Congenital Anomalies of Nose

Congenital malformations of the nose occur due to aberrations in the normal developmental pathways of the skeleton. Radiological evaluation plays an important role in the diagnosis and management of congenital malformations of the nose. A lot of congenital malformations of the nose alter the routine life cycle of the kids because of social stigma and the infections associated with them. Choanal atresia is one of the most common congenital anomalies in infants. The infant will present to the clinic with difficulty breathing and the reason for the difficulty is narrowing or blockage of the nasal pathway.

Choanal Atresia

• Choana is a communication between the nose anteriorly and the nasopharynx posteriorly. Atresia is a term used to define something which is not developed.
• Choanal atresia is the known communication between the nose and esophagus.

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 Pathophysiology

• It can be either Bony 90% or membranous 10%.
• Bony choanal atresia is either due to excessive thickness of Vomer bone or bowing of the lateral wall of the nose that results in atresia.
• Membranous atresia occurs due to the persistence of the bucconasal membrane which separates the nasal cavity from the oral cavity.

Types of Choanal Atresia

• Unilateral atresia- In this kind, only one side of the choana communicates with the nasopharynx. It is not considered as an emergency.
• Bilateral Atresia -In this kind of atresia choana are not communicating with the nasopharynx on both sides.
• It is an emergency condition.
• It will be present as respiratory distress and stridor at birth.
• It is part of a syndrome called CHARGE syndrome which includes:
  • Coloboma Iridis
•   Heart defects
  • Atresia(Choanal)
  • Retardation
  • Genital malformations
  • Ear abnormalities.

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Clinical Features/Symptoms

• Bilateral atresia leads to respiratory obstruction.
• Respiratory distress in rest but disappearing on crying is a typical feature of Choanal Atresia.

Diagnosis

• The patient presents with clinical features of Choanal atresia.
• There will be the presence of mucoid discharge in the nose and there will be the absence of air bubbles in the nasal discharge.
• The patient will present with failure to pass an infant feeding tube from the nose to the pharynx.
• Radiopaque dye is installed into the nose and checked whether it has reached the nasopharynx or not by taking a lateral X-ray.
• Radiologically X-ray and CT scans are done for the diagnosis.
• A CT scan will show bilateral Choanal atresia.

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Treatment

• Surgery is the only long-term treatment option for babies with choanal atresia.

• Emergency airway by using McGovern’s technique (A feeding nipple with a large hole provides a good oral airway) or Tracheostomy.
• To repair choanal atresia, the surgeon will drill a hole through the tissue or bone obstructing your baby's airway. The roof of the mouth (trans palatal) or the nose (transnasal) can be used for this. A stent may occasionally be inserted into your baby's nasal passage to keep the airway open while the wound heals. A few months after implantation, the stents are surgically removed. Under general anesthesia, choanal atresia is performed in an operating room.
• Definitive treatment is Correction of atresia at 1.5 years of age.

Hope you found this blog helpful for your ENT residency head and neck preparation. For more informative and interesting posts like these, keep reading PrepLadder’s blogs.

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