Abstract Introduction: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder with an inexorably progressive course which leads to a progressive neuromuscular weakness. Weight loss is one of the major bad prognostic factors in ALS. The placement of percutaneous endoscopic gastrostomy (PEG) is of paramount importance in patients with dysphagia to improve the disease outcomes, although some fear exists regarding the possible ventilatory complications during the procedure. The aim of this study was to evaluate the safety and effectiveness of PEG tube insertion under non-invasive ventilation (NIV) in patients with ALS and severe ventilatory impairment. Methods: A retrospective study of all consecutive PEGs placed in our department from May 2011 to January 2018 in patients with ALS was performed. The procedure was performed under non-invasive positive-pressure ventilation for ventilatory support. Results: We included 59 patients with ALS with severe ventilatory impairment, 58% were female, with a mean age of 67.2 ± 10.1 years and a median follow-up of 6 [2-15] months. The main indication for PEG placement was dysphagia (98%). The median time for PEG tube insertion since the established diagnosis of ALS was 12 [6-25] months and 4 [2-18] months since the beginning of bulbar symptoms. The majority of the patients had placed a 20-Fr PEG (63%) and under mild sedation with midazolam (80%), all under NIV. There were no immediate complications during and after the procedure (no episodes of aspiration or orotracheal intubation) and mortality. Conclusion: The placement of PEG is a very important procedure in patients with ALS and severe ventilatory impairment. The interdisciplinary department collaboration permitted the placement of PEG under NIV, in a safe and effective procedure in this special population.

Resumo Introdução: A esclerose lateral amiotrófica (ELA) é uma doença neurodegenerativa com um curso inexorável que leva a fraqueza neuromuscular progressiva. A perda de peso é um dos principais fatores de mau prognóstico na ELA. Apesar do receio de complicações ventilatórias durante o procedimento, a colocação de gastrostomia percutânea endoscópica em doentes com disfagia é extremamente importante para melhorar o prognóstico. O objetivo deste estudo é avaliar a segurança e eficácia da colocação de gastrostomia percutânea endoscópica (GEP) sob ventilação não invasiva (VNI) em doentes com ELA e disfunção ventilatória grave. Métodos: Estudo retrospetivo de todas as gastrostomias percutâneas endoscópicas colocadas em doentes com ELA no nosso departamento entre Maio 2011 e Janeiro 2018. O procedimento foi realizado sob VNI para suporte ventilatório. Resultados: Foram incluídos 59 doentes com ELA e disfunção ventilatória grave, 58% do sexo feminino, com uma idade média de 67.2 ± 10.1 anos e um follow-up mediano de 6 [2-15] meses. A principal indicação para colocação de gastrostomia percutânea endoscópica foi disfagia (98%). O tempo mediano para a colocação de GEP desde o diagnóstico de ELA foi 12 [6-25] meses e 4 [2-18] meses desde o início dos sintomas bulbares. A maioria dos doentes colocaram uma GEP de 20 Fr (63%) e sob sedação com midazolam (80%), todos sob VNI. Não se verificaram complicações imediatas durante e após o procedimento (sem episódios de aspiração ou entubação orotraqueal) e mortalidade. Conclusão: A colocação de GEP é um procedimento muito importante em doentes com ELA e disfunção ventilatória grave. A colaboração interdisciplinar permitiu a colocação de GEP sob ventilação não invasiva, tornando-o um procedimento seguro e eficaz nesta população especial.

Abstract Introduction: Confocal laser endomicroscopy is an optical imaging technique that allows in vivo, real-time, microscope-like images of the upper aerodigestive tract’s mucosa. The assessment of morphological tissue characteristics for the correct differentiation between healthy and malignant suspected mucosa requires strict evaluation criteria. Objective: This study aims to validate an eight-point score for the correct assessment of malignancy. Methods: We performed confocal laser endomicroscopy between March and October 2020 in 13 patients. 197 sequences (11.820 images) originated from the marginal area of pharyngeal and laryngeal carcinomas. Specimens were taken at corresponding locations and analyzed in H&E staining as a standard of reference. A total of six examiners evaluated the sequences based on a scoring system; they were blinded to the histopathological examination. The primary endpoints are sensitivity, specificity, and accuracy. Secondary endpoints are interrater reliability and receiver operator characteristics. Results: Healthy mucosa showed epithelium with uniform size and shape with distinct cytoplasmic membranes and regular vessel architecture. Confocal laser endomicroscopy of malignant cells demonstrated a disorganized arrangement of variable cellular morphology. We calculated an accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of 83.2%, 81.3%, 85.5%, 86.7%, and 79.7%, respectively, with a κ-value of 0.64, and an area under the curve of 0.86. Conclusion: The results confirm that this scoring system is applicable in the laryngeal and pharyngeal mucosa to classify benign and malignant tissue. A scoring system based on defined and reproducible characteristics can help translate this experimental method to broad clinical practice in head and neck diagnosis.