Puligandla P S, Nguyen L T, St-Vil D, Flageole H, Bensoussan A L, Nguyen V H, Laberge J M
Divisions of Division of Pediatric Surgery and Pathology, The Montreal Children's Hospital, Montreal, Quebec, Canada.
J Pediatr Surg. 2003 May;38(5):740-4. doi: 10.1016/jpsu.2003.50197.
BACKGROUND/PURPOSE: The aim of this study was to review the presentations of gastrointestinal duplication (GID) and to assess the influence of prenatal diagnosis on treatment.
Retrospective review of all GID at 2 pediatric hospitals from 1980-2002 was conducted.
Seventy-three patients (M43:F30) were identified: 21 neonates, 28 infants (1 to 24 months), 15 children (1 to 10 years), 9 adolescents (>/=11 years). GID location by frequency was ileum (31.5%), ileocaecal valve (30.2%), duodenum (9.6%), stomach (8.2%), jejunum (8.2%), colon (6.8%), and rectum (5.5%). In neonates and infants, vomiting and distension were the most common presentations. Volvulus, caused by a duplication, occurred in 23.8% of neonates and caused the death of one neonate. Intussusception was identified in 10.9% of patients. In older children and adolescents, pain and vomiting were the most common associations. Six of these patients were being treated for Crohn's disease, with the diagnosis of duplication made at laparotomy. Eighteen patients had a prenatal diagnosis by ultrasound scan, with 77.2% of these asymptomatic after birth. Most prenatal diagnoses occurred after 1991 (77.8%). When comparing an earlier period (1980 to 1991; 29 patients) with the current (1992 to 2002; 44 patients), a greater proportion of the latter patients were asymptomatic (36.4 v 13.8%) and had a lower incidence of complications (volvulus/intussusception).
GID can lead to life-threatening complications. Prenatal diagnosis should lead to expeditious postnatal investigation and treatment before the onset of symptoms or complications. GID in older children can mimic Crohn's disease. Laparoscopy/laparotomy should be considered in patients with atypical Crohn's disease or when the diagnosis of an intraabdominal mass is unclear.
背景/目的:本研究旨在回顾胃肠道重复畸形(GID)的临床表现,并评估产前诊断对治疗的影响。
对1980年至2002年两家儿科医院的所有GID病例进行回顾性研究。
共确定73例患者(男43例,女30例),其中新生儿21例,婴儿(1至24个月)28例,儿童(1至10岁)15例,青少年(≥11岁)9例。GID的好发部位按频率依次为回肠(31.5%)、回盲瓣(30.2%)、十二指肠(9.6%)、胃(8.2%)、空肠(8.2%)、结肠(6.8%)和直肠(5.5%)。在新生儿和婴儿中,呕吐和腹胀是最常见的表现。由重复畸形引起的肠扭转发生在23.8%的新生儿中,导致1例新生儿死亡。肠套叠在10.9%的患者中被发现。在大龄儿童和青少年中,疼痛和呕吐是最常见的症状。其中6例患者正在接受克罗恩病的治疗,在剖腹手术时确诊为重复畸形。18例患者通过超声扫描进行了产前诊断,其中77.2%在出生后无症状。大多数产前诊断发生在1991年之后(77.8%)。将早期(1980年至1991年;29例患者)与当前(1992年至2002年;44例患者)进行比较时,后一组患者中无症状的比例更高(36.4%对13.8%),并发症(肠扭转/肠套叠)的发生率更低。
GID可导致危及生命的并发症。产前诊断应促使在症状或并发症出现之前进行快速的产后检查和治疗。大龄儿童的GID可能类似克罗恩病。对于非典型克罗恩病患者或腹部肿块诊断不明确的患者,应考虑进行腹腔镜检查/剖腹手术。