eMedicine Specialties > Pediatrics: Surgery > General Surgery
Small Left Colon Syndrome: Treatment
Updated: Sep 30, 2008
Treatment
Medical Therapy
As for any newborn with intestinal obstruction, the first priority of treatment should include resuscitation, such as intravenous fluids, nasogastric decompression, and intravenous antibiotics (if clinically indicated). Once plain film abdominal radiography has revealed a distal obstruction without pneumoperitoneum, the infant should undergo contrast enema.
Fortunately, for most patients with neonatal small left colon syndrome (NSLCS), the contrast enema is not only diagnostic of the condition but also therapeutic. The vast majority of infants experience spontaneous passage of meconium after the examination, their abdominal distension resolves, and, soon after, enteral feedings can be cautiously introduced and carefully advanced.
As noted above, excluding other diagnoses, even if the infant has the expected clinical response to a contrast enema, is important; thus, a suction rectal biopsy and 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:
- 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.
- 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
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.2
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References
Davis WS, Allen RP, Favara BE, Slovis TL. Neonatal small left colon syndrome. Am J Roentgenol Radium Ther Nucl Med. Feb 1974;120(2):322-9. [Medline].
Philippart AI, Reed JO, Georgeson KE, Slovis TL. Neonatal small left colon syndrome: intramural not intraluminal obstruction. J Pediatr Surg. Oct 1975;10(5):733-40. [Medline].
Schofield DE, Yunis EJ. Intestinal neuronal dysplasia. J Pediatr Gastroenterol Nutr. Feb 1991;12(2):182-9. [Medline].
Burge D, Drewett M. Meconium plug obstruction. Pediatr Surg Int. Feb 2004;20(2):108-10. [Medline].
Berdon WE, Slovis TL, Campbell JB, et al. Neonatal small left colon syndrome: its relationship to aganglionosis and meconium plug syndrome. Radiology. Nov 1977;125(2):457-62. [Medline].
Rangecroft L. Neonatal small left colon syndrome. Arch Dis Child. Aug 1979;54(8):635-7. [Medline].
Stewart DR, Nixon GW, Johnson DG, Condon VR. Neonatal small left colon syndrome. Ann Surg. Dec 1977;186(6):741-5. [Medline].
Woodhurst WB, Kliman MR. Neonatal small left colon syndrome: report of two cases. Am Surg. Jul 1976;42(7):479-81. [Medline].
Falterman CG, Richardson CJ. Small left colon syndrome associated with maternal ingestion of psychotropic drugs. J Pediatr. Aug 1980;97(2):308-10. [Medline].
Ellerbroek C, Smith WL. Neonatal small left colon in an infant with cystic fibrosis. Pediatr Radiol. 1986;16(2):162-3. [Medline].
Sokal MM, Koenigsberger MR, Rose JS, et al. Neonatal hypermagnesemia and the meconium-plug syndrome. N Engl J Med. Apr 13 1972;286(15):823-5. [Medline].
Swischuk LE. Meconium plug syndrome: a cause of neonatal intestinal obstruction. Am J Roentgenol Radium Ther Nucl Med. Jun 1968;103(2):339-46. [Medline].
Further Reading
Keywords
small left colon syndrome, neonatal small left colon syndrome, NSLCS, imperforate anus, colonic atresia, colonic stenosis, Hirschsprung disease, colonic obstruction, gestational diabetes mellitus, meconium plug, dysmotility syndrome, hypoglycemia, constipation, enterocolitis, infants of diabetic mothers, cystic fibrosis, eclampsia, abdominal distension, intestinal perforation
Treatment: Small Left Colon Syndrome