History and Physical Examination
The clinical features of infants described in seven English-language case series of neonatal small left colon syndrome (NSLCS) are summarized in Table 1 below.
Table 1. Clinical Features of Infants Described in English-Language Case Series of Neonatal Small Left Colon Syndrome (Open Table in a new window)
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 |
Most infants with NSLCS are born at or near term and are of normal birth weight. Approximately 50% have a history of maternal diabetes mellitus, and other maternal comorbidities (usually eclampsia), which contribute to neonatal stress, may also be present. All patients do not pass meconium within the first 24 hours of life, and they all develop abdominal distention with bilious vomiting or nasogastric aspirates. A small number of infants develop progressive distention leading to perforation, typically in the cecum, within the first 24-36 hours of life.
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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 |
