Choanal atresia
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Jaspinder Kaur, MBBS[2]
Synonyms and keywords: Atresia, Choanal; Atresias, Choanal; Choanal Atresias; Bosma arhinia microphthalmia syndrome; Bosma Henkin Christiansen syndrome; Congenital absence of nose and anterior nasopharynx (OMIM: 603457)
Overview
The word “Choana” is derivative of a greek word “Xovan” which states the funnel and hence, the term “Posterior Choana” is known as posterior nasal aperture or posterior funnel. Choanal atresia (CA) is the most common form of congenital nasal airway abnormality which presents with variable clinical features ranging from acute airway obstruction to chronic recurrent sinusitis depending upon the degree of obstruction. It is caused by the abnormal rupturing of the buccopharyngeal or naso-buccal membrane which results in the failed recanalization of the nasal fossae during the embryological period and hence, occlude the posterior nasal cavity to communicate with the nasopharynx. CA presents with severe respiratory distress, difficulty feeding, and failure to thrive when the obstruction is bilateral; and chronic persistent nasal discharge in unilateral cases. Establishing an airway is an acute otolaryngological emergency because newborns are obligate nasal breathers and experiences the paradoxical cyanosis episodes while feeding. The diagnosis requires a high index of suspicion and usually initial clinical evaluation done by introduction of a six or eight Fr suction catheter via the nostrils, methylene blue dye test, cotton wisp test, and laryngeal mirror test. The obstruction may be further visualized with a narrow flexible nasopharyngoscope after the nasal cavity has been suctioned of mucus and the nasal mucosa has been constricted with a nasal decongestant (e.g., oxymetazoline). However, the final confirmation of the diagnosis is done by CT scan of the nasal cavity which will demonstrate the atresia, define the type of tissue (bony or membranous), and show the configuration of the entire nasal cavity. The treatment of choanal atresia is essentially surgical which can be divided into emergent and elective definitive categories. It may be successfully treated by removing the obstructing tissue by using the nasal endoscopic approach via transnasal route. However, when the thick bony plate extremely narrows posterior nasal cavity, a transpalatal repair technique is more direct. An intraoperative topical application of mitomycin to inhibit fibroblast proliferation has shown to be an effective adjunct to the surgical repair of choanal atresia. Stents fashioned from endotracheal tubes are placed and secured alongwith sutures to the septum in order to prevent postoperative re-stenosis chances which are removed after 6 weeks of duration. The stents must be moistened with saline and suctioned several times daily to prevent mucus plugging and acute respiratory distress.
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Choanal atresia from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Other Imaging Findings | Other Diagnostic Studies
Presentation
It can be unilateral or bilateral.
Sometimes, a unilateral choanal atresia is not detected until much later in life because the baby manages to get along with only one nostril available for breathing.
Bilateral choanal atresia is a very serious life-threatening condition because the baby will then be unable to breathe directly after birth as babies are obligate nasal breathers (they must use their noses to breathe). These babies usually require airway resuscitation right after birth.
Treatment
The only possible treatment is surgery to correct the defect. [1]
See also
References
Template:Congenital malformations and deformations of respiratory system