A case report of a patient with near total intestinal aganglionosis followed by the role of extended myectomy and synbiotics therapy

We report our experience with a patient with near-total intestinal aganglionosis who underwent extended myectomy-myotomy of the proximal jejunum. At 45 days of age, the patient underwent rejejunostomy at 34 cm from the ligament of Treiz (LOT) because of enteritis and the bowel rehabilitation therapy was started. Because the patient established the swelling and eating enough at 8 months of age, an extended myectomy-myotomy was performed when she was 245 days old. A 12-cm myectomy from the jejunostomy located at 27 cm from the LOT was coupled with a 30-cm antemesenteric border myotomy. This ultimately left the patient with 65 to 70 cm of small bowel from the LOT. Synbiotics therapy was performed to prevent bacterial translocation, and then central line infection or enteritis was not observed at all. The patient was doing well, weighing 9.65 kg, at 2 years of age. In conclusion, extended myectomy-myotomy has extended the patient's survival period, but it is important and necessary to prevent bacterial translocation and to improve intestinal adaptation by synbiotics therapy.