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[Spinal arteriovenous malformations in Klippel-Trénaunay-Weber syndrome: case report].
No Shinkei Geka. 1990 Sep; 18(9):877-81.NS

Abstract

A 19-year-old man was admitted with the chief complaint of a sudden onset of thoracic pain and paraplegia. His right lower limb had progressively enlarged since he was 3 months old. Transient paraparesis had occurred recurrently since the age of 3. Physical examination on admission revealed that there were multiple warts on his face, trunk and right extremities. The right lower limb was thicker and longer than the left one, and the temperature was 1 degree C higher in the right side. Neurological examination revealed paraplegia and severe impairment of all sensory modalities below Th4 with bilateral clonus. Radiological examination showed lordosis of the thoracic spines with thinning of the pedicles of Th4 to Th6. Selective spinal angiography showed a juvenile type of arteriovenous malformation involving the cord of Th2 to Th6 with multiple feeding arteries, and a glomus type of the lower thoracic cord with the feeding arteries from Adamkiewicz's artery and the right Th10 intercostal artery. MRI showed the cord enlargement of Th2 to Th6 and within it the nidus was identified. Spinal arteriovenous malformations associated with Klippel-Trénaunay-Weber syndrome were noticed. The patient underwent interruption of the main feeding artery and removal of the dilated vessels located in the dorsum of the thoracic cord. Postoperative course was uneventful with slight improvement of clinical symptoms. Klippel-Trénaunay-Weber syndrome is manifested by a triad of symptoms: 1) hemihypertrophy of the extremities, 2) cutaneous hemangiomas and 3) arteriovenous fistula or varicose veins of the involved limbs. Nineteen cases of Klippel-Trénaunay-Weber syndrome associated with spinal arteriovenous malformation have been reported. The pathogenesis has been suggested to be due to developmental abnormalities.

Authors+Show Affiliations

Tan EC
Department of Neurosurgery, Nagoya City University Medical School.
Takagi T
No affiliation info available
Nagai H
No affiliation info available

MeSH

AdultArteriovenous MalformationsHumansKlippel-Trenaunay-Weber SyndromeMagnetic Resonance ImagingMaleSpinal Cord

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

jpn

PubMed ID

2172852

Citation

Tan, E C., et al. "[Spinal Arteriovenous Malformations in Klippel-Trénaunay-Weber Syndrome: Case Report]." No Shinkei Geka. Neurological Surgery, vol. 18, no. 9, 1990, pp. 877-81.
Tan EC, Takagi T, Nagai H. [Spinal arteriovenous malformations in Klippel-Trénaunay-Weber syndrome: case report]. No Shinkei Geka. 1990;18(9):877-81.
Tan, E. C., Takagi, T., & Nagai, H. (1990). [Spinal arteriovenous malformations in Klippel-Trénaunay-Weber syndrome: case report]. No Shinkei Geka. Neurological Surgery, 18(9), 877-81.
Tan EC, Takagi T, Nagai H. [Spinal Arteriovenous Malformations in Klippel-Trénaunay-Weber Syndrome: Case Report]. No Shinkei Geka. 1990;18(9):877-81. PubMed PMID: 2172852.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Spinal arteriovenous malformations in Klippel-Trénaunay-Weber syndrome: case report]. AU - Tan,E C, AU - Takagi,T, AU - Nagai,H, PY - 1990/9/1/pubmed PY - 1990/9/1/medline PY - 1990/9/1/entrez SP - 877 EP - 81 JF - No shinkei geka. Neurological surgery JO - No Shinkei Geka VL - 18 IS - 9 N2 - A 19-year-old man was admitted with the chief complaint of a sudden onset of thoracic pain and paraplegia. His right lower limb had progressively enlarged since he was 3 months old. Transient paraparesis had occurred recurrently since the age of 3. Physical examination on admission revealed that there were multiple warts on his face, trunk and right extremities. The right lower limb was thicker and longer than the left one, and the temperature was 1 degree C higher in the right side. Neurological examination revealed paraplegia and severe impairment of all sensory modalities below Th4 with bilateral clonus. Radiological examination showed lordosis of the thoracic spines with thinning of the pedicles of Th4 to Th6. Selective spinal angiography showed a juvenile type of arteriovenous malformation involving the cord of Th2 to Th6 with multiple feeding arteries, and a glomus type of the lower thoracic cord with the feeding arteries from Adamkiewicz's artery and the right Th10 intercostal artery. MRI showed the cord enlargement of Th2 to Th6 and within it the nidus was identified. Spinal arteriovenous malformations associated with Klippel-Trénaunay-Weber syndrome were noticed. The patient underwent interruption of the main feeding artery and removal of the dilated vessels located in the dorsum of the thoracic cord. Postoperative course was uneventful with slight improvement of clinical symptoms. Klippel-Trénaunay-Weber syndrome is manifested by a triad of symptoms: 1) hemihypertrophy of the extremities, 2) cutaneous hemangiomas and 3) arteriovenous fistula or varicose veins of the involved limbs. Nineteen cases of Klippel-Trénaunay-Weber syndrome associated with spinal arteriovenous malformation have been reported. The pathogenesis has been suggested to be due to developmental abnormalities. SN - 0301-2603 UR - https://www.unboundmedicine.com/medline/citation/2172852/[Spinal_arteriovenous_malformations_in_Klippel_Trénaunay_Weber_syndrome:_case_report]_ DB - PRIME DP - Unbound Medicine ER -