Publique en esta revista
Información de la revista
Vol. 53. Núm. 8.Agosto 2017
Páginas 413-470
Compartir
Compartir
Descargar PDF
Más opciones de artículo
Vol. 53. Núm. 8.Agosto 2017
Páginas 413-470
Clinical image
DOI: 10.1016/j.arbres.2017.01.015
Vanishing Lung Syndrome in a Cystic Fibrosis Patient
Síndrome del pulmón evanescente en un paciente con fibrosis quística
Visitas
642
Libor Filaa,
Autor para correspondencia
libor.fila@fnmotol.cz

Corresponding author.
, Vojtech Suchanekb, Miloslav Marela
a Department of Pneumology, 2nd Faculty of Medicine, Charles University in Prague, and University Hospital Motol, Prague, Czech Republic
b Department of Radiology, 2nd Faculty of Medicine, Charles University in Prague, and University Hospital Motol, Prague, Czech Republic
Este artículo ha recibido
642
Visitas
Información del artículo
Texto Completo
Bibliografía
Descargar PDF
Estadísticas
Texto Completo

Vanishing lung syndrome is a rare condition characterized by unilateral or bilateral asymmetric upper lobe involvement with the formation of multiple large bullae replacing normal pulmonary architecture and compressing surrounding parenchyma.1

In this paper, we report the case of a male cystic fibrosis (CF) patient with severe sinopulmonary disease, malnutrition, diabetes mellitus and asthenozoospermia. He was diagnosed with CF at 3 years of age due to malnutrition and recurrent respiratory infections. His sweat chloride concentration was 101mmol/L and CFTR gene mutations were F508del and 2935del11. At the age of 30, he was evaluated for a lung transplant and chest imaging studies were performed, see Fig. 1. Previous chest X-rays had showed slowly progressing changes in the upper areas of both lung fields over the past 3 years.

Fig. 1.
(0,27MB).

Chest X-ray and chest CT. (a) Posteroanterior chest X-ray shows hyperlucent upper parts of both lung fields, approx. upper half on the right side and upper third on the left side. Residual lung parenchyma has increased bronchovascular markings; (b) Chest CT (sagittal reconstruction) at the level of left hilum shows vanishing lung in the upper third of left lung; (c) Chest CT (coronal reconstruction) at the level of main bronchi shows bronchiectasis in the left upper lobe ending in severe emphysematous changes of lung parenchyma, vanishing lung in both upper zones, large bulla in the right lower lobe and consolidation in basolateral part of the right upper lobe.

In patients with CF, the typical CT picture is bronchiectasis, bronchial wall thickening, mucus plugging, atelectasis, consolidation and air trapping.2 Emphysematous and bullous changes are not consistent with CF lung disease. Our patient was a never-smoker and had normal levels of serum α1-antitrypsin, with no symptoms of other diseases that may be complicated by vanishing lung syndrome or giant bullae (e.g. sarcoidosis, granulomatosis with polyangiitis or Marfan and Ehlers–Danlos syndromes).

References
[1]
L. Roberts,C.E. Putman,J.T. Chen,L.R. Goodman,C.E. Ravin
Vanishing lung syndrome: upper lobe bullous pneumopathy
Rev Interam Radiol, 12 (1987), pp. 249-255
[2]
T.E. Robinson
Computed tomography scanning techniques for the evaluation of cystic fibrosis lung disease
Proc Am Thorac Soc, 4 (2007), pp. 310-315 http://dx.doi.org/10.1513/pats.200612-184HT
Copyright © 2017. SEPAR
Idiomas
Archivos de Bronconeumología

Suscríbase al Newsletter

Opciones de artículo
Herramientas
Política de cookies
Utilizamos cookies propias y de terceros para mejorar nuestros servicios y mostrarle publicidad relacionada con sus preferencias mediante el análisis de sus hábitos de navegación. Si continua navegando, consideramos que acepta su uso. Puede cambiar la configuración u obtener más información aquí.

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?