International Journal of Head and Neck Surgery

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VOLUME 14 , ISSUE 3 ( July-September, 2023 ) > List of Articles


Klippel–Trenaunay Syndrome with Hearing Loss: Is it the Cause? A Rare Case Report

Trilok C Guleria, Mahender Singh, Ramesh Azad, Narender K Mohindroo

Keywords : Case report, Hemangiomatous, Nevus, Osteohypertrophique, Tranuanay, Variqueux

Citation Information : Guleria TC, Singh M, Azad R, Mohindroo NK. Klippel–Trenaunay Syndrome with Hearing Loss: Is it the Cause? A Rare Case Report. Int J Head Neck Surg 2023; 14 (3):59-61.

DOI: 10.5005/jp-journals-10001-1557

License: CC BY-NC 4.0

Published Online: 10-10-2023

Copyright Statement:  Copyright © 2023; The Author(s).


Two French physicians, Maurice Klippel and Paul Trenaunay, described two patients with hemangiomatous lesions of the skin and coined the term “nevus variqueux osteohypertrophique.” Klippel–Trénaunay syndrome (KTS) is characterized by a triad of capillary malformations, venous malformations, and soft tissue or bony hypertrophy. The etiology of the syndrome is unknown. Diagnosis of KTS is mainly clinical. An appropriate multidisciplinary approach to treatment and prevention of possible complications of KTS provides optimal care for the patient. KTS with hearing loss is a very rare presentation. We describe a case report of KTS in a 5-year-old boy associated with bilateral conductive hearing loss.

  1. Arasu A, Khalil-Khan A, G KI, et al. A rare case of Klippel-Trenaunay Syndrome. Cureus 2022;14(10):e30128. DOI: 10.7759/cureus.30128
  2. Jacob AG, Driscoll DJ, Shaughnessy WJ, et al. Klippel-Trenaunay syndrome: spectrum and management. Mayo Clin Proc 1998;73(1):28–36. DOI: 10.1016/S0025-6196(11)63615-X
  3. Asghar FAqeel R, Farooque U, et al. Presentation and management of Klippel-Trenaunay syndrome: a review of available data. Cureus 2020;12(5):e8023. DOI: 10.7759/cureus.8023
  4. Berry SA, Peterson C, Mize W, et al. Klippel-Trenaunay syndrome. Am J Med Genet 1998;79(4):319–326. DOI: 10.1002/(SICI)1096-8628(19981002)79:4<319::AID-AJMG15>3.0.CO;2-U
  5. Cha SH, Romeo MA, Neutze JA. Visceral manifestations of Klippel-Trenaunay syndrome. Radiographics 2005;25(6):1694–1697. DOI: 10.1148/rg.256055042
  6. Biesecker LG, Happle R, Mulliken JB, et al. Proteus syndrome: diagnostic criteria, differential diagnosis, and patient evaluation. Am J Med Genet 1999;84(5):389–395. DOI: 10.1002/(sici)1096-8628(19990611)84:5<389::aid-ajmg1>;2-o
  7. Lee BB, Do YS, Byun HS, et al. Advanced management of venous malformation with ethanol sclerotherapy: mid-term results. J Vasc Surg 2003;37(3):533–538. DOI: 10.1067/mva.2003.91
  8. Ishimoto S, Ito K, Matsuzaki M, et al. Sensorineural hearing loss with intracranial venous malformations in Klippel-Trenaunay syndrome. Ann Otol Rhinol Laryngol 2002;111(6):558–562. DOI: 10.1177/000348940211100613
  9. Suver DW, Perkins J, Manning SC. Klippel-Trenaunay-Weber syndrome with labyrinthine bony overgrowth and mixed hearing loss, a case report. Int J Pediatr Otorhinolaryngol 2004;68(8):1075–1079. DOI: 10.1016/j.ijporl.2004.03.003
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