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NEIL

Associated

of Four B.

Cases

KAPPELMAN,’2

with

Celiac

and Review

MORTON

AND

sprue

in which

Three

of

ported Lindner port celiac

disease. case

bowel In

of

“marked 1953

is rarely

1941

O’Sullivan

what

was

redundancy Glazer

dilated

and

and

probably and

in patients Moore

[1]

celiac

dilatation

Adlersberg

[2]

with

of reported

the

colon.”

megacolon

in sprue four

but

patients

Am

Department

J Roentgenol

of

Radiology, 128:65-68,

Waterbury January

Hospital.

1977

Waterbury,

underwent

case

of

associated

did

not

with

cite

volvulus.

uncomplicated

with

sprue

was

12]. Manshak of large bowel

specific

megacolon

Case Case

in

cases. association

reand di-

We

rewith

In

cases

A 55-year-old

of

mittent

17, 1976. Hospital and

Yale

University

Johns

Hopkins

Hospital.

Connecticut

06720.

65

Reports

1 man

diarrhea

for

School

with

noted 1

1/2

weight loss, weakness, years. Stools were loose

dilatation

of mesenteric Barium

of celiac sprue. B. demonstrated.

of Medicine.

333.

06510. 3

A

sprue.

Fig. 1.-Case 1. A, Upper gastrointestinal study showing small bowel changes typical of sprue folds, and evidence of hypersecretion. Thickened folds in descending duodenum also characteristic redundant colon. Contrast cleared completely on evacuation film. No intrinsic colonic abnormalities Recieved April 19, 1976; accepted after revision August I Department of Diagnostic Radiology, Yale-New Haven Address reprint requests to M. Burrell. 2 Present address: Department of Diagnostic Radiology,

colon

died.

a

showing

four

redundant

patients

celiac

described

sprue

the

the

TOFFLER3

by Canlson and Ziter in 1970 [4] mentioned the occurrence

latation

large

ROBERT

nonobstructive

Introduction The

of the Literature

BURRELL,1

Four cases of megacolon associated with celiac sprue are described and the literature reviewed. On barium enema examination, the colon is dilated and redundant with normal evacuation. Dilatation may be related to increased stool bulk delivered to the colon and/or to an intrinsic colonic abnormality in celiac sprue.

Sprue:

Baltimore.

Maryland

21205.

Cedar

Street,

small enema

New

and interand non-

bowel, normal sized showing capacious

Haven,

Connecticut

KAPPELMAN

66

ET

AL.

hepatomegaly elevated and bumin,

calcium,

sweat

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was noted. The stool fat content the prothrombin time was prolonged.

test

cholesterol,

was

Barium

and

carotene

were

low.

The

normal.

enema

revealed

a very

large

but without evidence of a transition hr revealed good evacuation. Small ent with celiac disease. Case

was greatly Serum al-

colon

zone bowel

filled

with

stool

(fig. 2). Films at 24 biopsy was consist-

3

A 21-month-old girl was admitted for evaluation of growth and motor retardation and signs of malabsorption since the age of 1 year. At that age she began having diarrhea with foul-smelling, ished The

pasty,

and

abdomen

was

distended

studies

cholesterol,

serum

duodenal

of

patient

was

2. Barium

abnormalities

demonstrated

enema

showing

demonstrated.

no evidence

of

large redundant

Profile

transition

views

zone.

Evacuation

of

colon.

No

rectosigmoid

film

normal.

bloody; they did not float nor were they bulky. After discontinuing alcohol consumption, his symptoms initially responded but soon became chronic. His brother had a biopsyproven diagnosis of celiac sprue and was successfully treated with a gluten-free diet. On physical examination the patient was cachectic with minimal mild

hepatomegaly. hypochromic

Laboratory microcytic

anemia,

determinations depressed

included calcium,

a

colon with no intrinsic abnormality opsy was characteristic of celiac well on a gluten-free diet. Case

(fig. 1B). Small bowel disease. The patient

A 13-month-old girl was noted to be cyanotic in the neontal period and cardiac catheterization showed tetralogy of Fallot. For the previous 2 months she had gained only 57 g despite a normal diet. She had large, loose, floating stools not related to any particular food intake. She was at the third percentile in weight and twenty-fifth percentile in height. Mild

fat

and

was

prolonged.

noted.

test

low

increased

She

had

a mild

were of

on

thickening. (fig. 3A)

the

changes

seen

24

hr

typical

with

on

the

upper

small

Barium with only

film.

of

After

was

enema a small

sprue

diet;

noted

demonresidual

a small

celiac

a gluten-free

gastrointes-

bowel

bowel

bi-

3B),

the

(fig.

A 6-year-old girl was evaluated for a 3-4 month progressive vomiting, diarrhea, weight loss, and

abdominal

4

distention.

Early

an apparent abdominal from three

upper respiratory infection she distention, somewhat relieved to four liquid, bloodless, yellow

daily.

She

growth

became

and

less

the

tenth

weight.

percentile

remarkable.

was

present

in the Serum

diminished,

showed The

an

child

stools

was

discharged

proximal

small

proximal

jejunal

bowel

from

(fig.

folds.

A

4A)

weight

dropped

in height

ml

and

occult

levels

Lactose sweat test

the

with

fecal

fat

were

slightly

and

was

responded

a moderately thickened

moderately

unblood

tolerance test was negative.

hospital

diet. showed

and

was

occasions;

calcium

the carotene. flat curve;

to a milk- and gluten-free Upper gastrointestinal series

by vomiting, with bowel movements

g/100

and

After painless

examination

on several

albumin

normal.

percentile

12.2

of

history

developed

her

Abdominal was

as was abnormally

were

and

twenty-fifth

Hemoglobin

increased.

well

in

development

energetic

from 21 to 18 kg. She was below

dilated

duodenal

dilated

colon

and

was

dem-

onstrated by barium enema (fig. 4B), and the evacuation was normal (fig. 4C). Biopsy was not performed.

film

bidid

2

was

sweat

cleared.

and

total serum protein level with an increased globulin level. Upper gastrointestinal series showed thickened folds in the proximal duodenum with moderate dilatation of the jejunum and excess secretions throughout the small bowel (fig. 1A). Barium enema revealed a capacious redundant dilated

folds dilatation

fold colon

treated

noun-

percentile.

organomegaly Stool

was

poorly

third

symptoms

Case

Fig. 2.-Case

barium

showed

no

a pale, the

anemia.

with slight mucosal strated a redundant amount

in

a normal

time

Minimal

opsy

was

carotene.

microcytic

series.

She were

but

and

prothrombin

Thickened tinal

weight

revealed

albumin,

hypochromic

intrinsic

stools.

Height

Laboratory and

pale

baby.

Discussion Colonic been

dilatation

recognized

common

and

nerstones [4] there

of

observed

were

infrequently. well that

prominent

“in transit

with Small

recognized,

radiologic

is prolonged

to ineffective marked small

in association and

diagnosis. patients time,

celiac

bowel forms

one

Marshak with which

sprue

has

dilatation

marked appears

of the and

is con-

Lindner

dilatation, to be due

penistaltic activity.” In our patients, neither bowel dilatation nor prolonged transit time features.

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Fig. 3.-Case 3. A, Barium enema showing B. Small bowel biopsy showing characteristic crypts. Cellular infiltrate in lamina propria also

increased

caliber

changes

of entire

of celiac sprue.

characteristic

colon.

No localized

Note flattening

transition

of mucosal

zone demonstrated. epithelial cells,

surface

Postevacuation study normal. absent villi, and hypertrophied

of sprue.

F,

Fig.

of colon

4.-Case 4. A. with no evidence

Upper

gastrointestinal

of localized

abnormality

study

showing or transition

small zone.

bowel changes characteristic C. Normal evacuation and

of sprue. B. Barium mucosal pattern.

enema

showing

uniform

dilatation

KAPPELMAN

68

In

malabsonption,

proximal rhea

and

is probably

absorbed

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stool

secretion

secondary

solutes

motility

and

intestinal

also

to

size

is

as the

the

a bulk

irritant

colon

osmotic

effect

stimulus

to of

colon

may

is probably

tion

to

increased

creased

represent

In several

barium

was

ability

of the

colon

of large to the

tients

with

ing

bowel

the

patient,

of the

colon

celiac

they

matory

may

of

the

colon

was

din

may

large

mucosal

and

of their

patients

colon

exposed

authors

to a lower

face

ultastructural columnar

in sprue

Differential Megacolon orders; tinction

can

several from

be

Some

features

evident in late may

cathartic

transition Acquired

zones are megacolon

of

be seen dilatation [8]. stool

the

colon

in patients is not

use.

the

is rare

with emotional uncommon in

through

coral ulcers and are unremarkable

total the

a number

some muscular pathologically.

for

hypertrophy,

dystrophica.

of

dilatation. of [10].

intestinal diabetes, colonic abnormalities

Volvulus

the

motility

may found

and

electrolyte

motility were

in

appeared

be

may been

redundant may

systemic

dilatation

anti-

has

markedly

Megacolon

involvement

as progressive

in colonic

[9], disease

also

such

re-

multi-

sclerosis

and

a common

de-

in such

disorders

disturbances. normal,

In and

no

detected.

O’Sullivan JE, Moore H: Gee-Thaysen disease (idiopathic steatorrhoea of adults and adolescents in nontnopical countries). Br Med J 1:183-187, 1941 2. Glazer I, Adlersberg D: Volvulus of the colon: a complication of spnue. Gastnoentenology 24:643-660, 1953 3. Carlson DH, Ziter FMH Jr: Non-tropical sprue as a cause of megacolon: report of a case. J Can Assoc Radiol 21:

sun-

of

dis-

allow dismegacolon

point

dilation

loss

REFERENCES

that

of

due

to super-

retention causes.

and Dilatabut

disturbances. patients with

Except

and

1.

of glia-

constipation

colonic

anus.

in

primarily

235-237,

1970

4.

fecal

in simple

diseases

electrolyte

as

fact

microvilli

the hallmark. may have various

segment

myotonia

patients

Occasional cases or early adulthood.

at this

However,

still

Radiographically, down

in infancy. childhood

result

cases,

dilatation

en-

cathartic

may

severe

Parkinson’s

a complication

systemic

myxedema,

[6].

with

cycle

dysfunction

and

gastrointestinal

our

postulated

which readily [7]. Congenital

be masked

imposed

tion

features sprue

from

as

Diagnosis

associated

have celiac

usually becomes will be discovered

of

patients

the Defe-

chronic

prolonged,

system

colonic

symp-

concentration

derangement

cells

as sigmoid

Diminished

and there were fewer absorptive cells to react. Electron microscopy studies of colon mucosa demon-

strated

generalized

ultra-

manner

by

reabsonbed,

a vicious to

with

or Fecal

inflam-

developed

tempered

cause

nominator

[5] studied and gliadin

in a similar

was

nervous medication,

in pa-

and

submucosal

The

reacted

this

bowel

and Rubin of wheat

Autonomic

response

cellular

involved

rectum

is painful.

and

In less

may

stimuli.

be

resort and

[7].

is

to

pain,

severe

the

these

defecation

patients

when

cholinergic

suit

malabsonb-

and

of

patients

from to

continues

to avoid these

colon

reported

provide

water

dilatation

right

stimuli

respond

because

These

haustration.

atonic

in pa-

in

in the

in the

discomfort.

the intestine,

load

Dobbins administration

Two

small

post-

abnormality

demonstrate

showed

the

by the

colonic

passively

fecal

studies

abdominal

although

bowel

dilate

of

sprue

response.

toms that

The

measure-

dynamics

which, colonic

the

with

bowel.

Antegrade

large

volume

structural abnormalities. rectal biopsies after enemas;

coating

Physiologic

and

in-

of

occurs

abuse.

hardened,

is delayed

abuse

dilatation of

to

follows,

Many

total

bulky

in the

as measured

measured.

histologic

with

mucosal fluid

sues.

had

input

neglect

becomes

cation

sprue.

increased

tients

colonic

motility

nature

patients

cathartic

decreased

the

adapta-

noticeably

unimpaired.

not

celiac

Although the

was

were

our

had

to increased

have

and of

by both

colonic

and

chronically retention

small bowel Redundancy

people,

constipation

stool

of chronic

of

two

to contract,

film times

a clue

to

due

un-

intestinal

malabsorbed

affected

form

four

patients

poor

evacuation, ments

All

frequency;

stools.

transit

another

bulk.

stool

of

AL.

In some

of Diar-

penistalsis.

amount of stool delivered to it from the the rate of elimination from the rectum. the

because

resorption.

effect

stimulating

of the

increased fluid

to

acting

contents

The

bulk

decreased

ET

may

is

noted

edited by Sleisenger MH, Fordtran JS, Philadelphia, Saunders, 1973, pp 1463-1472 8. Ehrentheil OF, Wells EP: Megacolon in psychotic patients. A clinical entity. Gastroentenology 29:285-293, 1955

Massive psychoses

occasional

Marshak RH, Lindner AE: Malabsorption syndrome: sprue, in Radiology of the Small Intestine, Philadelphia, Saunders, 1970, pp 9-29 5. Dobbins WO, Rubin CE: Studies of the rectal mucosa in celiac sprue. Gastroenterology 47 :47 1 -479, 1964 6. Pittman FE, Pittman JC: A light and electron microscopic study of sigmoid colonic mucosa in adult celiac disease. Scand J Gastnoenterol 1 :21-27, 1966 7. Davidson M, Sleisenger MH: Megacolon, in Gastrointestinal Disease: Pathophysiology, Diagnosis, Management,

9. as

ster-

specimens

10.

Grossman HJ, Limosani MA, Shore M: Megacolon as a manifestation of familial autonomic dysfunction. J Pediatr 49:289-296, 1956 Caplan LH, Jacobson HG, Rubenstein BM, Rotman MZ: Megacolon and volvulus in Parkinson’s disease. Radio!ogy 85:73-78, 1965