Cholelithiasis in Children With Sickle Cell Disease Gungor Karayalcin, MD;
Nasser
Hassani, MD; Martin Abrams, MD; Philip Lanzkowsky,
\s=b\ A group of 47 children with homozygous sickle cell disease ranging in age from 2 to 18 years was studied for the prevalence of gallstones. All of these patients had oral cholecystogram and cholecystosonogram. Eight of the 47 patients (17%) had gallstones both on oral cholecystography and on cholecystosonography. These eight patients had a history of recurrent abdominal pain usually localized to the right upper quadrant. All were admitted on several occasions for sickle cell abdominal crises and four of these were admitted for acute hepatic crisis. These patients have undergone elective cholecystectomy and gallstones were found in every patient. The patients have been followed up from seven to 17 months after cholecystectomy and none have had abdominal symptoms or required hospitalization for abdominal crises. (Am J Dis Child 133:306-307, 1979)
carried out to determine the incidence and clinical manifestations of cholelithia-
The
present study
was
From the Division of Pediatric Hematology\x=req-\ Oncology, Department of Pediatrics, Long Island Jewish-Hillside Medical Center (Drs Karayalcin and Lanzkowsky), New Hyde Park, NY, and the Departments of Pediatrics (Drs Karayalcin and Lanzkowsky), Radiology (Dr Hassani), and Surgery (Dr Abrams), School of Medicine of the Health Sciences Center, State University of New York, Stony Brook. Reprint requests to Department of Pediatrics, Long Island Jewish-Hillside Medical Center, New Hyde Park, NY 11040 (Dr Lanzkowsky).
sis in children with sickle cell disease, to evaluate the diagnostic role and accuracy of cholecystosonography, and to assess the results of cholecys¬ tectomy in children with cholelithia¬ sis. SUBJECTS AND METHODS A group of 47 children with homozygous sickle cell disease were attending the pedi¬ atrie sickle cell clinic. Their ages ranged from 2 to 18 years (mean of 8.7 years). Although many of these patients had a history of abdominal crises, none had been suspected of having gallstones. All patients had oral cholecystograms obtained and in two patients in whom the gallbladder could not be seen on a double dose of contrast material, intravenous cholangiography was performed. Cholecystosonography was per¬ formed in all patients. Younger children required sedation for satisfactory chole¬ cystosonography. The children were scan¬ ned with a commercially available Greyscale ultrasound unit using a 3.25-MHz focused transducer, with mineral oil cou¬ pler. The patients were scanned while fast¬ ing and in the supine position. With the patient in deep inspiration, longitudinal and transverse scans were obtained of the right upper quadrant. Interpretations of the ultrasonograms were made without knowledge of the roentgenographic find¬ ings. An informed consent was obtained from the parents for these studies.
RESULTS
Oral
cholecystography eight of the
stones in
showed 47
Downloaded From: http://archpedi.jamanetwork.com/ by a UQ Library User on 06/15/2015
gall¬ patients
MD
(Table). Two
were
nonopaque and six
Cholecystosonogram showed calculi in all of these patients. Two of the 47 patients had no visual¬ ization on oral cholecystogram and both the intravenous cholangiogram and cholecystosonogram were nor¬ were
opaque.
mal. The eight patients with gallstones had a total of 38 hospital admissions (range, two to 12 admissions per patient) for severe abdominal crises in the five years prior to cholecystecto¬ my. In addition, they experienced bouts of right upper quadrant abdom¬ inal pain varying in severity from dull "gas" pain to sharp colicky pain, last¬ ing from a few hours to 48 hours. The frequency of these pains was from four to 36 times a year. Four of these patients were also admitted to the hospital for episodes consisting of
right upper quadrant pain, enlargement of liver, and direct hyperbilirubinemia ("liver crisis"). All eight patients underwent elec¬ tive cholecystectomy. They all had preoperative partial exchange trans¬ fusion to reduce the S hemoglobin severe
level to below 40% to minimize the
complications that may occur during operative and postoperative periods in patients with sickle cell disease.1 Gall¬ stones were found in each patient. In three of the four patients who previously had liver crises, calculi
Clinical Summary for Eight Patients With Sickle Cell Disease With Cholelithiasis
Age, yr/Sex
Cholecystogram Opaque stones Opaque stones
Oral
4/F
6/M
Nonopaque
10/F
Cholecystosonogram
Clinical Status
Positive Positive
Symptomatic Symptomatic*
Surgery Cholecystectomy Cholecystectomyt and choledochotomy
Positive
Symptomatic*
Cholecystectomyt
stones
and
choledochotomy_
14/F 16/M 16/F 17/F
Opaque stones Opaque stones Nonopaque stones Opaque stones
Positive Positive Positive Positive
Symptomatic Symptomatic Symptomatic* Symptomatic*
18/M
Opaque
Positive
Symptomatic
stones
Cholecystectomy Cholecystectomy Cholecystectomy Cholecystectomy and choledochotomy Cholecystectomy
Outcome and Period of Follow-up, mo Asymptomatic, 17 Asymptomatic, 13
Asymptomatic, 14
Asymptomatic, Asymptomatic, Asymptomatic, Asymptomatic,
8
Asymptomatic,
15
12 11 7
"Patient also had liver crisis. also had stones in common duct.
tPatient were
also found in the
common
duct.
Choledochotomy as well as cholecys¬ tectomy was performed in these patients. The patients have been followed from
seven
to 17 months since chole¬
cystectomy and none have had any abdominal symptoms or required hos-
pitalization for abdominal crises. COMMENT
Cholelithiasis is
a
well-recognized
in patients with sickle cell disease; the prevalence increases with age. The real incidence in chil¬ dren remains somewhat controversial. The present study indicates that eight of 47 (17%) of our patients had gall¬ stones. In children 10 years or older, gallstones occurred in six of 21 chil¬ dren (28%). These results are substan¬ tially higher than that reported by Mintz et al2 who showed a 10% inci¬ dence of gallstones in children aged 3
complication
to 16 years with sickle cell disease and Phillips and Gerald' who demon¬ strated a 17% incidence in children 10 to 19 years of age. Symptomatic biliary tract disease in children with sickle cell anemia has been considered by many to be unusual. However, this report indi¬ cates that symptomatic biliary tract disease is common and that common duct obstruction by gallstones may
frequently. Furthermore, the patients who were incorrectly diagnosed as having liver crisis, were occur
three
found to have common duct obstruc¬ tion. Since it is difficult to distinguish between painful abdominal sickle crises and gallbladder disease, all patients who present with frequent abdominal crises, especially those old¬ er than 10 years of age, should have routine oral cholecystography. Al¬ though cholecystography is still the
procedure of choice in detecting gall¬ stones,' cholecystosonography is of
value in cases of nonvisualization of the gallbladder or when patients are unable to tolerate oral or intravenous cholecystography. This study and pre¬ vious work46 confirm the accuracy of cholecystosonography and its useful¬ ness under these circumstances. Since the incidence of severe symptoms and or complications of cholelithiasis is about 50% with an overall mortality of 2.7% within the first five years in unoperated patients,7 the presence of gallstones in children with sickle cell disease is an indication for elective
cholecystectomy. This study was supported by grant 3-792 by the Long Island Jewish-Hillside Medical Center. Anju Khanijou, MD, Young Ju Kim, MD, and Alfredo Kalafatie, MD, provided clinical assist¬ ance. Frieda Ruderman typed this manuscript, and Sonia Suga provided technical assistance in ultrasonography.
References 1. Lanzkowsky P, Shende A, Karayalcin G, et al: The use of partial exchange transfusion in children with serious complications of sickle cell anemia. Am J Dis Child, to be published. 2. Mintz AA, Church G, Adams ED: Cholelithiasis in sickle cell anemia. J Pediatr 47:171-177, 1955. 3. Phillips J, Gerald EB: The incidence of
cholelithiasis in sickle cell disease. Am J Roent-
genol 113:27-28, 1971. 4. Goldberg BB, Harris K, Brocker W: Ultrasonic and radiographic cholecystography. Radiology 111:405-409, 1974. 5. Bartrum RJ, Crow HC, Foote SR: Ultrasound examination of gall bladder: An alternative to "double-dose" oral cholecystography.
Downloaded From: http://archpedi.jamanetwork.com/ by a UQ Library User on 06/15/2015
JAMA 236:1147-1148, 1976. 6. Lawson TL: Gray scale cholecystosonography: Diagnostic criteria and accuracy. Radiology 122:247-251, 1977. 7. Lund J: Surgical indication in cholelithiasis: Prophylactic cholecystectomy elucidated on the basis of long-term follow up on 526 nonoperated cases. Ann Surg 151:153-162, 1960.
Nasser
Hassani, MD; Martin Abrams, MD; Philip Lanzkowsky,
\s=b\ A group of 47 children with homozygous sickle cell disease ranging in age from 2 to 18 years was studied for the prevalence of gallstones. All of these patients had oral cholecystogram and cholecystosonogram. Eight of the 47 patients (17%) had gallstones both on oral cholecystography and on cholecystosonography. These eight patients had a history of recurrent abdominal pain usually localized to the right upper quadrant. All were admitted on several occasions for sickle cell abdominal crises and four of these were admitted for acute hepatic crisis. These patients have undergone elective cholecystectomy and gallstones were found in every patient. The patients have been followed up from seven to 17 months after cholecystectomy and none have had abdominal symptoms or required hospitalization for abdominal crises. (Am J Dis Child 133:306-307, 1979)
carried out to determine the incidence and clinical manifestations of cholelithia-
The
present study
was
From the Division of Pediatric Hematology\x=req-\ Oncology, Department of Pediatrics, Long Island Jewish-Hillside Medical Center (Drs Karayalcin and Lanzkowsky), New Hyde Park, NY, and the Departments of Pediatrics (Drs Karayalcin and Lanzkowsky), Radiology (Dr Hassani), and Surgery (Dr Abrams), School of Medicine of the Health Sciences Center, State University of New York, Stony Brook. Reprint requests to Department of Pediatrics, Long Island Jewish-Hillside Medical Center, New Hyde Park, NY 11040 (Dr Lanzkowsky).
sis in children with sickle cell disease, to evaluate the diagnostic role and accuracy of cholecystosonography, and to assess the results of cholecys¬ tectomy in children with cholelithia¬ sis. SUBJECTS AND METHODS A group of 47 children with homozygous sickle cell disease were attending the pedi¬ atrie sickle cell clinic. Their ages ranged from 2 to 18 years (mean of 8.7 years). Although many of these patients had a history of abdominal crises, none had been suspected of having gallstones. All patients had oral cholecystograms obtained and in two patients in whom the gallbladder could not be seen on a double dose of contrast material, intravenous cholangiography was performed. Cholecystosonography was per¬ formed in all patients. Younger children required sedation for satisfactory chole¬ cystosonography. The children were scan¬ ned with a commercially available Greyscale ultrasound unit using a 3.25-MHz focused transducer, with mineral oil cou¬ pler. The patients were scanned while fast¬ ing and in the supine position. With the patient in deep inspiration, longitudinal and transverse scans were obtained of the right upper quadrant. Interpretations of the ultrasonograms were made without knowledge of the roentgenographic find¬ ings. An informed consent was obtained from the parents for these studies.
RESULTS
Oral
cholecystography eight of the
stones in
showed 47
Downloaded From: http://archpedi.jamanetwork.com/ by a UQ Library User on 06/15/2015
gall¬ patients
MD
(Table). Two
were
nonopaque and six
Cholecystosonogram showed calculi in all of these patients. Two of the 47 patients had no visual¬ ization on oral cholecystogram and both the intravenous cholangiogram and cholecystosonogram were nor¬ were
opaque.
mal. The eight patients with gallstones had a total of 38 hospital admissions (range, two to 12 admissions per patient) for severe abdominal crises in the five years prior to cholecystecto¬ my. In addition, they experienced bouts of right upper quadrant abdom¬ inal pain varying in severity from dull "gas" pain to sharp colicky pain, last¬ ing from a few hours to 48 hours. The frequency of these pains was from four to 36 times a year. Four of these patients were also admitted to the hospital for episodes consisting of
right upper quadrant pain, enlargement of liver, and direct hyperbilirubinemia ("liver crisis"). All eight patients underwent elec¬ tive cholecystectomy. They all had preoperative partial exchange trans¬ fusion to reduce the S hemoglobin severe
level to below 40% to minimize the
complications that may occur during operative and postoperative periods in patients with sickle cell disease.1 Gall¬ stones were found in each patient. In three of the four patients who previously had liver crises, calculi
Clinical Summary for Eight Patients With Sickle Cell Disease With Cholelithiasis
Age, yr/Sex
Cholecystogram Opaque stones Opaque stones
Oral
4/F
6/M
Nonopaque
10/F
Cholecystosonogram
Clinical Status
Positive Positive
Symptomatic Symptomatic*
Surgery Cholecystectomy Cholecystectomyt and choledochotomy
Positive
Symptomatic*
Cholecystectomyt
stones
and
choledochotomy_
14/F 16/M 16/F 17/F
Opaque stones Opaque stones Nonopaque stones Opaque stones
Positive Positive Positive Positive
Symptomatic Symptomatic Symptomatic* Symptomatic*
18/M
Opaque
Positive
Symptomatic
stones
Cholecystectomy Cholecystectomy Cholecystectomy Cholecystectomy and choledochotomy Cholecystectomy
Outcome and Period of Follow-up, mo Asymptomatic, 17 Asymptomatic, 13
Asymptomatic, 14
Asymptomatic, Asymptomatic, Asymptomatic, Asymptomatic,
8
Asymptomatic,
15
12 11 7
"Patient also had liver crisis. also had stones in common duct.
tPatient were
also found in the
common
duct.
Choledochotomy as well as cholecys¬ tectomy was performed in these patients. The patients have been followed from
seven
to 17 months since chole¬
cystectomy and none have had any abdominal symptoms or required hos-
pitalization for abdominal crises. COMMENT
Cholelithiasis is
a
well-recognized
in patients with sickle cell disease; the prevalence increases with age. The real incidence in chil¬ dren remains somewhat controversial. The present study indicates that eight of 47 (17%) of our patients had gall¬ stones. In children 10 years or older, gallstones occurred in six of 21 chil¬ dren (28%). These results are substan¬ tially higher than that reported by Mintz et al2 who showed a 10% inci¬ dence of gallstones in children aged 3
complication
to 16 years with sickle cell disease and Phillips and Gerald' who demon¬ strated a 17% incidence in children 10 to 19 years of age. Symptomatic biliary tract disease in children with sickle cell anemia has been considered by many to be unusual. However, this report indi¬ cates that symptomatic biliary tract disease is common and that common duct obstruction by gallstones may
frequently. Furthermore, the patients who were incorrectly diagnosed as having liver crisis, were occur
three
found to have common duct obstruc¬ tion. Since it is difficult to distinguish between painful abdominal sickle crises and gallbladder disease, all patients who present with frequent abdominal crises, especially those old¬ er than 10 years of age, should have routine oral cholecystography. Al¬ though cholecystography is still the
procedure of choice in detecting gall¬ stones,' cholecystosonography is of
value in cases of nonvisualization of the gallbladder or when patients are unable to tolerate oral or intravenous cholecystography. This study and pre¬ vious work46 confirm the accuracy of cholecystosonography and its useful¬ ness under these circumstances. Since the incidence of severe symptoms and or complications of cholelithiasis is about 50% with an overall mortality of 2.7% within the first five years in unoperated patients,7 the presence of gallstones in children with sickle cell disease is an indication for elective
cholecystectomy. This study was supported by grant 3-792 by the Long Island Jewish-Hillside Medical Center. Anju Khanijou, MD, Young Ju Kim, MD, and Alfredo Kalafatie, MD, provided clinical assist¬ ance. Frieda Ruderman typed this manuscript, and Sonia Suga provided technical assistance in ultrasonography.
References 1. Lanzkowsky P, Shende A, Karayalcin G, et al: The use of partial exchange transfusion in children with serious complications of sickle cell anemia. Am J Dis Child, to be published. 2. Mintz AA, Church G, Adams ED: Cholelithiasis in sickle cell anemia. J Pediatr 47:171-177, 1955. 3. Phillips J, Gerald EB: The incidence of
cholelithiasis in sickle cell disease. Am J Roent-
genol 113:27-28, 1971. 4. Goldberg BB, Harris K, Brocker W: Ultrasonic and radiographic cholecystography. Radiology 111:405-409, 1974. 5. Bartrum RJ, Crow HC, Foote SR: Ultrasound examination of gall bladder: An alternative to "double-dose" oral cholecystography.
Downloaded From: http://archpedi.jamanetwork.com/ by a UQ Library User on 06/15/2015
JAMA 236:1147-1148, 1976. 6. Lawson TL: Gray scale cholecystosonography: Diagnostic criteria and accuracy. Radiology 122:247-251, 1977. 7. Lund J: Surgical indication in cholelithiasis: Prophylactic cholecystectomy elucidated on the basis of long-term follow up on 526 nonoperated cases. Ann Surg 151:153-162, 1960.
