Biomaterial Database

[Spinal arteriovenous malformations in Klippel-Trénaunay-Weber syndrome: case report]. 1990

E C Tan, and T Takagi, and H Nagai

Department of Neurosurgery, Nagoya City University Medical School.

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.

UI	MeSH Term	Description	Entries
D007715	Klippel-Trenaunay-Weber Syndrome	A congenital disorder that is characterized by a triad of capillary malformations (HEMANGIOMA), venous malformations (ARTERIOVENOUS FISTULA), and soft tissue or bony hypertrophy of the limb. This syndrome is caused by mutations in the VG5Q gene which encodes a strong angiogenesis stimulator.	Klippel-Trenaunay Disease,Angio-Osteohypertrophy Syndrome,Angioosteohypertrophy Syndrome,Congenital Dysplastic Angiopathy,KTW Syndrome,Klippel Trenaunay Syndrome,Klippel-Trenaunay Syndrome,Klippel-Trénaunay-Weber Syndrome,Angio Osteohypertrophy Syndrome,Angio-Osteohypertrophy Syndromes,Angioosteohypertrophy Syndromes,Angiopathies, Congenital Dysplastic,Angiopathy, Congenital Dysplastic,Congenital Dysplastic Angiopathies,Disease, Klippel-Trenaunay,Dysplastic Angiopathies, Congenital,Dysplastic Angiopathy, Congenital,KTW Syndromes,Klippel Trenaunay Disease,Klippel Trenaunay Weber Syndrome,Klippel Trénaunay Weber Syndrome,Klippel-Trenaunay Syndromes,Syndrome, Angio-Osteohypertrophy,Syndrome, Angioosteohypertrophy,Syndrome, KTW,Syndrome, Klippel Trenaunay,Syndrome, Klippel-Trenaunay,Syndrome, Klippel-Trenaunay-Weber,Syndrome, Klippel-Trénaunay-Weber,Syndromes, Angio-Osteohypertrophy,Syndromes, Angioosteohypertrophy,Syndromes, KTW,Syndromes, Klippel-Trenaunay
D008279	Magnetic Resonance Imaging	Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques.	Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D008297	Male		Males
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D001165	Arteriovenous Malformations	Abnormal formation of blood vessels that shunt arterial blood directly into veins without passing through the CAPILLARIES. They usually are crooked, dilated, and with thick vessel walls. A common type is the congenital arteriovenous fistula. The lack of blood flow and oxygen in the capillaries can lead to tissue damage in the affected areas.	Arteriovenous Malformation,Malformation, Arteriovenous,Malformations, Arteriovenous
D013116	Spinal Cord	A cylindrical column of tissue that lies within the vertebral canal. It is composed of WHITE MATTER and GRAY MATTER.	Coccygeal Cord,Conus Medullaris,Conus Terminalis,Lumbar Cord,Medulla Spinalis,Myelon,Sacral Cord,Thoracic Cord,Coccygeal Cords,Conus Medullari,Conus Terminali,Cord, Coccygeal,Cord, Lumbar,Cord, Sacral,Cord, Spinal,Cord, Thoracic,Cords, Coccygeal,Cords, Lumbar,Cords, Sacral,Cords, Spinal,Cords, Thoracic,Lumbar Cords,Medulla Spinali,Medullari, Conus,Medullaris, Conus,Myelons,Sacral Cords,Spinal Cords,Spinali, Medulla,Spinalis, Medulla,Terminali, Conus,Terminalis, Conus,Thoracic Cords