FEBRUARY, 1974

NEONATAL SMALL LEFT COLON SYNDROME*

By WILLIAM S. DAVIS, M.D., R. PARKER ALLEN, M.D., BLAISE E. FAVARA, M.D.,
and THOMAS L. SLOVIS, M.D.
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DENVER, COLORADO

]/fOST radiologists examining newborn

splenic flexure suggesting obstruction with
.1. YL. infants have encountered difficulty meconium at that point. In both these
in evaluating the diagnostic possibilities instances, with extra manipulation, the
when the initial roentgenograms showed remaining portion of the colon was readily
intestinal obstruction. The plain film roent- filled. Also, in only i of these 19 patients
genograms are often helpful in determining was a significant rubbery meconium plug
the level of this obstruction.3 They, along removed with the enema. In the remainder,
with clinical symptoms, usually dictate the barium flowed readily through the splenic
type of contrast study first undertaken. If transition zone, filling the entire colon. In
the obstruction is in the colon, the common all, evacuation was then easily accom-.
diagnostic possibilities are the meconium plished.
plug syndrome, Hirschsprung’s disease, A similar roentgenographic appearance
low colonic atresia or stenosis, or the entity has been noted before in discussions of
being described in this paper-the neonatal meconium plug syndrome, but the impor-
small left colon syndrome. tance of the small left colon was either not
emphasized or was thought to be the result
MATERIAL AND METHOD ofinspissated meconium.2”8”

Nineteen infants presented in the first to CLINICAL FINDINGS AND COURSE

third days of life, each of whom had essen-
The patients presented with signs and
tially the same clinical history suggesting
symptoms of low colonic obstruction. Sev-
low colonic obstruction. A barium enema
eral had not passed meconium for 24 to
examination made in each showed identical
72 hours. All had mild to moderate abdomi-
roentgenographic findings. In one addi-
nal distention and several had vomited. In
tional patient the small left colon was an
some the vomitus was bile stained. Two of
incidental finding at autopsy. Pathologic
the patients underwent surgery, I in this
studies were obtainable in 4 of the 20 cases
hospital and i elsewhere, with the incorrect
and will be discussed.
diagnosis of Hirschsprung’s disease. One
patient died from hyaline membrane dis-
ROENTGENOGRAPHIC FINDINGS
ease (Case VI), but the remaining 17 pa-
Barium enema studies showed the colon tients stooled normally following diagnostic
from anus to splenic flexure to be of enema study and all signs and symptoms
markedly diminished caliber with a sharp secondary to obstruction disappeared. Fol-
zone of transition at the splenic flexure. In low_up histories showed that no further
most the left colon also appeared shortened, stooling difficulties were encountered with
lacking the usual tortuosity seen in normal any patients.
newborn infants. The margins were smooth Of particular interest is the finding that 8
and not jagged or serrated as has been de- of the 20 patients were infants of diabetic
scribed in Hirschspung’s disease.6 The mothers. Statistically, this is very signifi-
colon proximal to the splenic flexure was cant considering the incidence of diabetes
always dilated and distended with me- in pregnancy (I per I,ooo live births). The
conium. In only 2 of the 19 living cases was chance of this happening as a random
significant hold-up encountered at the occurrence’ is approximately j”l2

* From the Departments of Radiology and Pathology, Denver Children’s Hospital, Denver, Colorado.

322
 VOL. 120, No. 2 Neonatal Small Left Colon Syndrome 323

Six illustrative case histories with roent-

genographic findings are reported.

REPORT OF CASES
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CASE I. RAS. This 7 pound male infant was

transferred to Denver Children’s Hospital at
3 hours of age, having passed no meconium
since birth.
Abdominal distention was noted at
i day of age. The mother, age 20, was an insulin
dependent diabetic of years’ duration, who
at the time of delivery required 30 units of U 8o

insulin daily, but had no difficulty with her dis-

ease during pregnancy.
Physical examination showed moderate ab-
dominal distention without tenderness. A small
amount of normal appearing meconium was
present on the examining finger. Plain film
roentgenognams of the abdomen showed several
dilated ioops of bowel that varied considerably
in their degree of distention, suggesting low
colonic obstruction.
Barium enema examination showed a small, FIG. 2. Case ,i. An erect roentgenogram shows the
abdomen distended by numerous dilated loops of
smooth bordered colon (Fig. i) from anus to
intestine that are of disparate size.
splenic flexure with an abrupt zone of transition
at this level. The left colon was approximately
one-third the diameter of the transverse. An made and colostomy was performed. Initial
initial diagnosis of Hirschsprung’s disease was frozen sections thought
were to show absence
of ganglion cells at the splenic flexure. Because
of the patient’s excellent condition at the time
ofsurgery, the left colon was removed in prepa-
ration for a later Duhamel procedure. Fixed
sections the following day showed a normal
number of ganglion cells but an unusual ap-
pearance of the myentenic plexuses which will
be discussed later. The subsequent Duhamel
procedure was successful and the infant is living
and well.

CASE II. JWC. This male infant was trans-

ferred to Denver Children’s Hospital at i hour
of age because of respiratory distress. The
mother had been insulin dependent for many
years and at the time of delivery was receiving
#{231}units of U $o Lente insulin daily.
The respiratory and chemical abnormalities
were promptly and properly managed. By 24
hours of age the infant had not passed meco-
nium and the abdomen was becoming distended
FIG. i. Case I. The left colon from the splenic flexure
and tense. Two episodes of vomiting had oc-
distally is of markedly diminished caliber and con-
tains only a few small clumps of meconium. The curred with some aspiration. Roentgenogram of
margins are smooth. A sharp transition zone is the abdomen (Fig. 2) showed numerous gas-
present at the splenic fiexure. The initial diagnosis filled loops that varied in size from 8 to 24 mm.,
of Hirschsprung’s disease was proven incorrect. suggesting low colonic obstruction.
The mother of this infant is diabetic. Barium enema roentgenogram (Fig. 3) showed
 324 W. S. Davis, R. P. Allen, B. E. Favara and T. L. Slovis FEBRUARY, 1974

normal except for mild

pyelonephnitis. The in-
fant had taken by mouth
water and on the day
of admission had vomited bile stained fluid. No
meconium had been passed. On physical ex-
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amination, the infant was in good condition

except for moderate abdominal distention with-
out tenderness.
The family history was normal. Specifically,
no diabetes was present.
Plain film roentgenogram of the abdomen
immediately after admission suggested low
colonic obstruction. Barium enema roentgeno-
gram (Fig. 4) showed a definite, persistently
narrowed small left colon from anus to splenic
flexure, at which point there was an abrupt zone
of transition. The transverse colon was approxi-
mately 3 cm. in diameter, while the small left
side was 8 to 10 mm. in diameter. The entire
colon filled readily without evidence of obstruc-
tion at any point. Evacuation was normal and
no further diagnostic studies were performed.
FIG. 3. Case Ii. Barium enema roentgenogram shows On the postevacuation roentgenogram (Fig. c)
the typical appearance of the small left colon with numerous small transverse folds were noted just
smooth margins and an abrupt zone of transition proximal to the splenic flexure. Oral milk feed-
at the splenic flexure. A large amount of meconium ing was started. The abdominal distention dis-
is proximal to this area. Following the enema, appeared and no further bowel difficulties were
evacuation was prompt, stooling progressed nor- encountered.
mally and all obstructive signs disappeared. The
mother of this infant is diabetic.

a small left colon, measuring no more than 6

mm. diameter.
in The barium flowed easily to
the splenic flexure where a definite obstruction
was encountered. This area was manipulated,
the meconium loosened, and barium flooded
into a distended transverse and ascending colon
measuring 23 mm. in diameter and containing
a large amount of meconium. The infant then
expelled an 8 cm. cylindrical plug of rubbery
meconium. Following this, stooling was normal.
The abdominal distention and vomiting dis-
appeared. Laboratory examination of the me-
conium demonstrated tryptic activity.
A follow-up examination i month later
showed some persisting be- disparity in size
tween the transverse and
the former left colon,
being about twice as large as the left side.
Evacuation was normal. The baby had no
stooling difficulties during the month.

CASE III. SlC. This hour old female infant

FIG. 4. Case III. The small left colon with smooth
was transferred to I)enver Children’s Hospital margins is shown with the transition zone at the
because of possible intestinal obstruction. She splenic flexure. ‘I’he left side is shorter than that
was delivered by cesarean section and was in seen in normal newborns and lacks the usual tor-
good condition. The mother’s pregnancy was tuosi ty.
 Voi. 120, No. 2 Neonatal Small Left Colon Syndrome 325

A repeat barium enema study at 2 months of

age (Fig. 6) showed the left colon to be larger
than it had been at birth, but still a definite
disproportion between the left side and the
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more proximal colon. Clinically the infant was

well and stooling normally.

CASE Iv. DSD. This 7 pound male infant

was transferred to Denver Children’s Hospital
at 48 hours of age because of increasing abdomi-
nal distention and failure to pass meconium.
The mother was pnimiparous, the pregnancy
uneventful. Family history was noncontnibu-
tory. Specifically there was no history of dia-
betes.
Physical examination was normal except for
marked abdominal distention. During the ex-
amination the infant vomited bilious material.
Abdominal roen tgenograms showed dilated
FIG. 6. Case III. Repeat barium enema roentgeno-
bowel loops of disparate size suggesting low
gram at 2 months of age shows the left colon to be
colonic obstruction. A barium enema examina-
now longer and more tortuous and approaching
tion made immediately after admission showed the diameter of the transverse colon. The transi-
the characteristic small left colon. In this infant tion point is less definite. Stooling is normal.
also, there was difficulty experienced with

temporary obstruction at the splenic flexure.

However, with manipulation the transverse
colon abruptly filled and the disproportionate
size between the proximal and left colon was
readily apparent. No abnormal inspissated me-
conium plug was passed. The infant then
stooled normally and was discharged in good
condition on the fifth day of life.
A follow-up barium enema roentgenogram at
ii months of age (Fig. 7) showed the left colon
to be still smaller than normal and to be shorter
and less tortuous than normally seen for this
age. Numerous circular folds were present in
the transverse colon.

CASE ‘/. LMH. This 8 pound 2 ounce male

infant was the first born of a diabetic mother,
who at the time of delivery was taking 40 units

of U 8o insulin and 10 units of regular insulin

daily. Three weeks prior to delivery she began
gaining weight rapidly, had peripheral edema
and a cesarean section ws performed at 37
weeks. The infant was in good condition at the
time of delivery.
He was transferred to Denver Children’s
FIG. 5. Case iii. An immediate postevacuation roent-
Hospital at 24 hours of age because of progres-
genogram shows partial expulsion of the barium
meconium mixture. Note the transverse folds of sive abdominal distention and failure to pass
hypertrophied mucosa in the distal transverse meconium. Physical examination was normal
colon designated by Hope ci al.6 as “work hyper- except for an obviously distended abdomen.
trophy” or “jejunalization of the colon.” Laboratory studies were normal except for the
 326 V. S. Davis, R. P. Allen, B. E. Favara and T. L. Slovis FEBRUARY, 1974

old mother by cesarean section following a 37

week pregnancy. The preceding sibling required
2 transfusions for isoimmunization. Family his.
tory otherwise was normal. Specifically, there
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was no history of diabetes.

She was transferred to Denver Children’s
Hospital at i hour of age, pale and cyanotic.
Respirations were depressed and bilateral rales
were present. The liver and spleen were en-
larged. No exchange transfusion was done be-
cause of the severe respiratory distress and she
expired at 6 hours of age.
At autopsy the ascending and transverse
colon was moderately dilated. At the splenic
flexure, however, there was marked reduction
in diameter of the colon from 18 to 4 mm. This
was associated with a slight kin king at the zone
of transition with a delicate membranous band
going from the more distal small descending
colon to the proximal enlarged transverse colon.
There was no intrinsic obstructive lesion. The
proximal colon contained viscid meconium and
Fic. 7. Case iv. A barium enema roentgenogram at the distal lumen was essentially empty (Fig.
birth had shown the usual pattern of the neonatal I o).
small left colon syndrome. This follow-up enema
roentgenogram was obtained at II months of age.
No bowel problems had been present since the
initial enema. The left colon is still smaller than
normal and appears shorter and less tortuous than
ordinarily seen at this age. “Jejunalization” is
present in the transverse colon. The mother of this
child is not diabetic.

blood sugars which varied between 30 and o

mg. per cent.
Abdominal roentgen ograms suggested low
colonic obstruction. A barium enema study
(Fig. 8) showed the smooth-walled left colon
with a definite zone of transition at the splenic
flexure. The left colon measured 8 to JO mm. in
diameter with the ascending and transverse
colon being approximately 3 times this size,
and filled with meconium. Immediate evacua-
tion was average. A follow-up roentgenogram at
24 hours of age showed that all barium had
been expelled. Repeat barium enema roentgeno-
gram at 6 years of age (Fig. 9) showed the en-
tire colon to be normal with no disparity be-
tween the proximal portion and the left side.
Growth and development had been excellent
FIG. 8. Case v. This roentgenogram at birth shows
and no stooling problem had ever been encoun-
the same roentgenographic appearance as the other
tered.
cases with some meconium in the rectum. A diag-
nosis of meconium plug syndrome was made (1965)
CASE VI. BE. This I hour old Caucasian fe- and no surgery done. Subsequent stooling was
male weighing pounds was born to a 25 year normal. The mother of this infant is diabetic.
 VOL. 120, No. 2 Neonatal Small Left Colon Syndrome 327

A contrast medium injection

of the inferior
mesenteric artery was but was un-
attempted
successful. A postmortem barium enema study
(Fig. I i) showed the roentgenographic charac-

I
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teristics of the neonatal small left colon with an

easily visible transition zone at the splenic
flexure.

PATHOLOGY

Tissue specimens were examined from 4

cases in the series including autopsy ma-
terial from Case vI and descending and
sigmoid colon and appendix from Case I. In r.
all instances the intermyenteric plexus was
characterized by an increased number of
small cells when compared to the larger,
multipolar ganglion cells. These small cells
included immature neuronal elements,
nerve sheath cells and supportive elements. s....0
Morphometric studies from these cases
were compared to similar studies done on
control material. This included 6 infants of
diabetic mothers without clinical or au-
topsy evidence of small colon, infants Fic. JO. Case VI. This infant died at 7 hours of age
because of hyaline membrane disease and Rh iso-
immunization. Autopsy showed distended loops
of small bowel. The colon was distended with
meconium to the splenic flexure (large arrow)
where the caliber abruptly changed from i8 mm.
to 4 mm. The small left colon (small arrows) was
of the same caliber from splenic flexure to anus
and contained essentially no meconium. The
mother was not diabetic.

who died of pulmonary disease at 32 weeks

gestation, 4 who died at 38 weeks gestation
and 6 infants who died of congenital heart
disease at term. The ratio of small cells to
multipolar ganglion cells was determined in
each group. It was concluded that the
hypercellularity observed in the infants
with the small left colon syndrome was
probably a reflection of immaturity. The
plexus resembled those observed in control
material at approximately 32 weeks gesta-
tional age.
In Case I the hypercellular plexus was
also the site of a chronic inflammatory
infiltrate in which lymphocytes, histio-
FIG. Case
#{231}. v. Repeat barium enema roentgenogram
cytes and plasma cells were recognized.
at 6 years of age shows a normal colon. The pa-
tient has had no gastrointestinal problems since These changes were confined to the distal
birth. or -narrow portion. Changes of this type
 328 W. S. Davis, R. P. Allen, B. E. Favara and T. L. Slovis FEBRUARY, 1974

described above. Surgery in similar cases

has been previously reported.7
It is essential to recognize, however, that
this roentgenographic picture is not that of
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Hirschsprung’s disease and is one that

requires only diagnostic barium enema
examination. Following the enema study
each nonoperative case stooled normally
without assistance and required no further
treatment. This further speaks against
Hirschsprung’s disease as a follow-up roent-
genogram in 24 hours will usually show
retention of barium above an aganglionic
segment. In 2 cases, temporary obstruction
was encountered at the splenic flexure, but
this was relieved with relative ease by the
enema. It is true that an actual mechanical
obstruction can be caused by inspissated
meconium at this level but a definite func-
tional obstruction can likewise result be-
cause of the disparity in size of the caliber
of the colon at this zone of transition. The
rubbery meconium plug encountered in i

FIG. II. Case vi. Postmortem barium enema roent- of our cases is thought to be secondary to
genogram shows the changes of the neonatal small inspissation of the meconium within the
left colon with the transition zone at the splenic small distal portion and not the primary
flexure. cause of the abnormality.
If our experience with this syndrome
were not observed in other cases and remain represents the true incidence in the general
unexplained. population, its expected occurrence in new-
In 2 instances where material permitted born infants would be I out of each
comparison, there was no difference in the deliveries.’ Its relationship to diabetes is
character of the neural plexus of the narrow most interesting and statistically quite
portion and the proximal dilated colon. significant but the reason for this is as yet
unclear. Approximately I delivery in each
DISCUSSION
i,ooo pregnancies is an infant of a diabetic
Nineteen newborn infants are reported mother.1’ Eight of these 20 patients were
who presented in the first 2 or 3 days of life infants of diabetic mothers. The possibility
with signs and symptoms of low colonic of this happening as a random occurrenc&
obstruction. These consisted of failure to is in the order of j_l2

pass meconium, abdominal distention and In another report4 II asymptomatic in-

vomiting. Another was admitted with respi- fants of diabetic mothers were examined.
ratory distress syndrome and expired. In None of these infants had gastrointestinal
each a barium enema examination was symptoms such as abdominal distention or
made and the roentgenographic appearance vomiting; the barium enema was done only
was virtually the same in each case. Two because they were infants of diabetic
underwent surgery with an incorrect diag- mothers. The small left colon syndrome
nosis of Hirschsprung’s disease and in both was found in 5 of these infants. Again,
cases, later fixed sections showed the pres- should this represent the true incidence
ence of ganglion cells with the changes of this syndrome in progeny of diabetic
 VOL. 120, No. 2 Neonatal Small Left Colon Syndrome 329

mothers it would appear that such infants Bowling in preparation of the manuscript
have about a 6oo fold chance of having the and Mr. James Barbour for the photog-
small left colon syndrome.’ raphy.
From the limited pathologic studies
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REFERENCES
made in these cases, we are unable to cor-
relate the neural plexus immaturity with I. ARCHER, P. G. Sc.I)., Division of Biometrics
the distribution of the small left colon. The University of Colorado, School of Medicine.
Personal communication.
degree of immaturity was similar in all 2. CLATWORTHY, H. W., HOWARD, W. H. R., and
areas of the colon, contracted and dilated LLOYD, J. Meconium plug syndrome. Surgery,
portions alike. 1956,39, 131-142.
3. DAVIs, W. S., and ALLEN, R. P. Conditioning
value of plain film examination in diagnosis of
SUMMARY
neonatal Hirschsprung’s disease. Radiology,
I.Twenty infants with symptoms and 1969, 93, 129-133.
roentgenographic signs of low colonic ob- 4. DAVIS, W. S., and CAMPBELL, J. C. Asympto-
matic neonatal small left colon syndrome in
struction are presented. infants of diabetic mothers. To he published.
2. A barium enema examination per- 5. ELLIS, D. G., and CLATWORTHY, H. W. Meco-
formed in each patient showed identical nium plug syndrome revisited. 7. Pediat.
roentgenographic findings. Surg., 1966, 1, 54-61.
3. This entity was shown not to be 6. HOPE, J. W., BORNS, P. F., and BERG, P. K.
Roen tgenologic manifestations of Hirsch-
Hirschsprung’s disease. Surgery is contra- sprung’s disease in infancy. AM. J. ROENT-
indicated. The only procedure required is GENOL., RAD. THERAPY & NUCLEAR MED.,
diagnostic barium enema examination, this 1965, 95, 217-229.
being also curative. 7. KOT’FMEIER, P. K., and CLATWORTHY, H. W.
Aganglionic and functional megacolon in chil-
. This entity is not the “meconium
dren-diagnostic dilemma. Pediatrics, 1965,
plug syndrome,” although plugs have been 36, 576-582.
removed in a few instances. 8. MIKITY, V. G., HODGMAN, J. E., and PACIULLI,
. A highly significant correlation exists J. Meconium blockage syndrome. Radiology,
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9. SINGLETON, E. X-ray Diagnosis of the Alimen-
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tary Tract in Infants and Children. Year
Book Publishers, Inc., Chicago, 1959, p. 314.
William S. Davis, M.D.
10. SWISCHUK, L. E. Meconium plug syndrome;
The Denver Children’s Hospital
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io6 East i9th Avenue
J. ROENTGENOL., RAD. THERAPY & NUCLEAR
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MED., 1968, 103, 339-346.
ii. WHITE, P. Pregnancy and diabetes, medical
The authors appreciate the assistance of aspects. Med. Clin. North America, 1965, 49,
Mrs. Helen Leschnik and Mrs. Sarah 1015-1024.