Weber T R, Tracy T, Connors R H
Department of Surgery, St Louis University School of Medicine, Mo.
Arch Surg. 1991 Jul;126(7):841-6. doi: 10.1001/archsurg.1991.01410310051007.
A number of disorders in childhood can result in short-bowel syndrome (small bowel length, less than 100 cm). Improved care has increased survival in patients with short-bowel syndrome, but the quality-of-life factors associated with such improved survival have not been examined, to our knowledge. Sixteen consecutive pediatric patients with short-bowel syndrome (bowel length range, 22 to 98 cm) were followed up for 2 to 10 years. The original diagnoses were as follows: necrotizing enterocolitis (n = 6), multiple intestinal atresias (n = 4), extensive aganglionosis (n = 2), meconium peritonitis (n = 2), and midgut volvulus (n = 2). The range of initial hospitalization was from 62 to 395 days, and 13 of 16 patients have required readmission (two to 14 times). All patients required multiple operations (range, two to 14 operations), including combinations of venous access, adhesiolysis, tapering enteroplasty, reversed intestinal segments, and pull-through procedure. Nine of 16 patients received home total parenteral nutrition, and 12 of 16 patients required home elemental diets, usually via pump feedings. Fifteen patients (94%) survived. Two survivors are deaf, and one of these has mild developmental delay. Seven survivors (age range, 6 to 10 years) attend a regular school, four while receiving total parenteral nutrition or an elemental diet. Ten of 15 survivors are off all nutritional support (including the child with a 22-cm small bowel), with four others weaning. The presence or absence of an ileocecal valve did not affect outcome. Modern nutritional support methods provide excellent survival and quality of life for children with short-bowel syndrome.
儿童期的一些疾病可导致短肠综合征（小肠长度小于100厘米）。据我们所知，改善护理已提高了短肠综合征患者的生存率，但与这种生存率提高相关的生活质量因素尚未得到研究。对16例连续性儿童短肠综合征患者（肠长度范围为22至98厘米）进行了2至10年的随访。最初诊断如下：坏死性小肠结肠炎（n = 6）、多发性肠闭锁（n = 4）、广泛性无神经节症（n = 2）、胎粪性腹膜炎（n = 2）和中肠扭转（n = 2）。最初住院时间为62至395天，16例患者中有13例需要再次入院（2至14次）。所有患者都需要多次手术（范围为2至14次手术），包括静脉通路建立、粘连松解术、肠管缩窄成形术、肠段翻转术和拖出术。16例患者中有9例接受家庭全胃肠外营养，16例患者中有12例需要家庭要素饮食，通常通过泵饲。15例患者（94%）存活。两名幸存者失聪，其中一名有轻度发育迟缓。7名幸存者（年龄范围为6至10岁）就读于普通学校，其中4名在接受全胃肠外营养或要素饮食。15名幸存者中有10名已停止所有营养支持（包括小肠长度为22厘米的儿童），另外4名正在断奶。回盲瓣的有无不影响预后。现代营养支持方法为短肠综合征儿童提供了良好的生存率和生活质量。
