Laryngeal obstruction in congenital plasminogen deficiency

Jonathan Cohen, Shlomo Cohen, Malena Cohen Cymberknoh, Menachem Gross, Nir Hirshoren, David Shoseyov

Research output: Contribution to journalArticlepeer-review

Abstract

Type 1 congenital plasminogen deficiency (CPD) is a rare autosomal recessive disease which causes formation of fibrin pseudomembranes that affect multiple systems/organs: the eyes, respiratory system, urinary and genital systems, gastrointestinal system, and the central nervous system. We present a rare manifestation of the disease-severe upper airway obstruction due to a rapidly growing mass in the supraglottic region-6 months after dental treatment under general anesthesia. The management of such a manifestation has not been discussed in the current literature. Due to deterioration in his clinical status, the patient eventually underwent both a tracheotomy in order to bypass the obstruction, and excision of the supraglottic mass. Within a few days the mass recurred with complete obstruction of the upper airway.

Original languageEnglish
Pages (from-to)923-925
Number of pages3
JournalPediatric Pulmonology
Volume47
Issue number9
DOIs
StatePublished - Sep 2012
Externally publishedYes

Keywords

  • airway
  • larynx
  • plasminogen
  • polyp
  • supraglottis
  • tracheotomy

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