|Year : 2007 | Volume
| Issue : 1 | Page : 28-30
Cauda equina syndrome due to implantation epidermoid tumor: After 38 years of surgery for lumbar meningocele in a neonate
Rabindra N Mohapatra1, Ranjan K Patra2, Manoja K Panigrahi3
1 Department of Neurosurgery, IGH, Rourkela, India
2 Department of Orthopaedics, IGH, Rourkela, India
3 Department of General Surgery, IGH, Rourkela, India
Rabindra N Mohapatra
Qr. No. C/91, Sector-19, Rourkela - 769 005, Orissa
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Intraspinal epidermoid tumors are rare. Most of these tumors occur spontaneously, while others may be acquired. Occurrence of epidermoid tumor following surgery for spinal dysraphism has been reported till 15 years from the day of surgery. We report here a case of Cauda equina syndrome due to intraspinal epidermoid tumor following 38 years of surgery for spinal dysraphism. Though epidermoid tumors grow linearly - unlike other tumors, which grow exponentially - we could not find any report in English literature where such a long time of 38 years was taken for the tumor to manifest clinically. The longest period reported for epidermoid tumors to manifest clinically following repair of spinal dysraphism is 15 years. We report this case because it is a case of the longest silent period taken by the implantation epidermoid tumor to manifest clinically.
Keywords: Acquired, Cauda equina syndrome, epidermoid cyst, spinal, spinal dysraphism
|How to cite this article:|
Mohapatra RN, Patra RK, Panigrahi MK. Cauda equina syndrome due to implantation epidermoid tumor: After 38 years of surgery for lumbar meningocele in a neonate. J Pediatr Neurosci 2007;2:28-30
|How to cite this URL:|
Mohapatra RN, Patra RK, Panigrahi MK. Cauda equina syndrome due to implantation epidermoid tumor: After 38 years of surgery for lumbar meningocele in a neonate. J Pediatr Neurosci [serial online] 2007 [cited 2022 Jan 22];2:28-30. Available from: https://www.pediatricneurosciences.com/text.asp?2007/2/1/28/32005
| Introduction|| |
Epidermoid tumors are believed to develop several years after spinal surgery, and lesions detected as long as 15 years after surgery for spinal dysraphism are documented in the literature.  The present case manifested clinically after 38 years of initial surgery for lumbar meningocele.
| Case Report|| |
A 40-year-old man was referred to our department for neurosurgical evaluation of his gait and urinary difficulties. He developed progressive weakness in right lower limb followed by left lower limb since 2 years and required support to walk. Since 8 months, he developed incontinence of urine, loss of bladder sensation, erectile dysfunction and diminished sensation on the parts of his lower limbs and perineum. He had undergone surgery for lumbar meningocele immediately after his birth with no residual neurological deficit. His examination revealed lower motor neuron type of weakness in both the lower limbs, right more than the left; sensory loss to touch and pin prick from L4 downwards on both the lower limbs; absent knee and ankle reflexes; mute plantar reflexes both the sides and impaired perianal reflex. X-rays of lumbosacral (L-S) spine showed spina bifida and evidence of widening of spinal canal. Magnetic resonance imaging (MRI) [Figure - 1],[Figure - 2] demonstrated a homogenous, well-demarcated tumor mass, hypointense on T1 and hyperintense on T2, nonenhancing, located at and completely filling up the dilated spinal canal from L2 to L4. The conus was at the normal position. He underwent surgery, wherein a laminectomy was performed and the intradural tumor mass was removed completely. The tumor was smooth and grayish white in color and had a shinning thin capsule containing cheesy material. It was situated posterior to nerve roots and was extending from the tip of conus to L5. Histopathological examination of the tissue revealed lining of stratified squamous epithelium without any evidence of dermal elements. Postoperative recovery was uneventful, and the patient was discharged from hospital on the eighth day. At follow-up after 2 years, weakness and numbness in lower extremities and his bladder functions recovered completely and he was able to walk without support. However, he still continues to have erectile dysfunction.
| Discussion|| |
Epidermoid cysts are rare intraspinal tumors with an incidence of 0.5-1% of spinal tumors.  These tumors can be congenital or acquired. Acquired epidermoid cysts are thought to be secondary to implantation of cutaneous elements in spinal canal during lumbar puncture or other surgical procedures. Epidermoid cysts following repair of spinal dysraphism can occur due to inadequate excision or implantation of cutaneous elements in spinal canal during surgery. However, Storrs et al .  studied excised placode and specimens from tethered cord and found hamartomatous lesions, which are a common feature of myelodysplastic sequence and are independent of surgery. Implantation epidermoid is reported following 23 years of lumbar puncture, and the same has been reported until 15 years of repair of meningomyelocele. ,, Scott et al .  proposed a classification of etiology according to the location of epidermoid in relation to placode. Accordingly, they presumed congenital epidermoids to be located anterior to placode and implanted ones to be located posteriorly. After primary repair of myelomeningocele or lipomyelomeningocele, late progressive neurological deterioration commonly occurs due to treatable causes. While tethered cord is the commonest cause of deterioration, additional pathology like dermoid tumor, hydromyelia, tight filum, etc., accounts for some.  In others it may be due to remnants or implantation of cutaneous elements. Lunardi et al .  analyzed long-term results of surgical treatment of spinal dermoid and epidermoid tumors. Out of 16 cases, part of the epidermoid and dermoid elements were left in situ in 9 cases, as it was not possible to separate the parts of tumor from neural elements. After 5 to 30 years' follow-up, only one patient demonstrated tumor recurrence.
Elsworth et al .  studied the growth rate of epidermoid tumors and opined that they grow linearly - unlike other tumors, which grow exponentially. They also found that epidermoid tumors grow at the rate equal to that of skin. This observation is true for both congenital as well as acquired epidermoid tumors. Since there was no adhesion of the tumor to neural elements and due to its posterior location in relation to neural elements and slow growth, we presume it to be the implantation of cutaneous elements during primary repair, probably of a simple meningocele. After primary repair of spinal dysraphism, cases of epidermoid cyst are reported until after 15 years of surgery. Since epidermoid tumors grow slowly, patients develop symptoms over a long time. Imaging, particularly, magnetic resonance imaging (MRI), is helpful in diagnosis of the lesion. Ependymoma, degenerative diseases of spine, tumors arising from Cauda equina are other differential diagnoses. Early diagnosis and surgical excision are accepted procedures till date. Elements adherent to neural elements can be left as such as recurrence is low. Histopathology usually reveals stratified squamous epithelium and keratin deposits in the lesion.
In our patient, neurological symptoms developed 38 years after the initial surgery. Probably this is the longest silent period, reported in English literature, between primary repair of spinal dysraphism and appearance of epidermoid tumor at the operation site. In between, he had a complete symptom-free period of more than three decades. Symptoms appeared and slowly progressed over a period of 2 years, leading to typical features of Cauda equina syndrome. Though documentation of previous findings and operative procedures was not available, the information obtained from the patient and the recent MRI were sufficient to show clear evidence of a developmental disease involving spine at the level of L4/L5. Equally evident was the normal ascent of conus to L2 level, excluding tethering of the cord. During surgery, the tumor was located posterior to neural elements and was not adherent to the conus. Taking all these findings and the long duration of tumor appearance into consideration, we think it was implanted during surgery.
Epidermoid tumor can occur as long as four decades from the date of primary repair of spinal dysraphism. Though etiology of epidermoid tumor is not entirely clear, during primary repair adequate care to prevent implantation of cutaneous elements in the spinal canal and early surgical excision if it occurs seems to be the only treatment available till date.
| Acknowledgment|| |
Dr. D. N. Mohapatra, Director, Medical and Health Services, IGH, Rourkela for granting permission to publish the case report.
| References|| |
|1.||Kachhara R, Unnikrishnan M. Acquired Cauda equina epidermoid cyst. Neurol India 1999;47:250. [PUBMED] [FULLTEXT]|
|2.||Storrs BB. Are dermoid and epidermoid tumors preventable complications of myelomeningocele repair? Pediatr Neurosurg 1994;20:160-2. [PUBMED] |
|3.||Manno NJ, Uihlein A, Kernohan JW. Intraspinal epidermoid tumors (cholesteatoma) in patients treated for tuberculous meningitis. Lancet 1956;2:437-9. |
|4.||Song JH, Kim MH, Shin KM. Intraspinal epidermoid cyst occurring 15 years after lipomyelomeningocele repair. Case report. J Neurosurg 1999;90:252-4. |
|5.||Argenta L, Glazier S, Marks M, Proffer P. Intrspinal epidermal cyst following intrauterine surgery as cause of difficult to heal myelomeningocele repair. Plast Surg 2003. |
|6.||Scott RM, Wolpert SM, Bartoshesky LE, Zimbler S, Klauber GT. Dermoid tumors occurring at the site of previous myelomeningocele repair. J Neurosurg 1986;65:779-83 [PUBMED] |
|7.||Herman JM, McLone DG, Storr BB, Dauser RC. Analysis of 153 patients with myelomeningocele or spinal lipoma reoperated upon for a tethered cord Presentation, management and outcome. Pediatr Neurosurg 1993;19:243-9. |
|8.||Lunardi P, Missori P, Gagliardi FM, Fortuna A. Long - term result of the surgical treatment of spinal dermoid and epidermoid tumors. Neurosurgery 1989;25:860-4 [PUBMED] |
|9.||Alvord EC Jr. Growth rates of epidermoid tumors. Ann Neurol 1977;2:367-70. [PUBMED] |
[Figure - 1], [Figure - 2]
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