THE ROLE OF ERCP IN THE EVALUATION, DIAGNOSIS AND THERAPY
OF BILIARY AND PANCREATIC DISEASES IN CHILDREN
Haissam Nourallah, MD, CES; Hussain Issa, MD;
Ahmed H. Al-Salem, FRCSI, FICS, FACS
The role of endoscopic retrograde cholangiopancreatography (ERCP) for the investigation and treatment of biliary
and pancreatic diseases is well established in adults.1 This,
however, is not the case in the pediatric age group, because
biliary and pancreatic diseases are less common in children.
In addition to this problem is the lack of pediatric
gastroenterologists who are skilled in performing ERCP in
children. Recently, however, and as a result of the routine
use of ultrasonography in the evaluation of children with
abdominal pain, biliary and pancreatic diseases are being
diagnosed more often in children,2 especially in areas where
hemolytic diseases which are known to be associated with
increased
frequency
of
cholelithiasis
and
choledocholithiasis are common.3-5
The present study describes our experience with ERCP
in the evaluation, diagnosis and treatment of pancreatobiliary disorders in children and demonstrates its value in
the era of laparoscopic cholecystectomy.
Patients and Methods
Over a four-year period from September 1993 to August
1997, 34 consecutive children less than 18 years old had
ERCP as part of their management at our hospital. The
records of these patients were reviewed retrospectively for
age, gender, indication for ERCP, pre-ERCP investigations,
results, complications of ERCP and post-ERCP
management.
Informed, written consent for ERCP was obtained from
the child’s parent. All ERCPs were performed in the
Radiology Department. For patients 10 years old and
below, ERCP was performed under general anesthesia with
nasotracheal intubation. In those older than 10 years, ERCP
was done under sedation only. Patients with sickle cell
disease were hydrated with intravenous fluids starting the
night before the procedure, at a rate of 1½ their
maintenance requirements, and blood transfusions were
given when necessary to increase their Hb to 10-12 g/dL.
From the Departments of Medicine and Surgery, Qatif Central Hospital,
Qatif, Saudi Arabia.
Address reprint requests and correspondence to Dr. Al-Salem: P.O.
Box 18342, Qatif 31911, Saudi Arabia.
Accepted for publication 29 November 1998. Received 24 February
1998.
With the patients properly sedated, using meperedine (1
mg/kg) and diazepam (0.1-0.2 mg/kg), the Olympus JF1
T20 side-viewing duodenoscope was used in all the
patients. After visualization, the ampulla of Vater was
cannulated with tapered catheters and the pancreatic and
biliary ducts were visualized by fluoroscopy during
injection of Hexabrix (320 mg was diluted to 50%).
Appropriate radiographs were obtained in all cases. Where
indicated, sphincterotomy was performed using 5F
sphincterotome (Olympus). Common bile duct stone
extraction was performed with basket and balloon catheters.
During the procedure, all patients were monitored with
pulse oximetry with a cardiorespiratory trolley at the
bedside.
Results
The 34 children who had ERCP at Qatif Central
Hospital as part of their management over the four-year
study period comprised 20 males and 14 females. Their
ages ranged from 5-18 years (mean 14.5 years). Of the 34
patients, 29 (85.3%) had sickle cell disease (SCD). Their
mean HbS level was 77.8% (range 66.1-90.7) and their
TABLE
1.
Indications for ERCP.
Indications for ERCP
# of patients
Obstructive jaundice
22
Pancreatitis
4
Cholangitis
3
Recurrent biliary colic
2
Post-liver injury bile lead
1
Post-laparoscopic cholecystectomy with bile leak
1
Total
34
TABLE
2.
Procedures performed during ERCP.
Procedure
# of patients
Endoscopic sphincterotomy only
10
Endoscopic sphincterotomy + stone extraction
9
Endoscopic sphincterotomy + stone extraction +
nasobiliary tube drainage
2
Endoscopic sphincterotomy + biliary stent
1
Endoscopic sphincterotomy + mechanical lithotripsy +
stone extraction
1
Annals of Saudi Medicine, Vol 19, No 2, 1999
163
NOURALLAH
ET AL
mean HbF level was 21% (range 7.2-37). The indications
for ERCP are shown in Table 1. Obstructive jaundice was
the most common indication in 22 patients (64.7%). The
mean total bilirubin for those patients with obstructive
jaundice was 14.4 mg/dL (range 6.6-36), and their mean
alkaline phosphatase was 325.8 IU (range 148-545)
(normal, 50-136 IU). In those with obstructive jaundice,
pre-ERCP abdominal ultrasound showed dilated CBD with
no CBD stones in seven patients, dilated CBD associated
with CBD stones in six patients, and normal CBD in nine
patients. Gallstones were diagnosed in 15 patients and
biliary sludge in two patients. ERCP, on the other hand,
showed dilated CBD with no CBD stones in seven patients,
two of them with enlarged inflamed papilla suggestive of
recent stone passage, dilated CBD and CBD stones in 11,
and normal CBD in four patients, one of them with an
enlarged inflamed papilla suggestive of recent passage of a
stone. Gallstones were diagnosed in 16 patients. While
ultrasound was accurate in diagnosing choledocholithiasis
in only six of 22 children (27.3%) with obstructive
jaundice, ERCP on the other hand diagnosed CBD stones in
11 patients (50%) (Figure 1). In three other patients, an
inflamed enlarged papilla suggestive of recent stone
passage was visualized. The remaining eight patients did
not have CBD stones. Of the 22 children with obstructive
jaundice, ultrasound showed CBD dilatation in 13 (59%),
while ERCP showed CBD dilation in 18 (81.8%) children.
Three other patients had obstructive jaundice and
ascending cholangitis. ERCP revealed a dilated CBD
with CBD stones in one, CBD dilation and papillary
stenosis in another, and was normal in the third patient.
Four patients had pancreatitis, and in one of them ERCP
showed gallstones only, while in another there was a dilated
CBD with no stones but an inflamed enlarged papilla
suggestive of recent stone passage. In the third, ERCP
revealed gallstones and dilated CBD but no stones. The
fourth was a five-year-old child with recurrent pancreatitis.
ERCP in this child showed a choledochocele. One patient
had a post-LC bile leak. ERCP in this patient revealed
retained CBD stones and bile leak from cystic duct stump
(Figure 2).
Another SCD patient presented with obstructive
jaundice following LC. ERCP in this patient showed dilated
CBD but no stone or stricture. One of our patients had
recurrent biliary colic with a total bilirubin of 1.8 mg/dL
and an alkaline phosphatase of 177 IU. An abdominal
ultrasound revealed a dilated CBD with biliary sludge.
ERCP in this patient showed a normal CBD, normal papilla,
normal pancreatic duct, and cholelithiasis, but no
choledocholithiasis. This patient subsequently had LC. The
other patient with recurrent biliary colic had in addition
abnormal liver function test. ERCP in this patient showed
normal CBD and gallstones, and he subsequently had LC.
ERCP in the patient with postliver injury bile leak showed
bile leak from the second order branch of the right hepatic
duct, but the CBD was normal. This patient was treated
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Annals of Saudi Medicine, Vol 19, No 2, 1999
with endoscopic sphincterotomy and biliary stent with
eventual sealing of the leak.
The different procedures performed during ERCP are
shown in Table 2. After the ERCP, none of our patients
with SCD developed complications related to SCD or
anesthesia. Two of our patients (5.9%) developed mild
attacks of post-ERCP pancreatitis which resolved
conservatively.
Discussion
In adults, endoscopic retrograde cholangiopancreatography (ERCP) has a well-defined role in the diagnosis and
management of biliary and pancreatic diseases.1 In the
pediatric age group, however, the use of ERCP is limited
because biliary and pancreatic diseases are less common in
children, and there is a general lack of skilled pediatric
gastroenterologists capable of performing ERCP in
children. In this study, we were successful in performing
ERCP for 34 children. Considering the rarity of biliary and
pancreatic diseases in children, our series is not small, and
this is because the majority of our patients (85%) had sickle
cell disease.
Cholelithiasis is known to be common in children with
SCD. The prevalence of cholelithiasis in patients with SCD
ranges from 17% to 55% and increases with age.5-9 A
19.7% frequency of cholelithiasis has been reported in
Saudi children with SCD.4 In children with non-hemolytic
gallstones, a frequency of about 7% of choledocholithiasis
has been reported.2 This is in comparison with 14% to 30%
in SCD patients with gallstones.3,10-12 In a previous study,
we reported a frequency of CBD stones of 30% in children
with SCD undergoing cholecystectomy,3 and based on this
high frequency we recommended routine intraoperative
cholangiogram. This may necessitate CBD exploration and
open transduodenal sphincterotomy and sphincteroplasty.
Considering the high prevalence of cholelithiasis and
choledocholithiasis in children with SCD, exclusion of
CBD stones prior to cholecystectomy is of great
importance. This is especially so in the era of LC. Like
others, we found ERCP to be useful both in the diagnosis
and management of CBD stones prior to LC.13,14 A 97%
cannulation success rate in our series is similar to the
experience of others.15-17 Although pediatric duodenoscopes are now available, ERCP was performed in our
patients without difficulty, using the adult-size viewing
duodenoscope under sedation, except in those children aged
10 years or younger, where this was done under general
anesthesia to protect against airway compression. The
youngest patient in our series was five years old. With the
recent advancement in pediatric endoscopes, it is now
possible for ERCP and endoscopic sphincterotomy to be
performed in infants.13
ERCP is clearly superior to ultrasound in diagnosing
CBD stones, but it cannot be used routinely as it is invasive,
costly and not without morbidity. Therefore, dilation of
CBD on ultrasound, an elevated alkaline phosphatase,
BRIEF REPORT: ROLE OF ERCP
pancreatitis and an elevated total bilirubin of more than 5
mg/dL, either singly or in combination, should raise the
possibility of CBD stones in these patients. These patients
together with those with CBD stones detected on ultrasound
should undergo ERCP to confirm and extract CBD stones
prior to LC. This combined endoscopic sphincterotomy and
stone extraction followed by LC has been reported in
adults,18,19 and recently in patients with SCD.14
We found ERCP valuable in the diagnosis and
management of CBD stones prior to LC. Eleven of our
patients with CBD stones had successful ERCP and stone
extraction prior to LC. One of them had mechanical
lithotripsy via ERCP, and stone extraction because of the
large size of CBD stones, followed by LC. Ten other
patients with dilated CBD but no stones had endoscopic
sphincterotomy only. This may prove to be useful in the
future for these patients, as they are likely to develop
recurrent bile duct stones or biliary sludge. This is
especially so in the presence of dilated CBD, and since
these stones are usually small to start with, they are likely to
pass spontaneously in the presence of sphincterotomy. Two
of our patients had ERCP following LC to diagnose and
treat retained CBD stones. One of them had bile leak from
the cystic duct stump following LC. ERCP in this patient
showed a retained CBD stone which was extracted with
subsequent closure of the bile leak. In the other patient,
ERCP was normal and jaundice in this patient was
secondary to hepatic crisis. In those with pancreatitis,
ERCP was useful in diagnosing the disease. In three of
them, there were gallstones, and in two the CBD was
dilated with an enlarged inflamed papilla suggestive of
recent stone passage. The fourth patient with recurrent
pancreatitis had choledochocele. ERCP in the child with
post-liver injury bile leak proved useful both for diagnosis
and treatment.
Recently the indications for ERCP in children have been
extended to include the investigations of a variety of
clinical conditions, including biliary atresia,20 choledochal
cyst,21 recurrent pancreatitis,22 cholestatic jaundice,23
traumatic pancreatic duct disruption,24 and the investigation
of bile leak following liver transplant.25 In our series, two
patients developed post-ERCP mild pancreatitis, which is
comparable to the 10% complication rate of endoscopic
sphincterotomy reported both in children and in
adults.17,26,27
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References
27.
1.
2.
3.
4.
5.
Cotton PB. Endoscopic retrograde cholangiography. Gut
1977;18:316-41.
Holcomb GW Jr, O’Neil JA Jr, Holcomb GW III. Cholecystitis,
cholelithiasis and common duct stenosis in children and adolescents.
Ann Surg 1980;191:626-35.
Al-Salem AH, Nangalia R, Kolar K, et al. Cholecystectomy in
children with sickle cell disease: perioperative management. Pediatr
Surg Int 1995;10:472-4.
Al-Salem AH, Qaisaruddin S, Al-Dabbous I, et al. Cholelithiasis in
children with sickle cell disease. Pediatr Surg Int 1996;11:471-3.
Barrett-Connor E. Cholelithiasis in sickle cell anemia. Am J Med
1968;45:889:98.
Karayalcin G, Hassani N, Abrams M, Lanzkowsky PH. Cholelithiasis
in children with sickle cell disease. Am J Dis Child 1979;133:306-7.
Phillips JC, Gerald BE. The incidence of cholelithiasis in sickle cell
disease. Am J Roetgenol Radium Ther Nucl Med 1971;113:27-8.
Rennels MB, Dunne MG, Grossman NJ, Schuntz AD. Cholelithiasis
in patients with major sickle cell hemoglobinopathies. Am J Dis
Child 1984;138:66-7.
Sarnaik S, Slovis T, Corbett DP, et al. Incidence of cholelithiasis in
sickle cell anemia using the ultrasound Gray Scale technique. J
Pediatr 1980;96:1005-8.
Cameron JL, Maddrey WC, Zuidema GD. Biliary tract disease in
sickle cell anemia: surgical considerations. Ann Surg 1971;174:70210.
Schubert TT. Hepatobiliary system in sickle cell disease.
Gastroenterology 1986;90:2013-21.
Ware RE, Schultz WH, Filston HC, et al. Diagnosis and management
of common bile duct stones in patients with sickle
hemoglobinopathies. J Pediatr Surg 1992;27:572-5.
Guelrud N, Rincones VZ, Jaen D, et al. Endoscopic sphincterotomy
and laparoscopic cholecystectomy in a jaundiced infant. Gastrointest
Endosc 1994;40:99-102.
Gholson CF, Grier JF, Ibach MB, et al. Sequential endoscopic/
laparoscopic management of sickle cell hemoglobinopathy-associated
cholelithiasis and suspected choledocholithiasis. Southern Med J
1995;88:1131-5.
Allendorph M, Werlin SL, Green JE, et al. Endoscopic retrograde
cholangiopancreatography in children. J Pediatr 1987;110:206-11.
Buckley A, Connon JJ. The role of ERCP in children and adolescents.
Gastrointest Endosc 1990;36:369-72.
Guelrud M, Mendoza S, Jaen D, Plaz J, Machuca J, Torres P. ERCP
and endoscopic sphincterotomy in infants and children with jaundice
due to common bile duct stones. Gastrointest Endosc 1992;38:450-3.
Duensing RA, Williams RA, Collins JC, Wilson SE. Managing
choledocholithiasis in the laparoscopic era. Am J Surg 1995;170:61923.
Aliperti G, Edmundowicz SA, Soper NJ. Combined endoscopic
sphincterotomy and laparoscopic cholecystectomy in patients with
choledocholithiasis and cholecystolithiasis. Ann Intern Med
1991;115: 783-4.
Guelrud M, Jaen D, Mendoza S, et al. ERCP in the diagnosis of
extrahepatic biliary atresia. Gastrointest Endosc 1991;37:522-6.
Thatcher BS, Sivak MV, Hermann RE, Esselstyn CB. ERCP in
evaluation and diagnosis of choledocal cyst: report of five cases.
Gastrointest Endosc 1986;32:27-31.
Guelrud M, Mujica C, Jaen D, Plaz J, Arias J. The role of ERCP in
the diagnosis and treatment of idiopathic recurrent pancreatitis in
children and adolescents. Gastrointest Endosc 1994;40:428-36.
Derky HHF, Hibregtse K, Taminian JAJM. The role of endoscopic
retrograde cholangiopancreatography in cholestatic infants.
Endoscopy 1994;26:724-8.
Hall RI, Lavelle MI, Venables CW. Use of ERCP to identify the site
of traumatic injuries of the main pancreatic duct in children. Br J
Surg 1986;73:411-23.
Starzl TE, Dominguez R, Young LW, et al. Pediatric liver
transplantation. II. Diagnostic imaging in post-operative management.
Radiology 1985;157:339-44.
Brown KO, Goldschmiedt M. Endoscopic therapy of biliary and
pancreatic disorders in children. Endoscopy 1994;26:719-23.
Freeman M, Nelson DB, Sherman S, et al. Complications of
endoscopic biliary sphincterotomy. N Engl J Med 1996;335:909-18.
Annals of Saudi Medicine, Vol 19, No 2, 1999
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