Approach Considerations
As discussed in subsequent sections, the indications for operation in neonatal small left colon syndrome (NSLCS) include intestinal perforation and failure of resolution of obstruction following contrast enema administration.
No absolute contraindications for surgical management of NSLCS are recognized. It must be kept in mind that the diagnosis is not definitive until Hirschsprung disease has been excluded by rectal biopsy findings; therefore, any surgical procedure must take into account this diagnostic similarity.
Apart from the need for a more definitive understanding of the pathophysiology of this condition, no real areas of controversy surround the treatment of NSLCS.
Medical Therapy
As for any newborn with intestinal obstruction, the first priority of treatment should include resuscitation, such as intravenous (IV) fluids, nasogastric decompression, and IV antibiotics (if clinically indicated). Once plain abdominal radiography has revealed a distal obstruction without pneumoperitoneum, the infant should undergo a contrast enema.
Fortunately, for most patients with NSLCS, the contrast enema is not only diagnostic of the condition but also therapeutic. [22] The vast majority of infants experience spontaneous passage of meconium after the examination, their abdominal distention resolves, and soon after, enteral feedings can be cautiously introduced and carefully advanced.
As noted above, it is important to exclude other diagnoses, even if the infant has the expected clinical response to a contrast enema; thus, a suction rectal biopsy and a workup for cystic fibrosis are always indicated.
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 elements.
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
Provided that 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 Hartmann 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 preserve all subsequent reconstructive options.
In the rare case of intestinal perforation occurring after the diagnosis of Hirschsprung disease has been satisfactorily excluded, the safest operative strategy would involve a formal stoma or exteriorization of the perforation.
Complications
After the diagnosis of 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. However, Philippart et al reported two 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. [7]
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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 |
