2020/07/19 · Board of Th e Egyptian Journal of Bronchology (EJB) Editor-in-Chief Tarek Safwat (Ain Shams) Deputy Editors Associate Editors Adel Kattab (Ain Shams Univ.) Adel

Board of Th e Egyptian Journal of Bronchology (EJB)

Editor-in-ChiefTarek Safwat (Ain Shams)

Deputy Editors

Associate Editors

Adel Kattab(Ain Shams Univ.)

Adel Saeed(Ain Shams Univ.)

Ashraf Hatem(Cairo Univ.)

Essam Gouda(Alex. Univ.)

Ashraf Madkour (Ain Shams Univ.)

EJB BOARDAbd El Hakim Mahmoud (Cairo Univ.)Abd El Moneim Rabie (Alex. Univ.)Abd El Rehim Yousef (Zakazik Univ.)Adel Salah (Zakazik Univ.)Ahmed Abdel Rahman (Monoufeya Univ.)Ahmed Al Halfawy (Cairo Univ.)Ahmed El Gazzar (Benha Univ.)Ahmed El Noury (Ain Shams Univ.)Amgad Abdel Raouf (Tanta Univ.)Amr Badr El Din (Banha Univ.)Ehab Atta (Alex. Univ.)Emad Koraa (Ain Shams Univ.)Gamal El Khouly (Tanta Univ.)Gamal Rabie Agmy (Assiut Univ.)Hafez Abdel Hafeez (Azhar Univ.)Hatem El Mallawany (Alex. Univ.)Hesham Tarraf (Cairo Univ.)Hoda Abou Yousef (Cairo Univ.)Ibrahim Radwan (Azhar Univ.)Khaled Eid (Cairo Univ.)Khaled Wagih (Ain Shams Univ.)Magda Yehia Elseify (Ain Shams Univ.)Magdy Abou Rayan (Alex. Univ.)Magdy Zedan (Mansoura Univ.)

Malak Shaheen (Ain Shams Univ.)Mamdouh Mahfouz (Cairo Univ.)Maysa Sharaf El Din (Cairo Univ.)Medhat Abdel Khalek (Cairo Univ.)Medhat Negm (Benha Univ.)Mohamed Abdel Sabour (Ain Shams Univ.)Mohamed Awad Ibrahim (Zagazig Univ.)Mohamed Dosouky Abou Shehata (Mansoura Univ.)Mohamed Khairy (Mansoura Univ.)Mohamed Metwally (Assiut Univ.)Nader Fasseeh (Alex. Univ.)Neveen Abd El Fattah (Ain Shams Univ.)Olfat El Shinawy (Assiut Univ.)Raef Hosni (Cairo Univ.)Ramadan Nafea (Zagazig Univ.)Salah Sorour (Alex. Univ.)Samiha Ashmawy (Ain Shams Univ.)Sayed Oraby (Ain Shams Univ.)Suzan Salama (Assiut Univ.)Tarek Mohsen (Cairo Univ.)Wafaa El Sheimy (Tanta Univ.)Walid El Sorougy (Cairo Univ.)Yasser Mostafa (Ain Shams Univ.)

ADVISORY BOARD INTERNATIONAL FACULTY BOARD

Mokhtar Madkour (Ain Shams Univ.)Mohamed Awad Tag El Din (Ain Shams Univ.)

Ahmed Boseila (Germany)Alaa El Gendy (USA)Atul Mehta (USA)Heinrich D. Becker (Germany)Henri G. Colt (USA)James R. Jett (USA)Majdy M. Idrees (KSA)Petr Pohunek (Czech Republic)Richard W. Light (USA)Roland M. du Bois (UK)

http://www.ejbronchology.eg.net/

Editorial CoordinatorAmr Shoukri (Ain Shams Univ.)

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Th e Egyptian Journal of Bronchology is will be published thrice a year, January, May and September to start with.

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Information about the Journal is also available at the Egyptian Scientifi c Society of Bronchology website at:http://www.ejbronchology.eg.net/

Th e Egyptian Journal of BronchologyThe Offi cial Journal of the Egyptian Scientifi c Society of Bronchology

Vol. (10), No. (3), September, 2016

Review Article

Review article206 Rapid on-site evaluation: what a microscope will

add to the bronchoscopy unit? a concise reviewMaged Hassan

Case reports212 Unusual presentations of lung lesions

in children: diffi cult to diagnose case series Shaimaa Kandil, Rasha H. Hassan, Ashraf Fouda, Magdy Zedan

223 Metastatic hepatoblastoma: a rare cause of lung mass in adults Upadhyay Hinesh N., Vakil Abhay P., Sherani Khalid M., Sherani Faraha K., Babury Mohammed M.

Interventional Bronchology and Pulmonology

Original article 225 The role of medical thoracoscopy in the diagnosis

of exudative pleural effusion at the Chest Department of Zagazig University Hospitals Abd El Rehim I. Yousef, Amani F. Morsi, Mohamed El-Shabrawy, Hadeer A. El Shahaat

Original article 232 Complications and follow-up of foreign body

inhalation Nehad M. Osman, Emad Eldin Korraa, Nevine M. Abd Elfattah

Original article 238 Correlation between pleural fl uid cytology and

magnitude of pleural invasion in patients with malignant pleural mesotheliomaAmr M. Shoukri, Nermine M. Riad

Airway Diseases

Original article 243 Role of comorbidities in acquiring pulmonary

fungal infection in chronic obstructive pulmonary disease patients Ashraf Z. Mohamed, Ahmad M. Moharrm, Maha K. Ghanem, Hoda A. Makhlouf, Ebtesam M. El-Gezawy, Sahar F. Youssif

Original article 251 Role of mean platelet volume in patients with

chronic obstructive pulmonary diseaseEman R. Ali

Original article 261 Assessment of serum vitamin d levels in different

severities of asthmatic patientsWaleed M. El-Sorougi, Hisham H. Eissa

Original article 266 Role of oxygen and continuous positive

airway pressure therapy in chronic obstructive pulmonary disease patients with nocturnal oxygen desaturation Randa Salah-Eldin Mohammad, Waleed Mohamed El-Sorougi, Abeer Salah El-Din Mohamed, Laila Anwar Mohamed Zaki

Original article 274 Role of transthoracic ultrasound in evaluating

patients with chronic obstructive pulmonary disease Hoda Ali Abou Youssuf, Esmat A. Abdelnabi,Ahmed M.Abd El Hafeez, Waleed F. Fathallah,Jumana H. Ismail

Original article 283 Acute exacerbations of chronic obstructive

pulmonary disease: etiological bacterial pathogens and antibiotic resistance in Upper Egypt Alaa T. Hassan, Sherif A.A. Mohamed, Mona S.E. Mohamed, Mohamed A. El-Mokhtar

Pulmonary Infections

Original article 291 Evaluation of lipoarabinomannan in the diagnosis

of tuberculosis Ayman A. Youssef, Mohammed H. Kamel, Hisham A. Eissa, Tarek S. Essawy, Hany H. Moussa

Pulmonary Critical care And Pulmonary vascular diseases

Original article 301 Study of ventilator-associated tracheobronchitis

in respiratory ICU patients and the impact of aerosolized antibiotics on their outcome

Hanaa Ali

Original article 310 Evaluation of role of computed tomography (CT)

in the diagnosis of pulmonary hypertension Ahmed G. Elgazzar, Mohammad Abd-Elmohsen Elmahdy,

Islam M. Elshazly, Ahmed M. Ramzy, Shaimaa M. Abo Youssef

Original article 319 Role of chest ultrasonography in differentiating

between acute cardiogenic pulmonary edema and acute respiratory distress syndrome

Taher El-Naggar, Samar H. Sharkawy, Hossam Mohamed Abdel-Hamid, Haitham S. El-Din Mohamad, Rasha Mustafa A. Mohamed

Sleep Medicine

Original article 324 Effectiveness of nocturnal oximetry in predicting

obstructive sleep apnea hypopnea syndrome: value of nocturnal oximetry in prediction of obstructive sleep apnea hypopnea syndrome

Suliman Lucy A., Shalabi Nesrien M., Elmorsy Saad A., Moawad Mona MK

Interstitial lung diseases

Original article 330 Correlation between high-resolution computed

tomography of the chest and pulmonary functions in idiopathic pulmonary fi brosis

Hoda Ali Abou Youssuf, Yousriah Yahia Sabry,Ahmed M. Abd El-Hafeez, Hadeel A. Mohamed

Lung function tests

Original article 337 Assessment of functional lung impairment in

patients with thyroid disorders Eman R. Ali

Miscellaneous

Original article 348 Are we with e-cigarette as a friend or against it

as a foe? Radwa A. Elhefny, Mohamed A. Ali, Assem F. Elessawy,

Esam G. El-Rab

Pleural diseases

Original article 355 Outcome of patients with spontaneous

pneumothorax admitted in Abbasia Chest Hospital

Emad E. Korraa, Ashraf M. Madkour, Amr M. Shoukri,Sameh E. Ahmed

Erratum

360 Erratum: Telomere length in chronic obstructive pulmonary disease

206 Review article

Rapid on-site evaluation: what a microscope will add to thebronchoscopy unit? a concise reviewMaged Hassan

Rapid on-site evaluation (ROSE) of samples obtained bytransbronchial needle aspiration during flexiblebronchoscopy or endobronchial ultrasound has beenpractised for more than two decades. Earlier studiesevaluating its role have reported a magical impact onimproving the diagnostic yield and the adequacy ofsamples produced by transbronchial needle aspiration.Subsequent studies with more rigorous methodologiesfailed to find a significant increase in sensitivity with ROSEbut consistently demonstrated a trend toward performingshorter procedures with fewer complications when ROSEis utilized. There are new exciting fronts for ROSE, suchas using it to direct molecular testing for lung cancer. Inthe future, we expect more centers to apply ROSE, nowthat pulmonologists have succeeded in doing so andtelecytopathology has become reality.

© 2016 Egyptian Journal of Bronchology | Published by Wolters Kluwer

Egypt J Bronchol 2016 10:206–211© 2016 Egyptian Journal of Bronchology

Egyptian Journal of Bronchology 2016 10:206–211

Keywords: bronchoscopy, endobronchial ultrasound, on-site cytology, rapidon-site evaluation, transbronchial needle aspiration

Department of Chest Diseases, Faculty of Medicine, Alexandria University,

Alexandria, Egypt

Correspondence to Maged Hassan, MSc, Department of Chest Diseases,

Faculty of Medicine, Khartoum Square, Azarita 21521, Alexandria, Egypt,

Tel: + +20 122 332 7822; fax: +2033352691;

e-mail: [email protected]

Received 23 April 2016 Accepted 3 June 2016

This is an open access article distributed under the terms of the Creative

Commons Attribution-NonCommercial-ShareAlike 3.0 License, which

allows others to remix, tweak, and build upon the work

noncommercially, as long as the author is credited and the new

creations are licensed under the identical terms.

IntroductionIn the era of modern medicine and development ofsophisticated diagnostic machines that are less invasiveand − as a consequence − acquire smaller samples, fine-needle aspiration (FNA) has become a well-establishedprocedure that is commonly used for investigatinglesions at many anatomical locations. It is regardedas safe and accurate and has a low complication rate.

Rapid on-site evaluation (ROSE) of cytologicalmaterials obtained using FNA procedures has beenused for some time for evaluating lesions located indifferent organs/structures in the body with the aim offine-tuning the sampling procedure [1].

The concept of FNA was introduced in flexiblebronchoscopy in 1983 by the innovation oftransbronchial needle aspiration (TBNA) with theaim of sampling abnormal structures beyond theairways (the mediastinum) [2]. It has become aprominent sampling tool for a variety of malignant,infectious, and granulomatous lesions, and in thesetting of nonsurgical staging of lung cancer TBNAhas been shown to decrease the need for diagnosticthoracic surgery [3].

Using ROSE during TBNA was first studied byDavenport [4] who was the first to publish aboutthe subject in a major journal. The positive results interms of improved diagnostic yield have encouragedlarge centers to incorporate ROSE in theirbronchoscopy units. More studies have later lookedinto the role of ROSE during TBNA.

The addition of real-time ultrasound guidance to theneedle during TBNA [called endobronchial ultrasound(EBUS)] was an immense technological breakthroughthat has dramatically refined the process of TBNA andhas allowed both examination and sampling of verysmall lesions [5]. The ‘blind’ procedure was calledconventional transbronchial needle aspiration(cTBNA) henceforth to differentiate it from EBUS-guided TBNA. The ultrasound technology did notalienate ROSE. On the contrary, it is now a markof excellence to have an EBUS machine in addition tothe capability to perform ROSE during TBNA.

This review aims at examining the exact role of ROSEduring TBNA, whether conventional or EBUS guided,and to point out the added value, if any, in improvingdiagnostic yield and decreasing complications ofendoscopic procedures.

Materials and methodsA search on Medline was performed from 1990 toApril 2016 with the following keywords: ‘TBNA’;‘ROSE’; and ‘on-site cytology’. Entries that were notin English or involved case series with less than 20patients were excluded. In total; 48 studies could beidentified. After examining the titles/abstracts; 21studies were excluded; and full texts of the

- Medknow DOI: 10.4103/1687-8426.193634

mailto:[email protected]

ROSE for TBNA Hassan 207

remaining articles [6] were retrieved for evaluation[4,6–31]. Data on the study design; diagnostic yield;complication rate; and number of patients in thesestudies are shown in Table 1.

Rapid on-site evaluationThe cellular material retrieved from the TBNA isconventionally smeared on a glass slide, directly‘wet-fixed’ in 95% ethanol, and then later sent to thecytopathologist who usually uses either theMay–Grunwald Giemsa or the Papanicolaou methodto stain slides [32]. Any of these techniques requiresaround 5min of preparation per slide. For the purposeof rapid and timely examination of the aspiratedmaterial ‘on-site’ (in the bronchoscopy unit),cytopathologists have devised a modification for theGiemsa method that allows slide preparation within30 s. There are various commercial kits available, andthe most commonly used one (which is reported inmore than half of the cited studies) is the Diff-Quikmethod [32]. In this method, three aliquots containingdifferent solutions are used. After smearing the TBNAmaterial on the slide, it is left to dry in air and thenimpregnated in each aliquot for 5–8 s, which can thenbe examined directly. Images obtained can be used todefine the adequacy of the sampled material byshowing either malignant cells or at least abundantlymphocytes. Sometimes a provisional diagnosis canalso be reached. Figures 1 and 2 show smears highlysuggestive of nonsmall and small-cell lung cancer,respectively. A smear composed predominantly ofred cells or bronchial cells (Fig. 3) denotes aninadequate sample.

Conventional transbronchial needle aspiration andrapid on-site evaluationInspecting the studies in Table 1 will clearly show twodistinct eras − the first from inception of the idea in1990 to 2010 and the latter from 2011 onward. Earlierstudies were observational in nature and their resultsshowed improved sensitivity with addition of ROSE toTBNA compared with procedures performed withoutROSE [4,7]. Diacon et al. [9] did not have acomparative group, but demonstrated that the overallcosts are significantly lower by having a cytopathologiston-site, avoiding the need for additional diagnosticprocedures once a diagnosis is reached.

Later studies in the ‘observational’ era were moreconservative and critical. Although the results ofChin et al. [8] favored ROSE for allowing betterdiagnostic yield, the authors identified a keyproblem − the extremely high risk of selection bias.

No parameters were set for the allocation of patientsinto the ROSE or no-ROSE arms, a practice thatmakes it impossible to rule out that more complex caseswere allocated to the ROSE arm or vice versa. Thequestion was made even more relevant when Baramet al. [10] failed to find any diagnostic superiority byusing ROSE during cTBNA. They confirmed,however, the earlier edge of enabling to conclude theprocedure after fewer biopsies. At this point, it was feltthat the success rate of cTBNA is influenced by anumber of factors besides ROSE, such as size andlocation of lymph nodes, experience of the examiner,the needle type used, underlying disease, andprevalence of the disease being ascertained [33].

The second era was marked by two randomized-controlled studies that were published almostsimultaneously. The first trial aimed at evaluatingthe usefulness of ROSE in clinically unselectedpatients with lymphadenopathy at computedtomography [12]. Neither diagnostic yield norspecimen adequacy was significantly different in thetwo study arms. The possibility to avoid biopsy fromadditional targets without loss in diagnostic yield wasthe most important benefit of using ROSE, as it wasassociated with a significant reduction in thecomplication rate of bronchoscopy. The other trial(which had fewer patients) reported a similar patternwith effect on diagnostic yield and hinted on a ‘trend’toward allowing fewer passes with ROSE [13].

Endobronchial ultrasound-transbronchial needleaspiration and rapid on-site evaluationDespite seeming intuitive that EBUS guidance shouldobviate the need for ROSE to confirm the value of thesampledmaterial, in real life, most centers that have thecapability for ROSE are the ones that are large enoughto have the EBUS technology. ROSE for EBUS-TBNA has had a good share of studies looking at it.Griffin et al. [15] were the earliest to study the utility ofROSE during EBUS. The authors retrospectivelystudied the outcomes of 294 EBUS-TBNAs ofwhich 140 had ROSE performed and unexpectedlyreported no remarkable difference in diagnostic yield,the number of sites sampled per patient, or clinicaldecision making between specimens collected throughEBUS-TBNA with or without ROSE. Similarfindings were reported from a later study that onlyobserved the outcome of EBUS-TBNA without acomparison group [19].

Other studies with observational design that exploredthe different aspects of ROSE with EBUS came withmore positive results. Eapen et al. [18] reported the

Table 1 Summary of the studies discussing rapid on-site evaluation for transbronchial needle aspiration

References Numberof

patients

Samplingtechnique

Study design and purpose Outcome

Davenport[4]

207 cTBNA Comparative, nonrandomized (73 withROSE vs. 134 without ROSE)

Improved diagnostic yield (56% for ROSE vs. 31%without ROSE)

Dietteet al. [7]

204 cTBNA Comparative, nonrandomized (81 ROSE) Improved diagnostic yield (80% for ROSE vs. 51%without ROSE)

Chin et al.[8]

55 cTBNA Comparative, nonrandomized (ROSE 55vs. non-ROSE 35)

Better yield (70 with ROSE vs. 25% without ROSE)Problem with bias possible

Diaconet al.[9]

90 cTBNA Observational Addition of ROSE allowed the procedure to beterminated early in 64% of cases

Baramet al. [10]

44 cTBNA Comparative, nonrandomized (32 withROSE vs.12 without ROSE)

No difference in yield. Fewer biopsies needed in theROSE group.

Cardosoet al. [11]

81 EBUS Comparative, nonrandomized (41 withROSE vs. 40 without ROSE)

93 vs. 80% sensitivity in favor ROSE

Trisoliniet al. [12]

168 cTBNA RCT No difference in diagnostic yieldLess number of passesand fewer complications with ROSE

Yarmuset al. [13]

68 cTBNA RCT No difference in diagnostic yieldTrend toward fewerpasses with ROSE

Griffinet al. [15]

294 EBUS Retrospective comparative (140 caseswith ROSE)

No difference in sensitivity or number of proceduresperformed

Brundynet al. [14]

48 cTBNA Safety and yield in SVC High yield, less need for biopsyNo complication

Plit et al.[16]

60 EBUS Prospective for sarcoidosis. ROSE versusfinal diagnosis by TBLB

Concordance rate 92%

Nakajimaet al. [17]

438 EBUS Retrospective comparative (ROSE vs.final diagnosis)

Concordance rate 94%

Eapenet al. [18]

1317 EBUS Acquire registry. Rate of complicationduring EBUS

Less complications with ROSE (less need for TBLB)

Josephet al. [19]

170 EBUS Retrospective observational ROSE did not impact sensitivity

Brunoet al. [20]

120 cTBNA RCT Improved sensitivity and less cost

Oki et al.[21]

108 EBUS RCT Study not powered to detect improvement in sensitivity.Lower need for additional procedures and punctures

Sindhwaniet al. [22]

40 cTBNA Observational ROSE improved yield and helped prevent repeatingprocedures

Khuranaet al. [23]

200 EBUS Comparative, nonrandomized(telecytology vs. conventional ROSE)

Comparative concordance

Bonifaziet al. [24]

84 cTBNA ROSE by pulmonologist vs.cytopathologist

80% agreement

Minamiet al. [25]

35 EBUS Role of Bioevaluator with ROSE Technique is useful to determine adequacy beforemicroscopy

Murakamiet al. [26]

77 EBUS Retrospective (Role of ROSE in SCLCcases)

No difference in sensitivityFewer passes and stationswith ROSE

Jeffuset al. [27]

118 EBUS RetrospectiveEvaluated the use ofstructured ROSE approach to defineadequacy

Improved sensitivity with structured approach

Trisoliniet al. [6]

126 EBUS RCT. Evaluated the suitability of samplesfor molecular markers

ROSE provided better samples for molecular markersand allowed fewer passes

Mallyaet al. [28]

77 EBUS Observational 85% sensitivity

Guo et al.[29]

245 EBUS Retrospective (122 patients with ROSE,123 without ROSE)

No difference in sensitivityFewer passes in ROSEgroup

Madanet al. [30]

41 cTBNA Retrospective, observational Sensitivity 78% with ROSE

Rokadiaet al. [31]

255 EBUS Retrospective, observationalgranulomatous disease

Concordance rate 80% ROSE with final

cTBNA, conventional transbronchial needle aspiration; EBUS, endobronchial ultrasound; RCT, randomized-controlled trials; ROSE, rapidon-site evaluation; SCLC, small-cell lung cancer; SVC, superior vena cava; TBLB, transbronchial lung biopsy.

208 Egyptian Journal of Bronchology

Figure 1

Nest of cells harboring features of malignancy − suggestive of non-small-cell lung cancer. Diff-Quik stain, ×40 magnification.

Figure 2

Nest of cells harboring features of malignancy with scantycytoplasm − suggestive of small-cell lung cancer. Diff-Quik stain,×40 magnification.

Figure 3

Bronchial cells by Diff-Quik stain.


findings of the acquire registry created by the AmericanCollege of Chest Physicians, where they found that therate of complications was significantly less duringEBUS-TBNA when ROSE was used, and they

explained that this was mainly due to performingless transbronchial biopsy (TBBX) procedures whenROSE was used. Both Murakami et al. [26] (whostudied specifically cases that were eventuallydiagnosed with small-cell lung cancer) and Guoet al. [29] found no significant increase in sensitivitywith ROSE, but its use allowed performing fewerneedle punctures and briefer procedures.

Two randomized-controlled trials exist in the literaturethat examined the role of ROSE during EBUS-TBNA. The earlier study found unequivocalevidence that ROSE was associated with asignificantly lower need for additional bronchoscopicprocedures and punctures [21].

Rapid on-site evaluation and lung cancer genotypingThe second randomized-controlled trial was carried outby Trisolini et al. [6] who designed their study to assessthe influence ofROSEon the yield ofEBUS-TBNAfora multigene molecular analysis of lung cancer samples.One hundred and twenty six patients with suspected orknown advanced lung cancer were randomized toundergo EBUS-TBNA without ROSE or withROSE. In addition to shortening the procedural time,ROSE prevented the need for a repeat invasivediagnostic procedure aimed at molecular profiling inat least one out of 10 patients and significantlyreduced the risk of retrieving samples that can be usedonly for pathologic subtyping [6]. An important point tonote in the former study was that only tissue coresretrieved during TBNA could be used for moleculartesting, whereas cytology specimens were used forpathological diagnosis.

In a subsequent pivotal study by Casadio et al. [34], 306patients with clinically diagnosed primary lung cancerunderwent the EBUS-TBNA procedure, and theEGFR and KRAS mutations were evaluated thistime on the cytological specimens produced.Although this study was not specifically evaluatingthe on-site cytology procedure, ROSE was central totheir methodology. Molecular testing was onlyperformed on the cytology if deemed adequate byROSE. The authors concluded that EBUS-TBNA(when combined with ROSE) can be effectivelyused not only for diagnosis but also for completemutational testing [34].

Rapid on-site evaluation in benign diseasesROSE during EBUS-TBNA for patients withsuspected sarcoidosis was prospectively studied byPlit et al. [16] who compared the diagnostic


accuracy of EBUS-TBNA with ROSE with the finalcytological assessment and with transbronchial andendobronchial biopsies in 60 patients. ROSE hadhigh diagnostic accuracy (88%), and agreement withother modalities was present in 91% of cases. Theyconcluded that ROSE can inform the bronchoscopistin theater whether additional diagnostic proceduresneed to be undertaken [16]. More recently, Rokadiaet al. [31] retrospectively examined 255 cases withgranulomatous disease as their final diagnosis whohad undergone EBUS-TBNA with ROSE duringtheir diagnostic workup. There was 81%concordance between the ROSE findings and thefinal diagnosis. The concordance was not impactedby needle size, lymph node size or station, number ofstations biopsied, or passes per lymph node [31].

Recent innovationsAmong the recent advances with ROSE was theintroduction of the Bioevaluator (MurazumiIndustrial Co. Ltd.; Osaka, Japan) system in a studyby Minami et al. [25]. It is a device used fordetermining whether the tissues obtained by EBUS-TBNA are appropriate for a pathological diagnosis. Aspecial light was used to examine the aspirated materialafter being smeared on a slide. Tissue areas appearingwhite and red through Bioevaluator were considered tobe appropriate and inappropriate, respectively.Checking aspirated samples using this new systemappeared useful for determining their adequacy forpathological diagnosis [25]. Another aspect that wasexplored was the use of telemedicine in ROSE. Real-time images of stained cytology smears were obtainedusing a digital camera attached to an Olympusmicroscope (Olympus; Tokyo, Japan) andtransmitted through ethernet by a cytotechnologistto a cytopathologist in a cytopathology laboratorywho rendered a preliminary diagnosis whilecommunicating with an on-site cytotechnologist[23]. The overall concordance between thepreliminary and final diagnoses was 96% fortelecytopathology and 93% for conventionalmicroscopy. It was concluded that telecytopathologyis comparable with conventional microscopy in ROSEwith EBUS-TBNA. It can serve as a valid substitutefor conventional microscopy for on-site assessment ofEBUS-TBNA [23].

Rapid on-site evaluation by the pulmonologistA recent study tried to verify whether a pulmonologistwith training in cytology can perform ROSE [24]. Atotal of 364 aspirations made by cTBNA were firstexamined through ROSE by a cytology-trained

pulmonologist. These smears were later examined bya board-certified cytologist. There was an 81% overallsubstantial agreement between observers. The studywas only designed to evaluate the feasibility of theconcept, and thus the authors did not comment on theimpact of ROSE on sensitivity or complications. Theimplications of this study are significant. Trainingpulmonologists to have a basic knowledge ofcytopathology can possibly obviate most difficultiesrelated to the involvement of cytopathologists inroutine diagnostic activities and may reduce the costsof the procedure [24]. Performance of ROSE by thepulmonologist during both cTBNA and EBUS-TBNA has gained some popularity and is nowperformed routinely in many centers, especially inEurope.

ConclusionDespite the overzealous outlook for the role of ROSE inTBNA in earlier studies, the accumulating evidence hasconfirmed its value for decreasing the number and varietyof bronchoscopy samplingmethods during both cTBNAand EBUS-TBNA. ROSE has shown acceptablesensitivity both for malignant and benign disease. Therole of ROSE is emerging in molecular testing for lungcancer, and the capacity of pulmonologists to performROSE using telemedicine technology will serve topropagate the application of the procedure.

AcknowledgementsThe author thanks Professor Grigoris Stratakos, Headof the Interventional Pulmonology Unit at SotiriaChest Hospital and Assistant Professor of Medicineat the National and Kapodistrian University of Athens,Greece, for hosting the author in his institution wherehe was introduced to and trained on the procedures thatare described in the review. Professor Stratakos alsogenerously provided micrographs of cases of ROSEperformed at his department.

Financial support and sponsorshipNil.

Conflicts of interestThere are no conflicts of interest.

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212 Case report

Unusual presentations of lung lesions in children: difficultto diagnose case seriesShaimaa Kandila, Rasha H. Hassana, Ashraf Foudaa, Magdy Zedanb

We present five cases of unusual and rare presentations ofdifferent congenital/acquired lung lesions. These cases wereencountered during our daily practice. They weremisdiagnosed initially and received wrong treatment. Therewere challenges encountered during the diagnosis and themanagement of these patients, which required differentmodalities, ranging from chest radiography, computedtomography to surgical exploration and biopsy, to reachthe final diagnosis. These cases range from one absentlung, bilateral intralobar pulmonary sequestrations,bronchogenic cyst, congenital right diaphragmatic hernia tocystic hydatidosis (hydatid cyst). There should be a high indexof suspicion when the patient has an abnormal/atypicalpresentation, a prolonged course of the disease orabnormal imaging. Healthcare providers should also thinkof rare chest diseases and refer such patients to a paediatricspecialist (pulmonologist) to help in the final diagnosis and


specific management, which may require invasiveprocedures or specific imaging.Egypt J Bronchol 2016 10:212–222© 2016 Egyptian Journal of Bronchology


Keywords: bronchogenic cyst, congenital diaphragmatic hernia and cystichydatidosis, congenital lung lesion, lung agenesis, pulmonary sequestration

aDepartment of Pediatric, Mansoura University Children’s Hospital, bAllergy,

Clinical Immunology and Respiratory Medicine Unit, Faculty of Medicine,

Mansoura University, Mansoura, Egypt

Correspondence to Dr Shaimaa Mohamed Kandil, MD, Lecturer of

Pediatrics, Pediatric Department, Mansoura Faculty of Medicine, Mansoura

University Children Hospital, Mansoura University, 35516, Al-Mansoura,

Egypt, Tel: 00966599544894;

E-mail: [email protected]

Received 31 December 2014 Accepted 23 February 2015

IntroductionWe present in this paper five cases of unusual and rarepresentations of different lung lesions (congenital/acquired lung lesions), which represent a wide range ofchest diseases. Therewere challenges encountered duringthediagnosisandthemanagementofthesepatients.Thesepatients were initially misdiagnosed with other commonillnesses or even received inappropriate or unnecessarytreatment. Different modalities of investigations wererequired, ranging from chest radiography (CXR) andchest computed tomography (CT) up to surgicalexploration and biopsy, to reach the final diagnosis. Asummary of the following cases is shown in Table 1.






Case 1A 2.5-year-old female patient presented with sudden-onset wheezes and respiratory distress (subcostal andintercostal retraction) for the past 4 days. She wasreferred to our hospital with the suspicion offoreign-body (FB) inhalation, although there was nohistory of choking. There was no history of similarconditions or chest diseases. Her perinatal history (theperiod immediately before and after birth) was normal.On examination, the respiratory rate (RR) was 25breaths/min, there was no fever or clubbing, andgrowth parameters (weight and height) were within50th percentiles. Breath sounds were absent over thewhole left lung, with an impaired percussion note.

CXR was performed (Fig. 1a). FB inhalation wassuspected, and so urgent rigid bronchoscopy was

performed, which revealed a sharp carina and anabsent right upper lobe bronchus with a blind end ofthe left main bronchus. Hence, CT chest wasrecommended (Fig. 1b). Echocardiography revealeda small restrictive subaortic ventricular septal defect.

Final diagnosis: agenesis of the left lungCommentOur case was referred with the suspicion of FBinhalation, due to sudden-onset respiratory distressfor few days, which was supported by an abnormalCXR finding of compensatory hyperinflation of the leftlung, shift of the heart and the mediastinum andcrowded ribs of the left side, which were suggestiveof a collapsed left lung. However, there was no historyof choking and no signs of respiratory distress;however, FB inhalation was highly suspected.

Paediatric airway FB aspiration is associated with a highrate of airway distress, morbidity andmortality, especiallyinchildrenyounger than3yearsof age [2].Thepresentingsymptoms of FB aspiration may vary depending on itslocation, size and chronicity. The child may becomfortable and in no apparent distress or may presentin extremis with impending airway failure. Coughing,wheezing, shortness of breath, fever and recurrent

- Medknow DOI: 10.4103/1687-8426.193644

mailto:[email protected]

Table 1 A summary of our cases

Age/sexOnset ofsymptoms

Symptoms General exam Chest exam Initial diagnosis Diagnostic test Final diagnosis/management

2.5 Y/F, Suddenfor 4 days

Wheezes andRD

RR 25 breaths/min, no fever,clubbing

Absent BS andimpaired noteover Lt side

FB inhalation CXR, CT chestbronchoscopy

Lt lung agenesis/conservative

11 Y/MChronicsince the age of4 months

Recurrentrespiratoryinfections,productivecough,dyspnoea andintermittentfever

RR 28 breaths/min, no pallor,cyanosis,clubbing, orpalpable lymphnodes

Barrel chest,retractions,bilateral basalconsonatingcrepetations

BA, supprutivelung disease,immundefeciency

CXR, CT/angiographybiopsy

Intralobarpulmonarysequestration/surgery

a4.5-month/FGradual since1 month age [1]

Noisybreathing withfeedingdifficulty

RR 60 breaths/min, HR 140beats/min; O2saturation 94%,inspiratory stridor,no cyanosis, fever

Retractions,hyper-resonantpercussion,inspiratory andexpiratory sibilantronchi

Hyper-reactiveairway, BA, FBinhalation

CXRbronchoscopy,CT chest

Bronchogenic cyst/surgery

3.5 Y/MRecurrentfor few weeks

Recurrentwheezes, chestinfections andabdominaldistension

RR 30 breaths/min, HR 90 beats/min, norespiratory,distress, fever orcyanosis

Diminished BSover Rt lung baseand BB over Rtmiddle zone

Lung abscess,hydropnemothorax

CXR, CT chest Congenitaldiaphragmatichernia/surgicalcorrection

4 Y/MIntermittentover 3 weeks

Low-gradefever, RD,cough,progressiveabdominalenlargementand Rtabdominal pain

Toxic look, RR 40breaths/min, nofever, Pallor,jaundice,cyanosis,abdominalenlargement,hepatomegaly

Diminished BSover Rt middleand basal lung,impairedpercussion note

Pnemonia/unresolvedresidualpnemonia, tumor

CXR, CTchest/abdomen,abdominal USpathology

Hydated cyst/antiparasiticalbendazole

BA, bronchial asthma; BB, bronchial breathing; BS, breath sound; CT, computerized tomography; CXR, chest radiography; F, female; FB,foreign body; HR, heart rate; Lt, left; M, male; RR, respiratory rate; Rt, right; US, ultrasound; Y, years. aPreviously published: Zedan et al. [1].

Difficult to diagnose cases Kandil et al. 213

pneumonia may each be the presenting symptoms.Parental recall of a choking or gagging event followedby a cough is highly suspicious.However, this initial eventmay be short lived and the childmay be asymptomatic forone or more weeks, often leading the parents to forgetabout the inciting episode [3].

The physical examination can be nonspecific and theradiological evaluation normal, which may result indelayed diagnosis. An elevated index of suspicion isnecessary in all cases of pneumonia, atelectasis orwheezing with atypical courses. Early diagnosis isfundamental for optimal management, consisting of theremovalof theFBassoonaspossible topreventrespiratorysequelae. Localized wheezes or diminished breathingsounds from the affected lung are the main findings [4].

Cervical and thoracic radiographical evaluation is themost important investigation in every patient suspectedof having an FB aspiration. Radiographical abnormalitiesare more frequent when the FB is endobronchial. Thislocation is alsomore frequent in cases of delayeddiagnosisand delayed removal. An expiratory CXR should berequested (in cooperative children) when the standard

inspired film is normal, as this strategy allows thevisualization of air trapped by a valve-like effect due topartial obstruction of the bronchial lumen. Amediastinalshift may also be seen. The most frequent radiologicalfindings are obstructive emphysema, atelectasis andconsolidation. However, normal findings may occur[4]. CT scans can be used in patients with prolongedsigns and symptoms to identify early sequelae or to detectradiolucent materials [5]. Rigid bronchoscopy wasperformed for the suspected FB, but the amazingfinding of an absent right upper lobe bronchus and ablindendof the leftmainbronchussuggestedanabnormallungpathologyordevelopment,which requiredaCTscanfor the chest. CT chest showed a compensatoryhyperinflated right lung and an absent left lung,confirming the final diagnosis.

Regarding pulmonary agenesis, the onset of symptoms isremarkably variable. In many cases, the presence of thisanomaly usually comes to light during infancy because ofrecurrent chest infections, cardiopulmonary insufficiencyor due to associated congenital anomalies. However,patients with one lung have been reported to survivewell into adulthood without much complaints [6].

Figure 1

(a) CXR AP reveals a left opaque hemithorax, a hyperinflated rightlung (arrow) and shift of the heart and themediastinum to the left side.(b) CT chest demonstrates an hyperinflated right lung (arrow) with theheart occupying the left side of the chest. AP, anteroposterior; CT,computed tomography; CXR, chest radiography.


The exact aetiology of this condition is unknown,although genetic factors, viral agents and dietarydeficiency of vitamin A during pregnancy have beenimplicated. Left-sided agenesis is more common andthese individuals have a longer life expectancy thanthose with right-sided agenesis. This is probably due tothe excessive mediastinal shift and malrotation of thecarina in right-sided agenesis, which hinders properdrainage of the functioning lung and increases thechances of respiratory infections [6]. Nearly 50% ofthe cases of pulmonary agenesis have associatedcongenital defects, involving the cardiovascular, theskeletal, the gastrointestinal and the genitourinarysystems [7]. Echocardiography for our patientrevealed a small ventricular septal defect as anassociated congenital anomaly.

The differential diagnosis of lung agenesis alsoincludes lung collapse, a thickened pleura, adestroyed lung and pneumonectomy. The finaldiagnosis can be established after bronchoscopyand/or bronchography. Surgery is seldom requiredfor agenesis or aplasia, as it can be managed onconservative lines. The prognosis in these casesdepends on the functional integrity of theremaining lung and the presence of associatedanomalies [6].

Case 2An 11-year-old male patient presented with recurrentepisodes of respiratory tract infections (up to two tothree episodes per month) since the age of 4 months.He had productive cough of excess whitish to greyishpurulent offensive sputum, associated with dyspnoeaand intermittent fever. There was no weight loss orchest pain. The patient was previously diagnosed tohave bronchial asthma, but without response tobronchodilators or steroids. There was no familyhistory of chest problems, with an irrelevantneonatal history.

The patient had average growth parameters. Onexamination, there was no pallor, no cyanosis, noclubbing or palpable lymph nodes. His vital signs wereas follows: temperature, 37.0°C; heart rate (HR), 110beats/min; RR 28 breaths/min, with an average bloodpressure for his age and height. Chest examinationrevealed a barrel-shaped chest, intercostal retractions,with a central trachea. There were bilateral basalmedium-sized consonating crepitations.

The white blood cell count was 9000/mm3 (66%polymorph). CXR (Fig. 2a) showed consolidationcollapse of the medial segment of the right middlelung lobe. Multislice CT and CT angiography of thechest showed multicystic air-filled lesions occupyingthe medial segment of the right lower lung lobe andthe posterior basal segment of the left lower lobe(Fig. 2b).

Bronchoscopy was performed and the bronchoalveolarlavage revealed suppurative smears with no atypical cells.

Surgical excision of the lesions was performed in twosessions.Pathologic findingsof theexcisedmass revealedacute and chronic inflammatory changes with purulentexudates and variable-sized spaces lined with cuboidaland pseudostratified columnar epithelium, a few smoothmuscle bundles and cartilaginous bronchus-likestructures (Fig. 2c).

Figure 2

(a) A preoperative CXR film showing segmental consolidation collapse of the medial segment of the right middle lung lobe (white arrow)and retrocardiac collapse in the left side (black arrow). (b) CT chest angiography demonstrates multicystic air-filled lesions occupyingthe medial segment of the right lower lung lobe and the posterior basal segment of the left lower lobe. These lesions were supplied bydescending branches of both the right and the left pulmonary arteries and drained into the right and the left pulmonary veins. Theangiographic diagnosis was bilateral intralobar PS. (c) Pathological examination of the excised PS shows acute and chronicinflammatory changes with purulent exudates and variable-sized spaces lined with cuboidal and pseudostratified columnar epithelium(arrow), a few smooth muscle bundles and cartilaginous bronchus-like structures (H&E stain, ×200). CT, computed tomography; CXR,chest radiography; PS, pulmonary sequestration.


Final diagnosis: intralobar pulmonary sequestrationComment

Pulmonary sequestration (PS) is a congenital malfor-mation consisting of a nonfunctioning bronchop-ulmonary mass separated from the normal pulmonarytissue.Hence the termPS is applied to a pulmonary lobeor a portion of a lobe that is supplied by an anomaloussystemic artery anddrains either into the systemic or intothe pulmonary veins. The conditions are divided intointralobar PS, in which the sequestration is situatedinside the visceral pleura of a normal lobe, andextralobar PS, in which the sequestration issurrounded by its own pleura. Most sequestrations areunilateral; bilateral ones are rare [8].

The child with recurrent chest infections presents theclinician with a difficult diagnostic challenge. Doesthe child have a simply managed cause for hissymptoms, such as recurrent viral respiratoryinfections or asthma, or is there evidence of a moreserious underlying pathology, such as bronchiectasis,PS, immune deficiency, cystic fibrosis and ciliaryabnormalities [9].

Repeated episodes of pneumonia are often thepresenting feature of structural airway abnormalities(localized bronchial stenosis, bronchomalacia, tracheo-bronchus and bronchiectasis) or parenchymal lunglesions [PS, cystic adenomatoid malformation and

Table 2 Differential diagnosis of congenital cystic lung disease and parenchymal lung lesions presented as recurrentpneumonia [1]

Disease Pathogenesis Classification Clinical aspect Radiology

Bronchogeniccyst

Remnants of the primitive foregutcontaining tissue normally found inthe trachea and bronchi

Single, multilocular ormultiple location:paratracheal, carinal,paraoesophageal, hilar

Early childhood, but symptomscan develop at any age cough,stridor or wheezing

Cyst appearsas an ovoid softtissue density

Pulmonarysequestration

Mass of nonfunctional embryoniclung tissue characterized bybronchiectasis and varyingdegrees of cystic changes and isusually supplied by an aberrantsystemic artery

Intralobar: lies within a lobeof the lung invested by itsvisceral pleuraExtralobar: has its owninvesting pleura outside thenormal lung parenchyma

Intralobar: asymptomaticproductive cough, fever,haemoptysis, recurrentpneumonia and chest painExtralobar: newborns, frequentlyassociated with other congenitalanomalies infection (when thereis oesophageal or gastriccommunication)

Soft tissuemass in theposterior basalsegment of thelung

Congenitalcysticadenomatoidmalformation(CCAM)

Solid, cystic or mixed masses thatcommunicate with the normaltracheobronchial tree

Type 1 (50%): one or morelarge cysts (2–10 cm)Type 2 (40%): multiplesmaller cysts (0.5–2 cm)Type 3 (10%): solid on grossexamination, but containmultiple 0.3–0.5 cm cysts onmicroscopic examination

Newborn: progressive respiratorydistressAfter the neonatal period:recurrent pulmonary infections

Multiple air orair fluid-filled,thin-walledcysts that varyin size

Congenitallobaremphysema(CLE)

Overdistension of (usually) onelobeThe most constant pathologicalfinding is cartilage deficiencyresulting in bronchomalacia

Left upper lobe: 41%Right middle lobe: 34%Right upper lobe: 21%Bilateral: 20%

Progressive, severe respiratory,distress in infants, withwheezing, cough or recurrentchest infection

Overinflation ofthe affectedlobe


bronchogenic cysts (BC)] [10]. Parenchymal lunglesions should be suspected if one lobe is repeatedlyinfected or if there is incomplete resolution aftertreatment. CT and magnetic resonance scanning arehelpful in defining the anomaly before surgical excision[11]. Differentiating features of such parenchymallesions are shown in Table 2.

There are many different causes of recurrent chestinfections in children. The clinician has todistinguish between children with self-limitingconditions and those with more severe,progressive, diseases such as parenchymatous lungdisease. Although PS is a rare condition, early andaccurate diagnosis by CT angiography is essential toensure that optimal treatment is given and tominimize the risk of progressive or irreversiblelung damage.

Case 3A 4.5-month-old female infant was presented withgradual onset of noisy breathing in association withfeeding difficulty. Her symptoms started since 1 monthof age with no cough, fever or cyanosis. Her noisybreathing sounds were interpreted as wheezes andtreated as asthma, suggested by a positive familyhistory of an older asthmatic brother, with noresponse to bronchodilators.

On presentation, she had inspiratory stridor and nocyanosis. She had no fever, the HR was 140 beats/min and RR 60 breaths/min. Her oxygen saturationwas 94%. Her growth parameters were normal. Chestexamination revealed a normal shape, with suprasternaland subcostal retractions. She had a centrally posedtrachea and a hyper-resonant percussion note. Herbreath sounds were harsh vesicular with bilateralinspiratory and expiratory sibilant rhonchi.

CXR revealed bilateral hyperinflation (Fig. 3a).Fibreoptic bronchoscopy revealed a wide carinaand narrowed right and left main bronchi withexternal compression on the posterior and themedial walls, caused by a posterior mediastinalmass. CT chest was recommended, which revealeda posterior mediastinal cystic lesion (2–3 cm),suggesting a BC, a duplication cyst or aneuroenteric cyst (Fig. 3b).

The cyst was removed surgically. Pathologicalexamination demonstrated a cystic structure linedwith pseudostratified ciliated columnar epithelium(respiratory mucosa). The cyst wall consisted offibrous tissue, smooth muscle fibres and nerve fibres,with no malignant transformation (Fig. 3c).

After surgery, the infant was stable with no retractionor stridor and a normal chest percussion note. Her

Figure 3

(a) CXR AP shows bilateral overinflation. (b) A CT scan of the chest shows a well-defined, spherical, cystic lesion in the mediastinum. (c)Pathological examination of the wall of the resected cyst shows the respiratory epithelium lining with a pseudostratified columnar epithelium withcilia (H&E stain, ×400). AP, anteroposterior; CT, computed tomography; CXR, chest radiography.


breath sounds turned normal vesicular and equal onboth sides.

Final diagnosis: a bronchogenic cystCommentsCongenital cystic lung diseases are rare, and vary inpresentation and severity. They consist of PS, cysticadenomatoid malformation, congenital lobar emphy-sema and BC. These four anomalies present adifferent clinical picture, are often difficult todiagnose and require surgical management [12].Although there are many similarities in terms oftheir presenting features, there are particulardifferences between the diagnostic groups that areimportant to highlight, especially in relationship tothe approaches to imaging and the long-term outcome[13,14] (Table 1).

BCs are remnants of the primitive foregut containingtissue normally found in the trachea and thebronchi [15]. Maier [16] classified BCs by anatomiclocations: hailer, paraoesophageal, tracheal, carinal ormiscellaneous. Carinal cysts are most likely to producerespiratory problems and are therefore the mostfrequently reported of BCs [17].

Up to one-third of the BCs remain asymptomatic [13].Symptomatic cases usually manifest in early childhood,but symptoms can develop at any age. BCs can causesymptoms either due to mass effects or due to directpressure on an airway leading to air trapping.Secondary infection can also occur. Two-thirds ofthe patients are diagnosed due to symptoms such ascough, wheezing, stridor, dyspnoea, dysphagia or evenchest pain [14].

BCs can cause stridor, which is an alarming and prom-inent symptom that requires prompt management,

especially in newborns and young children. Thedifferential diagnosis of stridor in infants includescongenital lesions of the upper and the lowerairway, inflammatory or neoplastic lesions, vocalcord paralysis, FB and vascular lesions. Thepresence of mediastinal BCs should also beincluded in this list [13].

The diagnostic workup of each child presenting withnoisy breathing and/ or stridor includes a CXR andfluoroscopy with a barium oesophagogram (or the less-irritant water-soluble gastrographin, especially forinfants). If the CXR and the oesophagogram arenegative, upper-airway endoscopy is mandated [18].

Case 4A 3.5-year-old previously healthy male child waspresented with the complaint of recurrent wheezes,chest infections and abdominal distension. He receivedsalbutamol nebulizer treatment several times withoutimprovement. At the time of admission, the RR was 30breaths/min and the HR 90 beats/min. There was norespiratory distress, fever or cyanosis.

Examination revealed no signs of respiratory distress,but there was a diminished breath sound over the rightlung base. Bronchial breathing was heard over the rightmiddle lung zone with no dullness on percussion.

Laboratory investigation showed a total leukocyticcount of 5900 cells/mm3 (lymphocytes 67%),hemoglobin 12 g/dl and platelets 203 000/μl. The C-reactive proteinwas negative and arterial blood gaseswerenormal.

CXR of the right-sided hydropnemothorax/subphrenic abscess was suggested (Fig. 4a). Hence,

Figure 4

(a) CXR AP shows air and fluid levels with a thin wall (arrow) right-sided hydropnemothorax/subphrenic abscess. (b) CT chest revealsmultiple dilated intestinal loops in the right lower lung (arrow), mainlyposterior, with a mild leftward mediastinal shift. The size and theshape of the left lung were normal, with normal parenchyma onthe left lung. No masses or abnormal cystic air spaces were present.AP, anteroposterior; CT, computed tomography; CXR, chestradiography.


she was started on vancomycin infusion for 1 weekwithout change in the radiological findings; hence,CT chest was recommended. CT chest revealedmultiple dilated intestinal loops in the right lowerlung, mainly posterior, with a mild leftwardmediastinal shift. The size and the shape of the leftlung were normal, with normal parenchyma on the leftlung. There were no masses or abnormal cystic airspaces (Fig. 4b).

The patient was referred to the paediatric surgerydepartment for surgical correction of diaphragmatichernia.

Final diagnosis: delayed presentation of right-sidedcongenital diaphragmatic herniaCommentsCongenital diaphragmatic hernia (CDH) is generallyregarded as a neonatal emergency. There is, however, asmall percentage of patients, who present beyond theneonatal period. Late-presenting CDH is associatedwith a much wider spectrum of clinical presentation,encompassing various combinations of respiratory andgastrointestinal symptoms. The serious respiratorydistress occurring in CDH is related to pulmonaryhypoplasia, often bilateral, combined with persistentfoetal circulation and mechanical respiratory disorders[19]. In 5–10% of the patients, the presentation ofCDH is delayed, whereas 7–10% of the patients haveno symptoms [20,21]. Most asymptomatic CDH areright-sided, with the diaphragmatic defect covered bythe liver [20]. Patients with right CDH often livelonger, are asymptomatic and have a normal life[22]. The overall survival of patients with left CDHis about 60% (which makes left CDH a seriousneonatal problem associated with high mortality) [23].

The clinical presentation of delayed CDH is notspecific and the diagnosis is often overlooked. Thereis also a very good respiratory compensatorymechanism [20,24].

Our case was misdiagnosed initially as a lung abscessdue to suggestive CXR. Lung abscess is an uncommoncondition that can occur at any age. It is believed to beless common in children than in adults. Fever andcough consistently predominate, but are not universal.Unlike the situation with adults, haemoptysis isuncommon as a presenting feature in children withlung abscess. The physical signs elicited in a child withlung abscess most commonly include tachypnoea, a dullpercussion note or reduced air entry locally, fever andlocalized crepitations [25]. The basic diagnostic test fora lung abscess is CXR. However, to distinguish a lungabscess from an empyema, necrotizing pneumonia,sequestration, pneumatocoele or an underlyingcongenital abnormality such as a BC, a contrast-enhanced CT scan is usually considered to be theinvestigation of choice [26,27].

Case 5A 4-year-old male child was presented with low-grade fever, respiratory distress and cough for about3 weeks. There was no history of traveling abroad orof animal contact. He was diagnosed in a generalhospital as having pneumonia and receivedantibiotics and antipyretics for 3 weeks, butwith no improvement. His mother noticed

Figure 5

(a) Chest radiograph shows two opacities related to the right lung (arrows). (b) CT chest and abdomen shows multiple cystic lesions in the lungand the liver. (c) Pathological examination of the sampled fluid shows scattered RBCs, lymphocytes with scattered inflammatory cells and manyrounded structures with amorphous appearance are seen (arrow); some of them contain rounded eosinophilic granules (picture suggestive ofhydatid disease). CT, computed tomography; RBCs, red blood cells.


progressive abdominal enlargement, and the childcomplained of right hypochondrial pain. Anabdominal ultrasound was performed, whichrevealed a mildly enlarged liver with a large well-defined hypoechoic cystic lesion in the right lobemeasuring 8×7 cm with a clear fluid inside and nointernal echoes, which cope with a large simplebenign cyst.

The patient was referred to our hospital for furtherevaluation of nonresponding residual pneumonia.

On admission, he had a toxic look with pallor, andthe RR was 40 breaths/min (mild tachypnea). Nofever was documented. There was no jaundice,cyanosis, purpura/echymosis or oedema in thelower limbs.

An abdominal examination showed diffuse abdominalenlargement, hepatomegaly (the right lobe spanwas12 cm and the left span was 5 cm below the costalmargin), firm in consistency with rounded borders withno splenomegaly or ascites.

A chest examination revealed diminished breath soundwith an impaired note on percussion over the rightmiddle and the basal lung zone.

A CXR was performed (Fig. 5a), which revealed awell-defined homogenous rounded lesion in theright middle lung zone and a nonhomogenousopacity occupying the right lower lung zone.Laboratory results showed a total leucocytic countof 19 000/μl (lymphocytes 18%, polymorphs 71%,esinophils 2%, monocytes 9% and basophils 0%),hemoglobin 12.9 g/dl, platelets 342 000/μl, ESR 65/95 and the C-reactive protein was 50mg/dl, with anormal serum creatinine of 0.6 mg/dl.

A CT chest and abdomen (Fig. 5b) revealed a largewell-defined cyst in the right middle lung loberelated to the anterior chest wall measuring about6 cm in diameter; another similar cyst measuring6×10 cm was seen in the posterior segment ofthe right lower lung lobe and a third cyst in theright liver lobe (mainly the anterior sector),measuring 9×6×6.6 cm. All cysts showed an outer


thin nonenhancing wall with content of near waterdensity. CT brain was normal.

A malignant liver cyst or a mass with lung metastasiswas suspected, and so an ultrasound-guidedcytological aspiration of the liver cyst was perfo-rmed, which revealed scattered red blood cells,lymphocytes with scattered inflammatory cells andmany rounded structures with an amorphousappearance were seen; some of them contain rou-nded eosinophilic granules (picture suggestive ofhydatid disease; Fig. 5c).

He was referred to the Infectious Disease Unit and hereceived albendazole 15mg/kg/day for 6 months,which caused marked improvement with regard tohis symptoms. Surgical excision was not possibleinitially because of multiple lesions in the lung andthe liver, and so it was planned to be performed afterthe end of the medical treatment, but the patient waslost to follow-up.

Final diagnosis: cystic hydatidosis (multiple hydatid cysts)CommentsHuman cystic echinococcosis (CE) (cystic hydatidosis)is a chronic zoonotic disease that results from infectionwith the larval stage of the dog tapeworm, Echinococcusgranulosus. The disease is highly endemic in most of thecountries of the Mediterranean basin, including NorthAfrica and the Middle East [28,29].

In human CE, the liver is the main organ affected,followed by the lung tissue [30].

Patients with hepatic CE frequently exhibit nosymptoms because of the slow progression of thecysts [29]; therefore, they may be discovered onlyaccidentally and frequently have complicated formsof the disease. Pulmonary hydatidosis may berevealed during thoracic radiography.

Most symptoms of pulmonary CE are caused bymass effect from the cyst volume, which exertspressure on the surrounding tissues. The mostcommon symptoms described by the literature arecough (53–62%), chest pain (49–91%), dyspnoea(10–70%) and haemoptysis (12–21%). Othersymptoms described less frequently includedyspnoea, malaise, nausea and vomiting andthoracic deformations [31]. The majority of thechildren and adolescents with lung lesions areasymptomatic despite having lesions of impressivesize, assumedly because of a weaker immune

response and the relatively higher elasticity of thelung parenchyma in children and teenagers [32,33].

Radiological studies are the primary step in thedetection and the evaluation of pulmonary CE cysts.In CXRs, cysts are well defined as a rounded mass ofuniform density that occupies a part of one or of bothhemithorax. Combined chest and abdominal CT is abetter tool to recognize certain details of the lesions andtheir surrounding structures, helping to excludealternative differential diagnoses and can alsouncover additional smaller cysts that were notdetected by conventional CXR [34,35].

Surgery is the main therapeutic approach. Surgicaltreatment of CE has two goals: to remove the parasitesafely, to prevent intraoperative dissemination and totreat the bronchipericyst pathology and other associatedlesions [36,37].

Surgery may involve excision of the cyst or resection ofthe cyst and the immediate surrounding parenchyma.Despite the lack of consensus, the currently mostaccepted surgical treatment for lung CE is completeexcision using parenchyma-preserving methods, suchas cystostomy, intact cyst enucleation or removal afterneedle aspiration, preserving as much lung parenchymaas possible [38,39].

Resection techniques such as pneumonectomy andsegmentectomy should be reserved to cysts involvingthe whole hemithorax or the whole segment,respectively, and lobectomy should be performed onlyin large abscessed cysts. To avoid recurrences, the use ofpresurgical chemotherapy reduces the chancesof seedingand recurrence [40].

In children, for simple, accessible cysts, ultrasound orCT-guided percutaneous aspiration, instillation of ascolicidal agent and reaspiration is the preferredtherapy. For conventional surgery, the inner cyst wall(laminate and germinal layers) can be peeled easily fromthe fibrous layer. The cavity should then be topicallysterilized and either closed or filled with omentum.Nonpregnant patients with cysts not amenable toaspiration or surgery or with contraindications can bemanaged with albendazole (15mg/kg day divided twicedaily orally for 1–6 months maximum 800mg/day). Afavourable response occurs in 40–60% of the patients.Alveolar hydatidosis is frequently incurable by anymodality, but radical surgery such as partialhepatectomy or lobectomy may cure early limiteddisease. Liver transplantation is also an option fordisease confined to the liver. Medical therapy with


albendazole may slow the progression of alveolarhydatidosis, but if at all feasible, removal of theinfected tissue provides the best outcome. Somepatients have been maintained on long-termsuppressive therapy, but the infection generally recurs ifalbendazole is stopped [41].

ConclusionDifferent congenital and acquired lung lesions canmanifest with a wide spectrum of presentations andare an important cause of morbidity and mortality ininfants and children. There are many difficulties andchallenges encounteredwhenconfrontedwith suchcasesfor the first time. There is always a need for followingevidence-basedmanagement protocols for childrenwithpersistent respiratory symptoms. The diagnosis and themanagement of these patients usually required differentmodalities to reach the final diagnosis. However, thereshould be a high index of suspicionwhen the patient hasanabnormal/atypical presentation, a prolongedcourse ofthe disease or abnormal imaging findings. Differentavailable modalities ranging from CXR, CT, MRI tosurgical exploration and biopsy should be used to reachthe final diagnosis.

Healthcare providers (Paediatricians/General Practit-ioners) should also think of rare chest diseases. Theyshould also refer such patients to a paediatric specialist(pulmonologist) to help in the final diagnosis andspecific management, which may require invasiveprocedures or specific imaging.

AcknowledgementsAll authors contributed equally in the writing and therevision of this manuscript.

Financial support and sponsorshipNil.

Conflicts of interestThere are no conflicts of interest.

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Case report 223

Metastatic hepatoblastoma: a rare cause of lung mass in adultsUpadhyay Hinesh N.1, Vakil Abhay P.1,4, Sherani Khalid M.2,Sherani Faraha K.3, Babury Mohammed M.2

Hepatoblastoma is a pediatric malignant tumor of the liverwith very few cases reported in adults. There are no casereports on isolated metastatic lung involvement withhepatoblastoma in an adult who had been previouslytreated for the pediatric form of this disease. We report thecase of a 27-year-old asymptomatic man who presented tothe hospital after a motor vehicle accident. Imaging studiesrevealed bilateral lung masses. He had been treated forhepatoblastoma at the age of 10 years. Histopathologicexamination of the lung biopsy revealed embryonalsubtype of hepatoblastoma. Further imaging studies failedto reveal the presence of any concomitant liver lesions.Egypt J Bronchol 2016 10:223–224© 2016 Egyptian Journal of Bronchology



Keywords: adult, hepatoblastoma, lung mass

1Departments of Medicine, 2Pulmonary and Critical Care Medicine,

Jamaica Hospital Medical Center, 3Department of Paediatrics, Winthrop

Hospital Medical Center, New York, New York, 4Department of Critical Care

Medicine, Mayo Clinic, Rochester, Minnesota, USA

Correspondence to Hinesh Upadhyay, MD, Department of Medicine,

Jamaica Hospital Medical Center, 8900 Van Wyck Expy, Jamaica, NY

11418. Tel: +1 718–206-6000; fax: +1 718-206-6787;

e-mail: [email protected]

Received 28 September 2015 Accepted 5 October 2015

IntroductionHepatoblastoma, a rare malignant tumor of the liver, ismost commonly known to occur before the age of 5years [1]. Metastatic involvement of the lung is knownto occur in pediatric population and is associated with apoor prognosis. Hepatoblastoma is very rare in adults,with less than 40 reported cases [2]. Metastaticinvolvement of the lungs in such cases is even rarer.To the best of our knowledge, this is the only casereport describing an adult who had previously treatedfor pediatric hepatoblastoma presenting with anisolated metastatic lung involvement.






Case historyA 27-year-old nonsmoker African American manpresented to the hospital after being involved in amotor vehicle accident. Chest radiography andcomputed tomography (CT) of the chest wereperformed as a part of trauma protocol. Chestradiograph (Fig. 1) and CT of the chest (Fig. 2)revealed a left upper lobe mass and two masses onthe right side without any lymphadenopathy.Abdominal and pelvic CT showed postsurgicalchanges with an absent right lobe of the liver. Therewas no lymphadenopathy and no other hepatic orsplenic lesions. On further questioning, the patientdenied any pulmonary or systemic symptoms,including cough, dyspnea, hemoptysis, fever, andweight loss. The patient was known to have a righthepatic lobectomy for hepatoblastoma at the age of 10years. He had received four cycles of cisplatin anddoxorubicin. He had been followed up for 5 yearsafter the treatment and was not known to have anyrecurrence.

The patient was hemodynamically stable. He was wellbuilt and well nourished. There was no palpablelymphadenopathy. Abdominal examination revealed awell-healed scar in the right upper quadrant without anyorganomegaly. The rest of the physical examinationswere within normal limits. Laboratory tests includingcomplete blood counts and markers of liver and renalfunction were within normal limits. Because of thepatient’s previous history of hepatoblastoma, serumα-fetoprotein was evaluated and was found to be710 ng/ml (normal,

Figure 3

Histopathologic examination of the lung biopsy showing epitheliodand spindle neoplasm with a nested architecture.

Figure 1

Chest radiograph revealing a left upper lobemass and twomasses onthe right side without any lymphadenopathy.

Figure 2

Computed tomography (CT) of the chest revealing a left upper lobemass and twomasses on the right side without any lymphadenopathy.


DiscussionIsolated metastatic lung involvement in an adult withpreviously treatedpediatric hepatoblastomahasnot beendescribed in the literature, with this being the first andthe only case report describing such involvement.Because of the rarity of the disease, there are nostandardized guidelines for the management ofhepatoblastoma in adults or for the management ofpulmonary involvement in such patients [3].

Pulmonary inv

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