[MIM 149 000]

(osteohypertrophic varicose nevus, Klippel-Trenaunay-Weber syndrome)

Sporadic. It is different from Parkes-Weber syndrome (see this term) with which it is often confused and which is characterized by arteriovenous malformations..

Association at the level of a (generally lower) limb of:

-        a low flow vascular malformation with capillary (wine stain, venous venous ectasies) and lymphatic (micro- and macrocysts) anomalies

-        hypertrophy of bone and fat components.

There is a risk of chronic subclinical fibrinolysis by local consumption of blood platelets and clotting factors. There is no association with arteriovenous malformations at the spinal level.

The presence of hemangiomas at the pulmonary, digestive, hepatic, or urinary level is described in about 20 to 30% of cases. The head and neck, and so the upper airway can also be affected (14 %).

A venous malformation is sometimes present at the anorectal level. It may be misdiagnosed with hemorroids: its sclerosis may induce a thrombosis of the portal vein !

Risk of local infectious complications and thrombosis with pulmonary emboli.

It is different from the Parkes-Weber syndrome (see this term) with which it is often confused and which presents arteriovenous malformations.

Anesthetic implications:

risk of surgical bleeding. It may be useful to start an antifibrinolytic (tranexamic acid), or thromboprophylactic (low molecular weight heparin) therapy, in the perioperative period: seek the advice of a specialist in hemostasis (measure the preoperative D-dimers level). Check the absence of perimedullar vascular malformations before performing any neuraxial block. Risk of hemoptysis. Risk of postoperative pulmonary embolism.

References : 

• Ezri T, Szmuk P, Panksy A, Itzhak IB, Geva D. 
  Anaesthetic management for Klippel-Trenaunay-Weber syndrome. 
  Pediatr Anesth 1996; 6:81-2.
• Christie IW, Ahkine PA, Holland RL. 
  Central regional anaesthesia in a patient with Klippel-Trenaunay syndrome. 
  Anaesth Intensive Care 1998; 26:319-21.
• Pereda Marin RM, Garcia Collada JC, Garrotte Martinez AI, Miralles Serrano EM, Morales Aguilar JL.
  Anesthetic management of Klippel-Trenaunay syndrome and attendant gastrointestinal hemorrhage.
  Minerva Anesth 2007; 73: 187-90.
• Alomari AI, Orbach DB, Mulliken JB, Bisdorrff A, Fishman SJ, Norbash A, Alokaili R, Lord DJ, Burrows PE. 
  Klippel-Trenaunay syndrome and spinal arteriovenous malformation : an erroneous association.
  AJNR 2010 ; 31 : 1608-12
• Barbara DW, Wilson JL. 
  Anesthesia for surgery related to Klippel-Trenaunay syndrome: a review of 136 anesthetics. 
  Anesth Analg  2011; 113: 98-102
• Holak EJ, Pagel PS.
  Successful use of spinal anesthesia in a patient with severe Klippel-Trenaunay syndrome associated with upper airway abnormalities and chronic Kasabach-Merritt coagulopathy.
  J Anesth 2010; 24: 134-8
• Dwivedi D, Sheshadi K, Tandon U, Chakraborty S.
  Klippel-Trenaunay syndrome: a rare entity with anesthetic considerations.
  J Clin Anesth 2016, 35 : 233-4
• Elajmi A, Clapuyt P, Hammer F, Bataille A-C, Lengele B, Boon LM.
  Prise en charge des anomalies vasculaires chez l’enfant.
  Ann Chir Plast Esth 2016 ; 61 :480-97.
• Mestdagh F, Moreau N.
  Anesthetic management of a parturient with hemihypertrophy and vascular malformation : a case report.
  Acta Anaesth Belg 2018 ; 69 : 225-9.
• Mukaihara K, Godai K, Moriyama T.
  Tracheal varicose veins associated with Klippel-Trenaunay syndrome.
  Anesthesiology 2021 ; 135 : 1121
• Wang H, Lin W, Guo Z.
  Klippel-Trenaunay syndrome with anorectum involvement and portal hypertension.
  Pediatrics International 2022 ; 64 e15165

Updated: July 2022