Six infants with TIA or near TIA treated in our institution between 1980 and 2007 were reviewed retrospectively. Surgery was performed as a simple jejunostomy, 65 to 70 cm below the ligament of Treitz (LOT) in 2 infants, and 30 cm below LOT in 1 without extended myectomy-myotomy(EMM). Jejunostomy with EMM 30 to 35 cm below LOT were performed in 3.
Two infants with jejunostomy 65 cm or 70 cm distal from LOT died of sepsis at 7 months and 8 months of age, respectively. One infant with jejunostomy 30 cm from LOT without EMM died of cholestatic liver failure at the age of 1 year and 8 months. To date, the remaining 3 infants with jejunostomy 30 cm or 35 cm distal from LOT in addition to EMM have survived 10 years, 3 years and 10 months, and 2 years of age, respectively. Nutritional managements such as parenteral nutrition with 80 to 100 kcal/kg/day and oral feeding with elemental diet(ED) were preferable to reduce the occurrence of enteritis, sepsis, and cholestatic liver dysfunction.
A good combination of cyclic parenteral nutrition and oral intake with elemental diet after short proximal jejunostomy with EMM may be a key for the survival of infants with TIA. In addition, in infants whose absorptive function was not ameliorated by EMM, medical management such as GH administration might be worth trying.