Choanal Atresia2

8/6/2019 Choanal Atresia2

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ExamExam

Mouth breathing, retracting slightly at neckMouth breathing, retracting slightly at neck

Shortened palpebral fissures with no visible globeShortened palpebral fissures with no visible globe

Left cleft nareLeft cleft nare Ankyloglossus Ankyloglossus

Low set earsLow set ears

Normal fingersNormal fingers

RRR no murmursRRR no murmurs

Abdomen soft, no organomegaly Abdomen soft, no organomegaly

Cannot pass catheter or FFO scope either nareCannot pass catheter or FFO scope either nare



Nasopharyngeal obstruction DDxNasopharyngeal obstruction DDx

Vascular Vascular -- hemangiomahemangioma InfectiousInfectious -- ChlamydiaChlamydia TraumaticTraumatic-- septal hematoma, dislocated nasal septumseptal hematoma, dislocated nasal septum Autoimmune Autoimmune sarcoid, RA, churgsarcoid, RA, churg--straussstrauss MetabolicMetabolic cystic fibrosiscystic fibrosis Iatrogenic/idiopathicIatrogenic/idiopathic septal hematoma, dislocated nasal septum,septal hematoma, dislocated nasal septum,

kartagenerskartageners NeoplasiaNeoplasia hamartoma, nasopharyngeal mass hairy polyp, teratoma,hamartoma, nasopharyngeal mass hairy polyp, teratoma,

chordoma, rhabdomyosarcoma, angiofibroma, hemangiopericytoma, sccchordoma, rhabdomyosarcoma, angiofibroma, hemangiopericytoma, scc CongenitalCongenital -- Choanal stenosis/atresia, Piriform aperture stenosis, NasalChoanal stenosis/atresia, Piriform aperture stenosis, Nasal

glioma, Encephalocele, Meningocele, dermoid, Micrognathia, Macroglossia,glioma, Encephalocele, Meningocele, dermoid, Micrognathia, Macroglossia,Treacher collins, Downs syndrome, Cystic Hygroma, Lingual thyroid,Treacher collins, Downs syndrome, Cystic Hygroma, Lingual thyroid,incisive canal cysts, nasolacrimal duct cyst, dentigerous cystsincisive canal cysts, nasolacrimal duct cyst, dentigerous cysts



Nasal endoscopyNasal endoscopy

Right Left



CT scanCT scan

Her scan

Different Pathology



CT scan evaluationCT scan evaluation

Choanal airspace measurement: meanChoanal airspace measurement: meannormal is 0.67cm. Mixed atresia is 1/3 of normal is 0.67cm. Mixed atresia is 1/3 of normal, bony atresia measures 0.normal, bony atresia measures 0.

Vomer width Vomer width Mean 0.23cm, bony atresiaMean 0.23cm, bony atresiamean 0.6cm, membranous atresia 0.3 cm.mean 0.6cm, membranous atresia 0.3 cm.

Slovis TL et al. Choanal Atresia: Precise CT Evaluation. Radiology. 1985;155:345-348.



EpidemiologyEpidemiology

1:50001:5000--1:8000 live births1:8000 live births

50% with other anomalies50% with other anomalies

6565--75% unilateral. 75% of bilateral cases have other75% unilateral. 75% of bilateral cases have otheranomalies.anomalies.

Polydactyly, nasalPolydactyly, nasal--auricular and palatal deformities,auricular and palatal deformities,crouzons, craniosynostosis, microencephaly,crouzons, craniosynostosis, microencephaly,meningocele, meningoencephalocele, facial asymmetry,meningocele, meningoencephalocele, facial asymmetry,

hypoplasia of orbit and midface, hypertelorism, cleft hypoplasia of orbit and midface, hypertelorism, cleft palatepalate

29% bony, 71% mixed (brown et al)29% bony, 71% mixed (brown et al)



Typical presentationTypical presentation

BilateralBilateral cyclic cyanosis relieved bycyclic cyanosis relieved bycryingcrying

UnilateralUnilateral chronic nasal obstruction,chronic nasal obstruction,thick tenatious mucousthick tenatious mucous



anatomyanatomy

Bounderies of the atresiaBounderies of the atresiaplate:plate:

Superior: sphenoidSuperior: sphenoid

Lateral: medial pterygoidLateral: medial pterygoidlaminalamina

Medial: vomerMedial: vomer Inferior: horizontal portionInferior: horizontal portion

of the palatal boneof the palatal bone



Anatomy Anatomy

Narrow nasal cavity lateralNarrow nasal cavity lateralobstruction by lat pterygoidobstruction by lat pterygoidplate, Vomer thickening,plate, Vomer thickening,

membranous obstructionmembranous obstruction Medial pterygoid plate andMedial pterygoid plate and

posterior vomer are expandedposterior vomer are expandedby endochondral boneby endochondral boneformation and fibroepithelialformation and fibroepithelialmembrane that obstructs themembrane that obstructs thechoanaechoanae



Further work upFurther work up

As always, AIRWAY first As always, AIRWAY first

Genetics evaluationGenetics evaluation

Cardiology (EKG, Echo)Cardiology (EKG, Echo)

Opthalmology evaluationOpthalmology evaluation

HearingHearing UA (kidney)UA (kidney)



Management Management

Unilateral delayed to allow theUnilateral delayed to allow thepatient to grow, and reducepatient to grow, and reducerisk of restenosisrisk of restenosis

BilateralBilateral creating oral airwaycreating oral airway--Mcgovern nipple with enlargedMcgovern nipple with enlargedhole or two side holes, surgicalhole or two side holes, surgicalcorrection vs tracheostomycorrection vs tracheostomy

Stimulate infant to cry,Stimulate infant to cry,intubate, bag ventilate if intubate, bag ventilate if obstructing in an emergencyobstructing in an emergency

Genetics consult, work up forGenetics consult, work up forother anomaliesother anomalies



Surgical ApproachesSurgical Approaches

TransnasalTransnasal

TranspalatalTranspalatal

TransseptalTransseptal



Transnasal/transseptalTransnasal/transseptal

Most common repair strategy now that Most common repair strategy now that endoscopes are usedendoscopes are used

Multiple techniquesMultiple techniques all use eitherall use eithermucosal flaps or stenting to prevent mucosal flaps or stenting to prevent restenosisrestenosis



TransnasalTransnasal



Transnasal pros/consTransnasal pros/cons

Punching through the atretic plate is essentiallyPunching through the atretic plate is essentiallyblind processblind process important to go inferomedial toimportant to go inferomedial to

avoid sphenopalatine artery, and drill parallel toavoid sphenopalatine artery, and drill parallel topalate to avoid entering clivus, sphenoidpalate to avoid entering clivus, sphenoid

Risks include all those of FESS, but anatomy isRisks include all those of FESS, but anatomy isabnormal and less predictableabnormal and less predictable

Does not impair development of dental archesDoes not impair development of dental arches

Uses equipment/techniques similar to FESSUses equipment/techniques similar to FESS



Transnasal/transseptalTransnasal/transseptal

Cedin AC, Fujita R, Cruz OL: Endoscopic transeptal surgery for choanal atresia with a stentless folded-over-flap technique. Otolaryngol Head NeckSurg 2006 Nov; 135(5): 693-8



TranspalatalTranspalatal

Tewfik et al., 2007

Include greater palatine for blood supply to flapInclude greater palatine for blood supply to flapSubperiosteal elevation to leading edge of hardpalateCutting burr used to drill the posterior vomer andatretic plate.Stents placedIncision made 5 mm from dental archIncision made 5 mm from dental arch



Transpalatal drawbackTranspalatal drawback

52% incidence of dental malocclusion resultingfrom narrowed maxillary dental arches in

patients younger than 5 years Palatal flap necrosis

Palatal fistula

Persistent submucous tunnel

Restenosis Upside: success rate of over 80% is comparable

to all other approaches



SyndromesSyndromes

CHARGECHARGE

Treacher CollinsTreacher Collins

CrouzonsCrouzons



CHARGE syndromeCHARGE syndrome

Coloboma and/or CNS abnormalitiesColoboma and/or CNS abnormalities Heart abnormalityHeart abnormality Atresia choanae Atresia choanae

Retardation of growthRetardation of growth Genital defects (males)Genital defects (males) Ear anomalies/deafnessEar anomalies/deafness

Mutations on the CHD7 gene (located on Chromosome 8) wereMutations on the CHD7 gene (located on Chromosome 8) werefound in 10 of 17 patients in a study conducted in the Netherlandsfound in 10 of 17 patients in a study conducted in the Netherlands

(Vissers, et al., 2004).(Vissers, et al., 2004). A study in the US of 110 individuals with CHARGE syndrome showed A study in the US of 110 individuals with CHARGE syndrome showed

that 60% of those tested had a mutation on the CHD7 gene (Lalanithat 60% of those tested had a mutation on the CHD7 gene (Lalaniet al., 2006).et al., 2006).



CHARGECHARGE

The percentages of the different anomalies inThe percentages of the different anomalies inCHARGE association are as follows:CHARGE association are as follows:

ColobomaColoboma -- 80%80% Heart defect Heart defect -- 58%58% Atresia of choanae Atresia of choanae -- 100%100% Mental retardationMental retardation -- 94%94%

Growth deficiencyGrowth deficiency -- 87%87% Genital hypoplasia in malesGenital hypoplasia in males -- 75%75% Ear anomaliesEar anomalies -- 88%88%



CHARGECHARGE

Other features: kidney anomalies, OCDOther features: kidney anomalies, OCDPalmar crease, esophageal atresia,Palmar crease, esophageal atresia,

tracheoesophageal fistula, scoliosis,tracheoesophageal fistula, scoliosis,chronic middle ear problems, cleftschronic middle ear problems, clefts




Malar hypoplasiaMalar hypoplasia

ColobomasColobomas

Medial 2/3 lash without lashesMedial 2/3 lash without lashes

Retrusive chinRetrusive chin

Class 2 malocclusionClass 2 malocclusion

External ear anomaliesExternal ear anomalies SNHLSNHL

Rarely choanal atresiaRarely choanal atresia



Which of the following are true of Which of the following are true of

Treacher collins syndromeTreacher collins syndrome A. Autosomal Recessive A. Autosomal Recessive

B. Mutation in the TCOF gene, coding treacleB. Mutation in the TCOF gene, coding treacle

C. Abnormal third branchial arch development C. Abnormal third branchial arch development

D. External ear anomolies are present but thereD. External ear anomolies are present but thereis no hearing lossis no hearing loss

E. Surgical correction typically at 7 years of age.E. Surgical correction typically at 7 years of age.



EmbryogenesisEmbryogenesis

Nasal placodes invaginate to formNasal placodes invaginate to formnasal pits 3nasal pits 3--4 weeks of gestation4 weeks of gestation

These enlarge and burrow intoThese enlarge and burrow intounderlying mesoderm, formingunderlying mesoderm, forming

nasal pouchesnasal pouchesFloor between nasal and buccal cavityFloor between nasal and buccal cavity

thins to form nasobuccal membranethins to form nasobuccal membraneMembrane ruptures at 6 weeksMembrane ruptures at 6 weeks

gestationgestationChoanal atresiaChoanal atresia cause unknown,cause unknown,

thought to be due either tothought to be due either topersistence of nasobuccalpersistence of nasobuccalmembrane, buccopharngealmembrane, buccopharngealmembrane, or due to errors inmembrane, or due to errors inmesoderm migrationmesoderm migration



EpidemiologyEpidemiology

1:50001:5000--1:8000 live births1:8000 live births 50% with other anomalies50% with other anomalies

6565--75% unilateral. 75% of bilateral cases have75% unilateral. 75% of bilateral cases haveother anomaliesother anomalies Polydactyly, nasalPolydactyly, nasal--auricular and palatalauricular and palatal

deformities, crouzons, craniosynostosis,deformities, crouzons, craniosynostosis,microencephaly, meningocele,microencephaly, meningocele,

meningoencephalocele, facial asymmetry,meningoencephalocele, facial asymmetry,hypoplasia of orbt and midface, hypertelorism,hypoplasia of orbt and midface, hypertelorism,cleft palatecleft palate



EmbryogenesisEmbryogenesis



Hemifacial microsomiaHemifacial microsomia

Orbit Orbit

MandibleMandible

EarEar

Facial nerveFacial nerve

Soft tissueSoft tissue Defect of first and second branchial archesDefect of first and second branchial arches

-- ? Vascular insult ? Vascular insult




Malar hypoplasiaMalar hypoplasia

ColobomasColobomas

Medial 2/3 lash without lashesMedial 2/3 lash without lashes

Retrusive chinRetrusive chin

Class 2 malocclusionClass 2 malocclusion External ear anomaliesExternal ear anomalies

SNHLSNHL

Choanal Atresia2

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