STUDY DESIGN: A retrospective analysis of a large single institution pediatric intramedullary tumor database. The database was searched for all pediatric intramedullary cavernous malformations. OBJECTIVES: To review the authors' experience with three pediatric intramedullary cavernous malformations, review the previous literature, and discuss the surgical management for these rare lesions. SUMMARY OF BACKGROUND DATA: Cavernous malformations can occur throughout the central nervous system; however, the intramedullary spinal cord is a rare location. More recent estimates indicate that cavernous malformations constitute 5% of all vascular malformations. There is no series that reports the management of pediatric intramedullary cavernous malformations. METHODS: There were 181 pediatric intramedullary lesions in the tumor database. A review revealed three children with a mean age of 13.3 years (range, 8-19 years) with intramedullary cavernous malformations. All these children presented with an acute motor deficit that prompted radiographic imaging and diagnosis. All the children underwent laminectomy and gross total removal of the lesion. All the malformations abutted the dorsal pial surface and created a blue discoloration, which was evident after opening the dura. RESULTS: Approximately 10% of all intramedullary cavernous malformations present in the pediatric population. Pediatric lesions, unlike those in adults, have an acute presentation, and the lesions are typically found in the cervical region. The surgical removal of these malformations results in a good functional outcome. The entire neuraxis should be screened because there is a high incidence of multiple lesions. CONCLUSIONS: The surgical removal of these malformations results in a good functional outcome. The entire neuraxis should be screened because there is a high incidence of multiple lesions.
STUDY DESIGN: A retrospective analysis of a large single institution pediatric intramedullary tumor database. The database was searched for all pediatric intramedullary cavernous malformations. OBJECTIVES: To review the authors' experience with three pediatric intramedullary cavernous malformations, review the previous literature, and discuss the surgical management for these rare lesions. SUMMARY OF BACKGROUND DATA: Cavernous malformations can occur throughout the central nervous system; however, the intramedullary spinal cord is a rare location. More recent estimates indicate that cavernous malformations constitute 5% of all vascular malformations. There is no series that reports the management of pediatric intramedullary cavernous malformations. METHODS: There were 181 pediatric intramedullary lesions in the tumor database. A review revealed three children with a mean age of 13.3 years (range, 8-19 years) with intramedullary cavernous malformations. All these children presented with an acute motor deficit that prompted radiographic imaging and diagnosis. All the children underwent laminectomy and gross total removal of the lesion. All the malformations abutted the dorsal pial surface and created a blue discoloration, which was evident after opening the dura. RESULTS: Approximately 10% of all intramedullary cavernous malformations present in the pediatric population. Pediatric lesions, unlike those in adults, have an acute presentation, and the lesions are typically found in the cervical region. The surgical removal of these malformations results in a good functional outcome. The entire neuraxis should be screened because there is a high incidence of multiple lesions. CONCLUSIONS: The surgical removal of these malformations results in a good functional outcome. The entire neuraxis should be screened because there is a high incidence of multiple lesions.
Authors: Erwin M J Cornips; Pauline A C P Vinken; Mariel Ter Laak-Poort; Emile A M Beuls; Jacobine Weber; Johannes S H Vles Journal: Childs Nerv Syst Date: 2009-10-29 Impact factor: 1.475