Kimura Osamu, Ono Shigeru, Furukawa Taizo, Higuchi Koji, Deguchi Eiichi, Iwai Naomi
Department of Pediatric Surgery, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto 602-0841, Japan.
J Pediatr Surg. 2009 Aug;44(8):1564-7. doi: 10.1016/j.jpedsurg.2008.11.050.
This study investigated appropriate management strategies for infants with total intestinal aganglionosis (TIA), focusing on surgical and medical managements.
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.
本研究调查了全肠无神经节症（TIA）婴儿的合适管理策略，重点关注手术和药物管理。
回顾性分析了1980年至2007年在我们机构接受治疗的6例TIA或接近TIA的婴儿。2例婴儿进行了简单的空肠造口术，在屈氏韧带（LOT）下方65至70厘米处，1例在LOT下方30厘米处进行，未进行扩大肌切除术-肌切开术（EMM）。3例在LOT下方30至35厘米处进行了带EMM的空肠造口术。
2例空肠造口术位于LOT远端65厘米或70厘米处的婴儿分别在7个月和8个月时死于败血症。1例空肠造口术位于LOT远端30厘米且未进行EMM的婴儿在1岁8个月时死于胆汁淤积性肝衰竭。迄今为止，其余3例空肠造口术位于LOT远端30厘米或35厘米处且进行了EMM的婴儿分别存活至10岁、3岁10个月和2岁。营养管理，如每天80至100千卡/千克的肠外营养和要素饮食（ED）口服喂养，有助于减少肠炎、败血症和胆汁淤积性肝功能障碍的发生。
在进行短段近端空肠造口术并联合EMM后，周期性肠外营养与要素饮食口服摄入的良好结合可能是TIA婴儿存活的关键。此外，对于EMM后吸收功能未改善的婴儿，生长激素给药等药物管理可能值得一试。
