Choanal atresia

Choanal atresia
Classification and external resources
ICD-10	Q30.0
ICD-9	748.0
OMIM	608911
DiseasesDB	31330
MedlinePlus	001642
eMedicine	ent/330

Choanal atresia is a congenital disorder where the back of the nasal passage (choana) is blocked, usually by abnormal bony or soft tissue formed during fetal development.

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). In some cases, this may present as cyanosis while the baby is feeding, because the oral air passages are blocked by the tongue, further restricting the airway. The cyanosis may improve when the baby cries, as the oral airway is used at this time. These babies may require airway resuscitation soon after birth.

Associated conditions

Sometimes babies born with choanal atresia also have other abnormalities:

• coloboma
• heart defects
• mental retardation
• growth impairment
• others (see also CHARGE syndrome)

Also any condition that causes significant depression of the nasal bridge or midface retraction can be associated with choanal atresia. Examples include the craniosynostosis syndromes such as Crouzon syndrome, Pfeiffer syndrome, and Antley-Bixler syndrome.

Diagnosis

Choanal atresia can be suspected if it is impossible to insert a nasal catheter. Also, if one notices a continuous stream of mucous draining from one or both nostrils, it could be a sign of an atresia. Diagnosis is confirmed by radiological imaging, usually CT scan.

Treatment

Temporary alleviation can be achieved by inserting an oral airway into the mouth. However, the only definitive treatment is surgery to correct the defect by perforating the atresia to create a nasopharyngeal airway. If the blockage is caused by bone, this is drilled through and stent inserted. The patient has to have this sucked out by an air vacuum machine . And in later life as a teenager or in early twenties the hole will have to be re-drilled larger.

A stent may be inserted^[1] to keep the newly formed airway patent or repeated dilatation may be performed.^[2]

References

1. ^ Sadek SA (January 1998). "Congenital bilateral choanal atresia". Int. J. Pediatr. Otorhinolaryngol. 42 (3): 247–56. doi:10.1016/S0165-5876(97)00142-0. PMID 9466228. http://linkinghub.elsevier.com/retrieve/pii/S0165-5876(97)00142-0. 
2. ^ Gosepath J, Santamaria VE, Lippert BM, Mann WJ (2007). "Forty-one cases of congenital choanal atresia over 26 years--retrospective analysis of outcome and technique". Rhinology 45 (2): 158–63. PMID 17708465.