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CASE REPORT
Year : 2017  |  Volume : 6  |  Issue : 2  |  Page : 129-132

A case report on giant C5 spinous process with partial block vertebrae at C2, C3, and C4 levels


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

Date of Web Publication13-Jun-2017

Correspondence Address:
K Chenchu Lakshmi
Department of Radiodiagnosis, Kurnool Medical College, Kurnool, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.208013

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  Abstract 

The spine is a complex and vital structure. Its function includes not only the structural support of the body as a whole but it also serves as a conduit for safe passage of the neural elements while allowing proper interaction with the brain. We report the case of a 7-year-old child who presented with a swelling in the posterior aspect of the neck. On lateral radiograph, there was elongated spinous process in the neck. Computed tomography examination better depicted this congenital variant and clearly showed giant spinous process of the C5 vertebrae. There was associated spina bifida at this level. Moreover, there was asymmetrical fusion of the posterior elements at C2, C3, and C4. Magnetic resonance imaging showed normal signal intensity of cervical cord. This is a very rare congenital anomaly. Very few cases have been reported previously.

Keywords: Cervical spine–congenital abnormality, CT, embryology, spine


How to cite this article:
Reddy O J, Gafoor J A, Suresh B, Lakshmi K C. A case report on giant C5 spinous process with partial block vertebrae at C2, C3, and C4 levels. J NTR Univ Health Sci 2017;6:129-32

How to cite this URL:
Reddy O J, Gafoor J A, Suresh B, Lakshmi K C. A case report on giant C5 spinous process with partial block vertebrae at C2, C3, and C4 levels. J NTR Univ Health Sci [serial online] 2017 [cited 2020 Apr 10];6:129-32. Available from: http://www.jdrntruhs.org/text.asp?2017/6/2/129/208013




  Introduction Top


The spine is a complex and vital structure. Its function includes not only the structural support of the body as a whole but is also serves as a conduit for safe passage of neural elements while allowing proper interaction with the brain. Congenital cervical spinal anomalies are relatively common and can be seen in upper and lower cervical regions. Among them, unilateral hyperplasia of lamina and spinous process of cervical vertebra in association with partial block vertebrae is rare and only few cases were reported in English literature.


  Case report Top


A male child of age 7 years was brought by her mother to the surgical outpatient department with a history of swelling over the back of the neck. She reported that the swelling was recognized since 1 year of age. No significant increase in the size of the swelling was noted since then. Mild restriction of neck movements was noted. On clinical examination, there was a hard palpable, immovable, posterior cervical mass measuring 12 × 10 mm located 3 cm above the C7 spinous process. Overlying skin and subcutaneous structures were normal. There was no local color changes or increase in temperature. There was no neurological deficit associated with the mass.

The patient was referred to the radiology department for X-ray of the cervical spine in a lateral view. On X-ray, there was an elongated spinous process at the C5 vertebral level [Figure 1]. There was also partial block vertebrae with fusion of the posterior elements of C2 and C3 vertebrae. In order to find other associated features, computed tomography (CT) of the cervical spine was advised. On CT, there was giant spinous process of the C5 vertebra [Figure 2]. There was associated spina bifida at this level [Figure 3]. There was asymmetrical fusion of the posterior elements of C2, C3, and C4 vertebrae [Figure 4]. On the right side, C2 and C3 vertebral posterior elements were fused. On the left side, C2, C3, and C4 vertebral posterior elements were fused. Three-dimensional rendering helped in better visualization and demonstration [Figure 5],[Figure 6],[Figure 7].
Figure 1: X-ray of the cervical spine (lateral view) showing elongated C5 spinous process (plus mark) with partial fusion of the posterior elements C2 and C3 vertebrae

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Figure 2: CT cervical spine sagittal section showing elongated C5 vertebral spinous process (plus mark)

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Figure 3: CT cervical spine axial section showing spina bifida at the C5 vertebral level

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Figure 4: CT cervical spine coronal section showing asymmetrical fusion of posterior elements of C2, C3, and C5 vertebrae. C2 and C3 vertebral posterior elements are fused on the right side and C2, C3, and C5 vertebral posterior elements are fused on the left side

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Figure 5: Three-dimensional rendering showing elongated spinous process of C5 vertebra with spina bifida at this level

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Figure 6: Three-dimensional rendering mode showing fusion of posterior elements of C2 and C3 vertebrae on the right side

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Figure 7: Three-dimensional rendering mode showing fusion of posterior elements of C2, C3, and C4 vertebrae on the left side

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Because of the vertebral fusion anomalies, more than two cervical vertebrae fusion anomalies, this condition might have been associated with Klippel–Feil syndrome. Magnetic resonance imaging (MRI) of the cervical spine was done to rule out cord abnormalities [Figure 8]. Normal cord signal intensity was noted. No significant changes were noted. Further, we investigated the entire body for several organ anomalies. However, we could not detect additional abnormalities such as congenital heart defects and renal anomalies.
Figure 8: MRI cervical spine T2-weighted in sagittal section showing normal cord signal intensity

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  Discussion Top


The spine is a complex and vital structure. Its function includes not only structural support of the body on the whole but also serves as a conduit for safe passage of the neural elements while allowing proper interaction with the brain. Anatomically, a variety of tissue types are represented in the spine. Embryologically, a detailed cascade of events must occur to result in the proper formation of both the musculoskeletal and neural elements of the spine. Alterations in these embryologic steps can result in one or more congenital abnormalities of the spine.

Malformations of the spine can be classified into three main groups:

  1. neural tube defects,
  2. failure of formation, and
  3. failure of segmentation.


Our case is of failure of segmentation.

Failure of segmentation

Failure of segmentation occurs when two or more vertebrae fail to fully separate and divide with concomitant partial or complete loss of a growth plate. Embryologically, if two adjacent somites or their associated mesenchyme do not separate properly, a segmentation defect will occur. These defects are classified depending on the region and quantity of vertebrae affected. Involvement of the entire vertebrae creates block vertebrae, whereas defects of specific regions of the vertebral ring create unilateral bars that act as an asymmetric rigid tether to normal growth.

Pathogenesis of giant spinous process is unknown. Only few cases have been reported regarding giant spinous process [Figure 9]. Failure of segmentation of two or more cervical vertebrae is usually associated with Klippel–Feil syndrome.
Figure 9: Table showing summary of case reports for unilateral hyperplasia of spinous process and laminal that we can found in the English literature

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Klippel–Feil syndrome

Klippel–Feil syndrome is a complex disorder that consists of congenital fusions of the cervical vertebrae with the patients having a short neck, limitation of the movement of the head or the neck, and a low posterior hairline. This syndrome is a result of failure of the normal segmentation of the cervical somites during the 3rd to 8th weeks of gestation.[1] Klippel and Feil first reported the clinical features of this syndrome in 1912. In 1919, Feil described additional cases and distinguished Klippel–Feil syndrome into three different groups according to the degree of involvement.[1],[2]

Type I: Massive fusion of many of the cervical and upper thoracic vertebrae

Type II: Fusion at one or two interspaces with occipitoatlantoid fusion, hemivertebrae, or other abnormalities in the cervical spine

Type III: Cervical fusion in combination with lower thoracic or lumbar fusion

The anterior concave indentation at the site of the absent or reduced interspace between the fused vertebrae is known radiologically as the wasp-waist sign. Presence of wasp-waist sign indicates complete fusion of vertebrae.

The clinical presentation of KFS is variable. Klippel–Feil syndrome includes a complex of anomalies such as scoliosis (60%), renal anomalies (35%), such as agenesis, dysgenesis, renal ectopia, malrotation, duplication, and Sprengel's deformity (30%), deafness (30%), congenital heart disease (14%), such as atrial septal defect and coarctation, cranial and facial asymmetry, (13%) and cleft palate (10%).[1],[3]

The important symptoms and anomalies are usually revealed incidentally on radiological examinations in the mild cases. However, the patients may develop neurological symptoms that are secondary to the degenerative disc disease of the adjacent mobile segments, spinal instability from the hypermobility or from trauma, and spinal stenosis in the later decades of life. Therefore, the early diagnosis of KFS is important because of the high risk for other associated diseases in the affected patients.[1],[4]

A high incidence (64%) of genitourinary-tract anomalies that are demonstrated by IVU and on physical examination is encountered in patients with Klippel–Feil syndrome. The incidence of these anomalies in the three types of the syndrome are essentially the same, and unilateral renal agenesis is the most common abnormality.[5] Many kinds of congenital cardiac anomalies are also prominent in this syndrome, and ventricular septal defect is the most commonly encountered lesion.

CT scans can delineate fine skeletal structures in great detail with its ability for multislice scanning, and CT may assume a new role for this purpose because of its superior three-dimensional spatial resolution. Nowadays, volume rendering is the preferred technique for musculoskeletal system examinations as it can improve visualization and better demonstrate subtle findings.


  Conclusion Top


In the presence of axial spine abnormality, such as unilateral hypertrophy of spinous process, the remainder of the spine should be evaluated for additional congenital abnormalities. If additional congenital abnormality is detected, related radiological examination should be done before surgery to prevent unexpected unpleasant outcomes.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Thomsen MN, Schneider U, Weber M, Johannisson R, Niethard FU. Scoliosis and congenital anomalies associated with Klippel-Feil syndrome types I-III. Spine 1997;22:396-401.  Back to cited text no. 1
    
2.
Nguyen VD, Tyrrel R. Klippel-Feil syndrome: Patterns of bony fusion and wasp-waist sign. Skeletal Radiol 1993;22:519-23.  Back to cited text no. 2
    
3.
Dahnert W. Radiology Review Manual. 2nd ed. Maryland: Williams & Wilkins; 1993. p. 130.  Back to cited text no. 3
    
4.
Pizzutillo PD, Woods M, Nicholson L, MacEwen GD. Risk factors in Klippel-Feil syndrome. Spine 1994;19:2110-6.  Back to cited text no. 4
    
5.
Moore WB, Matthews TJ, Rabinowitz R. Genitourinary anomalies associated with Klippel-Feil syndrome. J Bone Joint Surg Am 1975;57:355-7.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]


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