Send to

Choose Destination
See comment in PubMed Commons below
J Neurosurg. 2005 Sep;103(3 Suppl):282-4.

Paraparesis caused by an angiolipomatous hamartoma in an adolescent with Proteus syndrome and scoliosis.

Author information

Department of Surgery, University of British Columbia, Canada.


This 16-year-old boy with Proteus syndrome suffered acute spinal cord compression secondary to hemorrhage into an extradural paraspinal angiolipomatous hamartoma. A magnetic resonance (MR) imaging study performed 12 years earlier had revealed the presence of this tumor at the apex of a mild thoracic scoliosis, which had been stable during the intervening decade. Spinal cord neurological deficit in Proteus syndrome is rare. It can arise either from vertebral abnormalities and kyphoscoliotic deformities or from tumor encroachment. The authors describe the temporal evolution of scoliosis and acute spinal cord compression caused by a paraspinal angiolipomatous hamartoma over a period of 12 years. A review of the literature suggests that regular follow up, including serial MR imaging, is indicated in children with Proteus syndrome and scoliosis. Earlier resection of extradural tumor mass encroaching on the cord is warranted in the treatment of these children.

[Indexed for MEDLINE]
PubMed Commons home

PubMed Commons

How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Atypon
    Loading ...
    Support Center