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.