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Choanal Atresia

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You are asked to see a neonate born at 36 weeks for intermittent cyanosis. ... hamartoma, nasopharyngeal mass hairy polyp, teratoma, chordoma, rhabdomyosarcoma, ... – PowerPoint PPT presentation

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Title: Choanal Atresia


1
Choanal Atresia
  • Essie Fine
  • May 31, 2007

2
Case Presentation
  • You are asked to see a neonate born at 36 weeks
    for intermittent cyanosis.
  • Birth history born to G4P3 mother, by c-section
    for placenta previa. Apgars are 8, 9. The neonate
    then desaturates at 7 minutes of life. She is bag
    ventilated and recovers.
  • History No birth defects in family. Uneventful
    pregnancy. No exposures.

3
Exam
  • Mouth breathing, retracting slightly at neck
  • Shortened palpebral fissures with no visible
    globe
  • Left cleft nare
  • Ankyloglossus
  • Low set ears
  • Normal fingers
  • RRR no murmurs
  • Abdomen soft, no organomegaly
  • Cannot pass catheter or FFO scope either nare

4
Nasopharyngeal obstruction DDx
  • Vascular - hemangioma
  • Infectious - Chlamydia
  • Traumatic- septal hematoma, dislocated nasal
    septum
  • Autoimmune sarcoid, RA, churg-strauss
  • Metabolic cystic fibrosis
  • Iatrogenic/idiopathic septal hematoma,
    dislocated nasal septum, kartageners
  • Neoplasia hamartoma, nasopharyngeal mass hairy
    polyp, teratoma, chordoma, rhabdomyosarcoma,
    angiofibroma, hemangiopericytoma, scc
  • Congenital - Choanal stenosis/atresia, Piriform
    aperture stenosis, Nasal glioma, Encephalocele,
    Meningocele, dermoid, Micrognathia, Macroglossia,
    Treacher collins, Downs syndrome, Cystic
    Hygroma, Lingual thyroid, incisive canal cysts,
    nasolacrimal duct cyst, dentigerous cysts

5
Nasal endoscopy
Left
Right
6
CT scan
Different Pathology
Her scan
7
CT scan evaluation
  • Choanal airspace measurement mean normal is
    0.67cm. Mixed atresia is 1/3 of normal, bony
    atresia measures 0.
  • Vomer width Mean 0.23cm, bony atresia mean
    0.6cm, membranous atresia 0.3 cm.

Slovis TL et al. Choanal Atresia Precise CT
Evaluation. Radiology. 1985155345-348.
8
Epidemiology
  • 15000-18000 live births
  • 50 with other anomalies
  • 65-75 unilateral. 75 of bilateral cases have
    other anomalies.
  • Polydactyly, nasal-auricular and palatal
    deformities, crouzons, craniosynostosis,
    microencephaly, meningocele, meningoencephalocele,
    facial asymmetry, hypoplasia of orbit and
    midface, hypertelorism, cleft palate
  • 29 bony, 71 mixed (brown et al)

9
Typical presentation
  • Bilateral cyclic cyanosis relieved by crying
  • Unilateral chronic nasal obstruction, thick
    tenatious mucous

10
anatomy
  • Bounderies of the atresia plate
  • Superior sphenoid
  • Lateral medial pterygoid lamina
  • Medial vomer
  • Inferior horizontal portion of the palatal bone

11
Anatomy
  • Narrow nasal cavity lateral obstruction by lat
    pterygoid plate, Vomer thickening, membranous
    obstruction
  • Medial pterygoid plate and posterior vomer are
    expanded by endochondral bone formation and
    fibroepithelial membrane that obstructs the
    choanae

12
Further work up
  • As always, AIRWAY first
  • Genetics evaluation
  • Cardiology (EKG, Echo)
  • Opthalmology evaluation
  • Hearing
  • UA (kidney)

13
Management
  • Unilateral delayed to allow the patient to grow,
    and reduce risk of restenosis
  • Bilateral creating oral airway- Mcgovern nipple
    with enlarged hole or two side holes, surgical
    correction vs tracheostomy
  • Stimulate infant to cry, intubate, bag ventilate
    if obstructing in an emergency
  • Genetics consult, work up for other anomalies

14
Surgical Approaches
  • Transnasal
  • Transpalatal
  • Transseptal

15
Transnasal/transseptal
  • Most common repair strategy now that endoscopes
    are used
  • Multiple techniques all use either mucosal
    flaps or stenting to prevent restenosis

16
Transnasal
17
Transnasal pros/cons
  • Punching through the atretic plate is essentially
    blind process important to go inferomedial to
    avoid sphenopalatine artery, and drill parallel
    to palate to avoid entering clivus, sphenoid
  • Risks include all those of FESS, but anatomy is
    abnormal and less predictable
  • Does not impair development of dental arches
  • Uses equipment/techniques similar to FESS

18
Transnasal/transseptal
Cedin AC, Fujita R, Cruz OL Endoscopic
transeptal surgery for choanal atresia with a
stentless folded-over-flap technique. Otolaryngol
Head Neck Surg 2006 Nov 135(5) 693-8
19
Transpalatal
  • Include greater palatine for blood supply to flap
  • Subperiosteal elevation to leading edge of hard
    palate
  • Cutting burr used to drill the posterior vomer
    and atretic plate.
  • Stents placed
  • Incision made 5 mm from dental arch

Tewfik et al., 2007
20
Transpalatal drawback
  • 52 incidence of dental malocclusion resulting
    from 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

21
Syndromes
  • CHARGE
  • Treacher Collins
  • Crouzons

22
CHARGE syndrome
  • Coloboma and/or CNS abnormalities
  • Heart abnormality
  • Atresia choanae
  • Retardation of growth
  • Genital defects (males)
  • Ear anomalies/deafness
  • Mutations on the CHD7 gene (located on Chromosome
    8) were found in 10 of 17 patients in a study
    conducted in the Netherlands (Vissers, et al.,
    2004).
  • A study in the US of 110 individuals with CHARGE
    syndrome showed that 60 of those tested had a
    mutation on the CHD7 gene (Lalani et al., 2006).

23
CHARGE
  • The percentages of the different anomalies in
    CHARGE association are as follows
  • Coloboma - 80
  • Heart defect - 58
  • Atresia of choanae - 100
  • Mental retardation - 94
  • Growth deficiency - 87
  • Genital hypoplasia in males - 75
  • Ear anomalies - 88

24
CHARGE
  • Other features kidney anomalies, OCD Palmar
    crease, esophageal atresia, tracheoesophageal
    fistula, scoliosis, chronic middle ear problems,
    clefts

25
Treacher Collins
  • Malar hypoplasia
  • Colobomas
  • Medial 2/3 lash without lashes
  • Retrusive chin
  • Class 2 malocclusion
  • External ear anomalies
  • SNHL
  • Rarely choanal atresia

26
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27
Which of the following are true of Treacher
collins syndrome
  • A. Autosomal Recessive
  • B. Mutation in the TCOF gene, coding treacle
  • C. Abnormal third branchial arch development
  • D. External ear anomolies are present but there
    is no hearing loss
  • E. Surgical correction typically at 7 years of
    age.

28
Embryogenesis
  • Nasal placodes invaginate to form nasal pits 3-4
    weeks of gestation
  • These enlarge and burrow into underlying
    mesoderm, forming nasal pouches
  • Floor between nasal and buccal cavity thins to
    form nasobuccal membrane
  • Membrane ruptures at 6 weeks gestation
  • Choanal atresia cause unknown, thought to be
    due either to persistence of nasobuccal membrane,
    buccopharngeal membrane, or due to errors in
    mesoderm migration

29
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34
Epidemiology
  • 15000-18000 live births
  • 50 with other anomalies
  • 65-75 unilateral. 75 of bilateral cases have
    other anomalies
  • Polydactyly, nasal-auricular and palatal
    deformities, crouzons, craniosynostosis,
    microencephaly, meningocele, meningoencephalocele,
    facial asymmetry, hypoplasia of orbt and
    midface, hypertelorism, cleft palate

35
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36
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37
Embryogenesis
38
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39
Hemifacial microsomia
  • Orbit
  • Mandible
  • Ear
  • Facial nerve
  • Soft tissue
  • Defect of first and second branchial arches - ?
    Vascular insult

40
Treacher Collins
  • Malar hypoplasia
  • Colobomas
  • Medial 2/3 lash without lashes
  • Retrusive chin
  • Class 2 malocclusion
  • External ear anomalies
  • SNHL
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