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ORIGINAL ARTICLE
Year : 2014  |  Volume : 3  |  Issue : 1  |  Page : 8-11

Tethered cord syndrome diagnosis and management


Department of Neurosurgery, Kurnool Medical College, Kurnool, Andhra Pradesh, India

Date of Web Publication10-Mar-2014

Correspondence Address:
Ramanjulu Mala
Associate Professor of Neurosurgery, Government General Hospital, Kurnool - 518 002, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.128414

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  Abstract 

Introduction: Tethered cord symptom complex, originally described by Brickner, presents with protean clinical manifestations. It is rare, and we have reviewed our experience in diagnosis, management, and outcome of these patients.
Materials and Methods: Patients presented with tethered cord syndrome at our neurosurgery department between 2007 and 2011 were analyzed and reviewed.
Results: Thirty cases of tethered cord syndrome were treated surgically over a period of 5 years. The clinical onset was insidious. The main clinical findings were neurological, skeletal, urological, and cutaneous manifestations. M.R.I. was the best diagnostic tool. T1-weighted sagittal and transverse images were useful. Untethering of cord was achieved in 30 cases, in which the filum terminale was divided in 10 patients. There were no serious post-operative complications. The mean post-operative follow-up was 9 months. Most of the patients had an improvement or an arrest of the progress of the symptoms. Patients with shorter duration of symptoms had a better prognosis.
Conclusions: Surgery for tethered cord is safe and effective in experienced hands. Early diagnosis and surgery when deficits are less severe the improvement is more likely.

Keywords: Filum terminale, hypertrophy, non-healing ulcer, tethered cord syndrome


How to cite this article:
Mala R, Lakshmi AM, Nagireddy NB, Chiniga V. Tethered cord syndrome diagnosis and management. J NTR Univ Health Sci 2014;3:8-11

How to cite this URL:
Mala R, Lakshmi AM, Nagireddy NB, Chiniga V. Tethered cord syndrome diagnosis and management. J NTR Univ Health Sci [serial online] 2014 [cited 2019 Apr 22];3:8-11. Available from: http://www.jdrntruhs.org/text.asp?2014/3/1/8/128414


  Introduction Top


Tethered cord symptom complex was originally described by Brickner in 1918. [1] Chute in 1921 described the involvement of the bladder in some types of spina bifida. [2],[3] The incidence of neural tube defects is 0.17-6.39 per 1000 live births worldwide. [4] There has been a decline in incidence over the past 20 years, largely due to folic acid supplementation, termination of pregnancy after prenatal diagnosis. Tethering of the spinal cord can occur from a thickened filum terminale, scarring due to myelomeningocele repair, lipoma, or diastematomyelia. [5] The clinical manifestations of the tethered cord are protean and commonly by motor and sensory dysfunction of the lower extremities, urinary and bowel incontinence, and orthopedic deformities. [6] M.R.I. offers the ready diagnosis. [7],[8] We have reviewed our experience in diagnosis, management, and outcome of these patients.


  Materials and Methods Top


Between 2007 and 2011, 30 patients presented with tethered cord syndrome at Department of Neurosurgery, Government General Hospital, Kurnool. All the case notes were thoroughly reviewed and analyzed.


  Results Top


Of the 30 patients, 16 were males and 14 were females. Their ages ranged from 10 months to 35 years with a mean age of 11.4 years.

The common clinical presentation was lower extremity pain, lower extremity weakness, sensory disturbance, and bladder dysfunction [Table 1]. Thirteen patients presented with back lesion in the form of lipoma, hypertrichosis [Figure 1], and lipomyelomeningocele. Nine had previously undergone surgery for myelomeningocele closure immediately after birth. Eighteen had foot deformities in the form of equinovarus. Six patients presented with non-healing ulcers on the feet [Figure 2]. One patient presented with hypertrophy of calf muscle on one side.
Table 1: Clinical presentation in 30 patients with tethered cord syndrome


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Figure1: Picture with hypertrichosis

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Figure 2: Trophic ulcers of foot

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Plain radiography of their spine showed spina bifida in 15 out of 30 (45.5%), and rest of patients had no abnormality in the spine [Figure 3]. All patients had M.R.I., and Low conus were seen in all patients [Figure 4].
Figure 3: Spina bifida

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Figure 4: MRI sagittal with syringomyelia and low lying cord

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Operative pathology

Thirty patients had untethering, and 10 of them also had division of filum terminale. All patients had low conus below L2. Thickened filum terminale was found in 10 patients (40%), lipomeningocele and adhesions of nerve roots to dura in 9 patients (30%), post-repair myelomeningocele adhesions in 6 patients (20%), aggregation of root bundles and adhesion of nerve roots to dura in 3 (10%), 3 cases with type I and type II split cord malformations as cause of tethering [Figure 5] and [Figure 6].
Figure 5: MRI axial view diastematomyelia with bony spurr

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Figure 6: Per-operative picture with diastematomyelia

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The mean post-operative follow up was 9 months. Twelve cases presented with back pain had relief of pain, 12 patients had improvement of motor symptoms, 6 patients had improved in sensory symptoms, 12 patients had improved control of bladder, ulcers healed in 4 patients, and none of the patients showed worsening of symptoms [Table 2]. There were 5 cases of C.S.F. leak in the immediate post-operative period; this was controlled with reinforcement sutures.
Table 2: Management out come

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  Discussion Top


Tethered cord syndrome (TCS) is most commonly seen in children, but adults have also been affected. [5] The onset of symptoms of TCS has been reported to be more common during growth spurts. The cord reaches the adult level of L1-L2 in most of the persons by approximately 2 months after birth. [9] A conus level lower than mid-L2 vertebral level is generally accepted as abnormal. [10] Normal filum thickening is stated to be 2 mm or less.

The common pathophysiology of this lesion is tethering of the neural elements. Whatever may be the tethering lesion, the cord is under traction and tension rather than the nerve roots. Tethering of the cord to the caudal dural sac by lipoma presumably involves an inclusion of adipose cells during process of neurilation and dysjunction. [11] Abnormal canalization and regression contribute to the isolated short thickened tethering of filum terminale. Patients who have undergone repair of myelomeningocele shortly after birth have a low conus and cord tethering at the site of previous repair. [5],[12],[13],[14] Yamada et al. demonstrated that in cord tethering, neural dysfunction correlated with mitochondrial anoxia in the conus. [15],[16] Perikariya (nerve cell bodies) are affected earlier than axons, and these may account for the earlier appearance of incontinence, muscle atrophy, and hyporeflexia as opposed to long tract sings such as hyperreflexia and the Babinski sign. Segmental muscle enlargement (calf hypertrophy), an unusual finding, is the result of partial denervation and continuous neuromuscular irritability, which is caused by tethered cord. [17]


  Conclusions Top


Surgery for tethered cord is safe and effective in experienced hands. Early diagnosis and surgery when deficits are less severe the improvement is more likely. Any infant having a midline lumbar cutaneous anomaly such a lipoma, hair patch, or sinus should be evaluated to rule out tethering of spinal cord. In any differential diagnosis of the etiology of the urinary incontinence in children, one must include the tethered cord syndrome. Segmental muscle hypertrophy is an unusual finding in tethered cord syndrome. Healing of cutaneous ulcers after untethering is a significant finding.


  Acknowledgments Top


I thank Professor Dr. W. Seetharam. M.Ch., for his suggestions and review of this paper and Dr. Ananth. M.Ch., DR. B. Nagaraju. M.Ch., Dr. Venkateswararao for preparing the manuscript.

 
  References Top

1.Brickner WM. Spina bifida occulta. Am J Med Sci 1918;155:473-502.  Back to cited text no. 1
    
2.Adamson AS, Gelister J, Hayward R, Snell ME. Snell Tethered cord syndrome: An unusual cause of Adult Bladder Dysfunction. Br J Urol 1993;71;417-21.  Back to cited text no. 2
    
3.Chute AL. The relation between spina bifida occulta and certain cases of urinary retention. J Urol 1921;5;317.  Back to cited text no. 3
    
4.Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome; a 25 year prospective. Pediatr Neurosurg 2001;34:114-20.   Back to cited text no. 4
    
5.Pang D, Wilberger JE Jr. Tethered cord syndrome in adults. J Neurosurg 1982;57:32-47.  Back to cited text no. 5
[PUBMED]    
6.French BM. Midline fusion defects, and defects of formation. In: Youmans JR, Editor. Neurological Surgery, 3 rd ed, Chapter 39. Philadelphis. W.D. Sunders; 1990. p. 1081-235.  Back to cited text no. 6
    
7.Raghavan N, Barkovich AJ, Edwards M, Norman D. MR Imaging in tethered spinal cord syndrome. AJR Am J Roentgenol 1989;152:843-52.  Back to cited text no. 7
    
8.Warder DE, Oakes WJ. Tethered cord syndrome the low lying and normally positioned conus. Neurosurgery 1994;34:597-600.  Back to cited text no. 8
    
9.Barson AJ. The vertebral level of termination of the spinal cord during normal and abnormal development. J Anatomy 1970;106;489497.  Back to cited text no. 9
    
10.Fitz CR. Harwood Nash DD. The tethered conus AJR Am J Roentgenol 1970;125:519-23.   Back to cited text no. 10
    
11.McLone DG. The subarachnoid space-a review. Childs Brain 1980;6:113-30.  Back to cited text no. 11
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12.Filler AG, Britton JA, Uttley D, Marsh HT. Adult post repair myelomeningocele and tethered cord syndrome. Br J Neurosurg 1995;9:659-66.   Back to cited text no. 12
    
13.Tamaki N, Shirataki K, Kojima N, Shouse Y, Matsumoto S. Tethered cord syndrome of delayed onset following repair of myelomeningocele. J Neurosurg 1988;69:393-8.  Back to cited text no. 13
    
14.Düz B, Gocmen S, Secer HI, Basal S, Gönül E. Tethered cord syndrome in adult. J Spinal Cord Med 2008;31:272-8.  Back to cited text no. 14
    
15.Yamada S, Zinke DE, Sanders D. Pathophysiology of tethered cord syndrome. J Neurosurg 1981;54:494-503.  Back to cited text no. 15
[PUBMED]    
16.Yamada S, Won DJ. What is the true tethered cord syndrome. Childs Nerv Syst 2007;23:371-5.  Back to cited text no. 16
    
17.Bertorini T, Woodhouse C, Horner L. Muscle Hypertrophy secondary to the tethered cord syndrome. Muscle Nerve 1994;17:331-5.  Back to cited text no. 17
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
 
 
    Tables

  [Table 1], [Table 2]



 

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Abstract
Introduction
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