Prenatal ultrasound diagnosis of fetal chest wall cystic lymphangioma: An Italian case series

Full

length

article

Prenatal

ultrasound

diagnosis

of

fetal

chest

wall

cystic

lymphangioma:

An

Italian

case

series

Gabriele

Saccone

*

,

Lavinia

Di

Meglio

,

Letizia

Di

Meglio

,

Fabrizio

Zullo

,

Mariavittoria

Locci

,

Fulvio

Zullo

,

Vincenzo

Berghella

,

Aniello

Di

Meglio

a

DepartmentofNeuroscience,ReproductiveSciencesandDentistry,SchoolofMedicine,UniversityofNaplesFedericoII,Naples,Italy

b

DiagnosiEcograficaePrenatalediA.DiMeglio,Naples,Italy

c

DivisionofMaternal-FetalMedicine,DepartmentofObstetricsandGynecology,SidneyKimmelMedicalCollegeofThomasJeffersonUniversity,Philadelphia, PA,USA ARTICLE INFO Articlehistory: Received14March2019 Accepted16March2019 Availableonlinexxx Keywords: MRI Fetalmalformation Abortion Genetic NIPT ABSTRACT

Fetallymphangiomaisararecongenitalmalformationoflymphaticsystemthatinvolvetheskinandthe

subcutaneous tissue. The vast majority of the lymphangioma occurs in the neck. More rarely

lymphangiomasmayoccurintheaxillaryregion,includingchestwall.Ourconsecutivecaseseriesstudy

includedthreecasesoffetalchestwallcysticlymphangiomas.Inourcohort,fetalchestwallcystic

lymphangiomaswerethe18.8%oftheallcasesoflymphangiomasofaxillaryregion.Inallthethreecases

nootherfetalabnormalitieswereevaluated,andthechestwallcysticlymphangiomaswereunilateral,

honeycombedinappearance,withmultipleecho-freeareaofvaryingsizeinthemass,withnocolorflow

onDopplersonography,andwithatrendtoincreaseduringthegestation.Theincidenceofchromosomal

abnormalitieswas33.3%,withonecaseoutofthethreebeingtrisomy21.Theliteraturereviewrevealed

onlysevencasesoffetalchestwallcysticlymphangiomas.Thecaseswerenotassociatedwithother

abnormalities,norwithabnormalkaryotypeandonlyonecaseoffetaldeathwasreported.Threewomen

deliveredvaginally.Insummary,fetalchestwallcysticlymphangiomaisaveryraremalformationswith

onlysevencasesreportedintheliterature.Thismalformationisusuallynotassociatedwithabnormal

karyotype or other abnormalities and the neonatal outcome is favorable after surgical removal.

Spontaneousvaginaldeliverymaybeasafeapproachfordeliverywomenwithfetalchestwallcystic

lymphangioma.

Condensation:Fetalchestwallcysticlymphangiomaisaveryraremalformationswithonlysevencases

reportedintheliterature.Thismalformationisusuallynotassociatedwithabnormalkaryotypeorother

abnormalities and the neonatal outcome is favorable after surgical removal.Spontaneous vaginal

deliverymaybeasafeapproachfordeliverywomenwithfetalchestwallcysticlymphangioma.

©2019ElsevierB.V.Allrightsreserved.

Introduction

Fetal lymphangioma is a rare congenital malformation of lymphaticsystemthatinvolvetheskin andthe subcutaneous tissue.Fetuses with congenital lymphangioma have a signi fi-cantlyincreasedriskofchromosomalabnormalities,including trisomy21[1].

Thevastmajorityofthelymphangiomaoccursintheneck, andareusually namedas cystichygromas[2,3].More rarely lymphangiomasmay occur in the axillary region, including

chest wall, trunk, pelvic, extremities, and retroperitoneal area [1,4].Outcomes ofbabieswithfetal lymphangiomaand with normal karyotype is usually good, with success of surgical intervention depends mainly on size and location [1,2]. The chestwall cystic lymphangiomais a rare location offetallymphangiomas, withveryfewcasesreportedinthe literature [5].

Objective

Theaimofthiscaseseriesstudy,withliteraturereview,was to report all consecutive cases of fetal chest wall cystic lymphangioma referred to our institution fromJanuary2010 toJanuary2019.

* Correspondingauthorat:DepartmentofNeuroscience,ReproductiveSciences andDentistry,SchoolofMedicine,UniversityofNaplesFedericoII,Naples,Italy.

E-mailaddress:gabriele.saccone.1990@gmail.com(G.Saccone).

https://doi.org/10.1016/j.ejogrb.2019.03.014

0301-2115/©2019ElsevierB.V.Allrightsreserved.

EuropeanJournalofObstetrics&GynecologyandReproductiveBiology236(2019)139–142

ContentslistsavailableatScienceDirect

European

Journal

of

Obstetrics

&

Gynecology

and

Reproductive

Biology

Caseseries

Duringthestudyperiod,16casesoflymphangiomasofaxillary regionwereidentified.Ofthemthree(18.8%)werefetalchestwall cysticlymphangiomas.

Case1.A29-year-oldprimagravidawomanwasreferredtoour institution at32 weeks of gestationforthird trimester scan. Previous sonographyat 13weeks was consistentwith gesta-tional age, and nuchal translucency was 1.1mm. Second trimesteranatomyscanwasregular.

Ultrasoundexaminationshowed a multilocular,cystmassof 48x19mminsize,locatedlaterallyintherightsideofthefetusin theareaofthechestwall,withoutcolorflowimaging(Fig.1).The placentaappearedwithnoabnormalities,andtheamnioticfluid amount was normal. No other anomalies were identified. Amniocentesis revealed normal karyotype (46, XY). 34 weeks scanshowednormalfetalgrowthwithenlargementofthecystic mass to 54x31mm. The woman underwent planned cesarean deliveryat39weeksofgestationduetobreechpresentation,anda 3,250g-sizedmaleinfantwasdeliveredwithAPGARscoreof9and 9,at1stand5thminutes,respectively.Thepatientpostoperative coursewasuncomplicated,andshewasdischargedatday4after cesareandelivery.

Thepediatricsurgeondecidedforaconservativemanagement withobservationof thecystic mass.At6 monthsfollow-upthe babypresented for the follow-up, and the lymphangioma had decreasedinsize(Figs.2and3).

Case2.A38-year-oldmultiparouswomanwasreferredtoour institution at 16 weeks of gestation.First trimester scan was consistentwithgestationalage,andnuchaltranslucencywas 3.9mm.

16-weeksultrasoundexaminationshowedamultilocular,cyst massof1325x31mminsize,locatedposteriorlyontheleftside ofthefetusinthelowerchestwall,withoutcolorflowimaging.

Ultrasound scan also showed short femur, and common atrioventricularcanal.

Amniocentesisrevealedtrisomy21,andthepatientoptedfor inducedterminationofpregnancy.Macroscopicandmicroscopic evaluationsrevealedafetalchestcavernouslymphangioma.

Case3.A27-year-oldprimagravidawomanwasreferredtoour institution at 22 weeks of gestation for second trimester anatomyscan.Previoussonographyat12weekswasconsistent withgestationalage,andnuchaltranslucencywas1.5mm.At13 weeksnon-invasiveprenataltestingusingcell-freefetal DNA showednormalkaryotype(46,XX).

The22-weekscanshowednormalgrowth,withtheamountof amnioticfluidandfetalmovementsshowingnoabnormalities.An abnormalunilateralleft-sidemassconnectedtothefetalchestof 1729x35mm was identified. The color Doppler revealed no flow.Nootherabnormalitieswereidentified.

Thecoupledecidedtointerruptthegestationbecauseofthe abnormalfetalfindinganddeclinedamniocentesis,andevaluation oftheabortedfetuses.

Literaturereview

The search for the systematic review was conducted using MEDLINE,EMBASE,Scopus,ClinicalTrials.gov,OVIDandCochrane Libraryaselectronicdatabases.Thecitationswereidentifiedwith theuseofacombinationofthefollowingtextwords:“fetalchest,” “lymph,” “lymphangioma,” “chest wall,” “circumscriptum” and “hygromas” from inception of each database through February 2019.Reviewofarticlesalsoincludedtheabstractsofallreferences retrieved from the search. No restrictions for language or geographiclocationwereapplied.

Six studies,[5–10] includingseven cases,were identified as relevantandincludedinthesystematicreview(Table1,Fig.4).Five of thesixstudieswerecase reports,while Goldsteinetal. [10] includedtwocases.Gestationalageatdiagnosisrangedfrom15to 38 weeksofgestation.Three caseswerediagnosedin thethird trimester, and in all of them second trimester anatomy scan revealednoabnormalities.

Fig. 1.Sonographicappearanceoffetalchestwallcysticlymphangiomaat32weeks ofgestation(Case1).

Fig.2.Appearanceofchestwallcysticlymphangiomaatthe6-monthsvisit follow-up,laterview(Case1).

Fig.3.Appearanceofchestwallcysticlymphangiomaatthe6-monthsvisit follow-up,frontalview(Case1).

In all the cases the mass was described as a multilocular septatedcysticmasshoneycombedinappearance,withmultiple echo-freeareaofvaryingsizeinthemass,andwithnocolorflow onDoppler.Sizeofthemassrangedfrom6mmtothegiantmassof 123mmdescribedbyLuetal.Inallthecases,themassesincreased throughthegestation.

Spontaneousvaginaldeliverywasthemostcommonmodeof delivery(3/7).Twowomenunderwentplannedcesareandelivery atterm,onewomanoptedforinducedterminationofpregnancy, andinonecaseat25weeksultrasoundexaminationrevealedfetal death with pleural effusion and labor was induced by using laminariatentsandprostaglandinE1.

Inallthecasesnoassociatedabnormalitieswerereportedatthe timeoftheultrasoundscans,butautopsyofthecasereportedby Hayashietal.revealedsyndactylyoftheleftthirdandfourth_finger. Noabnormalkaryotypewasreportedinanyoftheincludedcases. In the 6 cases of the live-born infantssurgical removal was successfulperformed.

Discussion

Ourconsecutivecaseseriesstudyincludedthreecasesoffetal chestwallcysticlymphangiomas.Inourcohort,fetalchestwall cystic lymphangiomas were the 18.8% of the all cases of lymphangiomasofaxillaryregion.Inallthethreecasesnoother

fetal abnormalities were evaluated, and the chest wall cystic lymphangiomas were unilateral, honeycombed in appearance, withmultipleecho-freeareaofvaryingsizeinthemass,andwith nocolorflowonDopplersonography.Theincidenceof chromo-somal abnormalities was33.3%, withonecase out of thethree beingtrisomy21.

Theliteraturereviewrevealedonlysevencasesoffetalchest wallcysticlymphangiomas.Thecaseswerenotassociatedwith otherabnormalities,norwithabnormalkaryotypeandonlyone case of fetal death was reported. Three women delivered vaginally. Notably, Kaufam et al. performed decompression of the fetal axillary lymphangioma before delivery to prevent dystocia.[8]

Lymphangiomas can occur anywhere in the skin and the mucousmembranes.Morethan80%ofthefetallymphangiomas arelocatedintheneck,andfetalchestwallcysticlymphangiomas areveryrare.Althoughhistologicallyitisabenigndisorder,ithasa propensityforrapidgrowthandlocalinvasionintothemuscle,and bone and may lead to decreased quality of life. Fetal chest lymphangiomasmaybeunilocularormultilocularandthelesions rage in size from several millimeters to much larger giant lymphangioma[1,2].Thebiggerfetalchestwallcystic lymphan-giomareportedintheliteraturewas123mm(Table1).

A significant association betweenlymphangiomasand chro-mosomalabnormalitieshavebeenreportedintheliterature.The

Table1

Studiesincludedinthesystematicreview. Study location Maternal age GAat diagnosis Characteristicsofthe cyst

Sizeofthecyst atdiagnosis

Sizeofthecystat USfollow-up Abnormal karyotype Associated abnormalities Pregnancy outcome Neonatal outcome Rasidaki 2004[5]

Greece 25years 28weeks Multilocular unilaterallocated posteriorlyontheleft sideofthefetalchest

1022x29mm 133343at30 weeks No No Scheduled CDat38 weeks Surgical removalat2 months Masood 2010[6]

Pakistan 38years 38weeks Multilocular unilateralmass extendingfromthe scapulatothe lumbosacralregionon theleftsideofthe fetalchestwall

140x110mm – No No SVDat40 weeks Surgical removalat day4 Hayashi 2005[7]

Japan 28years 18weeks Multilocularbilateral locatedanteriorlyon thefetalchest

54x30mm (left); 34x30mm (right) 94x45mm(left); 70x48mm (right);at24 weeks No Syndactylyof theleftthird andfourth finger IUDat25 weeks – Kaufman 1996[8]

USA 30years 36weeks Septatedcysticmass intheleftfetalchest

78x6mm – No No SVDat38

weeks

Surgical removalat weeks2 Lu2015[9] China 31years 19weeks Multilocular

unilaterallocatedon theleftsideofthe fetalchest 2932x31mm 12392x110mm No No Scheduled CDat38 weeks Surgical removalat day4 Goldstein 200610 # #1

Israel 25years 15weeks Multilocularbilateral locatedonthefetal chest

Notreported Notreported No No I-TOP –

Goldstein 200610 #

#2

Israel 30years 22weeks Unilateralmass locatedonfetalchest

Notreported Notreported No No SVDat40

weeks

Surgical removala month1 Saccone#1 Italy 29 32weeks Multilocular

unilateralmass locatedontheright sideofthefetalchest

48x19mm 54x31mmat34 weeks No No Scheduled CDat39 weeks Expectant management

Saccone#2 Italy 38 16weeks Multilocular unilaterallocated posteriorlyontheleft sideofthefetalchest

1325x31mm – Trisomy 21 Common atrioventricular canal I-TOP –

Saccone#3 Italy 27 22weeks Multilocular unilaterallocated posteriorlyontheleft sideofthefetalchest

1729x35mm – Not

performed

No I-TOP –

US,ultrasound;GA,gestationalage;SVD,spontaneousvaginaldelivery;IUD,intrauterinefetaldeath;CD,cesareandelivery;I-TOP,inducedterminationofpregnancy. G.Sacconeetal./EuropeanJournalofObstetrics&GynecologyandReproductiveBiology236(2019)139–142 141

mostcommontypeisTurnersyndromeandotherabnormalities include trisomy 21,trisomy 18, trisomy 13, triploidy, and copy number variants. Therefore, when lymphangiomas were diag-nosedinthe antennalperiod, karyotypeexaminationis recom-mendedalongwithserialultrasoundexaminations.Ourincaseof fetal chest wall cystic lymphangiomas no cases of abnormal karyotype were reported.In ourcase series the onlyone case associatedwithabnormalkaryotype,beingtrisomy21,shortfemur andcommonatrioventricularcanalwerenoticed.

Regardingtheneonataloutcome,thefavoredtreatmentforfetal chest lymphangiomas is complete surgical excision. However, expectantmanagementmaybeofferedtothesmall lymphangio-mas.Recentadvanceinsclerotherapyhaveexpanded contempo-rarymanagementoptions.

Conclusion

Fetalchestwallcysticlymphangiomaisaveryrare malforma-tions with only seven cases reported in the literature. This malformationisusuallynotassociatedwithabnormalkaryotype orotherabnormalitiesandtheneonataloutcomeisfavorableafter surgical removal. Spontaneous vaginal delivery may be a safe approach for delivery women with fetal chest wall cystic lymphangioma.

Disclosure

Theauthorsreportnoconflictofinterest. Financialsupport

Nofinancialsupportwasreceivedforthisstudy. References

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