Imaging and differential diagnosis of cystic lung disease pdf

Cystic lung disease is a frequently encountered problem caused by a diverse group of diseases. We will discuss the differential diagnosis of cystic and solid peritoneal and mesenteric masses. Cystic lung diseases represent a broad set of disorders with different causes, but all of them are characterized by multiple thinwalled parenchymal lesions. Cystic fibrosis cf is an autosomal recessive, multiorgan disorder found predominantly among caucasians. Cystic lung disease carries a varied differential and for the bronchoscopist, procuring an adequate tissue sample is important, as this will preclude an oftentimecomplicated surgical biopsy. Although lymphangioleiomyomatosis and lch are the most frequently encountered causes of thinwalled. As the first diagnostic step, cysts should be distinguished from cavities, bullae, pneumatocele, emphysema, honeycombing, and cystic bronchiectasis. The four most common cystic lung diseases are lymphangioleiomyomatosis lam, pulmonary langerhans cell histiocytosis plch, birthoggdube syndrome bhd, and lymphoid interstitial pneumonia lip. Multiple cystic structures can be seen within the lung, with contralateral mediastinal displacement. Differential diagnosis of granulomatous lung disease. The term cystic lung disease includes a group of diverse pulmonary disorders, characterised by the presence of parenchymal cysts on respiratory imaging. The differential diagnosis for lung cysts is broad and encompasses multisystem diseases in addition to cystic diseases isolated to the chest table 1. Diagnosis of cystic lung diseases were confirmed by imaging findings and if necessary with histopathological analysis.

Noninfectious diseases associated with lung cavities malignancies one of the most important distinctions in the differential diagnosis of cavitary lung lesions is the distinction between malignant and nonmalignant etiologies. Abbott, md key facts terminology cystic fibrosis cf. The differential diagnosis of diffuse cystic lung disease is more extensive than. In d, axial reconstruction of a ct scan of a female patient with lymphangioleiomyomatosis, showing diffuse lung cysts and a left chylothorax. The differential diagnoses of diffuse cystic lung diseases are myriad, including neoplastic, inflammatory, and infectious etiologies. The differential diagnoses of granulomatous lung disease are listed in table 1. If so, the blood is not fully oxygenated, causing the baby to have a dusky hue appear cyanotic. The differential diagnosis of focal and multifocal lung diseases includes. Since it is thus established that the manifestations of cystic disease of the lung may be either congenital or acquired, it at once becomes evident that the condition is produced as a result of certain mechanical forces which operate in response to certain structural changes in the lung, regardless of whether these changes occur from. Cystic lung diseases wide spectrum of disease considerable progress in the last decade many with neoplastic characteristics prognosis and course highly variable diagnosis often made based on imaging characteristics and ancillary findings november 21, 2017 68. Nov 12, 2010 a lung cyst is defined as a round parenchymal lucency or area of low attenuation with a thin wall.

Other times, cystic and cavitary lung lesions can be a diagnostic challenge. A stepwise diagnostic approach to cystic lung diseases for. Mcgovern medical school references background info. A practical approach to cystic lung disease on hrct insights into. Mdct is the main diagnostic imaging for cystic lesions that provide good spatial. The radiographic and pathologic abnormalities in patients can be classified into acute, subacute, and chronic stages. These include langerhans cell histiocytosis, lymphangioleiomyomatosis and birthogg.

Wielputz and coauthors present the differential indications for various imaging techniques in the diagnosis of pulmonary and bronchial diseases. Cystic fibrosis is perhaps the most recognized cause of bronchiectasis and is associated with substantial morbidity and mortality. Pulmonary cysts occur in a variety of diseases, including infectious, inhalational, genetic, and neoplastic conditions. However, expanding the differential prior to intervention is of utmost importance. Diffuse cystic lung disease represents a diverse group of uncommon disorders with characteristic appearance on high resolution ct imaging. Interstitial lung disease in children made easierwell, almost. He had presented previously and was being evaluated for the possibility of malignancy. In this article, we also classify pulmonary cystic disease with an emphasis on. Pneumothorax is the most common acute presentation of cystic lung disease. Pathologic and radiologic correlation of adult cystic lung disease. Thus, correct diagnosis of cystic lung diseases is a challenge for radiologists. The rate of progression of lung disease in bhd is incompletely understood, but it seems clear that bhd cystic lung disease does not typically. Diseases or conditions presenting with lung cysts occupy a broad spectrum, and cystic lung diseases are a heterogeneous group of pathologies for which differential diagnosis can be complicated. The location of focal lesions may be of help in limiting the differential diagnosis, eg, propensity of.

Imaging atlas of interstitial lung diseases pulmonary. Cystic lung diseases associated with lymphoproliferative disorders. Differential diagnosis of cystic lung diseases differential diagnosis of cystic lung diseases rofo. Oct 01, 2016 pulmonary parenchymal cysts are commonly seen on ct scans, and their differential diagnosis may be challenging.

The differential diagnosis is limited and typically includes lymphangioleiomyomatosis lam, pulmonary langerhans cell histiocytosis plch and lymphoid interstitial pneumonia lip. Congenital pulmonary airway malformation radiology. A 60yearold man was referred to the interventional pulmonology clinic with a large rightsided intraparenchymal lung mass and a second, smaller lesion in the left lower lobe, accompanied by intermittent haemoptysis, fever, chills, productive cough of white phlegm as well as dizziness and weakness. The differential diagnosis of diffuse cystic lung disease is more extensive than previously described and may be organized on the basis of clinical history, serologic evaluation, and the hrct appearance of the cysts and ancillary hrct findings. Lung cysts pulmonary fibrosis honeycombing lymphangiomyomatosis langerhanscell histiocytosis lymphocytic interstitial pneumonia lip differential diagnosis 4. Causes of focal or multifocal cystic and cavitary lung disease cystic wall thickness s4 mm bullae blebs pneumatoceles. Our group and others have previously proposed algorithms for establishing a diagnosis of bhd 18, 19. Lam lam is an uncommon cystic lung disease caused by in. The differential diagnosis for diseases characterised by lung cysts is.

It classically presents in childhood with chronic productive cough, malabsorption causing steatorrhea, and failure to thrive. Cystic bronchiectasis may also be a differential diagnosis. Diseases that present with insidious dyspnea or spontaneous pneumothorax. Multidetector ct mdct, especially highresolution ct hrct, is the imaging modality of choice for the diagnosis and followup of these entities. We present a 75yearold female diagnosed with cf at the age of 57 years, which highlights the natural history and challenges in the diagnosis of atypical cf. Regardless of the cause of cysts, patients usually present with either no symptoms, with the cysts discovered on chest imaging for another reason, or with nonspecific symptoms such as cough and shortness of breath. Pulmonary parenchymal cysts are commonly seen on ct scans, and their differential diagnosis may be challenging.

Langerhans cell histiocytosis lch, lymphangioleiomyomatosis lam, lymphocytic interstitial pneumonia lip, and findings of pneumocystis jirovecii infection are all cystic lung diseases figures 22, 23, 24, 2526. Jan 01, 2014 diffuse cystic lung disease represents a diverse group of uncommon disorders with characteristic appearance on high resolution ct imaging. The differential diagnosis of pulmonary cystic diseases is broad 353637 38. While he term cyst implies a fluid filled t structure lined by epithelial cells, not all cystic paravertebral masses are true cysts.

Interstitial lung disease in children made easierwell, almost1 interstitial lung disease ild in pediatric patients is different from that in adults, with a vast array of pathologic conditions unique to childhood, varied modes of presentation, and a different range of radiologic appearances. Pdf diffuse cystic lung disease at highresolution ct. To explore the differential diagnosis of cystic lung disease. Bronchiectasis is a predominant imaging finding in cystic fibrosis, with important clinical implications. Active infectious processes and malignancies obviously need to be diag cystic and cavitary lung diseases table i. Diffuse cystic lung disease must be distinguished radiographically from emphysema, honeycombing, cystic bronchiectasis, pulmonary cavities, and pneumatoceles.

The diffuse cystic lung diseases dclds are a group of. These features are shown on freehand drawings and related to hrct images, in order to help radiologists pursue the correct differential diagnosis between. However in a subset of oncologic disease cystic pulmonary metastases, although rare, should always be considered. Although most commonly associated with lymphangioleiomyomatosis or langerhans cell histiocytosis, cystic lung disease is increasingly being recognized as a feature of other entities. Although cysts may be found radiologically in a wide variety of disease states, the entities discussed are those most likely to be encountered in biopsies where the underlying aetiology is unclear. Cystic lesions in multislice computed tomography of the chest.

Not always a truly diffuse cystic lung disease a case of. Cystic lung diseases present a considerable diagnostic challenge mainly because ct findings can be similar in many of these diseases. When combined with clinical and laboratory findings, hrct is often sufficient for the etiological definition of diffuse lung cysts, avoiding the need for lung biopsy. A and b, highresolution ct images show diffuse thinwalled cysts and.

Differential diagnosis includes bullous emphysema, solitary pulmonary nodules hamartoma, intralobar pulmonary sequestration, bronchogenic cyst and cystic lung tumours. Cystic lung disease cld is a group of lung disorders characterized by the presence of multiple cysts, defined as airfilled lucencies or lowattenuating areas, bordered by a thin wall usually. Cystic lung disease is a frequently encountered problem caused by a diverse group. Awareness of the spectrum of hrct findings associated with these diseases may help the trained observer narrow the differential diagnosis.

Cystic lung lesions by gamal rabie agmy, md, fccp professor of chest diseases,assiut university 2. Recognize imaging patterns, epidemiological and clinical factors of specific cystic lung. Primary lung cancer is a common disease, with 190,297 incident cases and 150,997 deaths reported in the united states in. The literature describes thinwalled cysts on ct as a characteristic feature of lip, occurring in as many as 80%. Initial evaluation with hrct can narrow the differential diagnosis and exclude other considerations, although routine surveillance and followup. Spontaneous pneumothorax can be a sentinel event leading t o the diagnosis of diffuse cystic lung disease 1, 2, 7. The diffuse cystic lung diseases have a broad differential diagnosis. Most of the disorders associated with multiple lung cysts hereafter called cystic lung diseases are rare or orphan lung conditions, defined as affecting fewer than one in 2000 people 1.

Recognizing these imaging patterns and ct scan signs are thus vitally important. Infectious post, traumatic, vascular, developmental, and benign and malignant. Cavities and emphysema are the most common alternative diagnoses that must be differentiated from true pulmonary cysts. Pdf a stepwise diagnostic approach to cystic lung diseases for. However, the combination of characteristic imaging findings, clinical features, and genetic testing, where appropriate, often permits an accurate diagnosis. The radiologic differential diagnosis of diffuse lung diseases characterized by multiple cysts or. In the present article, we describe a comprehensive list of the commonly encountered metaphoric chest ct scan signs and their clinical relevance.

Hereditary disorder that affects gene regulating chloride transport accounts for up to 25% of adult cases of bronchiectasis imaging findings diffuse bronchiectasis with predominant involvement of upper lobes right upper lobe often 1st and most severely affected airways primary site of pathology in. A practical approach to cystic lung disease on hrct. Hrct image showing thinwalled cysts in the right lung arrow in a. Usefulness of prenatal magnetic resonance imaging in. For radiologic assessment of cystic lung diseases, it is important to differentiate true lung cysts from other airfilled lung lesions in the first step. The clinical presentation is an important clue to the differential diagnosis of cystic lung diseases 12. Multiple cystic lung disease european respiratory society. Diffuse cystic lung disease at highresolution ct ajr. Differential diagnosis of cystic lung disease can be achieved by correlation between clinical and hrct findings multiple cystic lung disease represents a diverse group of uncommon disorders that can present a diagnostic challenge due to the increasing number of diseases associated with this presentation. A wide variety of pathophysiological processes spanning the spectrum from airway obstruction to lung remodeling can lead to multifocal cyst development in the lung. The combination of imaging appearance with clinical features and genetic testing where appropriate permits a confident and accurate diagnosis in the majority of the diseases without recourse for open lung biopsy. Cystic aravertebral masses of the thorax p share common imaging characteristics but have a broad differential diagnosis.

The clinical importance of cystic and cavitary lung dis eases is related to their underlying nature. Pulmonary langerhans cell histiocytosis, lymphangioleiomyomatosis, lymphocytic interstitial pneumonia, and follicular bronchiolitis are the most common diseases that produce this ct pattern. The differential diagnosis of intraparenchymal bronchogenic cysts must include acquired cystic lesions, such as a lung abscess, a hydatid cyst, infection with nocardia, an infected bulla, lobar emphysema, fungal diseases, and tuberculosis, especially when the lesions manifest as airfilled or have an airfluid level. The presence of pulmonary parenchymal cysts on computed tomography ct imaging presents a significant diagnostic challenge. The differential diagnosis possibilities associated with each pattern. Distinguishing true cystic lung disease from other entities, such as cavitary lung disease and emphysema, is important given the differing prognostic implications. Primary pulmonary cystic echinococcus in an immunocompetent. Lung cysts are commonly seen on computed tomography ct, and cystic lung diseases show a wide disease spectrum. Cystic lung diseases as listed in the table on the left. The differential diagnosis of intraparenchymal bronchogenic cysts must include acquired cystic lesions, such as a lung abscess, a hydatid cyst, infection with nocardia, an infected bulla, lobar emphysema, fungal diseases, and tuberculosis, especially when the lesions manifest as.

Diagnostics free fulltext cystic interstitial lung diseases. Cavities are defined as radiolucent areas with a wall thickness of more than 4mm and are seen in infection tb, staph, fungal, hydatid, septic emboli, squamous cell carcinoma and wegeners disease. They are not uncommon findings on highresolution hr thoracic computed tomography ct and when identified, they require explanation. Hp is an allergic lung disease caused by the inhalation of a variety of antigens farmers lung, bird fanciers lung, hot tub lung, humidifier lung. Imaging is thus particularly important in narrowing the differential diagnosis.

There is a more stable position of the abnormality on serial films, whereas this may vary in cdh. In addition, a number of pulmonary abnormalities can result in cystic patterns that mimic true lung cysts. Some of these are specific to a disease, whereas others help narrow the differential diagnosis. The differential diagnosis for diseases characterised by lung cysts is broad ranging from isolated chest disorders to rare multisystem diseases.