69) 31.1 (6.78) 0.69 Median (IQR, range) 31 (11, 18–51) 30 (4, 18–52) QoL total score Mean (sd) 61.2 (5.73) 60.7 (4.90) 0.77 Median (IQR, range) 62 (8, 50–70) 60 (7, 49–68) Within the CM group, there were no statistically significant differences in QoL total score between patients who experienced surgical complications (N = 2) and patients who did not (Welch t-test, long -term complications Yes vs No, difference 1.45 CI 95% -4.54; 1.64, p = 0.34). Six women had a normal pregnancy and delivery, and all their children are in good health. Seven patients (33%) (6 women and 1 man) declared themselves uncomfortable with their surgical scars, however, the QoL score of these patients was in any case comparable with the CG (Welch t-test, p = 0.55). Discussion The present study reports our 43-year single-center experience of 113 children with CM, for whom Open complete excision of the Dilated Part of the Extrahepatic Biliary Tree and RHJ provided excellent results even at a very long-term follow-up. In our series, we had a low rate of long-term POCs, such as HJ stricture (2.9%), IHBD stones (2.9%) cholangitis (2.9%), pancreatitis (1.5%) and biliary cancer (0%). Cholangitis and IHBD stones were observed only in patients with stricture of the HJ, and not related to ascending cholangitis due to a reflux of gastrointestinal secretions into the limb of the Roux-and-Y as far as the liver. We think that the early surgical refinements we adopted helped to keep the rate of POCs low.[ 19 ][ 20 ] The first strategy was to perform an IEE in order explore and clean the IHBD, the distal CBD and the LBPCC. IEE also facilitated the identification of the pancreatic-biliary junction to avoid leaving an excessively long CBD distal stump. Furthermore, any biliary stricture at hepatic hilum can be easily detected, better evaluated, and surgically treated at the same time. The second strategy − as suggested by some authors to reduce the incidence of ascending cholangitis in biliary atresia − was the construction of a spur valve to prevent reflux of gastrointestinal secretions into the Roux-and-Y ascending limb. [ 21 ] The final strategy is to fashion the HJ anastomosis very close to the blind pouch of the jejunal limb to avoid anastomotic stricture and/or a redundant blind loop that can lead to bile stasis and the onset of repeated cholangitis of the IHBD that leads to intrahepatic lithiasis. [ 22 ] [ 23 ] [ 24 ] Chronic inflammation of the bile duct is the high-risk factor for carcinogenesis. [ 8 ] [ 13 ] [ 25 ] [ 26 ] [ 27 ] Timing of surgical repair in patients with antenatal diagnosis is controversial. [ 4 ] [ 28 ] [ 29 ] [ 30 ] Nevertheless, in our experience and in accordance with the Japanese Society of Hepato-Biliary-Pancreatic Surgery [ 4 ], we recommend early operation (3–6 months) even in asymptomatic patients, to avoid the progression of liver fibrosis and reduce the possibility of complications such as cholangitis, biliary obstruction, and pancreatitis. [ 26 ] In our series, of 9 patients with antenatal diagnosis, the surgical complication rate was 0%. Among authors, the debate continues about which techniques of biliary reconstruction should be preferred [ 3 ] [ 31 ] [ 32 ] [ 33 ] [ 34 ]. With the increasing trend of MIS, HD has been widely preferred over HJ because it is easier to perform laparoscopically and can be completed without the need for extracorporeal enteric sutures [ 31 ] [ 35 ] [ 12 ]. However, HD may predispose towards duodenogastric bile reflux (DGBR), gastritis and esophagitis. [ 31 ] Authors have shown a high risk of carcinoma in the residual stomach after a distal gastrectomy, secondary to exposure of the gastric mucosa to bile and pancreatic secretions due to DGBR [ 33 ]. Hamada et al, reported an incidence of DGBR in all his 17 patients who underwent HD; reflux was confirmed by endoscopic examination. Seven of these patients were subsequently converted with a new surgery to HJ at a mean of 13 years after the initial excisional surgery due to the presence of significant abdominal symptoms. [ 34 ] We agree with several authors that any type of surgery that favors DGBR should be avoided in children because of a potential long-term risk of gastric cancer. [ 32 ] [ 33 ] [ 34 ] [ 36 ] Furthermore, Todani et al, after having detected a case of hilar adenocarcinoma secondary to HD, hypothesized that such reconstruction could also promote the reflux of bile and pancreatic juice in the biliary epithelium, which would then suffer chronic inflammation, responsible for malignant degeneration. Therefore, they abandoned this technique. [ 33 ] [ 25 ] In this regard, there are two major advantages to HJ over HD: it enables the separation of the pancreatic juice from bile, allowing the latter to mix at the level of the intestinal lumen and consequently, along the length of the Roux limb, the risk of reflux into the bile ducts is reduced [ 37 ] Secondly, no bile reflux in the stomach is observed. Therefore, we believe that HD should no longer be performed in children with CM. In addition, another risk factors for the occurrence of biliary cancer secondary to CM resection may include the incomplete excision of the CM or, less frequently, an undetected subclinical disease at the time of the first operation. [ 26 ] [ 38 ] [ 39 ] Complete excision of the CM − including the need to leave the choledochal stump as short as possible − is critical because it allows the removal of the portion most frequently affected by cancer. [ 39 ] [ 40 ] Our exclusive pediatric series had no incidence of biliary cancer even at follow-up beyond 40 years. However other studies have demonstrated a non-negligible risk of biliary cancer. [ 37 ] [ 40 ] [ 41 ] Other recent data suggest a lifelong elevated risk of up to 4% of cancer in patients who underwent CM excision in pediatric age [ 8 ]. The most frequently described type of tumor is cholangiocarcinoma. [ 8 ] [ 27 ] However, rare cases of mucinous cystadenoma, metachronous cholangiocarcinoma and bile duct schwannoma have been reported after the first operation. [ 8 ] [ 42 ] [ 43 ] [ 44 ]. Tumors were reported primarily in Asian series, where the incidence of CM is higher. Patients were all adults with an age range of 18 to 60 years. [ 8 ] [ 27 ] [ 43 ] Considering this evolution, it is mandatory that, not only pediatric surgeons, but also general surgeons be aware of this pathology and its implications in adult life. We therefore agree with authors who argue that lifelong follow-up should be mandatory.[ 8 ] Furthermore, according to our experience, an additional point that supports the argument for continuing long-life follow-up is the long timespan between the original surgery and the development of stricture of the bilio-digestive anastomosis. Several publications on pediatric CMs in recent years have concerned the laparoscopic approach, but relatively few data are available on the long-term outcome with only a few papers reporting follow-up longer than 5 years [ 3 ] [ 12 ] [ 31 ]. In some cases, the long-term outcomes of open surgery are being overshadowed by the cosmetic benefits and potential short-term advantages of the laparoscopic approach. [ 3 ] [ 32 ] [ 45 ] [ 46 ] [ 12 ] However, concern arises because it seems that laparoscopic approach may lead, in case where inflammations is present, to an incomplete excision of the dilated CBD, leaving an excessively long choledochal stump. [ 3 ] This would increase the formation of stones and possibly the later development of cancer in the stump, besides pancreatitis. So, although MIS has been reported to be a feasible and safe surgical option [ 3 ] [ 12 ] [ 32 ] [ 45 ] [ 46 ], care should be taken before dispensing with standard open techniques, which have proven minimal complications and long-term benefits. The laparoscopic approach in children with CM, can be reserved for favourable cases only (adequate body weight, size of the hepatic duct at the level of biliodigestive anastomosis, absence of previous complications and no need of biliary ductoplasty) by surgeons trained in open and MIS. It is likely that robotic surgery is going to overcome all these issues, by adopting excellent magnification, 3D visions and easier and more precise and distally extended CBD dissection. Robotics can also improve HJ fashioning and enable a wide HJ to be performed using the hilar portion of the proximal hepatic duct. Our adult patients who answered the QoL questionnaire achieved scores that are comparable to controls. In our study, adverse long-term QoL concerns does not seem to be related to surgical complications. This is in contrast with Baba et al, whose study concluded that only patients without surgical complications have a QoL comparable with controls. [ 45 ] One possible explanation could be the lower rate of POCs occurring in our series. The only complaint from our patients was surgical scarring: 7 out of 21 (33%) patients stated that they feel different and embarrassed about taking off their clothes in the locker room or on the beach. Since 2015, we offer a plastic surgeon consultation for teenager and adult patients with non-cosmetic surgical scarring. We are aware that our study presented some inherent limitations. Firstly, the retrospective nature of the study implies that some patients were lost during follow-up. Similarly, the low collection rate of questionnaires from patients who are now adults could undermine the efficacy of the QoL evaluation. Some of the oldest patient’s data have been lost due to the lack of digital storage, resulting in the impossibility to recover contacts. Eventually, the loss of some information is due to the absence of a precise transitional protocol from pediatric to the adult care. Moreover, the long period of the study implies variation in diagnostic assessment, surgical expertise, and postoperative follow-up. Not surprisingly, the highest rate of surgical complications occurred in the first decades of the study. Despite these limitations, our 42-year experience suggests that open complete excision of the Dilated Part of the Extrahepatic Biliary Tree with RHJ offers excellent results in terms of few POCs even at very long-term follow-up. However, we are aware and convinced that international cooperation is required to define a precise follow-up protocol when pediatric patients reach adulthood to investigate and early detect long-term complications, especially the occurrence of biliary cancer. Abbreviations Choledochal Malformations (CMs); quality of life (QoL); postoperative complications (POCs); hepaticojejunostomy (HJ); Roux-en-Y hepaticojejunal anastomosis (RHJ); Pancreatico-Biliary Maljunction (PBM); Long Bilio-Pancreatic Common Channel (LBPCC); intrahepatic bile duct (IHBD); hepatico-duodenostomy (HD); ultrasound (US); Computed Tomography Scan (CT); Magnetic Resonance Cholangio-Pancreatography (MRCP); Endoscopic Retrograde Cholangio-Pancreatography (ERCP); common bile duct (CBD); common hepatic duct (CHD); intraoperative endoscopic evaluation (IEE); adhesive small bowel obstruction (ASBO); Control Group (CG); duodenogastric bile reflux (DGBR). Declarations Acknowledgments: We would like to express our gratitude to Professor Yamataka Atsuyuki (Full Professor of Pediatric Surgey. Juntendo University; Tokyo) and Professor Nicola De Angelis (Full Professor of General Surgey. University Paris Cite; Paris) for their suggestion in reviewing this study. 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Can J Surg 53(1):51–56 Ariake K, Unno M, Yoshida H (2020) Risk factors and characteristics of young patients with the biliary tract carcinoma: results of a project study for biliary surgery by the Japanese Society of Hepato-Biliary-Pancreatic Surgery. J Hepatobiliary Pancreat Sci 27(9):571–580 Tanaka H, Sasaki H, Wada M (2015) and e. al., "Postnatal management of prenatally diagnosed biliary cystic malformation," J Pediatr Surg, 50(4):507–510. Okada T, Sasaki F, Ueki S (2004) and e. al., "Postnatal management for prenatally diagnosed choledochal cysts," J Pediatr Surg., 39(7):1055–1058 Chen C (2003) Clinical and operative findings of choledochal cysts in neonates and infants differ from those in older children. Asian J Surg 26(4):213–217 Narayanan S, Chen Y, Narasimhan K (2013) and e. al., "Hepaticoduodenostomy versus hepaticojejunostomy after resection of choledochalcyst: a systematic review and meta-analysis.," J Pediatr Surg, 48(11):2336-42. Stringer M (2017) Laparoscopic management of choledochal cysts: is a keyhole view missing the big picture? Pediatr Surg Int 33(6):651–655 Shimotakahara A, Yamataka A, Yanai T (2005) Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy for biliary reconstruction during the surgical treatment of choledochal cyst: which is better? Pediatr Surg Int 21(1):5–7 Hamada Y, Hamada H, Shirai T (2017) and e. al., "Duodenogastric regurgitation in hepaticoduodenostomy after excision of congenital biliary dilatation (choledochal cyst).," J Pediatr Surg, 52(10):1621–1624. Tan H, Shankar K, Ford W (2003) Laparoscopic resection of type I choledochal cyst. " Surg Endosc 17(9):1495 Takada K, Hamada Y, Watanabe K (2005) and e. al., "Duodenogastric reflux following biliary reconstruction after excision of choledochal cyst.," Pediatr Surg Int, 21:1–4. Ten Hove A, de Meijer V, Hulscher J (2018) and e. al., "Meta-analysis of risk of developing malignancy in congenital choledochal malformation," Br J Surg., 105(5):482–490. He X, Wang L, Liu W (2014) and e. al., "The risk of carcinogenesis in congenital choledochal cyst patients: an analysis of 214 cases.," Ann Hepatol., 13(6):819 – 26. Todani T, Watanabe Y, Urushihara N (1994) and e. al., "Choledochal cyst, pancreatobiliary malunion, and cancer.," J Hepatobiliary Pancreat Surg, 1:247–251 Watanabe Y, Toki A, Todani T (1999) Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg 6:207–212 Ono S, Fumino S, Shimadera S (2010) and e. al., "Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up.," J Pediatr Surg, 45:376–378. Tajiri T, Tate G, Inagaki T (2004) Mucinous cystadenoma of the pancreas 17 years after excision of gallbladder because of a choledochal cyst. " J Gastroenterol 39(02):181–187 Ng D, Chiow A (2016) Poh and e. al., Metachronous cholangiocarcinoma 13 years post resection of choledochal cyst-is long-term follow-up useful? a case study and review of the literature. Surg Case Rep 2(1):60 Otani T, Shioiri T, Mishima H (2005) and e. al., "Bile duct schwannoma developed in the remnant choledochal cyst-a case associated with total agenesis of the dorsal pancreas," Dig Liver Dis, 37(09):705–708 Baba T, Mukai M, Masuya R (2019) The long-term health-related quality of life in patients operated for choledochal cyst. Pediatr Surg Int 35(5):539–546 Shen H, Xu M, Zhu H (2015) and e. al., "Laparoscopic versus open surgery in children with choledochal cysts: a meta-analysis.," Pediatr Surg Int, 31:529–534. Additional Declarations No competing interests reported. Supplementary Files COLEDOCOSCOPIADEFINITIVA.mp4 Cite Share Download PDF Status: Published Journal Publication published 19 Jan, 2024 Read the published version in Pediatric Surgery International → Version 1 posted Editorial decision: Revision requested 02 Dec, 2023 Reviews received at journal 28 Nov, 2023 Reviewers agreed at journal 21 Nov, 2023 Reviewers invited by journal 21 Nov, 2023 Editor assigned by journal 16 Nov, 2023 Submission checks completed at journal 16 Nov, 2023 First submitted to journal 15 Nov, 2023 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-3615808","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Method Article","associatedPublications":[],"authors":[{"id":250035264,"identity":"a85eba59-cba6-417b-82c7-555d9eaa1d55","order_by":0,"name":"Maria Vittoria Stern","email":"data:image/png;base64,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","orcid":"","institution":"“Spedali Civili” Children's Hospital","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Maria","middleName":"Vittoria","lastName":"Stern","suffix":""},{"id":250035266,"identity":"daa6796e-12bc-4608-81c7-19e144a70c3a","order_by":1,"name":"Giovanni Boroni Boroni","email":"","orcid":"","institution":"“Spedali Civili” Children's Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Giovanni","middleName":"Boroni","lastName":"Boroni","suffix":""},{"id":250035270,"identity":"34385cda-4032-44ee-93fd-c7c04eb867fe","order_by":2,"name":"Filippo Parolini","email":"","orcid":"","institution":"“Spedali Civili” Children's Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Filippo","middleName":"","lastName":"Parolini","suffix":""},{"id":250035272,"identity":"26feccef-26dd-4f64-8e4d-f5ab28c6dc6b","order_by":3,"name":"Fabio Torri","email":"","orcid":"","institution":"“Spedali Civili” Children's Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Fabio","middleName":"","lastName":"Torri","suffix":""},{"id":250035273,"identity":"afa5b8fa-f551-4c6d-a559-357b83aa73ea","order_by":4,"name":"Stefano Calza","email":"","orcid":"","institution":"“Spedali Civili” Children's Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Stefano","middleName":"","lastName":"Calza","suffix":""},{"id":250035274,"identity":"0bd62ad0-1c83-479d-8470-d6ae6361a560","order_by":5,"name":"Daniele Alberti","email":"","orcid":"","institution":"“Spedali Civili” Children's Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Daniele","middleName":"","lastName":"Alberti","suffix":""}],"badges":[],"createdAt":"2023-11-15 14:59:26","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-3615808/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-3615808/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1007/s00383-023-05622-8","type":"published","date":"2024-01-19T15:01:50+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":46806901,"identity":"f9da3152-31e3-4043-bd7b-51bda6eaac73","added_by":"auto","created_at":"2023-11-20 21:28:14","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":892656,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eDissection of CM\u003c/strong\u003e. We resected the CHD before beginning the CBD dissection, to ensure a safer dissection of its posterior wall from the portal vein and hepatic artery.\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-3615808/v1/6c2ad51e1c12f70c1ea11afd.png"},{"id":46806902,"identity":"2b6ae832-154a-48a2-bf63-8d3a3e312bf7","added_by":"auto","created_at":"2023-11-20 21:28:14","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":961312,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eDissection of CM.\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe aim was to remove the distal part of CBD next to the junction with the main pancreatic duct, avoiding leaving an excessively long choledochal stump. A: Cystic CM; B: Fusiform CM\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-3615808/v1/558367dc578658e9f64d3123.png"},{"id":49978845,"identity":"ddb2c2bf-dfb8-4d2c-a926-1218d5707431","added_by":"auto","created_at":"2024-01-22 15:09:34","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1975902,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-3615808/v1/7c26c896-f72c-4b6a-9437-d021ddcda8a0.pdf"},{"id":46806904,"identity":"48a92853-dab1-4b87-bac9-b9a9852b285b","added_by":"auto","created_at":"2023-11-20 21:28:15","extension":"mp4","order_by":1,"title":"","display":"","copyAsset":false,"role":"supplement","size":7677186,"visible":true,"origin":"","legend":"","description":"","filename":"COLEDOCOSCOPIADEFINITIVA.mp4","url":"https://assets-eu.researchsquare.com/files/rs-3615808/v1/9845ad2d8cd707aaba09b893.mp4"}],"financialInterests":"No competing interests reported.","formattedTitle":"\u003cp\u003eLong-term Outcome for Children Undergoing Open Hepatico-jejunostomy for Choledochal Malformations: a 43-year Single-center Experience\u003c/p\u003e","fulltext":[{"header":"Introduction","content":"\u003cp\u003eFormerly called choledochal cyst, Choledochal Malformations (CMs) have been more recently defined as congenital morphological abnormalities of the biliary tract characterized by abnormal dilatation (cystic or fusiform) of the choledochus in the absence of any mechanical obstruction [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e] [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e] [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. Most CM cases are associated with a Pancreatico-Biliary Maljunction (PBM) and a Long Bilio-Pancreatic Common Channel (LBPCC); cases with concomitant intrahepatic bile duct (IHBD) dilatation are included in this entity. [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e] [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eThe incidence of CMs shows significant geographical variation. In Western populations they are increasingly diagnosed in childhood probably due to a higher index of suspicion and improved imaging techniques. [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e] [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e] [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e] Regardless the time of presentation and diagnosis, early surgery is essential to mitigate potential damage to the liver and prevent severe complications and cancer. [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e] [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e] [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eOpen complete excision of the Dilated Part of the Extrahepatic Biliary Tree and restoration of intestinal bile drainage with a Roux-en-Y hepaticojejunal anastomosis (RHJ) is still the gold standard treatment [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e], even if minimally invasive approaches such as laparoscopic and robotic RHJ or hepatico-duodenostomy (HD) have gained popularity.[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e][\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e][\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e] .\u003c/p\u003e \u003cp\u003eIn this study, we present our 43-year single-center experience with a large cohort of children with CM managed using an open surgery approach with follow-up into adulthood. To the best of our knowledge, this is one of the largest pediatric single-center series and the first Italian study of CM pediatric patients with very long-term follow-up.\u003c/p\u003e"},{"header":"Material and Methods","content":"\u003cp\u003e\u003cem\u003eData Source and Study Population\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eAll consecutive pediatric patients (\u0026lt;18 years) with CM who underwent surgical treatment in our Tertiary care Pediatric Surgery Department between October 1980 and\u0026nbsp;December 2022\u0026nbsp;were enrolled in this retrospective study. Institutional Review Board approval was obtained (NP4246/2020).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained before all stages of the study.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eFor each patient, we collected demographic, clinical data, imaging results, information on surgical procedures, POCs and clinical outcomes.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eData\u0026nbsp;were retrieved from patient medical records, outpatient clinic check-ups, telephone, and email interviews. For patients reaching adulthood, follow-up data were supplemented by information from the records held by their general practitioners.\u003c/p\u003e\n\u003cp\u003eEven though diagnostic assessment and postoperative therapy varied through the period of the study, protocols were implemented to improve the standard of care.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003ePreoperative assessment included laboratory tests, abdominal ultrasound (US), abdominal Computed Tomography Scan (CT) and \u0026ndash; in the last 15 years \u0026ndash; Magnetic Resonance Cholangio-Pancreatography (MRCP) and Endoscopic Retrograde Cholangio-Pancreatography (ERCP).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eScheduled postoperative follow-up included blood tests and abdominal US initially at 1, 3, 6 months, and then at 12-month intervals for the first 5 years, every 2 years up to 10 years and subsequently every 5 years. In the last 4 years, we routinely added CA 19-9 to the laboratory exams.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eCMs were classified according to Todani classification.\u0026nbsp;\u0026nbsp;[13]\u003c/p\u003e\n\u003cp\u003eCholangitis was defined as presence of fever, abdominal pain with or without jaundice, increased levels of total and conjugated bilirubin and gamma glutamyl transferase. [14]\u0026nbsp;\u003c/p\u003e\n\u003cp\u003ePancreatitis was defined as presence of abdominal pain, plasma amylase levels over three times the upper limit of normal and a positive US. [15]\u003c/p\u003e\n\u003cp\u003ePseudopancreatitis was defined as plasma amylase levels over three times the upper limit of normal but with normal US.\u003c/p\u003e\n\u003cp\u003eSurgical complications were referred according to Clavien-Dindo Classification; Grades III and IV were major complications. [16]\u003c/p\u003e\n\u003cp\u003eLong term POCs were those occurring\u0026nbsp;at least 5 years after surgery.\u003c/p\u003e\n\u003cp\u003eQuality of life (QoL)\u0026nbsp;-\u0026nbsp;evaluated only for patients once they came into adulthood\u0026nbsp;-\u0026nbsp;was assessed with a 26-item self-reported Word Health Organization Quality of life questionnaire (WHOQOL Bref), evaluating the subjective perception of individual health in four areas: physical, psychological health, social relationships, and environment. [17]\u003c/p\u003e\n\u003cp\u003eThe results were compared with a control group (CG) of healthy people, composed by\u0026nbsp;medical students and other survey volunteers,\u0026nbsp;of the same age and sex, to whom the questionnaire was also administered. Twenty-one people were enrolled in the CG.\u0026nbsp;\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe questionnaire was sent by email and participants were asked to select from multiple choice answers. Informed consent was obtained from all participants.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eStatistical Analysis\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eQuantitave data were described as mean (standard deviation), median (IQR).\u0026nbsp; Median follow-up was estimated using Kaplan-Meier reverse estimator. Association between categorical data were estimated using\u0026nbsp;\u0026chi;2 test with Monte Carlo p-values computations or Fisher exact test, while quantitave variable were compared among groups using a Welch t-test for independent group. All tests were two sided and assumed a significance level of 5%.\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eSurgical procedure\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eOpen complete excision of the Dilated Part of the Extrahepatic Biliary Tree and RHJ is our surgical procedure of choice. During the first 10 years, the procedure involved a right subcostal incision, extended to the left rectus abdominis muscle. Later, we used a self-retaining retractor (Thompson, Thompson Surgical Instrument, MI), that enabled us to perform smaller incisions. A bile sample was taken from the gallbladder or from the CM for amylase dosage. Surgical dissection went from the gallbladder fundus down to the common bile duct (CBD); the common hepatic duct (CHD) was then dissected and encircled with an umbilical tape or a vessel loop, observing its position in relation to the right hepatic artery, because it can cross the duct both above and below it. \u0026nbsp;Since the 2000s, we have performed an intraoperative endoscopic evaluation (IEE) of the CM in all cases using a 7 or 7.5 Fr pediatric operating cystourethroscopy (Karl Storz GmbH \u0026amp; Co-KG, Tuttlingen, Germany). \u0026nbsp;The use of IEE improved the detection and cleaning of debris, stones, or biliary sludge from the IHBD, the distal choledochus and the LPBCC; any bile duct strictures at the hepatic hilum are detected. \u0026nbsp;Eventually IEE enabled us to visualize the outlet of the main pancreatic duct into the choledochus and explore the LBPCC. (\u003cstrong\u003eVideo 1\u003c/strong\u003e). We resected the CHD before beginning the CBD dissection, to ensure a safer dissection of its posterior wall from the portal vein and hepatic artery (\u003cstrong\u003eFig. 1\u003c/strong\u003e).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe aim was to remove the distal part of CBD next to the junction with the main pancreatic duct, avoiding leaving an excessively long choledochal stump (\u003cstrong\u003eFig. 2\u003c/strong\u003e). The Lilly technique for CM was adopted in case of bleeding during this step of surgery. \u0026nbsp;[18]\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;Open complete excision of the Dilated Part of the Extrahepatic Biliary Tree was followed by an end-to-side or end-to-end RHJ with a jejunal loop of 35-50 cm in length according to the patient age, fashioned with 4/0 or 5/0 Polyglycol in the first 10 years and later with 5/0 or 6/0 Polydioxanone suture. \u0026nbsp;Interrupted stitches, tied outside, were adopted when the CHD diameter was less than 1 cm, while two continuous hemisutures were used in all other cases. In case of end-to-side anastomosis, the ascending limb of the Roux-en-Y was opened as close as possible to the end of the blind jejunal pouch. When biliary stenosis was detected at hepatic hilum, biliary ductoplasty was performed with 7/0 polydioxanone interrupted stitches. Since 2000, we have added a 5-7 cm long jejunal \u0026ldquo;spur valve\u0026rdquo; approximating the native jejunum to the Roux-en-Y limb, to prevent the reflux of air and GI secretion into the biliary tree.\u003c/p\u003e"},{"header":"Results","content":"\u003cdiv id=\"Sec7\" class=\"Section2\"\u003e \u003ch2\u003ePopulation\u003c/h2\u003e \u003cp\u003eOne-hundred-thirteen patients with CM were managed at our department.\u003c/p\u003e \u003cp\u003eCM was suspected during prenatal US assessment in 9 patients (8%), at a median of 28 gestational weeks (range 27\u0026ndash;34). In the remaining 104 patients, the median age at diagnosis was 29 months (0.5\u0026ndash;190 months). Eighty-nine patients (78.7%) were symptomatic at time of the diagnosis: 73 (64.6%) presented jaundice and/or increased values of serum markers of obstructive jaundice; 28 patients (24.7%) were diagnosed with true pancreatitis or pseudopancreatitis. Fifteen patients (13.3%) presented non-specific symptoms (e.g., recurrent abdominal pain, nausea, vomiting).\u003c/p\u003e \u003cp\u003eIn 24 patients (21%), CM was an incidental US finding.\u003c/p\u003e \u003cp\u003eEpidemiological and Clinical data are summarized in Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eDemographic and clinical data\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"2\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients, n\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003e113\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSex (F)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e89 female (78.7%),\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAge at diagnosis, median (range)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e29 months (0-190)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAntenatal diagnosis, n (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e9 (8%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eGestational week, median (range)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e28 gestational week (27\u0026ndash;34)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eIncidental diagnosis (asymptomatic), n (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e24 (21%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eWeight (at surgery)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e13.8 kg (3.4\u0026ndash;49)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTodani Classification\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u0026middot; Todani I a :53\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u0026middot; Todani I b: 5\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u0026middot; Todani I c: 35\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u0026middot; Todani II: 1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u0026middot; Todani IV a: 16\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u0026middot; Todani IV b: 3\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec8\" class=\"Section2\"\u003e \u003ch2\u003eSurgical details\u003c/h2\u003e \u003cp\u003eAll patients underwent surgery at a median age of 39 months (0.5\u0026ndash;193), with a median weight of 13.8 kg (4\u0026ndash;49). Patients with antenatal diagnosis were operated, at a median age of 3 months (0.5\u0026ndash;32) (\u003cem\u003ep\u0026thinsp;=\u003c/em\u003e\u0026thinsp;0.002). Ten patients (8.8%) had undergone previous treatments at other hospitals: 2 had undergone a cholecysto-jejunostomy; 2 a cysto-jejunostomy; 2 a cholecystectomy; 1 an hepaticojejunostomy and 3 an ERCP with papillo-sphincterotomy.\u003c/p\u003e \u003cp\u003eAll the patients in our series underwent open complete excision of the Dilated Part of the Extrahepatic Biliary Tree followed by an RHJ.\u003c/p\u003e \u003cp\u003eLPBCC was detected in all patients. No patient had biliary carcinoma at the time of the operation.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec9\" class=\"Section2\"\u003e \u003ch2\u003eComplications and QoL\u003c/h2\u003e \u003cp\u003eSixty-eight patients (60%) completed a follow-up longer than 5 years, 45 (39.8%) completed a 10-year follow up and 28 (24.7%) a follow-up longer than 20 years and up to 42 years.\u003c/p\u003e \u003cp\u003eThe overall survival rate of our series was 100%.\u003c/p\u003e \u003cp\u003eIn the study period, major surgical complications (Clavien Dindo grade III-IV) occurred in 8 patients (7%). Seven of these 8 occurred in patients that underwent surgery before 2000. Since then, only 1 case of adhesive small bowel obstruction (ASBO) was encountered. (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eLong-term complications, treatment, outcomes and follow up\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"7\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c6\" colnum=\"6\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c7\" colnum=\"7\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMajor complications\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eN (%)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eTodani\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eAge at surgery\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eLong-term POC\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\"\u003e \u003cp\u003eTreatment\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c7\"\u003e \u003cp\u003eAge at Follow-up \u0026amp; Outcome\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eHJ stenosis with IHBD stones and cholangitis\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e2 (2.9%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 A\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e23rd POY\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eRedo HJ\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e27.4 yrs, alive and well, NLE\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 A\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e107 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e24th POY\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003ePTHC and HJ stenting\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e30.8 yrs, alive and well, NLE\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePancreatitis\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e \u003cp\u003e1 (1.5%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 A\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e16 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e14th POY\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eERCP with papillo- sphincterotomy and stone removal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e21.5 yrs, alive and well, NLE\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eASBO\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e2 (2.9%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 C\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e95 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e6th POY\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eOpen adhesiolysis\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e15.6 yrs, alive and well, NLE\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 A\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e7 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e8th POY\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eOpen adhesiolysis\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e26 yrs, alive and well, NLE\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eIntussusception\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e \u003cp\u003e1 (1.5%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 A\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e50 months\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e5th POY\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eOperative reduction\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e21 yrs, alive and well, NLE\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTotal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e \u003cp\u003e6 (8.8%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003e \u003cb\u003eLegend\u003c/b\u003e: POY: postoperative year; HJ: hepaticojejunostomy; IHBD: intrahepatic bile ducts; ASBO: adhesive small bowel obstruction; JJ: jejunojejunostomy; NLE: normal liver enzymes.\u003c/p\u003e \u003cp\u003eFocusing on patients with a follow up of at least 5 years, we observed 6 major complications (8.9%). Two patients (2.9%) developed stricture of the hepaticojejunal anastomosis (HJ) with cholangitis 23 and 24 years after the initial operation. One underwent successful open redo of the HJ and stone removal and the other underwent percutaneous transhepatic stone removal with lithotripsy and HJ stenting. One patient (1.5%) operated on when he was 3 years old, complained of recurrent pancreatitis with a pseudocyst formation 14 years after surgery. A stone was detected in the CBD stump. He underwent ERCP with papillo-sphincterotomy and stone removal followed by the insertion of a \u0026ldquo;pigtail\u0026rdquo; drain in the pancreatic pseudocyst. So far, 8 years after the endoscopic procedure, he is doing well with no recurrence of pancreatitis or stones. He was operated on before we routinely used IEE. Two patients (2.9%) with ASBO, and patient (1.5%) with an ileo-ileal intussusception underwent surgery respectively 6, 8 and 5 years after the CM treatment.\u003c/p\u003e \u003cp\u003eOne patient underwent an open redo of HJ anastomosis because of a major abdominal trauma with liver and bile duct injury caused by a car accident at the age of 45, 30 years after the first surgery. At present, at 35 years of follow-up, she is doing well with normal liver function tests and US.\u003c/p\u003e \u003cp\u003eWe did not find any relationship between type of CM, coded as I and IV, and HJ anastomotic stricture (Fisher exact test, p\u0026thinsp;=\u0026thinsp;1), and age at surgery (Welch t-test, IV vs I, difference \u0026minus;\u0026thinsp;6.39, CI\u003csub\u003e95%\u003c/sub\u003e -16.17; 28.95, p\u0026thinsp;=\u0026thinsp;0.57).\u003c/p\u003e \u003cp\u003eOne patient developed a non-Hodgkin Lymphoma 20 years after surgery. He\u0026rsquo;s currently disease free with normal liver function tests and liver US at follow-ups, 16 years after his lymphoma diagnosis and 36 years after surgery.\u003c/p\u003e \u003cp\u003eNo cases of hepatic cirrhosis, portal hypertension and malignant transformation were recorded. At the last follow-up, 61 patients (90%) are symptom free, 6 (9%) reported dyspepsia and/or 4 (6%) recurrent abdominal pain.\u003c/p\u003e \u003cp\u003eGiven that we decided to administer the questionnaire only to patients aged over 18 years, currently 82 patients (72%) fall into this subset; 60 of these did not answer and 1 was excluded because of severe mental retardation. The remaining 21 patients (25.6%) completed the questionnaire.\u003c/p\u003e \u003cp\u003eThe median follow-up for this subset of patients after surgery was 29.28 years (IQR, 24.34\u0026ndash;33.67, range\u0026thinsp;=\u0026thinsp;8-38.5).\u003c/p\u003e \u003cp\u003eTwenty-one people were enrolled in the CG. The mean age of the CG was 31.1 years (SD\u0026thinsp;=\u0026thinsp;6.78), median age 30 years (IQR\u0026thinsp;=\u0026thinsp;4, range\u0026thinsp;=\u0026thinsp;18\u0026ndash;52).\u003c/p\u003e \u003cp\u003eNo statistically significant differences were found between groups in term of sex (proportion of males 19.0% Cases in vs 23.8% CG, Fisher exact test, p\u0026thinsp;=\u0026thinsp;1) and age (CG vs Cases, Welch t-test, difference \u0026minus;\u0026thinsp;0.95, CI\u003csub\u003e95%\u003c/sub\u003e -5.81; 3.90, \u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.69).\u003c/p\u003e \u003cp\u003ePatients with CM achieved total scores comparable to the CG (Table\u0026nbsp;\u003cspan refid=\"Tab3\" class=\"InternalRef\"\u003e3\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab3\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 3\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eComparison of Quality of Life among CM patients and the Control Group\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"4\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eCM patients (n\u0026thinsp;=\u0026thinsp;21)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eControl Group (n\u0026thinsp;=\u0026thinsp;21)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003e\u003cem\u003ep\u003c/em\u003e value\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMales, n (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (19.0%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e5 (23.8%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAge\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMean (sd)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e31.6 (8.69)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e31.1 (6.78)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0.69\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMedian (IQR, range)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e31 (11, 18\u0026ndash;51)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e30 (4, 18\u0026ndash;52)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eQoL total score\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMean (sd)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e61.2 (5.73)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e60.7 (4.90)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0.77\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMedian (IQR, range)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e62 (8, 50\u0026ndash;70)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e60 (7, 49\u0026ndash;68)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eWithin the CM group, there were no statistically significant differences in QoL total score between patients who experienced surgical complications (N\u0026thinsp;=\u0026thinsp;2) and patients who did not (Welch t-test, long -term complications Yes vs No, difference 1.45 CI\u003csub\u003e95%\u003c/sub\u003e -4.54; 1.64, \u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.34).\u003c/p\u003e \u003cp\u003eSix women had a normal pregnancy and delivery, and all their children are in good health.\u003c/p\u003e \u003cp\u003eSeven patients (33%) (6 women and 1 man) declared themselves uncomfortable with their surgical scars, however, the QoL score of these patients was in any case comparable with the CG (Welch t-test, \u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.55).\u003c/p\u003e \u003c/div\u003e"},{"header":"Discussion","content":"\u003cp\u003eThe present study reports our 43-year single-center experience of 113 children with CM, for whom Open complete excision of the Dilated Part of the Extrahepatic Biliary Tree and RHJ provided excellent results even at a very long-term follow-up.\u003c/p\u003e \u003cp\u003eIn our series, we had a low rate of long-term POCs, such as HJ stricture (2.9%), IHBD stones (2.9%) cholangitis (2.9%), pancreatitis (1.5%) and biliary cancer (0%).\u003c/p\u003e \u003cp\u003eCholangitis and IHBD stones were observed only in patients with stricture of the HJ, and not related to ascending cholangitis due to a reflux of gastrointestinal secretions into the limb of the Roux-and-Y as far as the liver. We think that the early surgical refinements we adopted helped to keep the rate of POCs low.[\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e][\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e] The first strategy was to perform an IEE in order explore and clean the IHBD, the distal CBD and the LBPCC. IEE also facilitated the identification of the pancreatic-biliary junction to avoid leaving an excessively long CBD distal stump. Furthermore, any biliary stricture at hepatic hilum can be easily detected, better evaluated, and surgically treated at the same time.\u003c/p\u003e \u003cp\u003eThe second strategy \u0026minus; as suggested by some authors to reduce the incidence of ascending cholangitis in biliary atresia \u0026minus; was the construction of a spur valve to prevent reflux of gastrointestinal secretions into the Roux-and-Y ascending limb. [\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eThe final strategy is to fashion the HJ anastomosis very close to the blind pouch of the jejunal limb to avoid anastomotic stricture and/or a redundant blind loop that can lead to bile stasis and the onset of repeated cholangitis of the IHBD that leads to intrahepatic lithiasis. [\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e] [\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e] [\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eChronic inflammation of the bile duct is the high-risk factor for carcinogenesis. [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e] [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e] [\u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e] [\u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e] [\u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eTiming of surgical repair in patients with antenatal diagnosis is controversial. [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e] [\u003cspan citationid=\"CR28\" class=\"CitationRef\"\u003e28\u003c/span\u003e] [\u003cspan citationid=\"CR29\" class=\"CitationRef\"\u003e29\u003c/span\u003e] [\u003cspan citationid=\"CR30\" class=\"CitationRef\"\u003e30\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eNevertheless, in our experience and in accordance with the Japanese Society of Hepato-Biliary-Pancreatic Surgery [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e], we recommend early operation (3\u0026ndash;6 months) even in asymptomatic patients, to avoid the progression of liver fibrosis and reduce the possibility of complications such as cholangitis, biliary obstruction, and pancreatitis. [\u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eIn our series, of 9 patients with antenatal diagnosis, the surgical complication rate was 0%.\u003c/p\u003e \u003cp\u003eAmong authors, the debate continues about which techniques of biliary reconstruction should be preferred [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e] [\u003cspan citationid=\"CR31\" class=\"CitationRef\"\u003e31\u003c/span\u003e] [\u003cspan citationid=\"CR32\" class=\"CitationRef\"\u003e32\u003c/span\u003e] [\u003cspan citationid=\"CR33\" class=\"CitationRef\"\u003e33\u003c/span\u003e] [\u003cspan citationid=\"CR34\" class=\"CitationRef\"\u003e34\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eWith the increasing trend of MIS, HD has been widely preferred over HJ because it is easier to perform laparoscopically and can be completed without the need for extracorporeal enteric sutures [\u003cspan citationid=\"CR31\" class=\"CitationRef\"\u003e31\u003c/span\u003e] [\u003cspan citationid=\"CR35\" class=\"CitationRef\"\u003e35\u003c/span\u003e] [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. However, HD may predispose towards duodenogastric bile reflux (DGBR), gastritis and esophagitis. [\u003cspan citationid=\"CR31\" class=\"CitationRef\"\u003e31\u003c/span\u003e] Authors have shown a high risk of carcinoma in the residual stomach after a distal gastrectomy, secondary to exposure of the gastric mucosa to bile and pancreatic secretions due to DGBR [\u003cspan citationid=\"CR33\" class=\"CitationRef\"\u003e33\u003c/span\u003e]. Hamada et al, reported an incidence of DGBR in all his 17 patients who underwent HD; reflux was confirmed by endoscopic examination. Seven of these patients were subsequently converted with a new surgery to HJ at a mean of 13 years after the initial excisional surgery due to the presence of significant abdominal symptoms. [\u003cspan citationid=\"CR34\" class=\"CitationRef\"\u003e34\u003c/span\u003e]\u003c/p\u003e \u003cp\u003e We agree with several authors that any type of surgery that favors DGBR should be avoided in children because of a potential long-term risk of gastric cancer. [\u003cspan citationid=\"CR32\" class=\"CitationRef\"\u003e32\u003c/span\u003e] [\u003cspan citationid=\"CR33\" class=\"CitationRef\"\u003e33\u003c/span\u003e] [\u003cspan citationid=\"CR34\" class=\"CitationRef\"\u003e34\u003c/span\u003e] [\u003cspan citationid=\"CR36\" class=\"CitationRef\"\u003e36\u003c/span\u003e] Furthermore, Todani et al, after having detected a case of hilar adenocarcinoma secondary to HD, hypothesized that such reconstruction could also promote the reflux of bile and pancreatic juice in the biliary epithelium, which would then suffer chronic inflammation, responsible for malignant degeneration. Therefore, they abandoned this technique. [\u003cspan citationid=\"CR33\" class=\"CitationRef\"\u003e33\u003c/span\u003e] [\u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eIn this regard, there are two major advantages to HJ over HD: it enables the separation of the pancreatic juice from bile, allowing the latter to mix at the level of the intestinal lumen and consequently, along the length of the Roux limb, the risk of reflux into the bile ducts is reduced [\u003cspan citationid=\"CR37\" class=\"CitationRef\"\u003e37\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eSecondly, no bile reflux in the stomach is observed. Therefore, we believe that HD should no longer be performed in children with CM.\u003c/p\u003e \u003cp\u003eIn addition, another risk factors for the occurrence of biliary cancer secondary to CM resection may include the incomplete excision of the CM or, less frequently, an undetected subclinical disease at the time of the first operation. [\u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e] [\u003cspan citationid=\"CR38\" class=\"CitationRef\"\u003e38\u003c/span\u003e] [\u003cspan citationid=\"CR39\" class=\"CitationRef\"\u003e39\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eComplete excision of the CM \u0026minus; including the need to leave the choledochal stump as short as possible \u0026minus; is critical because it allows the removal of the portion most frequently affected by cancer. [\u003cspan citationid=\"CR39\" class=\"CitationRef\"\u003e39\u003c/span\u003e] [\u003cspan citationid=\"CR40\" class=\"CitationRef\"\u003e40\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eOur exclusive pediatric series had no incidence of biliary cancer even at follow-up beyond 40 years. However other studies have demonstrated a non-negligible risk of biliary cancer. [\u003cspan citationid=\"CR37\" class=\"CitationRef\"\u003e37\u003c/span\u003e] [\u003cspan citationid=\"CR40\" class=\"CitationRef\"\u003e40\u003c/span\u003e] [\u003cspan citationid=\"CR41\" class=\"CitationRef\"\u003e41\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eOther recent data suggest a lifelong elevated risk of up to 4% of cancer in patients who underwent CM excision in pediatric age [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. The most frequently described type of tumor is cholangiocarcinoma. [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e] [\u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e] However, rare cases of mucinous cystadenoma, metachronous cholangiocarcinoma and bile duct schwannoma have been reported after the first operation. [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e] [\u003cspan citationid=\"CR42\" class=\"CitationRef\"\u003e42\u003c/span\u003e] [\u003cspan citationid=\"CR43\" class=\"CitationRef\"\u003e43\u003c/span\u003e] [\u003cspan citationid=\"CR44\" class=\"CitationRef\"\u003e44\u003c/span\u003e]. Tumors were reported primarily in Asian series, where the incidence of CM is higher. Patients were all adults with an age range of 18 to 60 years. [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e] [\u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e] [\u003cspan citationid=\"CR43\" class=\"CitationRef\"\u003e43\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eConsidering this evolution, it is mandatory that, not only pediatric surgeons, but also general surgeons be aware of this pathology and its implications in adult life. We therefore agree with authors who argue that lifelong follow-up should be mandatory.[\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eFurthermore, according to our experience, an additional point that supports the argument for continuing long-life follow-up is the long timespan between the original surgery and the development of stricture of the bilio-digestive anastomosis.\u003c/p\u003e \u003cp\u003eSeveral publications on pediatric CMs in recent years have concerned the laparoscopic approach, but relatively few data are available on the long-term outcome with only a few papers reporting follow-up longer than 5 years [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e] [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e] [\u003cspan citationid=\"CR31\" class=\"CitationRef\"\u003e31\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eIn some cases, the long-term outcomes of open surgery are being overshadowed by the cosmetic benefits and potential short-term advantages of the laparoscopic approach. [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e] [\u003cspan citationid=\"CR32\" class=\"CitationRef\"\u003e32\u003c/span\u003e] [\u003cspan citationid=\"CR45\" class=\"CitationRef\"\u003e45\u003c/span\u003e] [\u003cspan citationid=\"CR46\" class=\"CitationRef\"\u003e46\u003c/span\u003e] [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eHowever, concern arises because it seems that laparoscopic approach may lead, in case where inflammations is present, to an incomplete excision of the dilated CBD, leaving an excessively long choledochal stump. [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eThis would increase the formation of stones and possibly the later development of cancer in the stump, besides pancreatitis.\u003c/p\u003e \u003cp\u003eSo, although MIS has been reported to be a feasible and safe surgical option [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e] [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e] [\u003cspan citationid=\"CR32\" class=\"CitationRef\"\u003e32\u003c/span\u003e] [\u003cspan citationid=\"CR45\" class=\"CitationRef\"\u003e45\u003c/span\u003e] [\u003cspan citationid=\"CR46\" class=\"CitationRef\"\u003e46\u003c/span\u003e], care should be taken before dispensing with standard open techniques, which have proven minimal complications and long-term benefits.\u003c/p\u003e \u003cp\u003eThe laparoscopic approach in children with CM, can be reserved for favourable cases only (adequate body weight, size of the hepatic duct at the level of biliodigestive anastomosis, absence of previous complications and no need of biliary ductoplasty) by surgeons trained in open and MIS.\u003c/p\u003e \u003cp\u003eIt is likely that robotic surgery is going to overcome all these issues, by adopting excellent magnification, 3D visions and easier and more precise and distally extended CBD dissection. Robotics can also improve HJ fashioning and enable a wide HJ to be performed using the hilar portion of the proximal hepatic duct.\u003c/p\u003e \u003cp\u003eOur adult patients who answered the QoL questionnaire achieved scores that are comparable to controls. In our study, adverse long-term QoL concerns does not seem to be related to surgical complications. This is in contrast with Baba et al, whose study concluded that only patients without surgical complications have a QoL comparable with controls. [\u003cspan citationid=\"CR45\" class=\"CitationRef\"\u003e45\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eOne possible explanation could be the lower rate of POCs occurring in our series.\u003c/p\u003e \u003cp\u003eThe only complaint from our patients was surgical scarring: 7 out of 21 (33%) patients stated that they feel different and embarrassed about taking off their clothes in the locker room or on the beach.\u003c/p\u003e \u003cp\u003eSince 2015, we offer a plastic surgeon consultation for teenager and adult patients with non-cosmetic surgical scarring.\u003c/p\u003e \u003cp\u003eWe are aware that our study presented some inherent limitations.\u003c/p\u003e \u003cp\u003eFirstly, the retrospective nature of the study implies that some patients were lost during follow-up. Similarly, the low collection rate of questionnaires from patients who are now adults could undermine the efficacy of the QoL evaluation. Some of the oldest patient\u0026rsquo;s data have been lost due to the lack of digital storage, resulting in the impossibility to recover contacts.\u003c/p\u003e \u003cp\u003eEventually, the loss of some information is due to the absence of a precise transitional protocol from pediatric to the adult care. Moreover, the long period of the study implies variation in diagnostic assessment, surgical expertise, and postoperative follow-up. Not surprisingly, the highest rate of surgical complications occurred in the first decades of the study.\u003c/p\u003e \u003cp\u003eDespite these limitations, our 42-year experience suggests that open complete excision of the Dilated Part of the Extrahepatic Biliary Tree with RHJ offers excellent results in terms of few POCs even at very long-term follow-up.\u003c/p\u003e \u003cp\u003eHowever, we are aware and convinced that international cooperation is required to define a precise follow-up protocol when pediatric patients reach adulthood to investigate and early detect long-term complications, especially the occurrence of biliary cancer.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003eCholedochal Malformations (CMs); quality of life (QoL); postoperative complications (POCs); hepaticojejunostomy (HJ); Roux-en-Y hepaticojejunal anastomosis (RHJ); \u0026nbsp;Pancreatico-Biliary Maljunction (PBM); \u0026nbsp;Long Bilio-Pancreatic Common Channel (LBPCC); intrahepatic bile duct (IHBD); hepatico-duodenostomy (HD); ultrasound (US); Computed Tomography Scan (CT); Magnetic Resonance Cholangio-Pancreatography (MRCP); Endoscopic Retrograde Cholangio-Pancreatography (ERCP); common bile duct (CBD); common hepatic duct (CHD); intraoperative endoscopic evaluation (IEE); adhesive small bowel obstruction (ASBO); Control Group (CG); duodenogastric bile reflux (DGBR).\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eAcknowledgments:\u0026nbsp;\u003c/strong\u003eWe would like to express our gratitude to Professor Yamataka Atsuyuki (Full Professor of Pediatric Surgey. Juntendo University; Tokyo) and Professor Nicola De Angelis (Full Professor of General Surgey. University Paris Cite; Paris) for their suggestion in reviewing this study.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n \u003cli\u003e\u003cspan\u003eSoares K, Arnaoutakis D, Kamel I (2014) and e. al., \u0026quot;Choledochal cysts: presentation, clinical differentiation, and management,\u0026quot; J Am Coll Surg, 219(6):1167-80.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eParolini F, Davenport M (2018) Choledochal malformation: terminology and aetiology. Ped Surg Int 34(1):117\u0026ndash;118\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eAtkinson J, Davenport M (2014) Controversies in choledochal malformation. S Afr Med J 104(11 Pt 2):816\u0026ndash;819\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eIshibashi H, Shimada M (2017) Kamisawa and e. a. 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Pediatr Surg Int 21(1):5\u0026ndash;7\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eHamada Y, Hamada H, Shirai T (2017) and e. al., \u0026quot;Duodenogastric regurgitation in hepaticoduodenostomy after excision of congenital biliary dilatation (choledochal cyst).,\u0026quot; J Pediatr Surg, 52(10):1621\u0026ndash;1624.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eTan H, Shankar K, Ford W (2003) Laparoscopic resection of type I choledochal cyst. \u0026quot; Surg Endosc 17(9):1495\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eTakada K, Hamada Y, Watanabe K (2005) and e. al., \u0026quot;Duodenogastric reflux following biliary reconstruction after excision of choledochal cyst.,\u0026quot; Pediatr Surg Int, 21:1\u0026ndash;4.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eTen Hove A, de Meijer V, Hulscher J (2018) and e. al., \u0026quot;Meta-analysis of risk of developing malignancy in congenital choledochal malformation,\u0026quot; Br J Surg., 105(5):482\u0026ndash;490.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eHe X, Wang L, Liu W (2014) and e. al., \u0026quot;The risk of carcinogenesis in congenital choledochal cyst patients: an analysis of 214 cases.,\u0026quot; Ann Hepatol., 13(6):819 \u0026ndash; 26.\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eTodani T, Watanabe Y, Urushihara N (1994) and e. al., \u0026quot;Choledochal cyst, pancreatobiliary malunion, and cancer.,\u0026quot; J Hepatobiliary Pancreat Surg, 1:247\u0026ndash;251\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eWatanabe Y, Toki A, Todani T (1999) Bile duct cancer developed after cyst excision for choledochal cyst. 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Surg Case Rep 2(1):60\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eOtani T, Shioiri T, Mishima H (2005) and e. al., \u0026quot;Bile duct schwannoma developed in the remnant choledochal cyst-a case associated with total agenesis of the dorsal pancreas,\u0026quot; Dig Liver Dis, 37(09):705\u0026ndash;708\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eBaba T, Mukai M, Masuya R (2019) The long-term health-related quality of life in patients operated for choledochal cyst. Pediatr Surg Int 35(5):539\u0026ndash;546\u003c/span\u003e\u003c/li\u003e\n \u003cli\u003e\u003cspan\u003eShen H, Xu M, Zhu H (2015) and e. al., \u0026quot;Laparoscopic versus open surgery in children with choledochal cysts: a meta-analysis.,\u0026quot; Pediatr Surg Int, 31:529\u0026ndash;534.\u003c/span\u003e\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"pediatric-surgery-international","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pesi","sideBox":"Learn more about [Pediatric Surgery International](http://link.springer.com/journal/383)","snPcode":"383","submissionUrl":"https://submission.nature.com/new-submission/383/3","title":"Pediatric Surgery International","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"choledochal malformation, long term follow up, Roux-en-Y hepaticojejunostomy, quality of life","lastPublishedDoi":"10.21203/rs.3.rs-3615808/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-3615808/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003ePurpose\u003c/h2\u003e \u003cp\u003eTo report on our 43-year single-center experience with children operated on for Choledochal Malformations (CMs), focusing on long term results and quality of life (QoL).\u003c/p\u003e\u003ch2\u003eMaterials and Methods\u003c/h2\u003e \u003cp\u003eAll consecutive pediatric patients with CMs who underwent surgical treatment at our center between October 1980 and December 2022 were enrolled in this retrospective study. We focused on long-term postoperative complications (POCs), considered to be complications arising at least 5 years after surgery. We analyzed QoL status once patients reached adulthood, comparing the results with a control group of the same age and sex.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e \u003cp\u003e113 patients underwent open excision of CMs with a Roux-en-Y hepaticojejunostomy (HJ). The median follow-up was 8.95 years (IQR: 3.74\u0026ndash;24.41). Major long-term POCs occurred in six patients (8.9%), with a median presentation of 11 years after surgery. The oldest patient is currently 51. No cases of biliary malignancy were detected. The QoL of our patients was comparable with the control group.\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e \u003cp\u003eOur experience suggests that open complete excision of CMs with HJ achieves excellent results in terms of long-term postoperative outcomes. However, since the most severe complications can occur many years after surgery, international cooperation is advisable to define a precise transitional care follow-up protocol.\u003c/p\u003e","manuscriptTitle":"Long-term Outcome for Children Undergoing Open Hepatico-jejunostomy for Choledochal Malformations: a 43-year Single-center Experience","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2023-11-20 21:28:10","doi":"10.21203/rs.3.rs-3615808/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2023-12-02T11:04:47+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2023-11-28T21:13:58+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"58c79602-6814-4e6e-b11e-9a1218f4cea1","date":"2023-11-21T11:50:41+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2023-11-21T11:43:05+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2023-11-16T20:31:27+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2023-11-16T14:07:34+00:00","index":"","fulltext":""},{"type":"submitted","content":"Pediatric Surgery International","date":"2023-11-15T14:57:31+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"pediatric-surgery-international","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pesi","sideBox":"Learn more about [Pediatric Surgery International](http://link.springer.com/journal/383)","snPcode":"383","submissionUrl":"https://submission.nature.com/new-submission/383/3","title":"Pediatric Surgery International","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"2bb88d2f-8cff-4168-8284-086abc852d9c","owner":[],"postedDate":"November 20th, 2023","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2024-01-22T15:05:47+00:00","versionOfRecord":{"articleIdentity":"rs-3615808","link":"https://doi.org/10.1007/s00383-023-05622-8","journal":{"identity":"pediatric-surgery-international","isVorOnly":false,"title":"Pediatric Surgery International"},"publishedOn":"2024-01-19 15:01:50","publishedOnDateReadable":"January 19th, 2024"},"versionCreatedAt":"2023-11-20 21:28:10","video":"","vorDoi":"10.1007/s00383-023-05622-8","vorDoiUrl":"https://doi.org/10.1007/s00383-023-05622-8","workflowStages":[]},"version":"v1","identity":"rs-3615808","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-3615808","identity":"rs-3615808","version":["v1"]},"buildId":"7rjqhiLT3MXkJMwkYKINL","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}