Emergent airway management in a case of fibrodysplasia ossificans progressiva

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Fibrodysplasia ossificans progressiva (FOP), or Stone man syndrome, is rare and one of the most disabling genetic conditions of the connective tissue due to progressive extraskeletal ossification. It usually presents in the first decade of life as painful inflammatory swellings, either spontaneously or in response to trauma, which later ossify and lead to severe disability. Progressive spinal deformity including thoracolumbar kyphoscoliosis leads to thoracic insufficiency syndrome, increasing the risk for pneumonia and right sided heart failure. We present the airway management in a 22-year-old male, diagnosed with FOP with severe disability, who required urgent airway intervention as a result of respiratory failure from pnuemonia. Tracheostomy triggers ossification and consequent airway obstruction at the tracheostomy site and laryngoscopy triggers temporomandibular joint ankylosis. Therefore, awake fiber-optic endotracheal intubation is recommended in these patients. Use of an airway endoscopy mask enabled us to simultaneously maintain non-invasive ventilation and intubate the patient in a situation where tracheostomy needed to be avoided.

Original languageEnglish (US)
Pages (from-to)565-567
Number of pages3
JournalJournal of Anaesthesiology Clinical Pharmacology
Volume30
Issue number4
DOIs
StatePublished - Oct 1 2014

Fingerprint

Myositis Ossificans
Airway Management
Tracheostomy
Osteogenesis
Noninvasive Ventilation
Laryngoscopy
Intratracheal Intubation
Airway Obstruction
Masks
Respiratory Insufficiency
Connective Tissue
Endoscopy
Pneumonia
Thorax
Heart Failure
Wounds and Injuries

All Science Journal Classification (ASJC) codes

  • Pharmacology, Toxicology and Pharmaceutics(all)
  • Pharmacology (medical)
  • Anesthesiology and Pain Medicine

Cite this

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title = "Emergent airway management in a case of fibrodysplasia ossificans progressiva",
abstract = "Fibrodysplasia ossificans progressiva (FOP), or Stone man syndrome, is rare and one of the most disabling genetic conditions of the connective tissue due to progressive extraskeletal ossification. It usually presents in the first decade of life as painful inflammatory swellings, either spontaneously or in response to trauma, which later ossify and lead to severe disability. Progressive spinal deformity including thoracolumbar kyphoscoliosis leads to thoracic insufficiency syndrome, increasing the risk for pneumonia and right sided heart failure. We present the airway management in a 22-year-old male, diagnosed with FOP with severe disability, who required urgent airway intervention as a result of respiratory failure from pnuemonia. Tracheostomy triggers ossification and consequent airway obstruction at the tracheostomy site and laryngoscopy triggers temporomandibular joint ankylosis. Therefore, awake fiber-optic endotracheal intubation is recommended in these patients. Use of an airway endoscopy mask enabled us to simultaneously maintain non-invasive ventilation and intubate the patient in a situation where tracheostomy needed to be avoided.",
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Emergent airway management in a case of fibrodysplasia ossificans progressiva. / Parekh, Uma ramesh; Read, Selina; Desai, Vimal; Budde, Arne.

In: Journal of Anaesthesiology Clinical Pharmacology, Vol. 30, No. 4, 01.10.2014, p. 565-567.

Research output: Contribution to journalArticle

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