Congenital Choanal Atresia: Paediatric Diagnosis and Surgical Management

Choanal atresia results embryologically from persistence of the bucconasal membrane (Hochstetter's membrane). Normally this membrane resorbs by day 38 of gestation, opening the nasal cavity into the nasopharynx. Persistence (usually around 6th gestational week) results in atresia.

Anatomic types: bony (90% — vomer and pterygoid plate hypertrophy, thick bony plate), membranous (10% — soft tissue), mixed. Classically 90% bony. Mean atretic plate thickness 4-6 mm.

Clinical picture — bilateral: neonatal emergency. Neonates are obligate nasal breathers (first 4-6 months; only a few learn mouth breathing). Classical triad: (1) postnatal cyanotic episodes — respiratory distress as no nasal breathing, oxygen saturation falls; (2) cry-relief — mouth-open breathing normalises saturation; (3) feeding-cyanosis recurrence — cannot breathe while sucking, cyanotic again.

A bilateral case is usually identified within hours. Resuscitation: oral airway (McGovern nipple) for temporising stabilisation, then planned emergency surgery.

Clinical picture — unilateral: usually late diagnosis. Unilateral congenital nasal obstruction, chronic unilateral purulent or clear discharge, recurrent unilateral sinusitis symptoms. As the child grows, compensation — other nostril and mouth-breathing maintain function, with functional limitation (exercise, sleep).

CHARGE syndrome association: 30-50% of choanal atresia infants have CHARGE (Coloboma, Heart defects, Atresia of choanae, Restricted growth, Genital, Ear). CHD7 gene mutation. Any bilateral atresia + additional anomalies must be evaluated for CHARGE.

Other associated syndromes: Treacher Collins, Crouzon, Apert, fetal alcohol syndrome, DiGeorge (22q11). Isolated choanal atresia is more commonly unilateral.

Sex distribution: female-to-male 2:1. Cause not fully understood — hormonal or genetic basis suspected. We expand on the clinical framework in our general ENT services.

Diagnosis is clinical + endoscopy + imaging. Bilateral atresia in a neonate is suspected by the classical triad; confirmed by tests.

Catheter test: 8 Fr or smaller catheter is passed through the nostril — meets resistance at 3-4 cm and cannot pass beyond. A bedside emergency test.

Nasal suction: no air movement on atretic side; mirror test (Glatzel) — no vapour from atretic side on exhalation.

Nasal endoscopy: flexible paediatric endoscope (2.4-2.7 mm) inspects anterior nasal cavity, septum, turbinates, choana. On the atretic side the posterior opening is absent; flat mucosa or bony barrier seen.

High-resolution maxillofacial CT: gold standard. Differentiates bony vs membranous, atretic plate thickness, septal thickness (often thick), pterygoid plate position, vomer thickness, concurrent sinus anomalies. Axial primary; coronal complementary. CT is mandatory for surgical planning.

CT findings: in bony atresia thickened vomer (>5-6 mm in normal neonate), medial pterygoid plate, lateral vomer thickening, mean atretic plate thickness 4-6 mm. In membranous atresia soft-tissue thickening, bone usually normal.

Other tests: hearing (BERA — particularly to exclude CHARGE), ophthalmology (CHARGE — coloboma), cardiovascular (CHARGE — cardiac anomalies), genetics consult (CHD7 analysis, karyotype). Any multi-anomaly infant warrants multidisciplinary paediatric genetics evaluation.

Differential: pyriform aperture stenosis (anterior nasal stenosis — different pathology, more anterior), septal deviation (usually unilateral, not acute obstruction), nasolacrimal cyst (intranasal cystic mass in neonate), nasal encephalocele or glioma (midline mass), large adenoids (later age — not infancy).

Timely diagnosis is essential: delayed diagnosis of bilateral atresia causes hypoxia, feeding failure, developmental delay. ENT consultation must be urgent when a neonate presents with the classical triad.

Modern choanal atresia surgery is endoscopic transnasal. Older transpalatal (palatal split), transseptal and sublabial approaches gave way to endoscopy — less invasive, better visualisation, fewer complications, better long-term outcomes.

Timing: bilateral atresia is neonatal (first week-month) — planned surgery after stabilisation. Unilateral atresia is not urgent; elective surgery if symptomatic or functionally limiting (usually 1-5 years).

Anaesthesia: general, endotracheal. Small airway and respiratory reserve mean paediatric anaesthesia expertise is critical.

Procedure: paediatric nasal endoscope (2.7 mm 0° or 30°). Vasoconstrictor (epinephrine) to decongest mucosa. Atretic plate visualised; flap planned — mucosal flaps over vomer or pterygoid plate (preserves vascularised cover).

Atrectomy: microdebrider, bone forceps, drilling (high-speed paediatric drill) to resect the atretic plate. Neochoanal diameter close to adult choanal (10-15 mm). Vomer resection often required (thick vomer is the commonest failure cause). Medial pterygoid plate resection for adequate width.

Stenting: controversial. Older 6-12 weeks nasopharyngeal silicone stent for restenosis prevention. Modern practice uses less — when mucosal flaps and adequate bone resection achieved, stent may be unnecessary. Indications: narrow neochoana, deficient flap, restenosis history.

Mitomycin C: intraoperative topical (0.4-1 mg/mL for 4-5 minutes) reduces scar — reported to lower restenosis. Often used in modern protocols.

Postoperative: 1-3 days admission (bilateral may need NICU monitoring), saline nasal irrigation 4-6×/day, antibiotic 7 days, weekly endoscopic check first 6 weeks. Stent removal (if placed) at 6-12 weeks.

Restenosis: the major problem in choanal atresia surgery. Reported 20-50% — higher in bilateral. Risk factors: thin mucosal flap, inadequate bony resection, thick vomer, CHARGE syndrome, restenosis tendency, GERD (postoperative irritation).

Revision: second endoscopic repair — more aggressive bone resection + mitomycin C + sometimes stent. Multiple revisions may be needed; a small group (5-10%) is challenging to keep patent. Step-by-step details: tonsillitis page.

Early postoperative (first 6 weeks): saline nasal irrigation is essential — 4-6× daily liberal washing, prevents crusting and mucus build-up. Parents trained; bilateral nasal aspirator + saline drops for the infant. Endoscopic check weekly or biweekly.

Stent management (if placed): crusts can accumulate inside stent; regular irrigation and instrumental cleaning needed. Stent may shift or extrude; parents asked to report immediately.

Antibiotic: 7-10 days oral antibiotic (amoxicillin-clavulanate) postop. With stent in place therapy revisited (long courses risk resistance).

GERD control: especially in CHARGE; nasopharyngeal acid irritation worsens restenosis. PPI (infant omeprazole) recommended 3-6 months postoperatively.

Long-term follow-up: first year 3-monthly endoscopic check; second year 6-monthly; then annual. Early restenosis can be managed with balloon dilatation or small revision.

Complications — early: bleeding (postop first week — especially vomer), infection (sinusitis, nasopharyngeal cellulitis), stent-related pressure injury, septal perforation, nasolacrimal duct injury.

Complications — late: restenosis (20-50%, commonest), nasal synechiae (adhesion — anterior cavity), pyriform aperture narrowing, nasal valve insufficiency, septal perforation, nasal saddle deformity (after aggressive resection — especially cartilage loss).

Prognosis: unilateral atresia overall success 80-90% (single procedure); bilateral 60-75% (typically reaches 80-90% after 1-2 revisions). CHARGE patients have lower success and higher revision need (anatomy + mucosal features).

Paediatric development: early intervention allows normal growth — feeding, weight gain, breathing, development; delayed bilateral atresia risks failure to thrive. Unilateral cases found socially in school age recover well after surgery.

Patient/family education: home care is critical. Saline irrigation, crust removal technique, alarm signs (bleeding, fever, distress), endoscopic follow-up programme, GERD management. Years-long follow-up.

Multidisciplinary team: paediatric ENT + paediatric anaesthesiologist + neonatologist or paediatrician + genetics (CHARGE suspicion) + GE (GERD) + SLP (long-term articulation or swallowing). Bilateral atresia is best managed in a tertiary paediatric centre. Related reading: our Istanbul ENT services.