VOL.

No.

125,

i

INTESTINAL

PERFORATION AS A COMPLICATION THE NEONATAL SMALL LEFT COLON SYNDROME*

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OF

GEORGE

By

W. and

NIXON, DAVID

M.D., VIRGIL R. STEWART,

SALT

LAKE

CITY,

R. CONDON, M.D.

M.D.,

UTAH

ABSTRACT:

Intestinal perforation can occur as a complication of the neonatal syndrome. Four such infants are reported. Early diagnosis and treatment, utilizing water soluble contrast tion, are recommended to reduce the frequency of this complication.

T

small enema

HE neonatal small left colon syndrome (SLCS) was recently reported by Davis et al.,3 as a condition producing

tion. She was the product tion and the pregnancy toxemia. The mother was

signs tion.

diabetic.

and symptoms of low colonic Affected infants are often

obstruc-

Mild

cyanosis

left

colon

examina-

of a 38 week gestawas complicated by an insulin dependent

secondary

to persistent

spring of diabetic mothers. amination of the abdomen dilated small bowel loops visualization of dilated transverse portions of the

the offRoentgen exshows multiple and sometimes ascending and colon. Contrast

fetal circulation syndrome and hypoglycemic cardiomyopathy was present during the first 24 hours. Only a minimal amount of meconium had been passed prior to her transfer to this hospital.

enema

a characteristic

peritoneu

study

demonstrates

Abdomen merous

pattern of a small left colon to the level of the splenic flexure where a sharp transition zone exists with the proximal colon being dilated. syndrome condition

This benign

in

has been considered which contrast enema

not

reported. tributed

A by

Dr.

mothers, Medical

fourth Davis

CASE

I.

*

Presented

3974. From Utah

OF

at the Seventeenth

a

center,

of Salt

Radiology

Lake

city,

Meeting and

Divisions

of the of

and

nu-

a pneumo-

as

to

abdominal

distention

a complication

had

of SLCS

in

our

i).

Exploratory

laparotomy

revealed

multiple

small perforations with necrotic margins in the ileum approximately 10 cm. proximal to the ileocecal valve. This segment of bowel was resected and an ileostomy done. The patient survived and is doing well at I year of age. Her ileostomy has been closed several months. A follow-up barium enema done at age weeks showed the entire colon to appear normal with no disparity in size between the right and left sides (Fig. 2).

at be

Society

due

occurred

(Fig.

CASES

Annual

demonstrated bowel

experience with infants of diabetic mothers. We felt that SLCS with perforation was an unlikely cause for the pneumoperitoneum and therefore elected to do a water soluble contrast enema to evaluate the possibility of aganglionosis and to rule out the colon as the site of perforation. This study showed characteristic findings of SLCS without extravasation of contrast medium into the peritoneal cavity

This 4,500 gram female infant was to Primary Children’s Medical Cen2 days because of abdominal disten-

the Departments Medical

seen will

of

previous

case, recently conwill also be reviewed.

REPORT

transferred ter at age

recently Center

tgenograms loops

m.

Perforation

examination was curative by stimulating meconium evacuation. We wish to supplement these concepts by reporting 4 cases with intestinal perforation as a complication of this condition. The case histories and roentgen findings in 3 patients, all infants of diabetic Primary Children’s

roen dilated

for

Pediatric

Pediatric Surgery,

Utah.

75

Radiology, Primary

San children’s

Francisco, Medical

california, center

September and

University

23,

of

G.

76

%V. Nixon,

V.

R.

Condon

and

D.

R.

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Exploratory

Stewart laparotomy

confirmed

intravascular

coagulation

Postmortem examination abscesses of the liver, lung subarachnoid with

were

Tissue co-author SLCS. The

FIG.

I.

the

Case i. A large pneumoperitoneum falciform ligament. The appearance

colon is typical syndrome. The with dilatation

outlines of the

of the neonatal small left colon colon is small to the splenic flexure proximal to the transition zone.

CASE

This

II.

ferred

to

gram

2,400

dependent

diabetic

Primary

female

infant

mother

was

Children’s

Medical

of an

by

the

Blaise

cells in the intermyenteric was reduced, as in

cases

of this

CASE

This

III.

born

Primary

hours

24

distention was

and

Favara,

left M.D.,

paper describing ganglion cells plexus first

their

and to

to

in this reported

to

a diabetic

fetal

female

pass

pregnancy

was

Medical

because

mother

circulation

infant

Children’s

of age

failure

with the Hypoglycemic

persistent

gram

4,400

to

at

bowel

syndrome.

transferred

Center

the

right E.

of the original ratio of multipolar

small patient

gestation toxemia. insulin

from

reviewed

a

asso-

noted.

specimens

were

and

age

candida and

Changes

diabetes

also

at

revealed and kidney

hemorrhage.

maternal

perforation colon

cecal

and control

disseminated II days.

ciated

the

a cecostomy was done. Ather chemical abnormalities, problems and peritonitis were and the patient died of sepsis and

perforation, tempts to cardiovascular unsuccessful

large

3975

SEPTEMBER,

of abdominal

She

meconium. after

a 36

week

complicated cardioniyopathy syndrome

by and resulted

transCenter

after intestinal perforation was identified. She was the product of a 36 week gestation which was complicated by polyhydramnios. Mild respiratory distress and cyanosis felt to be secondary to hypoglycemic cardiomyopathy and persistent fetal circulation syndrome were initially present, and were controlled by supportive

medical

measures.

age the patient had abdominal distention enemas

were

dition.

not

unsuccessful

Roentgen

At

hours

24

passed meconium, was noted. Small in

examination

relieving of

this the

of

and saline con-

abdomen

at this time revealed very mild dilatation of the cecum with the remainder of the intestinal gas pattern appearing normal. At 48 hours of age a pneumoperitoneum was present on an abdomen film (Fig. 3). A gastrografin enema done at another hospital was reported to show transition zone at the splenic flexure with the proximal coion of larger caliber. Contrast medium entered the peritoneal cavity after

reaching

postoperative firmed the

(Fig.

5).

the

cecum

abdomen splenic

(Fig. roen flexure

4).

A subsequent

tgenogram transition

conzone

Fic.

2.

barium of age.

Case

I.

enema

Normal colon

appearance examination

of a follow-up done at 5 weeks

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VOL.

No.

325,

Intestinal

i

Perforation

in cyanosis Abdomen

during the first roentgenograms

day.

lated

bowel

loops

to the

region (Fig.

of the colon (Fig. 6). A contrast 7) showed the distal colon to be

ately

small

colon

with

to

transition

the

the

zone.

terminal

contrast

level

of

Contrast

ileum.

No

the

Following of meconium

this

to

enema study, was expelled, but passage

and her condition roentgenogram

pneumoperitoneum peritoneal cavity

the

enema

Exploratory

laparotomy

abdominal

study

and contrast (Fig. 8).

was

a large despite

progressed An abdomen contrast

of

cavity

meconium

the

the into

extravasation

peritoneal

distention teriorated. after

77

enema

refluxed

continued

hours

Colon

flexure

proximal

definite the

Left

di-

splenic

medium

into

Small

modermid-descending

dilatation

medium

noted. amount

demonstrated

proximal

some

of Neonatal

dedone

6

showed

medium

a

in the

revealed

a

large

cecal perforation. The peritoneum was lavaged and the cecum exteriorized at the perforation site. Postoperatively, the patient’s condition continued

to

deteriorate

and

she

died

at

48

hours of age. Hirschsprung’s ruled out in

Autopsy permission was denied. disease was not completely this patient. The facts that the

patient

female,

was

duration,

and

the

strongly support than Hirschsprung’s

infant

the

premature

by

of

a diabetic

diagnosis disease.

Fic. . veals

Case ii. extensive

IV.

This

2,820

gram

gestation mother

of SLCS

male

contrast enema reinto the peritoneal

cavity.

rather

the

product

though

there

mother

was

abdominal CASE

Water soluble extravasation

infant

An

was

abdomen

bowel to

of not

week

37-40

a positive diabetic.

distention was roentgenogram

loops

the

a

was

with

region

of

the

colon

the

splenic

gestation.

family At

24

Al-

history, hours

the of

age

noted. revealed appearing flexure.

dilated involved Contrast

-4

Fic. Case demonstrates

FIG.

3.

II.

abdomen pneumoperitoneum

Supine

a large

roentgenogram

(arrows).

.

Case

roentgenogram splenic flexure

II.

Follow-up postoperative confirms the transition area of the colon.

abdomen zone in the

G. W.

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78

Nixon,

V. R. Condon

and

D.

R. Stewart

of age

again

domen

roentgenogram

showing

SEPTEMBER,

changes done

of SLCS. later

that

1975

An day

abdem-

onstrated contrast medium in the peritoneal cavity (Fig. lo). Following the diagnosis of intestinal perforation this baby was transferred to Denver

Children’s

Hospital.

Laparotomy

revealed

the

perforation site to be in the cecum. A cecostomy was done and a rectal biopsy showed ganglion cells to be present. This patient survived and has done well, currently being 3 months of age. DISCUSSION

There intestinal more

frequently

gastric

perforation

nium

Supine

iii.

shows colon

typical

of the neonatal

enema

examination

strated

a small

colon region Abdominal peat

abdomen

roentgenogram

numerous dilated loops of small to the splenic flexure region. This

contrast

small

at left

colon

with proximal distention enema

was

2

left colon

days to the

of

bowel pattern

and is

syndrome.

age

demon-

dilatation progressed performed

ileus,

etiologies of gastroin the newborn. The

reported of

small

bowel

causes

the

newborn, or

colon

include mecoatresia,

necrotizing enterocolitis, Hirschsprung’s disease, imperforate anus, trauma, and the meconium plug syndrome. Intestinal perforation associated with the neonatal small left colon syndrome has not been previously reported. It should be noted, however, that previous cases reported as idiopathic

or

small

mid-descending

are multiple perforation

spontaneous

bowel

or

perforations

proximal

colon

of

the

certainly

(Fig. 9). and a reat

3 days

nation reveals the left colon to be small to the middescending region without apparent extravasation or pneumoperitoneum.

FIG.

8.

Case

contrast up

abdomen

in. Pneumoperitoneum medium are demonstrated

roentgenogram.

and extravasated on this follow-

VOL.

No.

325,

may

Intestinal

i

have

occurred

Perforation

of Neonatal

Small

Left

Colon

79

secondary

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syndrome.4-7

Intestinal tation

perforation

and

focal

to a mechanically

colon.

other

necrosis

testinal

can

than may

perforation

in infants

circulatory

reflex”)

occurs

shock.6 monly

wit

syndrome that ischemia

in

seli (“ti

with

neonates

h

Infants of diabetic mot’ have respiratory disease,

cemic

cardiomyopathy

circulation trigger this Perforation colon This

and

syndrome asphyxial secondary

lesion usually was the location

With may

dilat

contribt

natal small left colon It has been postulated testinal

occur

or functionally

Factors

pressure

secondar

necrosis

associated be

bowel

involved

the perforation

persis

(PFCS)5 wh defense m to an o occurs in th in 3 of t’ ischemia,

as in our occurred

other in the

dis

io. Case

FIG.

reveals

peritoneal

v.

Follow-up

extravasation

abdomen of contrast

roentgenogram medium

into

the

cavity.

These cases of the neonatal small left colon syndrome with perforation demonstrate the potentially serious nature of this condition and the need for early diagnosis (mechanical)

OBSTRUCTION

(functional)

Dilatation

Bowel

PERFORATION

Wall Ischemia

SELECTIVE

CIRCULATORY “the

diving

ISCHEMIA reflex”

RDS PFCS Hypoglycemia

FIG.

.

Case

IV.

Contrast

enema

shows a small left colon with zone near the splenic flexure.

roentgenogram

an abrupt

transition

FIG.

ii.

tributing in SLCS.

Schematic

factors

diagram

resulting

showing

in intestinal

possible conperforation

G. W.

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8o

Nixon,

V. R.

Condon

and treatment. The mortality rate in newborns with gastrointestinal perforation is high.4’7 This is especially true in infants of diabetic mothers with associated serious complicating medical problems. Early diagnosis and treatment are accomplished with a water soluble contrast enema examination. We recommend that this study be done immediately in newborns who develop clinical findings of colon obstruction or fail to pass significant meconium within 24 to 48 hours. This is particularly critical in infants of diabetic mothers. This aggressive approach hopefully should reduce the incidence of intestinal perforation as a complication of the neonatal small left colon syndrome. Repeat

contrast

enema

these agents in diagnosing

cians

must

be

are

informed

less accurate aganglionosis.’

that

if

signs

than

Cliniand

D.

R.

Stewart

symptoms

of obstruction

examination

using

SEPTEMBER,

persist,

the

1975

a follow-up

is

barium

evaluate more accurately long segment Hirschsprung’s

essential

to

possibility disease.

of

W. Nixon, M.D. Department of Radiology

George

Primary 320

Salt

Children’s

Medical

Avenue Lake City, Utah

Center

12th

We Dr.

wish William

to S.

Hospital,

84103

express Davis,

Denver,

our appreciation Denver Children’s

Colorado,

for

Case

to

iv.

REFERENCES i.

W. E., and Diagnosis.

BERDON,

X-Ray

Year

Book pp.

1972,

examinations

may occasionally be necessary to relieve the obstructive signs in these babies.2 Also, undiluted or I 1 diluted water soluble contrast medium may result in irritability and spasm of the distal colon. Further dilution to i: has subsequently been successful in diagnosis and treatment.2 Although we recommend the use of water soluble contrast media in the evaluation and management of SLCS it must be noted that barium

and

Medical

DAvis,

W.

D&vis,

W. S., T.

L.

drome.

AM.

J.

Surg.,

LLOYD,

Gynec.

small

Chicago,

left

ROENTGENOL., 1974,

120,

& Obst.,

RAD.

7.

Pediat.,

J. R. Etiology

B. E., and colon

J. G., and in infancy. 623-630. fetal circulation: 82, 1103-1106.

113, of 1973,

of gastrointestinal 7. Pediat. Surg.,

in newborn.

syn&

THERAPY

322-329.

1961,

W. M. Persistence

commentary.

tions

Neonatal

MED.,

CONDON,

6.

J. Caffey.

R. J., RAFFENSPERGER, J. B. Pneumoperitoneum

FREEARK,

GERSONY,

By

Inc.,

communication. R. P., FAvARA,

ALLEN,

SLovIs,

.

Publishers,

S. Personal

2.

NUCLEAR

edition.

1515.

3.

4.

D. H. In: Pediatric

BAKER,

Sixth

perfora4,

1969,

77-84.

7.

THOMAS,

C.

Idiopathic Surg.,

1966,

C., JR., perforation

z6,

and BROCKMAN, of colon in infancy.

853-858.

S.

K.

Ann.

Intestinal perforation as a complication of the neonatal small left colon syndrome.

Intestinal perforation can occur as a complication of the neonatal small left colon syndrome. Four such infants are reported. Early diagnosis and trea...
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