|Year : 2006 | Volume
| Issue : 3 | Page : 24-26
Giant cervical intramedullary lipoma with intracranial extension
TK B. Ganapathy, C Balasubramaniam
Departments of Pediatric Intensive Care and Pediatric Neurosurgery, Kanchi Kamakoti CHILDS Trust Hospital 12-A , Nageswara Road, Nungambakkam, Chennai -600 034, India
New No. 14 Second Main Road, C I T Colony, Mylapore, Chennai 600 004,
Source of Support: None, Conflict of Interest: None
Intramedullary lipomas are rare tumours of the spinal cord. The account for about 1% of spinal neoplasms (Neurosurgery, 12, 1983, 460). Their association with a dysraphic state is well known and is common but their occurrence in the absence of a neural tube defect is very rare. They can occur anywhere along the length of the spinal cord but their extension into posterior cranial fossa is very rare. We are herewith reporting a case of intradural cervical spinal lipoma with extension into the posterior cranial fossa. The diagnosis was established by magnetic resonance imaging. The intraspinal portion was resected and the child is awaiting surgery for the intracranial part.
Keywords: cervical lipoma; intracranial extension; intramedullary lipoma.
|How to cite this article:|
B. Ganapathy T K, Balasubramaniam C. Giant cervical intramedullary lipoma with intracranial extension. J Pediatr Neurosci 2006;1, Suppl S1:24-6
Lipomas occurring as a part of the dysraphic state are well known. But their occurrence as isolated tumours of the spinal cord is very rare. They account for about 1% of all spinal neoplasms and can occur anywhere along the length of the spinal cord. Rarer still is the extension into the intracranial compartment.
| » Case report|| |
A 11-year-old boy was referred for difficulty in using the left upper limb of 3-year duration. The weakness was predominantly in using the fingers in addition; he found lifting the arm above the shoulder increasingly difficult. He had also noticed stiffness of both lower extremities. He had not experienced any sensory symptoms or disturbance of bowel or bladder functions.
In the past a lymphangioma had been excised from the back of the neck.
Examination revealed intact cranial nerve functions. Examination of the motor system revealed hypotonia of the left upper limb and wasting of the small muscles of the left hand. The power was grade 3/5 in the muscles of the left upper limb. The power and tone were normal in the right upper limb. The tone was increased in both lower limbs with normal power. All the tendon reflexes in the upper limbs were exaggerated while patellar and ankle clonus were present on both sides. The plantar response was extensor on both sides. The sensations were intact.
Plain X-rays of the cervical spine revealed widening of the cervical canal from C1 to C7.
Magnetic resonance imaging showed a hyperintense lesion on T1 weighted study, extending from C7-T1 junction to the Pons. There was no hydrocephalus [Figure - 1]A, B.
It was decided to operate on the lesion in two stages. Since the cervical cord component was symptomatic it was operated on first. From C1 to C7 laminotomy was done to expose the tumour. The tumour bulged out on opening the dura [Figure - 2]. The tumour was subtotally resected from the cervical cord. Dissection had to be terminated at this point as the plane between the neoplasm and the cord became indistinct. Pial sutures were placed to reform the neural tube. After dural closure the laminae were replaced and fixed.
Postoperatively there was transient deterioration of the neurologic state but it improved gradually. The child is now undergoing rehabilitation. The intracranial part will be removed in a second stage.
| » Discussion|| |
Intraspinal lipomas occurring as a part of the dysraphic state are well known. Here there is a subcutaneous component, which extends intradurally to get attached to the spinal cord and this is often seen in the lumbar and lumbosacral regions. However their occurrence as isolated neoplasms are rare. Lipomas constitute about 1% of all spinal neoplasms when they occur as isolated tumours. ,, They may occur anywhere along the spinal axis. They may be located intradurally or extradurally, the former constituting three fifths. Out of 100 cases of intradural lipomas 55% occurred in second and third decades, 24% in the first year and 16% in the fifth decade.
True intramedullary lipomas are rare and most have a subpial surface. Rarer still is the occurrence of multiple subpial lipomas. A subcutaneous component may not be present. Cervical intramedullary lipomas are very rare and intracranial extension is rarer still., But when they occur cervical lipomas may have a propensity to extend into the posterior cranial fossa., Intradural cervical lipomas represent 12% of all lesions. Those occurring above the thoracolumbar region are extramedullary and located on the posterior aspect of the cord. Those at dorsal and cervical levels are often unassociated with spinal dysraphism.,, Peak incidence is in the first 5 years and another peak is seen in the fifth decade. ,, The embryologic basis may be a less severe form of the those seen in association with a dysraphic state.
The clinical manifestation is like any other intramedullary spinal neoplasm and the symptoms and signs depend on the location of the tumour. The duration of symptoms may be long standing and progression may be slow., A long symptomatic period with rapid worsening prior to presentation has also been recorded. In cervical lipomas symptoms are of 10 years in 80%, 58% present as a slowly progressive compressive myelopathy, 9.5% as a syringomyelic syndrome a Brown Sequard syndrome has been seen in 6.5% of cases and atypical clinical features are encountered in 26% of patients.
These tumours are often indolent for a long time. In addition to the mass effect the tumour may cause tethering of the cord, which contributes to the symptoms.
Screening of the posterior fossa should be done in those with a cervical lipoma to rule out intracranial extension. Hydrocephalus may not be seen. Since the progress of symptoms is slow and the tumours can be indolent for a long time aggressive removal may not be essential. A generous decompression may suffice.
The aim of surgery must be only to prevent further progress of the deficit since normalization of function is impossible. Early surgery may avoid the development of a permanent neurologic deficit.,
These tumours are intimately blend with the neural elements hence attempts at total resection will result in an unacceptable deficit. Tumours may be infiltrative in nature and the lack of a plane to facilitate dissection precludes total removal. The surgeon has to use his or her judgment in stopping dissection.
Total resection is impossible and attempts to do so may result in a poor outcome. Total resection is not advisable., Total resection may result in an unacceptable deficit since no cleavage plane is obvious decompression will suffice.
Subtotal removal may result in stabilization of the neurologic state and good long-term survival.
Lee have speculated that these tumours being congenital in nature may replace the normal pathways thus resulting in a poorer physiologic reserve.
Usually, there is no improvement after surgery., Fixed deficits may not go away after surgery.,
Restoration of normal neurologic status may not occur and the patients are often left with a residual or fresh deficit., The prognosis of this group of lipomas seems to be worse than those associated with a dysraphic state. Probably because these represent a distinct entity.
| » Conclusion|| |
Intradural lipomas unassociated with a dysraphic state are rare. The symptoms are slowly progressive. Those occurring in the cervical cord may have propensity to extend into the posterior cranial fossa. Generous decompression will suffice since total removal is often impossible.
| » References|| |
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[Figure - 1], [Figure - 2]
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