Keywords
epidermoid - tethered Cord - lipoma - spinal tumor - cutaneous stigmata - congenital
spinal lesion
Introduction
Tethered cord syndrome is defined as progressively increasing neurological deficits
due to limitation of spinal cord movements and traction caused by anatomical or physiological
reasons.[1] It may be associated with different pathologies. Spinal intradural lipoma is rare
and slow growing tumor comprising less than 1% of all intraspinal tumors.[2] Epidermoid tumors are rare benign lesions, which are common in the intracranial
region and account for less than 1% of all intraspinal tumors.[3]
In this paper, we are presenting a case of a 50-year-old female who was suffering
from tethered cord with intradural epidermoid cyst, lipoma and syringomyelia. There
has been only one such case report before, and we look forward to contribute.[4]
Case Report
A 50-year-old female, presented with complaints of insidious onset, gradually progressing,
dull aching and persistent low-backache for 8 years. Around 6 months back, she developed
nonprogressive tingling sensations in her left lower limb. One week back, she suffered
a fall, following which, her complaints aggravated acutely, and she developed tingling
sensation in both her lower limbs. She also developed stiffness in both her lower
limbs, resulting in experiencing difficulty in walking. There was no history of recent
trauma or any aggressive exercise. On neurological examination, there was a dermal
pit and a tuft of hair present in the lumbosacral region. There was no wasting in
lower limbs. She had spastic paraparesis with power of 4+ / 5 in the right lower limb
and 4/5 in the left lower limb. No sensory deficit was present. Deep tendon reflexes
were exaggerated in both lower limbs. The rest of the neurological examination was
normal. MRI of lumbosacral spine was done, which revealed two distinct lesions at
L3 and L4 levels, of which the superiorly placed lesion was slightly hyperintense
on T1 and hypointense on T2 sequences. Inferiorly placed lesion was hyperintense on
T1 and hyperintense with an area of hypointensity on T2 sequences ([Fig. 1]). The cord was seen extending to the lower L2 level. She was then taken up for surgery
where L3, L4 and L5 laminectomy was done, followed by midline posterior durotomy.
There were two lesions, cranial gray–white firm cystic mass and caudal was yellow,
fibrofatty lesion. Excision of the lesions and detethering of cord was done, followed
by primary duroplasty. Histopathology was suggestive of cranial epidermoid cyst and
caudal lipoma. Postoperatively, her paresthesia decreased in intensity and disappeared
on 6 months of follow-up.
Fig. 1 (A) MRI T1-weighted image of midsagittal lumbosacral spine showing epidermoid cyst and
lipoma tethering the cord. (B) MRI T2-weighted image of midsagittal lumbosacral spine showing epidermoid cyst and
lipoma tethering the cord.
Discussion
Spinal region lipomas are usually associated with spinal dysraphisms, wherein the
intraspinal component of the tumor communicates with the subcutaneous part of the
tumor through a defect in the posterior elements of spine. It is thought that lipomas
develop when there is premature disconnection of the cutaneous ectoderm from the forming
neural tube.[5] Dysraphic lipomas are generally associated with cutaneous anomalies like subcutaneous
fatty masses, dermal sinus, dimple or hypertrichosis.[6] Moreover, lipomas occurring predominantly in the lumbosacral region have more likelihood
to be associated with congenital factors. Clinical presentation is usually with symptoms
secondary to mass effect, owing to the size of the lesion. These tumors present with
symptoms of progressive myelopathy, including gait difficulties, motor weakness, sensory
symptoms, and sphincter incontinence.
Epidermoid cysts are rare of the central nervous system (CNS) tumors, benign in nature,
with cranial being more common than spinal lesions; intraspinal ones account for less
than 1% of all intraspinal lesions.[3] Epidermoid cysts are mostly located in the thoracic region, while their presence
in lumbar region is found to be very rare.[7] The symptoms of the cyst are usually not specific and are secondary to mass effect
on the cord, due to the progressively increasing size of the lesion.[8]
[9] Patients are usually symptomatic for a longer duration, an average time of around
6 years, due to their slow growing nature.[8]
[9]
Concomitant existence of an epidermoid cyst adjacent to lipoma can be due to a form
of disjunction, which leads to sequestration of multipotent cells in between the tissue
layers that separate the skin from the deeper neuroectodermal structures.[10]
With the recent advances in imaging techniques, it has become increasingly easier
to correctly diagnose a patient with such presentation. In spite of theoretical possibility
of coexistence of the two lesions, there has been only one case report citing such
incidence, that too in a young patient, stressing on the rarity of such cases.[4]
Conclusion
The coexistence of intradural lipoma and epidermoid cyst, in spite of theoretical
possibility, is not commonly found. Such lesions should be considered in diagnosis
when dealing with a patient with lumbar region pain and paresthesia in lower limbs,
irrespective of the age. With the new advanced imaging techniques, accurate diagnosis
of such lesions is now possible. The management in such cases should be planned accordingly
for complete cure of the patient with least chances of recurrence.
