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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 43  |  Issue : 2  |  Page : 210-212
Disc prolapse and cord contusion in a case of Klippel-Feil syndrome following minor trauma


1 Department of Surgery , Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha, India
2 Department of Anesthesiology, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha, India

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   Abstract 

Klippel-Feil syndrome (KFS) is defined as congenital fusion of two or more cervical vertebrae and patients with KFS are frequently asymptomatic. However, these patients are especially prone to cervical cord injury after a minor fall or a major traumatic episode. We report an unusual case of KFS where the patient had disc prolapse between two Klippel-Feil segments and discuss the difficulties in the management of this case.

Keywords: Cervical disc, Klippel-Feil anomaly, trauma

How to cite this article:
Agrawal A, Badve AM, Swarnkar N, Sarda K. Disc prolapse and cord contusion in a case of Klippel-Feil syndrome following minor trauma. Indian J Orthop 2009;43:210-2

How to cite this URL:
Agrawal A, Badve AM, Swarnkar N, Sarda K. Disc prolapse and cord contusion in a case of Klippel-Feil syndrome following minor trauma. Indian J Orthop [serial online] 2009 [cited 2020 Feb 21];43:210-2. Available from: http://www.ijoonline.com/text.asp?2009/43/2/210/50857

   Introduction Top


 Klippel-Feil syndrome More Details (KFS) is defined as congenital fusion of two or more cervical vertebrae and results from faulty segmentation of the vertebral axis during weeks 3-8 of gestation. [1],[2] Although patients with KFS are frequently asymptomatic, these patients are especially prone to cervical cord injury after a minor fall or a major traumatic episode. [2],[3],[4] We report an unusual case of KFS where the patient had disc prolapse between two Klippel-Feil segments and discuss the difficulties in the management of this case.


   Case Report Top


A 40-year-old man presented with complaints of weakness, tingling and numbness in all four limbs following fall from bicycle. Following the fall he had quadriparesis with muscle power grade 4+/5. The deep tendon jerks were exaggerated in all four limbs. He used to walk with support. The bowel and bladder functions were normal. The posterior column sensations were moderately impaired in the lower limbs. Bilateral plantars were extensor. The gag and palate reflexes were normal. X-ray films of the cervical spine showed fusion of cervical vertebrae at two levels i.e. the C3 and C4 vertebrae and C5, 6 and C7 vertebrae with spinal instability [Figure 1a] and [Figure 1b]. Magnetic resonance imaging (MRI) of the cervical spine showed congenital fusion of the same vertebral bodies and in addition it also showed disc prolapse at C4-5 level with cord contusion at the same level [Figure 2]. The patient underwent right anterior cervical approach, C4-5 discectomy, fusion with autologus bone graft and C3-C4 fixation with titanium plate and screws [Figure 1c]. Following surgery hard cervical collar was applied and all activities related to neck movements were restricted for a period of six weeks. The patient made gradual recovery. At four months follow-up the power in all four limbs was grade 4+/5 and he was able to walk without support and had full control of bowel and bladder. The posterior column sensation recovered. However, because of screw pull out we had to remove the implants once there was evidence of bony fusion [Figure 1d].


   Discussion Top


Spondylotic and discogenic changes occur in the junctional segments in association with cervical instability resulting from hypermobile segments adjacent to the fused vertebrae. A symptomatic herniated cervical disc may develop from an excessive mechanical load stress in KFS patients with multiple fused segments. [3],[5] As in the present case patients with KFS may be at an increased risk of neurological injury as a result of hypermobility of the various cervical segments. This predisposition to spinal cord injury has been attributed to the fused segments and the resultant altered mechanical force transfer that makes the adjacent nonfused segments excessively mobile [Figure 3]. [2],[3],[4] The cervical spine is unable to compensate for excessive flexion, extension, rotation and lateral bending. The block vertebrae transfer forces through long mechanical lever arms to the adjacent hypermobile segments. The more the levels fused, the more patients are predisposed to excess motion and overloading at the remaining mobile segment which leads to accelerated discogenic degenerative disease with bulging or herniated disks with subsequent risk of post-traumatic neurological sequelae. [4],[6] Treatment regimens depend on the severity of symptomatic segmental instability or neurological compromise, varying from modification of activities to extensive spinal surgery. [7] As described we opted for microsurgical removal of the herniated disc via an anterior approach that was followed by interbody fixation with anterior plating. [5],[8] However, in the present case short neck and distorted vertebral anatomy posed a difficult challenge both in the exposure of the spine and in the placement of plate. In KFS cases arthroplasty with the Bryan artificial disc has been reported as the most suitable choice in young patients with the aim to preserve the motion in the remaining cervical spine segments which is important for maintaining neck function and quality of life. [9],[10] However, this was not feasible because of high cost in our case. As in the present case, persons with Klippel-Feil syndrome may be at increased risk of sustaining a neurological deficit even after minor trauma and appropriate guidance should be provided to alter their behavior to avoid any episode of neurological compromise. [6]

 
   References Top

1.Ulmer JL, Elster AD, Ginsberg LE, Williams DW 3rd. Klippel-Feil syndrome: CT and MR of acquired and congenital abnormalities of cervical spine and cord. J Comput Assist Tomogr 1993;17:215-24.  Back to cited text no. 1  [PUBMED]  
2.Guilles T, Miller A, Bowen JR, Forlin E, Caro PA. The natural history of Klippel-Feil syndrome: Clinical, roentgenographic and magnetic resonance imaging findings at adulthood. J Pediatr Orthop 1995;15:617-25.  Back to cited text no. 2    
3.Pizzutillo PD, Woods M, Nicholson L. MacEwen GD. Risk factors in Klippel-Feil syndrome. Spine 1994;19:2110-6.  Back to cited text no. 3    
4.Karasick D, Schweitzer ME, Vaccaro AR. The traumatized cervical spine in Klippel-Feil syndrome: Imaging features. AJR Am J Roentgenol 1998;170:85-8.   Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Samartzis D, Lubicky JP, Herman J, Kalluri P, Shen FH. Symptomatic cervical disc herniation in a pediatric Klippel-Feil patient: The risk of neural injury associated with extensive congenitally fused vertebrae and a hypermobile segment. Spine 2006;31:E335-8.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Vaidyanathan S, Hughes PL, Soni BM, Singh G, Sett P. Klippel-Feil syndrome-the risk of cervical spinal cord injury: A case report. BMC Fam Pract 2002;3:6.   Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Tracy MR, Dormans JP, Kusumi K. Klippel-Feil syndrome: Clinical features and current understanding of etiology. Clin Orthop Relat Res 2004;424:183-90.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Neroni M, Gazzeri R, Galarza M, Alfieri A. Intradural cervical disc herniation in a Klippel-Feil patient. Spine 2007;32:E608-10.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Leung CH, Ma WK, Poon WS. Bryan artificial cervical disc arthroplasty in a patient with Klippel-Feil syndrome. Hong Kong Med J 2007;13:399-402.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.Yi S, Kim SH, Shin HC, Kim KN, Yoon DH. Cervical arthroplasty in a patient with Klippel-Feil syndrome. Acta Neurochir (Wien) 2007;149:805-9.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]

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Correspondence Address:
Amit Agrawal
Division of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha - 442004,Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5413.50857

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    Figures

  [Figure 1a], [Figure 1b], [Figure 1c], [Figure 1d], [Figure 2], [Figure 3]

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    Abstract
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    References
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