Wu Qiang,Zhong Wei,Lyu Junjian,et al.Programmatic management of cervical lymphatic malformations with airway obstruction[J].Journal of Clinical Pediatric Surgery,2023,22(08):726-730.[doi:10.3760/cma.j.cn101785-202306043-005]
伴气道梗阻的颈部淋巴管畸形患儿产前产后程序化管理
- Title:
- Programmatic management of cervical lymphatic malformations with airway obstruction
- Keywords:
- Lymphatic Malformations; Airway Obstruction; Programmed; Treatment; Prognosis
- 摘要:
- 目的 总结伴气道梗阻的颈部淋巴管畸形（cervical lymphatic malformation with airway obstruction，CLMAO）患儿从产前评估到出生后干预程序化诊疗的临床经验。方法 回顾性分析2015年7月至2023年3月广州市妇女儿童医疗中心新生儿外科监护室收治的22例CLMAO患儿的临床资料。其中9例于本院出生，13例由外院转入。收集并分析患儿胎儿期评估、产时处理、出生后治疗及预后等资料。根据出生后治疗方案分为早期治疗组（6例）和程序化治疗组（16例），比较两组治疗结果、呼吸机辅助时间及住院时间等。结果 9例本院出生的患儿均在产前行胎儿MRI评估气管食管位移指数（tracheoesophageal displacement index，TEDI）或羊水量。其中，8例存在TEDI>12 mm或羊水过多，出生后因呼吸困难实施产时子宫外处理（ex-utero intrapartum treatment，EXIT），1例TEDI ≤ 12 mm且羊水量正常者于断脐后出现呼吸困难而行气管插管。产前对气道梗阻评估准确性为88.9%（8/9）。程序化治疗组最终3例经手术治疗脱离呼吸支持。程序化治疗组与早期手术治疗组相比，气管造口例数、呼吸机辅助时间及住院时间差异无统计学意义（P>0.05）；随访6~39个月，两组治愈率及好转率差异亦无统计学意义（P>0.05）。结论 对怀疑为CLMAO的胎儿应进行TEDI及羊水量评估。与早期手术相比，程序化治疗可取得相似预后，可考虑作为CLMAO的一种新治疗方案。
- Abstract:
- Objective To summarize the clinical experiences of managing cervical lymphatic malformation with airway obstruction(CLMAO) from prenatal assessments to postnatal interventions.Methods Retrospective analysis was conducted for clinical data of 22 hospitalized CLMAO infants from July 2015 to March 2023.Nine cases were born at our hospital while another 13 infants were transferred from other hospitals.They were assigned into two groups of early surgical treatment(n=6) and protocol treatment(n=16).Fetal assessments, intrapartum managements and postnatal interventions were recorded.Surgical complications, duration of mechanical ventilation, length of hospital stay and other prognostic factors were compared between two groups.Results Nine infants born at our hospital were examined by prenatal magnetic resonance imaging(MRI) for assessing tracheoesophageal displacement index(TEDI) or amniotic fluid volume.Eight infants had TEDI>12 mm or polyhydramnios.Ex-utero intrapartum treatment(EXIT) was offered for postnatal dyspnea.One case of TEDI ≤ 12 mm and normal amniotic fluid volume had dyspnea after umbilical cord clamping and underwent tracheal intubation.The accuracy of prenatal assessment for airway obstruction was 88.9%(8/9).Three cases were operated in protocol treatment group.As compared with early treatment group, no significant inter-group differences existed in proportion of tracheostomy, duration of mechanical ventilation or length of hospital stay.During a follow-up period of(6-39) months, there was no significant difference in cure rate or improvement rate. Conclusion Fetuses with suspected CLMAO should be evaluated for TEDI and amniotic fluid volume.As compared with early surgery, programmed therapy may obtain comparable outcomes.It offers a novel therapeutic option for CLMAO.
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备注/Memo
收稿日期:2023-06-27。
基金项目:广州市基础研究计划-基础与应用基础研究项目（202102080511）
通讯作者:何秋明,Email:qiuminghe@foxmail.com