Korean J Clin Neurophysiol.  2015 Dec;17(2):76-79. 10.14253/kjcn.2015.17.2.76.

Recurrent Myelopathy in a Patient with Klippel-Trenaunay Syndrome

Affiliations
  • 1Department of Neurology, Ajou University School of Medicine, Suwon, Korea. isjoo@ajou.ac.kr

Abstract

Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation syndrome involving blood and lymph vessels, which is characterized by triad of cutaneous hemangioma, venous varicosities, and overgrowth of the affected limbs. Because vascular malformation in KTS can be located anywhere except the face and brain, the clinical presentation could be extremely variable. But there are only rare case reports that KTS is associated with spinal cord lesion. We report a case of recurrent myelopathy in a patient with KTS.

Keyword

Myelopathy; Klippel-Trenaunay syndrome; Vascular malformation

MeSH Terms

Brain
Extremities
Hemangioma
Humans
Klippel-Trenaunay-Weber Syndrome*
Spinal Cord
Spinal Cord Diseases*
Vascular Malformations

Figure

  • Fig. 1. Photograph (A) and venography images (B, C) of the right lower extremity. The patient shows cutaneous “port wine” lesion with varicose veins (A). In venography, there are anomalous and dilated superficial veins of the right lower extremity at the level of upper thigh (B) and below the knee (C).

  • Fig. 2. Sagittal T1- (A) and T2-weighted images (B) of thoraco-lumbar spine MRI at first onset of myelopathy. T2-weighted image shows longitudinally extensive hyperintense lesion in T10-L1 (B, arrow heads) and subcutaneous serpentine vasculature at lower back suggesting congenital vascular malformation (C, arrow).

  • Fig. 3. Sagittal images of thoraco-lumbar spine MRI at the time of recurrence of myelopathy (A, B) and at the follow-up after 2 months (C, D). T1-weighted image shows linear internal hyperintense lesion with focal dark signal intensity suggesting hemorrhage in T10-L1 (A, arrow). Ill-defined hyperintense lesion is shown in T10-L1 at T2WI (B, arrow heads). The previous T2 hyperintense lesion has changed into T1 intermediate to low (C, arrow heads) and T2 dark (D, arrow heads).


Reference

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