Choanal atresia is a congenital disorder where the back of the nasal passage (choana) is blocked, usually by abnormal bony or soft tissue (membranous) due to failed hole development of the nasal fossae during prenatal development. It causes persistent rhinorrhea, and with bilateral choanal atresia, an obstructed airway that can cause cyanosis and hypoxia.
Choanal atresia is diagnosed based on the inability to place a nasal catheter, and radiology results (particularly CT scans). Treatment involves maintaining an open airway, and may involve surgery to reopen the airway, potentially with a stent. Choanal atresia is a fairly rare condition, affecting between 1 in 7,000 to 1 in 5,000 live births. It is more common in females and is more often unilateral.
Presentation
Choanal atresia can be unilateral or bilateral.
- A unilateral choanal atresia may not be detected until much later in life because the baby manages to get along with only one nostril available for breathing. Symptoms are minor, including persistent rhinorrhea (mainly normal mucus) and chronic sinusitis.
- Bilateral choanal atresia is a life-threatening condition because the baby will be unable to breathe directly after birth as babies are obligate nasal breathers (they mainly use their noses to breathe). Another epidemiological report in 2010 found even higher associations between increased incidents of choanal atresia and exposure to second-hand-smoke, coffee consumption, high maternal zinc and B-12 intake and exposure to anti-infective urinary tract medications. The anti-thyroid medication methimazole has been associated with the development of choanal atresia in rare cases if given during the first trimester of pregnancy.
Mechanism
Choanal atresia causes closure of the posterior choanae in the nasal cavity. Around 30% of these affect just the bone, while around 70% affect both bone and membranes. Bones affected can include the body of the sphenoid bone, the vomer, the medial pterygoid process of the sphenoid bone, and the horizontal plate of the palatine bone. particularly with bilateral choanal atresia. Repeated dilatation may be performed. CT guidance may be used.