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. 2022 May 7:78:103732.
doi: 10.1016/j.amsu.2022.103732. eCollection 2022 Jun.

Klippel-Trenaunay Syndrome: Case series from a university hospital of Nepal

Robin Man Karmacharya  1 , Satish Vaidya  2 , Swechha Bhatt  3 , Ashish Tamang  3 , Rohit Bhasink Shrestha  3 , Niroj Bhandari  3 , Bijaya Paudel  3 , Manish Shah  3 , Gaurav Nepal  4
Affiliations

Klippel-Trenaunay Syndrome: Case series from a university hospital of Nepal

Robin Man Karmacharya et al. Ann Med Surg (Lond). .

Abstract

Introduction: Klippel Trenaunay Syndrome (KTS) is a rare congenital malformation with capillary and venous malformations and soft tissue/bony overgrowth with or without lymphatic malformation. Cutaneous vascular stain, varicosities and tissue hypertrophy represent its main clinical features. Besides, the patient can develop thromboembolic pathologies, recurrent bouts of infection, stasis eczema, limb length discrepancy and intolerable pain typical of intraosseous involvement.

Methods: Here, we report a case series of seven patients aged 10-45 who presented to our centre with clinical features suggestive of KTS. Out of them, six patients had involvement of unilateral lower limb, while only one had involvement of bilateral lower limb. They all had typical cutaneous vascular stains and underlying venous malformation, while one patient had developed complications with multiple ulcer formation.

Outcomes: An interdisciplinary team of vascular surgeons, dermatologists, interventional radiologists, orthopaedics, and physiotherapists managed the cases. We performed an individualized treatment as per the patient's presentation, which included a combination of supportive, medical, interventional radiologic, and surgical interventions. The follow-up outcomes of all the patients revealed significant resolution of symptoms.

Conclusion: Patients with KTS can have diverse presentations. Therefore, clinicians should ensure an individualized treatment with the involvement of a multidisciplinary team for proper management and prevention of complications.

Keywords: Case series; Compression bandages; Klippel-trenaunay syndrome; Port-wine stain; Sclerotherapy.

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Figures

Fig. 1
Fig. 1
Port-wine stain in the bilateral lower limb with soft tissue swelling in the right lower limb a) anterior view, b) posterior view.
Fig. 2
Fig. 2
a) Pre radiofrequency ablation picture of lateral marginal vein of Servelle b) Post radiofrequency ablation showing decreased prominence of the vein.
Fig. 3
Fig. 3
CT venogram showing right-sided Persistent embryonic lateral marginal Vein of Servelle.
Fig. 4
Fig. 4
Doppler Ultrasonography showing persistent embryonic lateral marginal vein of Servelle along with other dilated veins and multiple perforators.
Fig. 5
Fig. 5
Lower limb with right-sided skin discolouration, prominent dilated vein and girth discrepancy compared to left limb a) anterior view b) posterior view.
Fig. 6
Fig. 6
Flowchart showing management algorithm for treatment of Klippel-Trenaunay Syndrome.
See this image and copyright information in PMC

References

    1. Bathi R.J., Agarwal N., Burde K.N. Klippel-Trénaunay syndrome (angio osteohypertrophy syndrome): a report of 3 cases. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod. 2002;93:276–280. doi: 10.1067/moe.2002.119003. - DOI - PubMed
    1. Asghar F., Aqeel R., Farooque U., et al. Presentation and management of klippel-trenaunay syndrome: a review of available data. Cureus. 2020;12 doi: 10.7759/cureus.8023. - DOI - PMC - PubMed
    1. Vahidnezhad H., Youssefian L., Uitto J. Klippel-Trenaunay syndrome belongs to the PIK3CA-related overgrowth spectrum (PROS) Exp. Dermatol. 2016;25:17–19. doi: 10.1111/exd.12826. - DOI - PubMed
    1. Gloviczki P., Driscoll D.J. Klippel-Trenaunay syndrome: current management. Phlebology. 2007;22:291–298. doi: 10.1258/026835507782655209. - DOI - PubMed
    1. Agha R.A., Sohrabi C., Mathew G., et al. The PROCESS 2020 guideline: updating consensus preferred reporting of CasESeries in surgery (PROCESS) guidelines. Int. J. Surg. 2020;84:231–235. doi: 10.1016/j.ijsu.2020.11.005. - DOI - PubMed