Abstract:A case of spinal arterio-venous malformation (AVM) initially diagnosed as unilateral cerebral palsy (CP) is reported. The presentation was of a long-standing spastic monoparesis of the left leg, with initial response to Botulinum toxin injections to the calf and tibialis posterior muscles. This was followed by progressive deterioration occurring over a 3-month period before further investigation and definitive diagnosis at 7 years. Imaging demonstrated a large extra-medullary spinal AVM compressing the mid-thoracic cord. This was successfully managed by embolisation with a non-adhesive polymer: ethylene-vinyl alcohol copolymer injected into the dominant feeding vessel. This case highlights the need to consider alternative diagnoses when a child with a diagnosis of CP presents with atypical clinical features such as monoparesis and has worsening or altered clinical signs. Moreover, a normal magnetic resonance imaging brain scan and the absence of ipsilateral upper limb neurological signs or functional impairment should raise suspicion even in the context of static lower limb signs. A literature review was performed on the management of spinal AVM in children and this will be is discussed.Key words: arterio-venous malformation; spine; cerebral palsy; child; intervention; embolisation.
Case ReportA male child was born by vaginal delivery at term after a normal pregnancy. There were no neonatal complications. Normal developmental milestones were achieved, walking at 14 months of age and speaking in phrases by 2 years of age. An initial diagnosis of left hemiplegic cerebral palsy (CP) was made at 2 years of age by a paediatric neurologist on the basis of lateral circumduction and a toe strike gait pattern affecting the left leg. The left lower limb had signs of an upper motor neurone syndrome, characterised by hyper-reflexia across the knee and ankle, an up-going plantar response and some reduction in muscle bulk of the limb when compared with the non-affected side.At 5 years of age, examination of the left arm revealed a slight reduction in arm swing when walking, but otherwise there was normal muscle tone, normal deep tendon reflexes and normal function as the non-dominant upper limb. The right arm and leg were normal. Gait was not restricted; however, it was qualitatively non-fluent. The boy, however, became an accomplished break dancer, suggesting normal upper limb co-ordination and muscle strength. Vision and hearing were formally assessed and were normal. He was toilet trained, with no reported bladder or bowel problems. At 5 years, his diagnosis was revised to spastic monoplegic CP affecting only the left lower limb. A magnetic resonance imaging (MRI) of his brain was normal. Neurovasculocutaneous stigmata were not seen.Over the next 2 years, treatments were required for spasticity and an equinus deformity of the left ankle. This included botulinum toxin A injections to the calf and tibialis posterior muscles and also serial casting with good response. The outcome of these interventions was sufficient...