Choanal Atresia: A Detailed Overview

Introduction

Choanal atresia is a congenital condition characterised by the blockage or narrowing of the posterior nasal apertures (choanae), which connect the nasal cavity to the nasopharynx. This abnormality can cause significant breathing difficulties, particularly in newborns, and often requires prompt recognition and intervention.

Definition and Types

Choanal atresia refers to the failure of the nasal cavity to communicate with the nasopharynx due to a physical obstruction. The obstruction may be:

• Bony: Caused by abnormal bone formation (most common type, accounting for approximately 90% of cases).
• Membranous: Due to a thin membrane of tissue blocking the passage (about 10% of cases).
• Mixed: A combination of both bony and membranous tissue.

The atresia can be unilateral (affecting one side) or bilateral (affecting both sides). Bilateral cases are more severe and present earlier in life.

Causes and Pathogenesis

Choanal atresia is a congenital defect, meaning it occurs during fetal development. The precise cause is not always known, but it is believed to result from abnormal persistence of the buccopharyngeal membrane or failure of the nasobuccal membrane to break down during embryogenesis. Genetic and environmental factors may contribute, and the condition can occur as an isolated defect or as part of a syndrome (such as CHARGE syndrome).

Clinical Presentation

The symptoms and severity depend on whether the atresia is unilateral or bilateral:

• Unilateral Choanal Atresia: May go unnoticed at birth. Affected infants may present later with persistent unilateral nasal discharge, chronic nasal obstruction, or recurrent sinus infections.
• Bilateral Choanal Atresia: Presents immediately after birth with severe respiratory distress, as neonates are obligate nasal breathers. Classic signs include:
• Cyanosis (bluish discolouration) that worsens during feeding and improves when crying (as the mouth opens for breathing).
• Difficulty in passing a nasal catheter through the nostril into the nasopharynx.
• Failure to thrive due to feeding difficulties.

Associated Conditions

Choanal atresia may occur as part of syndromes, most notably CHARGE syndrome (Coloboma, Heart defects, Atresia choanae, Retarded growth and development, Genital and Ear anomalies). Other anomalies may include craniofacial abnormalities, cleft palate, and congenital heart defects.

Diagnosis

Diagnosis is based on clinical suspicion and confirmed by investigations:

• Inability to pass a soft catheter through the nasal passage into the pharynx.
• Nasal endoscopy to visualise the obstruction.
• Imaging, especially CT scan of the nose and paranasal sinuses, to determine the type (bony or membranous) and extent of atresia and to plan surgical intervention.

Management

The primary treatment of choanal atresia is surgical correction. The approach depends on the severity and type of atresia:

• Emergency Management: For bilateral cases in neonates, immediate airway management is essential. An oral airway or McGovern nipple may be used as a temporary measure until surgery is performed.
• Surgical Repair: The preferred approach is transnasal endoscopic surgery to remove the obstruction and create a patent airway. Stenting may be used post-operatively to maintain patency, though it is controversial.
• Postoperative Care: Includes monitoring for restenosis, infection, and ensuring airway patency. Regular follow-up and endoscopic examination are essential.

Prognosis

With timely intervention, the prognosis for infants with choanal atresia is generally good. However, those with associated syndromic conditions may have additional health challenges. Early detection and management are crucial to prevent complications such as chronic respiratory problems, feeding difficulties, and failure to thrive.

Nursing Care of Patients with Choanal Atresia

Choanal atresia poses unique challenges for nursing care, requiring meticulous assessment, vigilant monitoring, and family-centered education to optimize outcomes.

Nursing Assessment

Thorough assessment is the cornerstone of effective nursing care. Key components include:

• Airway Assessment: Monitor for signs of airway compromise, noisy breathing (stertor), nasal flaring, retractions, and cyanosis, especially during feeding.
• Respiratory Status: Assess respiratory rate, effort, oxygen saturation, and color at regular intervals.
• Feeding Ability: Observe feeding patterns, noting any difficulty, choking, or fatigue.
• Growth and Development: Monitor weight gain, hydration status, and developmental milestones.
• Family History and Syndromic Features: Assess for features suggestive of associated syndromes.

Immediate Nursing Interventions

For neonates with suspected or confirmed bilateral choanal atresia, prompt intervention is essential.

• Maintain Airway Patency: Use an oral airway (oropharyngeal airway, McGovern nipple) to bypass the obstruction and keep the airway open.
• Positioning: Place the infant in a prone or side-lying position to promote oral breathing and prevent aspiration.
• Airway Suctioning: Suction oral secretions as required to prevent aspiration and maintain airway clearance.
• Oxygen Therapy: Administer supplemental oxygen as indicated to maintain adequate saturation (>94%).
• Monitoring: Continuous cardiorespiratory monitoring for signs of distress, bradycardia, or desaturation.
• Readiness for Resuscitation: Have resuscitation equipment readily available in case of acute airway compromise.

Preoperative Nursing Care

Surgical correction (transnasal or transpalatal repair) is the definitive treatment for choanal atresia. Preoperative nursing goals focus on stabilization, infection prevention, and family preparation.

• Stabilize the Patient: Ensure airway patency with oral airway or endotracheal tube as needed. Maintain NPO (nothing by mouth) status if surgery is imminent.
• Hydration and Nutrition: Provide intravenous fluids to maintain hydration. For longer-term management, discuss options for enteral feeding (e.g., orogastric tube).
• Monitor for Infection: Watch for signs of respiratory tract infections. Maintain strict aseptic technique during suctioning or airway management.
• Preparation for Surgery: Prepare the child and family for the surgical procedure, explaining the process, expected outcomes, and postoperative care.
• Support for Family: Provide emotional support, address parental anxiety and questions, and offer resources for additional information or counseling.

Postoperative Nursing Care

After surgical repair, nursing priorities shift to airway management, infection prevention, and supporting recovery.

Airway and Breathing

• Monitor for airway patency and signs of obstruction from edema, secretions, or surgical complications.
• Observe for stridor, increased work of breathing, or desaturation.
• Suction gently as needed, following the surgeon’s guidelines to avoid disrupting surgical sites.
• Humidify inspired air to keep secretions moist and facilitate clearance.
• Monitor for dislodgement or blockage of nasal stents, if placed.

Pain Management

• Assess pain using age-appropriate scales (e.g., FLACC, NIPS).
• Administer prescribed analgesics (acetaminophen, opioids) and monitor for side effects.
• Provide comfort measures such as swaddling, pacifiers, or parental presence.

Nutrition and Hydration

• Gradually reintroduce oral feeds as tolerated, starting with clear fluids.
• Monitor for signs of aspiration or feeding intolerance.
• Ensure adequate hydration via oral or intravenous fluids.

Prevention of Infection

• Monitor for fever, purulent nasal discharge, or increased respiratory distress.
• Maintain aseptic technique for all airway interventions.
• Administer prophylactic antibiotics if prescribed.

Monitoring and Early Detection of Complications

• Watch for complications such as restenosis, bleeding, septal hematoma, or synechiae formation.
• Monitor for adverse reactions to anesthesia or medications.

Long-Term Nursing Management

Some patients may require prolonged follow-up and support due to risk of airway complications or associated syndromic conditions.

• Airway Surveillance: Regular ENT follow-up to assess for restenosis, evaluate nasal airway patency, and manage stents if applicable.
• Developmental Monitoring: Assess growth, feeding ability, speech development, and hearing.
• Education: Teach parents to recognize early signs of airway compromise, infection, or feeding difficulties. Provide clear instructions for stent or nasal care if discharged home with devices in place.
• Psycho-social Support: Counsel families on coping strategies, linking them with support groups or counseling if needed.
• Coordination of Care: Collaborate with multidisciplinary teams, including neonatology, ENT, audiology, nutrition, and social work.

Family-Centered Care and Education

Family involvement is critical in ensuring optimal outcomes for patients with choanal atresia. Nurses should:

• Communicate clearly and compassionately about the diagnosis, treatment plan, and prognosis.
• Empower families to participate in care decisions and daily monitoring.
• Provide step-by-step demonstrations for airway or stent care, feeding techniques, and monitoring for warning signs.
• Offer written resources and contact information for urgent concerns.

Special Considerations

• Patients with syndromic choanal atresia often require genetic counseling and more extensive medical support.
• Assess for associated anomalies (cardiac, eye, ear, genital, growth) and coordinate specialist referrals.
• Recognize the emotional impact on families facing chronic or complex medical needs.

REFERENCES

1. Alsubaie HM, Almosa WH, Al-Qahtani AS, Margalani O. Choanal Atresia Repair With Stents and Flaps: A Systematic Review Article. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8842470/ Allergy Rhinol (Providence). 2021;12:21526567211058052.
2. Andaloro C, La Mantia I. Choanal Atresia. https://www.ncbi.nlm.nih.gov/books/NBK507724/ In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-.
3. Urbančič J, Vozel D, Battelino S, Boršoš I, Bregant L, Glavan M, Iglič Č, Jenko K, Lanišnik B, Soklič Košak T. Management of Choanal Atresia: National Recommendations with a Comprehensive Literature Review. Children (Basel). 2023 Jan 2;10(1):91. doi: 10.3390/children10010091. PMID: 36670642; PMCID: PMC9856561.
4. Choanal atresia. (2017).
   http://www.gosh.nhs.uk/medical-information/choanal-atresia
5. Rose E. Pediatric Upper Airway Obstruction and Infections. In: Walls R, ed. Rosen’s Emergency Medicine: Concepts and Clinical Practice. 10th ed. Philadelphia, PA: Elsevier; 2023. 2078-2089.e2.
6. Urbančič J, Vozel D, Battelino S, et al. Management of Choanal Atresia: National Recommendations with a Comprehensive Literature Review. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9856561/ Children (Basel). 2023;10(1):91.
7. Yoon PJ, Scholes MA, and Hermann BW. Ear, Nose, & Throat. In: Bunik M, Hay WW, Levin MJ, Abzug MJ, eds. Current Diagnosis & Treatment: Pediatrics. 26th ed. New York, NY: McGraw-Hill Education; 2022.

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