Acute paraplegia due to spinal arteriovenous fistula in two patients with hereditary hemorrhagic telangiectasia

Autor: Henri Plauchu, Alice Poisson, Francis Brunelle, Sophie Dupuis-Girod, Ashok Vasdev
Rok vydání: 2008
Předmět:
Male
congenital
hereditary
and neonatal diseases and abnormalities
medicine.medical_specialty
Subarachnoid hemorrhage
Spinal Cord Vascular Diseases
medicine.medical_treatment
Arteriovenous fistula
Magnetic resonance angiography
Diagnosis
Differential
otorhinolaryngologic diseases
medicine
Humans
Embolization
Family history
Child
Telangiectasia
Genes
Dominant
Chromosome Aberrations
Neurologic Examination
Paraplegia
medicine.diagnostic_test
Spinal Cord Ischemia
business.industry
Infant
Magnetic resonance imaging
medicine.disease
Embolization
Therapeutic
Magnetic Resonance Imaging
Surgery
Spinal Cord
Acute Disease
Arteriovenous Fistula
Pediatrics
Perinatology and Child Health
Telangiectasia
Hereditary Hemorrhagic
Presentation (obstetrics)
medicine.symptom
business
Magnetic Resonance Angiography
Follow-Up Studies
Zdroj: European Journal of Pediatrics. 168:135-139
ISSN: 1432-1076
0340-6199
DOI: 10.1007/s00431-008-0863-2
Popis: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by recurrent epistaxis, cutaneous telangiectasia, and visceral arteriovenous malformations (AVM). Of these, spinal AVM is a rare manifestation that concerns mainly children. In this report, we describe two cases of spinal AVM revealed by acute paraparesis due to subarachnoid hemorrhage in children with HHT and reviewed the literature on spinal arteriovenous malformations in HHT. In most of the cases reported, the clinical presentation was acute in the pediatric population and insidious during adulthood. The prognosis of spinal AVM mainly depends on the presence or not of medullar signs and symptoms and on the delay before treatment. In conclusion, any child with a family history of HHT should be considered at risk for spinal AVM in order to improve management of such complications and to decrease the risk of neurological sequellae.
Databáze: OpenAIRE
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