Congenital Intramedullary Lipoma of the Dorsocervical Spinal Cord with Intracranial Extension

Abstract

The unusual computed tomographic findings of an extensive intramedullary lipoma of the dorsocervical spinal cord, which extended into the brain stem, in a 9-month-old infant are described and correlated with the surgical findings. The operative therapeutic options are discussed.

Intramedullary Lipoma of Dorsocervicothoracic Spinal Cord with Intracranial Extension and Hydrocephalus

“…Most of the reported intradural lipomas of the cord were subpial [9,10] and not truly intramedullary. A few reports of cervical cord lipomas with an intracranial extension appeared [7,11,12], but none was as extensive in the neonatal period as the present case.…”

“…Several hypotheses regarding the genesis of spinal cord lipomas have been proposed [7,8]. Many character- istics of the present case and its presence at birth suggested a hamartomatous origin.…”

“…Many character- istics of the present case and its presence at birth suggested a hamartomatous origin. Maldevelopment with the resultant inclusions of embryonic remains of fat cells during the formation of the neural tube is considered the most probable explanation of the origin of these lipomas [7,8]. Some investigators feel that lipomas arise due to the loss of control over the normally present multipotential pericapillary mesenchymal cells and argue that the source is not neural in origin [1].…”

“…Extensive intramedullary lipomas of the cervical spinal cord extending into the posterior fossa are extremely rare, with only 8 cases reported in the literature [1,[3][4][5][6][7]. Most of the cases reported in the literature [7,8] were confined to four or five segments of the cord, presented in adult life, and extended rarely into the posterior fossa. Most of the reported intradural lipomas of the cord were subpial [9,10] and not truly intramedullary.…”

Nondysraphic intramedullary spinal cord lipomas: a review

“…One group presents during infancy and childhood, often as tetraplegia or floppy baby syndrome; the neurological manifestations are thought to be due to birth trauma to the spinal cord. 10,12 In the child with quadriplegia described by Mori, et al, 10 these authors diagnosed floppy infant syndrome; when the child experienced seizures at the age of 7 years, MR imaging revealed a cervicomedullary lipoma with intracranial extension.…”