完全性结肠无神经节细胞症的长期转归:32年回顾  

Long-term outcomes in total colonic agangl-ionosis: A 32- year experience

作　　者：Escobar M.A. Grosfeld J.L. West K.W. 郭战宏 

机构地区：[1]Department of Surgery, Indiana University School of Medicine, J. W. Riley Hospital for Children, Indianapolis, IN 46202, United States

出　　处：《世界核心医学期刊文摘（儿科学分册）》2005年第12期41-42,共2页

摘　　要：Background/Methods: A 32- year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed. Results: Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum (26), mid-small bowel (8), midjejunum (1), and entire bowel (1). Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81% )- had a pull-through at 15± 6 months (modified Duhamel 20, Martin long Duha-mel 4, and Soave 5). Six had a Kimura patch. Postoperative complications (including enterocolitis)- were more common after long Duhamel and Soave procedures. Seven (19% ; 2 with Down’s syndrome) died (3 early, 4 late)- from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1). Mean follow-up was 11± 9 years (range, 6 months- 29 years). Twenty-four (83% ) of 29 patients exhibited growth by weight of 25% or more, 21 (91% ) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81% )- of 21 were continent. In 5 of 6, the Kimura patch provided functional benefit with proximal disease. Conclusion: Longterm survival was 81% . The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.Background/Methods: A 32- year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed. Results: Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum (26), mid-small bowel (8), midjejunum (1), and entire bowel (1). Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81% )- had a pull-through at 15± 6 months (modified Duhamel 20, Martin long Duha-mel 4, and Soave 5). Six had a Kimura patch. Postoperative complications (including enterocolitis)- were more common after long Duhamel and Soave procedures. Seven (19% ; 2 with Down's syndrome) died (3 early, 4 late)- from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1). Mean follow-up was 11± 9 years (range, 6 months- 29 years). Twenty-four (83% ) of 29 patients exhibited growth by weight of 25% or more, 21 (91% ) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81% )- of 21 were continent. In 5 of 6, the Kimura patch provided functional benefit with proximal disease. Conclusion: Longterm survival was 81% . The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.

关 键 词：神经节细胞 完全性 SOAVE 长段型 根治术 肠运动 小肠结肠炎 短肠综合征 唐氏综合征 肠造口术 

分 类 号：R726.5[医药卫生—儿科]