Key words: Computed tomography, Congenital intramedullary lipomas, Floppy infant syndrome, Hamartoma, Intracranial extension, Lipoma, Spinal cord tumors THE UNUSUAL COMPUTED tomographic findings of an extensive intramed- ullary 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. (Neurosurgery 34: 1081-1084,1994) Congenital Intramedullary Lipoma of the Dorsocervical Spinal Cord with Intracranial Extension: Case Report FIGURE 1. X-rays of the cervical/upper dorsal spine showing a marked in- crease of the interpedicular distance (A) and the anteroposterior diameter (B). was impaired. Her sensation was im- paired up to the mandible/upper cervi- cal region. Her respiration was shallow, quick, and respiratory movement was confined mostly to lower chest. Plain x- rays of the spine revealed a marked in- crease of the interpedicular distance and of the anteroposterior diameter of the cervicothoracic spinal canal (Fig. 1). Cra- nial and spinal computed tomographic (CT) scans (Fig. 2) disclosed an enor- This 9-month-old infant girl, who had a normal full-term delivery and an un- remarkable prenatal history, was admit- ted to the neurosurgical ward with dense tetraparesis and respiratory difficulty. Before admission, the infant was treated elsewhere for hypotonia and lack of limb movement observed soon after birth. A diagnosis of floppy infant syndrome was made, but the cause remained obscure for many months. A physical examina- tion revealed a tetraplegic child with flailing areflexic upper limbs and spastic lower limbs, marked ankle clonus, and bilateral upgoing plantar response was noticed. Her bladder and bowel control CASE REPORT sented in the neonatal period or was as extensive as the present case. Naim-Ur-Rahman, F.R.CS., F.R.CS.Ed., F.A.CS., Mustafa A.M. Salih, M.P.CH., MD., Abdul Hakim Jamjoom, F.R.CS. Ed., Zain Alabedin Jamjoom, MD. Departments of Neurosurgery (N-U-R, AHj, ZAJ) and Pediatrics (MAMS), College of Medicine, King Saud University, Riyadh, Saudi Arabia Extensive intramedullary lipomas of the cervicothoracic spinal cord, which extended into the brain stem, were not associated with spinal dysra- phism, and presented in the infancy / neonatal period, are extremely rare and poorly documented. Most of the cases reported in the literature (1, 2, 5, 6, B), were confined to four or five segments of the cord (3), presented in adult life (1,3, 5), and extended rarely into the posterior fossa and brain stem (16). Most of the reported intradural lipomas of the cord were subpial (3, 7, 9) and not truly in- tramedullary. Thus, in McLone's (9) se- ries of 130 spinal lipomas, 5 were totally intradural. Of these five, only one was completely surrounded by spinal cord tissue. A few reports of cervical cord li- pomas with an intracranial extension have appeared (11, 12, 16), but none pre- Neurosurgery, Vol. 34, No. 6, June 1994