RevPaulPediatr.2016;34(2):243---246
REVISTA
PAULISTA
DE
PEDIATRIA
www.rpped.com.br
CASE
REPORT
Atypical
presentation
of
intra-abdominal
extralobar
pulmonary
sequestration
detected
in
prenatal
care:
a
case
report
Márcio
Rodrigues
Costa
∗,
Théo
Rodrigues
Costa,
Mauricio
Sérgio
Brasil
Leite,
Fernandes
Rodrigues
de
Souza
Filho,
Alexandre
Magno
Bahia
Reis,
Bruno
Paiva
Pereira,
Arthur
Magalhães
de
Oliveira
HospitaldasClínicas,UniversidadeFederaldeGoiás(UFG),Goiânia,GO,Brazil
Received27February2015;accepted20April2015 Availableonline24February2016
KEYWORDS Bronchopulmonary sequestration; Abdominal malignancies; Congenital abnormalities; Child
Abstract
Objective: To describe an unusual clinical presentation of intra-abdominal extralobar pul-monary sequestration ina 2-year,9month-oldpatient andassessdiagnosticandtreatment aspectsofthispathology.
Casedescription: Anundefinedintra-abdominalmasswasidentifiedintherightadrenalregion inamalefetus.Postnatalevaluationwithultrasoundimages,computedtomography,magnetic resonanceimagingandlaboratorytestingwasinsufficienttodeterminethenatureofthelesion. Aftertwoyears,laparoscopicresectionofthemassandhistopathologicalexaminationofthe surgicalspecimenallowedtoestablishthediagnosisofintra-abdominalextralobarpulmonary sequestration.
Comments: Thismalformationcanbemonitoredclinically;however,surgicalexcisionisoften performed,probablyduetotheimpossibility ofattaining diagnosiswithnon-invasive meth-ods, such as in the present case, inwhich thelesion appeared in anunusual position for intra-abdominalextralobarpulmonarysequestration.Therefore,thesurgicalapproachseems to bethe keytoattain thediagnosis and establishthe conduct for thistype ofcongenital malformation.
©2015SociedadedePediatriadeSãoPaulo.PublishedbyElsevierEditoraLtda.Thisisanopen accessarticleundertheCCBYlicense(https://creativecommons.org/licenses/by/4.0/).
∗Correspondingauthor.
E-mail:[email protected](M.R.Costa).
http://dx.doi.org/10.1016/j.rppede.2016.02.008
244 CostaMRetal.
PALAVRAS-CHAVE Sequestro
broncopulmonar; Neoplasias abdominais; Anormalidades congênitas; Crianc¸a
Apresentac¸ãoatípicadesequestropulmonarextralobarintra-abdominaldetectado nopré-natal:relatodecaso
Resumo
Objetivo: Descrever apresentac¸ãoclínica incomumdesequestropulmonar extralobar intra-abdominalem um paciente dedoisanosenovemeseseavaliaraspectosdiagnósticosede tratamentodessapatologia.
Descric¸ãodocaso: Umamassaintra-abdominalindefinidaemtopografiasuprarrenaldireitade fetomasculino.Aavaliac¸ãopós-natalcomimagensdeultrassom,tomografiacomputadorizada, ressonância magnética etestes laboratoriais não foi suficiente para determinara natureza dalesão.Apósdoisanos, aressecc¸ãolaparoscópicadamassa eoexamehistopatológicodo espécime cirúrgico permitiramestabelecer odiagnóstico de sequestropulmonar extralobar intra-abdominal.
Comentários: Essamalformac¸ãopodesermonitoradaclinicamente;entretanto,aexcisão cirúr-gica frequentemente é feita, provavelmente devido à impossibilidade de diagnóstico com métodos não invasivos, como ocorreu nopresente caso,na qual a lesão apresentou-se em posic¸ãonãohabitualparasequestropulmonarextralobarintra-abdominal.Dessemodo,a abor-dagemcirúrgicapareceserachaveparaodiagnósticoeaconduc¸ãodesdetipodemalformac¸ão congênita.
©2015SociedadedePediatriadeSãoPaulo.PublicadoporElsevierEditoraLtda.Esteéumartigo OpenAccesssobalicençaCCBY(https://creativecommons.org/licenses/by/4.0/deed.pt).
Introduction
Pulmonary sequestrationis arare eventanditsincidence in live births is estimated to be 0.15---1.7%.1 This con-genital malformation is characterized by focal areas of dysplasia and nonfunctioning pulmonary parenchyma, not connectedwiththebronchialtreeor pulmonaryarteries.2 Extralobarsequestration, represented by 25% of cases of pulmonary sequestration, is characterized by pulmonary parenchymainvolvedbypleuraindependentofthenormal lung.3,4 Among thecases of extralobarpulmonary seques-tration,8% arebelowthe diaphragm.This presentation is extremelyunusualandnot allof itsdiagnostic and thera-peuticaspectshavebeenclarified.5
Intra-abdominal extralobar sequestration is usually asymptomatic and complications such as malignancy or infection are exceptionally rare.6,7 The most often per-formed treatment is surgical resection; probably because mostcases need histopathological analysis toconfirm the diagnosis.8,9 Therefore, intra-abdominal extralobar pul-monarysequestrationispotentiallyagoodcandidateforan expectantconduct.
Therefore,theaimofthereportwastodescribeacase ofintra-abdominalextralobarpulmonarysequestrationand highlighttheaspectsofdiagnosis andmanagementofthis malformation.
Case
description
A25-year oldpregnantwomanin thesecond trimester of pregnancyof amalefetus, without complications, under-wentroutineprenatalultrasoundthatdetectedamassinthe rightadrenal glandofthefetus. Vaginaldeliveryoccurred
atterm(40weeks)withoutcomplications.Thenewbornwas submittedto postnatalultrasound onday oflife 5, which showeda2.6cmovalmassinitslargestdiameter, peripher-allyechogenicwithananechoiccore.Abdominalcomputed tomographyassessmentcarriedoutatdayoflife30,showed ahypodensenodule(60pre-contrastHounsfieldunits),with slightenhancementafterintravenouscontrast administra-tion.Throughthismethod,thelesionshowedtobe2.5cm initslargestdiameterandnoapparentcleavageplanewith therightliverlobeorright adrenal.Themetabolic assess-ment, which included themeasurement ofsex hormones, catecholamines, cortisol,aldosterone andmetanephrines, performed before the computed tomography (CT) assess-ment, showed no abnormalities. All monitoring and all examinations in the perinatal and neonatal periods were carried out at another health care service. After a long timewithoutfollow-upcare,thepatientreturnedfor assis-tanceattwoyearsandninemonthsofage,whenmagnetic resonanceoftheupperabdomenwasrequested.This exam-inationshowedthepresenceofanodulewiththefollowing characteristics: largest diameter of 3.4cm,located above theright adrenal,partiallydefinedcontours,hyperintense signal onT2, hypointenseonT1,heterogeneous enhance-mentafterintravenouscontrastinjectionandnocleavage plane with the right liver lobe or right adrenal (Fig. 1). Theadrenalmetabolicassessmentperformedatthattime, remainedunaltered.
Atypicalpresentationofintra-abdominalextralobarpulmonarysequestration 245
Tumor
Adrenal Liver
Kidney
Figure1 Closeassociationbetweenintra-abdominal extralo-bar pulmonary sequestration and right adrenal gland in magneticresonanceimaging(T2images).
regionabovetherightkidney.Afterdissection,contactof themasswiththediaphragmaticcrusandtherightliverlobe wasobservedsuperiorly,withnoadherencetotheseorgans, whileinferiorly,thelesion showednocleavageplanewith therightadrenal.Themasswasremovedtogetherwiththe adrenalglandandthentheywereseparatedonthesurgical table.Major,higher-caliberanddominantvesselswerenot identifiedduringtheprocedure.Thematerialwassentfor histopathologicalanalysisandthegrossexaminationshowed thepresenceofan11gtumorwith4.3cminitslargest diam-eter,ofbrowncolorandregular,smooth,heterogeneousand shinysurface.Microscopicexaminationshowedthepresence oflungtissuewithalveolifilledwithmacrophages(Fig.2). Theadrenalidentifiedinthehistopathologicalexamination showedusualaspect. The resultsfoundin thehistological sectionsdefinedthediagnosisofintra-abdominalextralobar pulmonary sequestration.The patientrecovered unevent-fullyandwasdischargedonthe2ndpostoperativeday.
Figure 2 Microscopy of intra-abdominal extralobar pul-monary sequestration showingrespiratory epithelium (arrow) andhyalinecartilage(asterisk),400×magnification.
Discussion
Abdominal tumors, including intra-abdominal extralobar pulmonary sequestration, are unusual among the malfor-mations of the perinatal period. According to Teel and Share,only5%offetalabnormalitiescorrespondto abdom-inal tumors visualized at the prenatal ultrasonography.10 Althoughinfrequent,morethan90%ofthetumorsdetected inthisphasearediagnosedcorrectly;gestationalage, ultra-sonographiccharacteristics, the location,blood supply of themassesandtheresultsoflaboratorytests,suchas mea-surementofvanillylmandelicacidareassessed.11
Theevaluationofabdominalmassesinneonatescanbe carriedoutby differentimagingmethods,but usuallythe ultrasoundexaminationisrequestedinitially.12 Tumorsthat representintra-abdominalextralobarpulmonary sequestra-tionattheultrasoundcommonlydisplayhyperechogenicity andsometimes a thinhyperechoic halo.9,13 When Doppler is added to the ultrasound evaluation, one can detect the arterial blood supply that originates from the aorta andestablishesthediagnosisofintra-abdominalextralobar pulmonarysequestration.9According tosome researchers, thereareno characteristic ultrasoundimages that define intra-abdominal extralobar pulmonary sequestration.14 In this case, the ultrasound images in the postnatal period showedanechogenichaloofthemasswithoutotherfindings thatmight indicate intra-abdominalextralobar pulmonary sequestration, whereas the lesion topography suggested thatitwasan adrenal neoplasia.This method,therefore, didnotallowthecorrectdiagnosistobeattained.
Additional tests to the perinatal ultrasound may be neededtoassessintra-abdominalmassesinneonates.12CT and/or magnetic resonance imaging of the abdomen are usually the subsequent tests used for this purpose. The diagnosis of intraabdominal extralobar pulmonary seques-trationthroughCTcanbedefinedbythepresenceofmass heterogeneity,withenhancement after contrastinjection associatedwiththeidentificationofbloodsupply originat-ingfromthe aorta.12 AccordingtoAmitaietal.,however, there are no CT and magnetic resonance images that unequivocallyrepresentthemalformation.15Supportingthis concept,thedatareportedbyChan etal., inareview of 13 cases of intraabdominal extralobar pulmonary seques-tration, showed that the findings of imaging tests were unspecific.16
ItisinterestingthatamongthecasesreviewedbyChan et al., only one was located on the right.16 This report showsthelesionintherightadrenalregion,consideredan unusualclinicalpresentationforintraabdominalextralobar pulmonarysequestration,makingitdifficulttosupposethe characteristicsofthisneoplasm.Probablytheunusual posi-tionoflesionandtheabsenceofcharacteristicaspectsat thecomputedtomographyand/ormagneticresonance imag-ingpreventedtheearlyattainmentofthediagnosis.
246 CostaMRetal.
of intra-abdominal extralobar pulmonary sequestration through imaging tests and/or biopsy without surgery is, however,theidealmethodofapproachinthesecases.15,18 Complicationsrelatedtoasymptomatic pulmonary seques-tration, not connected with the lung such as malignant degeneration, are rare and therefore the conservative management of the malformation is an advantageous possibility.7 How to diagnose and treat intra-abdominal extralobar pulmonary sequestration remains a controver-sialtopic.19 In thepresent case, thelaparoscopic surgical excision of the mass allowed the definitive diagnosis to beattained.Histopathologicalexaminationof thesurgical specimenconfirmedthenatureofthelesion.
Therefore,itcanbeconcludedthat,althoughitis com-montoestablishthenatureofabdominalmassesinneonates andfetusesthroughnoninvasivemethods,inthispatientit wasnotpossibletodeterminethenatureofmassevenafter theusingseverallaboratory andimagingtests.Inthecase ofintra-abdominalextralobarpulmonarysequestration, sur-gical excision seems to be the key to the diagnosis and managementofthesecases.
Funding
Thisstudydidnotreceivefunding.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
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