Home » A Very Difficult Airway: Klippel-Trènaunay Syndrome and Upper Airway Bleeding

A Very Difficult Airway: Klippel-Trènaunay Syndrome and Upper Airway Bleeding

|Quality & Safety

Anesthesiology News | Posted: December 11, 2020 | By: Rutuja R. Sikachi, MD and Madina Gerasimov, MD

Madina Gerasimov, MD


According to the 2018 classification by the International Society for the Study of Vascular Anomalies, Klippel-Trènaunay syndrome (KTS) is a rare congenital disorder characterized by a combination of vascular malformations and limb overgrowth from soft tissue and bone hypertrophy.1

In the past, if arteriovenous malformations were seen along with other features of KTS, it was diagnosed as Klippel-Trènaunay-Weber syndrome. Currently, it is known as Parkes-Weber syndrome, which by itself is a distinct entity.2 KTS can potentially affect any organ system including lower and upper extremities, abdomen, chest, neck, cranium, spinal canal, bowel, bladder or other pelvic organs.2,3 The severity and extent of these malformations depend on the timing of in utero arrest in vascular development.4 Anesthesiologists may encounter these patients presenting for KTS-related and unrelated surgeries, deliveries, procedures and pain management.5-8 Due to possible multisystem involvement with varying severity, successful anesthetic management of these patients demands a multidisciplinary approach and adequate preparation.9 This article describes a case of difficult airway management in a patient with KTS secondary to bleeding from upper airway hemangiomas.

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References

  1. Sadick M, Muller-Wille R, Wildgruber M, et al. Vascular anomalies (part I): Classification and diagnostics of vascular anomalies. Rofo. 2018;190(9):825-835.
  2. Jacob AG, Driscoll DJ, Shaughnessy WJ, et al. Klippel-TrÈnaunay syndrome: spectrum and management. Mayo Clin Proc. 1998;73(1):28-36.
  3. Gloviczki P, Driscoll DJ. Klippel-TrÈnaunay syndrome: current management. Phlebology. 2007;22(6):291-298.
  4. Gloviczki P, Duncan A, Kalra M, et al. Vascular malformations: an update. Perspect Vasc Surg Endovasc Ther. 2009;21(2):133-148.
  5. Dispenza K. Severe pain related to Klippel-TrÈnaunay-Weber syndrome: a case study. Pain Manag Nurs. 2011;12(2):112-114.
  6. Dwivedi D, Sheshadri K, Tandon U, et al. Klippel-TrÈnaunay syndrome: a rare entity with anesthesia concerns. J Clin Anesth. 2016;35:233-234.
  7. Notarnicola A, Pesce V, Maccagnano G, et al. Klippel-TrÈnaunay syndrome: a rare cause of disabling pain after a femoral fracture. Arch Orthop Trauma Surg. 2012;132(7):993-996.
  8. Teixeira C, Braga AFA, Braga F, et al. Anesthesia for cesarean delivery in a patient with Klippel-TrÈnaunay syndrome. Rev Bras Anestesiol. 2018;68(6):641-644.
  9. Sangeetha RP, Baskar N, Kamath S, et al. Craniotomy in Klippel-TrÈnaunay syndrome: concerns and challenges. Indian J Anaesth. 2019;63(12):1033-1035.
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