Laboratory Studies
Basic laboratory investigations are indicated, including complete blood count (CBC) and differential, C-reactive protein (CRP), coagulation profiles, and blood cultures. Serum levels of glucose, calcium, and magnesium should be measured in infants of mothers with diabetes mellitus or eclampsia or in infants who otherwise appear stressed. Cross-matched blood should be available.
Plain Radiography
Two-view abdominal radiography should be promptly performed. Typically, distal intestinal obstruction with air-fluid levels is revealed (see the image below). Occasionally, infants (especially those in whom the recognition of intestinal obstruction has been delayed) have pneumoperitoneum on plain film radiography, which may be the result of a cecal perforation.
Supine shoot-through lateral abdominal radiograph of infant with abdominal distention, bilious nasogastric aspirates, and failure to pass meconium at 24 hours of life. Distended loops of bowel with air-fluid levels are evident.
Contrast Enema
If plain film radiography does not reveal perforation, the infant should undergo a contrast enema examination, usually with a water-soluble medium. [20] Because appropriate choice of contrast medium and sufficient experience with neonatal conditions are critical to the interpretive accuracy and safety of the examination, the contrast enema examination should be performed by a radiologist who has pediatric expertise.
The diagnosis of neonatal small left colon syndrome (NSLCS) on contrast enema examination is based on the following fluoroscopic findings (see the image below):
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Proximal dilation of the colon (and, to a lesser extent, the small bowel) with abundant intraluminal meconium
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Abrupt cone-shaped caliber transition at or just distal to the splenic flexure
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Constricted but smooth-contoured, and often foreshortened, descending and sigmoid colon devoid of meconium with a slightly larger-caliber rectum
Contrast enema of infant who presented with abdominal distention, bilious nasogastric aspirates, and failure to pass meconium at 24 hours of life demonstrates normal-caliber rectum and small-caliber sigmoid and descending colon, with abrupt caliber transition at splenic flexure. These findings are characteristic of neonatal small left colon syndrome (NSLCS). Supine shoot-through lateral abdominal radiograph had revealed distended loops of bowel with air-fluid levels.
Other Tests
Because Hirschsprung disease with a splenic flexure transition zone is clinically and radiologically indistinguishable from NSLCS, all infants must undergo a suction rectal biopsy to exclude aganglionosis. Cystic fibrosis that produces a colonic variant of meconium ileus should be considered, and the appropriate DNA testing should be performed, as well as a sweat chloride test when the infant is older, if appropriate. [21]
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Supine shoot-through lateral abdominal radiograph of infant with abdominal distention, bilious nasogastric aspirates, and failure to pass meconium at 24 hours of life. Distended loops of bowel with air-fluid levels are evident.
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Contrast enema of infant who presented with abdominal distention, bilious nasogastric aspirates, and failure to pass meconium at 24 hours of life demonstrates normal-caliber rectum and small-caliber sigmoid and descending colon, with abrupt caliber transition at splenic flexure. These findings are characteristic of neonatal small left colon syndrome (NSLCS). Supine shoot-through lateral abdominal radiograph had revealed distended loops of bowel with air-fluid levels.
Tables
Investigator |
Number of Cases |
Median Gestational Age (wk) |
Maternal History of Diabetes Mellitus |
Other Perinatal History |
Intestinal Perforation |
Mortality |
Surgery Performed |
Davis et al [5] |
20 |
Not stated |
8 |
1 (Rh disease) |
0 |
1 (death due to unrelated cause) |
2 |
Berdon et al [15] |
11 |
37 |
3 |
4 (eclampsia) |
0 |
0 |
2 |
Philippart et al [7] |
8 |
38 |
8 |
6 (eclampsia) |
2 |
0 |
4 |
Rangecroft [16] |
5 |
37 |
1 |
0 |
0 |
0 |
1 |
Stewart et al [17] |
4 |
37 |
3 |
2 (eclampsia) |
3 |
1 |
3 |
Woodhurst and Kilman [18] |
2 |
36 |
0 |
0 |
1 |
1 |
2 |
Falterman and Richardson [19] |
2 |
37 |
0 |
2 (psychotropic drug use) |
0 |
0 |
0 |
Total |
52 |
38 |
23 (44%) |
15 (29%) |
6 (12%) |
3 (6%) |
14 (27%); 7 of 14 underwent colostomy for incorrect diagnosis of Hirschsprung disease |
