Arteriovenous fistula of the vertebral artery in a female infant with hypotonia and cephalocorporal disproportion

Background: Congenital arteriovenous fistulas are exceptional in childhood and imply a therapeutic challenge. Case report: A 9‐month‐old female infant was studied for cephalocorporal disproportion, hypotonia, progressive muscular atrophy and hyperreflexia. Computed tomography of the brain and electroencephalography were normal. Electromyographic patterns suggested proximal myopathic involvement. A continuous murmur with systolic reinforcement was audible in the neck. Angioresonance detected intracranial aneurysmal dilatations behind the bulbo‐medullary junction and cerebral panangiography evidenced a direct vertebrovertebral fistula with extra‐ and intra‐cranial varices and extreme medullary compression. Occlusion of the afferent vessel to the aneurismal sack was successfully achieved with a mixture of Histoacryl and 75% lipiodol via a microcatheter. Conclusion: Clinical signs of an arteriovenous fistula may be atypical. Considerable cephalocorporal disproportion and a bruit in the cervical or retromastoidal regions must suggest its existence. Complete obliteration with endovascular embolization permits somatic and neurological recovery.