Diagnosis
and Management of Common Bile Duct Stones in Patients With Sickle Hemoglobinopathies By Russell E. Ware, William H. Schultz, Howard C. Filston, and Thomas Durham, North Carolina and Knoxville, Tennessee
0 Nine of 35 patients with sickle hemoglobinopathies and cholelithiasis were found to have concomitant common bile duct (CBD) stones. We describe the diagnosis and management of these patients with CBD stones. Historical information, physical examination, and routine laboratory tests were unable to identify accurately the patients with CBD stones. Similarly, ultrasonography of the hepetobiliary system was an insensitive method for detecting CBD stones, as only 3 of 8 patients were correctly identified. In contrast, both an intraoperative cholangiogram and endoscopic retrograde cholangiopancreatography were sensitive procedures for detection of CBD stones. We conclude that CBD stones are relatively common in patients with sickle cell disease and cholelithiasis, and clinicians should have a high index of suspicion for their presence. Copyright o 1992 by W.B. Saunders Company INDEX WORDS: Choledocholithiasis; cholelithiasis; sickle cell disease; intraoperative cholangiography; endoscopic retrograde cholangiopancreatography.
T
HE PRINCIPAL manifestations of sickle cell disease include chronic hemolytic anemia and both acute and chronic tissue damage secondary to vasoocclusion produced by the abnormally shaped red blood cells.’ The chronic hemolysis predisposes affected individuals to pigmented cholelithiasis.2,3 Symptoms of cholelithiasis may be difficult to distinguish from those produced by intraabdominal vasoocclusion.4 Once gallstones are identified in these patients, cholecystectomy should be performed to reduce both morbidity and mortality from complications associated with cholelithiasis and emergency cholecystectomy.5Jj Despite the high frequency of gallstones in the sickle cell population, the diagnosis and treatment of
From the Department of Pediatrics, Duke University Medical Center, Durham, NC, and the Department of Surgery, University of Tennessee, Knoxville, TN. Date accepted: February 22, 1991. Supponed in part by a grant (PO 60-HL-28391) from the National institute of Heart, Lung, and Blood Diseases, Sickle Cell Disease Branch, National Institutes of Health. R. W. is the recipient of Physician Scientist Award (Kll-HLO2015) from the National Institutes of Health. Presented in part at the 15th National Sickle Cell Centers Meeting in Oakland, CA. Address reprint requests to Russell E. Ware, MD, PhD, Comprehensive Sickle Cell Center, PO Box 2916, Duke lJniversi@ Medical Center, Durham, NC 27710. Copyright o 1992 by W.B. Saunders Company 0022-3468/9212705-0009$03.00/0 572
R. Kinney
choledocholithiasis in these patients has not been addressed. In this paper, we summarize our experience with the identification and management of common bile duct (CBD) stones in nine patients. We emphasize the usefulness of intraoperative cholangiography (IOC), endoscopic retrograde cholangiopancreatography (ERCP), and endoscopic sphincterotomy. MATERIALS
AND METHODS
Patient Population We reviewed the records of all patients on the pediatric service with sickle cell disease and gallstones treated at Duke University Medical Center from 1977 through 1989. Data were abstracted pertaining to the clinical history, diagnosis, and management of patients with both cholelithiasis and choledocholithiasis.
Imagkg Procedures Ultrasonography of the gallbladder and biliary system was performed using real-time scanning. IOC was done through a catheter placed in the cystic duct at the time of cholecystectomy.
Patient Preparation for Procedures Prior to cholecystectomy, each patient received several packed erythrocyte transfusions that increased the hemoglobin concentration to between 10 and 12 g/dL and increased the percentage of hemoglobin A to at least 70% of the total hemoglobin mass. The details of this preoperative transfusion regimen have been reported previously.5 All patients were hydrated intravenously at a rate 1.5 times maintenance beginning 12 hours prior to cholecystectomy or ERCP. Intravenous hydration was continued after these procedures until the patient was able to take oral fluids well. RESULTS
Patient Characteristics
There were 35 patients with sickle hemoglobinopathies who were identified with gallstones, 9 of whom (26%) had associated CBD stones (Table 1). There were 6 males and 3 females. Eight of the patients were homozygous for the sickle cell gene (Hb SS), and one patient had Hb S in association with S-p thalassemia. The patients ranged in age from 8 to 23 years (mean, 15 years; median, 15 years). No patient had direct evidence for sepsis or cholangitis when the diagnosis of CBD stones was established. Diagnostic Evaluation for CBD Stones
All patients had total bilirubin levels measured prior to ERCP or cholecystectomy (Table 2). The total bilirubin levels ranged from 1.6 to 42 mg/dL Journal of Pediatric Surgery,
Vol27. No
5
(May), 1992: pp 572-575
COMMON
BILE DUCT STONES
573
Table 1. Hemoglobin Phenotype, Sex, and Age of Patients With Sickle Hemoglobinopathies
Table 3. Imaging Studies Performed on Patients Wiih Common Bile
and Common Bile Duct Stones
Patient
Hemoglobin
NO.
Phenotype
Duct Stones Hepatobiliary
Age Iv)
Sex
1
ss
F
8
2
ss
F
16
3
ss
F
19
4
ss
M
12
5
ss
M
6
ss
M
Ultrasound
Bladder
Common BileDuct
1
Stones
Dilated,
13
2
Stones
WNL
WNL(a)
Stones
15
3
Sludge
Dilated
Stones(b)
WNL
Patient
NO.
Gall
ERCP ND
IOC Stones
stones
7
SS
M
15
4
ND
ND
ND
Stones
6
ss
M
23
5
Stones
WNL
ND
Stones
9
S Sp
M
18
6
Stones
WNL
ND
Stones
7
Stones
WNL
ND
Stones
8
Stones
Stones
Stones(b)
WNL
9
Stones
Dilated,
Stones(b)
WNL
Thal
(mean, 15.9; median, 7.8 mg/dL). The direct bilirubin level was elevated in 2 patients, normal in 2 patients, and not measured in 5 patients (Table 2). Only modest increases in alkaline phosphatase, alanine aminotransferase, and amylase were noted in these patients. Eight patients had hepatobiliary ultrasonography performed prior to cholecystectomy or ERCP (Table 3). Only 3 (38%) were reported to have CBD stones, 2 of whom also had dilated biliary ducts, 1 had a dilated CBD duct with no demonstrable CBD stones, and 4 (50%) had a normal biliary system. The 9th patient did not have a preoperative sonogram, but had numerous gallstones visualized by an abdominal radiograph. IOC was performed routinely at the time of cholecystectomy. In 6 patients, the IOC demonstrated CBD stone(s) at the ampulla (Table 3). The IOC confirmed passage of the CBD stones in the 3 patients who had ERCP and endoscopic sphincterotomy prior to cholecystectomy. ERCP was performed prior to cholecystectomy on 3 patients and after surgery on 1 patient (Table 3). In all instances when ERCP was performed prior to surgery, CBD stones were demonstrated. Ultrasonography had demonstrated CBD stones in 2 of these patients and a dilated CBD in the other. The CBD Table 2. Laboratory Data on Patients With Common Bile Duct Stones Alanine Total
Direct
Alkaline
Amino-
Patient
Bilirubin
Bilirubin
Phosphatase
transferase
Amylase
NO.
(mg/dL)
(mg/dL)
w/u
(U/L)
(U/L)
1
34.0
23
222
ND
ND
2
15.6
ND
49
45
36
3
7.8
ND
100
33
228
4
3.3
ND
130
32
ND
5
1.6
and Management of Common Bile Duct Stones in Patients With Sickle Hemoglobinopathies By Russell E. Ware, William H. Schultz, Howard C. Filston, and Thomas Durham, North Carolina and Knoxville, Tennessee
0 Nine of 35 patients with sickle hemoglobinopathies and cholelithiasis were found to have concomitant common bile duct (CBD) stones. We describe the diagnosis and management of these patients with CBD stones. Historical information, physical examination, and routine laboratory tests were unable to identify accurately the patients with CBD stones. Similarly, ultrasonography of the hepetobiliary system was an insensitive method for detecting CBD stones, as only 3 of 8 patients were correctly identified. In contrast, both an intraoperative cholangiogram and endoscopic retrograde cholangiopancreatography were sensitive procedures for detection of CBD stones. We conclude that CBD stones are relatively common in patients with sickle cell disease and cholelithiasis, and clinicians should have a high index of suspicion for their presence. Copyright o 1992 by W.B. Saunders Company INDEX WORDS: Choledocholithiasis; cholelithiasis; sickle cell disease; intraoperative cholangiography; endoscopic retrograde cholangiopancreatography.
T
HE PRINCIPAL manifestations of sickle cell disease include chronic hemolytic anemia and both acute and chronic tissue damage secondary to vasoocclusion produced by the abnormally shaped red blood cells.’ The chronic hemolysis predisposes affected individuals to pigmented cholelithiasis.2,3 Symptoms of cholelithiasis may be difficult to distinguish from those produced by intraabdominal vasoocclusion.4 Once gallstones are identified in these patients, cholecystectomy should be performed to reduce both morbidity and mortality from complications associated with cholelithiasis and emergency cholecystectomy.5Jj Despite the high frequency of gallstones in the sickle cell population, the diagnosis and treatment of
From the Department of Pediatrics, Duke University Medical Center, Durham, NC, and the Department of Surgery, University of Tennessee, Knoxville, TN. Date accepted: February 22, 1991. Supponed in part by a grant (PO 60-HL-28391) from the National institute of Heart, Lung, and Blood Diseases, Sickle Cell Disease Branch, National Institutes of Health. R. W. is the recipient of Physician Scientist Award (Kll-HLO2015) from the National Institutes of Health. Presented in part at the 15th National Sickle Cell Centers Meeting in Oakland, CA. Address reprint requests to Russell E. Ware, MD, PhD, Comprehensive Sickle Cell Center, PO Box 2916, Duke lJniversi@ Medical Center, Durham, NC 27710. Copyright o 1992 by W.B. Saunders Company 0022-3468/9212705-0009$03.00/0 572
R. Kinney
choledocholithiasis in these patients has not been addressed. In this paper, we summarize our experience with the identification and management of common bile duct (CBD) stones in nine patients. We emphasize the usefulness of intraoperative cholangiography (IOC), endoscopic retrograde cholangiopancreatography (ERCP), and endoscopic sphincterotomy. MATERIALS
AND METHODS
Patient Population We reviewed the records of all patients on the pediatric service with sickle cell disease and gallstones treated at Duke University Medical Center from 1977 through 1989. Data were abstracted pertaining to the clinical history, diagnosis, and management of patients with both cholelithiasis and choledocholithiasis.
Imagkg Procedures Ultrasonography of the gallbladder and biliary system was performed using real-time scanning. IOC was done through a catheter placed in the cystic duct at the time of cholecystectomy.
Patient Preparation for Procedures Prior to cholecystectomy, each patient received several packed erythrocyte transfusions that increased the hemoglobin concentration to between 10 and 12 g/dL and increased the percentage of hemoglobin A to at least 70% of the total hemoglobin mass. The details of this preoperative transfusion regimen have been reported previously.5 All patients were hydrated intravenously at a rate 1.5 times maintenance beginning 12 hours prior to cholecystectomy or ERCP. Intravenous hydration was continued after these procedures until the patient was able to take oral fluids well. RESULTS
Patient Characteristics
There were 35 patients with sickle hemoglobinopathies who were identified with gallstones, 9 of whom (26%) had associated CBD stones (Table 1). There were 6 males and 3 females. Eight of the patients were homozygous for the sickle cell gene (Hb SS), and one patient had Hb S in association with S-p thalassemia. The patients ranged in age from 8 to 23 years (mean, 15 years; median, 15 years). No patient had direct evidence for sepsis or cholangitis when the diagnosis of CBD stones was established. Diagnostic Evaluation for CBD Stones
All patients had total bilirubin levels measured prior to ERCP or cholecystectomy (Table 2). The total bilirubin levels ranged from 1.6 to 42 mg/dL Journal of Pediatric Surgery,
Vol27. No
5
(May), 1992: pp 572-575
COMMON
BILE DUCT STONES
573
Table 1. Hemoglobin Phenotype, Sex, and Age of Patients With Sickle Hemoglobinopathies
Table 3. Imaging Studies Performed on Patients Wiih Common Bile
and Common Bile Duct Stones
Patient
Hemoglobin
NO.
Phenotype
Duct Stones Hepatobiliary
Age Iv)
Sex
1
ss
F
8
2
ss
F
16
3
ss
F
19
4
ss
M
12
5
ss
M
6
ss
M
Ultrasound
Bladder
Common BileDuct
1
Stones
Dilated,
13
2
Stones
WNL
WNL(a)
Stones
15
3
Sludge
Dilated
Stones(b)
WNL
Patient
NO.
Gall
ERCP ND
IOC Stones
stones
7
SS
M
15
4
ND
ND
ND
Stones
6
ss
M
23
5
Stones
WNL
ND
Stones
9
S Sp
M
18
6
Stones
WNL
ND
Stones
7
Stones
WNL
ND
Stones
8
Stones
Stones
Stones(b)
WNL
9
Stones
Dilated,
Stones(b)
WNL
Thal
(mean, 15.9; median, 7.8 mg/dL). The direct bilirubin level was elevated in 2 patients, normal in 2 patients, and not measured in 5 patients (Table 2). Only modest increases in alkaline phosphatase, alanine aminotransferase, and amylase were noted in these patients. Eight patients had hepatobiliary ultrasonography performed prior to cholecystectomy or ERCP (Table 3). Only 3 (38%) were reported to have CBD stones, 2 of whom also had dilated biliary ducts, 1 had a dilated CBD duct with no demonstrable CBD stones, and 4 (50%) had a normal biliary system. The 9th patient did not have a preoperative sonogram, but had numerous gallstones visualized by an abdominal radiograph. IOC was performed routinely at the time of cholecystectomy. In 6 patients, the IOC demonstrated CBD stone(s) at the ampulla (Table 3). The IOC confirmed passage of the CBD stones in the 3 patients who had ERCP and endoscopic sphincterotomy prior to cholecystectomy. ERCP was performed prior to cholecystectomy on 3 patients and after surgery on 1 patient (Table 3). In all instances when ERCP was performed prior to surgery, CBD stones were demonstrated. Ultrasonography had demonstrated CBD stones in 2 of these patients and a dilated CBD in the other. The CBD Table 2. Laboratory Data on Patients With Common Bile Duct Stones Alanine Total
Direct
Alkaline
Amino-
Patient
Bilirubin
Bilirubin
Phosphatase
transferase
Amylase
NO.
(mg/dL)
(mg/dL)
w/u
(U/L)
(U/L)
1
34.0
23
222
ND
ND
2
15.6
ND
49
45
36
3
7.8
ND
100
33
228
4
3.3
ND
130
32
ND
5
1.6
