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An Evolving Paradigm

Christopher J. Hartnick, MD; Michael Rutter, MD; Florian Lang, MD; J. Paul Willging, MD; Robin T. Cotton, MD

Arch Otolaryngol Head Neck Surg. 2002;128:567-570.

Objectives  To refine the classic definition of, and provide a working definition for, congenital high airway obstruction syndrome (CHAOS) and to discuss the various aspects of long-term airway reconstruction, including the range of laryngeal anomalies and the various techniques for reconstruction.

Design  Retrospective chart review.

Patients  Four children (age range, 2-8 years) with CHAOS who presented to a single tertiary care children's hospital for pediatric airway reconstruction between 1995 and 2000.

Conclusions  To date, CHAOS remains poorly described in the otolaryngologic literature. We propose the following working definition for pediatric cases of CHAOS: any neonate who needs a surgical airway within 1 hour of birth owing to high upper airway (ie, glottic, subglottic, or upper tracheal) obstruction and who cannot be tracheally intubated other than through a persistent tracheoesophageal fistula. Therefore, CHAOS has 3 possible presentations: (1) complete laryngeal atresia without an esophageal fistula, (2) complete laryngeal atresia with a tracheoesophageal fistula, and (3) near-complete high upper airway obstruction. Management of the airway, particularly in regard to long-term reconstruction, in children with CHAOS is complex and challenging.

From the Department of Otolaryngology, Harvard Medical School and Massachusetts Eye and Ear Infirmary, Boston (Dr Hartnick); the Department of Pediatric Otolaryngology, Children's Hospital Medical Center, Cincinnati, Ohio (Drs Rutter, Willging, and Cotton); and the Otorhinolaryngology Service, Centre Hospitalier Universitaire, Lausanne, Switzerland (Dr Lang).

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