Recurrent Myelopathy in a Patient with Klippel-Trenaunay Syndrome

Yue Kyung Kim; Young In Eom; In Soo Joo

doi:10.14253/KJCN.2015.17.2.76

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Kim, Eom, and Joo: Recurrent Myelopathy in a Patient with Klippel-Trenaunay Syndrome

Original Article

Korean J Community Nutr 2015;17(2):76-79.

Published online: 18 December 2015

DOI: https://doi.org/10.14253/KJCN.2015.17.2.76

Recurrent Myelopathy in a Patient with Klippel-Trenaunay Syndrome

Yue Kyung Kim, Young In Eom, In Soo Joo

Department of Neurology, Ajou University School of Medicine, Suwon, Korea

Address for correspondence; In Soo Joo Department of Neurology, Ajou University School of Medicine, 206 World cup-ro, Yeongtong-gu, Suwon 16499, Korea Tel: +82-31-219-5172 Fax: +82-31-219-5178 E-mail: isjoo@ajou.ac.kr

Received 4 November 2014 Revised 16 June 2015 Accepted 8 July 2015

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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. (Korean J Clin Neurophysiol 2015;17:76-79)

Keywords: Myelopathy, Klippel-Trenaunay syndrome, Vascular malformation

REFERENCES

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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).

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