PROGRESSIVE AIRWAY OBSTRUCTION DUE TO PLEXIFORM NEUROFIBROMA IN 7-YEARS-OLD FEMALE
Putu Diah Pratiwi,Ida bagus Subanada,Siadi Purniti,Ayu Setyorini,I Gde Ardika Nuaba
Departement of Child Health Udayana Univ Department of OtolaryngologyHead and Neck Surgery
Background : Plexiform neurofibroma is a variant of congenital neurofibroma type 1 (NF1), occurred in 25% to 50% of children with NF.1 Plexiform neurofibromas is a nonmetastatic and locally invasive tumor that can occur on the skin or along the peripheral nerves. Plexiform neurofibromas which extended to larynx are extremely rare, accounting for only 0,03 to 0,1% of benign tumors of the larynx. Up to our knowledge, this is the first case of invasive plexiform neurofibroma that caused progressive airway obstruction. Surgery is the only modality that has been shown to achieve at least temporary control of tumor growth and symptom progression. Complete tumor resection was possible only in patient with small plexiform neurofibroma.
Case Presentation Summary : A 7-years-old girl was admitted to the hospital with dyspnea, treated as pneumonia with poor clinical outcome. Further physical examination revealed wide area resembling subcutaneous neurofibroma lesion on the right neck extended to half of the chest. Similar dermatological feature observed paternally. Imaging studies revealed bone deformity with massive cervical spine plexiform neurofibroma lesion that caused external compression of the larynx. Massive airway obstruction was later found endoscopically. Later on, patient’s airway was narrowed by greater than 50% and urgent placement of tracheostomy tube planned, but unfortunately patient deteriorated swiftly due to progressive airway obstruction at the lower site of existing tracheostomy. Despite adequate resuscitation performed, death due to progressive airway obstruction could not be avoided.
Learning Points/Discussion : Airway compression and spontaneous bleeding are direct lethal complication of plexiform neurofibroma. In our case, tracheostomy already performed but airway obstruction continued progressively below the tube. Complete resection was unable because neurofibroma located in head/neck/face cannot be completely removed surgically as it difficult to separate from normal tissue due to lack a well-defined capsule of the tumor. A very attentive airway evaluation in plexiform neurofibroma may warrant a better outcome and prevent lethal complication.