Airway management of laryngeal manifestation of a patient with Klippel-Trénaunay syndrome

Introduction Klippel-Trénaunay syndrome (KTS) is a rare disease with vascular malformations associated with hyperplasia, usually of one extremity.

Case presentation A 49-year-old patient with KTS with known pharyngeal, laryngeal and cervical venous malformation and left vocal cord paresis presented with a 4-month-progressive dyspnea. Approximately 30 years before, a similar constellation made him need an emergency tracheostomy with perioperative massive blood transfusion and the performance of 6 revision surgeries. The glottis was not sufficiently visible. The patient rejected both the controlled-planned re-tracheostomy and a therapy trial with sirolimus. Systemic corticosteroids slightly improved the symptoms. For the permanent improvement of the airway situation we recommended the sclerotherapy with bleomycin. The patient required testing it before in another part of the body far from the respiratory tract. For non-clinical airway management, the thinnest point of passage from the outer neck to the trachea was permanently marked with a tattoo in a CT-controlled procedure, together with the neuroradiology. Together with anesthesia team an emergency ID card prepared, and the patient and his wife were provided with a Melker cricothyrotomy set and instructed to use it. The sclerotherapy with bleomycin in the region of the trunk was carried out under fluoroscopy. A neu appointment to assess its effect was arranged.

Conclusions The presented case of difficult airway management exemplifies complex situations that may arise in vascular anomalies and thus require interdisciplinary management within a specialized center.

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Publication History

Article published online:
10 June 2020