Klippel Trenaunay syndrome (KTS) is a congenital vascular malformation associated with abnormal growth in the extremities. A case with KTS accompanying skeletal anomaly and nerve root compression symptoms is presented along with a review of the literature. A 31-year-old male patient, who was being followed for KTS, presented to the neurology outpatient clinic com- plaining of numbness at the left arm, low back pain, and numbness and pain spreading down both legs. Physical examination revealed port wine stain lesions, varicose veins, and an increased circum- ference and length at the left lower extremity. The patient had thoracic scoliosis with apex to the right. Neurological examination showed hypoactive biceps and triceps tendon reflexes at the left side, hypoesthesia at the left C7 dermatome, and positive Lasegue"s sign bilaterally. Cervical and lum- bar MRI showed multiple disc protrusions. It should be kept in mind that accompanying pathological conditions in KTS may include scoliosis and nerve root compression as well as visceral involvement and musculoskeletal deformities.


Karabacak K., Genç G., Özben S., Sungur M., Kadan M., Cingöz F.

Turkish journal of vascular surgery, cilt.21, sa.3, ss.1-5, 2012 (Hakemli Dergi)

  • Yayın Türü: Makale / Vaka Takdimi
  • Cilt numarası: 21 Sayı: 3
  • Basım Tarihi: 2012
  • Dergi Adı: Turkish journal of vascular surgery
  • Sayfa Sayıları: ss.1-5
  • Acıbadem Mehmet Ali Aydınlar Üniversitesi Adresli: Hayır

Özet

Klippel Trenaunay syndrome (KTS) is a congenital vascular malformation associated with abnormal growth in the extremities. A case with KTS accompanying skeletal anomaly and nerve root compression symptoms is presented along with a review of the literature. A 31-year-old male patient, who was being followed for KTS, presented to the neurology outpatient clinic com- plaining of numbness at the left arm, low back pain, and numbness and pain spreading down both legs. Physical examination revealed port wine stain lesions, varicose veins, and an increased circumference and length at the left lower extremity. The patient had thoracic scoliosis with apex to the right. Neurological examination showed hypoactive biceps and triceps tendon reflexes at the left side, hypoesthesia at the left C7 dermatome, and positive Lasegue"s sign bilaterally. Cervical and lum- bar MRI showed multiple disc protrusions. It should be kept in mind that accompanying pathological conditions in KTS may include scoliosis and nerve root compression as well as visceral involvement and musculoskeletal deformities.