Venous Malformation in Upper Airway – Anesthetic Challenges and Management: A Case Report

S. Nirmal Kumar, Mohammed Elias, K. Senthil Kumar

Department of Anaesthesiology, Kauvery Hospitals, Trichy

Abstract

We present a case of venous malformation involving upper airway, who developed airway obstruction post extubation requiring re-intubation and tracheostomy. Our reflection on the management of this case highlights several important learning points. These include the importance of clear communication between different medical specialties to promote shared situation awareness, the importance of training anaesthesiologists in the limitations of standard difficult airway management algorithms, and the implications of the skill set mix of doctors responding to airway emergencies.

Keywords: venous malformation, airway vascular malformation

Background

Venous malformation involving airway deserve special consideration as proper recognition and management can be life saving. Venous malformation of head and neck can thrombose, engorge causing dis-figurement, and cause pain and obstruction of airway. Multidisciplinary approach is ideal in managing these patients.

Case Presentation

A 9 yr old child presented with venous malformation over lower lip, tip of the tongue and right side of neck, present since birth. He was planned for sclerotherapy under anesthesia. Patient’s milestones were normal and asymptomatic in regard to swallowing, breathing and phonation. Also, mother gave history of one episode of partial obstruction of airway during vigorous crying in the past.

On examination, apart from lesion over the lower lip, tip of the tongue & other areas visualized with tongue depressor were free from malformation. MRI showed extensive venous malformation over right side of neck, lip, tongue and over right parotid region.

Fig. 1. Lesion over lower lip.

Fig. 2. MRI images showing malformation.

Procedure was planned under GA with ETT and controlled ventilation.ENT surgeon back up was ensured during procedure. After induction gentle laryngoscopy revealed diffuse malformation over lateral pharynx, either side of vallecula and beneath arytenoids. Intubation done with cuffed endotracheal tube and proceeded with procedure. Sclerosant injection done over lip & tongue by Plastic Surgery team and ultrasonography guided sclerosant injection done over Right side of neck by Radiologist team. Post procedure, child awakened well and with ENT physician presence, extubation was done.

Post-extubation patient seemed to have partial obstruction which worsened within few minutes. The lesion present over lip and tongue doubled in size further complicating airway obstruction.

Fig. 3. Glottic image post extubation.

Fig. 4. Engorged lesion.

Immediately fibre optic bronchoscopy was done that showed extensive engorgement of malformations. Reintubation was done under fibre optic guidance. Due to non resolving engorgement, Tracheostomy was done after 48 hours. On follow up after 1 month, 80% of lesion had resolved over tongue and lip. Upper airway endoscopy was done which revealed resolving lesion over vallecula and beneath arytenoids. Tracheostomy decannulation was done by ENT surgeon and discharged after observation.

Fig. 6. Follow up lesion.

Fig. 7. Follow up endoscopy.

Discussion

Venous malformation is the most common type of congenital vascular malformation with incidence of 1 to 2 in 10000 and prevalence of 1% [1,2]. Venous malformation involving airway deserve special consideration as proper recognition and management can be lifesaving [3].

Howuton [4] reported extubation difficulty in patient with venous malformation involving airway, causing obstruction and requiring re-intubation. Davidson et al [5] reported a case of rapidly compromised upper airway due to rupture of arteriovenous malformation involving inferior thyroid artery requiring cricothyroidotomy following failed attempts multiple intubation attempts. Diagnostic imaging of malformation helps in managing patients accordingly. Endotracheal intubation during first attempt was successful and uneventful in our patient.

Complications are more reported during extubation. Our patient had venous engorgement with multiplying size of lesion mostly due to obstruction of airway causing increased intra-thoracic pressure leading to increased venous filling with further added interstitial edema due to sclerotherapy. Meta analysis done by Lucia et al [6] reported temporary local swelling rates were high after sclerotherapy injection.

Gorostidiet et al [7] suggested complex and/or multilevel lesions obstructing the upper airway are difficult to treat and are best addressed under a tracheotomy-secured airway. Performing a tracheotomy in case of a large vascular anomaly of the neck can itself be challenging. In our patient ENT surgeon made incision over left side as midline approach was difficult due to venous engorgement from left side of neck crossing midline.

Conclusion

Venous malformations are present at birth and grow commensurately with the child, causing pain and discomfort. Ultrasonography and Magnetic Resonance imaging are the prime imaging techniques in the work up of venous malformation. We suggest a Pre operative upper airway endoscopy evaluation in these patients in order to be prepared for the complication peri-operatively. Multidisciplinary approach and advanced airway equipment preparation will make management of these venous malformation involving the airway easier for Anesthesiologists.

References

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Eifert S, et al. Prevalence of deep venous anomalies in congenital vascular malformations of venous predominance. J Vasc Surg. 2000;31:462-71.
Behravesh S, et al. Venous malformations: clinical diagnosis and treatment. Cardiovasc Diagn Ther. 2016;6(6):557-69.
Howton M, et al. Intraoral vascular malformation and airway management: A case report and review of the literature. J Clin Anesth. 1992;4(6):498-502.
Davidson A, et al. Rapid upper airway obstruction after arteriovenous malformation rupture in a patient with neurofibromatosis. Anaesth Rep. 2021;9(2).
De Maria L, et al. Sclerotherapy for low-flow vascular malformations of the orbital and periocular regions: Systematic review and meta-analysis. Surv Ophthalmol. 2020;65(1):41-7.
Gorostidi F, et al. Pediatric vascular anomalies with airway compromise. J Oral Pathol Med. 2022.

Dr. S. Khaja Mohideen

Anaesthesiologist

Dr. K. Senthil Kumar

Head of the Department, Anaesthesiology and Toxicology

Venous Malformation in Upper Airway – Anesthetic Challenges and Management: A Case Report