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头颈部淋巴管畸形的产时宫外治疗(EXIT):三例病例讨论及EXIT-团队时间程序(TTP)清单建议

EXIT (Ex utero Intrapartum Treatment) in lymphatic malformations of the head and neck: discussion of three cases and proposal of an EXIT-TTP (Team Time Procedure) list.

作者信息

Stefini Stefania, Bazzana Tullia, Smussi Cinzia, Piccioni Michela, Frusca Tiziana, Taddei Fabrizio, Tomasoni Gabriele, Recupero Daniela, Cavazza Alessandra, Villani Paolo, Nicolai Piero, Eivazi Behfar, Wiegand Susanne, Werner Jochen Alfred, Schmidt Stephan, Maier Rolf F, Torossian Alexander

机构信息

Department of Otorhinolaryngology, University of Brescia - Spedali Civili of Brescia, Italy.

出版信息

Int J Pediatr Otorhinolaryngol. 2012 Jan;76(1):20-7. doi: 10.1016/j.ijporl.2011.09.015. Epub 2011 Nov 10.

DOI:10.1016/j.ijporl.2011.09.015
PMID:22078744
Abstract

OBJECTIVES

Ex utero Intrapartum Treatment (EXIT) is a technique to secure the fetal airway while oxygenation is maintained through utero-placental circulation. The aim of the study is to present three cases of fetal lymphatic malformation of the head and neck that required EXIT and to summarize EXIT details.

METHODS

The cases were studied before the delivery and EXIT was planned with a multidisciplinary team. The key factors of EXIT are considered and the type, stage and clinical score of the three lymphatic malformations are defined.

RESULTS

In the three cases of EXIT the time working on placental support to secure the airway was 9, 7, and 9 min, respectively (from the hysterotomy to clamping the umbilical cord). Procedures performed on the airway were laryngo-tracheo-bronchoscopy in the first case, laryngoscopy and intubation in the second one, laryngoscopy, drainage of the lymphatic macro-cyst, and intubation in the third case. A sketching to detail the EXIT steps are presented: EXIT-Team Time Procedure list (EXIT-TTP list). Lymphatic malformations were classified as mixed (micro/macro-cystic) in two cases, and macro-cystic in one. de Serres Stage was IV, V and II. Therapy varied in the three neonates (surgery alone, surgery+Picibanil+Nd-YAG, or Picibanil alone).

CONCLUSIONS

In case of prenatal suspicion of airway obstruction, EXIT should be planned with a multidisciplinary team. The EXIT-Team Time Procedure list (EXIT-TTP list), reviews the most critical phases of the procedure when different teams are working together. The type of lymphatic malformation, the anatomic location and the clinical score predict the outcome.

摘要

目的

产时宫外治疗(EXIT)是一种在通过子宫 - 胎盘循环维持氧合的同时确保胎儿气道安全的技术。本研究的目的是介绍三例需要EXIT治疗的头颈部胎儿淋巴管畸形病例,并总结EXIT的详细情况。

方法

在分娩前对病例进行研究,并由多学科团队制定EXIT计划。考虑EXIT的关键因素,并确定三种淋巴管畸形的类型、分期和临床评分。

结果

在三例EXIT病例中,依靠胎盘支持确保气道安全的时间分别为9分钟、7分钟和9分钟(从子宫切开术到夹住脐带)。第一例进行的气道操作是喉 - 气管 - 支气管镜检查,第二例是喉镜检查和插管,第三例是喉镜检查、淋巴管大囊肿引流和插管。给出了一个详细描述EXIT步骤的示意图:EXIT团队时间程序清单(EXIT - TTP清单)。两例淋巴管畸形分类为混合型(微/大囊型),一例为大囊型。德塞雷斯分期分别为IV期、V期和II期。三名新生儿的治疗方法各不相同(单独手术、手术 + 匹鲁卡品 + 钕钇铝石榴石激光,或仅用匹鲁卡品)。

结论

如果产前怀疑气道阻塞,应与多学科团队共同制定EXIT计划。EXIT团队时间程序清单(EXIT - TTP清单)回顾了不同团队协同工作时该程序最关键的阶段。淋巴管畸形的类型、解剖位置和临床评分可预测预后。

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