2 pulmon, vol. 20, issue 1, jan - apr 2018 - apccm

Pulmon, Vol. 20, Issue 1, Jan - Apr 2018 1

Editorial

5 Medical Thoracoscopy -The Window of Opportunity in Pleural DiseasesRavindran Chetambath

Review Article

10 High Flow Nasal Cannula for Oxygen Delivery inPulmonary and Critical Care PracticeRajesh V.

Original Article

18 Medical Thoracoscopy :Diagnostic Utility in a Tertiary Care Setting in South IndiaRennis Davis K.

Radiology Pearl

24 Radiology PearlVenu Gopal P.

Self Assessment Quiz

27 Vishnu Sharma M.

Case Reports

29 Thoracic Ewings Sarcoma with Right Atrial Invasion -A Case ReportSmitha Nair P.G.

33 An Unusual Bronchiectasis of Azygos LobeKesavan Nair V.

36 Cannonball Lesions in a Young Patient : An Unusual CauseMathew Ninan

Guidelines for authors

Contents

Vol 20, Number 1, (Jan - Apr) 2018 ISSN 0973 - 3809

PulmonThe Journal of Respiratory Sciences

2 Pulmon, Vol. 20, Issue 1, Jan - Apr 2018


Editorial

Medical Thoracoscopy -The Window of Opportunity in Pleural Diseases

Ravindran ChetambathProfessor & HeadDept. of Pulmonary MedicineDM Wayanad Institute of Medical SciencesWayanad, Kerala.

Pleural disease remains common, affecting over 3000 personsper million population each year. It therefore contributes significantlyto the workload of a Respiratory physician. To find out the cause ofpleural effusion, thoracocentesis for biochemical/microbiologicalanalyses of pleural fluid and closed pleural biopsy were usuallyemployed.However, this initial analysis has low sensitivity to detecttuberculosis and malignancies; the two most important causes ofpleural effusions in India. The advent of medical thoracoscopy hasrevolutionized the diagnostic approach of pleural diseases acrossthe globe.

The concept of medical thoracoscopy (MT) is a simplification ofvideo-assisted thoracic surgery (VATS), as it is done under conscioussedation through a single port by the chest physician. The 2010 updateof British Thoracic Society (BTS) guidelines for pleural diseasesrecommended medical thoracoscopy (MT), surgical thoracoscopy(VATS) or radiology guided pleural biopsy in preference to closedpleural biopsy with Abram’s needle in the management of undiagnosedexudative pleural effusion1. There are very few studies comparingthe diagnostic yield of these procedures. In a prospective study in 40cases from South Africa in tuberculosis, medical thoracoscopy had adiagnostic yield of 98% in comparison with an 80% diagnostic yieldwith Abram’s needle biopsy2. In an intra-patient comparison study intuberculosis, a histological diagnosis of tuberculosis was obtained in94 % of the cases by thoracoscopic biopsy compared to 38 % withclosed pleural biopsy using Trucut needle3. Loddenkemper et al


reported a diagnostic yield of 95% for thoracoscopic biopsy comparedto 44% with closed pleural biopsy alone and 74% when closed pleuralbiopsy was combined with fluid cytology in malignant pleural effusions4.The sensitivity of MT for malignancy is approximately 93%- 95%compared with pleural fluid cytology at 57.6% and closed pleural biopsyat 43%5. The diagnostic yield of MT in pleural tuberculosis is 100%specific and sensitive based on histology and bacteriology comparewith 79% with Abram’s needle biopsy. A randomized controlled studyconducted in South India reported a diagnostic yield of 86.2% with acomplication rate of 10.3% with medical thoracoscopy when comparedto a diagnostic yield of 62.1% and a complication rate of 17.2% withclosed pleural biopsy group6. The relatively low yield of closed pleuralbiopsy is due to several factors, including minimal and non-uniformpleural involvement in early disease. These limitations can beovercome by MT. The markedly improved yield of MT over othermodalities is likely owing to the size of specimens and ability to reliablyperform a biopsy of abnormal area under direct vision7. Whencompared to surgical thoracoscopy (VATS), medical thoracoscopyhas the advantage of being performed under local anesthesia andconscious sedation, in an endoscopy suite. Thus, it is considerablyless invasive and less expensive. Hence in many centers, anondiagnostic pleurocentesis is followed by thoracoscopy. In additionto its high diagnostic yield, thoracoscopy can be used for therapeuticprocedures too. In this issue of the journal, Rennis D et al present thedata of 250 thoracoscopies from his centre in the article “Medicalthoracoscopy: Diagnostic utility in a tertiary care setting in SouthIndia”. A single center study on 250 subjects undergoing thoracoscopyis one of the few studies from South India. His result also reiteratesthe high positive yield of MT in exudative pleural effusion.

Rigid thoracoscopy, with or without video assistance hastraditionally been the procedure of choice8. A semi-rigid thoracoscope(pleuroscope) combining the features of rigid thoracoscope andflexible bronchoscope has been available since its first report in 19989.Two recent systematic reviews have concluded that the semi-rigidthoracoscope is a safe, easy-to-handle, and accurate tool in thediagnosis of pleural effusions of undetermined origin10.Anotherretrospective study11 found comparable histologic yields of the rigidand the semi-rigid thoracoscopes. The decision to choose semi-rigidor rigid thoracoscopy depends on the extent of the adhesions foundon imaging. It is suggested that semi-rigid thoracoscopy is unlikely tohelp in those with extensive adhesions, and one should directly resortto rigid thoracoscopy or VATS12.

MT is mainly indicated for diagnosis of pleural effusions ofunknown etiology. MT is also useful for evaluation of spontaneouspneumothorax and empyema. For those familiar with the technique,MT can also be used for diagnostic biopsies from the lungs, diaphragm,

Ravindran Chetambath - Medical Thoracoscopy - The Window of Opportunity in Pleural Diseases



mediastinum and pericardium. The commonest therapeuticprocedures for which MT can be used are pleurodesis with talcpoudrage for symptomatic recurrent malignant pleural effusion13 andrecurrent pneumothorax.

Contraindications to MT are uncommon and rarely absolute. Themain limitation is the size of the pleural space, which must be at least10 cm in depth. If extensive adhesions are present MT can be carriedout without creating a pneumothorax, but this requires special skillsand should not be undertaken without special training14. Several factorsthat make it necessary to delay MT,but are rarely prohibitive includea persistent cough, hypoxemia, hypocoagulability and cardiacabnormalities. Great care should be taken in presence of hypercarbia.Depending on its severity, respiratory failure proves to be an absolutecontra-indication, except in patients with a tension pneumothorax ormassive pleural effusion, in whom MT may provide a therapeuticbenefit. In such conditions, premedication should be given judiciously.Contraindications to pulmonary biopsy are arteriovenous pulmonaryaneurysms, vascular tumors, hydatid cysts and stiff fibrotic lung15.Relative contraindications include previous systemic steroid orimmunosuppressive therapy, as the resulting bronchopleural fistulasmay heal poorly.

MT is a safe and effective treatment modality in the diagnosisand treatment of several pleuropulmonary diseases if standardcriteria are fulfilled. Most of the series have reported a mortality rateof less than 0.1%. The reported complications in various studies werebleeding from a biopsy site, arrhythmias, hypotension, hypoxemia,persistent air leak of over 7 days, subcutaneous emphysema,postoperative fever, empyema, pulmonary infections and malignantinvasion of the scar. In case of smaller persistent bleeding,electrocoagulation may be necessary. MT was associated withmortality and complication rates of 0.37% and 5.6%, respectively,whereas the complication rate with Closed-Blind Pleural Biopsy was8.3% with no mortality16.

In conclusion, MT is the procedure of choice in the evaluation ofundiagnosed exudative pleural effusion, due to its higher successrate and an acceptable safety profile. Hence MT has an irrefutablerole in the management of exudative pleural effusion and should bethe next investigation after an initial inconclusive pleural fluid study.

References

1. Maskell NA, Butland RJA, on behalf of the British Thoracic SocietyPleural Disease Group. BTS guidelines for the investigation of a


unilateral pleural effusion in adults.Thorax. 2003; 58: (Suppl. II)ii8–ii17.

2. Walzl G, Wyser C, Smedema J, Corbett C, van de Wal B.Comparing the diagnostic yieldof Abrams needle pleural biopsyand thoracoscopy. Am J RespirCrit Care Med 1996;153: A460

3. Loddenkemper R, Grosser H, Gabler A, Mai J, Preussler H, BrandtHJ. Prospective evaluation of biopsy methods in the diagnosis ofmalignant pleural effusions- Intra-patient comparison betweenpleural fluid cytology, blind needle biopsy and thoracoscopy. AmRev Respir Dis 1983; 127 (Suppl. 4):114-19.

4. Loddenkemper R, Grosser H, Mai J, Preussler H, Wundschock M,Brandt HJ. Diagnosis of tuberculous pleural effusion: prospectivecomparison of laboratory chemical, bacteriologic, cytologic andand histologic study results Prax Klin Pneumol 1983; 37:1153-56.

5. Parkash UB, Reiman HM. Comparison of needle biopsy withcytologic analysis for the evaluation of pleural effusion: analysisof 414 cases. MyoClinProc1985; 60(3):158-64.

6. Nithya Haridas, Suraj K P, Rajagopal T P, James P T, RavindranChetambath. Medical Thoracoscopy vs Closed Pleural Biopsy inPleural Effusions: A Randomized Controlled Study. Journal ofClinical and Diagnostic Research 2014;8(5): MC01-MC04. Googlescholar.

7. Gaetane M, David M Berkowitz, Armin Ernst.Pleuroscopy fordiagnosis and therapy for pleural effusions. Chest 2010; 138(5):1242-46

8. Mootha VK, Agarwal R, Singh N, Aggarwal AN, Gupta D, JindalSK. Medical thoracoscopy for undiagnosed pleural effusions:experience from a tertiary care hospital in north India. Indian JChest Dis Allied Sci 2011;53(1):21-24. PubMed Google Scholar

9. McLean AN, Bicknell SR, McAlpine LG, Peacock AJ. Investigationof pleural effusion: an evaluation of the new Olympus LTFsemiflexible thoracofiberscope and comparison with Abram'sneedle biopsy. Chest 1998;114(1):150-53.PubMedGoogle Scholar

10. Mohan A, Chandra S, Agarwal D, Naik S, Munavvar M. Utility ofsemirigid thoracoscopy in the diagnosis of pleural effusions: asystematic review. J Bronchology Interv Pulmonol 2010;17(3):195-201.PubMedGoogle Scholar

11. Rozman A, Camlek L, Marc-Malovrh M, Triller N, Kern I. Rigidversus semi-rigid thoracoscopy for the diagnosis of pleural



disease: a randomized pilot study. Respirology 2013;18(4):704710.PubMedGoogle Scholar

12. Sahajal Dhooria, Navneet Singh, Ashutosh N Aggarwal, DheerajGupta and RiteshAgarwal. A Randomized Trial Comparing theDiagnostic Yield of Rigid and Semirigid Thoracoscopy inUndiagnosed Pleural Effusions. Respiratory Care 2014, 59 (5) 756-64.

13. Loddenkemper R. Thoracoscopy state of art. EurRespir J1998;11:213-21.

14. Janssen JP,Boutin C: Extended thoracoscopy: A biopsy method tobe used in case of pleural adhesions.EurRespir J 1992; 5:763- 66.

15. LoddenkemperR,BoutinC. Thoracoscopy- Present diagnostic andtherapeutic indications.EurRespir J 1993; 6:1544-45.

16. Maturu VN, Dhooria S, Bal A, Singh N, Aggarwal AN, Gupta D,Behera D, Agarwal R. Role of medical thoracoscopy and closed-blind pleural biopsy in undiagnosed exudative pleural effusions:a single-center experience of 348 patients. J Bronchology IntervPulmonol. 2015 Apr;22(2):121-29



Review Article

High Flow Nasal Cannula for Oxygen Delivery inPulmonary and Critical Care Practice

Rajesh V.1, Divya R.2, Jolsana Augustine2, Melcy Cleetus3

Senior Consultant and HOD1, Consultant2, Junior Specialist3

Department of Pulmonary Medicine, Rajagiri Hospital, Aluva, Kochi

Abstract

Oxygen therapy is the mainstay of treatment in critically ill hypoxic patients with differentprimary disease states. The therapeutic benefits of oxygen therapy depend heavily on its judicioususage. The delivery device has a key role in the success of oxygen therapy. The choice of oxygendelivery device depends on a multitude of factors including the magnitude of oxygen requirement,efficacy of the device, reliability, ease of therapeutic application, cost of therapy and patient acceptance.Although design plays an important role in selection of these devices, clinical assessment of performanceand response is crucial to success. Oxygen delivery devices are categorized into three basic typesbased on their design: low-flow, reservoir, and high-flow. Regarding the FiO2 range oxygen systems canbe divided into three indicated for low oxygen delivery (<35%), moderate delivery (35%-60%) or highdelivery (>60%). Some devices can deliver a wide range of oxygen percentages.

The high flow nasal cannulas are high flow oxygen delivery devices that deliver adequatelyheated and humidified medical gas at up to 60 L/min of flow. High-flow nasal cannula (HFNC) oxygentherapy comprises an air/oxygen blender, an active humidifier, a single heated circuit, and a nasalcannula. It is easy to administer and is well tolerated by patients.

Key words

Respiratory failure, oxygen therapy, respiratory circuits, oxygen delivery devices

Background

Supplemental oxygen therapy is the cornerstone and first-line treatment for hypoxemic respiratoryfailure due to a multitude of aetiologies. Oxygensupplementation allows immediate correction ofhypoxia, rapidly stabilises the hypoxic subject andprovides time for definitive therapeutic measures to

act. Oxygen may be supplemented via a variety ofdelivery devices, each having its own unique advantagesand disadvantages. The present review briefly touchesupon the various currently available delivery devicesand attempts to enlist the shortcomings of existingdevices. We also provide a detailed description of highflow nasal cannula, a relatively new oxygen deliverydevice, which circumvents many of the disadvantages


Rajesh V. - High Flow Nasal Cannula for Oxygen Delivery in Pulmonary and Critical Care Practice

of conventional ones. Emphasis is placed on availableevidences and practical recommendations for bedsideusage.

Supplemental Oxygen therapy

Oxygen delivery devices may be broadlygrouped into low flow and high flow devices. High flowdevices deliver oxygen at flow rates sufficient to meetthe inspiratory demand of the subject. They therefore,tend to deliver reliable FiO2 levels, although minorfluctuations may be expected based on the degree oftight-fit of the interface. On the other hand, low flowdevices deliver O2 at flow rates less that inspiratoryrequirements and hence deliver imprecise FiO2 levels.The common low flow devices include simple O2 cannula,O2 catheter, simple face mask etc, whereas the venturimask and non-rebreathing reservoir bag mask are highflow devices. It has to be noted that high flow device isnot synonymous with high FiO2 and vice versa. We candeliver low levels of precise FiO2 via a high flow deliverydevice like venturi mask.

The conventional devices pose a lot ofchallenges to oxygen delivery.1

During spontaneous breathing, inspired gashas to move through the upper air passages andtrachea which effectively warm the air to bodytemperature and humidify it with water vapour. This issuccessfully achieved even when the ambient air is coldand dry. Supplemental oxygen is made up of dry gasand is not usually humidified when administered at lowflow rates. Dry or poorly humidified medical gas mayhave adverse medical consequences, such as dry nose,dry throat, and nasal pain, and consequent poortolerance of oxygen therapy. Unconditioned gasincreases airway resistance and contributes toworsening work of breathing.2 Breathing dry air is knownto reduce nasal and bronchial mucociliary clearance.3

Using conventional devices, the maximum attainableoxygen flow is limited to 15 L/min. The inspiratory flowdemand for critically ill respiratory patients varies widely,ranging from 30 to 120 L/min, which cannot be deliveredvia the above mentioned devices. This may translate tolower delivered FIO2 than the real patient needs andfluctuating / unreliable FIO2 levels. All these may haveadverse effects on clinical outcomes.

High flow nasal cannula

High flow nasal cannula is a relatively newoxygen delivery device that is slowly establishing itsplace in recent years as a safe and efficacious mode ofadministering supplemental O2.4 It delivers conditionedgas at near body temperatures (37 °C) containing 44 mgH2O/l [100% relative humidity] using a heated humidifierand a heated inspiratory circuit. This is delivered atpatient’s nares through a wide bore nasal cannula atvery high flow (up to 60 l/min) at a predeterminedconstant oxygen concentration (21 to 100%). Thistechnique of delivery, as discussed below, has a lot ofphysiological and clinical benefitsin many disease states.

Pathophysiological mechanisms of clinicalbenefits

High flow nasal cannula (HFNC) supportivetherapy exerts its beneficial effects through a variety ofpotential mechanisms.

1. Provision of high and precise FiO2 – HFNC with itshigh flow rate of upto 60 litres / minute can deliverhigh FiO2 as well as precise FiO2 (because theinspiratory flow rate can be met even insignificantly hyperventilating subjects). WithHFNC, especially at high flow, actual delivered FiO2

is almost equal to calculated (predicted) FiO2. Inpublished literature, it was seen that during nosebreathing, at inspiratory flow above 30 L/min, themeasured FiO2 was close to the delivered FiO2

with HFNC.5

2. Dead space wash out and CO2 clearance - Byproviding a high fresh air flow during expiration,HFNC is capable of effectively washing out thecarbon dioxide contained in the anatomical deadspace, thus contributing to CO2 clearance.6Further,the gaseous admixture achieved by continuousstream of a high flow gas mixture facilitatesoxygenation and CO2 clearance.

3. PEEP effect - HFNC therapy contributes to apositive pharyngeal and airway pressure duringexpiration due to its constant high gas flow. Themagnitude of PEEP attained is determined by theflow rate and the expiratory flow exhaled by thepatient. Lower pressures are observed when the



patient keeps the mouth open.7Despite thisuncertainty about how much positive end-expiratory pressure (PEEP) can really be offeredby HFNC, studies8 have consistently shownincreasing end expiratory lung impedance withhigher flow rate of HFNC, which is a surrogatemarker of an increase in end expiratory lungvolume.

4. Effects of humidification - Conditioning of the gasreduced the chance of bronchospasm, improvesairway calibre and reduces the work of breathing.Furthermore, conditioned gas improvesmucociliary function and thereby promotesclearance of secretions. All these translate tolesser incidence of atelectasis, resulting in a goodventilation/perfusion ratio and better oxygenation.In other words, conditioning gases may be viewedupon as a lung recruitment strategy.

5. Reduction of nasopharyngeal resistance - Anothercrucial effect noticed with HFNC therapy is thereduction in nasopharyngeal air flow resistance.Research on obstructive sleep apnea subjects byanalysing flow volume loops in the nasopharynxhas revealed that the nasopharynx is expansiblethereby accounting forvariations in resistance.9

The most probable mechanism by which HFNCreduces inspiratory nasopharynx resistance islikely to be by increasing inspiratory flow.10

Indications for O2 therapy with HFNC

HFNC as an oxygen delivery device has beentried in a multitude of clinical conditions and a criticalappraisal of each and every published trial is beyondthe scope of this review. However, the following generalcomments can be made regarding the publishedevidences. HFNC has to its credit a higher body ofevidence in hypoxemic respiratory failure thanhypercapneic failure. The clinical scenarios where HFNChas been employed have been summarised in table 1.

1. Acute hypoxemic respiratory failure due topneumonia and ARDS

2. Acute hypoxemic respiratory failure due tocardiogenic pulmonary edema

3. Post extubation respiratory compromise

4. During airway and upper gastrointestinalendoscopies

5. During anaesthesia induction and airwayinstrumentation

6. Immunocompromised subjects with respiratoryfailure

7. End of life care patients

The first entity has the largest published bodyof evidence in contemporary medical literature.

Table 1 : Usual Indications for Using HFNC

Acute hypoxemic respiratory failure due topneumonia and ARDS

Acute hypoxemic respiratory failure due tocardiogenic pulmonary edema

During anaesthesia induction and airwayinstrumentation

During invasive airway procedures (bronchoscopies)

Post extubation respiratory compromise

Respiratory failure in emergency department

Immunocompromised subjects with respiratoryfailure

End of life care

Evidences on HFNC

Early studies of HFNC in patients with acuterespiratory failure focused on the effects onphysiological variables and reported improvements inoxygenation with reductions in respiratory rate and noobserved alterationsin PaCO2.11 The first randomizedcontrolled trial (RCT) was conducted on postoperativecardiac surgery patients with mild to moderate acute



respiratory failure.12This trial observed that HFNCpatients were less likely to need escalation to NIV andhad fewer desaturations than those who receivedsupplemental O2 via a standard high flow face mask. Inanother analysis of a cohort of 37 lung transplant patientsreadmitted to the ICU because of acute respiratoryfailure, HFNC therapy was the only variable at ICUadmission associated with a decreased risk ofmechanical ventilation in the multivariate analysis.13

In the authors’ opinion the real landmark trialthat attracted the attention of the scientific world to thismodality of therapy was published in 2015.14 This studyhad the advantage of being a large RCT published in ajournal with high impact factor. The study included 310patients with acute hypoxemic acute respiratory failurehaving a PaO2/FiO2 ratio ≤ 300 mmHg with 10 litre perminute of O2, a clinical scenario that would probably fitinto the Berlin definition of ARDS. The study hadstringent exclusion criteria, which would raise concernfor its use in many critical care scenarios, but is probablyjustified considering this as an early path breaking study.Patients with a history of chronic respiratory disease,including COPD, as well as patients with acutecardiogenic pulmonary edema, severe neutropenia andhypercapnia (PaCO2 > 45 mmHg) were excluded, aswere patients with other organ failures, includinghemodynamic instability or vasopressors at the time ofinclusion. The commonest etiology of acute respiratoryfailure was pneumonia. Study subjects were randomisedto supplemental O2 via HFNC versus NIV. Although theprimary outcome, the intubation rate, did not differbetween the treatment arm and controls, the 238patients with a PaO2/FiO2 ratio ≤ 200 mmHg found thatHFNC reduced intubation rates(p = 0.009). In the entirecohort, HFNC increased ventilator free days, reduced90 day mortality and was associated with better comfortand lower dyspnea severity. In contrast, NIV patientshad higher 90 day mortality rates than HFNC patients.

HFNC has been used in the peri-intubationsetting and has the advantage, as opposed to bag andmask ventilation, that it continues to deliver high FIO2levels even when the process of endotracheal tubeinsertion is ongoing. Although not routine, HFNC is anacceptable way to provide oxygen to patients undergoingintubation, both before (preoxygenation) and during theprocedure (to prevent desaturation). Data areconflicting regarding the value of HFNC for

preoxygenation prior to intubation. One randomizedsingle centre study compared four minutes ofpreoxygenation with HFNC (100 percent FiO2 at 60 L/minute) together with concomitant NIV (10 cm H2Opressure support ventilation and 5 cm H2O PEEP) withNIV alone prior to intubation. HFNC/NIV resulted inhigher peripheral oxygen saturations (100 versus 96percent) and fewer patients with episodes ofdesaturation below 80 percent (0 versus 21 percent).15

In contrast, in a multicentre study of 124 patientsundergoing intubation who had severe hypoxemia(PaO2:FiO2 ratio <300 mmHg, respiratory rate >30 breaths/minute, and a FiO2>50 percent to achieve a saturation of>90 percent), HFNC did not reduce the lowest saturationduring intubation when compared with preoxygenationusing a conventional high-flow oxygen face mask.16

HFNC has also been used to successfullyoxygenate hypoxemic patients undergoing fiberopticbronchoscopy. Such patients are at high risk ofrespiratory decompensation requiring ventilatorsupport as a consequence of the procedure. In arandomized trial of 40 patients with hypoxemicrespiratory failure who underwent bronchoscopy, therewas no difference in peripheral oxygen saturationbetween patients who were supported with NIV andpatients supported with HFNC.17 One HFNC patientdeteriorated and required NIV; however, three NIVpatients required endotracheal intubation within 24 hoursof bronchoscopy versus one in the HFNC group. Therole for HFNC during high-risk bronchoscopy remainsto be defined with larger studies.

Studies have looked at the success of HFNC inpost extubation respiratory failure subjects. The firstRCT comparing HFNC with conventional oxygendevices after extubation included patients with acuterespiratory failure due to pneumonia and trauma whowere mechanically ventilated for a mean of almost fivedays before extubation.18 In these patients, the use ofHFNC was associated with better comfort, betteroxygenation, fewer desaturations and interfacedisplacements, and a lower reintubation rate.Hernandez et al.19 compared HFNC with NIV in patientsat high risk for reintubation in a non inferiority trial. Thisstudy confirmed that the reintubation rate was noninferior in the HFNC group compared to the NIV groupwithin 72 h (22.8% vs 19.1%). For postextubationrespiratory failure, the authors reported a lower rate in



the HFNC group compared to the NIV group (26.9% vs39.8%), suggesting that the postextubation respiratoryfailure rate could be even higher in the NIV group.

Table 2 : Major trials of HFNC in acute hypoxemic respiratory failure and cardiac surgery

Tables 2 and 3 given below summarise some ofthe major trials conducted with HFNC in respiratory andcritical care arenas.

ETIOLOGYYEAR

(NUMBER) NATURE OF STUDY OUTCOME

2015 (37) Roca et al Retrospective cohort Positive

2015 (313) Frat et al RCT Positive

2015 (100) Lemiale et al RCT No change

2015 (175) Kang et al Retrospective observational study Negative

2015 (178) Mokart et al Retrospective propensity matched Positive

2013 (340) Parke et al RCT Positive

2015 (155) Corley et al RCT No change

2015 (830) Stephan et al Randomised non inferiority No change

STUDYGROUP

AcuteHypoxemicRespiratory

Failure

CardiacSurgery

Table 3 : Major trials of HFNC in Post extubation subjects, ED and during invasive procedures

ETIOLOGYYEAR

(NUMBER) NATURE OF STUDY OUTCOME

2015 (124) Vourch et al RCT No change

2014 (105) Maggiore et al RCT Positive

2014 (17) Rittayamai et al RCT Positive

2010 (50) Tiruvoipati et al RCT No change

2016 (527) Hernandez et al RCT Positive

2012 (45) Lucangelo et al RCT Positive

2014 (40) Simon et al RCT Negative

2015 (40) Rittayamai et al RCT Positive

2015 (303) Jones et al RCT Positive

STUDYGROUP

Preoxygenation

Duringbronchoscopy

Postextubation

EmergencyDepartment



Instrumentation

HFNC is being commercially marketed byFisher and Paykel health care under the trade nameAIRVO 2. The AIRVO 2 has a humidifier with integratedflow generator that delivers high flow warmed andhumidified respiratory gases to spontaneouslybreathing patients through a variety of patientinterfaces.

Fig 1 : High flow nasal cannula device (AIRVO 2)

Fig 2 : Water chamber with heatedbreathing tube and patient interface

Fig 3 : Pictorial representation of HFNC devicewith its accessories connected to a patient

It has an inbuilt heated humidifier with portsfor oxygen inlet, heated breathing tube and a slot tomount the auto fill water chamber. Reusable accessoriesinclude the water chamber, the heated breathing tubeand the patient interface. The interface is available inpaediatric and adult sizes. The device has no batteryback-up and mandatorily needs a power source to work.Control panel exhibits the temperature of delivered gas,flow rates and FiO2. Flow rates and temperature can bedirectly set whereas the FiO2 can be adjusted bycontrolling the O2 inflow rate into the blender.

Bedside Institution

There are no set recommendations for itspractical application; thus, the suggestions below is anopinion of the authors based on sound rationale andour own practice. Although HFNC can be administeredon an unmonitored floor, in our opinion, it is best appliedin a monitored setting such as the intensive care unit,high dependency unit, or emergency department.20Ourrecommendation is based upon the rationale thatpatients in need of HFNC are at high risk of severerespiratory failure or mechanical ventilation, in whichcase a closely monitored setting is often needed.However, once the patient improves and needs seriallyreduced oxygen requirements (e.g, 50 L/minute at 60percent fraction of inspired oxygen [FiO2]), HFNC maybe delivered in a less monitored setting.

Oxygen flows from the source, is heated andhumidified, and is then delivered to the patient throughwide bore nasal cannulae, which are generally made ofsofter, more pliable plastic than cannulae for low-flowsystems. The cannulae fit snugly into the nares and areheld in place with a head strap. Two parameters need tobe set for optimal care are the flow rate through thecannula and the FiO2. The temperature also needs to beset, but is more of a concern for patient comfort. Weprefer to set the flow rate first, typically at 20 to 35 L/minute (range 5 to 60 L/minute). The FiO2 (range 21 to100 percent) is next set to target a desired peripheraloxygen saturation. The flow rate can subsequently beincreased in 5 to 10 L/minute increments if therespiratory rate fails to improve, oxygenation fails toadequately improve, or breathing remains laboured.Increasing the flow rate and FiO2 will both result inimproved peripheral oxygen saturation; we prefer tomaximize the flow rate first in an attempt to keep the



FiO2 ≤ 60 percent; however, an increase in FiO2 may benecessary to achieve adequate oxygenation. Patientsmay be switched to conventional low-flow nasal cannulasystem once the flow rate reaches ≤ 20 L/minute andFiO2 ≤ 50 percent.

Predictors of success

Reliance on early predictors of HFNC successis important given the fact that delayed intubation mayworsen the prognosis of patients treated with HFNC.21

Therefore, the ability to describe accurate predictors ofHFNC success thatcan allow timely endotrachealintubation in patients whoare likely to fail is of paramountimportance. Sztrymfet al11 reported that respiratory rateas well as the percentage of patients exhibiting thoracoabdominalasynchrony as early as 30 and 15 min afterthe beginning of HFNC were significantly higher inpatients who required endotracheal intubation.Moreover, the PaO2/FiO2 ratio 1 h after the start of HFNCwas significantly lower in patients requiring invasivemechanical ventilation. Interestingly, a recentprospective study showed that patients with severepneumonia who had a ROX index (defined as the ratioof SpO2/FiO2 to respiratory rate) ≥ 4.88 after 12 h ofHFNC therapy were less likely to be intubated, evenafteradjusting for potential covariates.22 From the previousevidences, it is clear that physiological and clinicalresponse in the first few hours of institution helps predictsuccess of HFNC and anyone who does not improvefast potentially needs early intubation and mechanicalventilation.

Shortcomings of HFNC

From a clinical stand point, O2 supplementationwith HFNC needs to be head to head compared withother O2 delivery systems as well as non-invasiveventilation (NIV) with regard to superiority andshortcomings. Clearly, HFNC is a superior O2 deliverydevice than O2 cannula, face mask, venturimask etc inits ability of being a reliable and comfortable device.The challenges dealt with relate to the economicimplications, HFNC being a costly device. There needsto be personnel who are familiar with the setting up anddisinfection of the HFNC apparatus and setting up takesmore time delay than conventional O2 devices.

With regard to NIV, HFNC therapy and NIV

have fundamental differences, each device havingsome advantages and limitations.23 The significantlybetter comfort and better tolerance of HFNC therapycompared with NIV, permitting nearly 24 h of daily use,are significant advantages and justify using HFNCtherapy as the first alternative if O2 supplementation isthe sole aim. The role of HFNC therapy during theweaning period is questionable with regard tophysiological rationale and evidences. Even if HFNCtherapy provides a continuous flow, pressure is low andnot continuous. The ability of HFNC therapy to reducework of breathing in patients with COPD and high levelof auto - PEEP is questionable.Most importantly,institution of HFNC should not delay intubation andmechanical ventilation, because unduly delayedmechanical ventilation has been shown to be deleteriousin multiple studies. The readers should keep in mind theuniversal problem with these kinds of studies with regardto near impossibility of blinding, introducing a majorbias.

Conclusion

Delivery of heated and humidified air-oxygengas mixture at high flow rates through HFNC isincreasingly being employed in adult patients with criticalrespiratory illness. Its mechanisms of action arephysiologically sound and potential clinical benefitsconsistently demonstrated in trials. It has the potentialto improve the management of patients with acuterespiratory failure or during the weaning phase. Withthe currently available evidence, there are prevailinguncertainties in many aspects of HFNC usage; there isstrong evidence for usage in some clinical indications,whereas the body of evidence is bleak in some others.Institution of HFNC treatment should be individualizedin each particular situation and due consideration needsto be given to local experience, expertise and resources.Notwithstanding all these shortcomings, HFNC therapyis still an innovative and powerful technique that iscurrently changing the management of patients withrespiratory failure.

References

1. Nishimura M. High-Flow Nasal Cannula OxygenTherapy in Adults: Physiological Benefits, Indication,Clinical Benefits, and Adverse Effects. Respir Care2016; 61 (4): 529-41



2. Fontanari P, Zattara-Hartmann MC, Burnet H,Jammes Y. Nasal eupnoeic inhalation of cold, dry airincreases airway resistance in asthmatic patients.Eur Respir J 1997;10(10):2250-2254

3. Salah B, Dinh Xuan AT, Fouilladieu JL, Lockhart A,Regnard J. Nasal mucociliary transport in healthysubjects is slower when breathing dry air. Eur RespirJ 1988;1(9):852-855

4. Gonzalo Hernández, Oriol Roca and Laura Colinas.High-flow nasal cannula support therapy: new insightsand improving performance. Critical Care (2017) 21:62

5. Ritchie JE, Williams AB, Gerard C, Hockey H.Evaluation of a humidified nasalhigh-flow oxygensystem, using oxygraphy, capnography andmeasurementof upper airway pressures. AnaesthIntensive Care. 2011;39:1103–10

6. Möller W, Celik G, Feng S, et al. Nasal high flow clearsanatomical dead space in upper airway models. JAppl Physiol. 2015;118:1525–32

7. Parke RL, McGuinness SP. Pressures delivered bynasal high flow oxygen during all phases of therespiratory cycle. Respir Care. 2013;58:1621–4

8. Riera J, Perez P, Cortes J, et al. Effect of high-flow nasalcannula and body position on end-expiratory lungvolume: a cohort study using electrical impedancetomography. Respir Care. 2013;58:589–96

9. Shepard Jr. JW and Burger CD. Nasal and oral flow-volume loops in normal subjects and patients withobstructive sleep apnea. Am Rev Respir Dis 1990;142(6): 1288–1293

10. Arora B, Mahajan P, Zidan MA, et al. Nasopharyngealairway pressures in bronchiolitis patients treated withhigh-flow nasal cannula oxygen therapy. PediatrEmerg Care 2012; 28: 1179–1184

11. Sztrymf B, Messika J, Bertrand F, et al. Beneficialeffects of humidified highflow nasal oxygen in criticalcare patients: a prospective pilot study.IntensiveCare Med. 2011; 37:1780–6.

12. Parke RL, McGuinness SP, Eccleston ML. Apreliminary randomizedcontrolled trial to assesseffectiveness of nasal high-flow oxygen in intensivecare patients. Respir Care. 2011; 56:265–70

13. Roca O, de Acilu MG, Caralt B, et al. Humidified highflow nasal cannula supportive therapy improvesoutcomes in lung transplant recipients readmitted

to the intensive care unit because of acuterespiratory failure.Transplantation. 2015; 99:1092–8

14. Frat JP, Thille AW, Mercat A, et al. High-flow oxygenthrough nasal cannulain acute hypoxemicrespiratory failure. N Engl J Med. 2015; 372:2185–96

15. Jaber S, Monnin M, Girard M, et al. Apnoeicoxygenation via high-flow nasal cannula oxygencombined with non-invasive ventilationpreoxygenation for intubation in hypoxaemicpatients in the intensive care unit: the single-centre,blinded, randomised controlled OPTINIV trial.Intensive Care Med 2016; 42:1877

16. Vourc'h M, Asfar P, Volteau C, et al. High-flow nasalcannula oxygen during endotracheal intubation inhypoxemic patients: a randomized controlled clinicaltrial. Intensive Care Med 2015; 41:1538.

17. Simon M, Braune S, Frings D, et al. High-flow nasalcannula oxygen versus non-invasive ventilation inpatients with acute hypoxaemic respiratory failureundergoing flexible bronchoscopy--a prospectiverandomised trial. Crit Care 2014; 18:712.

18. Maggiore SM, Idone FA, Vaschetto R, et al. Nasalhighflow versus Venturimask oxygen therapy afterextubation. Effects on oxygenation, comfort, andclinicaloutcome. Am J Respir Crit Care Med. 2014;190:282–8

19. Hernandez G, Vaquero C, Colinas L, et al. High flowconditioned oxygentherapy for prevention ofreintubation in critically il patients at high riskforextubation failure: a multicenter randomisedcontrolled trial. JAMA. 2016;316:1565–74

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Original Article

Medical Thoracoscopy :Diagnostic Utility in a Tertiary Care Setting in South India

Rennis D. K.*, Suresh Abhay A.**, Muhammed Anaz***Professor & Head, **Junior ResidentPulmonary Medicine, Amala Institute of Medical SciencesThrissur, India

Abstract

IntroductionMedical thoracoscopy is a minimally invasive procedure for inspecting the pleura, diagnosing

and treating pleural diseases, especially undiagnosed exudative pleural effusions.

ObjectiveTo analyse the results of medical thoracoscopy in our hospital over a specific time period.

Materials and MethodsA hospital record based descriptive study with respect to patients who underwent medical

thoracoscopy between 2011 and 2018 in Amala Medical College, Thrissur.

ResultsAmong the 250 patients who underwent medical thoracoscopy, malignant effusions were seen

in 88 (35.2%); Tuberculosis was seen in 68(27.2%); Chronic inflammation in 35 (14%); Empyema in 10 (4%);benign cells in 8 (3.2%); inconclusive result in 35 (14%); normal study in (2%) and Silicosis in 1 patient.

ConclusionMedical thoracoscopy is a useful procedure for the diagnosis of pleural diseases due to its

safety, shorter hospital stay and high diagnostic yield.

Key wordsMedical Thoracoscopy, Diagnostic Utility, Tuberculosis, Malignancy, South India


Introduction

Thoracoscopy is a procedure that involvesaccess to the pleural space with an endoscope andallows direct visualization of the pleural space andintrathoracic structures and aids in obtaining tissue orperforming interventions under direct visual guidance1.Pleural effusions are a frequently encountered conditionin pulmonary medicine outpatient clinics .Thoracentesisis often able to establish a diagnosis and is anappropriate first step, but how to approach undiagnosedexudative pleural effusions remains a source of debate2.The recent rapid growth in the field of interventionalpulmonology along with the evolution of sedationtechniques and development of flexirigid thoracoscopeshas brought Medical thoracosocopy (MT) to theforefront1.

Over the past two decades, medicalthoracoscopy has developed very rapidly in India, andhas gradually become the first-choice for pleural biopsyinstead of video-assisted thoracoscopic surgery(VATS).With thoracoscopy, one can visualise the entirevisceral and parietal pleura and take pleural biopsy fromsuspicious sites under vision. Larger pleural biopsyspecimen taken under direct vision allows greaterdiagnostic yield up to 90 percent. Diagnosis of pleuraltuberculosis can be achieved in 99% of patients withthoracoscopy, which is higher than the 51% yield forclosed pleural biopsy.

Similarly, yield of thoracoscopic pleural biopsyis higher in patients with suspected pleural malignancy.A diagnosis could be achieved in 95% of patients asagainst 44% patients using closed pleural biopsy. Medicalthoracoscopy can also be used for therapeuticprocedures, such as adhesiolysis and evacuation ofpleural fluid in patients with empyema, pleurodesis inpatients with malignant pleural effusion andspontaneous pneumothorax3. This study aims to explorethe diagnostic profile of this procedure, with respect topatients who underwent the same in a tertiary carehospital setting.

Objective

To analyse the results of medical thoracoscopyfor a variety of pleural diseases in a tertiary carehospital setting and to explore the diagnostic profile,

Rennis D.K. - Medical Thoracoscopy : Diagnostic Utility in a Tertiary Care Setting in South India

safety and complications of this procedure.

Materials and Methods

Conceived as a hospital record baseddescriptive study of the medical thoracoscopies doneduring the period 2011-2018, all patients with pleuraldiseases who underwent medical thoracoscopyduring this study period in Amala Medical Collegewere included in the study. Descriptive statisticalmethods were used in the data analysis (mean,Standard Deviation etc.). Being a record based study,permission of the gatekeeper of information wasobtained and patient identifiers were not extracted.The diagnosis of pleural disease was established bythe presence of the positive findings of respectivediseases in pleural biopsy. All patients underwentdetailed clinical evaluation with history and clinicalexamination. Computed tomography (CT) of the chestwas performed when necessary to assess feasibilityof thoracoscopy.All patients undergoing thoracoscopywere investigated with complete blood count includingprothrombin time (PT), activated partialthromboplastin time (aPTT) and platelet count to ruleout bleeding diathesis. Patients with platelet count lessthan 50,000/mm3and those with PT oraPTT prolongedby more than four seconds above control were notsubjected to thoracoscopy. Patients were kept fastingfor 4 hours prior to the procedure. Vascular accesswas achieved with intravenous cannula inserted inthe upper limb opposite to the side of thoracoscopy.Patients were positioned in lateral decubitus withdiseased side up and with the arm on the side ofthoracoscopy positioned above the patient’s head.This allowed better access and widens the intercostalspaces. Thoracoscopy was conducted under conscioussedation with intravenous midazolam, small doses ofpentazocine(for pain relief) and promethazine.Theskin, subcutaneous tissue, intercostal muscle andparietal pleura were anesthetised with 2% lignocaine.A single port was used for visualising and takingpleural biopsy or for sampling of pleural fluid. A 1.5cmto 2cm long skin incision along the line of intercostalspace was given in 4th or 5th intercostal space in mid-axillary line (Triangle of safety) using sterile surgicalblade. After blunt dissection of subcutaneous tissueand the intercostal muscles with curved arteryforceps, a cannula of 10mm diameter with blunt trocaris inserted into the pleural cavity. The trocar was then



replaced with semi flexible scope. Pleural fluid wassuctioned to enable clear visualization of entirepleural surface. Thoracoscope was manoeuvered tosee visceral, costal, diaphragmatic pleura as well asthe costophrenic recess. Adhesions were gently lysedusing thoracoscope or biopsy forceps to allow bettervisualizations of pleura.After selecting suitable siteon parietal pleura for biopsy, biopsy forceps wasintroduced through working channel of thethoracoscope. The pleura was grasped under visionand biopsy was taken with a lateral movement of thethoracoscope. After the procedure was completed,thoracoscope and the cannula were removed and a28 to 32 Fr chest tube was inserted. Chest drain wasconnected to an underwater-seal. Once the lung hadexpanded and drain output had decreased to less than150mL over 48hours, chest drain was removed. Formalignant pleural effusions(as per histopathologicalreport) pleurodesis is achieved by instilling 2-4 gm oftalc through the intercostal drain and clamped for 12hours before removal.

Results

A total of 250 patients (62% males; 38%females) underwent medical thoracoscopy. The meanage of the study sample was 57.6yrs. These patientshad a variety of pleural diseases varying fromundiagnosed pleural effusions (240), empyemas (10)to persistent air leaks (3). Out of the 250 patients whounderwent the procedure, 88 (35.2%) had malignantpleural effusion (see Table 1). Out of these 42 (47.78%)were found to have Adenocarcinoma; 17 (19.31%)Metastasis; 10 (11.36%) Mesothelioma; 6(6.81%) poorlydifferentiated carcinoma; 5(5.6%) Small cell carcinomaand 4(4.54%) were reported as Non Small CellCarcinoma after pathological diagnosis. Only 2 patientswere diagnosed with Squamous Cell Carcinoma andthere were just one case each of lymphoma andsarcoma. Relevant cases underwent further Immunohistochemistry typing for planning of treatmentpurposes.

A diagnosis of Tuberculosis as evidenced bygranulomas on biopsy was seen in 68 (27.2%) patients.Out of those who were diagnosed with Tuberculosis bybiopsy, 44 patients had high clinical suspicion.

The number of mesotheliomas diagnosed bythoracoscopy was 10. The diagnosis of Silicosis wasobtained in 1 patient.

10 (4%) patients had Empyema, chronicinflammation was seen in 35 (14%), an inconclusive resultwas seen in 35 (14%) possibly due to transudative natureof pleural effusion screened to look for any additionalpathology. 5 (2%) patients had a normal histopathologicalreport. 8 (3.2%) patients had a pathology report of benignreactive cells and 1 patient had silicotic noduleconfirming silicosis.

Chronic inflammation, a normal pleura andbenign reactive cells were seen in patients withpersistent air leak, in patients with recurrentpneumothorax and in some patients with exudativepleural fluid picture where no cause could bedetermined. It should be noted that some of thesepatients had an underlying chronic kidney disease orcoronary heart disease, but evaluation of its significanceis beyond the scope of this study.Fig 1 :


Table - 1 : Diagnosis after hisptopathology among the 250 patients who underwent thoracoscopy

Tuberculosis 68 27%

Malignancy 88 35% %(95%CI)

Adenocarcinoma 42 48%(37.5-58.4)

Squamous cell Carcinoma 2 2%(0-4.9)

Small Cell Carcinoma 5 6%(1.03-10.9)

Lymphoma 1 1% (0-3.07)

Sarcoma 1 1%(0-3.07)

Mesothelioma 10 11%(4.46-17.5)

Poorly Diff Carcinoma 6 7%(1.66-12.3)

NSCLC 4 5%(0.44-9.55)

Metastasis from other organs 17 19%(10.8-27.1)

Silicosis 1

Empyema 10 4%

Inconclusive 35 14%

Normal 5 2%

Benign reactive cells 8 3%

Chronic Inflammation 35 14%

Confirmed Diagnosis Number (n - 250)


Table - 2 : Table 2. Pleural fluid cytologyof patients who had positive results

A haemorrhagic appearance was seen in 42patients (17.5%) straw coloured in 190 patients (79.16%)while pus was obtained in 8 patients. Among the patientswith haemorrhagic effusion 41 (97.6%) were diagnosedwith malignancy.

Pleural fluid cytology was able to diagnose TBsuccessfully in 37 patients with thoracoscopy confirmedresults (see Table 2). In thoracoscopy confirmedmalignancy only 12 patients got a successful positiveresult. This is possibly due to presence of paramalignanteffusions. Atypical cell clusters were seen in patientswith malignancy and in those with inconclusive biopsyresults. Eosinophilic effusion was seen in a patient withNon small Cell Lung Cancer.

Discussion

Medical thoracoscopy is a procedure

Lymphocytic effusion s/o TB 37

Malignancy 12

Inflammatory cells 5

Atypical Cell clusters 7

Eosinophilic effusion 1

NumberPleural Fluid Cytology



increasingly being used in India. Since the prognosisfor patients with malignant pleural effusion is poor, anefficacious procedure that can establish a definitediagnosis as early as possible with a minimum risk anddiscomfort would be highly desirable. If a patient withundiagnosed pleural effusion is suspected as malignant,cytologic examination of pleural fluid is the first choiceof investigation. Although repeated thoracentesis canenhance the sensitivity of cytology, it is usually onlyupto 50 to 70%. CT or ultrasound-guided pleural biopsiesare quite sensitive and safe, with the only reportedcomplications being local hematoma and minorhemoptysis. The limitation of the image guided pleuralbiopsy is the blindness of the procedure and chances ofiatrogenic pneumothorax4.

Closed percutaneous needle biopsy wastraditionally performed blindly by an Abrams or cope’sneedle, but its diagnostic yield is only 50%5,6. The medicalthoracoscopies are useful in this context, and help in apositive diagnosis in 86% of cases (215/250). There wasa yield of 35.2% with respect to malignancy. These resultsare corresponding to the results obtained by Wu et al(41%)4 which is a larger scale similar study conducted inChina.

With respect to the morphology, a wide patternwas seen including large nodules, small sago grain likegranular nodules, septations, adhesions, plaques,pleural hyperemia and thickening. Large nodular lesionsappear to be more in favour of a malignant etiology (68/88), while sago grain/granular nodules seemed to favoura diagnosis of tuberculosis (23/68). India is a regionprevalent in tuberculosis, which also explains its highyield 27.2%. The patients with tuberculosis also werefound to have septations, adhesions, loculations andsome amount of hyperemia.

It has been reported that carcinoma from anyorgan can metastasize to the pleura, but lung and breastcarcinomas and lymphomas are the most commoncauses, digestive and ovary carcinomas are lessfrequent. Our study confirms that the most commonsites of metastasis is from the lung. Other sourcesinclude breast, lymphoma, sarcoma and colorectalcancers. Primary pleural malignancy was seen in only10 patients. It was noted that no etiological causes ofpleural effusions could be identified in 14%. This wassimilar to the results reported by Wu et al (7.4%)4. The

patients with inconclusive results after this procedurefurther underwent bronchoscopy (n=22) or CT guidedbiopsy (n=2) for diagnosis. There was a suspicion ofintraluminal extension of mass or peripheral nodulesby CT scan in these cases.

With respect to complications post procedure,1 patient developed bronchopleural fistula, 1 patientdeveloped seizure unrelated to procedure and 1 patientdeveloped hypoxia which responded to oxygen therapy.No serious adverse events were observed, and transientchest pain induced by the indwelling chest tube was themost frequent minor complication. Complications likebleeding, subcutaneous emphysema or post procedureinfection were not seen though described in variousother studies.VATS had to be done in 4 patients postthoracoscopy for bullae excision or bronchopleuralfistula.

Medical thoracoscopy has an excellent safetyprofile when performed by a trained physician. Mortalityrate associated with medical thoracoscopy is about 0.8%7.The strength of this study is that, it is a large scale studyin South India which examines the efficacy of a relativelynew procedure.At the same time, no longterm follow upof patients was done, which can be taken as a limitationof this study. Though we had data of patients with bothbiopsy proven tuberculosis and malignant etiologies ascauses for effusion, their pleural fluid analysis were notalways done at our centre in ~20% of patients prior toprocedure. Before undergoing thoracoscopy, blindneedle biopsies or image assisted biopsies wereperformed only in a few patients, due to financialconstraints. Moreover this study included data from onlya single pulmonologist of the same centre due tofeasibility issues.

Conclusion

In India, a large number of pleural diseasesexist of which malignancy and tuberculosis still appearto be the most common. Since medical thoracoscopy isa safe, easy to perform procedure with a very high yield,this procedure should be performed more actively inall cases where non invasive techniques fail to reach adiagnosis.



Acknowledgements

The Authors would like to acknowledge theguidance and help received from Dr. Sanjeev Nair,Associate Professor, Department of Pulmonary Medicine,Government Medical College, Thiruvananthapuram &Dr. Hisham Moosan, Technical Expert - Epidemiology,Regional Technical Resource Centre for HTA, AchuthaMenon Centre for Health Science Studies, Sree ChitraTirunal Institute for Medical Sciences and Technology,Trivandrum, in analysis & drafting of the manuscript ofthis article.

References

1. Alraiyes AH, Dhillon SS, Harris K, Kaphle U, Kheir F.Medical Thoracoscopy. PLEURA [Internet]. 2016 May6 ; 3:237399751663275. Available from: http://journals.sagepub.com/doi/10.1177/2373997516632752

2. Murthy V, Bessich JL. Medical thoracoscopy and itsevolving role in the diagnosis and treatment of pleuraldisease. J Thorac Dis [Internet]. 2017 Sep; 9(Suppl10):S1011–21. Available from: http://www.ncbi.nlm.nih.gov/pubmed/29214061

3. Agarwal A, Gupta D, Jindal SK, Mootha VK, AgarwalR, Singh N, et al. Medical thoracoscopy forundiagnosed pleural effusions: Experience from atertiary care hospital in North India. 2010; Available

from: https://www.researchgate.net/publication/50905855

4. Wu Y-B, Xu L-L, Wang X-J, Wang Z, Zhang J, Tong Z-H, et al. Diagnostic value of medical thoracoscopy inmalignant pleural effusion. BMC Pulm Med [Internet].2017 Dec 4; 17(1):109. Available from: http://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-017-0451-1

5. Mungall IP, Cowen PN, Cooke NT, Roach TC, CookeNJ. Multiple pleural biopsy with the Abrams needle.Thorax [Internet]. 1980 Aug; 35(8):600–2. Availablefrom: http://www.ncbi.nlm.nih.gov/pubmed/7444827

6. Prakash UBS, Reiman HM. Comparison of NeedleBiopsy With Cytologic Analysis for the Evaluation ofPleural Effusion: Analysis of 414 Cases. Mayo ClinProc [Internet]. 1985 Mar; 60(3):158-64. Available from:http:/ / l inkinghub.elsevier.com/retr ieve/pi i /S0025619612602122

7. Marel M, Štastny B, Melinová L, Svandová E, LightRW. Diagnosis of Pleural Effusions. Chest [Internet].1995 Jun 1; 107(6):1598–603. Available from: http://l i n k i n g h u b . e l s e v i e r . c o m / r e t r i e v e / p i i /S0012369216357816


A 79 year old female, presented with persistentnon pleuritic, non cardiac central chest pain, chronicdry cough and MMRC grade 2 dyspnea on exertion forthe last 6 months.

Respiratory examination was normal.

Relevant images of her digital Xray of chestand contrast enhanced CT scan of thorax given belowwas diagnostic.

What is the diagnosis?

Fig 1 :

Radiology Pearl

Venu Gopal P.*, Athul Thulasi**, Shajahan P.S.***, Bindu C.G.*****Professor & HOD, **Senior Resident, ***Associate Professor, ****Assistant ProfessorPulmonary Medicine, Government TD Medical College, Alappuzha

Fig 2 :

Fig 3 :


Venu Gopal P. - Radiology Pearl

Answer: Hiatus Hernia (Sliding – Type I)

The chest X-ray shows a retrocardiac shadowsuggestive of an esophageal abnormality. The CT Scanshows widening of esophageal hiatus with gastric rugalfolds extending above the level of gastro esophagealjunction. This finding is diagnostic of hiatus hernia. Thediagnosis was confirmed by endoscopy.

Discussion

Hiatus hernia is a condition in which contentsof the abdominal cavity, most commonly the stomachherniates through a widening of the right crus of thediaphragm into the mediastinum. Among the threeopenings in the diaphragm -caval, aortic and esophageal,the esophageal is the weakest, and therefore, the mostsusceptible to visceral herniation1.

Prevalence of hiatus hernia has been reportedto be between 10 to 50% of the population2, the exactprevalence is difficult to determine. Incidence increaseswith advance in age and this may be explained by thedecrease in collagen fibers with increasing age. Otherrisk factors include central obesity, previousgastroesophageal surgery (antireflux procedures,esophagomyotomy, partial gastrectomy), thoracoabdominal trauma, skeletal deformities and congenitalconditions such as scoliosis, kyphosis, and pectusexcavatum3. Hiatus hernias can occur congenitally alsoin children, and may be associated with other

malformations, such as intestinal malrotation4.

There are four types of esophageal hiatalhernias: sliding (type I), paraesophageal (type II),combined (type III), which include elements of types Iand II, and giant paraesophageal (type IV)5.

Sliding hernias are the commonest, accountingfor more than 85%. The common symptoms of a slidinghernia are of gastroesophageal reflux, esophagitis andesophageal ulceration.

Paraesophageal hiatal hernias (type II) arepredominantly seen in adults older than 40 years. Unlikein sliding hernia, here the gastroesophageal junctionremains in its normal position. So the dilated stomachwhich lies between the pericardium and the spine,compresses the esophagus and prevents gastric reflux. Because of this mechanism, gastroesophageal refluxsymptoms are rare in paraesophageal herniapatients.The main complaints being postprandialfullness, palpitation, shortness of breath, pain, anddysphagia. Paraesophageal herniation may causeserious complications like gastric volvulus andinfarction.

Mixed hiatal hernias (type III) are acombination of sliding and paraesophageal hiatalhernias and so may have symptoms of both.

Giant paraesophageal hiatal hernias (type IV)are those which include more than one-third to one-halfof the stomach6. They commonly include herniation ofupper abdominal organs into the thorax, including colon,omentum, small intestine, liver and even spleen. Theircharacteristic symptoms include GERD and irondeficiency anemia due to chronic gastric bleeding7.

Most of the hiatus hernias are asymptomatic.Many patients with hiatus hernia first present to apulmonologist, because of symptoms like chest pain,persistent cough or unexplained dyspnea. As thestomach moves into the chest, respiratory symptomsmay predominate as a result of lung compression anddecreased forced vital capacity (FVC). Large hiatalhernias are often visible in routine chest radiograph asa retrocardiac opacity8. Presence of retrocardiac aircorresponding to the gastric bubble suggest thediagnosis.

Fig 3 :


Venu Gopal P. - Radiology Pearl

Computed tomography helps to demonstratethe widened esophageal hiatus, hiatal size and content,the orientation of the stomach, and the location ofhernia.The characteristic CT features of a sliding hiatushernia include dehiscence of the diaphragmatic crura,evident as widening of esophageal hiatus on crosssection (normal value being less than 15 mm) andprojection of a part of stomach into the mediastinum9.These findings are visualized in the CT of the citedpatient (Fig 2 & 3).

Diagnosis is established with the use of bariumswallow contrast chest radiographs, in which, hiatalhernias appear as a shadow in the posteriormediastinum. Upper gastro intestinal endoscopy alsohelps in diagnosis. High resolution manometry and refluxmonitoring is done to decide the mode of treatment.Radiological differential diagnoses of hiatus herniainclude Bochdalek diaphragmatic hernia andbronchogenic cysts.

Treatment

Treatment is not indicated for asymptomaticsliding hernias. Para esophageal hernias might needinterference due to fear of potential complications likeobstruction. Medical management of GERD is thecornerstone for treatment of hiatus hernia. This includesproton pump inhibitors (PPIs), histamine 2 receptorantagonists and antacids. Surgical treatment consistsof restoring the stomach into the abdominal cavity bylaparoscopic fundoplication10.

References

1. Drake RL, Vogl AW, Mitchell AWM (2010) Gray’sanatomy for students, 2nd edn. Churchill Livingstone,Philadelphia, p 352

2. Chase Dean, Denzil Etienne, Bianca Carpentier, JerzyGielecki, Shane Tubbs R, Marios Loukas:

3. Sabine Roman, Peter J Kahrilas.The diagnosis andmanagement of hiatus hernia. BMJ 2014;349:g6154doi: 10.1136/bmj.g6154

4. Chandrasekar S, Welch RJ, Watson H (2006)Congenital mixed hiatus hernia in a neonate. ArchDis Child Fetal Neonatal 91:F317

5. Jong Jin Hyun and Young-Tae Bak. Clinical Significance of Hiatal Hernia. Gut and Liver, Vol. 5, No. 3,September 2011, pp. 267-277

6. Maziak DE, Todd TR, Pearson FG (1998) Massive hiatushernia: evaluation and surgical management. JThorac Cardiovasc Surg 115:53–60

7. Sinaki B, Jayabose S, Sandoval C (2010) Iron-defciency anemia associated with hiatal hernia: casereports and literature review. Clin Pediatr 49:984–985

8. Samujh R, Kumar D, Rao KL (2004) Paraesophagealhernia in the neonatal period: suspicion on chest X-ray. Indian Pediatr 41:189–191

9. W Richard Webb,Charles b Higgins. ThoracicImaging: Pulmonary and Cardiovascular radiology.Second edition. Lippincot Williams & Wilkins, 2005, p272

10. Morrow EH, Oelschlager BK. Laparoscopicparaesophageal hernia repair. Surg Laparosc EndoscPercutan Tech 2013;23:446-8.


Self Assessment Quiz

Vishnu Sharma M.Professor and Head, Dept. of Respiratory Medicine,A J Institute of medical sciences, MangaloreKarnataka, 575 004

Fig 1 : CT scan thorax

Question 1 :

This 72 year old man was admitted with chroniccough . What is the most likely diagnosis from the aboveimage?

1. Bronchogenic carcinoma

2. Thymic cyst

3. Bronchogenic cyst

4. Aneurysm involving arch of aorta and descendingthoracic aorta

5. Posterior mediastinal tumor

Answer : 4

Contrast enhanced CT scan thorax shows

aneurysm involving arch of aorta and descendingthoracic aorta

Question 2

Which is a WRONG statement about aorticaneurysm?

1. More common in elderly males

2. Smokers have higher risk to develop aneurysm

3. Uncontrolled hypertension is a risk factor

4. May be associated with abdominal aortic aneurysm

5. All aortic aneurysms once diagnosed should betreated by surgery

Answer : 5

Small asymptomatic aortic aneurysms lessthan 5.5cm in diameter without any risk factors can bemanaged conservatively1. Medical managementincludes proper control of blood pressure and treatmentof hyperlipidemia. Smoking cessation in smokers,proper treatment of infection in cases where aneurysmis due to infection (mycotic aneurysm) is essential. Sizeof the aneurysm should be regularly monitored by CTscan or MRI scans every six months in these patients.

Question 3 :

Which of the following is LEAST LIKELY causefor descending thoracic aortic aneurysm?

1. Ehlers-Danlos syndrome type IV

2. Marfan’s syndrome


Vishnu Sharma M. - Self Assessment Quiz

3. Genetic (Family history of thoracic aortic aneurysm)

4. Aortitis

5. Tertiary syphilis

Answer : 5

Except syphilis all of the above conditions canlead to descending thoracic aortic aneurysm. In somefamilies, there appears to be an autosomal dominanttrait causing thoracic aortic aneurysm2. Syphilitic aorticaneurysm almost always involves the ascending aortaor aortic root3. Syphilis does not cause descendingthoracic aortic aneurysm. The prevalence of tertiarysyphilis is now very low.

Question 4 :

Which is a rare symptom in thoracic aorticaneurysm?

1. Chronic cough

2. Hemoptysis

3. Chest pain

4. Hoarseness of voice

5. Back pain

Answer : 2

If the aneurysm erodes into a bronchus leadingto aorto-bronchial fistula hemoptysis can occur3. This isvery rare and can be fatal. Compression over a bronchuscan lead to chronic cough. Acute severe chest pain is asymptom in acute aortic dissection. Compression ofrecurrent laryngeal nerve can lead to hoarseness ofvoice. Constant pressure and compression of vertebralbody can lead to erosion of vertebral body causing backpain4.

Question 5 :

Which of the following is NOT a pressuresymptom in descending thoracic aortic aneurysm?

1) Hoarseness of voice

2) Dysphagia

3) Raised left dome

4) Dyspnea

5) Superior vena cava obstruction

Answer : 5

Superior vena cava is on right side where asdescending thoracic aorta is on left side. Hencedescending thoracic aortic aneurysm can’t compresssuperior vena cava4.Compression of phrenic nerve canlead to raised left dome due to phrenic nerve palsy onleft side. Dyspnea can occur due to compression of leftmain bronchus and or phrenic nerve palsy due tocompression of left phrenic nerve. Dysphagia can occurdue to compression over the esophagus.

Question 6 :

Which of the following is NOT considered asan indication for surgery in aortic aneurysm?

1. Acute symptoms due to aortic aneurysm

2. Age less than 60 years

3. Pressure symptoms due to aortic aneurysm

4. Aneurysm growth rate 0.5 centimeters over aperiod of six months to one year

5. Presence of genetic disorders or familial history ofthoracic aneurysms

Answer : 2

Age is not considered as an indication forsurgery4. Except this, all of the above require surgerybecause there is a higher risk for aortic rupture ordissection4.

References

1. Booher AM, Eagle KA; Diagnosis and managementissues in thoracic aortic aneurysm. Am Heart J. 2011Jul;162(1):38-46.e1. Epub 2011 Jun 15.

2. Aortic Aneurysm, Familial Thoracic 2, AAT2 ; OnlineMendelian Inheritance in Man (OMIM)

3. Jackman JD, Radolf JD. Cardiovascular syphilis, AmJ Med, 1989, vol. 87. 425- 433

4. Thoracic aortic aneurysm: reading the enemy'splaybook. Yale J Biol Med. 2008 Dec;81(4):175-86.


Case Report

Thoracic Ewings Sarcoma with Right Atrial Invasion -A Case Report

Smitha Nair P.G.*, Rajani M.**, Manoj D.K. ****Junior Resident, **Professor, ***Professor & HODDepartment of Respiratory Medicine, Pariyaram Medical College.

Corresponding Author :Dr. Rajani M.Professor, Department of Respiratory MedicinePariyaram Medical College.Phone Nubmer : 0497 2800662Email ID : [email protected]

AbstractA 28 year old male manual labourer, presented with progressive breathlessness for 1 month,

cough and abdominal pain for 2 weeks. Clinical picture suggestive of right sided pleural effusion withcollapse. Chest X-ray showed right mediastinal mass with pleural effusion. Echocardiography suggestiveof right atrial mass protruding to right ventricle with pericardial effusion. Computed tomography (CT)thorax showed large lobulated heterogeneously enhancing lesion in anterior mediastinum extendingupto the upper mediastinum and left pulmonic recess with mild pleural effusion. Lesion was encasingand compressing the right pulmonary artery. There was also invasion into right atrium and ventriclewith pericardial effusion. Ultrasonography guided fine needle aspiration cytology was suggestive ofsmall round cell tumor, possibly of lymphoma. On immunohistochemistry of the biopsy specimen,tumor cells expressed CD99 and vimentin positivity consistent with the diagnosis of primitiveneuroectodermal tumor (PNET).

Key wordsPleural effusion, mediastinal mass, immunohistochemistry, primitive neuroectodermal tumour

Introduction

Primitive neuroectodermal tumor (PNET)represents a family of tumors with varying degrees ofneuronal differentiation, presenting as a bone or soft

tissue mass in the trunk or axial skeleton in adolescentsand young adults1. Peripheral Primitive neuroectodermal tumor (pPNET) are the rarest class of PNETbelonging to the Ewing’s or Askin’s family of tumors.They are highly aggressive and have an average life


expectancy of 8 months4 Intrapulmonary PNET is a veryrare entity. PNET is a highly malignant neoplasm and itis composed of small, round, uniform cells3. Diagnosisof the tumor is confirmed using various immunohistochemical studies and detecting the presence of atranslocation, t(11;22) through fluorescent in situhybridization (FISH)4. PNET can be treated with variouscombinations of radical surgical resection, neoadjuvantand adjuvant chemotherapy, and radiation5.

A 28-year-old male manual laborer presentedwith progressive breathlessness of one month durationand scanty mucoid expectoration with tinge of bloodstreaks on few occasions for the last 2 weeks. He alsocomplained of generalized abdominal pain for 2 weeks.He had loss of appetite and unintentional weight losswhich was not quantified. He was a smoker with smokingindex of 65 and a alcoholic. No significant family historieswere obtained. On general examination he was notedto have elevated non pulsatile JVP with facial puffinessand bipedal edema. On chest examination, trachea wasshifted to the right side. Apex beat was not palpable.Chest movements were decreased in right hemithorax.Percussion note was impaired anteriorly from secondintercostal space and stony dull on right infra axillaryand infra scapular area. On auscultation, tubular breathsounds was heard in the right infra clavicular area. Vocalresonance was diminished in all areas except right infra

Fig. 1 : X-RAY CHEST

Smitha Nair P.G. - Thoracic Ewings Sarcoma with Right Atrial Invasion - A Case Report

clavicular area on the right side. Heart sounds weremuffled. Examination of other systems includinglymphoreticular system and testicular examination wereall within normal limits. The clinical picture wassuggestive of right sided upper lobe mass with effusion.Chest X-ray postero anterior view revealed ahomogenous opacity with broad base towards themediastinum at the upper, mid and lower zones of rightlung field with distinct superior, lateral and inferiorborders. Lesion was forming an obtuse angle with thelung margin. There was moderate ipsilateral pleuraleffusion.

Pleural fluid aspiration under USG guidanceyielded 250 ml of hemorrhagic fluid which wasneutrophilic exudate. (Total cell count – 500/cu.mm,polymorph – 88%, lymphocyte - 12%, protein 4.5g/dL,sugar – 166mg/dL ADA – 20IU/L, LDH – 890 IU/L).Cytology was negative for malignant cells. Aconcomitant screening echo done at that time showeda right atrial mass protruding into right ventricle.Cardiology opinion with proper echocardiogram wasdone which reported large right atrial mass protrudingto right ventricle (78 x 48 mm), moderate to largepericardial effusion, fair left ventricular systolic functionand bilateral pleural effusion.

Fig. 2 :

An emergency contrast enhanced CT (CECT)was done which showed large lobulatedheterogeneously enhancing coalescent lesion seen inthe right hilum extending up to the upper mediastinumand left pulmonic recess. Lesion was encasing andcompressing the right pulmonary artery. There was alsoinvasion into right atrium & ventricle. Lesion wasabutting and compressing the carina, bilateral mainbronchus and bilateral superior pulmonary veins.



Heterogeneously enhancing pleural based nodularlesions morphologically like the mediastinal lesion werenoted. Mild pleural effusion was present. Few tiny calcificfoci were noted within.

Fig. 3 : CECT Thorax

No acid fast bacillus was found in sputum smearmicroscopy. Hematological evaluation showedmarkedly elevated LDH with normal beta HCG and AFP.USG abdomen revealed no significant abnormalities.USG guided FNAC of the mass was suggestive of smallround cell tumor, possibly lymphoma. CT guided truecut biopsy of the lesion was performed which showedfragments of cellular neoplasm with small round cellsarranged in sheets and in perivascular pattern andscattered singly, moderate to scanty cytoplasm withmild to moderate nuclear pleomorphism.

Fig. 4 :

On immunohistochemistry the tumor cellsexpressed CD 99 strong diffuse positivity and vimentinpositive and immuno-negative for leukocyte commonantigen and EMA/CK. The findings were consistent with

primitive neuroectodermal tumor.(Fig.4). CT brain wasthen done which was within normal limits.Patient wasreferred to the oncology department. Relatives refusedfurther treatment and patient died after one month ofpresentation.

Discussion

PNET is a highly malignant tumor arising fromthe germinal matrix cells of primitive neural tube. It hasa very poor prognosis. PNET most commonly arisesfrom soft tissue and bone with case reports of it arisingfrom intra thoracic region being rare. The most commonsite of origin from intrathoracic region is chestwall6.Peripheral PNET are small round cell malignantneoplasm of neuroectodermal origin and are membersof ES/PNET family of tumors. Primary pulmonary PNETare very rare with few case reported till date. Review ofliterature have shown that it occurs more commonly inmales with a ratio of 1.8:1, with mean age of 28.2 (8-56years)7. Fever, cough, dyspnea, hemoptysis and chestpain were noted to be the most common presentingcomplaints.The differential diagnosis of PNET of lunginclude small cell carcinoma and other small round celltumors such as malignant lymphoma,Langerhans cellhistiocytosis, granulocytic sarcoma, rhabdomyosarcoma,neuroblastoma and synovialsarcoma.3

The diagnosis of PNET is facilitated byhistological and IHC markers and antibodies like O13,HBA-T1 and 12E7 that recognize the cell surface antigensdefined by CD998. In imaging studies,primary PNETpresents as circumscribed solitary mass withheterogenous appearance on both contrast and non-contrast CT. Intra lesion calcification or ipsilateral pleuraleffusion have been demonstrated.Very infrequently themass is shown to have invaded the adjacent structures.9

Chromosomal arrangements such asdemonstration of the t(11;22) (q24;q12) chromosomaltranslocation (EWS-FLI1 gene rearrangement) is highlyspecific for ES/PNET as it is encountered in more than90% of the neoplasms10,11.

The treatment of choice for these tumors arevarious combinations of radical surgical resection,neoadjuvant and adjuvant chemotherapy and radiation.12



This case shows the importance of consideringa differential diagnosis of primary PNET in personspresenting with mediastinal mass. Once diagnosed thetreatment options are different from others as PNETsare more chemo sensitive. A retrospective study of 24patients with extra skeletal (ES) PNET showed an overallfive-year survival rate of 61%. Wide excision with tumorfree margins in conjunction with multi-agentchemotherapy are necessary for good clinicaloutcomes.13

Extra skeletal ES/PNET should be consideredin the differential diagnosis of primary mediastinalneoplasms especially in children or young adults.Primary mediastinal PNETs are rare and usually locatedin the posterior mediastinum. However they can alsobe found in the anterior mediastinum. Histopathologicalconfirmation is mandatory especially in cases of unusuallocation.

We could not find in literature such apresentation of Ewing’s sarcoma with cardiac invasion13,14.

References

1. Thyavihally Y.B, Tongaonkar H.B, Gupta.S et al.,“Primitive neuroectodermal tumor of the kidney: asingle institute series of16patients,” Urology,vol.71,no.2,pp.292–296,2008.

2. Ganick DJ, Silverman JF, Holbrook CT, et al. Clinicalutility of fine needle aspiration in the diagnosis andmanagement of neuroblastoma. Med PediatrOncol1988;16:101-6.

3. Tsuji. S, Hisaoka. M, Morimitsu. Y et al., “Peripheralprimitive neuroectodermaltumour of the lung:report of two cases,” Histopathology, vol.33, no.4,pp.369-374,1998.

4. Cambruzzi. E, Guerra E.E, Hilgert. H. C et al.“Primitive neuroectodermal tumor of the liver :acasereport, ”CaseReports in Medicine, vol.2011,Article ID748194,4pages,2011.

5. Weissferdt A., Moran C. A., “Primary PulmonaryPrimitive Neuroectodermal Tumor (PNET): aclinicopathological and immunohistochemical study

of six cases,” Lung, vol. 190, pp. 677–683,2012.

6. Askin F.B, Rosai J, Sibley R.K, Dehner L.P, McAlisterW.H, “Malignant small cell tumor of thethoracopulmonary region in childhood. A distinctiveclinicopathologic entity of uncertain histogenesis,”Cancer, vol. 43, no. 6, pp. 2438–2451, 1979.

7. Imamura F, Funakoshi T, Nakamura S.I, Mano M,Kodama K, Horai T, “Primary primitiveneuroectodermal tumor of the lung: report of twocases,” Lung Cancer, vol. 27, no.1,pp.55–60,2000.

8. Stephenson C.F, Bridge J.A, Sandberg A.A,“Cytogenetic andpathologicanalysis of Ewing’ssarcomaandn euroectodermal tumors, ”HumanPathology, vol.23, pp.1270–1277,1992.

9. Stephenson C.F, Bridge J.A, Sandberg A.A: Primaryextraosseous Ewing sarcoma of the lung: Casereport and literature review, Radiology CaseReports, Volume 8, Issue 2, 2013.

10. Ushigome S, Machinami R, Sorensen PH. Ewing'ssarcoma primitive neuroectodermaltumour (PNET).In: Fletcher CDM, Unni KK, Mertens F, editors. WHOClassification of Tumours. Pathology and Genetics.Tumours of Soft Tissue and Bone. Lyon: IARC Press;2002. p. 298-300.

11. Folpe AL, Goldblum JR, Rubin BR, Shehata BM, LiuW, Dei Tos AP, et al. Morphologic andimmunophenotypic diversity in Ewing familytumors: A study of 66 genetically confirmed cases.Am J SurgPathol 2005;29:1025-33

12. Applebaum M.A, Worch J, Goldsby R et al., “Clinicalfeatures and outcomes in patients with extraskeletalEwingsarcoma,” Cancer,vol.117,pp.3027–3032,2011.

13. Sudipta Pandit et al. A rare mediastinal tumour in ayoung male mimicking massive pleural effusion inlung india 2012 edition volume 29 p 66-69

14. Kubra ErolKalkan, etal Thoracic PrimitiveNeuroectodermalTumor: An Unusual Case andLiterature Review Case Reports in PulmonologyVolume 2013, Article ID 326871, 4 pages


Case Report

An Unusual Bronchiectasis of Azygos Lobe

Kesavan Nair V.*, Smitha Sarvade***Professor & Senior Consultant, **ResidentDept. of Respiratory MedicineKerala Institute of Medical Sciences (KIMS)Thiruvananthapuram - 29

Corresponding Author - Dr. V. Kesavan Nair

Abstract

We are reporting a rare case of Bronchiectasis affecting the Azygos lobe. Though the patienthad bronchiectasis affecting the Right Middle lobe and Left lower lobe, this rare entity which can createconfusion regarding the diagnosis if CT thorax is not done with the problems it can produce is discussed.The typical appearance of bronchiectasis of the azygos lobe is clear from the CT Thorax.

Key words

Azygos lobe, bronchiectasis, Paratracheal opacity, CT Thorax

Case Report

A 35 year old,housewife, presented with feverand cough of 1 week duration. Fever was mild with nochills and rigors.The cough was associated with copiousamounts of mucopurulent sputum. Cough and sputumincreased in supine position. No h/o haemoptysis.Cough was associated with wheezing. Dyspnoea onexertion was present. No h/o orthopnoea /PND or chestpain.

She had recurrent episodes of cough withmucopurulent sputum and wheezing, for 10 years whichused to subside with medications and inhalers. No h/o

Pulmonary TB / sinusitis / discharge from ears. Noprevious h/o of hospitalisations.

Examination showed a tall ,thin female whowas pale with no icterus /cyanosis / clubbing /pedaloedema or lymphadenopathy. She had tachypnoea ,notachycardia and no tracheo mediastinal shift. Coarsecrepitations were present in Lt infra axillary , mammaryareas and Rt mammary and Rt infraclavicular areas.Wheezes were heard bilaterally .

Her chest Xray showed a right paratrachealopacity and the CT Thorax showed bronchiectasisaffecting left lower lobe, right middle lobe andsurprisingly of an azygos lobe.


Kesavan Nair V. - An Unusual Bronchiectasis of Azygos Lobe

The presence of bronchiectasis in azygoslobe is extremely rare and hence this case report.

Azygos lobe is a normal anatomical variant ofright upper lobe of lung, and is very rare with a CXRprevalence 0.4% and 1.2% on HRCT 2.

Embryology

Azygos lobe occurs when right posteriorcardinal vein, one of the precursors of azygos vein, failsto migrate over the apex of the lung and penetrates thelung, carrying along pleural layers that entrap a portionof the right upper lobe2.

Clinical importance of azygos lobe

It can be a cause of recurrent haemoptysis, itcan lead to bleeding during thoracic surgery.

It can produce an ‘empty azygos fissure’ or‘vanishing azygos’ due to atelectasis.

It can mimic mass, scar, pneumothorax, andoesophageal dilatation as in achalasia cardia.

Detection of this anomaly and clarification ofits precise anatomical features are important not onlyto differentiate this anomaly from other pathologicalconditions, but also to alert the surgeon to potentialproblems during surgery. With the progress in minimallyinvasive thoracoscopic surgery, azygos lobeidentification has become important for anaesthetistsalso5.

Some of the complications associated withazygos lobe are6.

• Infarction of azygos lobe due to pulmonaryembolism,

• Lung cancer in azygos lobe,

• Azygos lobe bullae causing spontaneouspneumothorax,

• Aneurysm of the azygos vein.

• Tuberculosis of azygos lobe

Fig. 1 : CXR showing Rt. Paratracheal opacity

Fig. 2 & 3 : CT Thorax showing bronchiectatic cysts inazygos lobe, right middle lobe and left lower lobe


Kesavan Nair V. - An Unusual Bronchiectasis of Azygos Lobe

References

1. Studdert T.C, Turnbull A.K. Pneumonia of the Lobeof the Azygos Vein. British Institute of RadiologyVolume 20, Issue 231

2. Mata J, Cáceres J, Alegret X, Imaging of the azygoslobe: normal anatomy and variations. AJR Am JRoentgenol 1991;156:931-7

3. Aziz A Ashizawa K. Nagaoki K et al. High resolutionCT anatomy of the pulmonary fissures. J. ThoracImaging 2004;19:186–91.

4. Mehmet Cimen, Hayat Erdil ,Turan Karatepe:Cadaver with azygos lobe and its clinicalsignificance,Anatomical Science International •January 2006:80(4):235-7 Source: PubMed

5. A El-Dawlatly, A Al-Dohayan. The Azygos Lobe:Anesthetic Considerations. The Internet Journal ofAnesthesiology. 2005 Volume 11 Number 1.

6. Tatiana Denega, Suzanne Alkul BS, EbtesamIslam,et al. Recurrent hemoptysis - a complicationassociated with an azygos lobe :SWRCCC2015;3(11):44-47

Fig. 4 : CT thorax showing bronchiectaticcysts in azygos lobe


Case Report

Cannonball Lesions in a Young Patient : An Unusual Cause

Mathew Ninan1, Meeta Thomas2, Annamma M.O.3,Geena Benjamin4, Sukumaran P.5

1Associate Professor, Pulmonary Medicine2Assistant Professor, Pathology3Professor, Pathology4Professor, Radiology5Professor, Pulmonary MedicinePIMS & RC, Thiruvalla, Kerala

Abstract

Cannonball metastases refer to well-defined spherical nodules scattered over both lungs, beinga classical presentation of hematogenous tumor spread. Striking progression of lung metastases withoutestablished primary malignancy can raise a diagnostic challenge. We herein report a case of a 25 yearold female patient with alveolar soft part sarcoma(ASPS), a rare soft tissue sarcoma of children & youngadults which manifested with respiratory symptoms & cannonball metastases at initial presentation.

Key words

Alveolar soft part sarcoma, cannonball lesions, metastasis lung

Introduction

Alveolar soft part sarcoma(ASPS) is a raredistinctive soft tissue sarcoma affecting mainlyadolescents & young adults with a female preponderance& a predilection for the soft tissues of the thigh. Earlymetastasis is a characteristic feature & in a good numberof cases, metastasis to the lung or brain is the firstmanifestation, hence, in spite of relatively slow growthof the tumour ultimate prognosis is poor.

Case Report

A 25 year old female presented to the outpatientdepartment with complaints of dry cough of 6 months

duration with one episode of hemoptysis a few monthsback. She also complained of dyspnoea on exertion of 1week duration. She gave a history of loss of appetite &loss of weight of 9 kg over the past 10 months.Examination of the respiratory system revealed bilateralreduction in air entry. Her routine blood examinationshowed normal blood counts. Chest X-ray showedmultiple nodular opacities with a cannon ballappearance. A contrast enhanced CT chest was donewhich showed multiple hypervascular soft tissue densitylesions of sizes varying from few mms to 6 cms diffuselyinvolving both lungs with multiple enlarged mediastinallymph nodes. A CT abdomen was done which showedno significant abnormality.


Mathew Ninan - Cannonball Lesions in a Young Patient : An Unusual Cause

Fig. 2 : Contrast CT Thorax ShowingMultiple Canon Ball Lesions

Fig. 3 : MRI Showing a Large Soft Tissue Lesionin the Anterior Compartment Of The Left Thigh

Fig. 1 : Chest X Ray PA View ShowingCannon Ball Lesions

Meanwhile, a CT guided biopsy was obtainedfrom the largest lung nodule which showed a neoplasmcomposed of epithelioid cells arranged in solid &organoid patterns with reticulin stain highlighting theorganoid pattern. Individual neoplastic cells showedabundant eosinophilic cytoplasm & vescicular nucleisome with prominent nucleoli. Immunohistochemistry(IHC) for synaptophysin was negative ruling out

pulmonary paraganglioma. PAS positive, diastaseresistant granules were noted in most of the cells withpresence of very occasional crystalline inclusions. Onfurther probing & on detailed clinical examination, anill-defined swelling was palpated in the left thigh whichwas deep to the muscles.

MRI of the left thigh showed a largeheterogeneous soft tissue lesion in the anteriorcompartment of the thigh involving the vastus lateralis,intermedius & medialis - suggestive of a soft tissuesarcoma. An ultrasound guided biopsy of the thigh massshowed a lesion with similar histology as that of thelung mass with more prominent alveolar & organoidpatterns. The morphology & histochemistry along withthe typical clinical presentation enabled us to confirmthe diagnosis of ASPS of thigh with lung metastasis. Thepatient was then referred to the oncologist for furthermanagement.



Fig. 4 : Biopsy from the thigh mass showing cellsarranged in organoid & pseudoalveolar patterns

Fig. 5 : PAS stain showing PAS positive,diastase resistant granules within the cytoplasm

Because it is a tumour that typically growsslowly & causes few to no local symptoms, the firstclinical manifestation of disease in ASPS is frequentlymetastasis to the lung or brain8. Other commonmetastatic sites are bone & liver5. These tumours areextremely vascular & occasionally present as a pulsatilemass with associated bruit6.

The histology of ASPS is unique in that it varieslittle in appearance from case to case & is in somerespects remarkable among soft tissue neoplasms forthe absence of described variants(4).As originallydescribed by Chritopherson et al(3) ASPS is charaterisedby uniform organoid nests of polygonal tumour cellsseparated by fibrovascular septae & delicate capillarysized vascular channels. Within the nests there isprominent cellular dyscohesion leading to the distinctive

psudoalveolar pattern for which it is named. In somecases the organoid pattern may be lost & the tumourmay be composed of sheets of epithelioid cells.Intravascular tumour extension is seen in most cases &is responsible for early metastasis. The histopathologicaldifferential diagnosis of ASPS includes any tumour witheosinophilic/clear cytoplasm in an organoid/pseudoalveolar pattern including adrenal corticalcarcinoma, hepatocellular carcinoma(HCC), malignantmelanoma, renal cell carcinoma (RCC) & paragangliomaamong others.4 Coarse PAS positive diastase resistantgranules with occasional rod shaped or crystallinestructures are the histological hallmarks of the tumour.The characteristic ultrastructural feature is the presenceof membrane bound or free rhomboid crystals with aperiodicity of 10 nm.

Cytogenetic studies of ASPS have identified aspecific unbalanced translocation der(17) t (X;17)(p11;q25) which results in the formation of ASPL-TFE3fusion gene7. This gene can be detected by moleculargenetics or IHC analysis.

Treatment of ASPS primarly is surgical withonly minor roles for chemotherapy & radiotherapy.Achievement of complete microscopic resection iscritical in localized alveolar soft part sarcoma, butincomplete excision due to lack of appreciation of thecorrect diagnosis is all too often encountered. Despitethe occurrence of metastases in up to 79% of patients,5-year overall survival rates range from 45 to 88%9.

Discussion

ASPS is a rare tumour of unknown histogenesiswith a distinctive & generally invariable histologicalappearance & having specific molecular characteristics.It can occur at any age but mainly affects adolescents& young adults with occurrence being rare before 5 &after 50 yrs of age1. There is a female predilection before30 yrs of age & a male predilection thereafter. In adultsthe most common site of involvement is the extremitiesespecially the deep soft tissue of the thigh while thehead & neck esp. the orbit & tongue are the mostcommon sites of origin in children & infants(1) .Otherunusual locations include the female genital tract ,mediastinum, lung, stomach, bone1.



References

1) Christopher D M Fletcher, K. Krishnan Unni. WHOPathology & genetics of tumours of soft tissue andbone:

2) Sharon Weiss, John Goldblum. Enzinger & Weiss’sSoft tissue tumors. 6th edition:

3) Chistopher DM Fletcher,Diagnostic Histopathologyof Tumors:Vol.2. 4th edition:

4) Folpe A L and Deyrup A T. Alveolar soft-partsarcoma: a review and update.J Clin Pathol. 2006Nov; 59(11): 1127–1132.

5) Jayaprakash B., Neena P., Thara Somanathan.Pulmonary multinodular disease of rare etiology.Pulmon. May - Aug 2015;17(2): 87-89.

6) Sidi V, Fragandrea I. Alveolar soft – part sarcomaof the extremity: a case report .Hippokratia. 2008Oct-Dec; 12(4): 251–253.

7) Ming Zhao,Qiu Rao. Alveolar soft part sarcoma oflung: report of a unique case with emphasis ondiagnostic utility of molecular genetic analysis forTFE3 gene rearrangement and immunohistochemistry for TFE3 antigen expression. DiagnosticPathology 2015.10:160

8) Don Hayes, Melissa V. A 19 year old woman withprogressive dyspnea and multiple pulmonarynodules. Chest 2011; 140(1):253-257.

9) Keila Torres, Raphael Pollock .Alveolar Soft PartSarcoma

2 pulmon, vol. 20, issue 1, jan - apr 2018 - apccm

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