7. Hiatal Hernia
• Inhomogeneous cardiac density
• Retrocardiac density
• In mediastinum in PA view
– Air Fluid Level
• Other findings include:
– Pleural fibrosis on right
Sunday, January 06, 2013
10. Lipoid Pneumonia
• Alveolar
• Mass like densities
• Chronic
• Lower lung fields
• Bilateral in this case
• Air space density projecting over RML and
lingula
Sunday, January 06, 2013
15. • Round lesions projected over lung fields are in
chest wall
• Lesions in chest wall along both sides and on
abdominal wall
• Posterior mediastinal mass: Para vertebral line
on right side
Black arrow points to posterior
mediastinal mass distorting
paravertebral line.
White arrows point to
neurofibromas in chest wall Sunday, January 06, 2013
18. chest clinical cases
Uncontrolled asthma,
recurrent rhinosinusitis, and
infertility in a young woman
Submitted by
P. Scott Meehan MD
Fellow
Division of Pulmonary, Allergy, Critical Care and Sleep Medicine
The Ohio State University Medical Center
Columbus, Ohio
Jennifer W. McCallister MD
Assistant Professor of Internal Medicine
Division of Pulmonary, Allergy, Critical Care and Sleep Medicine
The Ohio State University Medical Center
Columbus, Ohio
19. History
• A 27 year old Caucasian woman with a history of uncontrolled asthma, allergic rhinitis,
gastroesophageal reflux disease and recurrent pneumonias was referred for evaluation in the
pulmonary clinic.
• She was diagnosed with asthma at age twelve.
• In the last six years she had been treated as an outpatient for pneumonia, sinusitis, and
bronchitis several times per year and had required systemic corticosteroids several times per year
for presumed asthma exacerbations.
• Since last year she described persistent dyspnea with exertion, daily cough, and thick, yellow
sputum production which had worsened.
• This had not improved despite multiple courses of antibiotics, systemic glucocorticoids, and
escalation of her inhaled corticosteroids.
• She also reported wheezing and recurrent episodes of left sided chest pain.
• There was no nighttime cough or limitation in daily activities.
• She had not had frequent ear infections, yeast infections, epistaxis or hearing loss.
• Her medications included a combination high dose inhaled corticosteroid and long acting beta
agonist, montelukast, a proton pump inhibitor, albuterol, a multi-vitamin and methylphenidate.
• She had been using short acting beta agonists multiple times per day without relief.
• She had a 5 pack-year smoking history and quit eight years prior.
• She was married and had been trying to conceive for about 15 months without success.
• She had no known occupational exposures and owned one dog.
• There was no known family history of asthma or other respiratory diseases.
Sunday, January 06, 2013
20. Physical Exam
• On physical exam, she was a well developed, obese (Body
Mass Index 32 kg/m2) woman in no acute distress.
• Her resting oxygen saturation (SpO2) was 97% on room air.
• Head and neck exam were normal.
• Heart sounds were normal without murmur, rub, or gallop
with sounds heard best at right sternal border.
• Auscultation of the lungs revealed inspiratory squeaks over
the left upper lung field, otherwise clear.
• Percussion and diaphragmatic excursion were normal.
• The abdomen was soft and non-tender with no
hepatosplenomegaly appreciated.
• There was no peripheral edema or clubbing.
Sunday, January 06, 2013
21. Lab
• White blood cells 6.2 K/Ul
• Differential, normal
• Hemoglobin12.4 g/dLHematocrit 39.4 %
• Platelets285 K/uL
• Alpha 1 Anti-trypsin117 mg/dL (80-240 mg/dL)
• Immunoglobulin G (IgG) 1001 mg/dL (700-1600 mg/dL)
• Immunoglobulin A312 mg/dL (70-400 mg/dL)
• Immunoglobulin M 116 mg/dL (40-230 mg/dL)
• Immunoglobulin E 18.4 IU/mL (7-135 IU/mL)
• Total hemolytic complement titer (CH50)115 units (51-150 units)
• Pneumococcal antibody titer normal
• Tetanus IgG antibody titer normal
• Diphtheria IgG antibody titer normal
Sunday, January 06, 2013
22. Pulmonary Function Testing
• Pulmonary Function Testing
• (FVC) …..3.54 L (109% predicted)
• (FEV1) 2.94 L (105% predicted)
• FEV1/FVC 81.4%
• (TLC) 4.5 L (104% predicted)
• (DLCO) 19 mL/min/mm Hg (82% predicted)
• Flow volume loop normal
Sunday, January 06, 2013
25. Question 1
• What is the most likely diagnosis?
• A. Uncontrolled Asthma
• B. Primary Ciliary Dyskinesia/Kartagener
Syndrome
• C. Cystic Fibrosis
• D. Bronchiectasis
• E. Chronic Sinusitis
Sunday, January 06, 2013
26. discussion
• Primary Ciliary Dyskinesia (PCD) is a rare, typically autosomal recessive disease characterized by
dysfunctional ciliary motility and impaired mucociliary clearance leading to multiple clinical
manifestations including sino-pulmonary disease, infertility, and anatomic defects.
• In about half of the cases it is associated with situs inversus, also known as Kartagener
syndrome.
• The link between Kartagener syndrome and immotile cilia was first described in 1976 .
• The prevalence of situs inversus appears to be random and thought to be associated with loss of
ciliary nodal function in embryogenesis .
• The incidence of PCD is estimated at 1/16,000 births based on the presence of bronchiectasis and
situs inversus which is likely underestimating the burden of disease given the heterogeneous
nature and difficulty in diagnosing the disease .
• Given this heterogeneity, diagnosis is often delayed until late childhood or early adulthood.
• This diagnosis should be considered in those patients with the following:
– Those with situs inversus/heterotaxia
– Those with unexplained neonatal respiratory distress or hydrocephaly
– Those with a chronic productive cough, bronchiectasis of unknown cause, or other severe upper airway
disease
– Males with immotile sperm
– Females with recurrent ectopic pregnancy and/or infertility with other features of PCD
Sunday, January 06, 2013
27. Question 2
• What is the best therapeutic management of
this patient?
• A. Bronchodilators
• B. Hypertonic saline
• C. Oral corticosteroids
• D. Lung transplantation
• E. Aggressive antibiotic therapy and airway
clearance maneuvers
Sunday, January 06, 2013
28. discussion
• There are no current medications specifically approved for use in PCD.
• Additionally there are no randomized control trials in the treatment of PCD.
• Treatment strategies are largely extrapolated from the cystic fibrosis literature and based on expert opinion.
• Goals of treatment are to detect and treat complications with hopes of restoring and maintaining normal lung
function.
• Generally, aggressive treatments of pulmonary infection as manifested by worsening cough and sputum
production, regular airway clearance maneuvers, and prevention of infection through proper immunization are
the primary management modalities.
• Sputum cultures should be obtained at baseline and with exacerbations to tailor antibiotic therapy.
• Haemophilus influenzae, Staphylococcus aureus, and Streptococcus pneumoniae are the most common bacteria
isolated from sputum.
• Psuedomonas aeruginosa is seen more often in adults and isolated in 15% of patients.
• Inhaled antibiotics in the presence of pseudomonas have been recommended based on cystic fibrosis evidence
that there is better penetration with deceased systemic effects .
• There is no evidence to recommend prophylactic antibiotics, however, there are some reports of azithromycin
causing slight improvements in pulmonary function and rates of exacerbations in non-cystic fibrosis
bronchiectasis.
• Inhaled hypertonic saline has been demonstrated to improve lung function and reduce frequency of
exacerbations in cystic fibrosis .
• No studies have evaluated the use of this medication in PCD.
• Additionally, rhDNase has anecdotally been shown to improve symptoms but no randomized studies are
available .
• If there is evidence of reversible airflow obstruction, true asthma, or wheezing, then it is recommended that the
patient with PCD be treated according to current asthma guidelines .
Sunday, January 06, 2013
29. Question3
• Currently, what is the "gold standard" for
diagnosis of primary ciliary dyskinesia?
• A. Exhaled nitric oxide measurement
• B. Genetic testing
• C. Respiratory epithelial biopsy with electron
microscopy
• D. Sweat chloride testing
• E. Pulmonary function testing
Sunday, January 06, 2013
30. Question 4
• What is the expected course of lung function
change in patients diagnosed with PCD?
• A. Stabilization of lung function
• B. Deterioration of lung function
• C. Improvement of lung function
• D. Initial improvement, then stabilization of
lung function
• E. Variable course of lung function
Sunday, January 06, 2013
31. discussion
• Observational studies have suggested that the earlier a patient is
diagnosed, the more favorable the outcome.
• Lung function is believed to stabilize when the patient is
diagnosed and managed at a multidisciplinary center .
• These observations have been based on small numbers of
patients.
• More recently a Danish group studied seventy-four children and
adults with PCD and measured 2,937 lung function measurements
over 30 years.
• This is the largest longitudinal study reported to date.
• They demonstrated that there was a variable course of lung
function (measured FEV1) with 34% showing decline, 57%
showing stability, and 10% showing improvement.
• Age of diagnosis and initial FEV1 were not related to course of
disease .
Sunday, January 06, 2013
32. Question 5
• For which non-respiratory complication is this
patient at highest risk?
• A. Chronic suppurative otitis media
• B. Ectopic pregnancy
• C. Speech delay
• D. Congenital heart disease
• E. Liver disease
Sunday, January 06, 2013
33. discussion
• Several non-respiratory complications can be seen in patients with PCD.
• The most common are related to chronic sinusitis and chronic middle ear
effusions and are most often observed in children.
• Evaluation by an otolaryngologist is essential.
• Chronic middle ear effusions in young children have been thought to contribute
to hearing loss with speech delay.
• The management of this is controversial.
• Myringototomy tubes sometimes lead to chronic otorrhea, but may improve
hearing and speech .
• Fertility issues in men and woman are a concern and frequent complication in
PCD.
• Although it is thought that males are universally infertile secondary to
dysfunctional sperm, the heterogeneity of the disease suggests that up to 50% of
males may conceive .
• Women are at a particular risk for ectopic pregnancies and may have reduced
fertility although not as prevalent as in males .
• Situs inversus totalis itself does not cause medical complications in those with
PCD, however, those with laterality defects may be at increased risk for
congenital heart disease and should be screened accordingly .
Sunday, January 06, 2013
37. HRCT-1
• Find and outline a group of coarse
centrilobular nodules in the right lung.
• Find a single coarse centrilobular nodule in
the left lung.
• Find a conglomerate mass that causes
deviation of the major fissure in the right lung.
40. • Find a group of fine centrilobular nodules
(rosette) in the left lung.
• Find a fine tree-in-bud pattern in the left lung
and a coarse one in the right lung.
• Find a conglomerate mass in the right lung.
• Find a conglomerate mass containing a cavity .
43. • This slice of lung has a mass of conglomerate nodules
in the posterior segment of the upper lobe. The
opaque white regions in the mass represent necrosis,
and the pinkish areas, viable inflammatory infiltrates.
The irregularly-shaped slits represent cavitation.
• Find two slits.
• Note the satellite centrilobular nodules, indicating in
this case an endobronchial spread of disease.
• Find 2 centrilobular nodules in a lobule outlined by
edematous, grey, interlobular septa. Note also the
centrilobular nodules in another lobule just below this
one.
46. • Outline and describe (name) the type of tissue
at the lower right.
• How would you describe the inflammatory
response in the rest of the picture?
• Find and name 4 of the large, multinucleated
cells in the inflammatory infiltrate.
• What types of cells compose the rest of the
infiltrate?
47. Differential diagnosis of centrilobular
nodules and cavitating masses on HRCT:
• Tuberculosis
• fungal,
• Non-tuberculous mycobacterial.
• chronic bacterial.
• Centrilobular nodules and cavitating masses can also
be seen with:
• Langerhans' cell histiocytosis.
• Sarcoidosis.
• Silicosis.
• coal workers' pneumoconiosis.
• Wegener's granulomatosis.
• rheumatoid arthritis.
50. Summary
• Summary of diagnostic features of
endobronchial tuberculosis on HRCT:
• Tree-in-bud pattern
• Clustered centrilobular nodules
• Mass-like areas of consolidation
• Cavitation in larger nodules or masses