Original Article
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Controversies in Choledochal Malformations: A Survey among Dutch Pediatric Surgeons Maria Hendrina Antoinette van den Eijnden1 Paul M. J. G. Peeters2 Jan B. F. Hulscher1
Ruben H. de Kleine2
1 Department of Pediatric Surgery, University Medical Centre
Groningen, Groningen, The Netherlands 2 Department of Hepatobiliary Surgery and Liver Transplantation, University Medical Center Groningen, Groningen, The Netherlands 3 Department of Paediatrics, University Medical Center Groningen, Groningen, The Netherlands 4 Department of Pediatric Surgery, Hôpitaux Universitaires de Genève, Geneva, Switzerland
Henkjan J. Verkade3
Jim C. H. Wilde4
Address for correspondence Jan B. F. Hulscher, MD, PhD, Department of Paediatric Surgery, University Medical Center Groningen, Hanzeplein 1, HPC BA20 Postbus 30.001, Groningen 9700 RB, The Netherlands (e-mail: [email protected]).
Abstract
Keywords
► choledochal malformation ► choledochal cysts ► controversies in choledochal malformation ► malformation
received June 2, 2014 accepted July 3, 2014 published online October 26, 2014
Background Choledochal malformation (CM) is a rare medical condition of which 80% are diagnosed in pediatric patients. There are several important controversies regarding diagnostic workup, management, and follow-up in these pediatric patients. To assess preferences and practices of Dutch pediatric surgeons regarding the diagnostic procedures, management, and follow-up of children with CM we conducted an electronic survey. Methods A questionnaire was sent to all the pediatric surgeons working in the academic centers and the only community hospital with a pediatric surgery service. The questionnaire included, items regarding incidence, diagnostic workup, interval between diagnosis and surgery, surgical techniques, and follow-up. We also assessed whether personal exposure influenced the preferences and practices. Results Overall 22 out of the 31 (71%) Dutch pediatric surgeons returned the questionnaire. Total 15 out of 22 (68%) encountered CM up to 2 times/y, whereas 7 out of 22 (32%) encountered it more than 2 times/y. Indications for surgery were significantly different between surgeons who encountered CM > 2 time/y versus those who did not: 6/6 (100%) of surgeons encountering CM > 2 times/y considered the presence of an asymptomatic CM an indication for surgery versus 5/14 (36%) of the pediatric surgeons who encountered a CM up to 2 times/y (p ¼ 0.01). Overall 12 out of the 22 (55%) respondents preferred surgery between 6 months and 2 years of age. The amount of exposure did not differ in preferred age at surgery or surgical technique. In the symptomatic child 10/22 (45%) of respondents preferred surgery within 3 months. Overall 7/22 (32%) favored laparoscopic resection. Hepaticojejunostomy with Roux-en-Y reconstruction was the preferred reconstruction for all the respondents. One-third stated that they never performed a parenchyma resection. Follow-up was limited to 10 years in almost half of the respondents. Conclusion Dutch pediatric surgeons demonstrate a wide variety of opinions regarding diagnostic workup, treatment, and follow-up of CM. While most surgeons encounter
© 2015 Georg Thieme Verlag KG Stuttgart · New York
DOI http://dx.doi.org/ 10.1055/s-0034-1387947. ISSN 0939-7248.
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Eur J Pediatr Surg 2015;25:441–448.
A Survey among Dutch Pediatric Surgeons for Controversies in CM
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CM up to 2 times/y, there is an association between exposure and several of the outcome parameters. Some of the answers are not in line with the expert opinion. This demonstrates that there is a need for evidence-based (inter)national guidelines regarding the diagnostic approach, management, and follow-up.
Introduction
Statistics
A choledochal malformation (CM) is a rare medical condition with an incidence of 100,000 to 150,000 live births in the Western population.1,2 Almost 80% of the CM is diagnosed before the age of 10 years.3 There are several important controversies regarding the diagnosis and management of CM in children. For instance, some favor a magnetic resonance cholangiopancreatography (MRCP), while others still prefer an endoscopic retrograde cholangiopancreatography (ERCP). Some favor laparoscopic resection, while others argue in the favor of an open approach.4,5 Some advocate early surgery, while others prefer to wait until the child is at least 6 months old before performing any surgery in the asymptomatic neonate.6,7 While there are expert opinions which are more or less accepted, evidencebased guidelines are lacking and outcome measures are not specified. This lack of an evidence-based nationwide guideline for the approach of CM might hamper the outcomes in both short and long term. To assess practices of the Dutch pediatric surgeons regarding the management of CM we have conducted an electronic survey on several controversial topics. We speculated that, given the rarity of this disease in combination with the lack of consensus regarding diagnostic and therapeutic strategies, different centers and different pediatric surgeons use different strategies. In this article, we do not attempt to provide answers to the controversies mentioned above, nor do we assess treatment outcomes. However, investigating nationwide preferences and practices can be the first step toward a common approach to a rare disease.
Methods Sampling The local medical ethics committee approved of this research. An electronic survey was sent to pediatric surgeons working in the six academic pediatric surgical centers and in the only Dutch community hospital with a pediatric surgery service. After 3 weeks a reminder was sent to the nonrespondents and after 6 weeks the questionnaire was closed. The survey was constructed by the authors.
Questionnaire Besides items regarding the frequency in which Dutch pediatric surgeons encounter CM, the questionnaire aimed to gain insight into the routine practices regarding diagnostic workup, preferred interval between diagnosis and surgery, surgical technique, and follow-up. European Journal of Pediatric Surgery
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Apart from descriptive statistics we tested associations between individual exposure and several of the outcome parameters. Percentages were calculated including the pediatric surgeons who did not answer all questions of the survey (this was considered the missing data) or answered “no opinion.” Nonresponders were not included in the analyses. The SPSS IBM 20 package (Armonk, New York, United States) was used for statistical analysis. To test the categorical variables Fisher exact test or chi-square statistics were used as appropriate.
Results Overall 22 (71%) of the 31 Dutch pediatric surgeons returned the questionnaire. Out of which 11 responded after the primary request and 11 after the reminder was sent. Two respondents did not answer all the questions.
Incidence and Diagnostic Workup The incidence of CM per surgeon and practices regarding the diagnostic workup are displayed in ►Table 1. A total of 68% (n ¼ 15) of the respondents encounter a CM up to 2 times/y and 32% (n ¼ 7) more than 2 times/y. The anatomical criteria when performing diagnostic workup on CM vary, with most surgeons looking for information regarding anatomical boundaries of CM, the possible presence of a common channel, and stenosis of the bile duct. Criteria for surgical intervention vary widely. For 50% (n ¼ 11) of the respondents the presence of an asymptomatic CM alone is a valid reason to consider surgical intervention. Other indications for surgical intervention are depicted in ►Table 2. A total of 36% of surgeons who encounter CM up to 2 times/y considered an asymptomatic CM an indication for surgery, versus all of the pediatric surgeons who encounter a CM more than 2 times/y (p ¼ 0.01). Opinions when to perform surgery in the asymptomatic neonate diagnosed with CM ranged from within 3 months after birth to when the child is older than 2 years. Overall 27% (n ¼ 6) prefers surgery before 6 months, whereas 55% (n ¼ 12) of the respondents prefers surgery between 6 months and 2 years of age. Surgeons who responded that an asymptomatic CM is a reason for surgery (i.e., surgeons with more CM exposure) preferred a significantly shorter interval between start of symptoms and surgery (p ¼ 0.03). When the neonate becomes symptomatic 45% (n ¼ 10) pediatric surgeons prefers surgery within 3 months.
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Table 1 Experience and practices of 22/31 active Dutch paediatric surgeons regarding the incidence and diagnostic work-up of CMa Number of CM encountered annually
Number of surgeons
0–1/year
5
1–2/year
10
3–5/year
6
>5 times a year
1 >2 times a year
Important
Not important
Important
Not important
Shape of CM
12
3
6
1
Boundaries/outline of the CM
15
0
6
1
Size of the CM
9
6
6
1
Stenosis of the biliary tract
15
0
6
1
Presence of sludge/cholelithiasis
6
9
5
2
Presence of enlarged lymph nodes
2
13
0
7
Thickness of the wall of the CM
4
11
2
5
Relation with vascular structures
12
3
4
3
Presence of a common channel
15
0
6
1
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2 times a year
Anatomic details that might be considered important
Criteria for surgical intervention Missing/No opinion
2 2 times a year
>2 times a year
Yes
No
Yes
No
Presence of CM without symptoms
5
9
6
0
Failure to thrive
4
10
2
4
Cholelithiasis
5
9
3
3
Cholangitis
12
2
4
2
Abdominal pain
6
8
2
4
Jaundice
13
1
5
1
Desirable interval for surgery in the asymptomatic infant Missing/ no opinion
3 2 times a year
>2 times a year
3 months after birth
1
1
3–6 months after birth
2
2
6 months–1 year after birth
3
2
1–2 years after birth
6
1
>2 years after birth
1
1
Desirable interval for surgery in the jaundiced neonate of 6 weeks old Missing/no opinion
4 2 times a year
>2 times a year
Within 1 month
6
2
Within 3 months
1
1
Within 3–6 months
3
2
Wait as long as possible
3
0
Preoperative laboratory investigations Missing/No opinion
1 2 times a year
>2 times a year (Continued)
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Table 1 (Continued) Number of CM encountered annually
Number of surgeons Yes
No
Yes
No
Infection parameters
14
1
6
0
Liver function tests
15
0
6
0
General blood tests
15
0
6
0
Kidney function tests
8
7
3
3
CA-19.9
1
14
0
6
Diagnostic workup after ultrasonography Missing/No opinion
1 2 times a year
>2 times a year
MRCP
12
3
ERCP
3
3
Abbreviations: CM, choledochal malformation; ERCP, endoscopic retrograde cholangiopancreatography; MRCP, magnetic resonance cholangiopancreatography. a Surgeons are subsequently divided into those operating 2 CM/year and those operating > 2 CM/year.
Surgical Technique and Intraoperative Investigations Preferred surgical techniques and intraoperative investigations are outlined in ►Table 3. Cholangiography and cholangioscopy were used by 12/22 (55%) and 1/22 (5%), respectively. Laparoscopic resection was favored by 32% (n ¼ 7) of the respondents. All favored hepaticojejunostomy with Roux-en-Y reconstruction as the preferred reconstruction method. One-third of the respondents were reluctant to perform parenchymal resections. Personal exposure did not show any significant differences between the preference for laparoscopic or open surgery and the need for adjacent resections.
Follow-Up Follow-up frequencies, duration, and preferred additional workup used during follow-up can be seen in ►Table 4. A general tendency of one visit every half year to a year can be seen. Overall 41% (n ¼ 9) of the pediatric surgeons prefer lifelong follow-up, with ultrasound and liver function tests as favored follow-up investigations. A total of 14% (n ¼ 3) regularly tests tumor markers, all performing lifelong
Table 2 Indications for surgical intervention in the group of pediatric surgeons encountering CM up to 2 times/y, 41% (n ¼ 9) who did not consider the presence of an asymptomatic CM as a primary indication for surgery Criteria for surgical intervention
Yes
No
Failure to thrive
3
6
Cholelithiasis
2
7
Cholangitis
8
1
Abdominal pain
2
7
Jaundice
9
0
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follow-up. The remaining 10 respondents preferred a follow-up for a period of 2 to 10 years. Personal case load did not show any significant differences between length, frequency, and favored investigations of follow-up.
Discussion This study investigates the exposure to and practices of Dutch pediatric surgeons in diagnostic workup, surgical treatment, and follow-up of patients diagnosed with CM. CM is a rare disease and there are several important controversies regarding diagnostics and treatment. The present survey demonstrated that preferences and practices are very diverse. Some of these preferences and practices may not be in line with the expert opinions about the approach to CM.4,7–12 In the Netherlands, exposure to CM is very low, with only 32% (n ¼ 7) of Dutch pediatric surgeons encountering CM more than 2 times/y. Most pediatric surgeons hardly ever encounter CM and if they do they might treat it together with a colleague with more experience or ask a colleague from another center for help (i.e., with laparoscopic surgery). But, according to our survey, even the pediatric surgeons with the most exposure hardly ever see > 5 cases of CM annually. At first, we investigated the preferences and practices regarding the diagnostic workup. In the literature, both ERCP and MRCP are advocated as adjuncts to ultrasonography. As expected, surgeons choose the imaging method they were most familiar with. In the Netherlands, neonatal ERCP is only available in one center and all pediatric surgeons from that center favored ERCP. In experienced hands, ERCP is capable of accurately delineating biliary anatomy.13–16 On the other hand, it requires general anesthesia and remains an invasive procedure with nonneglectable morbidity such as cholangitis.13,17,18 Full visualization of bigger CM requires high dye loads, which further increases this risk.19,20 In
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Table 3 Practices of 22/31 active Dutch pediatric surgeons regarding surgical technique and intraoperative investigations Preferred surgical approach Missing/no opinion
4 Up to 2 times/y
> 2 times/y
Laparoscopic
4
3
Open
8
3
Intraoperative investigations > 2 times/y
Up to 2 times/y
Missing/no opinion
Yes
No
Yes
No
Cholangioscopy
1
12
0
6
3
Cholangiography
9
3
3
3
4
Liver biopsy
8
4
3
3
4
Missing/no opinion
7 Up to 2 times/y
> 2 times/y
Never
4
4
With an intrahepatic dilatation
0
0
When the intrahepatic dilatation does not improve after extrahepatic dilatation
4
1
The presence of an intrahepatic dilatation and when after extrahepatic reconstruction the intrahepatic dilatation does not improve
1
1
Preferred surgical technique Missing/no opinion
2 Up to 2 times/y
> 2 times/y
HD
0
0
HJ
14
6
Abbreviations: HD, hepaticoduodenostomy; HJ; hepaticojejunostomy.
patients with recurrent infections, it can be more difficult to perform an ERCP due to scarring.13,14,21,22 ERCP fails in visualizing the pancreaticobiliary duct junction in 5 to 30% of the cases.8,23 MRCP has sensitivity for diagnosing CM of 90 to 100%.24 New technology no longer requires breath holding maneuvers.14 This allows for more convenient imaging and older, cooperative, children no longer need anesthesia.14,24 MRCP is relatively poor at imaging tortuous ducts, stones or ducts smaller than 5 mm and visualizing possible abnormal pancreaticobiliary junction.19,20,25 Most important, MRCP has no immediate therapeutic options for treatment of a biliary problem. To our surprise, half of the respondents did not consider the presence of an asymptomatic CM per se an indication for surgery. This seems in contrast with the widely held opinion that CM should be excised as it can lead to significant CM-related morbidity such as cholelithiasis or cholangitis, and is by many considered a premalignant condition.7,26,27 All the pediatric surgeons with more exposure considered the sheer presence of CM an indication for surgery. Perhaps in line with the previous item, 41% (n ¼ 9) of the Dutch pediatric surgeons prefer postponing surgery until the
asymptomatic neonate is at least 1 year. However, all of the pediatric surgeons who considered the presence of an asymptomatic CM an indication for surgery, also choose a significant shorter interval between detection and surgery (p ¼ 0.03). According to some authors, early surgery might have a higher morbidity, such as the development of strictures and obstructive jaundice.28,29 In children undergoing liver transplantation, smaller size (< 10 kg) is clearly associated with a higher incidence of biliary complications.29 This is probably due to the technical difficulties of performing a biliary anastomosis in small children.28,29 Therefore, surgical intervention at a later age has been advocated. On the other hand, postponing resection until the age of 12 months (> 10 kg) can lead to a higher incidence of CM-related complications such as perforation, cholangitis, and progressive liver fibrosis, perhaps even more so in children with an antenatal or immediately postnatal-detected CM.7,12 With the advance of antenatal ultrasound, there will be more neonates presenting with an antenatal-detected cystic lesion.12 When a cystic form of biliary atresia has been ruled out, CM diameter, jaundice, and liver function can be monitored closely. However, it is not European Journal of Pediatric Surgery
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Parenchyma resection
A Survey among Dutch Pediatric Surgeons for Controversies in CM Table 4 Practices of 22/31 active Dutch pediatric surgeons regarding follow-up and research used during follow-up Frequency of follow-up after surgery Missing/no opinion
2 Up to 2 times/y
> 2 times/y
Once every half year
8
2
Yearly
6
3
Once in 2 years
0
1
Duration of follow-up Missing/no opinion
3 Up to 2 times/y
> 2 times/y
2–5 years
3
2
5–10 years
3
2
Lifelong
7
2
Investigations used during follow-up Missing/no opinion
3 Up to 2 times/y
> 2 times/y
Yes
No
Yes
No
Ultrasonography
10
3
5
1
Liver function tests
12
1
5
1
ERCP
1
12
0
6
MRCP
1
12
0
6
CT-scan
1
12
0
6
CA-19.9
3
10
0
6
Abbreviations: CT, computed tomography; ERCP, endoscopic retrograde cholangiopancreatography; MRCP, magnetic resonance cholangiopancreatography.
clear if or when CM-related complications will occur in the asymptomatic infants with prenatally diagnosed CM.12 To our knowledge there are no large multicenter series from Europe or the United States comparing early with delayed surgery in terms of preoperative and postoperative complications and long-term outcome. Intraoperative imaging using cholangioscopy or cholangiography was not often performed according to our survey. Cholangioscopy offers the possibility to visualize the biliary tree to delineate possible intrahepatic extension of abnormal epithelium and the possibility to rinse the biliary tract of sludge/cholelithiasis.30–32 It also enables the surgeon to recognize a possible biliary stenosis, which can be found with an intrahepatic dilation.33 Several studies33,34 concluded that intraoperative cholangioscopy can reduce long-term complications by removing sludge and cholelithiasis. Despite these benefits, only one Dutch pediatric surgeon uses cholangioscopy. Cholangiography is useful for identifying an abnormal pancreaticobiliary duct junction or ductal-filling defects.35 There is a relation between the presence of an abnormal European Journal of Pediatric Surgery
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pancreaticobiliary duct junction and a long common channel with histopathological liver changes.7,36–38 Intraoperative cholangiography can be considered when preoperative workup does not provide sufficient information on the presence of an abnormal pancreaticobiliary duct junction and/or a long common channel.36,39,40 When (if ever) to perform a parenchyma resection in the pediatric patient when intrahepatic dilation is present (type IVa) remains a hot topic. The debate is heated up even more when “adult” hepatobiliary surgeons become involved. Persistent intrahepatic dilations can lead to stasis leading to cholelithiasis or cholangitis.34 The risk of biliary malignancy might also be related to the presence of intrahepatic biliary dilatations.4,41–45 However, literature describes a significant decrease in intrahepatic duct diameter after resection of the concomitant stenosis.10,46 To prevent biliary malignancy, removal of the stenosis (leading to a decrease in dilatation) might be sufficient, and resection of the parenchyma surrounding the dilated part might not be necessary.34,44,47,48 Acker et al49 stated that the preoperative presence of an intrahepatic dilatation is not predictive of postoperative intrahepatic involvement. Hill et al10 also demonstrated that intrahepatic duct dilatation could reverse after resection of the CM. Thus, it can be very difficult to distinguish between type IVa (hepatic involvement) or a type I CM with diffuse biliary dilation, which is often seen at presentation.47,50 The need for partial liver resection in pediatric patients with intrahepatic dilatations therefore remains debatable. While most pediatric surgeons favor an open approach, there is a significant minority of 32% (n ¼ 7) that favors a laparoscopic approach. In the Netherlands, there is limited experience with the laparoscopic approach, most centers perform open procedures or ask for the aid of one of two surgeons who do perform laparoscopic resection. The high number of surgeons favoring the laparoscopic approach was therefore quite surprising. Hepaticojejunostomy seems to be the favorite reconstruction for Dutch pediatric surgeons. A recent review suggested that hepaticoduodenostomy might have slightly better short-term outcomes (e.g., hospital stay and postoperative cholangitis) but might also be associated with a significant higher rate of reflux and gastritis.11,51 Most articles addressing this issue are retrospective case series from Asia, data from Western high volume centers is scarce. A total of 45% (n ¼ 10) of the pediatric surgeons stated to follow-up their patients for only a period of 2 to 10 years and 41% (n ¼ 9) would perform lifelong follow-up. From this it can be concluded that some CM patients will not undergo regular follow-up after these first 10 years. With well known (but not well described) long-term complications, such as biliary strictures, and the possible development of malignancy this is a disturbing thought, even while the exact incidence of malignancy developing after CM is unknown in the Western world.27
Conclusions While only 71% of Dutch pediatric surgeons completed the questionnaire, we feel that the present survey provides
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Conflict of Interest None.
16 Matsufuji H, Araki Y, Nakamura A, Ohigashi S, Watanabe F.
17
18
19
20
21
22
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important information on the preferences and practices in the management of CM in the Netherlands. As expected, the survey demonstrates that exposure is limited and that there is a wide variety of opinions. We did not investigate treatment outcomes, nor did we attempt to answer some of the controversies. We only aimed at investigating exposure and current preferences and practices, as this could provide the stepping stone for defining a common approach to this rare disease. The findings from this survey demonstrate that there is a need for evidence-based (inter)national guidelines regarding management of this rare disease. These guidelines can only be established via (inter)national cooperation using nationwide databases to achieve enough statistical power to answer several of the questions raised.
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37 38
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to surgery: the overlooked aspect? Pediatr Surg Int 2014;30(2): 205–211 Nambirajan L, Taneja P, Singh MK, Mitra DK, Bhatnagar V. The liver in choledochal cyst. Trop Gastroenterol 2000;21(3):135–139 Todani T, Watanabe Y, Toki A, Ogura K, Wang ZQ. Co-existing biliary anomalies and anatomical variants in choledochal cyst. Br J Surg 1998;85(6):760–763 Sharma N, Bhatnagar V, Srinivas M, Agarwala S, Singh MK, Sharma R. Correlation of intracystic pressure with cyst volume, length of common channel, biochemical changes in bile and histopathological changes in liver in choledochal cyst. J Indian Assoc Pediatr Surg 2014;19(1):10–16 Davenport M, Stringer MD, Howard ER. Biliary amylase and congenital choledochal dilatation. J Pediatr Surg 1995;30(3):474–477 Kawarada Y, Das BC, Tabata M, Isaji S. Surgical treatment of type IV choledochal cysts. J Hepatobiliary Pancreat Surg 2009;16(5): 684–687 Lenriot JP, Gigot JF, Ségol P, Fagniez PL, Fingerhut A, Adloff M; French Associations for Surgical ResearchBile duct cysts in adults: a multi-institutional retrospective study. Ann Surg 1998;228(2): 159–166 Kawamoto S, Hiraoka T, Maruta S, Watanabe E, Kanemitsu K, Tsuji T. A case of early cancer in cystic intrahepatic duct associated with congenital choledochal cyst. Hepatogastroenterology 1998; 45(20):428–432
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cinoma arising 34 years after excision of a type IV-A congenital choledochal cyst: report of a case. Surg Today 2009;39(3):247–251 Chijiiwa K, Komura M, Kameoka N. Postoperative follow-up of patients with type IVA choledochal cysts after excision of extrahepatic cyst. J Am Coll Surg 1994;179(6):641–645 Koshinaga T, Inoue M, Ohashi K, et al. Persistent biliary dilatation and stenosis in postoperative congenital choledochal cyst. J Hepatobiliary Pancreat Sci 2011;18(1):47–52 Wiseman K, Buczkowski AK, Chung SW, Francoeur J, Schaeffer D, Scudamore CH. Epidemiology, presentation, diagnosis, and outcomes of choledochal cysts in adults in an urban environment. Am J Surg 2005;189(5):527–531, discussion 531 Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg 1999; 6(3):207–212 Acker SN, Bruny JL, Narkewicz MR, Roach JP, Rogers A, Karrer FM. Preoperative imaging does not predict intrahepatic involvement in choledochal cysts. J Pediatr Surg 2013;48(12): 2378–2382 Visser BC, Suh I, Way LW, Kang SM. Congenital choledochal cysts in adults. Arch Surg 2004;139(8):855–860, discussion 860–862 Todani T, Watanabe Y, Urushihara N, Noda T, Morotomi Y. Biliary complications after excisional procedure for choledochal cyst. J Pediatr Surg 1995;30(3):478–481
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Controversies in Choledochal Malformations: A Survey among Dutch Pediatric Surgeons Maria Hendrina Antoinette van den Eijnden1 Paul M. J. G. Peeters2 Jan B. F. Hulscher1
Ruben H. de Kleine2
1 Department of Pediatric Surgery, University Medical Centre
Groningen, Groningen, The Netherlands 2 Department of Hepatobiliary Surgery and Liver Transplantation, University Medical Center Groningen, Groningen, The Netherlands 3 Department of Paediatrics, University Medical Center Groningen, Groningen, The Netherlands 4 Department of Pediatric Surgery, Hôpitaux Universitaires de Genève, Geneva, Switzerland
Henkjan J. Verkade3
Jim C. H. Wilde4
Address for correspondence Jan B. F. Hulscher, MD, PhD, Department of Paediatric Surgery, University Medical Center Groningen, Hanzeplein 1, HPC BA20 Postbus 30.001, Groningen 9700 RB, The Netherlands (e-mail: [email protected]).
Abstract
Keywords
► choledochal malformation ► choledochal cysts ► controversies in choledochal malformation ► malformation
received June 2, 2014 accepted July 3, 2014 published online October 26, 2014
Background Choledochal malformation (CM) is a rare medical condition of which 80% are diagnosed in pediatric patients. There are several important controversies regarding diagnostic workup, management, and follow-up in these pediatric patients. To assess preferences and practices of Dutch pediatric surgeons regarding the diagnostic procedures, management, and follow-up of children with CM we conducted an electronic survey. Methods A questionnaire was sent to all the pediatric surgeons working in the academic centers and the only community hospital with a pediatric surgery service. The questionnaire included, items regarding incidence, diagnostic workup, interval between diagnosis and surgery, surgical techniques, and follow-up. We also assessed whether personal exposure influenced the preferences and practices. Results Overall 22 out of the 31 (71%) Dutch pediatric surgeons returned the questionnaire. Total 15 out of 22 (68%) encountered CM up to 2 times/y, whereas 7 out of 22 (32%) encountered it more than 2 times/y. Indications for surgery were significantly different between surgeons who encountered CM > 2 time/y versus those who did not: 6/6 (100%) of surgeons encountering CM > 2 times/y considered the presence of an asymptomatic CM an indication for surgery versus 5/14 (36%) of the pediatric surgeons who encountered a CM up to 2 times/y (p ¼ 0.01). Overall 12 out of the 22 (55%) respondents preferred surgery between 6 months and 2 years of age. The amount of exposure did not differ in preferred age at surgery or surgical technique. In the symptomatic child 10/22 (45%) of respondents preferred surgery within 3 months. Overall 7/22 (32%) favored laparoscopic resection. Hepaticojejunostomy with Roux-en-Y reconstruction was the preferred reconstruction for all the respondents. One-third stated that they never performed a parenchyma resection. Follow-up was limited to 10 years in almost half of the respondents. Conclusion Dutch pediatric surgeons demonstrate a wide variety of opinions regarding diagnostic workup, treatment, and follow-up of CM. While most surgeons encounter
© 2015 Georg Thieme Verlag KG Stuttgart · New York
DOI http://dx.doi.org/ 10.1055/s-0034-1387947. ISSN 0939-7248.
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CM up to 2 times/y, there is an association between exposure and several of the outcome parameters. Some of the answers are not in line with the expert opinion. This demonstrates that there is a need for evidence-based (inter)national guidelines regarding the diagnostic approach, management, and follow-up.
Introduction
Statistics
A choledochal malformation (CM) is a rare medical condition with an incidence of 100,000 to 150,000 live births in the Western population.1,2 Almost 80% of the CM is diagnosed before the age of 10 years.3 There are several important controversies regarding the diagnosis and management of CM in children. For instance, some favor a magnetic resonance cholangiopancreatography (MRCP), while others still prefer an endoscopic retrograde cholangiopancreatography (ERCP). Some favor laparoscopic resection, while others argue in the favor of an open approach.4,5 Some advocate early surgery, while others prefer to wait until the child is at least 6 months old before performing any surgery in the asymptomatic neonate.6,7 While there are expert opinions which are more or less accepted, evidencebased guidelines are lacking and outcome measures are not specified. This lack of an evidence-based nationwide guideline for the approach of CM might hamper the outcomes in both short and long term. To assess practices of the Dutch pediatric surgeons regarding the management of CM we have conducted an electronic survey on several controversial topics. We speculated that, given the rarity of this disease in combination with the lack of consensus regarding diagnostic and therapeutic strategies, different centers and different pediatric surgeons use different strategies. In this article, we do not attempt to provide answers to the controversies mentioned above, nor do we assess treatment outcomes. However, investigating nationwide preferences and practices can be the first step toward a common approach to a rare disease.
Methods Sampling The local medical ethics committee approved of this research. An electronic survey was sent to pediatric surgeons working in the six academic pediatric surgical centers and in the only Dutch community hospital with a pediatric surgery service. After 3 weeks a reminder was sent to the nonrespondents and after 6 weeks the questionnaire was closed. The survey was constructed by the authors.
Questionnaire Besides items regarding the frequency in which Dutch pediatric surgeons encounter CM, the questionnaire aimed to gain insight into the routine practices regarding diagnostic workup, preferred interval between diagnosis and surgery, surgical technique, and follow-up. European Journal of Pediatric Surgery
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Apart from descriptive statistics we tested associations between individual exposure and several of the outcome parameters. Percentages were calculated including the pediatric surgeons who did not answer all questions of the survey (this was considered the missing data) or answered “no opinion.” Nonresponders were not included in the analyses. The SPSS IBM 20 package (Armonk, New York, United States) was used for statistical analysis. To test the categorical variables Fisher exact test or chi-square statistics were used as appropriate.
Results Overall 22 (71%) of the 31 Dutch pediatric surgeons returned the questionnaire. Out of which 11 responded after the primary request and 11 after the reminder was sent. Two respondents did not answer all the questions.
Incidence and Diagnostic Workup The incidence of CM per surgeon and practices regarding the diagnostic workup are displayed in ►Table 1. A total of 68% (n ¼ 15) of the respondents encounter a CM up to 2 times/y and 32% (n ¼ 7) more than 2 times/y. The anatomical criteria when performing diagnostic workup on CM vary, with most surgeons looking for information regarding anatomical boundaries of CM, the possible presence of a common channel, and stenosis of the bile duct. Criteria for surgical intervention vary widely. For 50% (n ¼ 11) of the respondents the presence of an asymptomatic CM alone is a valid reason to consider surgical intervention. Other indications for surgical intervention are depicted in ►Table 2. A total of 36% of surgeons who encounter CM up to 2 times/y considered an asymptomatic CM an indication for surgery, versus all of the pediatric surgeons who encounter a CM more than 2 times/y (p ¼ 0.01). Opinions when to perform surgery in the asymptomatic neonate diagnosed with CM ranged from within 3 months after birth to when the child is older than 2 years. Overall 27% (n ¼ 6) prefers surgery before 6 months, whereas 55% (n ¼ 12) of the respondents prefers surgery between 6 months and 2 years of age. Surgeons who responded that an asymptomatic CM is a reason for surgery (i.e., surgeons with more CM exposure) preferred a significantly shorter interval between start of symptoms and surgery (p ¼ 0.03). When the neonate becomes symptomatic 45% (n ¼ 10) pediatric surgeons prefers surgery within 3 months.
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Table 1 Experience and practices of 22/31 active Dutch paediatric surgeons regarding the incidence and diagnostic work-up of CMa Number of CM encountered annually
Number of surgeons
0–1/year
5
1–2/year
10
3–5/year
6
>5 times a year
1 >2 times a year
Important
Not important
Important
Not important
Shape of CM
12
3
6
1
Boundaries/outline of the CM
15
0
6
1
Size of the CM
9
6
6
1
Stenosis of the biliary tract
15
0
6
1
Presence of sludge/cholelithiasis
6
9
5
2
Presence of enlarged lymph nodes
2
13
0
7
Thickness of the wall of the CM
4
11
2
5
Relation with vascular structures
12
3
4
3
Presence of a common channel
15
0
6
1
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2 times a year
Anatomic details that might be considered important
Criteria for surgical intervention Missing/No opinion
2 2 times a year
>2 times a year
Yes
No
Yes
No
Presence of CM without symptoms
5
9
6
0
Failure to thrive
4
10
2
4
Cholelithiasis
5
9
3
3
Cholangitis
12
2
4
2
Abdominal pain
6
8
2
4
Jaundice
13
1
5
1
Desirable interval for surgery in the asymptomatic infant Missing/ no opinion
3 2 times a year
>2 times a year
3 months after birth
1
1
3–6 months after birth
2
2
6 months–1 year after birth
3
2
1–2 years after birth
6
1
>2 years after birth
1
1
Desirable interval for surgery in the jaundiced neonate of 6 weeks old Missing/no opinion
4 2 times a year
>2 times a year
Within 1 month
6
2
Within 3 months
1
1
Within 3–6 months
3
2
Wait as long as possible
3
0
Preoperative laboratory investigations Missing/No opinion
1 2 times a year
>2 times a year (Continued)
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Table 1 (Continued) Number of CM encountered annually
Number of surgeons Yes
No
Yes
No
Infection parameters
14
1
6
0
Liver function tests
15
0
6
0
General blood tests
15
0
6
0
Kidney function tests
8
7
3
3
CA-19.9
1
14
0
6
Diagnostic workup after ultrasonography Missing/No opinion
1 2 times a year
>2 times a year
MRCP
12
3
ERCP
3
3
Abbreviations: CM, choledochal malformation; ERCP, endoscopic retrograde cholangiopancreatography; MRCP, magnetic resonance cholangiopancreatography. a Surgeons are subsequently divided into those operating 2 CM/year and those operating > 2 CM/year.
Surgical Technique and Intraoperative Investigations Preferred surgical techniques and intraoperative investigations are outlined in ►Table 3. Cholangiography and cholangioscopy were used by 12/22 (55%) and 1/22 (5%), respectively. Laparoscopic resection was favored by 32% (n ¼ 7) of the respondents. All favored hepaticojejunostomy with Roux-en-Y reconstruction as the preferred reconstruction method. One-third of the respondents were reluctant to perform parenchymal resections. Personal exposure did not show any significant differences between the preference for laparoscopic or open surgery and the need for adjacent resections.
Follow-Up Follow-up frequencies, duration, and preferred additional workup used during follow-up can be seen in ►Table 4. A general tendency of one visit every half year to a year can be seen. Overall 41% (n ¼ 9) of the pediatric surgeons prefer lifelong follow-up, with ultrasound and liver function tests as favored follow-up investigations. A total of 14% (n ¼ 3) regularly tests tumor markers, all performing lifelong
Table 2 Indications for surgical intervention in the group of pediatric surgeons encountering CM up to 2 times/y, 41% (n ¼ 9) who did not consider the presence of an asymptomatic CM as a primary indication for surgery Criteria for surgical intervention
Yes
No
Failure to thrive
3
6
Cholelithiasis
2
7
Cholangitis
8
1
Abdominal pain
2
7
Jaundice
9
0
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follow-up. The remaining 10 respondents preferred a follow-up for a period of 2 to 10 years. Personal case load did not show any significant differences between length, frequency, and favored investigations of follow-up.
Discussion This study investigates the exposure to and practices of Dutch pediatric surgeons in diagnostic workup, surgical treatment, and follow-up of patients diagnosed with CM. CM is a rare disease and there are several important controversies regarding diagnostics and treatment. The present survey demonstrated that preferences and practices are very diverse. Some of these preferences and practices may not be in line with the expert opinions about the approach to CM.4,7–12 In the Netherlands, exposure to CM is very low, with only 32% (n ¼ 7) of Dutch pediatric surgeons encountering CM more than 2 times/y. Most pediatric surgeons hardly ever encounter CM and if they do they might treat it together with a colleague with more experience or ask a colleague from another center for help (i.e., with laparoscopic surgery). But, according to our survey, even the pediatric surgeons with the most exposure hardly ever see > 5 cases of CM annually. At first, we investigated the preferences and practices regarding the diagnostic workup. In the literature, both ERCP and MRCP are advocated as adjuncts to ultrasonography. As expected, surgeons choose the imaging method they were most familiar with. In the Netherlands, neonatal ERCP is only available in one center and all pediatric surgeons from that center favored ERCP. In experienced hands, ERCP is capable of accurately delineating biliary anatomy.13–16 On the other hand, it requires general anesthesia and remains an invasive procedure with nonneglectable morbidity such as cholangitis.13,17,18 Full visualization of bigger CM requires high dye loads, which further increases this risk.19,20 In
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Table 3 Practices of 22/31 active Dutch pediatric surgeons regarding surgical technique and intraoperative investigations Preferred surgical approach Missing/no opinion
4 Up to 2 times/y
> 2 times/y
Laparoscopic
4
3
Open
8
3
Intraoperative investigations > 2 times/y
Up to 2 times/y
Missing/no opinion
Yes
No
Yes
No
Cholangioscopy
1
12
0
6
3
Cholangiography
9
3
3
3
4
Liver biopsy
8
4
3
3
4
Missing/no opinion
7 Up to 2 times/y
> 2 times/y
Never
4
4
With an intrahepatic dilatation
0
0
When the intrahepatic dilatation does not improve after extrahepatic dilatation
4
1
The presence of an intrahepatic dilatation and when after extrahepatic reconstruction the intrahepatic dilatation does not improve
1
1
Preferred surgical technique Missing/no opinion
2 Up to 2 times/y
> 2 times/y
HD
0
0
HJ
14
6
Abbreviations: HD, hepaticoduodenostomy; HJ; hepaticojejunostomy.
patients with recurrent infections, it can be more difficult to perform an ERCP due to scarring.13,14,21,22 ERCP fails in visualizing the pancreaticobiliary duct junction in 5 to 30% of the cases.8,23 MRCP has sensitivity for diagnosing CM of 90 to 100%.24 New technology no longer requires breath holding maneuvers.14 This allows for more convenient imaging and older, cooperative, children no longer need anesthesia.14,24 MRCP is relatively poor at imaging tortuous ducts, stones or ducts smaller than 5 mm and visualizing possible abnormal pancreaticobiliary junction.19,20,25 Most important, MRCP has no immediate therapeutic options for treatment of a biliary problem. To our surprise, half of the respondents did not consider the presence of an asymptomatic CM per se an indication for surgery. This seems in contrast with the widely held opinion that CM should be excised as it can lead to significant CM-related morbidity such as cholelithiasis or cholangitis, and is by many considered a premalignant condition.7,26,27 All the pediatric surgeons with more exposure considered the sheer presence of CM an indication for surgery. Perhaps in line with the previous item, 41% (n ¼ 9) of the Dutch pediatric surgeons prefer postponing surgery until the
asymptomatic neonate is at least 1 year. However, all of the pediatric surgeons who considered the presence of an asymptomatic CM an indication for surgery, also choose a significant shorter interval between detection and surgery (p ¼ 0.03). According to some authors, early surgery might have a higher morbidity, such as the development of strictures and obstructive jaundice.28,29 In children undergoing liver transplantation, smaller size (< 10 kg) is clearly associated with a higher incidence of biliary complications.29 This is probably due to the technical difficulties of performing a biliary anastomosis in small children.28,29 Therefore, surgical intervention at a later age has been advocated. On the other hand, postponing resection until the age of 12 months (> 10 kg) can lead to a higher incidence of CM-related complications such as perforation, cholangitis, and progressive liver fibrosis, perhaps even more so in children with an antenatal or immediately postnatal-detected CM.7,12 With the advance of antenatal ultrasound, there will be more neonates presenting with an antenatal-detected cystic lesion.12 When a cystic form of biliary atresia has been ruled out, CM diameter, jaundice, and liver function can be monitored closely. However, it is not European Journal of Pediatric Surgery
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Parenchyma resection
A Survey among Dutch Pediatric Surgeons for Controversies in CM Table 4 Practices of 22/31 active Dutch pediatric surgeons regarding follow-up and research used during follow-up Frequency of follow-up after surgery Missing/no opinion
2 Up to 2 times/y
> 2 times/y
Once every half year
8
2
Yearly
6
3
Once in 2 years
0
1
Duration of follow-up Missing/no opinion
3 Up to 2 times/y
> 2 times/y
2–5 years
3
2
5–10 years
3
2
Lifelong
7
2
Investigations used during follow-up Missing/no opinion
3 Up to 2 times/y
> 2 times/y
Yes
No
Yes
No
Ultrasonography
10
3
5
1
Liver function tests
12
1
5
1
ERCP
1
12
0
6
MRCP
1
12
0
6
CT-scan
1
12
0
6
CA-19.9
3
10
0
6
Abbreviations: CT, computed tomography; ERCP, endoscopic retrograde cholangiopancreatography; MRCP, magnetic resonance cholangiopancreatography.
clear if or when CM-related complications will occur in the asymptomatic infants with prenatally diagnosed CM.12 To our knowledge there are no large multicenter series from Europe or the United States comparing early with delayed surgery in terms of preoperative and postoperative complications and long-term outcome. Intraoperative imaging using cholangioscopy or cholangiography was not often performed according to our survey. Cholangioscopy offers the possibility to visualize the biliary tree to delineate possible intrahepatic extension of abnormal epithelium and the possibility to rinse the biliary tract of sludge/cholelithiasis.30–32 It also enables the surgeon to recognize a possible biliary stenosis, which can be found with an intrahepatic dilation.33 Several studies33,34 concluded that intraoperative cholangioscopy can reduce long-term complications by removing sludge and cholelithiasis. Despite these benefits, only one Dutch pediatric surgeon uses cholangioscopy. Cholangiography is useful for identifying an abnormal pancreaticobiliary duct junction or ductal-filling defects.35 There is a relation between the presence of an abnormal European Journal of Pediatric Surgery
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pancreaticobiliary duct junction and a long common channel with histopathological liver changes.7,36–38 Intraoperative cholangiography can be considered when preoperative workup does not provide sufficient information on the presence of an abnormal pancreaticobiliary duct junction and/or a long common channel.36,39,40 When (if ever) to perform a parenchyma resection in the pediatric patient when intrahepatic dilation is present (type IVa) remains a hot topic. The debate is heated up even more when “adult” hepatobiliary surgeons become involved. Persistent intrahepatic dilations can lead to stasis leading to cholelithiasis or cholangitis.34 The risk of biliary malignancy might also be related to the presence of intrahepatic biliary dilatations.4,41–45 However, literature describes a significant decrease in intrahepatic duct diameter after resection of the concomitant stenosis.10,46 To prevent biliary malignancy, removal of the stenosis (leading to a decrease in dilatation) might be sufficient, and resection of the parenchyma surrounding the dilated part might not be necessary.34,44,47,48 Acker et al49 stated that the preoperative presence of an intrahepatic dilatation is not predictive of postoperative intrahepatic involvement. Hill et al10 also demonstrated that intrahepatic duct dilatation could reverse after resection of the CM. Thus, it can be very difficult to distinguish between type IVa (hepatic involvement) or a type I CM with diffuse biliary dilation, which is often seen at presentation.47,50 The need for partial liver resection in pediatric patients with intrahepatic dilatations therefore remains debatable. While most pediatric surgeons favor an open approach, there is a significant minority of 32% (n ¼ 7) that favors a laparoscopic approach. In the Netherlands, there is limited experience with the laparoscopic approach, most centers perform open procedures or ask for the aid of one of two surgeons who do perform laparoscopic resection. The high number of surgeons favoring the laparoscopic approach was therefore quite surprising. Hepaticojejunostomy seems to be the favorite reconstruction for Dutch pediatric surgeons. A recent review suggested that hepaticoduodenostomy might have slightly better short-term outcomes (e.g., hospital stay and postoperative cholangitis) but might also be associated with a significant higher rate of reflux and gastritis.11,51 Most articles addressing this issue are retrospective case series from Asia, data from Western high volume centers is scarce. A total of 45% (n ¼ 10) of the pediatric surgeons stated to follow-up their patients for only a period of 2 to 10 years and 41% (n ¼ 9) would perform lifelong follow-up. From this it can be concluded that some CM patients will not undergo regular follow-up after these first 10 years. With well known (but not well described) long-term complications, such as biliary strictures, and the possible development of malignancy this is a disturbing thought, even while the exact incidence of malignancy developing after CM is unknown in the Western world.27
Conclusions While only 71% of Dutch pediatric surgeons completed the questionnaire, we feel that the present survey provides
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Conflict of Interest None.
16 Matsufuji H, Araki Y, Nakamura A, Ohigashi S, Watanabe F.
17
18
19
20
21
22
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important information on the preferences and practices in the management of CM in the Netherlands. As expected, the survey demonstrates that exposure is limited and that there is a wide variety of opinions. We did not investigate treatment outcomes, nor did we attempt to answer some of the controversies. We only aimed at investigating exposure and current preferences and practices, as this could provide the stepping stone for defining a common approach to this rare disease. The findings from this survey demonstrate that there is a need for evidence-based (inter)national guidelines regarding management of this rare disease. These guidelines can only be established via (inter)national cooperation using nationwide databases to achieve enough statistical power to answer several of the questions raised.
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cinoma arising 34 years after excision of a type IV-A congenital choledochal cyst: report of a case. Surg Today 2009;39(3):247–251 Chijiiwa K, Komura M, Kameoka N. Postoperative follow-up of patients with type IVA choledochal cysts after excision of extrahepatic cyst. J Am Coll Surg 1994;179(6):641–645 Koshinaga T, Inoue M, Ohashi K, et al. Persistent biliary dilatation and stenosis in postoperative congenital choledochal cyst. J Hepatobiliary Pancreat Sci 2011;18(1):47–52 Wiseman K, Buczkowski AK, Chung SW, Francoeur J, Schaeffer D, Scudamore CH. Epidemiology, presentation, diagnosis, and outcomes of choledochal cysts in adults in an urban environment. Am J Surg 2005;189(5):527–531, discussion 531 Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg 1999; 6(3):207–212 Acker SN, Bruny JL, Narkewicz MR, Roach JP, Rogers A, Karrer FM. Preoperative imaging does not predict intrahepatic involvement in choledochal cysts. J Pediatr Surg 2013;48(12): 2378–2382 Visser BC, Suh I, Way LW, Kang SM. Congenital choledochal cysts in adults. Arch Surg 2004;139(8):855–860, discussion 860–862 Todani T, Watanabe Y, Urushihara N, Noda T, Morotomi Y. Biliary complications after excisional procedure for choledochal cyst. J Pediatr Surg 1995;30(3):478–481
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