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Combined surgical and endovascular treatment for spinal arteriovenous fistula associated with Parkes Weber syndrome

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Abstract

Background

Parkes Weber syndrome (PWS) is a rare and congenital vascular malformation manifesting as hemihypertrophy of the extremities, cutaneous hemangiomas, varicose veins, and arteriovenous fistula of the affected limbs. The incidence rate of spinal arteriovenous fistula (AVF) associated with PWS is extremely rare.

Case presentation

We reported a case of an adolescent girl with PWS who presented with a rupture spinal perimedullary AVF at the level of T12-L1. She was successfully treated with emergent surgical decompression and subsequent endovascular embolization. The clinical features and treatment of spinal AVF associated with PWS were discussed and a brief literature review was presented.

Conclusion

Based on this case report, we suggested that the management of spinal AVF in PWS should also be individualized and be tailored according to the condition and expectation of the patients as well as the angioarchitecture of the vascular malformation.

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References

  1. Wassef M, Blei F, Adams D, Alomari A, Baselga E, Berenstein A, Burrows P, Frieden IJ, Garzon MC, Lopez-Gutierrez JC, Lord DJ, Mitchel S, Powell J, Prendiville J, Vikkula M, Board I, Scientific C (2015) Vascular anomalies classification: recommendations from the international society for the study of vascular anomalies. Pediatrics 136:e203–e214

    Article  Google Scholar 

  2. Courivaud D, Delerue A, Delerue C, Boon L, Piette F, Modiano P (2006) Familial case of Parkes Weber syndrome. Ann Dermatol Venereol 133:445–447

    Article  CAS  Google Scholar 

  3. Revencu N, Boon LM, Mulliken JB, Enjolras O, Cordisco MR, Burrows PE, Clapuyt P, Hammer F, Dubois J, Baselga E, Brancati F, Carder R, Quintal JM, Dallapiccola B, Fischer G, Frieden IJ, Garzon M, Harper J, Johnson-Patel J, Labreze C, Martorell L, Paltiel HJ, Pohl A, Prendiville J, Quere I, Siegel DH, Valente EM, Van Hagen A, Van Hest L, Vaux KK, Vicente A, Weibel L, Chitayat D, Vikkula M (2008) Parkes Weber syndrome, vein of Galen aneurysmal malformation, and other fast-flow vascular anomalies are caused by RASA1 mutations. Hum Mutat 29:959–965

    Article  CAS  Google Scholar 

  4. Li ZF, Li Q, Xu Y, Hong B, Huang QH, Liu JM (2017) Spinal arteriovenous malformation associated with Parkes Weber syndrome: report of two cases and literature review. World Neurosurg 107:1043 e1047–1043 e1013

    Google Scholar 

  5. Klippel CT (1900) Du naevus variqueux osteohypertrophique. Archives Generales de Medicine (Ser 2):641–672

  6. Parkes-Weber F (1918) Haemangectatic hypertrophy of limbs congenital phlebarteriectasis and so-call congenital varicose. Br J Child Dis 15:12–23

    Google Scholar 

  7. Mullins JF, Naylor D, Redetski J (1962) The Klippel-Trenaunay-Weber syndrome: naevus vasculosus osteohy-pertrphicus. Arch Dermatol 86:202–206

    Article  CAS  Google Scholar 

  8. Benhaiem-Sigaux N, Zerah M, Gherardi R, Bellot J, Hurth M, Poirier J (1985) A retromedullary arteriovenous fistula associated with the Klippel-Trenaunay-Weber syndrome. A clinicopathologic study. Acta Neuropathol 66:318–324

    Article  CAS  Google Scholar 

  9. Nakstad PH, Hald JK, Bakke SJ (1993) Multiple spinal arteriovenous fistulas in Klippel-Trenaunay-Weber syndrome treated with platinum fibre coils. Neuroradiology 35:163–165

    Article  CAS  Google Scholar 

  10. Rohany M, Shaibani A, Arafat O, Walker MT, Russell EJ, Batjer HH, Getch CC (2007) Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome: a literature search and report of two cases. AJNR Am J Neuroradiol 28:584–589

    CAS  PubMed  PubMed Central  Google Scholar 

  11. Tokunaga K, Hishikawa T, Sugiu K, Date I (2015) Combined transarterial and transvenous approach for curative obliteration of Klippel-Trenaunay-Weber syndrome-associated spinal perimedullary arteriovenous fistulas. Clin Neuroradiol 25:291–294

    Article  CAS  Google Scholar 

  12. Bagherpour AN, Rodriguez GJ, Moorthy C, Trier TT, Maud A (2016) Combined surgical and endovascular treatment of complex high-flow conus medullaris arteriovenous fistula associated with Parkes Weber syndrome: case report. J Neurosurg Spine 25:234–238

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Neurosurgery, Shanghai Ninth People’s Hospital, Affiliated to Shanghai JiaoTong University School of Medicine, NO.639 Shanghai Zhizaoju Road, Huangpu District, Shanghai, China

    Chenlong Liao, Huoniu Ouyang & Wenchuan Zhang

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  1. Chenlong Liao
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  2. Huoniu Ouyang
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  3. Wenchuan Zhang
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Contributions

Chenlong Liao: conceptualization, formal analysis, investigation, writing-original draft.

Huoniu Ouyang: data curation, writing-review.

Wenchuan Zhang: supervision, validation, writing-review.

Corresponding author

Correspondence to Wenchuan Zhang.

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This study was approved by Ethics Committee of Shanghai Ninth People’s Hospital

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Informed consent was obtained from the patient included in this study.

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Informed consent was obtained from the patient included in this study.

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The authors declare that they have no conflict of interest.

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Liao, C., Ouyang, H. & Zhang, W. Combined surgical and endovascular treatment for spinal arteriovenous fistula associated with Parkes Weber syndrome. Childs Nerv Syst 38, 677–681 (2022). https://doi.org/10.1007/s00381-021-05187-8

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  • DOI: https://doi.org/10.1007/s00381-021-05187-8

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