Heterotaxia syndrome: the role of screening for intestinal rotation abnormalities
Heterotaxia syndrome: the role of screening for intestinal rotation abnormalities
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DOI：
10.1136/adc.2004.067504
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发表时间：
2005-08-01
影响因子：
5.2
通讯作者：
Langer, JC
中科院分区：
文献类型：
--
作者：
Choi, M;Borenstein, SH;Langer, JC
背景：内脏异位综合征涉及多种异常，包括心脏畸形和肠旋转异常。大多数作者建议常规放射学评估，剖腹手术和拉德程序，如果旋转abnormalism.Aims：以确定是否有必要进行常规放射学筛查，如果有一组儿童，可以安全地管理expectorious.Methods：回顾性图表审查所有儿童异位综合征从1968年至2002年。25例（14%）有新生儿胃肠道症状（喂养不耐受、呕吐）。其中11人进行了胃肠道造影检查，其中7人异常并导致手术。在152名无症状新生儿中，9名进行了放射学筛查，其中6名异常。只有一个人被认为有一个狭窄的基础肠系膜，但没有接受手术，由于心脏病。本组随访无肠道并发症。其他143名无症状儿童没有接受放射学筛查，并密切跟踪。4例随后出现胃肠道症状，并进行了对比研究;其中只有1例旋转不良，并接受了拉德手术。其余139例无症状，60例（43%）死于心脏病，没有发展肠道症状或并发症相关的旋转不良followup.Conclusion：无症状儿童异位综合征有低风险的不良后果相关的肠旋转异常。只要密切随访，并对发生胃肠道疾病的患者进行及时检查，可能不需要常规筛查。
Background: Heterotaxia syndrome involves multiple anomalies, including cardiac malformations and intestinal rotation abnormalities. Most authors recommend routine radiological evaluation, with laparotomy and Ladd procedure if a rotation abnormality is found.Aims: To determine if routine radiological screening is necessary, and if there is a group of children that can safely be managed expectantly.Methods: Retrospective chart review of all children with heterotaxia syndrome from 1968 to 2002.Results: Complete data were available for 177 patients. Twenty five (14%) had neonatal gastrointestinal symptoms ( feeding intolerance, vomiting). Eleven of these had gastrointestinal contrast studies, of which seven were abnormal and led to surgery. Of the 152 asymptomatic neonates, nine had radiological screening and six of these were abnormal. Only one was thought to have a narrow based mesentery, but did not undergo surgery due to cardiac disease. There were no intestinal complications on follow up in this group. The other 143 asymptomatic children did not undergo radiological screening and were closely followed. Four subsequently developed gastrointestinal symptoms and had contrast studies; only one of these had malrotation and underwent a Ladd procedure. Of the remaining 139 patients who remained asymptomatic, 60 (43%) died of cardiac disease and none developed intestinal symptoms or complications related to malrotation on follow up.Conclusion: Asymptomatic children with heterotaxia syndrome have a low risk of adverse outcome related to intestinal rotation abnormalities. Routine screening may not be necessary as long as close follow up is done, and prompt investigation is performed for those that develop gastrointestinal symptomatology.