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SMALL LEFT COLON

SYNDROME

BACKGROUND
Intestinal obstruction is one of the most frequent reasons for obtaining
surgical consultation in newborns

Distal intestinal obstruction of the newborn may be anatomic (eg,


imperforate anus, colonic atresia, colonic stenosis) or functional. Most
cases of functional colonic obstruction are caused by Hirschsprung
disease

a subset of term or near-term babies experience colonic obstruction with


a characteristic caliber reduction in the sigmoid and descending colon,
unrelated to meconium inspissation or aganglionosis.

In 1974, Davis coined the term small left colon syndrome in his initial
description of 20 infants with colonic obstruction not caused by a
meconium plug or Hirschsprung disease. [5] In all patients, a contrast
enema revealed a narrowed descending and sigmoid colon, with a
caliber transition at the splenic flexure; of these, 17 patients
experienced immediate and complete resolution of obstruction
without operation. Although Davis observed that 8 of the 20 infants
were born to mothers with diabetes mellitus, he hypothesized that the
apparent dysmotility syndrome was neurogenically determined.

In 1975, Philippart et al reported 8 patients with a similar clinical and


radiographic presentation, all born to mothers treated for gestational diabetes
mellitus with either insulin or oral hypoglycemic agents. [6] In 4 of 8 cases,
resolution of obstruction after contrast enema was uncomplicated. One
patient presented with a cecal perforation requiring emergency operation,
one patient underwent operation for suspected Hirschsprung disease (which
was subsequently disproved by biopsy), and 2 patients required a delayed
operation (one for persistent obstruction and the other for sudden distension
and cecal perforation occurring after institution of oral feedings).

Neonatal small left colon syndrome is an uncommon cause of


neonatal intestinal obstruction characterized by an abrupt
intestinal caliber transition at or near the splenic flexure and
associated, in approximately half of cases, with a maternal
history of gestational diabetes mellitus.

EPIDEMIOLOGY
The frequency with which neonatal small left colon syndrome
occurs is difficult to estimate because the entire subject
literature contains only case reports and a few case series

ETIOLOGY
Although the precise cause of this form of neonatal intestinal
obstruction, which has a typical radiologic picture but is
distinctly unusual, is unknown, numerous theories have been
proposed, including neural, humoral, and drug-induced
etiologic mechanisms.

In 1974, Davis et al reported the association of neonatal small left colon syndrome
with abnormalities of intestinal neurohistology.[5] Their initial report described increased
numbers of immature small ganglion cells in the myenteric plexus (in both the
narrowed and dilated portions of the colon) in 4 of 20 patients with neonatal small left
colon syndrome. They compared the histology from patients with small left colon
syndrome with that of control subjects, including infants of diabetic mothers without
colon changes, premature infants, and term infants. They concluded that the
hypercellularity observed in the specimens from patients with neonatal small left colon
syndrome most closely resembled the histology observed in the colons of premature
infants. Despite this conclusion, they did not provide gestational age data on the
patients; therefore, at least some of them assumably were premature

In a 1975 report, Philippart et al focused on humoral and autonomic nervous system changes,
which occur in response to neonatal hypoglycemia in developing a mechanistic explanation. [6]

Glucagon release and sympathoadrenal stimulation are typical in vivo responses to


hypoglycemia, and both result in blood glucose stabilization through hepatic gluconeogenesis
and glycogenolysis. Along with several effects on the GI tract, glucagon release is known to
decrease motility in the jejunum and left colon. Hypoglycemia also stimulates sympathetic and
parasympathetic arms of the autonomic nervous system. Maximal vagal (parasympathetic)
stimulation results in increased motility in its area of distribution, which ends at the splenic
flexure, whereas sympathetic stimulation results in diminished motility. Therefore, a composite
effect of glucagon release with sympathetic and parasympathetic nervous system stimulation
would hypothetically be an overall diminution in intestinal motility, with a functional block in the
colon beyond the splenic flexure.

In 1991, Schofield and Yunis reported an association in 7 patients with clinical


and radiographic neonatal small left colon syndrome and suction rectal biopsy
histology demonstrating intestinal neuronal dysplasia (IND). [7] In all 7 cases,
the biopsies, which were stained with hematoxylin and eosin (H&E) and
acetylcholinesterase (AChE), demonstrated an increase in the number of
AChE-stained fibers in the mucosa and increased submucosal ganglia or large
ganglia. These changes are also observed with prematurity, and because most
of the infants with neonatal small left colon syndrome in this report were
indeed premature, gestational age seems to have had a confounding effect on
the biopsy results.

DIAGNOSTIC IMAGING
The diagnosis of neonatal small left colon syndrome on contrast
enema examination is based on the following fluoroscopic
findings:
(1) proximal dilation of colon (and, to a lesser extent, small bowel) with

abundant intraluminal meconium;


(2) an abrupt cone-shaped caliber transition at or just distal to the
splenic flexure; and
(3) a constricted but smooth contoured, and often foreshortened,
descending and sigmoid colon devoid of meconium with a slightly larger
caliber rectum

Contrast enema of an infant who presented with


abdominal distension, bilious nasogastric
aspirates, and failure to pass meconium at 24
hours of life demonstrates a normal caliber rectum,
a small caliber sigmoid and descending colon with
an abrupt caliber transition at the splenic flexure.
These findings are characteristic of neonatal small
left colon syndrome (NSLCS

MEDICAL THERAPY
IV fluid
NGT decompression
IV antibiotics
Contrast enema, not only diagnostic but also can be
therapeutic

SURGICAL THERAPY
Surgery is reserved for infants with intestinal perforation or for those in whom
obstruction is refractory or recurrent, despite appropriate conservative
measures. Surgery in infants with intestinal perforation includes the following

Following appropriate fluid, antibiotic, and, if necessary, blood product


resuscitation, the infant is taken to the operating room and explored, usually
through a transverse upper abdominal incision.

Once the point of perforation has been identified, consideration should be


given to determining the diagnosis because, in most cases, Hirschsprung
disease still has not been excluded at this time.

Assuming that the infant's condition is stable and a pathologist with the appropriate
expertise is available, seromuscular biopsy samples from the distal colon, in what
obviously would appear to be aganglionic bowel, should be obtained for frozen
section examination. If a distal biopsy is aganglionic, biopsy samples should be
obtained near the transition zone and proximally to localize the transition zone, and a
stoma should be created in ganglionic bowel. If the transition zone is at the splenic
flexure, leaving the distal aganglionic bowel in place as a long Hartman pouch is
reasonable; however, if the transition zone is in the small bowel, a decompressive
mucous fistula should be created. In cases of long segment disease, the aganglionic
colon should be left undisturbed so as to retain all subsequent reconstructive options.

COMPLICATIONS
Refractory or recurrent obstruction
After the diagnosis of neonatal small left colon syndrome (NSLCS) has been
adequately established, the need for surgical intervention seems unlikely.
Historically, most operations have been performed because of a mistaken
diagnosis of Hirschsprung disease, although Philippart et al reported 2 patients
who required delayed operations and did not have aganglionosis; one patient
developed recurrent obstruction after initial successful enema decompression
and required transition zone colostomy, and the other developed an
obstruction with cecal perforation 6 days after institution of oral feedings

OUTCOME AND PROGNOSIS


Most infants completely respond to contrast enema decompression
and are able to progress quite rapidly to full enteral feedings.
Numerous patients who have undergone follow-up examinations of
their colon have demonstrated normalization of caliber within a few
weeks. In a 1975 report, Philippart et al warned of the small
number of patients who experience delayed complication, either
recurrent or persistent obstruction or delayed perforation,
mandating close surveillance during the first week or 2 of life

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