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Laryngeal obstruction in congenital plasminogen deficiency

Jonathan Cohen1, Shlomo Cohen, Malena Cohen Cymberknoh

  • 1Department of Otolaryngology/Head and Neck Surgery, Hadassah Medical Center, Hebrew University, Jerusalem, Israel.

Pediatric Pulmonology
|February 14, 2012
PubMed

Insights

Type 1 congenital plasminogen deficiency (CPD) can cause fibrin pseudomembranes affecting multiple organs. A rare case presented severe upper airway obstruction from a rapidly growing supraglottic mass, requiring urgent intervention.

Area of Science:

  • Rare genetic disorders
  • Hematology
  • Otorhinolaryngology

Background:

  • Type 1 congenital plasminogen deficiency (CPD) is a rare autosomal recessive disorder.
  • CPD leads to fibrin pseudomembrane formation impacting various organ systems.
  • Manifestations include ocular, respiratory, urinary, gastrointestinal, and central nervous system involvement.

Observation:

  • A patient with Type 1 CPD developed severe upper airway obstruction.
  • The obstruction was caused by a rapidly growing supraglottic mass.
  • This rare complication occurred six months after dental treatment under general anesthesia.

Findings:

  • The supraglottic mass led to complete upper airway obstruction.
  • Surgical excision and tracheotomy were performed to manage the obstruction.
  • The mass recurred rapidly within days, causing re-obstruction.

Implications:

  • This case highlights a previously undocumented, severe airway complication of Type 1 CPD.
  • Management strategies for this rare manifestation require further investigation.
  • Prompt recognition and intervention are crucial for patients with CPD presenting with airway compromise.

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