Acute Abdomen Due to Acute Cholangitis in a Leukemic Child

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EXPERIENCE AND REASON 469 4. Lord Brain, Walton JN (eds): Diseases of the Nervous

System, ed 7. London, Oxford University Press,

1969, p

227.

5. Berman PH, Banker BQ: Neonatal meningitis. Fed iatrcs

38:6, 1966.

6. Minckler

J:

Pathology of the Nervous System. New York, McGraw-Hill, 1968, vol 1, p 429.

7. Lyons EL, Leeds NE: The angiographic demonstration

of

arterial vascular disease in purulent meningitis.

Radiology 88:935, 1967.

8. Gado M, Axley

J,

Appleton DB, Prensky AL:

Angiogra-phy in the acute and post-treatment phases of

Hemophilus

influenzae meningitis. Radiology 110:439, 1974.

ACKNOWLEDGMENT

We thank Dr. Tom Carlton, Children’s Clinic, Salisbury,

North Carolina, for referring this infant to the North

Carolina Baptist Hospital.

Acute

Abdomen

Due to Acute

Cholangitis

in a Leukemic

Child

When

a

leukemic

child

develops

an

“acute

surgical abdomen,” the odds are against him;

mortality is reported to be as high as 66% for all

leukemics,

and

approaches

100%

for

leukemics

in

relapse.’ The acute abdomen may be due to one

of the

more

common

surgical

conditions

of

child-hood,

including

appendicitis

or

intussusception,

or to a rarer

problem

to the

leukemia

or

its therapy,2

such

as necrotizing

enteropathy

or

hemorrhagic

pancreatitis.

We

report

an

unusual

cause

of

acute

abdomen

in

a

leukemic

child-acute

cholangitis.

CASE REPORT

Acute lymphocytic leukemia (ALL) was diagnosed in this

5-year-old girl in February 1972. Her presenting signs

included hepatosplenomegaly and abnormal liver functions

(

Table I). She responded well to induction and maintenance

chemotherapy, according to our 1972 protocol for treatment

of ALL, using vincristine, cytoxan, prednisone,

methotrex-ate, and mercaptopurine. “Pulses” of vincristine, prednisone,

and intrathecally administered methotrexate were given

every three months.

She remained in remission and did well clinically, despite

asymptomatic abnormalities of liver function (Table I).

Twenty months after diagnosis, after a “pulse” of drug

therapy, she remained listless for two days, then developed

fever, vomiting, and profuse diarrhea. Several other family

members had had “flu” with diarrhea and vomiting.

She was hospitalized the following day with a temperature

of 40.5 C, headache, dehydration, profuse watery diarrhea,

vomiting, and bilateral CVA tenderness without urinary

symptoms. Hepatosplenomegaly was not present, but the

abdomen was tense and bowel sounds were hypoactive.

White blood cell count was 2,100/cu mm, with 18%

segmented neutrophils, 18% bands, 56% atypical

lympho-cytes, and 8% metamyelocytes. Hemoglobin was 10.0 gm/

100 ml; hematocrit, 28.4%; platelet count, 177,000/cu mm;

and ESR 85 mm/mm. Liver function tests were abnormal

(Table I). After cultures were obtained, she was treated with

intravenously given fluids, ampicillin and gentamicin, and

250 cc of packed red blood cells. Spiking fevers continued.

On the third hospital day, chest roentgenogram showed

small bilateral pleural effusions and lower-lobe infiltrates.

Multiple cultures, including lung aspirate, yielded no

orga-nisms. Nasogastric suction was begun for increased

abdom-inal distention.

On the fourth day, abdominal pain and tenderness were

localized in the right upper quadrant. She had an enlarged

tender liver and a morbilliform rash. Liver scan showed

diffusely abnormal uptake with multiple filling defects

consistent with abscesses. Amylase was 61 Close-Street units

per 100 ml (normal, 6 to 33).

Because of her worsening condition and localized right

upper abdominal tenderness, exploratory laparotomy was

performed. The liver was swollen and mottled, but grossly

free of abscesses. The gallbladder was tense and edematous

with an area of serosal dullness, but contained no stones.

There was marked edema of the porta hepatis and mild

edema of the head of the pancreas, but there was no

retroperitoneal hemorrhage or necrosis, and no omental fat

necrosis. The small intestine and colon were grossly normal.

Cholecystostomy (tube drainage of the gallbladder) for

biliary decompression was performed. Liver biopsy showed

focal inflammation consistent with ascending cholangitis and

inflammatory bowel disease (Fig. 1). Cultures of bile, liver,

gallbladder, and peritoneal fluid for viral, bacterial, amid

fungal agents showed no growth.

She improved steadily after the operation, although

tran-sient fevers during the next week were associated with mild

elevations of serum amylase. A cholangiogram one week

postoperatively showed a thread-like common bile duct and

no hepatic duct visualization (Fig. 2). The profuse bile

drainage gradually diminished as she tolerated intermittent,

then continuous, clamping of the tube. A repeat

cholangio-gram six weeks postoperatively showed normal caliber of the

common bile duct and hepatic ducts. The tube was removed

on the 50th postoperative day.

She is well nine months postoperatively. Her leukemia has

remained in remission, and liver functions have returned to

relatively normal levels (Table I). Chemotherapy with

mercaptopurine and cyclophosphamide has been resumed

with no ill effects. Methotrexate was withheld because of

potential hepatic toxicity.

DISCUSSION

Acute cholangitis was documented by liver

biopsy and cholangiography. The patient’s

post-operative

course

was

compatible

with

a slowly

subsiding inflammation of the biliary tree. The

operative

findings

supported

neither

a diagnosis

of

primary

acute

cholecystitis

nor

of

acute

pancreatitis.

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470 ACUTE CHOLANGITIS IN LEUKEMIA

Fic. 1. Liver biopsy during acute illness. There are acute inflammatory aggregates in the portal

area shown. The liver parenchyma is minimally changed. No leukemic infiltrates are seen.

(hematoxylin-eosin,

x

20).

Two

possible

pathomechanisms

for

cholangitis

in this patient must be considered: (1) vascular

spread of infection along portal venules or

lymphatics following intestinal damage from viral

enteritis or antileukemic drugs

and

(2) ascending

cholangitis via the common duct. An infectious

agent is likely in this immunosuppressed host.

Prior

antibiotic

therapy

may

have

suppressed

bacterial growth in cultures obtained

at

the

operation.

The differential diagnosis of the leukemic with

acute “right upper quandrant syndrome” (as

distinguished

from “right lower quandrant

syndrome’ ‘‘and ‘‘ileocecal syndrome”) included:

(1)

acute hepatitis; (2) liver abscess; (3) leukemic

infiltration of the liver; (4) acute pancreatitis; (5)

perforation of a steroid-induced ulcer; (6)

appen-dicitis associated with a malrotated cecum; (7)

acute cholecystitis; and (8) acute cholangitis.

In

this

patient,

the

preoperative

diagnosis

of

fulminant acute hepatitis and acute pancreatitis

were

considered

unlikely

because

of the

relatively

mild

abnormalities

in

serum

enzymes

and

amylase.

The

roentgenographic

findings

sug-gested neither a perforated ulcer nor malrotation.

Leukemic infiltration of the liver, although not

ruled

out

until

laparotomy

and

biopsy,

does

not

present as a catastrophic illness. Thus our

strong-est diagnostic considerations were liver abscess,

acute cholecystitis, and, subsequently, acute

cholangitis.

The initial treatment for both acute

cholecys-TABLE I

LIVER FUNCTION TESTS

Date

Total Bilirubin

(mg/100 ml)

Alkaline Phosphatase (IU/100 ml)

SGOT (lU/mi)

LDH (lU/mi)

February 1972

March 1973

August 1973

February 1974

0.2

2.1

4.6

0.7

68

98

77

101

25

88

37 28

184

139

123 128

Normal range 0.2 to 0.8 82 to 192 5 to 18 29 to 67

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EXPERIENCE AND REASON 471

Fmc.

2.

Tube cholangiogram one week postoperatively.

Contrast material filled the gallbladder, but did not enter the

thread-like common duct until a fatty meal was given. A

small amount of contrast was seen in the duodenum 45

minutes following fatty meal stimulation. The hepatic ducts did not visualize.

titis

and

acute

cholangitis

is nonoperative,

with

nasogastric

suction

and

intravenously

adminis-tered

broad-spectrum

antibiotics.

If the

infection

is not controlled, however, prompt operation is indicated, and may be life-saving. The gallbladder

should

not

be

removed

unless

it

contains

stones

or

is necrotic.

Cholecystostomy

for

decompres-sion

of the

infected

biliary

tree

is the

procedure

of

choice

in the

critically

ill patient.

SUMMARY

Acute

cholangitis,

an

unusual

cause

of

acute

abdomen,

occurred

in

a

5-year-old

child

with

acute

lymphocytic

leukemia

in

remission.

Possi-ble

pathomechanisms

of

cholangitis

were

(1)

vascular

spread

or (2) ascending

ductal

spread

of

infection

from

the

gastrointestinal

tract

in

an

immunosuppressed host. The patient improved

following

surgical

decompression

of

her

biliary

tree.

She

survives

in continuous

complete

remis-sion

for

27 months and is nine months

postopera-tive

without

complications.

ADDENDUM. Since the submission of this

manu-script,

the

patient

had

a relapse

of her

ALL

at 30

months after diagnosis. Her marrow reverted to

normal after re-induction therapy.

ANN

M.

KosLosiu,

M.D.

WILLIAM

J.

ZWARTJES,

M.D.

Departments

of Surgery

and

Oncology,

Denver

Children’s

Hospital

Denver, Colorado

This study was supported by National Institutes of

Health-National Cancer Institute grant CA-12247-02 and by the

Leukemia Society of America for Patient Aid. Dr. Zwartjes is

an American Cancer Society Clinical Fellow for

1974-1975.

ADDRESS FOR REPRINTS: (W.J.Z.) Department of

Oncology, Denver Children’s Hospital, Denver, Colorado

80218.

REFERENCES

1. Sherman NJ, Williams K, \Voolley MM: Surgical

complications in the patient with leukemia.

J

Pediatr Surg 8:235, 1973.

2.

Mauer AM: Pediatric Hematology. New York,

McGraw-Hill, 1969.

3. Lampkin B, McWilliams NB, Mauer AM: Treatment of

acute leukemia. Pediatr Clin North Am 19:1123,

1972.

4. Amromin GD, Solomon RD: Necrotizing enteropathy:

A complication of treated leukemia or lmphoma

patients. JAMA 182:23, 1962.

5. Bierman RH, Amromin (;D: Ileocecal syndrome in

leukopathic conditions. Clin Res 8:134, 1960.

6. Denver Children’s Hospital Oncology Center: Protocol

AL-2 for Combination Treatment of Acute

Lymphocytic Leukemia in Childhood.

ACKNOWLEDGMENT

We wish to thank William S. Davis, M.D., for his

assistance with the roentgenograms, and Charlene P. Holton,

M.D., and John D. Burrington, M.D., for reading the

manuscript.

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1975;56;469

Pediatrics

Ann M. Kosloske and William J. Zwartjes