Long-Term Outcomes of Congenital High Airway Obstruction Syndrome at a Single Comprehensive Fetal Center.

IF 1.7 3区医学 Q3 OBSTETRICS & GYNECOLOGY

Fetal Diagnosis and Therapy Pub Date : 2026-05-29 DOI:10.1159/000552628

Monica L Wagner, Jose L Peiro, Beth A Rymeski, Matthew M Smith, Alessandro de Alarcon, David N McKinney, Mounira A Habli, Foong-Yen Lim

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引用次数: 0

Abstract

Introduction: Congenital high airway obstruction syndrome (CHAOS) is characterized by over-distended lungs leading to impaired cardiac return and fetal hydrops. Survivors have been reported following prenatal spontaneous fistulization, fetal procedures to decompress the airway, or ex utero intrapartum treatment (EXIT). The long-term outcomes of survivors are unclear.

Methods: We performed a retrospective chart review on patients diagnosed with CHAOS in our center between 2005 and 2025.

Results: Of the 28 patients with CHAOS, three (10.7%) underwent a fetal procedure to decompress the airway. Three (10.7%) patients had evidence of spontaneous fistulization. Four (14.3%) patients terminated the pregnancy and four (14.3%) had in utero fetal demise. Twenty (71.4%) patients were live-born; of these, 14 (70%) died shortly after delivery and two (10%) died in the neonatal period. Seven (35%) patients underwent EXIT-to-tracheostomy at our center, of which four (57.1%) are long-term survivors ranging in age from 4 to 19 years old. Three patients have undergone airway reconstruction between 1.6 and 5.6 years of age; one remains tracheostomy dependent due to recurrent airway stenosis, 1 patient has undergone reconstruction and is likely to be decannulated soon, and 1 patient had successful reconstruction and was decannulated. The fourth patient has not yet undergone airway reconstruction.

Conclusion: CHAOS remains a highly morbid diagnosis, but long-term survivorship and liberation from tracheostomy is possible.

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先天性高气道阻塞综合征（CHAOS）在单一综合胎儿中心的长期预后。

目的：先天性高气道阻塞综合征（CHAOS）的特点是肺部过度扩张导致心脏回流受损和胎儿水肿。据报道，幸存者在产前自发瘘，胎儿手术减压气道，或子宫外分娩时治疗（EXIT）。幸存者的长期预后尚不清楚。方法：我们对我院2005-2025年间诊断为CHAOS的患者进行回顾性分析。结果：在28例CHAOS患者中，3例（10.7%）接受了胎儿手术来减压气道。3例（10.7%）有自发性瘘管形成的证据。终止妊娠4例（14.3%），宫内死胎4例（14.3%）。活产20例（71.4%）；其中，14例（70%）在分娩后不久死亡，2例（10%）在新生儿期死亡。7例患者（35%）在本中心接受了出口-气管切开术，其中4例（57.1%）是4 - 19岁的长期幸存者。3例患者在1.6 - 5.6岁之间进行了气道重建；1例患者因气道狭窄复发，仍需气管造口术；1例患者进行了气管重建，可能很快会去管；1例患者重建成功，并已去管。第四名患者尚未进行气道重建。结论：混沌仍然是一个高度病态的诊断，但长期生存和从气管切开术中解放是可能的。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Fetal Diagnosis and Therapy 医学-妇产科学

CiteScore

4.70

自引率

9.10%

发文量

审稿时长

6-12 weeks

期刊介绍： The first journal to focus on the fetus as a patient, ''Fetal Diagnosis and Therapy'' provides a wide range of biomedical specialists with a single source of reports encompassing the common discipline of fetal medicine.