Síndrome de hamman en neumonía por SARS-COV-2 / Hamman's syndrome in SARS-CoV-2 pneumonia

Resumen El síndrome de Hamman, o neumomediastino es pontáneo, es la presencia de aire en mediastino en pacientes sin antecedentes de enfermedad pulmonar previa, trauma torácico o iatrogenia. Se ha descrito como una complicación rara en pacientes con neumo nía por COVID-19. Se postula que un aumento en la presión de la vía aérea asociado a daño alveolar difuso generado por el virus, producen una fuga de aire hacia el mediastino. El dolor torácico y disnea, asociado a enfisema subcutáneo, deben hacer sospechar al médico tratante. Presentamos un paciente de 79 años que du rante su internación por neumonía secundaria al virus SARS-CoV-2 evolucionó súbitamente con disnea, dolor torácico, accesos de tos y broncoespasmo con hallaz go de neumomediastino espontáneo en la tomografía de tórax. Evolucionó favorablemente con tratamiento broncodilatador y oxigenoterapia. El síndrome de Ham man es una causa poco frecuente de progresión de insuficiencia respiratoria en pacientes con neumonía por COVID-19. Su identificación es crucial para imple mentar el tratamiento adecuado.

Abstract Hamman's syndrome, or spontaneous pneumome diastinum, is the presence of air in the mediastinum without a history of previous pulmonary pathology, chest trauma or iatrogenesis. It has been described as a rare complication in patients with COVID-19 pneu monia. It is postulated that an increase in airway pres sure associated with diffuse alveolar damage caused by the virus produces an air leak into the mediastinum. Chest pain and dyspnea associated with subcutaneous emphysema should make the treating physician suspi cious. We introduce a 79-year-old patient who, during hospitalization for pneumonia secondary to COVID-19, suddenly developed dyspnea, chest pain, coughing spells and bronchospasm with the discovery of spontaneous pneumomediastinum on chest tomography. He evolved favorably with bronchodilator treatment and temporary oxygen therapy. Hamman's syndrome is a rare cause of respiratory failure progression in patients with COVID-19 pneumonia. Its recognition is crucial to implement the appropriate treatment.

[Hamman's syndrome in SARS-CoV-2 pneumonia]. / Síndrome de Hamman en neumonía por SARS-CoV-2.

Hamman's syndrome, or spontaneous pneumomediastinum, is the presence of air in the mediastinum without a history of previous pulmonary pathology, chest trauma or iatrogenesis. It has been described as a rare complication in patients with COVID-19 pneumonia. It is postulated that an increase in airway pressure associated with diffuse alveolar damage caused by the virus produces an air leak into the mediastinum. Chest pain and dyspnea associated with subcutaneous emphysema should make the treating physician suspicious. We introduce a 79-year-old patient who, during hospitalization for pneumonia secondary to COVID-19, suddenly developed dyspnea, chest pain, coughing spells and bronchospasm with the discovery of spontaneous pneumomediastinum on chest tomography. He evolved favorably with bronchodilator treatment and temporary oxygen therapy. Hamman's syndrome is a rare cause of respiratory failure progression in patients with COVID-19 pneumonia. Its recognition is crucial to implement the appropriate treatment.

El síndrome de Hamman, o neumomediastino espontáneo, es la presencia de aire en mediastino en pacientes sin antecedentes de enfermedad pulmonar previa, trauma torácico o iatrogenia. Se ha descrito como una complicación rara en pacientes con neumonía por COVID-19. Se postula que un aumento en la presión de la vía aérea asociado a daño alveolar difuso generado por el virus, producen una fuga de aire hacia el mediastino. El dolor torácico y disnea, asociado a enfisema subcutáneo, deben hacer sospechar al médico tratante. Presentamos un paciente de 79 años que durante su internación por neumonía secundaria al virus SARS-CoV-2 evolucionó súbitamente con disnea, dolor torácico, accesos de tos y broncoespasmo con hallazgo de neumomediastino espontáneo en la tomografía de tórax. Evolucionó favorablemente con tratamiento broncodilatador y oxigenoterapia. El síndrome de Hamman es una causa poco frecuente de progresión de insuficiencia respiratoria en pacientes con neumonía por COVID-19. Su identificación es crucial para implementar el tratamiento adecuado.

Proteinosis alveolar pulmonar / Pulmonary alveolar proteinosis

Resumen La proteinosis alveolar pulmonar (PAP) es una enfermedad pulmonar difusa, infrecuente, secundaria a una alteración en la homeostasis del surfactante. Se presenta el caso de una mujer de 69 años que ingresó a sala de internación por disnea progresiva hasta clase funcional III, de tres meses de evolución, asociada a tos no productiva. Se constató insuficiencia respiratoria tipo I. Como hallazgos en tomografía de tórax se evidenció engrosamiento del intersticio pulmonar intra e interlobulillar, opacidades en vidrio esmerilado y áreas con tendencia a la consolidación bilateral. Se realizó biopsia pulmonar con diagnóstico histológico de PAP y se efectuó tratamiento con lavado pulmonar total, logrando mejoría clínica. Se destaca la necesidad de tener presente diagnósticos diferenciales de insuficiencia respiratoria e infiltrados pulmonares en el contexto de la pandemia por COVID-19, incluidas las entidades muy poco prevalentes como lo es la PAP.

Abstract Pulmonary alveolar proteinosis (PAP) is a rare, diffuse pulmonary disease due to abnormal surfactant homeostasis. We present the case of a 69-year-old woman who was admitted to the hospital for progressive dyspnea with marked limitation in activity, and non-productive cough, of three months of evolution. Type I respiratory failure was confirmed. Chest tomography findings were interlobular and intralobular septal thickening, ground glass opacities and bilateral consolidation. Histological diagnosis was made and whole-lung lavage was performed with clinical improvement. We highlight the need to keep in mind differential diagnoses of respiratory failure and pulmonary infiltrates during COVID-19 pandemic, even rare entities such as PAP.

[Pulmonary alveolar proteinosis]. / Proteinosis alveolar pulmonar.

Pulmonary alveolar proteinosis (PAP) is a rare, diffuse pulmonary disease due to abnormal surfactant homeostasis. We present the case of a 69-year-old woman who was admitted to the hospital for progressive dyspnea with marked limitation in activity, and non-productive cough, of three months of evolution. Type I respiratory failure was confirmed. Chest tomography findings were interlobular and intralobular septal thickening, ground glass opacities and bilateral consolidation. Histological diagnosis was made and whole-lung lavage was performed with clinical improvement. We highlight the need to keep in mind differential diagnoses of respiratory failure and pulmonary infiltrates during COVID-19 pandemic, even rare entities such as PAP.

La proteinosis alveolar pulmonar (PAP) es una enfermedad pulmonar difusa, infrecuente, secundaria a una alteración en la homeostasis del surfactante. Se presenta el caso de una mujer de 69 años que ingresó a sala de internación por disnea progresiva hasta clase funcional III, de tres meses de evolución, asociada a tos no productiva. Se constató insuficiencia respiratoria tipo I. Como hallazgos en tomografía de tórax se evidenció engrosamiento del intersticio pulmonar intra e interlobulillar, opacidades en vidrio esmerilado y áreas con tendencia a la consolidación bilateral. Se realizó biopsia pulmonar con diagnóstico histológico de PAP y se efectuó tratamiento con lavado pulmonar total, logrando mejoría clínica. Se destaca la necesidad de tener presente diagnósticos diferenciales de insuficiencia respiratoria e infiltrados pulmonares en el contexto de la pandemia por COVID-19, incluidas las entidades muy poco prevalentes como lo es la PAP.

[Dermatomyositis associated with anti-MDA5 autoantibody]. / Dermatomiositis asociada al autoanticuerpo anti-MDA5.

Dematomyositis is an idiopathic inflammatory myopathy with a variable clinical spectrum. In recent years, a number of myositis-specific antibodies have been identified including anti-MDA5, which is us eful for diagnosis, prognosis and classification of the diverse clinical forms of the disease. This antibody is associated with cutaneous ulcers, rapidly progressive interstitial lung disease, early mortality and poor prognosis, so the detection of this antibody in a suitable clinical context, raises the need for an aggressive immunosuppressive treatment. We describe a case of dermatomyositis classified as hypomyopathic (i.e. involving mild muscle weakness), presenting specific skin lesions, interstitial lung disease, and presence of anti-MDA5 antibody that had a favorable response to combined treatment with cyclophosphamide, gamma globulin and corticosteroids.

Dermatomiositis asociada al autoanticuerpo anti-MDA5 / Dermatomyositis associated with anti-MDA5 autoantibody

La dematomiositis es una miopatía inflamatoria idiopática con espectro clínico variable. En los últimos años se ha identificado un número de autoanticuerpos específicos de miositis útiles para el diagnóstico, la clasificación y el pronóstico de las diversas formas de la enfermedad, entre los que se encuentra el anti-MDA5. Este anticuerpo se asocia al desarrollo de úlceras cutáneas, enfermedad intersticial pulmonar rápidamente progresiva, mortalidad temprana y mal pronóstico por lo que la detección del mismo, en un contexto clínico adecuado, plantea la necesidad de un tratamiento inmunosupresor agresivo. Describimos un caso de dermatomiositis hipomiopática, (es decir, con afección muscular leve) que presentaba compromiso cutáneo específico, enfermedad pulmonar intersticial y anticuerpo anti-MDA5 que respondió favorablemente al tratamiento combinado con ciclofosfamida, gamaglobulina y corticoides.

Dematomyositis is an idiopathic inflammatory myopathy with a variable clinical spectrum. In recent years, a number of myositis-specific antibodies have been identified including anti-MDA5, which is us eful for diagnosis, prognosis and classification of the diverse clinical forms of the disease. This antibody is associated with cutaneous ulcers, rapidly progressive interstitial lung disease, early mortality and poor prognosis, so the detection of this antibody in a suitable clinical context, raises the need for an aggressive immunosuppressive treatment. We describe a case of dermatomyositis classified as hypomyopathic (i.e. involving mild muscle weakness), presenting specific skin lesions, interstitial lung disease, and presence of anti-MDA5 antibody that had a favorable response to combined treatment with cyclophosphamide, gamma globulin and corticosteroids.

Perforación esofágica por cuerpo extraño / Esophageal perforation by body strange

Mujer de 43 años que consultó a guardia por dolor en el compartimiento central del cuello, región retroesternal y disfagia de 48 hs. de evolución, asociado a fiebre y escalofríos. Refirió ingesta de carne molida (hamburguesa) antes del comienzo de los síntomas. En el examen físico se constato temperatura de 38 °C, eritema en región paratraqueal izquierda con dolor a la palpación y enfisema subcutáneo y en el laboratorio tenía un recuento de glóbulos blancos de 13.000/mm3. En las imágenes de cuello y tórax se confirmo la presencia de enfisema subcutáneo en región cervical izquierda, cuerpo extraño en esófago cervical y rarefacción de la grasa paratraqueal izquierda (Fig. 1 a y b, flechas). Se realizó esofagoscopia rígida para remoción del cuerpo extraño (vidrio), drenaje de absceso cervical y mediastino superior, esofagostoma cervical y gastrostomía de alimentación (Fig. 2 a y b, flechas). El hallazgo fue una perforación en el borde izquierdo de 2 cm de diámetro y otra de 5 mm en cara posterior del esófago cervical con absceso paraesofágico. La paciente evoluciono favorablemente, cumplió 7 días de tratamiento antibiótico y se otorgo alta institucional con control ambulatorio

[Clinical manifestations of organizing pneumonia]. / Manifestaciones clínicas de la neumonía en organización.

Organizing pneumonia is a clinical entity asociated with nonspecific symptoms and radiological findings and abnormalities in pulmonary function tests. It is defined by the characteristic histopathological pattern: filling of alveoli and respiratory bronchioles by plugs of granulation tissue. It can be idiopathic (COP) or secondary to other causes (SOP). It is an unusual finding and the clinical and radiographic findings are nonspecific. For specific diagnosis an invasive procedure has to be done, but often empirical treatment is started when there's a clinical suspicion. We describe the clinical characteristics of 13 patients with histological diagnosis of organizing pneumonia. Data was obtained from their medical records. The median age was 76 years and the median time to diagnosis from the onset of symptoms was 31 days. In 10 cases the diagnosis was made by transbronchial biopsy. 8 patients required hospitalization, 4 of them received high doses of steroids and 3 required ventilatory support. One patient died from a cause attributable to this entity and 5 relapsed. Dyspnea, cough and fever were the most frequent symptoms. Most patients had more than one tomographic pattern being the most common ground glass opacities and alveolar consolidation. Nine patients were diagnosed with COP and 4 with SOP. The most frequent underlying cause of SOP was drug toxicity. The clinical characteristics of the reported cases are consistent with previously published series. As an interesting feature, there was a group of patients that needed high doses of steroids and ventilatory support.

Manifestaciones clínicas de la neumonía en organización / Clinical manifestations of organizing pneumonia

La neumonía en organización es una entidad clínica asociada a síntomas y hallazgos radiológicos inespecíficos y anomalías en las pruebas de función pulmonar. El patrón histopatológico característico se define por tapones intra-alveolares de tejido de granulación. Puede presentarse sin una etiología determinada -neumonía en organización criptogénica (COP, cryptogenic organizing pneumonia)- o en otra condición clínica -neumonía en organización secundaria (SOP: secondary organizing pneumonia). Es poco frecuente y las manifestaciones clínico-radiológicas son poco específicas. Para su confirmación se requieren procedimientos invasivos, siendo habitual el tratamiento empírico con esteroides sistémicos ante la sospecha clínica. Se presentan 13 casos con diagnóstico histológico de neumonía en organización describiendo las características clínicas. La mediana de edad fue 76 años y la de tiempo al diagnóstico desde el inicio de los síntomas: 31 días. En 10 casos el diagnóstico fue por biopsia transbronquial (BTB). Ocho pacientes requirieron internación, 4 de ellos recibieron pulsos de esteroides y soporte ventilatorio. Uno falleció por una causa atribuible a la entidad y 5 presentaron recaídas. Disnea, tos y fiebre fueron los síntomas más frecuentes. La mayoría presentó más de un patrón tomográfico, siendo los más habituales vidrio esmerilado y consolidación alveolar. En 9 se realizó el diagnóstico de COP y en 4 de SOP. La causa secundaria en todos los casos fue toxicidad por drogas. Las características clínicas de los casos comunicados son consistentes con las series previamente publicadas. Cabe destacar la necesidad de tratamiento con dosis altas de esteroides y soporte ventilatorio en un grupo de pacientes.

Organizing pneumonia is a clinical entity asociated with nonspecific symptoms and radiological findings and abnormalities in pulmonary function tests. It is defined by the characteristic histopathological pattern: filling of alveoli and respiratory bronchioles by plugs of granulation tissue. It can be idiopathic (COP) or secondary to other causes (SOP). It is an unusual finding and the clinical and radiographic findings are nonspecific. For specific diagnosis an invasive procedure has to be done, but often empirical treatment is started when there’s a clinical suspicion. We describe the clinical characteristics of 13 patients with histological diagnosis of organizing pneumonia. Data was obtained from their medical records. The median age was 76 years and the median time to diagnosis from the onset of symptoms was 31 days. In 10 cases the diagnosis was made by transbronchial biopsy. 8 patients required hospitalization, 4 of them received high doses of steroids and 3 required ventilatory support. One patient died from a cause attributable to this entity and 5 relapsed. Dyspnea, cough and fever were the most frequent symptoms. Most patients had more than one tomographic pattern being the most common ground glass opacities and alveolar consolidation. Nine patients were diagnosed with COP and 4 with SOP. The most frequent underlying cause of SOP was drug toxicity. The clinical characteristics of the reported cases are consistent with previously published series. As an interesting feature, there was a group of patients that needed high doses of steroids and ventilatory support.

Blunted Hypercapnic Respiratory Drive Response in Subjects With Late-Onset Pompe Disease.

BACKGROUND: Patients with late-onset Pompe disease develop progressive hypercapnic respiratory failure that can be disproportionate to the respiratory muscle compromise and/or thoracic restriction. Although recent studies have reported the presence of a blunted hypercapnic respiratory response in some subjects with neuromuscular disorders and chronic hypercapnia, no study has evaluated the integrity of the respiratory drive in subjects with late-onset Pompe disease. Thus, we endeavor to determine the CO2 rebreathing response in subjects with late-onset Pompe disease. METHODS: Respiratory muscle strength was assessed by measuring the maximum inspiratory pressure, and the maximum expiratory pressure. The maximum inspiratory pressure reflects the strength of the diaphragm and other inspiratory muscles, whereas the maximum expiratory pressure reflects the strength of the abdominal muscles and other expiratory muscles. We studied the hypercapnic drive response (measured as the ratio of the change in airway-occlusion pressure 0.1 s after the start of inspiration and end-tidal PCO2 in 13 subjects with late-onset Pompe disease and 51 healthy controls. RESULTS: Overall inspiratory muscle strength was within normal limits or slightly diminished in the late-onset Pompe disease group. Five subjects (38.5%) were chronically hypercapnic, and 9 (69.2%) had an increased breath-holding time. Compared with controls, the change in airway-occlusion pressure 0.1 s/change in end-tidal CO2 pressure slope (hypercapnic respiratory drive) was lower in the late-onset Pompe disease group (median 0.050 [interquartile range 0.027-0.118] vs 0.183 [0.153-0.233], P < .001). Nine subjects (69.2%) had a blunted change in airway-occlusion pressure 0.1 s/change in end-tidal carbon dioxide pressure slope. CONCLUSIONS: Subjects with late-onset Pompe disease had an impaired hypercapnic respiratory drive response. The clinical impact of this phenomenon in this subject subset deserves further investigation.

Recomendaciones de vacunación en adultos con enfermedades respiratorias: Documento de la Asociación Argentina de Medicina Respiratoria para los neumonólogos / Recommendations on Vaccination for Adults with Respiratory Diseases. Document of the Argentina Association of Respiratory Medicine for Pulmonologists

El neumonólogo de adultos acostumbra a prescribir vacunas. Este documento hecho por expertos en aspectos de la especialidad que involucran vacunar a pacientes con enfermedades respiratorias, perteneciente a la Asociación Argentina de Medicina Respiratoria, resumió la información disponible proponiendo una participación activa en la vacunación contra influenza (VAG), neumococo (VAN), pertusis y zoster. El Ministerio de Salud (MSAL) en Argentina, como el CDC y su comité de consulta sobre inmunización (ACIP) en Estados Unidos, elaboran calendarios y recomendaciones para vacunación. La ACIP recomienda la VAG a mayores de 6 meses sin contraindicaciones; el MSAL a mayores de 65 años y a quienes tengan comorbilidades (incluye enfermedades respiratorias y tabaquismo) o contacto con personas vulnerables. La clásica VAN polisacárida de 23 serotipos es recomendada para adultos con riesgo de enfermedad invasiva, incluyendo a mayores de 65 años, revacunando a los inmunosuprimidos y una única vez a los mayores de 65 que hubieran sido vacunados 5 años antes o más; la ACIP recomienda dar la VAN conjugada de 13 serotipos, más inmunogénica, secuencialmente con la polisacárida de 23, en adultos con factores de riesgo y en mayores de 65 años. Sugerimos usarla en menores de 65 con comorbilidad respiratoria. El neumonólogo debe recordar al menos 2 vacunas más: dar el refuerzo decenal contra difteria y tétanos (DT) en mayores de 18, una vez con vacuna triple acelular (difteria, pertusis y tétanos) protegiendo contra pertusis y reduciendo su transmisión. El herpes zoster produce un rash cutáneo vesicular doloroso. Uno cada 2 mayores de 85 sufrirán al menos un ataque de herpes zoster. La vacuna reduce más del 50% la incidencia y más del 60% la neuralgia post herpética; el ACIP la recomienda en mayores de 60 años. Un gran número de los pacientes con afecciones pulmonares crónicas tienen esa edad.

The pulmonologist uses to prescribe vaccines to adult patients. Experts of the Argentina Association of Respiratory Medicine who are specialists in areas involving vaccination of patients with respiratory diseases prepared this document which summarizes the available information and proposes an active prescription of the infuenza, pneumococcus, pertussis and herpes zoster vaccinations. The Ministry of Health in Argentina as the CDC and its Advisory Committee on Immunization Practices (ACIP) in the USA, made recommendations on vaccination indications and schedules. The ACIP recommends influenza vaccination to persons older than 6 months of age without any contraindication. The Ministry of Health recommends this vaccination to persons over 65 years of age, to those with morbidities (including respiratory diseases and smoking habit) and to persons in contact with high risk people. The classic 23-valent polysaccharide pneumococcal vaccine is recommended for adults at risk of invasive disease, including persons over 65 years of age. Revaccination is recommended to immunosuppressed patients and persons over 65 years of age at 5-year intervals. The ACIP recommends vaccination with the 13-valent serotypes polysaccharide pneumococcal vaccine, which is more immunogenic, sequentially with the 23-valent vaccine in adults with risk factors and over 65 years of age. We suggest this practice in patients under 65 years of age with respiratory morbidities. The pulmonologist must remember at least two other vaccines: a booster vaccination every 10 years of diphtheria and tetanus vaccine to persons over 18 years of age, and once the triple acellular vaccine (diphtheria, pertussis and tetanus) to protect against pertussis and reduce transmission. Herpes zoster (shingles) causes a painful vesicular rash; 50% of persons over 85 years suffer at least one bout of herpes zoster. The vaccine reduces more than 50% incidence and more than 60% postherpetic neuralgia. This vaccine is recommended by ACIP for persons over 60 years. In this age group there are many patients with chronic lung conditions.

[Thymic neuroendocrine carcinoma with Pompe's disease of the adult]. / Carcinoma neuroendocrino de timo con enfermedad de Pompe del adulto.

Pompe disease (glycogenosis type II) is an inherited autosomal recessive lysosomal storage disease caused by a deficiency of acid alpha-glucosidase. Thymic neuroendocrine tumors, are primary thymic neoplasms with neuroendocrine differentiation that generally present as a mass within the anterior mediastinum. Both diseases are considered rare. To our knowledge the co-existence of Pompe disease and thymic neuroendocrine tumor in the same patient has not been previously reported. We could not find biological plausibility between both diseases. Further studies are needed to confirm the finding and to further increase our understanding of this association. Clinical data from epidemiological studies, case reports, case series and small formal open or controlled clinical trials may define both clinical plausibility and causality between the two conditions.

Carcinoma neuroendocrino de timo con enfermedad de pompe del adulto / Thymic neuroendocrine carcinoma with Pompe's disease of the adult

La enfermedad de Pompe (glucogenosis tipo II) es una enfermedad de depósito lisosomal, autosómica recesiva causada por una deficiencia de ácido alfa-glucosidasa. Los tumores neuroendocrinos tímicos son neoplasias primarias con diferenciación neuroendocrina que generalmente se presentan como una masa en el mediastino anterior. Ambas enfermedades se consideran raras en sí mismas. En nuestro conocimiento, la enfermedad de Pompe y un tumor neuroendocrino del timo en el mismo paciente no ha sido antes comunicada. No pudimos encontrar la plausibilidad biológica entre ambas enfermedades. Se necesitan más estudios para confirmar el hallazgo y para aumentar aún más nuestra comprensión de esta asociación. Los datos clínicos de los estudios epidemiológicos, los informes de casos, las series de casos y los pequeños ensayos clínicos abiertos o controlados pueden definir tanto la plausibilidad clínica como la causalidad entre las dos enfermedades.

Pompe disease (glycogenosis type II) is an inherited autosomal recessive lysosomal storage disease caused by a deficiency of acid alpha-glucosidase. Thymic neuroendocrine tumors, are primary thymic neoplasms with neuroendocrine differentiation that generally present as a mass within the anterior mediastinum. Both diseases are considered rare. To our knowledge the co-existence of Pompe disease and thymic neuroendocrine tumor in the same patient has not been previously reported. We could not find biological plausibility between both diseases. Further studies are needed to confirm the finding and to further increase our understanding of this association. Clinical data from epidemiological studies, case reports, case series and small formal open or controlled clinical trials may define both clinical plausibility and causality between the two conditions.

[Intrathoracic schwannoma]. / Schwannoma intratorácico.

The schwannoma (neurilemmoma) is a slow-growing benign tumor originating from Schwann sheath whose location in the chest cavity is exceptional. It is generally asymptomatic and is discovered incidentally but can cause symptoms when the lesion grows or invade underlying structures. Its importance lies in the possibility of confusion with malignant tumors. We present a patient who complains of chest pain caused by a prolonged course schwannoma. The tomographic image is suggestive of extrapulmonary tumor, so the schwannoma in this location should be considered in the differential diagnosis of metastatic or primary pleural tumors such as lipoma, solitary fibrous tumor and mesothelioma.

Schwannoma intratorácico / Intrathoracic schwannoma

El schwannoma (neurilemoma) es un tumor benigno de lento crecimiento originado en la vaina de Schwann, cuya localización en la cavidad torácica es excepcional. En general es asintomático y se descubre en forma incidental, aunque puede ocasionar síntomas cuando la lesión crece o invade estructuras subyacentes. Su importancia radica en la posibilidad de confusión con tumores malignos. Presentamos una paciente con dolor torácico de evolución prolongada causado por un schwannoma. La imagen tomográfica es sugestiva de tumor extrapulmonar, por ello el schwannoma en esta localización debe ser considerado en el diagnóstico diferencial de tumores pleurales metastásicos o primarios como el lipoma, el tumor fibroso solitario y el mesotelioma.

The schwannoma (neurilemmoma) is a slow-growing benign tumor originating from Schwann sheath whose location in the chest cavity is exceptional. It is generally asymptomatic and is discovered incidentally but can cause symptoms when the lesion grows or invade underlying structures. Its importance lies in the possibility of confusion with malignant tumors. We present a patient who complains of chest pain caused by a prolonged course schwannoma. The tomographic image is suggestive of extrapulmonary tumor, so the schwannoma in this location should be considered in the differential diagnosis of metastatic or primary pleural tumors such as lipoma, solitary fibrous tumor and mesothelioma.

Schwannoma intratorácico / Intrathoracic schwannoma

El schwannoma (neurilemoma) es un tumor benigno de lento crecimiento originado en la vaina de Schwann, cuya localización en la cavidad torácica es excepcional. En general es asintomático y se descubre en forma incidental, aunque puede ocasionar síntomas cuando la lesión crece o invade estructuras subyacentes. Su importancia radica en la posibilidad de confusión con tumores malignos. Presentamos una paciente con dolor torácico de evolución prolongada causado por un schwannoma. La imagen tomográfica es sugestiva de tumor extrapulmonar, por ello el schwannoma en esta localización debe ser considerado en el diagnóstico diferencial de tumores pleurales metastásicos o primarios como el lipoma, el tumor fibroso solitario y el mesotelioma.(AU)

The schwannoma (neurilemmoma) is a slow-growing benign tumor originating from Schwann sheath whose location in the chest cavity is exceptional. It is generally asymptomatic and is discovered incidentally but can cause symptoms when the lesion grows or invade underlying structures. Its importance lies in the possibility of confusion with malignant tumors. We present a patient who complains of chest pain caused by a prolonged course schwannoma. The tomographic image is suggestive of extrapulmonary tumor, so the schwannoma in this location should be considered in the differential diagnosis of metastatic or primary pleural tumors such as lipoma, solitary fibrous tumor and mesothelioma.(AU)

Schwannoma intratorácico. / [Intrathoracic schwannoma].

The schwannoma (neurilemmoma) is a slow-growing benign tumor originating from Schwann sheath whose location in the chest cavity is exceptional. It is generally asymptomatic and is discovered incidentally but can cause symptoms when the lesion grows or invade underlying structures. Its importance lies in the possibility of confusion with malignant tumors. We present a patient who complains of chest pain caused by a prolonged course schwannoma. The tomographic image is suggestive of extrapulmonary tumor, so the schwannoma in this location should be considered in the differential diagnosis of metastatic or primary pleural tumors such as lipoma, solitary fibrous tumor and mesothelioma.

[Analysis of factors associated with chronic hypercapnia in patients with myotonic dystrophy]. / Análisis de factores relacionados con hipercapnia crónica en la distrofia miotónica.

Myotonic dystrophy (DM) is the most common dystrophy in adults. Several factors may explain the chronic CO2 retention. The selection of patients, different clinical stages and evaluation forms may explain the differing results obtained. Our objectives were to characterize respiratory function and to evaluate factors associated with chronic retention of CO2 in DM. We included 27 consecutive ambulatory and stable patients who were allocated into normocapnic and hypercapnic groups (PaCO2 ≥ 43 mmHg). Forced vital capacity (FVC), maximum static pressure, voluntary apnea time, Epworth scale and arterial blood gases were measured. The CO2 chemosensitivity was assessed using CO2 rebreathing (Read method). The slope ΔP0.1/ΔPCO2 expressed the CO2 chemosensitivity. A 59.3% (16/27) presented hypercapnia. FVC and respiratory muscle strength were normal or showed mild to moderate decrease. No significant differences in these variables were found in both groups. Inadequate response to CO2 (slope ΔP0.1/ΔPCO2 low (< 0.1 cm H2O/mmHg) or flat) was associated with hypercapnia (p < 0.005). Chronic retention of CO2 represented 11.56 times higher risk of inadequate response to CO2. The group with low-flat slope ΔP0.1/ΔPCO2 showed higher PaCO2 (p = 0.0017) and more prolonged voluntary apnea time (p = 0.002). We conclude that in our patients with DM, chronic CO2 retention was associated with the presence of abnormalities of the central control of breathing. Our results allow explaining previous reports describing the striking frequency of postoperative respiratory failure and difficulties in the process of weaning from mechanical ventilation.