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Cholelithiasis in Infants, Children, and

Adolescents
George W. Holcomb, Jr, MD,* and George W. Holcomb, In, MDt

hemolytic disease is a necessary pre-


The questions below should help requisite for gallstone formation in
EDUCATIONAL OBJECTIVE
focus the reading of this article. children. In 1 966, our report1 called
1. What factors dispose to choleli- attention to the increased number of 90. The pediatrician should have
thiasis in infancy, childhood, and ad- children and teenagers with nonhem- an appropriate recollection of the
olescence? ohytic chohehithiasis and unexplained problems associated with cholell-
2. What clinical features of illness in abdominal symptoms. Since that thiasis and methods of diagnosis
infants should prompt consideration time, more children and adolescents by ultrasonography (Recent Ad-
of cholelithiasis in the differential di- have been found to have gallstones vances, 89/90).
agnosis? according to reports from this country
3. What diagnostic studies are indi- and Europe. Many gallstones of non-
cated for infants, children, or adoles- hemolytic origin are idiopathic, in ad-
cents in whom a diagnosis of chole- dition to those that develop in relation
lithiasis is suspected? to the risk factors.
4. What features of cholelithiasis sug-
gest a need for surgical intervention? TPA, and anatomic anomalies of the
CHOLELITHIASIS IN THE
bihiary system.
ABSENCE OF HEMOLYTIC
The presence of gallstones was
DISEASE
diagnosed in six prematurely born 5-
Although chohehithiasis has been Although a precise classification of to 30-week-old infants weighing less
thought to occur infrequently in the nonhemolytic cholehithiasis cannot be than 1600 g.3’4 These infants had re-
pediatric age group, its incidence has made because of some overlapping ceived TPA for 3 to 10 weeks and
increased during the past two dec-
characteristics, a general grouping each had respiratory distress syn-
ades. Pediatricians and pediatric sur- relative to age is helpful. Accordingly, drome. All but one of these babies
geons now should consider gall- gallstones occurring in infancy (birth had radiopaque stones. Pellerin et a16
stones in the differential diagnosis for to 2 years), childhood (2 to 13 years), described the cases of two infants
every child or adolescent with vague and adolescence (1 4 to 1 6 years) will and two 7-year-old children who had
or colicky upper abdominal pain, and be discussed. not received TPA infusion but who
particularly for those with associated had bihiary calculi following extensive
risk factors (Table). In addition to the small bowel resection. Boyle et a17
factors shown in the Table, chohehi- Infancy (Birth to 2 Years of Age)
described a 3-week-old premature in-
thiasis may result from hemolytic dis- Although rare, calculi are being de- fant in whom gallstones developed in
eases such as congenital spherocy- tected in infants in greater numbers the absence of bowel resection or of
tosis, sickle cell disease, or thalas-
than in the past, largely because of cholestatic jaundice. This infant was
semia.
the frequent use of ultrasonography. treated for only 13 days with periph-
In 1971 , attention was called to the eral venous infusion of amino acids
complication of hepatobihiary dys- and 1 1 days with intravenous lipid.
Approximately 80% of function with cholestasis and cirrho- However, in one 6-week-old infant
gallstones in children are not sis in a patient receiving total paren- and another 6-day-old neonate (nei-
due to hemolytic disease; the teral ahimentation (TPA). Subse- ther with any risk factors or hemolytic
remaining 20% are related to quently, this dysfunction was noted diseases) idiopathic stones, perfora-
recurring hemolysis. to be more severe in low birth weight tion of the gallbladder, and bile pen-
babies. Benjamin2 reported observa- tonitis developed.5 Thus, it seems
tions at the time of either autopsy or that factors other than TPA or intes-
One reason that gallstones were liver biopsy in 15 patients with cho- tinal resection may be involved in the
not considered as a possible cause hestasis who had received TPA. He pathogenesis of gallstones in some
of jaundice or right upper quadrant concluded that hepatobihiary dysfunc- infants.
discomfort in the past may have been tion is common when TPA is used for The major similarity between in-
the emphasis placed on the belief that more than 60 days. He also empha- fants in whom cholehithiasis develops-
sized that histologic changes of cho- following ileal resection and those re-
lestasis may mimic extrahepatic bihi- ceiving TPA therapy is the loss of
‘Clinical Professor of Pediatric Surgery, Van- ary atresia and may progress to cir- normal gastrointestinal function with
derbilt University School of Medicine, Nash- rhosis. Since then, gallstones have development of bihiary stasis and dis-
ville, TN.
been found to occur in association appearance of the normal entero-
tAssistant Professor of Pediatric Surgery, Van-
derbilt University School of Medicine, Nash- with extensive small bowel resection, hepatic circulation of bile salts.
ville, TN. necrotizing enterocolitis, long-term Benjamin2 concluded that intrave-

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GASTROINTESTINAL DISEASE

TABLE. Risk Factors Associated With Nonhemolytic Cholehithiasis


Infants and Children Adolescents
Total parenteral ahimentation Total parenteral ahimentation
Gallbladder stasis, starvation Gallbladder stasis, starvation
Dehydration, vomiting, furosemide Dehydration, vomiting
medication Cholestasis, cirrhosis
Prematurity, cholestasis, cirrhosis Ileal resection: Crohn disease,
Ileal resection: necrotihizing entero- trauma
colitis, volvuluv, trauma Pregnancy, oral contraceptives
Hyahine membrane disease, broncho- Obesity
pulmonary dysplasia Sex, racial and genetic influences
Polycythemia, possible phototherapy Hemobihia, liver trauma
Bihiary tract anomalies, choledochal Prolonged immobilization, spinal fu-
cyst, stenosis, diverticuha sion

nous amino acids and lipids probably difficult to evaluate. Hyperalimenta-


play only a minor roll in chohestasis. tion with development of jaundice is Fig 1. Radiopaque gallstones in 18-month-old
Thus, it is becoming more apparent often associated with temporary cho- girl following several surgical procedures for
that the use of the term TPA-induced lestasis. However, when direct hy- repair of omphalocele and bladder extrophy.
cholelithiasis should be replaced by perbihirubinemia is persistent, bihiary
TPA-associated hithiasis. atresia or common duct obstruction
is also frequently encountered. This
with gallstones would also be consid-
is a fluid substance consisting of cal-
ered. Although the frequency of neo-
cium bihirubinate, which layers in the
natal sepsis in general and of infec-
The major similarity between dependent portion of the gallbladder,
tion related to venous catheters in
infants with cholehithiasis resulting in low-amplitude echoes.
particular suggest that sepsis is a
following ileal resection and Sludge often can be distinguished
likely explanation for fever and icte- from multiple small gallstones by the
those receiving total
rus, the possibility of gallstones still
parenteral ahimentation acoustic shadowing effect of the lat-
should be considered carefully when
therapy is the loss of normal ter. However, this differential finding
jaundice is prolonged. is not always distinguishable, and
gastrointestinal function with
Most gallstones in infants and
development of biliary stasis false interpretations are rendered on
young children are composed primar-
and disappearance of normal occasion.
ily of calcium bihirubinate pigment with
enterohepatic circulation of
varying amounts of cholesterol and
bile salts.
calcium carbonate. Although scintig-
raphy is not helpful in detecting gall- Radiopaque stones are
stones, it is beneficial in evaluating evident on plain
Diagnostic Considerations. The di- the functional status of the bihiary roentgenograms, but the
agnosis of cholehithiasis in an infant tract, diagnosing cholecystitis, and most reliable and most
receiving TPA may be difficult to es- demonstrating ductal obstruction and readily accessible diagnostic
malformations. However, the results study for nonopaque calculi
of technetium-labeled paraisopropyl- is abdominal
acetanihidoiminodiacetic acid (PIP- ultrasonography.
Routine ultrasonographic IDA) scintigraphy may be confusing,
evaluation should be and false indications of gallbladder
obtained at intervals for all disease may be found in fasting pa- During a 1 -year period, Matos and
children who received total tients without bihiary disease because associates8 performed a prospective
parenteral ahimentation for of nonvisuahization. study of the bihiary tract in 41 neo-
more than 4 weeks, Although radiopaque stones will be nates using ultrasonography. Gall-
particularly those with ileal evident on roentgenograms (Fig 1), bladder sludge appeared in 18 of 41
resection or with chronic the most reliable and most readily (44%) of those infants who had re-
enteritis accessible confirmatory study for cal- ceived TPA infusion for a mean period
(Crohn disease). culi is abdominal ultrasonography. It of 10 days. In 5 (12%), an evolution
is important that the presence of of sludge to “sludge balls” was ob-
movable, echogenic structures within served. In 2 infants (5%), uncompli-
tablish, primarily because cholehi- the gallbladder be identified, usually cated gallstones developed. Sponta-
thiasis is not commonly considered in with shadowing, to confirm the pres- neous resolution occurred in one of
the differential diagnosis in this age ence of gallstones (Fig 2). With the these infants by 6 months after the
group and because symptoms are so use of real-time sonography, “sludge” examination; calculi persisted in the

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Cholehithiasis

cystokinin when enteral fluids cannot


be given. We are not aware of cho-
lecystokinin being used for this pur-
pose in infants, although it has had
some success in adults. Variations in
the concentrations of parenteral
amino acids, fats, and glucose sohu-
tions are not thought to be beneficial
in reducing gallstone formation.
Several observers have reported
spontaneous resolution of gallstones
without treatment, from 2 weeks to
6 months following the initial discov-
ery. Also, multiple echogenic struc-
tures, presumably fetal gallstones,
have been seen on sonograms at 37
weeks’ gestation with confirmation
after birth and disappearance at 6
weeks of age.1#{176}
These apparent gall-
stones may actually have been
acoustic sludge balls that disap-
Fig 2. Real-time sonogram demonstrates several echogenic gallstones with distal shadowing in peared when enteral feedings were
16-month-old girl with leukemia. instituted. Because some of these
acoustic sludge balls will progress to
the caloric intake is administered en- calculus formation, ultrasonographic
other baby. Matos and associates
monitoring is advised.
found that sludge develops more rap- terally as soon as possible to stimu-
late gallbladder contraction. Theoret- At Vanderbilt Children’s Hospital,
idly in neonates than in adults after a
we advise chohecystectomy for symp-
mean time of TPA infusion in neo- ically, prevention of sludge formation
could be achieved by stimulating gall- tomatic infants and young children.
nates of 10 days, as compared with
Also, we recommend cholecystec-
more than 6 weeks in adults. This
tomy for the asymptomatic child with
finding is probably related to the fact
TPA-associated gallstones if echo-
that neonates, and especially pre-
The key to management of genic shadows have been present for
mature infants, are particularly sus-
infants is awareness by the at least 1 2 months following resump-
ceptible to the cholestatic effect of
TPA because of the immaturity of the pediatrician and tion of oral feedings or when the gall-
neonatologist that total stones are radiopaque. In each situ-
enterohepatic circulation of bile salts.
Bihiary stasis is thought to be an im- parenteral ahimentation, ation, it is unlikely either will resolve
dehydration, bowel resection, spontaneously. Complications of
portant factor in the development of
polycythemia, and the use of common bile duct obstruction, pan-
gallstones, and sludge is likely to be
the stimulating event. There certainly furosemide without enteral creatitis, perforation with bile perito-
feedings may lead to the nitis, and life-threatening sepsis may
seems to be a progression from bile
development of gallstones. thus be prevented.
stasis to sludge to calcium bihirubi-
nate gallstone formation in some pa-
Children (2 to 13 Years of Age)
tients.
Clinical Management. The key to Gallstones in this age group pri-
management of infants is awareness manly are composed of mixtures of
by the pediatrician and neonatologist Prevention of biliary calculus calcium bihirubinate (pigment) with
that TPA, dehydration, bowel resec- formation is important. varying amounts of calcium carbon-
tion, pohycythemia, and the use of Gallbladder stasis and ate and cholesterol (Fig 3). The diag-
furosemide when enteral feedings are gastroentenc dysfunction are nosis currently is best accomplished
withheld may lead to development of likely prime etiologic factors by ultrasonography, although some
gallstones. in the pathogenesis of opaque gallstones can be detected
Prevention of biliary calculus for- cholelithiasis in infants and on plain roentgenograms.
mation in infants should be empha- children. Early resumption of During the past 6 years, the devel-
sized. It is likely that gallbladder enteral feedings may prevent opment of gallbladder disease with
stasis and gastroenteric dysfunction gallstone formation. gallstones has been emphasized as
are prime etiologic factors in the path- occurring with frequency in associa-
ogenesis of cholehithiasis in infants. tion with starvation and administra-
This suspicion prompted Ament9 to bladder contractions with intermittent tion of hyperahimentation. Roslyn and
suggest that cholehithiasis will de- oral feedings of fat or protein or by associates11 reported that 12 children
crease if as much as 10% to 15% of intravenous administration of chohe- (mean age 8.2 years) required chole-

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GASTROINTESTINAL DISEASE

or subscapular regions. The pain was tween 1 0% and 20%, reaching as


most often dull and intermittent, but high as 40% with advanced age.
one 5-year-old boy described right Diets that are high in cholesterol and
subcostal colicky pain. The duration low in fiber predominate in the West-
of symptoms varied from 10 days to em world, possibly contributing to the
6 years. higher occurrence of mixed choles-
Two girls, ages 9 and 1 1 years, terol stones.
were jaundiced and both had gall- Sex. The female to male ratio of
stones impacted in the ampulla of adults in whom gallstones develop is
Vater. Fatty food intolerance was re- generally accepted as 4:1 This ratio .

ported in only three of the older chil- is considerably higher in adolescents:


dren, two of whom were obese. Bihi- in Sweden, Soderlund and Zetter-
ary pancreatitis was discovered in strom reported a 19:1 ratio, and in
one 9-year-old girl and in one obese the United States, Odom et al5 found
13-year-old girl. One 13-year-old boy a ratio of 22:1 and Holcomb found a
with gallstones previously had had a radio of 14:1.
mesocaval shunt. The etiology of Pregnancy. As early as 1966, at-
these gallstones was unknown. tention was called to the frequent
Cholecystectomy was performed association of bihiary stones with
in each of these eight children, and teenage pregnancy.1 In a more recent
the procedure was well tolerated. study of 1 4- to 1 8-year-old girls with
Fig 3. Top, Blackish-green gallstones in 3-
There were no postoperative comphi- gallstones,13 45 (65%) were or had
year-old boy with upper abdominal pain. His- cations except for the recurrence of been pregnant, and none of these
tory of weighing 1400 g at birth, necrotilizing a common duct stone in one child 9 teenagers had hemolytic anemia.
enterocolitis, bowel resection, total parenteral years after removal of common duct Gallstones have been produced ex-
nutrition and no hemolytic diseases. Bottom,
stones and cholecystectomy. perimentally in rabbits by the pro-
Higher magnification of stones. Although dark
gross appearance suggested bilirubinate longed injection of progesterone- and
stones, they were 100% calcium carbonate. Adolescents (14 to 16 Years of estradiol-simulating hormones of pla-
From Holcomb,5 reproduced with permission. Age) cental origin.
In this age group, the symptoms Obesity. It has been suspected that
obesity in adolescents predisposes to
and physical findings are similar to
cystectomy, the youngest being a 9- those generally found in adults. Pain the formation of cholesterol calculi.
month-old boy who received TPA is usually easily described by the pa- Body weight in excess of 1 0% of
since soon after birth following duo- tient as being subcostal in location normal for height and age has been
denal trauma. Most of these young and sometimes with radiation around found in 33% of teenagers with gall-
children had short bowel syndrome the ribs to the subscapular area. stones. Neither hyperlipidemia nor
secondary to ileocohic resection. The Complaints of nausea and vomiting hyperchohesterolemia seems to be an
diagnosis of cholehithiasis was often may occur and intolerance to fats or associated factor in production of
delayed in spite of abdominal pain, greasy foods may be mentioned. gallstones in persons younger than
nausea, emesis, and fever. The prob- 1 6 years of age. Although the precise
hem initially suspected in 5 of the 7 mechanism of gallstone formation is
Etiology and Risk Factors
children less than 6 years of age was not known, obesity, gallbladder
venous catheter sepsis. King and Demographic Features. The mci- stasis, dehydration, and pregnancy
associates12 also reported cholehi- dence of cholehithiasis varies from seem to accentuate the hereditary
thiasis developing in 11 of 84 children country to country and with different predisposition in female adolescents.
(1 3%)who received hyperahimentation races and sexes. Nonhemolytic cho- Billary Pancreatitis. Pancreatitis
for more than 4 weeks. Roslyn et ah lehithiasis is less common in American secondary to passage of small gall-
advised early elective cholecystec- black children. Also, the African Ma- stones down the common duct and
tomy in any child with chohehithiasis sai secrete only half-saturated bile obstruction at the sphincter of Oddi
who received hyperahimentation. and bihiary calculi rarely develop. In is an uncommon development in
In a previous study reported in contrast, 5% to 8% of adolescent childhood. Indeed, one review14 of
1980,13 we reviewed the history and girls and 25% to 30% of young pancreatitis in 30 children indicated
findings in eight 5- to 1 3-year-old chil- women in Chile have gallstones re- no instances of bihiary pancreatitis.
dren with nonhemolytic gallstones sulting from cholesterol hypersecre- On the other hand, in the Vanderbilt
(mean age 10.6 years). The initial tion. In Arizona, the female Pima In- Children’s Hospital study, 6 adoles-
complaint in each child was abdomi- dians secrete supersaturated bile cent girls between 13 and 18 years
nal pain. Location of the pain varied and, as they grow older, the inci- of age were found to have bihiary
from epigastric to vague abdominal dence of cholesterol stones in- pancreatitis. The acute pancreatitis
sites in younger children. The older creases to 80%. The occurrence of was allowed to subside, after which
patients usually were more specific in gallstones among white adults in the cholecystectomy was performed and
localizing pain to the right subcostal United States and Europe varies be- gallstones were found in each gall-

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Cholehithiasis

bladder (Fig 4). These patients re- sidered in the differential diagnosis. lecystitis, especially with obstruction
covered without complications. In one study,5 disease of the gallblad- of the cystic duct. Cholescmntigraphy
Congenital Deformities. Formation der was suspected initially in only is also effective in determining pa-
of gallstones has been observed in one-half of the children (30 of 60), tency of the common duct in the pres-
association with stenosis of the cystic whereas in the other half, as many as ence of jaundice, detecting congeni-
duct, choledochal cysts, and divertic- 10 years elapsed before the correct tal choledochal cysts, and assessing
ula of the gallbladder or the cystic diagnosis was made. Also, it has trauma.
duct. Lilly15 reported primary com- been reported that a 4-year-old girl
mon duct gallstones without cholehi- and a teenager with undiagnosed ab-
Treatment
thiasis which developed in four chil- dominal pain were referred for psy-
dren with chronic liver diseas’ or with chiatric guidance. Both were hater Nonoperative Treatment. Dissohu-
bihiary tract malformations. found to have bihiary colic, which tion of gallstones with high doses of
Pathology and Composition of Gall- gives additional emphasis to the dif- chenodeoxychohic and/or ursodeox-
stones. Most idiopathic gallstones in ficulty of this diagnosis. ychohic acids, given orally, is undergo-
older children are composed of vary- Physical examination may indicate ing intensive study in adults. The suc-
ing proportions of pigment (calcium some tenderness in the right subcos- cess rate has been a disappointing
bihirubinate), calcium, and cholesterol. tal region. The temperature may be 13%. At this time, we are not aware
Cholesterol gallstones are soft, pale slightly elevated, as may be the leu- of its use in children.
yellow, and often small in size and kocyte count or the sedimentation Extracorporeal shock wave hitho-
occur infrequently in the pure form. rate. Jaundice is not often encoun- tripsy in combination with oral doses
Most cholesterol gallstones are of the tered but may occur with common of bile acids has been introduced as
mixed variety and occur in older chil- duct obstruction. Serum cholesterol a new nonsurgical therapy for disso-
dren and teenagers without hemo- and lipid levels are seldom elevated. lution of nonopaque gallstones. A re-
lytic diseases. The differential diagnosis should in- cent report from West Germany mdi-
Histiohogic evaluation of the gall- dude hepatitis, subhepatic appendi- cated 81 treatments were performed
bladder containing gallstones usually citis, pancreatitis, and chronic consti- for solitary and 24 treatments for mul-
reveals chronic chohecystitis, some- pation. A teenager with vague or in- tiple gallstones.16 The youngest pa-
times with thickening, and only rarely termittent right abdominal pain tient was 1 7 years of age. Gallstones
is acute cholecystitis evident. Occa- should have cholehithiasis listed in the disintegrated in all patients except
sionally, normal mucosa is found. differential diagnosis, particularly one and completely disappeared in
when one or more risk factors are 91 % by 12 to 1 8 months. Complica-
Diagnostic Considerations present. tions of pancreatitis developed in 2
Plain abdominal roentgenograms patients and gross hematuria in 3
In adolescents, as in young chil- may accurately show opaque gall- patients, while one third experienced
dren, cholehithiasis is not often con- stones, but lucent calculi must be bihiary colic. Further evaluation will be
diagnosed by other studies. Cur- necessary before this form of therapy
rently, the most universally used and is universally accepted, particularly
most accurate diagnostic aid in de- for the pediatric age group. In the
termining the presence of gallstones future, hithotripsy possibly will be
is ultrasonography. When the gall- used primarily for elderly persons and
bladder can be seen by ultrasonog- those adults who select this treat-
raphy, a gallstone discovery rate as ment. For children or teenagers with
high as 98% is expected. False-pos- a long life expectancy, removal of the
itive and false-negative interpreta- gallbladder may offer a more defini-
tions occur, but when real-time ultra- tive solution, because a diseased
sonic imaging shows echogenic foci gallbladder may develop recurrent
within the gallbladder that move with gallstones after dissolution.
positional change and demonstrates Operative Treatment. The surgical
distal shadowing, the findings indi- treatment of adolescents with chole-
cate gallstones. Ultrasonography is hithiasis is somewhat controversial. A
also helpful in determining the size of few surgeons have advised cholecys-
gallstones while detecting gallbladder totomy with simple removal of gall-
distention and common duct size. stones when the gallbladder is not
Ultrasonography is not, however, a acutely inflamed.17 Although this pro-
test of gallbladder function and can- cedure may temporarily benefit the
not be used to diagnose acute cho- patient, gallstones do recur, neces-
lecystitis. Chohescintigraphy, with sitating removal of the gallbladder at
Fig 4. Top, Multiple mixed cholesterol gall- one of the technetium 99tm-habeled a later time with increased risk of
stones in 15-year-old girl with pancreatitis and
no hemolytic diseases. Bottom, Higher magni-
acetanihide iminodiacetic acid deriva- complications. Accordingly, we
fication illustrates coalescence of small aggre- tives is the most accurate method of would reserve cholecystotomy with
gates to form larger stones. evaluating the patient with acute cho- removal of gallstones (leaving the

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GASTROINTESTINAL DISEASE

disorders most commonly associated


gallbladder intact) for chronically ill raphy using real-time scanning tech-
children with high-risk, complicated with development of gallstones. nique is preferred for the initial diag-
conditions, such as congenital heart nostic test. It is recommended that
Hereditary Spherocytosis
disease, chronic lung disease, or children older than 1 0 years of age
chronic renal impairment. The number of boys and girls with with sickle cell disease be screened
hereditary spherocytosis is either routinely for gallstones. By late ado-
about equal or slightly higher for girls. hescence, cholehithiasis occurs in as
As many as 60% of the children with many as 40% of these patients, with
Cholecystectomy is the
spherocytosis are estimated to have 75% becoming symptomatic as they
procedure of choice for
hemolytic gallstones, but in most re- age.
symptomatic chohelithiasis.
ports the incidence is approximately Pain, fever, nausea, jaundice,and
Cholecystectomy is
recommended for the 30%. Although hemolytic calculi have leukocytosis are often present with
been found in infants younger than 1 both abdominal sickle cell crisis and
asymptomatic child younger
than 3 years of age when year of age, usually several years are bihiary calculi. Unfortunately, these
required for the calculi to develop. findings offer little assistance to dif-
echogenic shadows have
Because of the basic hemolytic proc- ferentiation of the two clinical syn-
been present for at least 12
ess, jaundice may occur intermit- dromes. When sonography fails to
months following resumption
tently. Hemolytic gallstones may be identify gallstones, radionuchide bili-
of oral feedings or when the
symptomatically silent and, therefore, ary tract scanning may suggest cho-
gallstones are radiopaque.
will be detected only by appropriate lecystitis in the absence of gahlblad-
Cholecystectomy is advised
diagnostic studies. Radiopaque cal- der visualization. However, with gall-
for asymptomatic children
cuhi in the gallbladder region will be bladder visualization, abdominal
older than 3 years of age
identified by plain abdominal roent- crisis is more likely to be the diagno-
with gallstones if
genograms but must be confirmed sis.
ultrasonographic studies
confirm that echogenic by ultrasonography. An ultrasono- Operative Treatment. Elective cho-
graphic evaluation is recommended hecystectomy is recommended for a
foci with shadowing are
for children older than 2 years of age symptomatic child with gallstones
true stones and not
who have spherocytosis and are can- and sickle cell disease because (1)
echogenic sludge.
didates for splenectomy. It is advisa- gallstone formation increases after
ble also for the surgeon to palpate the age of 1 0 years, (2) the differen-
Cholecystectomy is the procedure the gallbladder to check for the pres- tiation of bihiary calculi from abdomi-
of choice for adolescents, and little ence of gallstones when splenectomy nal sickle cell crisis may be increas-
risk is incurred when the operation is is performed because of hemolytic ingly difficult with advancing age, and
performed by an experienced pedi- disease. If gallstones are found, con- (3) the likelihood of morbidity or mor-
atric surgeon. Most patients tolerate current cholecystectomy should be tahity is less at a younger age, partic-
cholecystectomy well. In the Vander- performed. In the younger child who ularly if a systematic preoperative
bilt group of 73 children and adohes- is symptomatic and complains of dis- transfusion schedule is adopted.
cents who underwent cholecystec- comfort in the gallbladder region, it Most clinicians report fewer compli-
tomy for nonhemolytic gallstones, may be necessary to perform chole- cations when attention is given to
morbidity was minimal and mortality cystectomy and leave the spleen in- preoperative preparation of the pa-
was zero. Two wound infections de- tact in view of concern about post- tient with programmed transfusions.
veloped, and one girl later required splenectomy sepsis, deferring sple- A reasonable plan is to transfuse with
enterolysis for relief of intestinal ob- nectomy until an older age. 10 to 15 mI/kg of packed red cells
struction. initially. If the hemoglobin concentra-
Sickle Cell Anemia tion is less than 1 1 g/dL after 1 week,
CHOLELITHIASIS AND This serious hereditary disorder af- a packed red blood cell transfusion is
HEMOLYTIC DISEASE fects about 50 ,000 children in the repeated. It is most important for the
In former years, it was taught that United States. Almost 10% of the hemoglobin S concentration to de-
hemolytic disease was the most fre- black population carry a gene for sic- crease to at least 30%. When this
quent cause of gallstone develop- kle cell disease, and about I in 400 occurs and the hemoglobin level
ment in children. However, during the has sickle cell disease. Calculi may reaches at least 11 g/dL, the patient
last two decades the proportion of be discovered in affected children as is ready for the operation. Infusion of
children and adolescents reported to young as 4 years of age. The 10% to 1.5 times the maintenance volume of
have “idiopathic” or nonhemolytic 35% prevalence of chohehithiasis in intravenous fluids the night before
cholehithiasis has increased to as high persons with this disease indicates cholecystectomy is advised to ensure
as 80% of the reported cases, as the need to screen for gallstones all adequate hydration. During the op-
opposed to a 20% frequency of children who complain of upper ab- eration and recovery period, hypoten-
cases in which gallstones resulted dominal pain before labeling them as sion, dehydration, hypoxia, hypother-
from hemolytic conditions.5 Heredi- suffering from abdominal vaso-occlu- mia, and acidosis should be pre-
tary spherocytosis, sickle cell anemia, sive crisis. vented to avoid sickling.
and thalassemia are the hemolytic Imaging Procedures. Ultrasonog- A right subcostah incision is pre-

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Cholelithiasis

ferred for chohecystectomy, but if with cholehithiasis, regardless of age. olescents. Ann Surg. 1 980;1 91:626-635
14. Buntain WL, Wood JB, Woolley MM. Pan-
splenectomy is to be accomplished Cholecystectomy is recommended
creatitis in childhood. J Pediatr Surg.
simultaneously for splenic sequestra- for the asymptomatic child younger 1 978;1 3:143-149
tion, a midline or transverse upper than 3 years of age when echogenic 1 5. Lilly JR. Common bile duct calculi in infants
abdominal incision may be used. In- shadows have been present for at and children. J Pediatr Surg. 1 980;1 5:
cidental appendectomy will be bene- least 12 months following resumption 577-580
1 6. Sackmann M, Delrus M, Sauerbruch T, et
ficial because of future difficulty in of oral feedings or when the gall-
al. N EngI J Med. 1988;31 8:393-397
distinguishing appendicitis from ab- stones are radiopaque. Also, chole- 1 7. Robertson JRF, Carachi CR, Sweet EM,
dominal crisis. cystectomy is advised for asympto- et al. Cholelithiasis in childhood. J Pediatr
The safety of elective cholecystec- matic children who are older than 3 Surg. 1 988;23:246-249
tomy with careful management has years of age if ultrasonographic stud- 1 8. Ware R, Filston HC, Schultz WH, et al.
Elective cholecystectomy in children with
been confirmed by recent reports ies confirm that echogenic foci with sickle hemoglobinopathies. Ann Surg.
from major pediatric surgical cen- shadowing are true stones and not 1 988;208:1 7-22
ters.18’19 Successful management of echogenic sludge. Complications of 19. Fullerton MW, Philappart Al, Samaik 5, et
six children after having chohecystec- common bile duct obstruction, pan- aI. Preoperative exchange transfusion in
sickle cell anemia. J Pediatr Surg. 1981;
tomies performed because of sickle creatitis, perforation with bile perito-
16:297-300
cell disease (including two undergo- nitis, and Iife-threatening sepsis may
ing simultaneous splenectomy for se- thus be prevented. Morbidity and
questration) at Vanderbilt Children’s mortality following cholecystectomy Self-Evaluation Quiz
Hospital during the past 7 years with- are expected to be relatively low in
6. Among the following, the factor least
out significant morbidity and no mor- the pediatric age group. likely to contribute to the increasing fre-
tahity also supports this recent trend. quency of diagnosis of cholelithiasis in in-
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trasonographic studies should be Cholelithiasis in infants. Radiology. 1982; cept:
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that approximately 80% of gallstones 9. Takiff H, Fonkalsrud EW. Gallbladder dis- tion.
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138:565-568 thiasis in children is relatively stable
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PIR 274 pediatrics in review #{149} vol. 11 no. 9 march 1990


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Cholelithiasis in Infants, Children, and Adolescents
George W. Holcomb, Jr and George W. Holcomb III
Pediatrics in Review 1990;11;268
DOI: 10.1542/pir.11-9-268

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Cholelithiasis in Infants, Children, and Adolescents
George W. Holcomb, Jr and George W. Holcomb III
Pediatrics in Review 1990;11;268
DOI: 10.1542/pir.11-9-268

The online version of this article, along with updated information and services, is located on
the World Wide Web at:
http://pedsinreview.aappublications.org/content/11/9/268

Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly
publication, it has been published continuously since 1979. Pediatrics in Review is owned, published, and
trademarked by the American Academy of Pediatrics, 345 Park Avenue, Itasca, Illinois, 60143.
Copyright © 1990 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0191-9601.

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