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Helmet-delivered heliox-CPAP in severe upper airway obstruction caused by PHACES syndrome

A Vivanco-Allende1, J Mayordomo-Colunga, A Coca-Pelaz

  • 1Pediatric Intensive Care Unit, Hospital Universitario Central de Asturias, Ovideo, Asturias, Spain. anaviall@hotmail.com.

Pediatric Pulmonology
|October 23, 2010
PubMed

Insights

PHACES syndrome in infants can cause severe airway obstruction due to hemangiomas. Helmet-delivered heliox-CPAP (HH-CPAP) effectively treated this obstruction in a 4-month-old patient.

Area of Science:

  • Pediatric Otolaryngology
  • Neonatal Respiratory Medicine
  • Medical Genetics

Background:

  • PHACES syndrome is a rare, complex disorder affecting multiple organ systems, including the airway.
  • Subglottic and tracheal hemangiomas are common in PHACES syndrome, potentially causing life-threatening airway obstruction.
  • Conventional therapies may have limitations for severe airway compromise in infants.

Observation:

  • A 4-month-old female infant presented with severe upper respiratory airway obstruction.
  • The obstruction was attributed to multiple subglottic and tracheal hemangiomas.
  • Standard treatments were considered, necessitating an alternative approach.

Findings:

  • Helmet-delivered heliox-CPAP (HH-CPAP) was administered to the infant.
  • HH-CPAP provided effective treatment for the severe upper respiratory airway obstruction.
  • The infant demonstrated significant clinical improvement with HH-CPAP.

Implications:

  • HH-CPAP represents a promising, non-invasive therapeutic option for infants with PHACES syndrome and airway hemangiomas.
  • This case highlights the potential utility of HH-CPAP in managing complex pediatric airway disorders.
  • Further research may explore the broader application of HH-CPAP in similar clinical scenarios.

Keywords:
PHACES syndromeairway obstructionhelioxhelmetnon-invasive ventilation

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