Braz. j. . vol.83 número3

BrazJOtorhinolaryngol.2017;83(3):364---366

www.bjorl.org

Brazilian

Journal

of

OTORHINOLARYNGOLOGY

CASE

REPORT

First

branchial

cleft

fistula:

a

difficult

challenge

夽

Fístula

na

primeira

fenda

branquial:

um

difícil

desafio

Corneliu

Mircea

Codreanu

,

Corneliu

Codreanu

,

Margareta

Codreanu

a_{Br˘ailaCountyHospital,DepartmentofOtolaryngology,Braila,Romania} b_{‘Dr.Codreanu’ENTClinic,Galati,Romania}

Received2June2015;accepted16July2015 Availableonline17October2015

Introduction

Firstbranchial cleftanomalies accountfor 8---10%1,2 _ofall

branchialcleftdefects,representingapproximately17%of

all pediatric cervical masses. The low incidence and the

variousclinicalpresentationsofthesecongenital

malforma-tionsofthebranchialapparatusmakethediagnosisdifficult

evenfortheexperimentedotolaryngologist. Dependingon

thetypeoftheanomaly(fistula,sinusorcyst),thefistulous

tractcanbelocatedmedialorlateraltothefacialnerve.

CTscansprovideusefulinformationonthelocationofthe

fistuloustract.

WereportararecaseoftypeIIfirstbranchialcleftfistula

presentingasatractwith2openings---acervicalopeningin

theleftsubmandibularregionandasmallfistulousopening

atthe floorof thecartilaginous portionof theleft

exter-nalauditorycanal(EAC).Treatmentconsistedincomplete

excisionofthefistuloustractandsuperficialparotidectomy

withpreservationofthefacialnerve.

夽

Pleasecitethisarticleas:CodreanuCM,CodreanuC,Codreanu M.Firstbranchialcleftfistula:adifficultchallenge.BrazJ Otorhi-nolaryngol.2017;83:364---6.

∗_{Correspondingauthor.}

E-mail:[email protected](C.M.Codreanu).

PeerReviewundertheresponsibilityofAssociac¸ãoBrasileirade OtorrinolaringologiaeCirurgiaCérvico-Facial.

Case

report

A4year-oldgirlpresentedintheENTclinicwithaleftupper

neckmassthatwasfirstnoticed6monthsago.Theparents’

interrogatory revealed that during acute rhinopharyngitis

episodesthemassbecameredandswollen,andawhitish,

recurrentdischargeleakedbothfromasmallopeninginthe

left laterocervical region and from the left EAC. Clinical

examinationshowedthatthemasswasovoidal,measuring

2.5---1cm,fluctuantandrelativelymobileondeepcervical

structures. Asmall cutaneousopening wasnoticed in the

leftsubmandibularregion.

Otoscopyrevealedasmalldepressiononthefloorofthe

leftEACwithanintacttympanicmembrane.

CTscansshowedafistulous tractconnectingthe

carti-laginousportionoftheleftEACtotheupperlaterocervical

regionthatcrossedthedeepparotidlobe.Thediagnosisof

firstbranchialcleftfistulawasmade.

Undergeneralanesthesia,thefistuloustractwasexcised

using aclassic parotidectomy incisiondescribed by Adson

andOtin1923andmodifiedbyRedonin1955.Becauseof

thedistancebetweentheincisionandtheinferiorcutaneous

openingofthefistula,anellipticalexcisionoftheopening

wasalsoperformed(Fig.1).

Aftertheidentificationanddissectionofthefacialnerve

trunk, we performed a superficial parotidectomyand

dis-covered the fistulous tract lying under the facial nerve

branches. The fistulous tract was excised along with the

http://dx.doi.org/10.1016/j.bjorl.2015.07.017

Firstbranchialcleftfistula:adifficultchallenge 365

Figure1 Intraoperative view:thebluearrowindicates the fistuloustract,thegreenarrowindicatestheleftfacialnerve trunkandtheblackarrowshowstheellipticalexcisionofthe inferioropeningofthefistula.

inferior cutaneous opening, taking care to preserve the

branchesofthefacialnerve.Finally,thesuperioropening

ofthefistulawasexcisedtogetherwithasmallportionof

thecartilaginousflooroftheleftEAC.

The postoperative care was uneventful. Histological

examination of the specimen revealed that the fistulous

tractwaslinedbysquamousepitheliumwithadnexal

struc-tures and cartilage disposed in hyaline lamellae, thus

confirmingthediagnosisoftypeIIfirstbranchialcleftfistula.

Discussion

Anomaliesoffirstbranchialcleftarerareentitiesresulting

fromincompleteclosure oftheectodermalportionof this

cleft. The first description of theseanomalies dates back

from1865(Virchow).In1929,HyndmanandLightgathered

108casesfromthemedicalliterature.

In1971, Arnotclassifiedtheanomaliesofthefirstarch

andfirstbranchialcleftin:type1anomalies(encountered

mostlyinadults),consistinginapretragal cystdrainingin

the parotid area,and type 2 anomalies (usually

develop-ing during childhood), appearing in the anterior cervical

triangle.

ThehistologicalclassificationwasmadebyWork:typeI

---ectodermaloriginoftheanomaly(squamousepithelium),

and type II --- ectodermal and mezodermal origin

(squa-mous epithelium,adnexial structuresandcartilage). Type

Ianomalypresentsasanepidermoidcystorfistulalocated

intheperiauralregion,withafistuloustractparalleltothe

EACthatendsinacul-de-sacatthelevelofthe

mesotym-panum.TypeIIlesionspresentasacyst,sinus(tractwithan

externalopening),afistuloustractwithasuperioropening

atthelevel ofthebony---cartilaginousjunctionoftheEAC

or a combination of theseelements. The inferioropening

oftypeIIlesions(asinourcasereport)issituatedina

tri-angularareadescribedbyE.Poncetthatisboundedbythe

EACabove,thementalregionanteriorlyandthehyoidbone

inferiorly.3_{ItisalsoimportanttoknowthattypeIIanomalies}

aresituatedabovethevascularstructuresoftheneckand

thedigastricmuscle,buttheirpositionregardingthefacial

nerveisvariable,withthenervesituatedabove,belowor

evencrossedbythelesion.

Finally,inathirdclassificationOlsendividedthedefects

ascysts,sinusesorfistulas.

The analysis of clinical manifestations (cervical ---

usu-allyalittledepressionnearthemandibularangle; parotid

--- small masses located in the parotid area, and

auricu-lar --- otorrhea and sometimes a membranous attachment

betweentheflooroftheEACandthetympanicmembrane

---10%ofthecasesintheseriesofTriglia),cervicalandparotid

areaCT scans or MRI and the fact that these lesions are

rarelyassociatedwithotherfacialmalformationscanlead

tothe diagnosis offirstbranchial cleftanomalies. In

chil-dren, differential diagnosis includes congenital ear cysts,

preauriculartagsandsecondbranchialcleftanomalies.

Concerningourcase,thelocationofthecervicalmass,its

characteristics,theotoscopicfindingsalong withCTscans

providedsolidelementsinfavorofthediagnosis.

It is generallyadmitted that fistulas areoften located

medialtothenerve,whereassinustractstendtorunlateral

toit.4,5_{Basedontheseobservations,weperformeda}

com-pletesurgical excision ofthefistulous tracttogether with

thesuperficialparotidlobe,becauseofthepositionofthe

mass(belowthe facialnerve).Thisparticular situationof

theanomalyexplainsthedifficultyofthiskindofsurgery,in

whichfacialnervedamagecanoccur(40%intheseriesof

Ford2_{and15%intheseriesofTriglia).}

Wemustinsistonthefactthatthereisasecondproblem

concerningthe completeexcision oftheselesionswithout

damaging the nerve, and that is in relationship with the

child’sage:theyoungerthepatient,themastoidtipisnot

completelydevelopedandthefacialnervetendstohavea

moresuperficialpositionasitarisesfromthestylomastoid

foramen. Inthis case, facialnerve monitoring becomesa

mustinfindingthenerve’strunkandin performingasafe

dissectionofitsbranches.6

Also,theriskofiatrogenicfacialpalsyduringthesurgical

removalofthetumorishigherifthereisahistoryof

recur-rentinfectionsandinadequatetreatment(incision,drainage

orincompleteexcision)thatmayleadtointensivescarring.

Fortunately,inourcase,thediagnosiswasearlybecausethe

generalpractitioner promptlyreferred the patient tothe

ENTclinicandtherewerenopreviousattemptstoremove

thelesion,thusminimizingtheriskofscarring.

Concerning the upper portion of the fistulous tract, it

isessentialtoremoveasmallcartilageportionoftheEAC

alongwiththefistulousopeningtopreventitsrecurrence.

In ourcase, we couldapproach the margins of the

supe-rioropeningaftertheexcisionwithoutcausingasecondary

stenosisoftheEAC.

Conclusion

Congenitalmalformationsofthefirstbranchialcleftregion

represententitiesthatanotolaryngologistmightencounter

366 CodreanuCMetal.

pathology and its management, recommending general

practitionersandclinicianstobeawareoftheconsequences

of misdiagnosing it or delaying the surgical treatment. A

thoroughknowledgeoftheembryologyofthecervicofacial

region ensures an early diagnosis and a complete

surgi-calexcisionoftheselesions,minimizingtheriskofserious

complicationssuchaspermanentfacialnervepalsy.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

References

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2.Ford GR, Balakrishnan A, Evans JNG, Bailey CM. Branchial cleft and pouch anomalies. J Laryngol Otol. 1992;106: 137---43.

3.Chen M-F, Ueng S-H, Jung S-M, Chen Y-L, Chang K-P. A type II branchial cleft cyst masquerading as an infected parotid Warthin’s tumor. Chang Gung Med J. 2006;29: 435---8.

4.D’SouzaAR, UppalHS,deR,ZeitounH.Updatingconceptsof firstbranchialcleft defects:a literaturereview.IntJPediatr Otorhinolaryngol.2002;62:103---9.

5.SolaresCA,ChanJ,KoltaiPJ.Anatomicalvariationsofthefacial nerveinfirstbranchialcleftanomalies.ArchOtolaryngolHead NeckSurg.2003;129:351---5.