Perforation Of Choledochal Cyst Research Articles

A nomogram for predicting choledochal cyst with perforation

BackgroundCholedochal cyst with perforation (CC with perforation) rarely occurs, early diagnosis and timely treatment plan are crucial for the treatment of CC with perforation. This study aims to forecast the occurrence of CC with perforation.MethodsAll 1111 patients were conducted, who underwent surgery for choledochal cyst at our hospital from January 2011 to October 2022. We conducted univariate and multivariate logistic regression analysis to screen for independent predictive factors for predicting CC with perforation, upon which established a nomogram. The predictive performance of the nomogram was evaluated using receiver operating characteristic (ROC) curves, calibration plots, and decision curve analysis (DCA) curves.ResultsThe age of children with choledochal cyst perforation is mainly concentrated between 1 and 3 years old. Logistic regression analysis indicates that age, alanine aminotransferase, glutamyl transpeptidase, C-reactive protein, vomiting, jaundice, abdominal distension, and diarrhea are associated with predicting the occurrence of choledochal cyst perforation. ROC curves, calibration plots, and DCA curve analysis curves demonstrate that the nomogram has great discriminative ability and calibration, as well as significant clinical utility.ConclusionThe age of CC with perforation is mainly concentrated between 1 and 3 years old. A nomogram for predicting the perforation of choledochal cyst was established.

Cholangiocarcinoma identified in perforated choledochal cyst in a 3-year-old boy

Cholangiocarcinoma in patients with Choledochal cysts is rare in childhood; however, it seriously affects the prognosis of the disease. The key to addressing this situation lies in completely removing the extrahepatic cyst. We herein present a case report of a 3-year-old boy with cholangiocarcinoma associated with a choledochal cyst (CDC). Preoperative 3D simulation, based on CT data, played an important role in the treatment of this patient.

Surgical outcomes of robotic-assisted cyst excisions and hepaticojejunostomies in patients with perforated choledochal cysts: a single-centerretrospective study.

Perforated choledochal cysts is usually treated by open approach through a one- or two-stage procedure. Laparoscopic procedures are gradually being used more commonly with advancements in minimally invasive technology, while the level of difficulty and conversion rate are still high due to adhesions and deranged anatomy. Robot-assisted choledochal cyst excisions have been proposed as another minimally invasive procedure that is thought to improve operability and precision compared with laparoscopic surgery. However, there is still a lack about the use of robotic assistance for bilio-enteric reconstruction in pediatric patients with perforated choledochal cyst excisions. The aim of this current study was to investigate the safety and effectiveness of robotic-assisted procedures in pediatric perforated choledochal cyst excisions. Patients suffering from perforated choledochal cysts and treated with surgical procedures including open, laparoscopic, and robotic procedures between January 2009 and December 2021 were retrospectively analyzed. Robotic cyst excisions and hepaticojejunostomies were mainly used to a one-stage procedure (the pseudocyst formation or intra-mural perforation) or in stage 2 of two-stage procedures (complete perforation). Data collection included patient characteristics, intraoperative outcomes, and postoperative complications. There were a total of 64 patients suffering from perforated choledochal cysts were treated at our institution. Thirty-one cases of cyst excisions and hepaticojejunostomies were completed by open procedures. Twenty-two cases of cyst excisions and hepaticojejunostomies were completed by laparoscopic procedures and 11 cases were completed by robotic procedures. The operating times were significantly longer in the laparoscopic procedures group (214.32 ± 51.33min) than found with either the open procedures group (130.55 ± 10.51min) or the robot-assisted procedures group (188.82 ± 16.55min) (p < 0.001). The time to oral intake, total complication, and hospital stay were similar among all three groups (3.53 ± 0.28days vs 3.47 ± 0.30days vs 3.46 ± 0.29days, 19.35% vs 27.27% vs 18.18%, 11.48 ± 1.29days vs 11.95 ± 2.75days vs 11.55 ± 2.38days, respectively) (p > 0.05). The number of biliary complications was higher in the laparoscopic procedures group (18.18%) than in both the open procedures and robot-assisted procedures groups (0.00%) (p = 0.016). Robotic-assisted cyst excision and hepaticojejunostomy procedures in patients with perforated choledochal cysts are both safe and feasible. What is more, they can achieve the same results as open procedures and also reduce the level of difficulty of operations and bring fewer biliary complications compared with laparoscopic procedures.

Multimodal imaging findings of perforated choledochal cyst with biliary lithiasis and secondary pancreatitis in a pediatric patient: A case report and literature review

Multimodal imaging findings of perforated choledochal cyst with biliary lithiasis and secondary pancreatitis in a pediatric patient: A case report and literature review

Value of serum GGT level in the timing of diagnosis of choledochal cyst perforation.

BackgroundCholedochal cyst perforation is extremely rare, and early diagnosis or prediction is important for the immediate therapeutic intervention of perforations. This study aimed to define the predictor(s) of an impending or complete spontaneous perforation of choledochal cyst and establish the optimal operative timing.MethodsAll 429 consecutive choledochal cyst patients from January 2015 to December 2021, were included. A retrospective study was performed based on Kaplan-Meier analysis, and Cox univariate and multivariate analyses.ResultsA total of 429 patients were included, among which, 21 had choledochal cyst perforations (group A), and 408 did not (group B). Compared to group B, the serum alanine aminotransferase, aspartate aminotransferase, direct bilirubin, gamma-glutamyl transpeptidase, indirect bilirubin, total bilirubin, and alkaline phosphatase were significantly higher in group A (p = 0.025, 0.006, < 0.0001, 0.0001, 0.001, < 0.0001, and 0.033). High serum gamma-glutamyl transpeptidase was negatively associated with perforation-free preoperative survival, and multivariate Cox regression revealed that serum gamma-glutamyl transpeptidase was an independent predictive factor for an impending or complete perforation (p = 0.042).ConclusionsA gamma-glutamyl transpeptidase level ≥ 346.5 U/L accompanied with significantly elevated liver enzymes and bilirubin levels was indicative of the possibility of an impending or complete choledochal cyst perforation, and a proactive surgical approach should be considered.

Comparison of Clinical and Radiologic Findings Between Perforated and Non-Perforated Choledochal Cysts in Children.

ObjectiveTo compare the clinical and radiologic findings between perforated and non-perforated choledochal cysts in children.Materials and MethodsFourteen patients (mean age ± standard deviation, 1.7 ± 1.2 years) with perforated choledochal cysts (perforated group) and 204 patients (3.6 ± 3.8 years) with non-perforated choledochal cysts (non-perforated group) were included between 2000 and 2019. All patients underwent choledochal cyst excision after ultrasound, CT, or MR cholangiopancreatography. Relevant data including demographics, clinical symptoms, laboratory findings, imaging findings, and outcomes were analyzed. Statistical differences were compared using the Mann-Whitney U test and Fisher’s exact test.ResultsCholedochal cyst perforation occurred only in children under the age of 4 years. Acute symptoms, including fever (p < 0.001), were more common in the perforated group than in the non-perforated group. High levels of white blood cells (p = 0.004), C-reactive protein (p < 0.001), and serum amylase (p = 0.002), and low levels of albumin (p < 0.001) were significantly associated with the perforated group. All 14 patients with perforated choledochal cysts had ascites, whereas only 16% (33/204) of patients in the non-perforated group had ascites (p < 0.001). In the subgroup of patients who had ascites, a large amount of ascites (p = 0.001), increase in the amount of ascites in a short time (p < 0.001), complex ascites (p < 0.001), and perihepatic pseudocysts (p < 0.001) were more common in the perforated group than in the non-perforated group.ConclusionChildren with perforated choledochal cysts have characteristic clinical and radiologic findings compared to those with non-perforated choledochal cysts. In young children with choledochal cysts, perforation should be differentiated in cases with acute symptoms, laboratory abnormalities, and characteristic ascites findings.

Choledochal cyst perforation: experience from a centre with limited emergency resources

Spontaneous perforation in Choledochal cyst (CDC) is a very rare initial manifestation and more commonly seen beyond infantile age. The management is challenging due to acute presentation, poor general condition of the patient and inflamed tissue that may jeopardize the appropriateness of any surgical intervention. The aim of the study was to analyse this rarity depending on clinical findings, diagnostic difficulty and optimum management plan in a limited resource set-up. It was a retrospective observational study where five patients presenting to the casualty department with biliary peritonitis between January 2015 and December 2020 were included. They were analysed with respect to symptomatology, laboratory parameters, radiology, emergency intervention with findings and definitive management plan. A female preponderance (60%) was found. Mean age was 5.4 years. One was a known case of CDC. Abdominal pain was the most common symptom. Inflammatory markers like Total leucocyte count (TLC) and Erythrocyte sedimentation rate (ESR) were raised in all (100%). Lipase was raised in 40% (2/5). Anaemia and low serum albumin were non-specific findings. A dilated Common bile duct (CBD) on Ultrasound (US) was seen in 80% (4/5). Magnetic resonance cholangio pancreaticography (MRCP) demonstrated type I CDC in all. All underwent laparotomy with lavage and external drainage followed by interval definitive surgery. Pre-operative diagnosis of a perforated CDC may not be possible. Strong clinical suspicion and bilious peritoneal fluid may point to this rare complication. Minimum exploratory manoeuvre with good lavage and external drainage should be the optimum emergency intervention. Definitive bilio-pancreatic reconstruction should be performed when tissue oedema subsides and general condition is improved. This approach usually achieves a rewarding outcome.

Short- and intermediate-term evaluation of the initial definitive operation for perforated choledochal cysts compared to two-stage management.

Recently, single definitive hepaticojejunostomy has been proposed to replace two-stage procedures, including initial urgent biliary drainage and final biliary reconstruction, with a reduced hospitalization time and corresponding overall hospitalization cost. We aimed to investigate the safety and efficacy of the initial definitive operation compared with the two-stage approach. The medical records of patients with perforated CDCs managed between 2010 and 2017 were retrospectively reviewed. The qualified samples were divided according to a single definitive operation or two-stage approach. We conducted a comparison of the clinical characteristics, including surgical parameters, length of stay, and short- and intermediate-term complications. A total of 117 patients with perforated CDCs were reviewed, with 48 cases of single-stage management and 69 cases of two-stage management. No differences in the baseline characteristics between the two groups were found, such as age, sex distribution, ultrasound presentation, or laboratory findings. The initial definitive operation was associated with a lower total duration of drainage, including abdominal drainage and biliary drainage (p < 0.001), and rate of unplanned readmission (OR, 0.056; 95% CI 0.007-0.435; p < 0.001) than those who underwent two-stage management. Furthermore, the initial definitive operation significantly reduced the postoperative hospital stay (p < 0.001), and the overall hospitalization cost was accordingly reduced compared with two-stage management (p < 0.001). The initial single definitive operation was safe and effective for most of the patients with perforated CDCs when expertise was available. Therefore, a single definitive operation should be considered the treatment of choice for perforated CDCs.

Robotic Excision of Choledochal Cyst with Hepaticoduodenostomy (HD): Report of HD Technique, Initial Experience, and Early Outcome.

Minimal access surgical approach to choledochal cyst (CC) is becoming a standard of care in pediatric age group. Robotic-assisted excision of CC is increasingly being practiced at centers which have access to the system. We present our experience and technique of hepaticoduodenostomy (HD). Over all initial experience, short-term outcomes and complications are also presented and discussed. Patients with CC and undergoing robotic excision were retrospectively studied. Patients with active cholangitis, liver dysfunction, and perforated CC were excluded for robotic procedures. All included patients were preoperatively evaluated as per the defined protocol. They underwent excision of CC with HD. The duodenal anastomosis was done after limited mobilization and emphasis was laid on anastomosing the distal D2 part to the common hepatic duct. This prevents bile reflux into stomach. The follow-up evaluation was done for these patients. Hepatobiliary iminodiacetic acid (HIDA) scan for duodenogastric reflux (DGR) was done only if patients reported symptoms related to it. A total of 19 patients (10 females) were studied. The mean age was 84 months. Type 1b was present in 12 patients and the rest were type IVb. Complete cyst excision with HD was done in all patients except conversion to open in one patient. The mean surgical time was 170 ± 40 minutes with console time of 140 ± 20 minutes. Median follow-up duration is 2.5 years (range: 3.5-0.5 years). HIDA scan was done in five patients who had reported epigastric pain. Of these five, one patient had a positive DGR. He is on conservative management. Robot-assisted CC excision with HD is feasible as proven by the outcome of 19 patients presented in this series. HD is to be done away from pylorus in distal part of down curving D2. This particular step prevents DGR and is the most important point of technique in doing HD. The presented series is the first report of robotic excision of CC with HD. The robot is a facilitator for complex and difficult operations as CC excision and HD.

Type C Pancreaticobiliary Maljunction Is Associated With Perforated Choledochal Cyst in Children

Background: Perforation of a choledochal cyst (CC) is not rare, but the pathogenesis of spontaneous perforation has not been established. Pancreaticobiliary maljunction (PBM) is commonly seen in association with choledochal cyst. To explore the relationship between PBM and perforated CC, a retrospective study was conducted.Methods: We analyzed all the patients with CC who underwent surgery in our hospital from 2014.06.01 to 2018.12.31. All patients were divided into two groups: group 1 were patients with perforated CC, and group 2 were patients with non-perforated CC. We recalled all the patients records to identify types of PBM. PBM was divided into four types [(A) stenotic type, (B) non-stenotic type, (C) dilated channel type, and (D) complex type] according to the classification proposed by the Committee on Diagnostic Criteria of the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) in 2015.Results: There were 186 patients with CC in all, and 116 patients showed PBM. Twenty patients in group 1 and 96 patients in group 2. There was an extremely higher percentage of type C PBM in group 1 than in group 2 (60 and 17.7%, respectively). More fusiform dilatation cases were found in group 1 (70%) than in group 2 (58.3%). Also there were more type C PBM in fusiform cases and type A PBM were frequently seen in cystic cases (P < 0.01).Conclusions: We found that Type C PBM and fusiform common bile duct maybe relate to the perforation of choledochal cyst. Patients with type C PBM and fusiform common bile duct should be treated more proactively, preferably before they perforate.

Perforated Choledochal Cyst: Its Clinical Implications in Pediatric Patient.

PurposePerforation of choledochal cyst (CC) is a relatively rare clinical presentation in pediatric populations and difficult to predict preoperatively. We assess the clinical implications by comparing clinical parameters based on a single-center experience between perforated and nonperforated CC to facilitate the appropriate management for future interventions.MethodsA total of 92 cases of CC in pediatric patients (aged <18 years) who received surgical management between January 2003 and December 2018 at a Pusan National University Children's Hospital were reviewed. After screening the clinical features of perforated cases, we compared the demographic findings, clinical characteristics, and some laboratory results between the perforated and nonperforated groups.ResultsPerforated CC was identified in 8 patients (8.7%), and nonperforated CC in 84 patients (91.3%). Perforation can be classified into three categories: free perforation of cyst (3 cases), pinpoint perforation of cyst (2 cases), and necrotic change of cyst (3 cases). CC perforation occurred significantly more commonly in patients aged <24 months. Clinically, the perforated group showed significantly higher frequency of fever and higher C-reactive protein (CRP) level during the initial visit.ConclusionPerforation is more likely to be suspected in patients aged <24 months presenting together with fever and high CRP level in the initial visit. It is also necessary to keep in mind that it indicates not only a possibility of complicated disease status regardless of its association with stones but also a difficulty of applying a minimal invasive procedure and relatively increased length of hospital stay.

Timing of Choledochal Cyst Perforation.

Timing of Choledochal Cyst Perforation.

Case Report: Spontaneous perforation of choledochal cyst in an infant: Successful management in a centre with limited means

Background: Biliary peritonitis due to a ruptured choledochal cyst (CC) is a rare occurrence. The difference between bile duct perforation (BDP) and ruptured choledochal cysts continues to be a matter of debate. Simple drainage, T tube placement and cholecystostomy have been proposed as the initial treatment of choice. Definitive surgery in the form excision of the CC and hepatico-enterostomy has been described as the ideal treatment option. We report a successful management of a unique case of perforated choledochal cyst in an infant who presented with biliary peritonitis. Case report: An 8 months old female child presented with biliary peritonitis as result of spontaneous perforation of a choledochal cyst. The patient was successfully managed initially by placement of T tube in the perforated cyst followed by a T tube cholangiogram. Definitive surgery was performed 5 weeks after the initial surgery in which cyst was excised and hepatico-duodenostomy was performed. The child is currently in follow up and doing well. Conclusion: Perforated CC can present as acute abdomen sometimes having only subtle signs. In absence of any previous established diagnosis of CC and trained radiological support the condition becomes challenging to diagnose preoperatively. External T-tube drainage followed by T-tube cholangiogram can help in delineating the anatomy. Cyst excision along with hepaticoportoentersomy remains the gold standard definitive treatment.

Single-incision laparoscopic hepaticojejunostomy for children with perforated choledochal cysts.

Conventionally, perforated choledochal cyst (CDC) is a contraindication of laparoscopic treatment. The current study is to evaluate efficacy of single-incision laparoscopic hepaticojejunostomy (SILH) in children with perforated CDCs. One hundred and thirty-three children with perforated CDCs who underwent SILHs in our hospital between August 2011 and August 2017 were reviewed. Fifteen (11.3%) patients were converted to open procedures due to severe adhesions and oozing. The mean age at SILH was 2.09years (range 2days-12.37years). The average operative time was 3.23h (range 2-5h). The mean postoperative hospital stay was 6.25days (range 4-16days). The mean time to full diet resumption was 2.18days (range 2-6days). The mean duration of drainage was 3.71days (range 3-10days). The median follow-up period was 24months. Postoperative liver function tests and serum amylase levels returned to normal within 1year. Three (2.5%) patients required blood transfusions because of extensive oozing from intramural micro-vessels of CDCs. Two (1.7%) patients encountered duodenal injuries because of severe adhesions. The duodenum was repaired with double-layer 5-0 PDS running sutures. One (0.8%) patient with giant CDC had abdominal fluids because of extensive dissection of intrapancreatic segment of CDC. He recovered after 10days of drainage. None of patients had bile leak, anastomotic stenosis, cholangitis, intrahepatic reflux, pancreatic leak, pancreatic calculi formation, pancreatitis, Roux-loop obstruction, or adhesive intestinal obstruction. Single-incision laparoscopic hepaticojejunostomy is safe and effective for selected patients with perforated CDCs in experienced hands.

Single-stage operation for perforated choledochal cyst.

The option of either single- or two-staged cyst excision has been proposed for perforated choledochal cysts (CCs), but which of the two methods is more effective remains controversial. We examined the complications and short-term outcomes of single-stage excision of perforated and non-perforated CCs. The medical records of patients treated for CCs from 2003 to 2016 were retrospectively reviewed. Outcomes were compared between patients with perforated CCs (Group A) and non-perforated CCs (Group B). The operative time, intraoperative bleeding, length of stay, and postoperative complications were analyzed. Group A comprised 6 patients (2 males, 4 females; mean age, 29months), and Group B comprised 26 patients (2 males, 24 females; mean age, 41months). All patients underwent single-stage complete excision with Roux-en-Y hepaticojejunostomy. There were no significant differences in the operative time, bleeding, and/or length of stay. There were no operative deaths or complications such as anastomosis leakage or postoperative cholangitis, but a pancreatic fistula developed in one patient in Group A and two in Group B. Single-stage excision for a perforated CC is feasible if the patient's condition is stable. Treatment Study, LEVELIII.

Endoscopic biliary drainage as a bridging procedure to single-stage surgery for perforated choledochal cyst: a case report and review of the literature.

Choledochal cyst (CC)—a congenital anomaly of the bile duct—is rare. We report a 28-year-old woman complaining of epigastralgia who was transferred to our hospital. Physical examination revealed severe tenderness to abdominal palpation without symptoms of diffuse peritonitis. Urgent contrast-enhanced abdominal computed tomography indicated the dilated common bile duct (CBD) was perforated, with a presumed diagnosis of perforated CC. Endoscopic external biliary drainage was performed immediately as a bridging procedure to the definitive surgery. Additional evaluations confirmed a type IVa CC, according to Todani’s classification, but no signs of malignancy. Twenty-two days after biliary drainage, laparotomy was performed. A large cystic mass was found in the CBD with a perforated scar on the right-side wall. Because inflammation around the pancreas head was too severe to perform cyst excision safely, the patient underwent subtotal stomach-preserving pancreatoduodenectomy. The postoperative course was uneventful, and the patient was discharged on the 29th postoperative day. Pathologic examination of a specimen showed no malignancy, and the patient has remained well during the 3-year follow-up. Our experience with this case suggests that definitive single-stage surgery for perforated CC in an adult can be performed safely owing to external biliary drainage as a bridging procedure, if manifestation of diffuse peritonitis is not evident.

Laparoscopic single stage procedure for perforated choledochal cyst

Spontaneous perforation of choledochal cyst is a rare presentation. Complicated choledochal cysts are routinely managed by staged open surgical procedures for excision and reconstruction [1, 2]. Complex laparoscopic surgery in infants is an evolving field but no reports have been published describing a minimal invasive approach to the management of spontaneous perforation of choledochal cyst. We report a case of perforated choledochal cyst in a 12 week old, female which was managed by single stage laparoscopic resection of the cyst with biliary reconstruction. She presented with fever and bilious vomiting for 4 days. She had chemical pancreatitis and further work up showed her to have a perforated choledochal cyst. After conservative treatment of her pancreatitis, she underwent laparoscopic resection of the choledochal cyst with reconstruction. She had an uneventful postoperative recovery. This is the first ever reported case of laparoscopic management of a perforated choledochal cyst in infants which was managed by single stage laparoscopic surgery with immediate reconstruction.

Péritonite biliaire par rupture traumatique d’un kyste du cholédoque

Choledochal cysts are rare congenital malformations of the biliary tract. Traumatic rupture of a choledochal cyst can be misleading. An 11-year-old boy was admitted for peritonitis and intestinal occlusion after blunt abdominal trauma, evolving over 48 h. Laparotomy revealed bile ascites and a suspected duodenal perforation. After referral to our center, a CT scan showed a perforated choledochal cyst. Six months later, a complete excision of the cyst was successfully performed. This treatment is mandatory because of the risk of further complications such as lithiasis, pancreatitis, cholangitis, biliary cirrhosis, and malignant transformation (cholangiocarcinoma).

Spontaneous gangrene and perforation of choledochal cyst: a rare presentation

Choledochal cyst is a rare entity. It generally presents in children with pain, jaundice and the occasional finding of a lump in right hypochondriac region. A very rare presentation is biliary peritonitis resulting from a perforated choledochal cyst. It is usually seen in younger children. Our patient was a 14 year old female who presented with an acute abdomen. Clinically she had signs of peritonitis. Chest radiograph did not show free gas under diaphragm. The USG showed a dilated CBD of 4.1cm with a calculus at its lower end of 2 x 2.6cm, USG guided diagnostic tap revealed bile. Emergency exploration was done, a gangrenous patch of size 3 * 2cm in the anteromedial wall of large choledochal cyst was found with a perforation in it. No calculous was found. The gangrenous wall of choledochal cyst was excised and primary suturing of choledochal cyst was done with a T-tube placed through separate incision in choledochal cyst. As there is no facility for intra-operative cholangiogram in the emergency setting in our institute, a post-operative cholangiogram was done on day 7. It revealed a dilated CBD, CHD as well as right and left hepatic ducts (choledochal cyst type IA). The patient was discharged and asked to follow-up electively for excision of choledochal cyst at a later date. A rare possibility of a perforated choledochal cyst should be kept in mind in cases of biliary peritonitis, especially in younger age groups. Bile drainage would be a safer procedure in emergency condition, especially when patient presented late to hospital with hemodynamic unstability and edematous wall.

One-staged or two-staged surgery for perforated choledochal cyst with bile peritonitis in children? A single center experience with 27 cases

To report the authors' experience in the management of perforated choledochal cyst with bile peritonitis (PCC) in children. Medical records of all children undergoing surgery for PCC at our hospital from May, 2005 to January, 2013 were reviewed. Twenty seven patients were identified, with a median age of 2 years. The clinical manifestations were abdominal pain (96.3 %), abdominal distention (92.6 %), vomiting (88.9 %), jaundice (74.1 %), fever (70.4 %), acholic stool (51.8 %), abdominal tenderness (92.6 %), peritoneal signs (74.1 %), and palpable mass (18.5 %). Duration of the acute symptoms ranged from 1 to 30 days (median 3 days). Median size of the choledochal cysts was 3 cm (range 1.5-18 cm). Before January 2008, four cases were treated by two-staged surgery and only one case by one-staged surgery. Since then, all remaining 22 cases were treated by the one-staged surgery. There was no anastomotic leakage and all patients were discharged in good health. The mean postoperative stay was 7.6 ± 2.5 days for the one-staged group. No complication was noted at a median follow-up of 24 months. One-staged definitive repair is feasible, safe and should be the treatment of choice for most cases of childhood PCC in experienced centers.