Bailey P V, Tracy T F, Connors R H, Mooney D P, Lewis J E, Weber T R
Department of Surgery, St Louis University School of Medicine, MO.
J Pediatr Surg. 1993 Jan;28(1):92-5. doi: 10.1016/s0022-3468(05)80364-1.
Although survival in infants with congenital intestinal obstruction has improved, duodenal obstruction continues to present unique challenges. One hundred thirty-eight newborns and infants (aged 0 to 30 days) were treated for congenital duodenal obstruction. Sixty-five were boys and 73 were girls. Sixty-one (45%) were premature. Forty-six had an intrinsic defect (atresia, web, stenosis, or duplication), 64 had an extrinsic defect (annular pancreas or malrotation with congenital bands), while 28 had various combinations of these. Presenting signs included vomiting (90%, bilious in 66%), abdominal distention (25%), dehydration (24%), and weight loss (17%). Although plain film abdominal x-ray was diagnostic in 58%, upper and/or lower gastrointestinal contrast studies were obtained in 71% of infants to confirm diagnosis. Thirty-eight percent of patients had associated anomalies, including Down's syndrome (11%), cardiac defects, other atresia, other trisomy syndrome, imperforate anus, and central nervous system anomalies. Fourteen patients (10%) had 3 or more other anomalies, many of which required additional surgical therapy. The operative repair of the various defects included Ladd's procedure for malrotation (31%), duodenoduodenostomy (14%), duodenojejunostomy (22%), gastrojejunostomy or gastroduodenostomy (4%), excision of the web and duodenoplasty (3%), or combination of the above (22%). Gastrostomy was placed in 61%. One hundred twenty-eight patients survived (93%). The causes of death were combinations of sepsis, pneumonia, brain hemorrhage, short bowel, and cardiac anomaly. Eight of 10 (80%) who died had other serious anomalies. Twenty patients (14%) required reoperation 5 days to 4 years postoperatively for obstructing lesions (5), wound dehiscence (3), anastomotic leak or dysfunction (6), other atresias (2), choledochal cyst (1), pyloric stenosis (1), and gastroesophageal reflux (2).(ABSTRACT TRUNCATED AT 250 WORDS)
尽管先天性肠梗阻患儿的生存率有所提高，但十二指肠梗阻仍然带来独特的挑战。138例新生儿和婴儿（年龄0至30天）接受了先天性十二指肠梗阻治疗。其中65例为男孩，73例为女孩。61例（45%）为早产儿。46例有内在缺陷（闭锁、蹼、狭窄或重复畸形），64例有外在缺陷（环状胰腺或旋转不良伴先天性束带），28例有这些情况的不同组合。主要症状包括呕吐（90%，其中66%为胆汁性呕吐）、腹胀（25%）、脱水（24%）和体重减轻（17%）。尽管腹部平片对58%的病例有诊断价值，但71%的婴儿进行了上消化道和/或下消化道造影检查以确诊。38%的患者有相关畸形，包括唐氏综合征（11%）、心脏缺陷、其他闭锁、其他三体综合征、肛门闭锁和中枢神经系统畸形。14例患者（10%）有3种或更多其他畸形，其中许多需要额外的手术治疗。各种缺陷的手术修复包括Ladd手术治疗旋转不良（31%）、十二指肠十二指肠吻合术（14%）、十二指肠空肠吻合术（22%）、胃空肠吻合术或胃十二指肠吻合术（4%）、蹼切除术和十二指肠成形术（3%）或上述方法的联合应用（22%）。61%的患者进行了胃造口术。128例患者存活（93%）。死亡原因是败血症、肺炎、脑出血、短肠和心脏畸形的综合作用。10例死亡患者中有8例（80%）有其他严重畸形。20例患者（14%）在术后5天至4年因梗阻性病变（5例）、伤口裂开（3例）、吻合口漏或功能障碍（6例）、其他闭锁（2例）、胆总管囊肿（1例）、幽门狭窄（1例）和胃食管反流（2例）需要再次手术。
