Linfangioleiomiomatosis. Similitudes y diferencias entre sus dos formas clínicas / Lymphangioleiomyomatosis. Similarities and differences between its two clinical forms

La linfangioleiomiomatosis (LAM) es una enfermedad pulmonar que se caracteriza por la proliferación de células musculares lisas atípicas en el pulmón y por la destrucción del tejido en forma de quistes. Existen dos formas clínicas de LAM, la esporádica (LAM-S) y la asociada a CET (LAM-CET), esta última es cinco veces más frecuente que la LAM-S, puede tener mutación tanto en el gen TSC1 o en el TSC2, mientras que la LAM-S solo en TSC2. La LAM-S es más sintomática, agresiva, presenta mayor ocupación quística, con peores valores en FEV1 y DLCO, menor número de NP (HMNM) y menor número de AML renales que la LAM-CET. No se han descrito diferencias en cuanto a la histología, fisiopatología, tipo de tratamiento o pronóstico

Lymphangioleiomyomatosis (LAM) is a lung disease characterized by the proliferation of atypical smooth muscle cells in the lung and the destruction of tissue in the form of cysts. There are two clinical forms of LAM, sporadic (LAM-S) and associated with TSC (LAM-CET), the latter is five times more frequent than LAM-S, it may have a mutation in both the TSC1 or TSC2 gene, while LAM-S only in TSC2 while LAM-S is more symptomatic and aggressive with greater cystic occupation, worse FEV1 and DLCO values, fewer pulmonary nodules (PN) and fewer renal AML than form LAM-CET. No differences have been described in terms of histology, pathophysiology, type of treatment or prognosis

Qué puede pasar tras el trasplante pulmonar y la importancia del tiempo transcurrido. Revisión radiológica de las complicaciones postrasplante / What can happen after lung transplantation and the importance of the time since transplantation: radiological review of post-transplantation complications

El trasplante pulmonar es la mejor opción de tratamiento para los estadios finales de patologías como la fibrosis quística, la hipertensión pulmonar, la enfermedad pulmonar obstructiva crónica o la fibrosis pulmonar idiopática. Una mejor técnica quirúrgica y el avance en las terapias inmunosupresoras aumentan la supervivencia de los receptores y hacen necesario un seguimiento más duradero, dado que las complicaciones pueden producirse en cualquier momento tras el trasplante. Las complicaciones se dividen de forma práctica en agudas (dos primeros meses postrasplante), tardías (pasados 2 meses) y aquellas que pueden producirse en cualquier momento. Muchas de ellas presentan manifestaciones clínico-radiológicas inespecíficas, por lo que el criterio temporal es clave para acotar el diagnóstico diferencial. Las pruebas de imagen permiten guiar procedimientos intervencionistas y detectar complicaciones precozmente. El objetivo del artículo es presentar las posibles complicaciones postrasplante desde un punto de vista clínico-radiológico con el fin de ser capaces de detectarlas lo antes posible (AU)

Lung transplantation is the best treatment option in the final stages of diseases such as cystic fibrosis, pulmonary hypertension, chronic obstructive pulmonary disease, or idiopathic pulmonary fibrosis. Better surgical techniques and advances in immunosuppressor treatments have increased survival in lung transplant recipients, making longer follow-up necessary because complications can occur at any time after transplantation. For practical purposes, complications can be classified as early (those that normally occur within two months after transplantation), late (those that normally occur more than two months after transplantation), or time-independent (those that can occur at any time after transplantation). Many complications have nonspecific clinical and radiological manifestations, so the time factor is key to narrow the differential diagnosis. Imaging can guide interventional procedures and can detect complications early. This article aims to describe and illustrate the complications that can occur after lung transplantation from the clinical and radiological viewpoints so that they can be detected as early as possible (AU)

Remodelado cardíaco inverso en pacientes con hipertensión pulmonar tras trasplante bipulmonar. Estudio con tomografía computarizada multidetector / Multidetector computed tomography shows reverse cardiac remodeling after double lung transplantation for pulmonary hypertension

Objetivo. Valorar mediante tomografía computarizada multidetector (TCMD) los cambios estructurales del corazón derecho y de las arterias pulmonares que se producen en los pacientes con hipertensión pulmonar (HP) grave tratados mediante trasplante bipulmonar (TxBP). Material y métodos. Estudio retrospectivo de 21 pacientes consecutivos diagnosticados de HP grave, a los que se realizó TxBP en nuestro centro hospitalario durante los años 2010-2014. Se analizó la TCMD realizada previa al trasplante pulmonar, y la primera disponible después. Se obtuvieron las siguientes variables: diámetro del tronco de la arteria pulmonar, relación diámetro tronco de la arteria pulmonar/diámetro de la aorta ascendente, diámetro del ventrículo derecho, relación diámetro ventrículo izquierdo/derecho e índice de excentricidad. Se realizó un análisis estadístico con comparación de medias de las diferentes variables recogidas. Resultados. En todos los casos analizados se observó, en la TCMD realizada, una media de 24±14 días post-TxBP, una reducción significativa del tamaño de las cavidades derechas, con mejoría de los índices de interdependencia ventricular y del tamaño del tronco de la arteria pulmonar (p<0,001 para todas las variables analizadas). Conclusión. Los pacientes con HP tratados mediante TxBP presentan un remodelado inverso precoz de los cambios estructurales cardíacos derechos y del árbol arterial pulmonar. La TCMD es útil para detectar dichos cambios (AU)

Objective. To use multidetector computed tomography (MDCT) to evaluate the structural changes in the right heart and pulmonary arteries that occur in patients with severe pulmonary hypertension treated by double lung transplantation. Material and methods. This was a retrospective study of 21 consecutive patients diagnosed with severe pulmonary hypertension who underwent double lung transplantation at our center between 2010 and 2014. We analyzed the last MDCT study done before lung transplantation and the first MDCT study done after lung transplantation. We recorded the following variables: diameter of the pulmonary artery trunk, ratio of the diameter of the pulmonary artery trunk to the diameter of the ascending aorta, diameter of the right ventricle, ratio of the diameter of the left ventricle to the diameter of the right ventricle, and eccentricity index. Statistical analysis consisted of the comparison of the means of the variables recorded. Results. In all cases analyzed, the MDCT study done a mean of 24±14 days after double lung transplantation showed a significant reduction in the size of the right heart chambers, with improved indices of ventricular interdependency index, and reduction in the size of the pulmonary artery trunk (p<0.001 for all the variables analyzed). Conclusion. Patients with pulmonary hypertension treated by double lung transplantation present early reverse remodeling of the changes in the structures of the right heart and pulmonary arterial tree. MDCT is useful for detecting these changes (AU)

Multidetector computed tomography shows reverse cardiac remodeling after double lung transplantation for pulmonary hypertension. / Remodelado cardíaco inverso en pacientes con hipertensión pulmonar tras trasplante bipulmonar. Estudio con tomografía computarizada multidetector.

OBJECTIVE: To use multidetector computed tomography (MDCT) to evaluate the structural changes in the right heart and pulmonary arteries that occur in patients with severe pulmonary hypertension treated by double lung transplantation. MATERIAL AND METHODS: This was a retrospective study of 21 consecutive patients diagnosed with severe pulmonary hypertension who underwent double lung transplantation at our center between 2010 and 2014. We analyzed the last MDCT study done before lung transplantation and the first MDCT study done after lung transplantation. We recorded the following variables: diameter of the pulmonary artery trunk, ratio of the diameter of the pulmonary artery trunk to the diameter of the ascending aorta, diameter of the right ventricle, ratio of the diameter of the left ventricle to the diameter of the right ventricle, and eccentricity index. Statistical analysis consisted of the comparison of the means of the variables recorded. RESULTS: In all cases analyzed, the MDCT study done a mean of 24±14 days after double lung transplantation showed a significant reduction in the size of the right heart chambers, with improved indices of ventricular interdependency index, and reduction in the size of the pulmonary artery trunk (p<0.001 for all the variables analyzed). CONCLUSION: Patients with pulmonary hypertension treated by double lung transplantation present early reverse remodeling of the changes in the structures of the right heart and pulmonary arterial tree. MDCT is useful for detecting these changes.

What can happen after lung transplantation and the importance of the time since transplantation: radiological review of post-transplantation complications. / Qué puede pasar tras el trasplante pulmonar y la importancia del tiempo transcurrido. Revisión radiológica de las complicaciones postrasplante.

Lung transplantation is the best treatment option in the final stages of diseases such as cystic fibrosis, pulmonary hypertension, chronic obstructive pulmonary disease, or idiopathic pulmonary fibrosis. Better surgical techniques and advances in immunosuppressor treatments have increased survival in lung transplant recipients, making longer follow-up necessary because complications can occur at any time after transplantation. For practical purposes, complications can be classified as early (those that normally occur within two months after transplantation), late (those that normally occur more than two months after transplantation), or time-independent (those that can occur at any time after transplantation). Many complications have nonspecific clinical and radiological manifestations, so the time factor is key to narrow the differential diagnosis. Imaging can guide interventional procedures and can detect complications early. This article aims to describe and illustrate the complications that can occur after lung transplantation from the clinical and radiological viewpoints so that they can be detected as early as possible.

Las diferentes manifestaciones de la aspergilosis pulmonar. Hallazgos en tomografía computarizada multidetector / The different manifestations of pulmonary aspergillosis: Multidetector computed tomography findings

La aspergilosis pulmonar es una infección micótica causada la mayoría de las veces por la inhalación de esporas de Aspergillus fumigatus. Sin embargo, al hablar de aspergilosis, en realidad nos referimos a un abanico de manifestaciones clínicas y radiológicas que dependen directamente del estado del sistema inmune del paciente, de la existencia de enfermedad pulmonar previa y de la virulencia del organismo. Son 4 los tipos de aspergilosis pulmonar (aspergiloma, aspergilosis broncopulmonar alérgica, aspergilosis necrosante crónica y aspergilosis invasiva), cada uno con hallazgos radiológicos distintos. En este artículo revisamos el espectro de signos de la aspergilosis pulmonar en la tomografía computarizada multidetector y los correlacionamos con los síntomas y la respuesta inmune del paciente. Se abordarán asimismo los principales diagnósticos diferenciales (AU)

Pulmonary aspergillosis is a fungal infection usually caused by inhaling Aspergillus fumigatus spores. However, when we talk about aspergillosis, we normally refer to the spectrum of clinical and radiological findings that depend directly on the patient's immune status, on the prior existence of lung disease, and on the virulence of the infective organism. There are four types of pulmonary aspergillosis (aspergilloma, allergic bronchopulmonary aspergillosis, chronic necrotizing pulmonary aspergillosis, and invasive aspergillosis), and each type has its own distinct radiologic findings. We review the signs of pulmonary aspergillosis on multidetector computed tomography and we correlate them with patients’ symptoms and immune responses. Likewise, we discuss the differential diagnoses (AU)

[The different manifestations of pulmonary aspergillosis: multidetector computed tomography findings]. / Las diferentes manifestaciones de la aspergilosis pulmonar. Hallazgos en tomografía computarizada multidetector.

Pulmonary aspergillosis is a fungal infection usually caused by inhaling Aspergillus fumigatus spores. However, when we talk about aspergillosis, we normally refer to the spectrum of clinical and radiological findings that depend directly on the patient's immune status, on the prior existence of lung disease, and on the virulence of the infective organism. There are four types of pulmonary aspergillosis (aspergilloma, allergic bronchopulmonary aspergillosis, chronic necrotizing pulmonary aspergillosis, and invasive aspergillosis), and each type has its own distinct radiologic findings. We review the signs of pulmonary aspergillosis on multidetector computed tomography and we correlate them with patients' symptoms and immune responses. Likewise, we discuss the differential diagnoses.

Varón joven con aumento del tamaño hiliar derecho y hemoptisis / Young male with increase of the right hilar size and hemoptysis

No disponible

Sonographically guided percutaneous biopsy of gastrointestinal tract lesions.

OBJECTIVE: Our objective was to evaluate the role and safety of sonographically guided percutaneous biopsy in the diagnosis of digestive tract lesions when the lesions are not suitable to biopsy by endoscopy and safely reachable by sonography. MATERIALS AND METHODS: We performed 42 biopsies in 41 patients (age range, 14-81 years; mean age, 57.5 years). We performed biopsies with real-time sonographic guidance using graded compression, with a 3.5-5-MHz microconvex transducer. In 39 biopsies, core specimens were obtained with an 18-gauge automatic needle gun; fine-needle aspiration biopsy was obtained in 28 patients with a 22-gauge needle and in the other four patients with a 21-gauge needle. In the remaining three patients, a coaxial technique with 20- and 22-gauge needles for cytology was used. RESULTS: In 40 (95.2%) of 42 core biopsies performed, a specific diagnosis was obtained. A positive diagnosis was obtained in 16 (45.7%) of 35 fine-needle aspirations. The lesions were located from the pharynx to the sigmoid colon. Twenty-eight patients had malignant lesions, and 13 had benign lesions. Only one serious complication, bile peritonitis, was observed. CONCLUSION: Percutaneous biopsy with sonographic guidance can be used safely and efficiently to diagnose digestive tract lesions that can be visualized on sonography and are not accessible endoscopically.

Evaluation of CT findings for diagnosis of pleural effusions.

Computed tomography studies are usually used to assess patients with pleural effusions, and radiologists should be aware of the significance of different CT findings for the diagnosis of the effusion. The purpose of this study was to evaluate CT findings for etiological diagnosis of pleural effusions. Contrast-enhanced CT of the chest of 211 patients with pleural effusion of definite diagnosis were evaluated. The CT images were evaluated for the presence and extent of pleural effusion, thickening or nodules, extrapleural fat and other changes in the mediastinum or lung. The CT scans were read by two independent observers and correlation between them was evaluated. Comparison of CT findings between benign and malignant effusions, between exudates and transudates, and between empyemas and the other parapneumonic effusions were carried out. Kappa values for most CT findings were > 0.85. Loculation, pleural thickening, pleural nodules, and extrapleural fat of increased density were only present in exudative effusions. Multiple pleural nodules and nodular pleural thickening were the only pleural findings limited to malignant pleural effusions. The signs were also more frequently seen in empyemas than in other parapneumonic effusions. Computed tomography findings can help to distinguish between transudates and exudates. Although there is some overlap between benign and malignant pleural effusions, pleural nodules and nodular pleural thickening were present almost exclusively in the latter. Although differences between CT findings of empyemas and the other parapneumonic effusions exist, there is no finding which can definitely differentiate between them.

Langerhans' cell histiocytosis of the temporal bone in pediatric patients: imaging and follow-up.

OBJECTIVE: The purpose of this study was to review the initial clinical and radiologic manifestations and the follow-up of pediatric patients with Langerhans' cell histiocytosis affecting the temporal bone. MATERIALS AND METHODS: We retrospectively studied 14 patients with Langerhans' cell histiocytosis affecting the temporal bone. All patients were examined initially and sequentially with CT. In six patients, MR imaging was also done. RESULTS: Temporal bone involvement was the initial form of presentation in 12 patients. In eight patients, temporal bone involvement presented as an isolated manifestation, and in four it was associated with multisystemic involvement. In the remaining two patients, temporal bone involvement appeared during the course of the Langerhans' cell histiocytosis. Bilateral involvement was seen in four patients. In two patients, the temporal bone was affected only at the petrous apex. CT showed destruction of bone in all 14 patients and an associated soft-tissue homogeneous mass after injection of i.v. contrast material in 12 patients. CT showed a heterogeneous appearance of the soft-tissue mass in two patients. The average period of follow-up was 5 years. In seven of the 14 patients, the disease had a satisfactory evolution in which the bony lesions of the temporal bone reossified and remodeled over the course of a year. CONCLUSION: In Langerhans' cell histiocytosis, involvement of the temporal bone is usually seen on radiographs as extensive lytic lesions associated with soft-tissue masses. The lesions that remit show early disappearance of the soft-tissue mass, followed by reossification and remodeling of the involved bone. Patients with limited initial involvement of the temporal bone have a better prognosis on long-term follow-up than do patients with the multisystemic form.