Reconstructing absence: Airway management in a rare craniofacial abnormality

Reshma Ponnusamy^*, K Senthil Kumar

Department of Anaesthesiology, Kauvery Hospital, Cantonment, Trichy

Background

Congenital arrhinia is an extremely rare malformation, with fewer than 50 cases reported in the literature since its first description in 1931. This anomaly is frequently associated with other midline abnormalities, including hypertelorism, microphthalmia, microtia, and a high-arched palate. Although the exact pathogenesis remains unclear, it is thought to arise from aberrant development of the nasal placodes during embryogenesis.

This condition poses significant challenges, particularly in the neonatal period, as neonates are obligate nasal breathers. Airway obstruction and feeding difficulties often necessitate immediate intervention to secure a viable airway. Beyond neonatal stabilization, definitive nasal reconstruction presents additional complexities, as both mask ventilation and intubation can be compromised.

Here, we present the case of an 8-year-old child with complete arrhinia who underwent staged nasal reconstruction. We highlight the preoperative evaluation, intraoperative airway management strategies, and postoperative course.

Case Presentation

An 8-year-old girl (weight: 23 kg) with complete congenital arrhinia was scheduled for endoscopic recanalization of the nasal airway as part of staged nasal reconstruction. She had first presented to our institution at 8 months of age. She was born at term by emergency cesarean section due to fetal distress, with unremarkable antenatal scans.

Neonatal Period

At birth, the absence of an external nose was noted, and she developed immediate respiratory distress with cyanosis. Emergent orotracheal intubation was performed, and she was admitted to the NICU. She remained intubated for one week, during which she received ventilatory support and orogastric feeding. Following extubation, she gradually adapted to oral breathing. Examination revealed hypertelorism, complete arrhinia, and a high-arched palate. Chromosomal analysis was not performed. Cardiac evaluation and laboratory investigations were normal.

First intervention (8 months)

At 8 months, she underwent Stage 1 nasal reconstruction. Open and endoscopic creation of bilateral neo-choanae was performed, with placement of nasal stents to maintain patency. Serial dilations were carried out, and she initially achieved a patent nasal airway. However, over the following months, restenosis occurred due to scarring. The family did not maintain follow-up; stents dislodged, and the nasal openings completely closed by age 2. Despite this, she continued to mouth-breathe adequately without hypoxia or growth impairment, demonstrating successful adaptation.

Fig (1): Clinical and intraoperative images at 8 months of age during Stage 1 nasal reconstruction.

(A) Lateral view showing complete arrhinia with absent external nasal structures and midfacial flattening.

(B) Frontal view of the patient showing hypertelorism and complete congenital arrhinia.

Presentation at 8 years

At 8 years, she re-presented for Stage 2 reconstruction. Examination showed a cooperative child with normal cognition. The midface was flat, with no external nose and obliterated nostril openings. Hypertelorism and bilateral microphthalmia (with residual vision requiring thick glasses) were noted. She had a high-arched palate without cleft, mild maxillary hypoplasia, and a small but mobile mandible with good mouth opening. She maintained normal oxygen saturations (98–100% on room air), with no stridor or distress. She fed orally, adapting by taking small bites and pausing to breathe. No systemic anomalies were identified. CT imaging revealed arrhinia, hypoplastic paranasal sinuses, and Grade II adenoid hypertrophy.

The surgical plan involved endoscopic re-cannulation of bilateral nasal passages with scar tissue debridement, followed by placement of a biodegradable dermal regeneration template wrapped around pediatric ETTs (sizes 3.0–3.5 mm) to maintain patency. A future Stage 3 would address external nasal reconstruction.

Fig (2): Clinical and intraoperative images at 8 years of age during Stage 2 nasal reconstruction.

(A) Lateral postoperative view demonstrating flat midface profile.

(B) Frontal postoperative view at 8 years showing obliterated nasal passage with visible residual surgical scar.

(C) Intraoperative image showing bilateral recanalization with dermal matrix stents placed in the newly created nasal passages.

Anaesthetic Management

Pre-operative Evaluation

The anaesthesia team assessed her the day prior to surgery. Difficult airway was anticipated due to complete arrhinia and craniofacial abnormalities. Mask ventilation could be compromised (poor seal), and nasotracheal intubation was impossible. High-arched palate and retrognathia raised concern for a difficult laryngoscopic view (Mallampati III). However, she had normal neck mobility and adequate mouth opening, with no prior anaesthetic complications.

A comprehensive airway plan was formulated. A difficult airway cart was prepared, including pediatric fiberoptic bronchoscope, video laryngoscope (C-MAC® with Miller 2 and 3 blades), cuffed south pole ETTs (5.0 mm and smaller), LMAs (sizes 2–2.5), and surgical airway equipment. ENT standby was arranged for possible emergency tracheostomy. Risks were discussed with family, and written consent obtained.

No sedative premedication was given to preserve airway tone. She followed standard fasting guidelines and was well-hydrated.

Induction

In the OR, ASA standard monitoring was applied. Preoxygenation was achieved with 100% O₂ via a well-fitted face mask. IV induction included midazolam 1.15 mg, glycopyrrolate 0.25 mg, fentanyl 45 µg, and propofol 45 mg. Bag-mask ventilation was adequate. Succinylcholine 45 mg facilitated intubation.

Video laryngoscopy provided a Cormack-Lehane grade II view. A 5.0 mm cuffed south pole ETT was successfully inserted orally and secured to the chin, away from the surgical field. Cis-atracurium 3 mg IV was administered for relaxation. A throat pack was placed.

Maintenance and Intraoperative Course

Anaesthesia was maintained with sevoflurane (1.5–2.5% ET, in 50:50 air/O₂). Ventilation was volume-controlled, maintaining ETCO₂ at 35–40 mmHg. Ondansetron (0.1 mg/kg) was given prophylactically.

The surgical team recanalized bilateral nasal tracts under endoscopic guidance, with intermittent bleeding controlled by suction. Dermal matrix was sutured to 3–3.5 mm ETT and neo-stents created were secured into place. Blood loss was ~30 ml. Vital signs remained stable throughout.

Emergence and Extubation

At completion, throat pack was removed and airway suctioned. Dexamethasone (0.1 mg/kg) was administered for edema prophylaxis. Neuromuscular blockade was reversed with neostigmine and glycopyrrolate. Extubation was performed once the patient was fully awake with intact reflexes. She breathed comfortably via her mouth, unaffected by nasal stents.

Postoperative Care

She was monitored in PACU with supplemental humidified oxygen. Pain was controlled with IV paracetamol (20 mg/kg). Oral fluids were resumed after 6 hours, and she tolerated soft feeds by the next morning.

Airway monitoring showed no compromise, as she continued effective mouth-breathing. After overnight high-dependency observation, she was transferred to the ward. On POD 2, she started ambulation and vitals were stable. The ENT team recommended saline drops and sprays to maintain stent patency, with follow-up planned at 1 week.

Discussion

The embryological development of nose and nasal cavity occurs between third to tenth weeks of gestation. Congenital arrhinia is one of the rare defects that occurs during early embryogenesis with less than 50 cases reported worldwide.

Early diagnosis allows for better education of the parents and allows the medical team to be better prepared for delivery and its complications.

Treatment of arrhinia usually involves the treatment of airway obstruction and the management of feeding difficulties. Because newborns breathe compulsorily through the nose, the simultaneous needs of sucking and breathing result in respiratory distress. Inhaling and exhaling through the oral passage alone can cause chest retraction, further exacerbating this distress. Our patient was intubated soon after birth and she required minimal ventilatory support.

In the first reported cases, arrhinia was addressed by means of the creation of a patent airway as a first procedure while reconstruction of nose pyramid was delayed at school age or adolescence, bridging the period with an epithesis.

Our case confirms what it has previously reported in the literature regarding the fact that patients who completely lack nasal airways may adapt to oral breathing and feeding. Other reports, on the contrary, are in favour of tracheostomy and a delay of surgery at a later date.

In patients who present with respiratory distress, creation of a new airway through the maxillary bone should be performed as soon as possible in the post-natal period, leaving tracheostomy as a second choice.

Nevertheless, in absence of such symptoms, we agree on placing an ideal timing for the whole reconstructive procedure before school age, as at an earlier age such a complex operation may interfere with normal facial development. On the other hand, we believe that surgery should not be further postponed to adolescence, as restoring an acceptably normal appearance at a young age gives the child a better opportunity for social integration.

We choose to provide the airway restoration and the nasal reconstruction in a staged procedure, as we strongly believe that nasal respiration always represents a functional improvement, while we do not feel that possible growth impairment due to surgery represents a main problem in these patients, as maxillary growth is intrinsically deficient and they will certainly need maxillary osteotomy at the end of the growth.

In our opinion, the major issues of the correction of this deformity are the long-term patency of the new airway and the growth potential of reconstructed nasal structures. It is likely that in a growing child the cartilage graft will not grow enough to keep pace with adjacent structures, even though a limited growth of cartilage grafts was noted in ear, mandibular and nasal reconstruction.

We hope to be able to follow up the patient, as future functional and aesthetic refinements will be probably necessary.

Conclusion

Congenital arrhinia, though rare, presents significant neonatal and reconstructive challenges. Airway management is paramount in early life. Definitive management requires staged internal and external nasal reconstruction, ideally performed before school age to balance functional and psychosocial outcomes.

Successful treatment demands a multidisciplinary approach involving anaesthesiologists, pediatricians, ENT surgeons, maxillofacial surgeons, and plastic surgeons.

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Reconstructing absence: Airway management in a rare craniofacial abnormality