Double jeopardy in airway management: Klippel- Feil syndrome with restricted neck extension and nasal polyp

Sasikumar palanivel*, Reshma ponnusamy

Department of Anaesthesiology, Kauvery hospital, Trichy, Tamil Nadu

Background

Klippel-Feil syndrome (KFS) is a rare congenital disorder (incidence 1 in 42,000 births) characterized by the triad of a short neck, low posterior hairline, and limited cervical range of motion due to vertebral fusion. These anatomical abnormalities contribute to a difficult airway, as limited neck extension and potential cervical spine instability may complicate direct laryngoscopy and increase the risk of neurological injury during intubation. Antrochoanal polyposis, a form of nasal polyp arising from the maxillary sinus and extending to the choana, is itself extremely rare and causes chronic nasal obstruction. The coexistence of KFS and nasal polyp poses a “double jeopardy” for airway management, as cervical fusion limits traditional oral intubation maneuvers while nasal obstruction precludes the use of nasal airways or fiberoptic routes. We present a case of a 19-year-old female with this dual pathology scheduled for functional endoscopic sinus surgery (FESS) under general anaesthesia, and discussing the airway management challenges and strategies employed.

Fig (1). Clinical features observed in patient. (A) Anterior view showing antrochoanal polyp and short webbed neck. (B) Posterior view showing fused cervical spine.

Case Presentation

A 19-year-old female presented with chronic nasal discharge, bilateral nasal obstruction, and mouth-breathing. She was diagnosed with antrochoanal nasal polyp by the ENT team and scheduled for FESS under general anaesthesia. On examination, she had a short webbed neck and pectus carinatum chest shape. Airway assessment revealed a Mallampati class II, adequate mouth opening, and thyromental distance of 5.2 cm. Neck mobility was markedly restricted, with very limited extension at the cervical spine. Systemic examination was unremarkable and vital signs were within normal limits. Cervical spine imaging confirmed congenital fusion of C2–C4 vertebrae (Klippel-Feil anomaly) and hypoplasia of the left scapula (Sprengel’s deformity), consistent with her syndrome. These findings indicated a potentially difficult airway due to reduced neck extension and altered upper spine anatomy.

Fig (2): Coronal CT showing left antrochoanal polyp extending from maxillary sinus into nasal cavity and choana.

Anaesthesia and surgical plans were explained to the patient, including the possibility of an awake intubation given the anticipated difficult airway. A comprehensive difficult airway cart was prepared, including a fiberoptic bronchoscope, video laryngoscope, supraglottic airway devices, and cricothyrotomy kit. The initial plan was to perform an awake fiberoptic intubation (FOI) with the patient breathing spontaneously, given that awake FOI is considered the gold standard in managing such difficult airways. However, upon arrival to the operating room, the patient became extremely anxious and refused awake instrumentation. Attempts at conscious sedation were difficult, and she remained uncooperative for awake FOI. Therefore, the airway plan was revised.

General anaesthesia was induced in a controlled manner (after thorough preoxygenation) using intravenous agents (fentanyl, propofol) while maintaining manual in-line stabilization of the neck in neutral position.

Fig (3): After induction of general anaesthesia, the patient was intubated in a neutral cervical position using a King Vision™ videolaryngoscope. The image shows 7.0 mm cuffed oral endotracheal tube secured in place through first attempt, with minimal neck extension and standard monitors applied.

A Cormack-Lehane grade 2a view of the glottis was obtained on the videolaryngoscope. No excessive force or neck manipulation was required. Following intubation, anaesthesia was maintained with sevoflurane in an oxygen/air mixture, and a continuous infusion of dexmedetomidine was used for analgesia and to promote hemodynamic stability

The FESS proceeded uneventfully over 2.5 hours. The surgical team removed large nasal polyp endoscopically (Figure 4), and achieved hemostasis in the sinonasal cavities. Throughout the procedure, mechanical ventilation was controlled and oxygenation remained adequate. At the end of surgery, neuromuscular blockade was fully reversed (neostigmine and glycopyrrolate) and the patient was carefully extubated when awake, obeying commands and with protective airway reflexes intact. Extubation was smooth and no airway compromise occurred. The patient was observed in recovery and had an uneventful postoperative course. She reported mild sore throat but no neurologic symptoms. Analgesia was maintained with paracetamol and intermittent fentanyl. She was discharged on the 3rd post-operative day with intact neurologic status and improved nasal breathing.

Fig (4): The excised left antrochoanal nasal polyp shown next to a 2mL syringe, a large, benign mass occupying the nasopharyngeal airway, explaining her complete nasal obstruction.

Discussion

Klippel-Feil Syndrome

Klippel-Feil syndrome is a rare congenital disorder characterized by classical triad: low posterior hairline, short neck, and restricted range of motion in the cervical spine. It predominantly affects females. Cervical spine CT imaging reveals complete fusion of the C2, C3, and C4 vertebrae, along with a reduced vertebral body diameter. This vertebral fusion limits neck extension and rotation, increasing the risk of cervical spine injury during airway management. The condition is frequently associated with craniofacial abnormalities, which may further complicate airway access.

Nasal Polyp

Nasal polyps can obstruct the nasal passages, leading to significant challenges during airway management. These include difficulty with nasal intubation, insertion of a nasopharyngeal airway, and performing nasal fibre optic intubation.

Airway challenges:

Airway challenges Causes
Restricted mouth openingPossible in KFS
Restricted neck extension Cervical bony fusion
Nasal obstruction Polyp
Airway axis alignment difficulty Fused cervical spine
Difficulty in rescue oxygenation Nasal obstruction limits oxygenation/ adjuncts

Airway planning

Preoperative Plan

  • Perform a thorough airway assessment
  • Prepare a difficult airway cart
  • Inform the patient that an awake intubation technique may be necessary

Airway Plan

  • Awake fibre optic intubation (FOI) via the oral route, as the nasal route is contraindicated due to nasal polyp
  • Maintain spontaneous ventilation throughout the procedure
  • Apply topical anaesthesia meticulously
  • Sedation with dexmedetomidine at 0.5 µg/kg/hr

Alternate Plan

  • Use video laryngoscopy with minimal neck manipulation

Rescue Plan

  • Supraglottic airway device (SAD)
  • Surgical airway intervention

Extubation Strategy

  • Extubate only after full recovery of consciousness and protective airway reflexes
  • Consider using an airway exchange catheter
  • Have a reintubation plan in place
  • Maintain a well-defined backup strategy.

Conclusion

Patients with Klippel-Feil syndrome present significant airway management challenges due to cervical spine fusion and limited neck mobility. When combined with anatomic nasal obstruction from antrochoanal polyps, the difficulty is compounded, as both oral and nasal approaches to the airway are compromised. This case highlights the importance of meticulous planning and adaptability: an awake fiberoptic intubation is ideal for KFS, but patient factors may necessitate switching to alternate strategies such as video laryngoscopy. Careful preparation with a graded airway plan (primary, backup, and rescue options) and vigilant extubation planning are essential. By anticipating potential pitfalls and having all necessary equipment and expertise on hand, we safely managed the “double jeopardy” airway and ensured a successful outcome. This case underlines the value of flexibility in airway management and the need for individualized strategies when multiple challenging conditions coexist.

References

  • Cherrabi K, Touihem N, Nakkabi I, Nadour K. Bilateral antrochoanal polyps: a case report. Egypt J Otolaryngol. 2021; 37:30.
  • Singh M, Prasad R, Jacob R. Anaesthetic challenges in a patient with Klippel-Feil syndrome undergoing surgery. Indian J Anaesth. 2005;49(6):511–514.
  • Ahuja V, Kazal S, Gombar S, Thapa D, Bahadur R. Glidescope® for predicted difficult airway in Klippel-Feil Syndrome. J Anaesthesiol Clin Pharmacol. 2012;28(4):532–534.
  • Shah TH, Badve MS, Olajide KO, Skorupan HA, Waters JH, Vallejo MC. Dexmedetomidine for an awake fiber-optic intubation of a parturient with Klippel-Feil Syndrome and Arnold–Chiari malformation. Clinics and Practice. 2011;1(3):e57.
  • Popat M, Mitchell V, Dravid R, Patel A, Swampillai C, Higgs A. Difficult Airway Society Guidelines for the management of tracheal extubation. 2012;67(3):318–340.

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