Neurofibromatosis laríngea pediátrica: reporte de localización atípica / Pediatric neurofibromatosis of the larynx: report of atypical location

Los neurofibromas laríngeos (NFL) son tumores benignos poco frecuentes de localización principalmente supraglótica. Se manifiestan con síntomas obstructivos de la vía aérea. El tratamiento es la resección completa del tumor mediante abordaje endoscópico; se reserva la cirugía abierta para tumores de gran extensión. Se presenta el caso de un paciente pediátrico con localización atípica de NFL asociado a neurofibromatosis tipo 1 (NF1). Se realizó resección endoscópica del tumor y la anatomía patológica informó neurofibroma plexiforme. Es importante sospechar de esta patología en todo niño con estridor inspiratorio atípico progresivo. Se sugiere seguimiento a largo plazo por la alta probabilidad de recidiva.

Laryngeal neurofibromas (LNFs) are rare benign tumors mainly located in the supraglottis. LNFs occur with airway obstruction symptoms. The treatment is complete resection via an endoscopic technique; the open approach is reserved for large tumors. Here we describe the case of a pediatric patient with LNF of atypical location associated with neurofibromatosis type 1 (NF-1). The tumor was resected with an endoscopic technique, and the pathological study reported a plexiform neurofibroma. It is important to suspect this condition in any child with atypical, progressive inspiratory stridor. Long-term follow-up is recommended due to the high rate of recurrence

Pediatric neurofibromatosis of the larynx: report ofatypical location. / Neurofibromatosis laríngea pediátrica: reporte de localización atípica.

Laryngeal neurofibromas (LNFs) are rare benign tumors mainly located in the supraglottis. LNFs occur with airway obstruction symptoms. The treatment is complete resection via an endoscopic technique; the open approach is reserved for large tumors. Here we describe the case of a pediatric patient with LNF of atypical location associated with neurofibromatosis type 1 (NF-1). The tumor was resected with an endoscopic technique, and the pathological study reported a plexiform neurofibroma. It is important to suspect this condition in any child with atypical, progressive inspiratory stridor. Long-term follow-up is recommended due to the high rate of recurrence.

Los neurofibromas laríngeos (NFL) son tumores benignos poco frecuentes de localización principalmente supraglótica. Se manifiestan con síntomas obstructivos de la vía aérea. El tratamiento es la resección completa del tumor mediante abordaje endoscópico; se reserva la cirugía abierta para tumores de gran extensión. Se presenta el caso de un paciente pediátrico con localización atípica de NFL asociado a neurofibromatosis tipo 1 (NF1). Se realizó resección endoscópica del tumor y la anatomía patológica informó neurofibroma plexiforme. Es importante sospechar de esta patología en todo niño con estridor inspiratorio atípico progresivo. Se sugiere seguimiento a largo plazo por la alta probabilidad de recidiva.

Spreader Graft for Severe Deviation of Nasal Septum with Obstruction of the Internal Nasal Valve: Clinical and Functional Results.

Difficulty in nasal ventilation is one of the most frequently occurring problems in otorhinolaryngology and its correct diagnosis is the key step to solve it. The dysfunctions in the valve area are a frequent cause of chronic nasal obstruction, though commonly ignored. The objective of the study is to analyze the clinical and functional outcomes in a group of patients with septal deviations and valve compromise treated with spreader graft with endonasal approach. Thirty-five patients with septal deviation with compromise of the internal nasal valve (INV; area II of Cottle), treated with spreader graft and a minimum follow-up of 12 months, were included for analysis. Patients were evaluated with video nasosinusal endoscopy, photography, the Nasal Obstruction Symptom Evaluation (NOSE) questionnaire, and rhinomanometry (RM). Postoperative complications were recorded. The results obtained in the pre- and postoperative NOSE scores showed significant differences (p = 0.001), as also in pre- and postoperative RM tests (p < 0.001). Two complications were reported in the 35 patients; thus the complication rate in our sample was 6%. The use of spreader grafts, with endonasal approach, as nasal septum's tutors improved perpendicular septal deviations with compromise of the INV (area II), reaching an effective functional improvement in the nasal airway, with low rate of complications.