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Progressive Airway Obstruction due to Plexiform Neurofibromain 7 Years-Old Female

Journal: International Journal of Science and Research (IJSR) (Vol.8, No. 2)

Publication Date:

Authors : ; ; ; ; ;

Page : 2289-2292

Keywords : neurofibroma; plexiform neurofibroma; airway obstruction;

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Background: Extention to larynx of Plexiform neurofibromas are extremely rare, approximately for only 0, 03 to 0, 1 % of benign tumors of the larynx, it is one of neurofibroma type 1 (NF-1) variant.1, 6 Surgery is the only choice to achieve temporary tumor growth control and symptom progression. Patient with small plexiform neurofibroma eligible for complete resection of tumor. Case: 7 years old female presented with dyspnea. Physical examination showed a lot of subcutaneous neurofibroma. Endoscopy shown massive airway obstruction, that was narrowed by greater than 50 % than decide emergency tracheostomy. Unfortunately, the patient getting worse because of progressive airway obstruction lower the site of tracheostomy and patient death because airway obstruction Learning Points: Tumors location in head/neck/face cannot be completely removed, this tumor had been reported difficult to separate from normal tissue because they lack a well-defined capsule. Airway compression and spontaneous bleeding are direct lethal complication of plexiform neurofibroma.

Last modified: 2021-06-28 17:24:41