Biank Vincent, Werlin Steven
Department of Pediatric Gastroenterology, The Medical College of Wisconsin, and The Children's Hospital of Wisconsin, Milwaukee, WI 53226, USA.
J Pediatr Gastroenterol Nutr. 2006 May;42(5):522-5. doi: 10.1097/01.mpg.0000221888.36501.f2.
Superior mesenteric artery syndrome (SMAS), Wilkie syndrome or cast syndrome is a rare condition that usually presents with symptoms of mid to upper gastrointestinal obstruction due to the compression of the duodenum between the abdominal aorta, posteriorly, and the superior mesenteric artery, anteriorly. The aim of this study was to analyze the clinical characteristics, means of diagnosis and management of SMAS in a pediatric population.
Retrospective chart review of all patients at the Children's Hospital of Wisconsin with SMAS from 1985 to 2005.
Twenty-two cases of SMAS where diagnosed at Children's Hospital of Wisconsin between 1985 and 2005 [14, (64%) female]. Symptoms developed 1 to 393 days (median 5 days) before diagnosis. Presenting symptoms included abdominal pain (59%), vomiting (50%), nausea (40%), early satiety (32%) and anorexia (18%). Diagnosis was made by upper-gastrointestinal radiography in 18 (82%), by computed tomography in 2 (9%) and at laparotomy in 2 (9%). One patient was treated surgically after medical management failed. Mean length of treatment was 65 days (range 13-169), with a mean length of hospitalization of 21 days (range 0-68 days).
SMAS usually presents more acutely than chronically with symptoms of small bowel obstruction. Weight loss is not necessary for SMAS development. Prior neurological injury may be a risk factor for development of SMAS. Upper gastrointestinal radiography remains the primary means of diagnosis. SMAS is typically successfully managed medically. Surgical intervention should be reserved for patients' refractory to medical therapy. The expected outcome of SMAS is excellent.
肠系膜上动脉综合征（SMAS），又称威尔基综合征或石膏综合征，是一种罕见疾病，通常表现为中、上消化道梗阻症状，这是由于十二指肠在后方的腹主动脉和前方的肠系膜上动脉之间受到压迫所致。本研究的目的是分析小儿肠系膜上动脉综合征的临床特征、诊断方法及治疗手段。
对1985年至2005年期间威斯康星儿童医院所有肠系膜上动脉综合征患者进行回顾性病历审查。
1985年至2005年期间，威斯康星儿童医院共诊断出22例肠系膜上动脉综合征患者[14例（64%）为女性]。症状在诊断前1至393天（中位数为5天）出现。主要症状包括腹痛（59%）、呕吐（50%）、恶心（40%）、早饱（32%）和厌食（18%）。18例（82%）通过上消化道造影确诊，2例（9%）通过计算机断层扫描确诊，2例（9%）通过剖腹手术确诊。1例患者在药物治疗失败后接受了手术治疗。平均治疗时间为65天（范围为13 - 169天），平均住院时间为21天（范围为0 - 68天）。
肠系膜上动脉综合征通常以小肠梗阻症状急性发作而非慢性发作。体重减轻并非肠系膜上动脉综合征发生的必要条件。既往神经损伤可能是肠系膜上动脉综合征发生的危险因素。上消化道造影仍是主要的诊断方法。肠系膜上动脉综合征通常通过药物治疗成功治愈。手术干预应仅用于药物治疗无效的患者。肠系膜上动脉综合征的预期预后良好。
