Congenital esophageal stenosis because of tracheobronchial remnant and treated by circular myectomy: a case report.

Congenital esophageal stenosis (CES) is a rare anomaly, and appropriate management is not well established. We performed myectomy of the esophageal wall in a child with critical esophageal stenosis caused by tracheobronchial remnant (TBR). An 18-month-old boy was admitted to our hospital having frequent vomiting and failure to thrive. Esophagography and esophagoscopy showed abrupt stenosis at the lower esophageal wall. Balloon dilatation was performed but was ineffective. Surgery was performed under a diagnosis of CES because of TBR. Cartilage was palpable in the stenotic esophageal wall, and extirpation of the muscular layer of the stenotic portion was performed, leaving the mucosal layer intact. The muscular layer was closed loosely using interrupted 5-0 absorbable sutures to match the oral and anal sides together. Postoperatively, the esophageal passage was improved to the point that the patient was able to take solid foods without vomiting. This successful outcome suggests that circular myectomy of the TBR is worth recommending as a surgical procedure for short segment and stenosis of patients with CES because of TBR.

Living-related intestinal transplantation for a patient with hypoganglionosis.

A 14-yr-old boy with total parenteral nutrition-dependent short-bowel syndrome associated with hypoganglionosis underwent the LR-IT by using a 150 cm segment of distal ileum taken from a healthy donor. The graft vessels were connected to infrarenal aorta and inferior vena cava. The immunosuppressive regimen consisted of daclizumab, tacrolimus, and steroid. The graft surveillance for ACR was accomplished using zoom endoscopy and mucosal biopsy. The blood trough level of tacrolimus was maintained between 20 and 25 ng/mL for the first 2 months, followed by 15-20 ng/mL thereafter. The 50 mg of daclizumab was administered on the day of operation, and same dosage was repeated at 2-wk intervals. The first ACR occurred on POD-9 and was progressive, and required a 14-day course of OKT-3 injection. After the treatment with OKT-3, the graft recovered from the ACR, and began to function well enough to discontinue the intravenous nutrition on POD-55. No infectious complication has occurred. The patient was discharged in POD-112, and currently tolerates full oral intake without requiring intravenous nutritional or fluid supplementation. The donor was discharged without any complications. The LR-IT could successfully be performed with minimal risk to the donor, and it can be a treatment of choice for patients with short-gut syndrome associated with hypoganglionosis.