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目的探讨先天性肠旋转不良的临床特点、诊治及转归。方法回顾性分析1993年1月-2010年12月本院收治的先天性肠旋转不良84例病例资料。84例均为小于3个月龄婴儿,均经手术或影像学检查证实为肠旋转不良。76例(90.5%)出现间歇性呕吐,69例(82.1%)有间歇性胆汁性呕吐,11例(13.1%)出现便血。63例行B超检查,阳性率为76.2%。51例次行上消化道造影或钡灌肠,总阳性率为98.0%。82例(97.6%)行Ladd’s手术治疗。结果先天性肠旋转不良术中发现肠扭转52例(63.4%);10例有肠管坏死、肠穿孔,6例有血便,严重肠坏死6例,2例死亡,4例术中放弃治疗。术中发现腹腔渗液22例,有乳糜样渗出4例,伴发消化道畸形22例。有肠坏死的患儿临床表现多伴便血、腹胀或发病时间长。结论间歇性胆汁性呕吐为肠旋转不良的主要表现,便血常预示肠扭转甚至肠坏死发生,需及时手术。B超对于患儿早期诊断有重要价值,消化道造影检出阳性率较高。并肠坏死、肠穿孔者病情危重,部分患儿预后不良。先天性肠旋转不良发现症状后早期诊断、手术治疗效果良好。
Objective To investigate the clinical features, diagnosis, treatment and prognosis of congenital intestinal malrotation. Methods A retrospective analysis of 84 cases of congenital intestinal dysplasia admitted to our hospital from January 1993 to December 2010 was conducted. 84 cases were less than 3 months old infants, were confirmed by surgery or imaging examination for bowel dysfunction. 76 cases (90.5%) had intermittent vomiting, 69 cases (82.1%) had intermittent biliary vomiting, and 11 cases (13.1%) had hematochezia. 63 routine B-ultrasound, the positive rate was 76.2%. 51 cases of upper gastrointestinal angiography or barium enema, the total positive rate was 98.0%. 82 cases (97.6%) underwent Ladd’s surgery. Results Intestinal torsion was found in 52 cases (63.4%) of patients with congenital intestinal malrotation. Ten cases had intestinal necrosis, intestinal perforation, 6 cases of bloody stool, 6 cases of severe intestinal necrosis, 2 died and 4 cases were given up for surgery. Intraoperative findings of 22 cases of peritoneal effusion, chylous exudation in 4 cases, associated with gastrointestinal malformation in 22 cases. Children with intestinal necrosis clinical manifestations more with blood in the stool, bloating or onset of time. Conclusion Intermittent biliary vomiting is the main manifestation of intestinal malrotation, hematochezia often indicates intestine twisting or even intestinal necrosis, requiring timely surgery. B ultrasound for the early diagnosis of children have important value, gastrointestinal imaging positive rate detected. And intestinal necrosis, intestinal perforation critically ill, some children with poor prognosis. Congenital intestinal malformations after the early detection of symptoms, surgical treatment is good.