Cholelithiasis in childhood

Cholelithiasis

in Childhood

Roger W. MacMillan,

MD, New York, New York

John N. Schullinger,

MD, New York, New York

Thomas V. Santulli, MD, New York, New York

Inflammatory

disease

curs in children cholecystitis tors:

of the biliary

usually

sepsis,

tract

rarely

oc-

[I ,2]. In this age group the cause of relates

anatomic

to one of several

anomalies,

metabolic

facdisor-

ders, hemolytic anemia, or hereditary tendencies [s]. Often, several factors may be implicated. In adults, cent

cholecystitis incidence

the reported large

of gallstones incidence

surveys

disease This

that

were sixteen

report

examines

cholelithiasis

at the

the recent

literature

with

whereas

a 93 per

in children

is 57 to 69 per cent

on biliary

reported

patients

is associated

tract

surgery

[4]. Two

for calculus

tion

who

study.

Five

males.

the surgical Babies

experience

Hospital

dealing

[5,6].

and

with reviews

with this aspect

of bil-

patients

were

black.

The

ages

Vague

distress

quadrant patients

presented

ease

but

with

normal

strated

radiologically

patient

two years

six

later.

Dyspepsia only

underwent,

of all patients

cholecystectomy

at this institu-

for

calculus

dis-

1950 to 1971. Excluded

Department

cal Center, Reprint The way.

New

and the

Medical

New

should

Medical

York,

127.

New

New

June

and York

1974

College

Pediatric

and Surgical

York,

requests

Children’s

Volume

of Surgery,

University,

The Children’s

in the

right

Eight

pa-

Three

biliary

tract

of dis-

cholecystograms. were

later

Six patients,

in two

one year before

gallstones

months

upper

Only

was made.

on oral

1.)

occurring

colic.

than

of having

patient

years

(Figure

biliary

disease

findings

in one

five

and fatty

patients,

sole complaint.

A history

was very unusual, Seven

and

demon-

in another

or 29 per cent,

these

of Physicians

Surgical

Center,

Service,

and

Surgeons,

patients,

Babies

Columbia-Presbyterian

Hospital, Medi-

were

although

York. be addressed Surgical 10032.

to Dr Santulli,

Center

of New

Babies

York,

3975

Hospital, Broad-

disease

teen

patients,

cholecystogram three

patients

patients

was never

had hemolytic of disease

in nineteen calculi were

evaluated

on admis-

had a history All of

and none function

of biliary

or 90 per cent. on either

cholangiogram. for

had

studies

bile duct.

was diagnostic

demonstrated

or intravenous

and vomiting

of the common

patients,

were

liver

in the

patients.

documented.

anemia

Other

examination

never

had jaundice

other

duct.

were

nausea,

in only three

this

were present

and

of fever,

Three

in the common

tract

cent,

or 33 per cent,

ten patients

Roentgenologic

intolerance

occurring

sion to the hospital. of jaundice

food

or 24 per

were not diagnostic Columbia

classic

this and

to sixteen

of the group.

for longer tract

of

females

complaint,

or 69 per cent

were suspected

had

six

pain

common

of biliary

Subsequently,

eleven

from

proce-

criteria

and a half years.

were symptomatic patients

the

were

or abdominal

patients

stones the

There

was the most

fourteen tients

met

ranged

cholecystec-

intra-abdominal

asymptomatic.

were those patients who had anomalies of the biliary tract, acalculus cholecystitis, volvulus of the gallbladder, and septic cholecys-

From

other

with the mean of eleven

these

was conducted

from

Twenty-one

ten

who had incidental

during

dures.

the diagnosis

of age or younger

patients

performed

0.15 and 0.22 per cent of the

Clinical Data

ease

and those

tomy

years

iary disease.

A review

titis

calcifications

In fiforal

Another in the

689

MacMillan,

6-7

Schullinger,

8-9

and Santulli

IO-11 AGE

Figure 7. The age cholelithiasis.

and sex

12-13

I

Male

El

female

J

:.:.: a-

14-15

.-.-.

16

in YEARS distribution

of patients

with

right upper abdominal quadrant noted on a flat plate of the abdomen and were found to have nonvisualization of the gallbladder. A single patient with congenital spherocytosis was admitted for evaluation of chronic pain in the right upper quadrant. A scout film of the abdomen revealed a gallbladder filled with “milk of calcium bile.” (Figures 2 and 3.) The remaining two patients in the series, both of whom had hemolytic anemia, were admitted with acute cholecystitis. Both had had normal findings on oral cholecystograms three years previously. Calcifications in the right upper quadrant seen in six patients during other radiologic procedures alerted the clinician to the need for further workup of the biliary tract. As illustrated in Table I, fifteen patients, or 71 per cent, had hemolytic anemia. Congenital hemolytic spherocytosis was the most common condition. The five black children in this series either had sickle cell anemia or were heterozygous for hemoglobin S and C. The single patient with thalassemia minor merits special comment. Although thalassemia minor is usually not considered a clinically significant form of hemolytic anemia, this patient had an elevated fetal and As hemoglobin level, an elevated reticulocyte count, and abnormal red blood cell morphology.

Of the two patients classified as having sepsis, one patient at five and a half years of age had a perforated appendix. Ten days later, after appendectomy and drainage of a large pelvic abscess, mechanical intestinal obstruction developed necessitating exploratory surgery for lysis of adhesions. Three days thereafter a small intestinal fistula developed and he was transferred to Babies Hospital for further care. A prolonged septic course ensued with ultimate spontaneous closure of the fistula six months later. Eight months after discharge the patient was readmitted for repair of an incisional hernia in the right lower quadrant. At operation stones were palpated in the gallbladder; cholecystectomy was performed subsequently. The second patient had an omphalocele repaired at birth. Malrotation was also noted, but she did well until four years of age when intestinal obstruction developed. At laparotomy multiple obstructing adhesions were lysed, but ten days later obstruction recurred. Reoperation revealed a gangrenous jejunal volvulus. Because of deterioration in the patient’s condition at the operating table, the area was drained in an attempt to establish a fistula. After a complicated, septic postoperative period of three months during which 11,400 ml of whole blood was administered, the fistula was closed surgically. Two years later the presence of gallstones was noted and the patient subsequently underwent an uncomplicated cholecystectomy. Pathologic

Findings

The pathologic diagnosis in each instance was based on histologic examination. (Table II.) The majority of patients, 52 per cent, had evidence of mucosal destruction of the gallbladder with round cell infiltration and fibrosis indicative of chronic inflammation. Seven patients, or 33 per cent, were found to have a normal gallbladder on histologic examination despite the presence of stones. All seven of these patients had hemolytic anemia. Of the patients with chronic cholecystitis, 73 per cent

Figure 2. A scout film of the abdomen demonstrating the “milk of calcium bile” gallbladder.

in

the

Figure 3. Roentgenogram of the operative specimen demonstrating the ‘mi/k of calcium bile” and a small calculus obstructing the cystic duct.

690

The American Journal of Surgery

Cholelithiasrs

synlptonlatic-; interestingly, however, of the patients with a normal gallbladder, ‘il per cent were also symptomatic. Histologic definition did not always correlate with the clinical picture. Cholelithiasis in every patient was treated by cholecystect,omy. There were five choledochotomies, two of which revealed stones. In one patient several common duct. calculi were demonstrated by cholangiography of the cystic duct. A single stone was palpated in the common duct of the second patient,. Of the three choledochotomies that gave negative results one was performed because of obscure anatomic findings. A false-positive cholangiogram of the cystic duct led to exploration in another. The third choledochotomy was performed on the basis of clinical data. This patient presented with a one week history of pain in the right upper quadrant and was found to have a bilirubin of 6.0 mg per 100 ml (mostly direct) and elevated enzyme levels. The common duct was narrowed presumably secondary to the adjacent inflammation. Among the twenty-one patients there were four postoperative complications, none of which prolonged the hospitalization. Pneumonia developed in two patients. One patient with thalassemia major had a marked decrease in the hematocrit level thought to be secondary to a hemolytic reaction. Transient hematobilia developed in a patient with spherocytosis after choledochotomy, and it subsided spontaneously. No complications occurred among the six patients who had splenectomy in conjunction with cholecystect.omy. There were no operative deaths. were

Comments The peak incidence of gallstones in our series occurred in patients between ten and thirteen years of age, which is in agreement with that reported by others [3,7-9,141. The ratio of females to males in this study was 11:lO contrasting with other reports in which females predominated [1&13]. None of the patients in this study had ever been pregnant. Kirtley and Holcomb [II] and Ahrens [7] expressed the opinion that blacks were less likely to have biliary tract disease. In our study the ratio of blacks to whites is in proportion to that of the general hospital population for both races. Heringman and Diken [14] reported an equal incidence of disease in both races. The symptoms of calculus disease in children are similar to those in adults [4,15,16]. Episodic right upper quadrant or abdominal pain is the

Volume

127,

June

1974

TABLE

I

in Childhood

Associated Conditions in ‘Twenty-One Patients with Gallstones Number cf

Clinical Uiagnosis Hemoiytic anemia Congenital spherocytosis Sickle cell (S-S) Sickle trait (S-C) Thalassemia major Thalassemia minor Pyruvate-kinase deficiency Glucose-6-phosphate dehydrogenase deficiency Sepsis None

TABLE

II

Patients

Per cent

15

71

2 4

10

2 1

19

Pathologic Findings in Twenty-One Patients with Calculi Findings

Chronic cholecystitis Subacute cholecystitis Acute cholecystitis Normal gallbladder

Number

Per cent

11 3 0 7

52.4 14.3 33.3

most common complaint. However, classic biliary colic, dyspepsia, nausea, vomiting, or descriptions of radiating shoulder pain are rare [ 21,131. Often the typical complaints of chronic cholecystitis remain unrecognized in the child because of a lack of suspicion by the clinician. The history or presence of jaundice is a frequent finding in children with gallbladder disease [14,17]. This is rarely ever secondary to choledocholithiasis. In our series 48 per cent of the patients either had a history of jaundice or had documented jaundice on admission to the hospital. None of these patients had common duct calculi and neither of the two patients with common duct calculi were in this group. In a review of 326 cases by Ulin, Nasal, and Martin [4] the incidence of jaundice was 26 to 4Fi per cent whereas that of choledocholithiasis was only 6 per cent. Most investigators conclude that jaundice is secondary to inflammation around the common duct, [4,7]. However, operative cystic duct cholangiograms should be considered when a question of common duct calculi exists. In our experience the most commonly associated condition with cholelithiasis in children was hemolytic disease. It was found in 71 per cent of the patients. Most authors agree with this experience [5,14,18] but there are several report,s to the contrary. Soderlund and Zetterstrom [8] presented a

691

MacMillan, Schullinger, and Santulli

series of sixty cases, fifty-seven of which had calculi, and of these, only three had hemolytic anemia. In Brenner and Stewart’s review [3] of 244 cases of cholecystitis 60 per cent had calculi but only 8.6 per cent had hemolytic anemia. Their conclusion was that gallstones occur more frequently in patients without hemolytic anemia. In the presence of hemolysis the increased destruction of red blood cells releases excessive quantities of free hemoglobin. This is ultimately degraded into bilirubin and excreted in the bile. It is known that patients with hemolytic anemia are more likely to have gallstones than is the general population. Dewey, Grossman, and Canale [ZS] studied twenty-six random patients with thalassemia major and found that 23 per cent had unsuspected cholelithiasis. The incidence of cholelithiasis in hereditary spherocytosis varies between 43 and 66 per cent and in sickle cell anemia between 10 and 37 per cent, as mentioned by Dewey, Grossman, and Canale [IS]. In the two cases of sepsis reported in our series, increased hemolysis might have led to stone formation. Oral cholecystography is the diagnostic method of choice [8,10-121. Nonvisualization of the gallbladder has the same significance in the child as in the adult [15]. In six patients in our series the diagnosis of biliary tract calculi was suspected from plain films of the abdomen. Hanson, Mahour, and Woolley [13] reported this finding in thirteen of their thirty-four patients, nine of whom had hemolytic anemia. Bile contains calcium in the same concentration as that found in the serum. In the presence of inflammation or subacute cystic duct obstruction the gallbladder secretes calcium. If the calcium precipitates with carbonate, a radiopaque calciumcarbonate paste results, the so-named milk of calcium bile. The case reported in this article represents one of the few reported cases occurring in children [19]. Once the diagnosis of cholelithiasis is established the treatment of choice is cholecystectomy. If indicated for hematologic reasons, splenectomy may be performed at the same operation with little increased risk to the patient [8,12]. Six patients in the present series had cholecystectomy and splenectomy without incident. Prior to elective splenectomy for hemolytic anemia, oral cholecystography should be performed.

692

Conclusions I. Cholelithiasis is a rare problem in children and is found equally in both sexes. There is no racial, predominance. 2. The diagnosis of calculi is best made by cholecystography. 3. Laboratory data are of little importance in the diagnosis of choledocholithiasis. 4. Cholelithiasis is most often associated with hemolytic anemia. 5. Cholecystectomy is the preferred treatment. 6. Prior to elective splenectomy for hemolytic diseases a study of the gallbladder is indicated. If cholelithiasis is found, cholecystectomy in conjunction with splenectomy is indicated. References 1. Hawkins PE, Graham FB, Holliday P: Gallbladder disease in children. Am J Surg 111: 74 1. 1966. 2. Kiesewetter WB: Cholecystitis and cholelithiasis, p 745. Pediatric Surgery, 2nd ed (Mustard WT, Ravitch MW, Snyder WH, et al, ed). Chicago, Yearbook Medical Publishers, 1969. 3. Brenner RW. Stewart CF: Cholecystitis in children. Rev Surg 21: 327, 1964. 4. Ulin A, Nasal JL, Martin W: Cholecystitis in childhood: associated obstructive jaundice. Surgery 3 1: 3 12, 1952. 5. Glenn F: 25 year experience in the surgical treatment of 5,037 patients with nonmalignant biliary tract disease. Surg Gynecol Obstef 110: 591, 1959. 6. Colcock B, McManus FE: Experiences with 1,356 cases of cholecystitis and cholelithiasis. Surg Gynecol Obstet 96: 161, 1955. 7. Ahrens W: Cholelithiasis and cholecystitis in infancy and childhood. C/in Proc Child Hosp Washington 13: 85, 1957. 8. Soderlund S: Zetterstrom B: Cholecystitis and cholelithiasis in childhood. Arch Dis Child37: 74.-1962. 9. Strauss RG: Cholelithiasis in childhood. Am J Dis Child 117: 689,

1969.

IO. Morales L et al: Cholecystitis and cholelithiasis in children. J Pediatr Surg 2: 565, 1967. 11. Kirtley J, Holcomb G: Surgical management of diseases of the gallbladder and common duct in children and adolescents. Am J Surg 111: 39, 1966. 12. Calabrese C, Pearlman P: Gallbladder disease below age of 21 years. Surgery70: 413, 1971. 13. Hanson BA, Mahour GH, Woolley MM: Diseases of the gallbladder in infancy and childhood. J Pediatr Surg 6: 277, 1971. 14. Heringman EC, Diken DW: Cholecystitis and cholelithiasis in the young. AmJ Surg74: 27, 1947. 15. Gravier L, Dorman GW, Votteler TP: Gallbladder disease in infants and children. Surgery 63: 690, 1968. 16. Gerrish E, Cole JW: Surgical jaundice in infants and children. Arch Surg 63: 529,

195 1.

17. Seiler I: Gallbladder disease in children. Am J Dis Chi/d 99: 662, 1960. 18. Dewey KW, Grossman H, Canale VC: Cholelithiasis in thalassemia major. Radiology96: 385, 1970. 19. Bass HN: Gallbladder disease in childhood. C/in Pediah 9: 229, 1970.

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