Laryngeal obstruction in congenital plasminogen deficiency.
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.
Department of Otolaryngology/Head and Neck Surgery, Hadassah Medical Center, Hebrew University, Jerusalem, Israel.
Jonathan
J
Cohen
Shlomo
S
Cohen
Malena Cohen
MC
Cymberknoh
Menachem
M
Gross
Nir
N
Hirshoren
David
D
Shoseyov
D000402:Airway Obstruction / Q000209:etiology* / Q000601:surgery; D002675:Child, Preschool; D003231:Conjunctivitis / Q000150:complications* / Q000601:surgery; D006801:Humans; D007818:Laryngeal Diseases / Q000209:etiology* / Q000601:surgery; D008297:Male; D010958:Plasminogen / Q000172:deficiency; D011127:Polyps / Q000209:etiology* / Q000601:surgery; D012873:Skin Diseases, Genetic / Q000150:complications* / Q000601:surgery; D014140:Tracheotomy
D010958:Plasminogen