• Users Online: 490
  • Print this page
  • Email this page

Table of Contents
Year : 2022  |  Volume : 30  |  Issue : 3  |  Page : 233-234

Sciatic nerve hemangioma, a challenge in klippel–Trenaunay syndrome for perioperative analgesia

Department of Anesthesiology, Sancheti Hospital, Pune, Maharashtra, India

Date of Submission09-Jun-2021
Date of Decision12-Jul-2021
Date of Acceptance04-Oct-2021
Date of Web Publication19-Jul-2022

Correspondence Address:
Dr. Nitin Gawai
Department of Anesthesiology, Sancheti Hospital, Pune - 411 005, Maharashtra
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jmu.jmu_130_21

Rights and Permissions

Children with Klippel–Trenaunay syndrome have vascular malformations, varicosities at unusual distribution, and unilateral soft and skeletal tissue hypertrophy of the lower extremity. Corrective surgery of the deformity is necessary to improve gait and wear acceptable footwear. Perioperative pain relief for debulking of the right great toe in a known case of KTS with sciatic hemangioma was achieved with ultrasound-guided sciatic nerve block in an avascular area.

Keywords: Klippel–Trenaunay syndrome, magnetic resonance imaging, ultrasound-guided sciatic nerve block, vascular malformations

How to cite this article:
Gawai N, Bhong G, Diwan S, Sancheti P. Sciatic nerve hemangioma, a challenge in klippel–Trenaunay syndrome for perioperative analgesia. J Med Ultrasound 2022;30:233-4

How to cite this URL:
Gawai N, Bhong G, Diwan S, Sancheti P. Sciatic nerve hemangioma, a challenge in klippel–Trenaunay syndrome for perioperative analgesia. J Med Ultrasound [serial online] 2022 [cited 2023 Jan 30];30:233-4. Available from: http://www.jmuonline.org/text.asp?2022/30/3/233/351397

  Introduction Top

The condition was first described by French physicians Maurice Klippel and Paul Trenaunay in 1900; they referred to it as nevus vasculosus osteohypertrophicus.[1] Klippel–Trenaunay syndrome (KTS) is a classical triad that consists of vascular malformations, varicosities at unusual distribution, and unilateral soft and skeletal tissue hypertrophy, usually of the lower extremity.[2],[3] Children need corrective orthopedic surgeries of the lower limb. Apart from subcutaneous hemangiomas in the abdominal area, neuraxial hemangiomas are a known occurrence in KTS.[4],[5] We report a case of KTS for debulking of the great toe under general anesthesia with perioperative pain relief achieved with an ultrasound-guided peripheral nerve block. Ultrasound may be preferable to landmark technique when aberrant anatomy is suspected.[6],[7]

  Case Report Top

An 8-year-old boy weighing 26.7 kg with KTS presented with right great toe hypertrophy along with soft-tissue swelling in the lower abdomen and medial aspect of the right thigh [Figure 1]a. Hypertrophy of the great toe hindered his gait and wearing proper footwear. He, therefore, was posted for osteotomy and debulking of the great toe. Laboratory reports and chest radiograph were normal. Examination revealed multiple compressible swellings on the right hypochondrium, lumbar and iliac quadrants of the abdomen, and right upper medial thigh [Figure 1]b. Magnetic resonance imaging (MRI) revealed multiple hemangiomas in the liver, spleen, and rectum.
Figure 1: (a) Hypertrophy of the right great toe. (b) Subcutaneous hemangiomas on the right abdominal quadrants. (c) Ultrasound detection of vascular malformations around the sciatic nerve. (d) Ultrasound-guided mid-thigh sciatic nerve block. ST: Semitendinosus muscle; SM: Semimembranosus muscle; BF-LH: Biceps femoris long head muscle, BF-SH: Biceps femoris short head, F: Femur, SN: Sciatic nerve, PA: Popliteal artery, LA: Local anesthetic

Click here to view

Written informed consent was obtained from parents for surgical procedures to be performed under general anesthesia and peripheral nerve block for perioperative pain relief. Anesthesia was induced with intravenous (IV) fentanyl 1 μg/kg and propofol 2 mg/kg, and a laryngeal mask airway (2.5) was inserted. Anesthesia was maintained with nitrous in oxygen and sevoflurane through a closed circuit. Since the surgical incision was planned on the medial side of the right great toe and forefoot, an adductor canal block and sciatic nerve block were planned for postoperative pain relief. In the left lateral decubitus position, the sciatic nerve was scanned in real-time posteriorly from the greater trochanter level until the popliteal fossa, with a linear probe (Sonosite M-Turbo, USA – 6–13 mHz). The scan revealed an elliptical sciatic nerve surrounded by several pulsatile vascular structures [Figure 1]c encircling the sciatic nerve throughout its course from the gluteal area to the popliteal fossa, except in one area at mid-thigh level. This was abnormal and considered a part of the KTY syndrome. Scan around the ankle, too, revealed anomalous vascular structures. The nonvascular zone at the mid-thigh level of the sciatic nerve was considered a safe zone to administer an in-plane sciatic nerve block. No vascular malformations were observed in the entire adductor canal. US-guided and neurostimulation-aided sciatic nerve block was performed in the identified safe zone [Figure 1]d and [Video 1] [Additional file 1], and 0.2% of 11-ml ropivacaine and 15 μg of clonidine were injected after eliciting plantar flexion at 0.4 mA. A saphenous nerve block was administered in the adductor canal in the mid-thigh area with 8 ml of 0.2% ropivacaine. At surgical incision, there was no rise in heart rate and blood pressure more than 20% of baseline. No additional analgesics were required in the intraoperative period. IV paracetamol 500 mg was administered 30 min before extubation.

Postoperatively in the recovery room, the patient was pain free with a Visual Analog Scale (VAS) score of 1–2. No motor block was observed. The child complained of pain in the foot for the first time at 14.5 h after the surgery at a VAS = 4. IV paracetamol 500 mg was injected every 8 h thereafter until the patient was discharged on the 3rd postoperative day. At discharge, sensations were preserved in the distribution of the femoral and sciatic nerve in the foot. Plantar and dorsiflexion of the foot and movements of toes were normal.

  Discussion Top

Regional anesthesia is a challenge in KTS because of associated vascular malformations reported in the spinal canal.[4],[5] Although MRI scanning can detect central neuraxial vascular malformation, it is prudent to avoid central neuraxial blocks when peripheral nerve blocks can provide equivalent analgesia.[8] We could detect vascular malformations around the entire length of the sciatic nerve [Figure 1]c from the gluteal area to the popliteal fossa, except in one area at the mid-thigh level [Figure 1]d. This was considered a safe zone to administer sciatic nerve blocks. With vascular malformations around the ankle joint, the ankle block was excluded. Vascular hemangiomas have been reported around the ankle joint.[9] This emphasizes the role of ultrasound in diagnosing vascular malformations before implementing a block in diagnosed KTY patients. A saphenous nerve block was added as a supplemental nerve block considering the incision on the medial aspect of the great toe and forefoot.

  Conclusion Top

This report emphasizes the role of the US in diagnosing vascular malformations in peripheral nerves, assisting regional anesthesia for improved analgesia, and an overall better outcome. Further, we would like to caution regional anesthesia enthusiasts to refrain from administrating neuraxial anesthesia and landmark-guided blocks in KTY syndromes or similar disorders associated with hemangiomas elsewhere.

Declaration of patient consent

The authors certify that they have obtained appropriate patient's guardian consent form. In the form, the guardian has given the consent for the child's images and other clinical information to be reported in the journal. The guardian understands that the child's name and initial will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Klippel M, Trénaunay P. Du naevus variqueux ostéohypertrophique. Arch Gén Méd 1900;3:641-72.  Back to cited text no. 1
You CK, Rees J, Gillis DA, Steeves J. Klippel-Trenaunay syndrome: A review. Can J Surg 1983;26:399-403.  Back to cited text no. 2
Liguori A, Jorgensen SA, Towbin AJ, Towbin R. Klippel-Trenaunay syndrome. Appl Radiol 2018;47:35-6.  Back to cited text no. 3
Nakstad PH, Hald JK, Bakke SJ. Multiple spinal arteriovenous fistulas in Klippel-Trenaunay-Weber syndrome treated with platinum fibre coils. Neuroradiology 1993;35:163-5.  Back to cited text no. 4
Rohany M, Shaibani A, Arafat O, Walker MT, Russell EJ, Batjer HH, et al. Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome: A literature search and report of two cases. AJNR Am J Neuroradiol 2007;28:584-9.  Back to cited text no. 5
Han DS, Wu WT, Hsu PC, Chang HC, Huang KC, Chang KV. Sarcopenia is associated with increased risks of rotator cuff tendon diseases among community-dwelling elders: A cross-sectional quantitative ultrasound study. Front Med 2021;8:630009.  Back to cited text no. 6
Chang PH, Chen YJ, Chang KV, Wu WT, Özçakar L. Ultrasound measurements of superficial and deep masticatory muscles in various postures: Reliability and influencers. Sci Rep 2020;10:14357.  Back to cited text no. 7
Pereda Marin RM, Garcia Collada JC, Garrote Martinez AI, Miralles Serrano EM, Morales Aguilar JL. Anaesthetic management of Klippel – Trenaunay syndrome and attendant gastrointestinal haemorrhage. Minerva Anestesiol 2007;73:187-90.  Back to cited text no. 8
Deka JB, Deka NK, Shah MV, Bhatnagar N, Nanni AL, Jimenez F. Intraneural hemangioma in Klippel–Trenaunay syndrome: Role of musculoskeletal ultrasound in diagnosis – Case report and review of the literature. J Ultrasound 2020;23:435-42.  Back to cited text no. 9


  [Figure 1]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded78    
    Comments [Add]    

Recommend this journal