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