• HPI

    • 6-year-old boy presents with capillary malformations on feet, develops macular lesions over 3 years
    • New onset of motor tics involving arms, progressive headaches and neck pain

    Figure 1

    1. Which of the following is highest on your differential based on imaging and history?

    2. What treatment would you offer?
     
    3. What other testing should be considered?
    4. Which of the following describes you?
    5. I practice in one of the following locations.
    6. Please add any suggestions or comments regarding this case:
     
    • This is a very huge spinal MAV and is determinant to operate because it has compressive effects on spinal cord.

    • In this part of the word every patient with what ever disease they got demands treatment/cure without surgery.When no all other expectation fails they opt for surgery taking they are going to die.But now a days endovascular procedures are accepted very well. As it is not operation.Though results of operations-mortality/morbidity are as that of other most developed countries.Could it be that in this patr of the world no health insurance is there?

    • Need more information regarding the angiogram

      Difficult to see the actual fistula sometimes with one film

      Cause for headache - ? other lesions

      Cause for so called motor tic  - ? other lesion

    • nice good wellcome for the new comer with an amazing and dashing quiz that the poor trainee has yet to see in his residency.

    • While no certain I have appreciated the exact architecture of the malformation I suspect it is a perimedullary or craniospinal AVF and  this will likely come to either endovascular or microsurgical closure of a craniospinal fistula (to be determined after full assessment of the angiogram). Further clinical exam, MR whole neuraxis and genetic testing have a place but one option only is available so I opted for a neuraxis MR as potentially the single most useful investigation. The association between a spinal AVF, capillary malformations  and RASA1 mutation is described and this is a possible diagnosis here

    • This is  a rare case in my country

    • any surgical intervention may carry a devastating risk.

    Pubmed References

    • Heros RC, Debrun GM, Ojemann RG, et al: Direct spinal arteriovenous fistula: a new type of spinal AVM: case report. Journal of Neurosurgery 64: 134, 1986.
       
    • Hodes JE, Merland JJ, et al: Spinal vascular malformations: endovascular therapy. Neurosurgery Clinics of North America 5: 497-509, 1994. Muraszko KH, Oldfield EH: Vascular Malformations of the spinal cord and dura. Neurosurgery Clinics of North America 1: 631-652, 1990.
       
    • Oldfield EH, Doppman JL: Spinal arteriovenous malformations. Clinical Neurosurgery 34: 161-183, 1988.
       
    • Thiex R, Mulliken JB, Revencu N, Boon LM, Burrows PE, Cordisco M, Dwight Y, Smith ER, Vikkula M, Orbach DB., A novel association between RASA1 mutations and spinal arteriovenous anomalies.AJNR Am J Neuroradiol. 2010 Apr;31(4):775-9. doi: 10.3174/ajnr.A1907.
     

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