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    Choanal Atresia2

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    pramod555
    This document discusses Choanal Atresia, a congenital nasal obstruction. Key points include: - It occurs in 1 in 5000-8000 births and is often associated with other anomalies. - Presentation includes cyanosis in bilateral cases and nasal obstruction in unilateral cases. - Exam findings include mouth breathing, retracting neck, and inability to pass a catheter through either nasal passage. - Imaging like CT scan can help determine if the atresia is bony or membranous. - Surgical approaches include transnasal, transpalatal, or transseptal repair. Syndromes like CHARGE and Treacher Collins are often associated.

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    Choanal Atresia2

    Uploaded by

    pramod555
    170 views40 pages
    This document discusses Choanal Atresia, a congenital nasal obstruction. Key points include: - It occurs in 1 in 5000-8000 births and is often associated with other anomalies. - Presentation includes cyanosis in bilateral cases and nasal obstruction in unilateral cases. - Exam findings include mouth breathing, retracting neck, and inability to pass a catheter through either nasal passage. - Imaging like CT scan can help determine if the atresia is bony or membranous. - Surgical approaches include transnasal, transpalatal, or transseptal repair. Syndromes like CHARGE and Treacher Collins are often associated.

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    This document discusses Choanal Atresia, a congenital nasal obstruction. Key points include: - It occurs in 1 in 5000-8000 births and is often associated with other anomalies. - Presentation includes cyanosis in bilateral cases and nasal obstruction in unilateral cases. - Exam findings include mouth breathing, retracting neck, and inability to pass a catheter through either nasal passage. - Imaging like CT scan can help determine if the atresia is bony or membranous. - Surgical approaches include transnasal, transpalatal, or transseptal repair. Syndromes like CHARGE and Treacher Collins are often associated.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    170 views40 pages

    Choanal Atresia2

    Uploaded by

    pramod555
    This document discusses Choanal Atresia, a congenital nasal obstruction. Key points include: - It occurs in 1 in 5000-8000 births and is often associated with other anomalies. - Presentation includes cyanosis in bilateral cases and nasal obstruction in unilateral cases. - Exam findings include mouth breathing, retracting neck, and inability to pass a catheter through either nasal passage. - Imaging like CT scan can help determine if the atresia is bony or membranous. - Surgical approaches include transnasal, transpalatal, or transseptal repair. Syndromes like CHARGE and Treacher Collins are often associated.

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    Choanal Atresia

    Essie Fine
    May 31, 2007
    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.
    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
    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, Down’s syndrome, Cystic Hygroma,
    Lingual thyroid, incisive canal cysts, nasolacrimal duct cyst,
    dentigerous cysts
    Nasal endoscopy

    Right Left
    CT scan

    Different Pathology

    Her scan
    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. 1985;155:345-348.
    Epidemiology
     1:5000-1:8000 live births
     50% with other anomalies
     65-75% unilateral. 75% of bilateral cases have
    other anomalies.
     Polydactyly, nasal-auricular and palatal
    deformities, crouzon’s, craniosynostosis,
    microencephaly, meningocele,
    meningoencephalocele, facial asymmetry,
    hypoplasia of orbit and midface, hypertelorism,
    cleft palate
     29% bony, 71% mixed (brown et al)
    Typical presentation
     Bilateral – cyclic cyanosis relieved by
    crying
     Unilateral – chronic nasal obstruction,
    thick tenatious mucous
    anatomy
    Bounderies of the atresia
    plate:
     Superior: sphenoid

     Lateral: medial

    pterygoid lamina
     Medial: vomer

     Inferior: horizontal

    portion of the palatal


    bone
    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
    Further work up
     As always, AIRWAY first
     Genetics evaluation
     Cardiology (EKG, Echo)
     Opthalmology evaluation
     Hearing
     UA (kidney)
    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
    Surgical Approaches
     Transnasal
     Transpalatal
     Transseptal
    Transnasal/transseptal
     Most common repair strategy now
    that endoscopes are used
     Multiple techniques – all use either
    mucosal flaps or stenting to prevent
    restenosis
    Transnasal
    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
    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
    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


    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
    Syndromes
     CHARGE
     Treacher Collins
     Crouzon’s
    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).
    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%
    CHARGE
     Other features: kidney anomalies,
    OCD Palmar crease, esophageal
    atresia, tracheoesophageal fistula,
    scoliosis, chronic middle ear
    problems, clefts
    Treacher Collins
     Malar hypoplasia
     Colobomas
     Medial 2/3 lash without lashes
     Retrusive chin
     Class 2 malocclusion
     External ear anomalies
     SNHL
     Rarely choanal atresia
    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.
    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
    Epidemiology
     1:5000-1:8000 live births
     50% with other anomalies
     65-75% unilateral. 75% of bilateral cases
    have other anomalies
     Polydactyly, nasal-auricular and palatal
    deformities, crouzon’s, craniosynostosis,
    microencephaly, meningocele,
    meningoencephalocele, facial asymmetry,
    hypoplasia of orbt and midface,
    hypertelorism, cleft palate
    Embryogenesis
    Hemifacial microsomia
     Orbit
     Mandible
     Ear
     Facial nerve
     Soft tissue
     Defect of first and second branchial
    arches - ? Vascular insult
    Treacher Collins
     Malar hypoplasia
     Colobomas
     Medial 2/3 lash without lashes
     Retrusive chin
     Class 2 malocclusion
     External ear anomalies
     SNHL

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