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Facial and Skull Cystic Lesions

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Facial and Skull Cystic Lesions Course: Facial and skull cystic lesions Faculty: TuT.T.Le,MD.,PhilippeJeanty,MD.,PhD. Course objectives: Aftercompletingthiscourse,theparticipantshouldbeable: ••• torecognizetheabnormalcystsofneckandface ••• todiscussdifferentialdiagnosesoftheseconditions ••• todiscusswiththeparentstheprognosisoftheseconditions Target audience: Physicians,sonographersandotherswhoperformand/or interpretOBultrasound. Instructions: ThisInternet-basedtutorialrequiresthatyoureadthroughthetextand relatedimagesintheirentirety.Youmayreaditfromyourbrowserorfromhardcopyafter printingitout.Inaddition,itissuggestedthatyourefertoreferencesinthebibliographyto reinforcetheinformationpresentedbytheauthor.Followingcompletionofthetutorial, clickon"TaketheQuiz".Afterscoringapassingscoreof70%orhigheryouwillbetaken toasecurepaymentpagewhereyouwillhavetheopportunitytopaytheCMEfeeand receiveyourcertificate. System requirements : In order to complete this program you must have a computer with arecent version of Chrome or Internet Explorer, andaprinter, which is configuredtoprintfromthebrowser. Volume Pricing: This course is $25.00 for 1 credit. You will receive your CME creditsandcertificateforthiscourse.Fourcredithoursfor$60.00.($15.00percredit).You willreceiveyourcreditsandcertificateforthiscourseplus2voucherswhichyoucanuse immediatelyoratafuturedate.Eightcredithoursare$80.00.($10.00percredit).Youwill receive your credit and certificate for this course plus 6 vouchers which you can use immediatelyoratafuturedate. Customer Assistance: Foranyquestionsorproblemsconcerningthisprogram,or for problems related to the printing of the certificate, please contact IAME at 802-824- 4433 or [email protected] . Estimated time for completion of tutorial : approximately120minutes . Date of review and release : March,2012. Expiration Date : March,2015. Disclosure: In compliance with the Essentials and Standards of the ACCME, the author of this CME tutorial is required to disclose any significant financial or other relationships they may have with the manufacturer(s) of any commercial product(s) or provider(s)ofanycommercialservice(s)discussedinthisprogram. Drs.LeandJeantyhaveindicatedthattheyhavenosuchrelationshipstodisclose. Facial and skull cystic lesions Mostlycysticmasses: • Cystichygroma • Vallecularcyst • Ranula • Epidermalscalpcyst • Cephalocele • Cysticheterotopicbraintissue • Dacryocystocele • Choanalatresia Mixedcysticandsolidlesions: • Hemangiolymphangioma. Mostlysolidlesionswithcysticcomponent: • Hemangioma • Cysticfacialteratoma • Epignathus • Epulis 1. Mostly cystic masses: 1.1. Facial cystic hygroma Foramorecompletediscussionofcystichygroma,seenuchalcyst-Part1.. 1.1.1. Case 1: cystichygroma,anterior. Afetuswithalargefacialmass.Thefetuswasdiagnosedwithseverelymphangiomaand hemangioma.Surgerywascompletedinitiallytotakeoutaportioninthechin,withhopes thattheswellingwouldgodown.Aweeklateratracheostomywasperformedduetothe weightofthemass.Thethirdweekanothersurgerywasdonetostopsomeofthebleeding in the left cheek. MRI confirmed a massive lymphomatous malformation consistent withcystichygromawithintheneckwithairwaycompromise. 1.1.2. Case 2: Thefollowingimagesrepresentaninterestingcaseofthe Cystichygroma diagnosedatthe 34 weeks of gestation. There was alarge roundmass protrudingto the right side of the neonate’sface.Deformingtherightcheekandeye. The Magnetic Resonance Imagingperformedafter delivery, describedaverylarge cystic mass,93x84x84mm,locatedattherightsideoftheneckandfacio-maxillaryregion.There was a notable thinning of the greater andlesser wings of the sphenoidbone, temporal, frontal bone and maxillary and zygomatic arch with excentric proptosis. There was no evidenceoftheintraorbitalorintracranialexpansion.Thedifferentialdiagnosisaccordingto theMRIwaslymphangioma,specificallycystichygroma. The neonate underwent the surgeryto remove the mass. The pathologist confirmedthe diagnosisofthecystichygroma. Images 1,2 :Image1showsanaxialviewofthefetalhead.Image2showsaroundmasson thesideofthehead . Image 3,4 :Imagesshowbothorbitsandlargeanechoicmassprotrudingontherightside ofthehead. Images 5,6 :3Dimagesofthefetalface,notetherightcheekwiththeprotrudingmass. Images 7,8 :MRIimagesshowingthecysticmasslocatedatthefacio-maxillaryregion. Image 9:showsaproptosisoftherighteye. Images 10,11,12,13:aneonatewithalargemassdeformingtherightsideofthefacebefore andaftersugery. Discussion Cystichygromasresultfromtheblockageofthelymphaticvessels.Theyarelocatedinthe regionswhichcontainlymphatictissue.Mostcommonlocationforcystichygromaisaneck region,headandaxilla.Thelocationofthecystichygromaatthepresentedcaseisavery unusualbecausetherearenolymphaticvesselsinthecentralnervoussystemormeninges. Lymphaticdrainageofthebrainisviaperivascularspacealongthebasalmembranesinthe walls of capillaries and cerebral arteries.Lymphatics are present within the nasal cavity, orbits,aroundthejugularforamenandleptomeningealsheathsaroundcranialnerves.One oftheabovementionedlocationsmaybeonoriginofthecystichygromainthepresented case . 1.2. Vallecular cyst: AdaptedandupdatedfromFabriceCuillier,MD 1.2.1. Synonyms: mucousretentioncyst,epiglotticcyst,base-of-the-tonguecyst, congenital cyst and ductal cyst 1. This later name originates from the classificationofDeSantoetal,inwhichtheygroupedlaryngealcystsaccording to their location and surface mucosa (Thyroid cartilage foraminal cysts; Saccularcysts;Ductalcysts) 1.Thisclassificationhasbecomepopularbuthas the limitationthat,it waslargelybasedonobservationinadults anddidnot recognizedifferentanatomicalsitesandvariationsinclinicalpresentation. 1.2.2. Definition Vallecular cyst is a unilocular cystic mass of variable size arising from the lingual surface of the epiglottis and containing clear, non- infectedfluid 1. 1.2.3. Prevalence : Notpreciselyknown Laryngealcystsareuncommoninchildren.Aryepiglotticcystisthemostcommon laryngeal cyst in children, followed by vallecular cyst (VC), ventricular cyst and subglottic cyst. Although these cysts are benign, they may cause serious airway obstructionandevendeathifnottreatedappropriately.Amortalityrateofabout40 %inchildrenwithlaryngealcystswasreported 2.Vallecularcystorpre-epiglotticcyst isararebutrecognizedcauseofrespiratorydistressininfancyorimmediatelyafter birth 3.Ithasbeenassociatedwithsuddenairwayobstructionresultingindeath. 1.2.4. Etiology : Sporadic. 1.2.5. Pathogenesis : Thereareseveraltheoriesforthepathogenesisofvallecularcyst.Theselesionsmost likely result from obstruction of mucous gland at the base of the tongue. The vallecularcystarelinedwithmucousglandsandwithcontinuedmucoussecretion, the lesion gradually increases in size. Other potential causes of cyst include embryological malformation (angiomatous, lymphatic malformation,…). Vallecular cystisconsideredtobemorecommoninadultsthanchildren 1.Butvallecularcyst withrespiratoryepitheliumarerare 4.. When the embryo is approximately three weeks old, the primordium of the respiratorysystemappearsasanendodermaloutgrowthfromtheventralwallofthe foregut immediately caudal to the hypobranchial eminence. The ventrallyplaced respiratory primordium then becomes separated from the dorsal portion, the esophagus, except at the entrance to the larynx, where it maintains its communicationwiththeforegutthroughthelaryngealorifice.Theremainingpartof thegutcontributestothedevelopmentofthestomachandduodenum,justcaudal to the liver 5.. Because of the proximity of the primitive gut and the pharyngeal 1 Guttiérrez J.P., Berkowitz R.G., Robertson C.F.- Vallecular cysts in newborns and young infants. Pediatr Pulmonol 1999 ; 27 : 282-5. 2 Kin Sun Wong, Hsueh Yu Li, Tsun Sheng Huang—Vallecular cyst synchronous with laryngomalacia : Presentation of two cases. Otolaryngol Head Neck Surg 1995 ; 113 : 621-4. 3 Ruben R.J., Kucinski S.A., Greenstein N.- Cystic lymphangioma of the vallecula. Can J Otol 1975 ; 4 : 180-4. 4 Manor Y., Buchner A., Peleg M., Taicher S.- Lingual cyst with respiratory epithelium: an entity of debatable histogenesis. J Oral Maxillofac Surg 1999 ; 57 : 124-7. 5 Naidoo L.C.D. - Medial lingual cyst: Review of the literature and report of a case. J Oral Maxillofac Surg 1997 ; 55 : 172-5 arches, which contain the developing tongue, embryonal rests of the gut may become misplaced in the developing tongue. It is believed that these epithelial remnantsmaysubsequentlycontributetothedevelopmentofcysticlesionsofthe tongue 4,5,6. Constantinides et al have suggested that the presence of ciliated epithelium,whichisthehallmarkoftheprimitiveforegut,warrantsuseoftheterm «intralingualcystofforegutorigin»5. 1.2.6. Associated anomalies :Associatedanomaliesarerare. 1.2.7. Pronosis: Due to the anatomical location of the cyst, an infant with vallecularcystisatriskofsuddenairwayobstructionanddeath 1. 1.2.8. Complications : ••• Polyhydramnios. ••• Airwayobstruction. ••• Pulmonaryhypoplasia. ••• Trachea,cervicalvesselsandhypoglossalnervecompression. 1.2.9. Differential diagnosis :The differential diagnosis of lesions in the vallecularregionincludesepidermoidcyst,dermoidcyst,thyroglossalductcyst, mucocele, enteric duplication cyst, lingual bronchogenic cyst, hemangioma, cystic hygroma, teratoma, hamartoma, lymphangioma and thyroid remnant cyst .1,4,5. ••• Hemangioma:The diagnosis of fetal neck hemangioma is made by ultrasonography as a translucent mass, in which pulsating Doppler flow signals is detected. MRI is recommended to plan perinatal treatment and particularly for obtaining precise information about tumor and adjacent organ 7. Nevertheless this type of mesenchymal tumor has never
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