Laryngeal Obstruction in Congenital Plasminogen Deficiency

Pediatr Pulmonol. 2012 Sep;47(9):923-5. doi: 10.1002/ppul.22522. Epub 2012 Feb 10.

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Airway Obstruction / etiology*
  • Airway Obstruction / surgery
  • Child, Preschool
  • Conjunctivitis / complications*
  • Conjunctivitis / surgery
  • Humans
  • Laryngeal Diseases / etiology*
  • Laryngeal Diseases / surgery
  • Male
  • Plasminogen / deficiency
  • Polyps / etiology*
  • Polyps / surgery
  • Skin Diseases, Genetic / complications*
  • Skin Diseases, Genetic / surgery
  • Tracheotomy

Substances

  • Plasminogen

Supplementary concepts

  • Plasminogen Deficiency, Type I