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新生儿期后出现的肠旋转不良。

Malrotation presenting beyond the neonatal period.

作者信息

Spigland N, Brandt M L, Yazbeck S

机构信息

Ste-Justine Hospital, Montreal, Quebec, Canada.

出版信息

J Pediatr Surg. 1990 Nov;25(11):1139-42. doi: 10.1016/0022-3468(90)90749-y.

Abstract

The diagnosis of malrotation is easily made in the neonatal period, but is often delayed in older patients. Among 82 patients treated for malrotation in this institution, 45 patients presented with symptoms related to their malrotation, seven were diagnosed at exploration for concomitant intrinsic duodenal obstruction, and 30 patients had malrotations discovered as incidental findings at laparotomy or autopsy. Among the 45 symptomatic patients, 25 (56%) underwent surgery in the first month of life, whereas 20 patients (44%) underwent surgery at an older age. In this last group, the mean age at surgery was 51.5 months (range, 2 months to 16 years), the mean age of onset of symptoms was 2 years (range, 0 to 15 years) and the mean delay in diagnosis was 1.7 years. Although bilious vomiting was the presenting symptom among all patients undergoing surgery in the neonatal period, clinical features of older patients included intestinal obstruction (7), chronic abdominal pain (4), malabsorption/diarrhea (3), peritonitis/septic shock (2), solid food intolerance (1), common bile duct obstruction (1), abdominal distention (1), and delayed transit postappendectomy (1). The frequency of midgut volvulus was equal among both groups. Unusual forms of malrotation were more frequent in patients undergoing surgery beyond the neonatal period. In this group there was evidence of chronic venous and lymphatic obstruction with one case of superior mesenteric vein thrombosis and two cases of intestinal gangrene. A Ladd's procedure was performed in all cases and the most frequent postoperative complication was adhesive intestinal obstruction. There were no deaths. Awareness of the unusual presentation in patients who present beyond the neonatal period may help reduce delays in diagnosis and surgical treatment. We believe that laparotomy is indicated in all patients with malrotation, even if they are asymptomatic.

摘要

旋转不良在新生儿期易于诊断,但在年龄较大的患者中常被延迟诊断。在本机构接受旋转不良治疗的82例患者中,45例患者出现与旋转不良相关的症状,7例在因合并先天性十二指肠梗阻而进行探查时被诊断,30例患者在剖腹手术或尸检时作为偶然发现被诊断为旋转不良。在45例有症状的患者中,25例(56%)在出生后的第一个月接受了手术,而20例患者(44%)在较大年龄时接受了手术。在最后一组中,手术时的平均年龄为51.5个月(范围为2个月至16岁),症状出现的平均年龄为2岁(范围为0至15岁),诊断的平均延迟时间为1.7年。尽管胆汁性呕吐是所有新生儿期接受手术的患者的主要症状,但年龄较大患者的临床特征包括肠梗阻(7例)、慢性腹痛(4例)、吸收不良/腹泻(3例)、腹膜炎/感染性休克(2例)、固体食物不耐受(1例)、胆总管梗阻(1例)、腹胀(1例)和阑尾切除术后转运延迟(1例)。两组中肠扭转的发生率相同。不典型的旋转不良形式在新生儿期后接受手术的患者中更为常见。在这组患者中,有慢性静脉和淋巴梗阻的证据,其中1例为肠系膜上静脉血栓形成,2例为肠坏疽。所有病例均进行了Ladd手术,最常见的术后并发症是粘连性肠梗阻。无死亡病例。认识到新生儿期后出现症状的患者的不典型表现可能有助于减少诊断和手术治疗的延迟。我们认为,所有旋转不良的患者即使无症状也应进行剖腹手术。

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