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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
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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
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Zedan (Mansoura Univ.)
Malak Shaheen (Ain Shams Univ.)Mamdouh Mahfouz (Cairo
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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.)
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(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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Solid-organ transplantation in HIV-infected patients. N Engl J Med.
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rst six authors followed by et al.Rose ME, Huerbin MB, Melick J,
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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
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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
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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
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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
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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.
ROSE for TBNA Hassan 209
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
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210 Egyptian Journal of Bronchology
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.
References1 Schmidt RL, Witt BL, Lopez-Calderon LE, Layfield LJ.
The influence of
rapid onsite evaluation on the adequacy rate of fine-needle
aspirationcytology: a systematic review and meta-analysis. Am J
Clin Pathol 2013;139:300–308.
2 Xia Y, Wang KP. Transbronchial needle aspiration: where are we
now? JThorac Dis 2013; 5:678–682.
-
ROSE for TBNA Hassan 211
3 Diacon AH, Schuurmans MM, Theron J, Brundyn K, Louw M, Wright
CA,Bolliger CT. Transbronchial needle aspirates: howmany passes per
targetsite? Eur Respir J 2007; 29:112–116.
4 Davenport RD. Rapid on-site evaluation of transbronchial
aspirates. Chest1990; 98:59–61.
5 Yasufuku K. Linear endobronchial ultrasound. In: Ernst A,
Herth FJ,editors. Principles and practice of interventional
pulmonology. NewYork, NY: Springer; 2013. 185–195. Available at:
http://link.springer.com/10.1007/978-1-4614-4292-9_18. [Accessed 5
April 2016].
6 Trisolini R, Cancellieri A, Tinelli C, de Biase D, Valentini
I, Casadei G, et al.Randomized trial of endobronchial
ultrasound-guided transbronchialneedle aspiration with and without
rapid on-site evaluation for lungcancer genotyping. Chest 2015;
148:1430–1437.
7 Diette GB, White P Jr, Terry P, Jenckes M, Rosenthal D, Rubin
HR. Utilityof on-site cytopathology assessment for bronchoscopic
evaluation of lungmasses and adenopathy. Chest 2000;
117:1186–1190.
8 Chin R Jr, McCain TW, Lucia MA, Cappellari JO, Adair NE,
Lovato JF,et al. Transbronchial needle aspiration in diagnosing and
staging lungcancer: how many aspirates are needed? Am J Respir Crit
Care Med2002; 166:377–381.
9 Diacon AH, Schuurmans MM, Theron J, Louw M, Wright CA, Brundyn
K,Bolliger CT. Utility of rapid on-site evaluation of
transbronchial needleaspirates. Respiration 2005; 72:182–188.
10 Baram D, Garcia RB, Richman PS. Impact of rapid on-site
cytologicevaluation during transbronchial needle aspiration. Chest
2005;128:869–875.
11 Cardoso AV, Neves I, Magalhães A, Sucena M, Barroca H,
Fernandes G.The value of rapid on-site evaluation during EBUS-TBNA.
Rev PortPneumol (2006) 2015; 21:253–258.
12 Trisolini R, Cancellieri A, Tinelli C, Paioli D, Scudeller L,
Casadei GP, et al.Rapid on-site evaluation of transbronchial
aspirates in the diagnosis ofhilar and mediastinal adenopathy: a
randomized trial. Chest 2011;139:395–401.
13 Yarmus L, van der Kloot T, Lechtzin N, Napier M, Dressel D,
Feller-Kopman D. A randomized prospective trial of the utility of
rapid on-siteevaluation of transbronchial needle aspirate
specimens. J BronchologyInterv Pulmonol 2011; 18:121–127.
14 Brundyn K, Koegelenberg CF, Diacon AH, Louw M, Schubert P,
BolligerCT, et al. Transbronchial fine needle aspiration biopsy and
rapid on-siteevaluation in the setting of superior vena cava
syndrome. DiagnCytopathol 2013; 41:324–329.
15 Griffin AC, Schwartz LE, Baloch ZW. Utility of on-site
evaluation ofendobronchial ultrasound-guided transbronchial needle
aspirationspecimens. Cytojournal 2011; 8:20.
16 Plit ML, Havryk AP, Hodgson A, James D, Field A, Carbone S,
et al. Rapidcytological analysis of endobronchial ultrasound-guided
aspirates insarcoidosis. Eur Respir J 2013; 42:1302–1308.
17 Nakajima T, Yasufuku K, Saegusa F, Fujiwara T, Sakairi Y,
Hiroshima K,et al. Rapid on-site cytologic evaluation during
endobronchial ultrasound-guided transbronchial needle aspiration
for nodal staging inpatients with lung cancer. Ann Thorac Surg
2013; 95:1695–1699.
18 Eapen GA, Shah AM, Lei X, Jimenez CA, Morice RC, Yarmus L, et
al.Complications, consequences, and practice patterns of
endobronchialultrasound-guided transbronchial needle aspiration:
results of theAQuIRE registry. Chest 2013; 143:1044–1053.
19 Joseph M, Jones T, Lutterbie Y, Maygarden SJ, Feins RH,
Haithcock BE,et al. Rapid on-site pathologic evaluation does not
increase the efficacy of
endobronchial ultrasonographic biopsy for mediastinal staging.
AnnThorac Surg 2013; 96:403–410.
20 Bruno P, Ricci A, Esposito MC, Scozzi D, Tabbì L, Sposato B,
et al.Efficacy and cost effectiveness of rapid on site examination
(ROSE) inmanagement of patients with mediastinal lymphadenopathies.
Eur RevMed Pharmacol Sci 2013; 17:1517–1522.
21 Oki M, Saka H, Kitagawa C, Kogure Y, Murata N, Adachi T, Ando
M. Rapidon-site cytologic evaluation during endobronchial
ultrasound-guidedtransbronchial needle aspiration for diagnosing
lung cancer: arandomized study. Respiration 2013; 85:486–492.
22 Sindhwani G, Rawat J, Chandra S, Kusum A, Rawat M.
Transbronchialneedle aspiration with rapid on-site evaluation: a
prospective study onefficacy, feasibility and cost effectiveness.
Indian J Chest Dis Allied Sci2013; 55:141–144.
23 Khurana KK, Kovalovsky A, Wang D, Lenox R. Feasibility of
dynamictelecytopathology for rapid on-site evaluation of
endobronchial ultrasound-guided transbronchial fine needle
aspiration. Telemed J E Health 2013;19:265–271.
24 Bonifazi M, Sediari M, Ferretti M, Poidomani G, Tramacere I,
Mei F, et al.The role of the pulmonologist in rapid on-site
cytologic evaluation oftransbronchial needle aspiration: a
prospective study. Chest 2014;145:60–65.
25 Minami D, Takigawa N, Inoue H, Hotta K, Tanimoto M, Kiura K.
Rapid on-site evaluation with BIOEVALUATOR(®) during endobronchial
ultrasound-guided transbronchial needle aspiration for diagnosing
pulmonary andmediastinal diseases. Ann Thorac Med 2014;
9:14–17.
26 Murakami Y, Oki M, Saka H, Kitagawa C, Kogure Y, Ryuge M, et
al.Endobronchial ultrasound-guided transbronchial needle aspiration
in thediagnosis of small cell lung cancer. Respir Investig 2014;
52:173–178.
27 Jeffus SK, Joiner AK, Siegel ER, Massoll NA, Meena N, Chen C,
et al.Rapid on-site evaluation of EBUS-TBNA specimens of lymph
nodes:comparative analysis and recommendations for standardization.
CancerCytopathol 2015; 123:362–372.
28 Mallya V, Kumar SP, Meganathan P, Shivkumar S, Mehta R. The
utility ofROSE (rapid on-site evaluation) in endobronchial
ultrasound (EBUS)-guided transbronchial needle aspiration (TBNA):
Is the picture rosy? JCytol 2015; 32:230–233.
29 Guo H, Liu S, Guo J, Li B, Li W, Lu Z, et al.Rapid on-site
evaluation duringendobronchial ultrasound-guided transbronchial
needle aspiration for thediagnosis of hilar and mediastinal
lymphadenopathy in patients with lungcancer. Cancer Lett 2016;
371:182–186.
30 Madan NK, Madan K, Jain D, Walia R, Mohan A, Hadda V, et al.
Utility ofconventional transbronchial needle aspiration with rapid
on-site evaluation(c-TBNA-ROSE) at a tertiary care center with
endobronchial ultrasound(EBUS) facility. J Cytol 2016;
33:22–26.
31 Rokadia H, Mehta A, Culver DA, Patel J, Machuzak M, Almeida
F, et al.Rapid on-site cytological examination (ROSE) in detection
of granulomasin the mediastinal lymph nodes. Ann Am Thorac Soc
2016; 13:850–855.
32 Kini SR. Color atlas of pulmonary cytopathology. New York,
NY: Springer;2002. Available at:
http://link.springer.com/10.1007/978-0-387-21641-6.[Accessed 4
March 2016].
33 Bonifazi M, Zuccatosta L, Trisolini R, Moja L, Gasparini S.
Transbronchialneedle aspiration: a systematic review on predictors
of a successfulaspirate. Respiration 2013; 86:123–134.
34 Casadio C, Guarize J, Donghi S, di Tonno C, Fumagalli C,
Vacirca D, et al.Molecular testing for targeted therapy in advanced
non-small cell lungcancer: suitability of endobronchial ultrasound
transbronchial needleaspiration. Am J Clin Pathol 2015;
144:629–634.
http://link.springer.com/10.1007/978-1-4614-4292-9_18http://link.springer.com/10.1007/978-1-4614-4292-9_18http://link.springer.com/10.1007/978-0-387-21641-6
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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
© 2016 Egyptian Journal of Bronchology | Published by Wolters
Kluwer
specific management, which may require invasiveprocedures or
specific imaging.Egypt J Bronchol 2016 10:212–222© 2016 Egyptian
Journal of Bronchology
Egyptian Journal of Bronchology 2016 10:212–222
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.
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.
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.
214 Egyptian Journal of Bronchology
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.
Difficult to diagnose cases Kandil et al. 215
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
216 Egyptian Journal of Bronchology
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.
Difficult to diagnose cases Kandil et al. 217
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.
218 Egyptian Journal of Bronchology
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.
Difficult to diagnose cases Kandil et al. 219
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
-
220 Egyptian Journal of Bronchology
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
-
Difficult to diagnose cases Kandil et al. 221
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.
References1 Zedan M, Elgamal MA, Zalata K, Nasef N, Fouda A.
Progressive stridor:
could it be a congenital cystic lung disease? Acta Paediatr
2009;98:1533–1536.
2 Zur KB, Litman RS. Pediatric airway foreign body retrieval:
surgical andanesthetic perspectives. Paediatr Anaesth 2009;
19:109–117
3 Fitzpatrick PC, Guarisco JL. Pediatric airway foreign bodies.
J La StateMed Soc 1998; 150:138–141.
4 Lima JA, Fischer GB. Foreign body aspiration in children.
Paediatr RespirRev 2002; 3:303–307.
5 Applegate KE, Dardinger JT, Lieber ML, Herts BR, Davros
WJ,Obuchowski NA, Maneker A. Spiral CT scanning technique in
the
detection of aspiration of LEGO foreign bodies. Pediatr Radiol
2001;31:836–840.
6 Kisku KH, Panigrahi MK, Sudhakar R, Nagarajan A, Ravikumar R,
Daniel JR.Agenesis of lung − a report of two cases. Lung India
2008; 25:28–30.
7 Sbokos CG, McMillan IK. Agenesis of the lung. Br J Dis Chest
1977;71:183–197.
8 Stern R, Berger S, Casaulta C, Raio L, Abderhalden S,
Zachariou Z.Bilateral intralobar pulmonary sequestration in a
newborn, case report andreview of the literature on bilateral
pulmonary sequestrations. J PediatrSurg 2007; 42:E19–E23.
9 Couriel J. Assessment of the child with recurrent chest
infections. Br MedBull 2002; 61:115–132.
10 Panitch HB. Evaluation of recurrent pneumonia. Pediatr Infect
Dis J 2005;24:265–266.
11 Zach MS, Eber E. Adult outcome of congenital lower
respiratory tractmalformations. Thorax 2001; 56:65–72.
12 Hashemzadeh S, Aslanabadi S, Jafari Rouhi AH, Azhough R,
Kaleibar NA.Congenital malformations of the lung. Indian J Pediatr
2007; 74:192–194.
13 Horak E, Bodner J, Gassner I, Schmid T, Simma B, Grässl G,
Sawyer SM.Congenital cystic lung disease: diagnostic and
therapeutic considerations.Clin Pediatr (Phila) 2003;
42:251–261.
14 Williams HJ, Johnson KJ. Imaging of congenital cystic lung
lesions.Paediatr Respir Rev 2002; 3:120–127.
15 ShanmugamG,MacArthur K, Pollock JC. Congenital
lungmalformations −antenatal and postnatal evaluation and
management. Eur J CardiothoracSurg 2005; 27:45–52.
16 Maier HC. Bronchogenic cysts of the mediastinum. Ann Surg
1948;127:476–502.
17 Estrera AS, Landay MJ, Pass LJ. Mediastinal carinal
bronchogenic cyst: isits mere presence an indication for surgical
excision? South Med J 1987;80:1523–1526.
18 Lazar RH, Younis RT, Bassila MN. Bronchogenic cysts: a cause
of stridorin the neonate. Am J Otolaryngol 1991; 12:117–121.
19 Levin DL. Morphologic analysis of the pulmonary vascular bed
incongenital left-sided diaphragmatic hernia. J Pediatr 1978;
92:805–809.
20 Osebold WR, Soper RT. Congenital posterolateral diaphragmatic
herniapast infancy. Am J Surg 1976; 131:748–754.
21 Ohura H, Kondo T, Iwabuchi S, Matsumura Y, Saito R, Okada Y,
et al. Twocases of the congenital posterolateral diaphragmatic
hernia were reported.Kyobu Geka 1996; 49:420–423.
22 Graivier L, Dorman GW, Votteler TP. Congenital diaphragmatic
hernia inchildren. Surg Gynecol Obstet 1971; 132:408–410.
23 Campbell WA, Sanders M, Rosenkrantz T, Leo MV, Odibo A, Ling
PY,et al. A center’s nine years outcome experience with
congenitaldiaphragmatic hernia. Am J Obstet Gynecol 1998;
178:163S
24 Golladay ES, Katz JR, Katz H, Haller JA Jr. Delayed
presentation ofcongenital posterolateral diaphragmatic hernia: a
dramatic cause offailure to thrive. J Pediatr Surg 1981;
16:503–505.
25 Patradoon-Ho P, Fitzgerald DA. Lung abscess in children.
Paediatr RespirRev 2007; 8:77–84.
26 Zuhdi MK, Spear RM, Worthen HM, Peterson BM. Percutaneous
catheterdrainage of tension pneumatocele, secondarily infected
pneumatocele,and lung abscess in children. Crit Care Med 1996;
24:330–333.
27 Trotman-Dickenson B. Radiology in the intensive care unit
(part 2). JIntensive Care Med 2003; 18:239–252.
28 Matossian RM, Rickard MD, Smyth JD. Hydatidosis: a global
problem ofincreasing importance. Bull World Health Organ 1977;
55:499–507.
29 Andersen FL. Introduction to cystic echinococcosis and
description ofcooperative research project in Morocco. In: Andersen
FL, Ouhelli H,Kachani M, eds Compendium on cystic echinococcosis in
Africa andMiddle Eastern countries with special reference to
Morocco. Provo, Utah:Brigham Young University; 1997. 1–17
30 Craig PS, Rogan MT, Allan JC. Detection, screening and
communityepidemiology of taeniid cestode zoonoses: cystic
echinococcosis, alveolarechinococcosis and neurocysticercosis. Adv
Parasitol 1996; 38:169–250.
31 Arinc S, Kosif A, Ertugrul M, Arpag H, Alpay L, Unal O, et
al. Evaluation ofpulmonary hydatid cyst cases. Int J Surg 2009;
7:192–195.
32 Arroud M, Afifi MA, El Ghazi K, Nejjari C, Bouabdallah Y.
Lung hydaticcysts in children: comparison study between giant and
non-giant cysts.Pediatr Surg Int 2009; 25:37–40.
33 Dopchiz MC, Elissondo MC, Andresiuk MV, Maiorini E, Gutiérrez
AM,Muzulin PM, et al. Pediatric hydatidosis in the south-east of
the
-
222 Egyptian Journal of Bronchology
Buenos Aires province, Argentina. Rev Argent Microbiol
2009;41:105–111.
34 Turgut AT,AltinokT, TopcuS,KosarU. Local complications of
hydatid diseaseinvolving thoracic cavity: imaging findings. Eur J
Radiol 2009; 70:49–56.
35 Zeyrek D1, Savas R, Gulen F, Demir E, Tanac R. ‘Air-bubble’
signs in theCT diagnosis of perforated pulmonary hydatid cyst:
three case reports.Minerva Pediatr 2008; 60:361–364.
36 Brunetti E, Kern P, Vuitton DA, Writing Panel for the
WHO-IWGE. Expertconsensus for the diagnosis and treatment of cystic
and alveolarechinococcosis in humans. Acta Trop 2010; 114:1–16.
37 Junghanss T, da Silva AM, Horton J, Chiodini PL, Brunetti E.
Clinicalmanagement of cystic echinococcosis: state of the art,
problems, andperspectives. Am J Trop Med Hyg 2008; 79:301–311.
38 Vasquez JC, Montesinos E, Peralta J, Rojas L, DeLaRosa J,
Leon JJ.Need for lung resection in patients with intact or ruptured
hydatid cysts.Thorac Cardiovasc Surg 2009; 57:295–302.
39 Dakak M, Caylak H, Kavakli K, Gozubuyuk A, Yucel O, Gurkok S,
et al.Parenchyma-saving surgical treatment of giant pulmonary
hydatid cysts.Thorac Cardiovasc Surg 2009; 57:165–168.
40 Dziri C, Haouet K, Fingerhut A, Zaouche A. Management of
cysticechinococcosis complications and dissemination: where is
theevidence? World J Surg 2009; 33:1266–1273.
41 Balanton R. Echinococcosis (Echinococcus granulosus and
Echinococcusmultilocularis). In: KliegmanRM,BehrmanRE,
JensonHB,StantonBF, eds.Nelson textbookofpediatrics.
18thed.Philadelphia,PA:SaundersElsevier;2007; chap 299:
516–1519.
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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
© 2016 Egyptian Journal of Bronchology | Published by Wolters
Kluwer
Egyptian Journal of Bronchology 2016 10:223–224
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.
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.
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.
224 Egyptian Journal of Bronchology
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