Indmedica Home | About Indmedica | Medical Jobs | Advertise On Indmedica
Search Indmedica Web
Indmedica - India's premier medical portal

Journal of the Anatomical Society of India

Congenital Abnormal Cervical Vertebrae - A Case Report

Author(s): Tiwari, A., Chandra, N., Naresh, M., Pandey, A., *Tiwari, K.

Vol. 51, No. 1 (2002-01 - 2002-06)

Department of Anatomy, M.L.N. Medical College Allahabad. *Orthopaedic Surgeon, Community Health Centre, Dadri, Noida-INDIA.

Abstract

Normal typical cervical vertebrae from 3rd to 6th are characterised by small body, triangular spinal canal, foramina transversarium, superior articular facet directed backward and upward, inferior articular facet directed forwards and downwards. The specimen shows two typical cervical vertebral laminae fused on left side along with the failure of development of pedicle and costal element and anterior tubercle of transverse process of lower cervical vertebrae. Foramen transversarium and intervertebral canal are absent on left side. On right side lamina of upper cervical vertebra is failed to fuse with the opposite side. Lamina of lower cervical vertebra is also failed to fuse with the opposite side. Foramen transversarium of right side lower cervical vertebra is quite small. These abnormalities lead to significant clinical signs and symptoms. therefore the case has been studied.

Key words: Cervical, Vertebrae, Foramina, Lamina, Pedicle, Fusion

Introduction:

The characteristics of the normal typical cervical vertebrae (3rd to 6th) can be enumerated as follows-smaller body, broader lateral dimension,triangular larger vertebral foramen exceeding the size of the body, longer and narrower lamina, foramina transversaria in transverse process and short bifid spine.

Abnormalities in any of the features may be associated with neurological signs and symptoms. The later can be variable according to the extent of pathology. We are presenting the details of one abnormal fused cervical vertebrae in the text.

Material and Methods:

In a routine annual survey of the bone room, department of Anatomy, M. L. N. Medical College, Allahabad, we found one abnormally fused two typical cervical vertebrae. We analysed this fused cervical vertebrae with the normal cervical vertebrae under the following headings-body, lamina, pedicle, transverse process, foramina transversarium and costal element. The specimen was photographed from different aspects.

Observation and Results:

We are presenting the details of one abnormally fused cervical vertebrae. The body of two typical cervical vertebrae are joined together through intervertebral disc. On the left side vertebral laminae of upper and lower cervical vertebrae are fused together. On the left side there is failure of developement of pedicle, costal element and anterior tubercle of transverse process of lower cervical vertebra. Foramen transversarium and intervertebral canal are absent on left side. On the right side lamina of upper cervical vertebra has failed to fuse with the opposite side and are small. Lamina of lower cervical vertebra has also failed to fuse with the opposite side. Foramen transversarium of right side lower cervical vertebra is quite small.

All these abnormalities may lead to clinical signs and symptoms. These are-shortening of spine in the cervical region, the trapezei may be unduly prominent laterally and give a webbed appearance-
webbed neck, limited neck motion, lowered hair line, osseous malformation (scoliosis, kyphosis and torticollis) leading to signs of peripheral nerve irritation such as,pain, burning sensations and cramp or signs of nerve compression such as hypoaesthesia/ anaesthesia, weakness/paralysis, fibrillations and reduced deep reflexes.

All the above findings have similarities with the specimen of Klippel-feil syndrome (congenital fusion of cervical vertebrae,brevicollis). It is a condition of congenitally fused and deformed cervical vertebrae that results in restricted neck motion and neurologic phenomena.

Discussion:

Authers have tried their best to search the literature for this type of work in the field of anatomy. But to the best of knowledge of Authors, this type of work in the field of Anatomy is reported for the first time. Though few cases have been reported on Klippel-Feil syndrome (KFS) in Orthopaedics. Klippel-feil syndrome was first described by Maurice Klippel, a French internist and Andre Feil, in a patient with congenital fusion of cervical vertebrae. (Patel & Laureman, 1995) Based on clinico-radiological features it is classified into:

  • Type 1 : Fusion of cervical and upper thoracic vertebrae with synostosis accounting for 40%.
  • Type 2 : Isolated cervical spine fusion accounting for 47%.
  • Type 3 : Cervical vertebrae with lower thoracic or upper lumbar fusion accounting 13%.

Type 2 has the lowest risk of the scoliosis (Thomsen et al, 1997). A fourth type has been suggested and is associated with sacral agenesis (Raas-Rothschild, et al, 1988). Our case showed features of type 2. Embryological vascular disorder like subclavian artery supply disruption sequence have been hypothesized to result in Klippel-Feil syndrome (Brill et al 1987). Pattern of bony fusion may involve more than one level giving rise to wasp waist sign which is a valuable radiological sign of Klippel-Feil Syndrome (Nyugen & Tyrrel, 1993). Cervical spondylosis, disc herniation and secondary degenerative changes are more at levels adjacent to fused vertebra (Ulmer et al, 1993). Risk increases in case of unstable fusions, cranio cervical anomalies, spinal stenosis and such cases should be adequately followed up (Shoul & Ritro, 1952). Neurological signs and symptoms are variable, depending on the degree of pathology (Mosberg, 1953). The 3rd, 4th and 5th cervical vertebrae are fused and 6th is in the form of two hemivertebrae in the KF syndrome (Smith & Griffin, 1992). In the surgical treatment of the Klippel Feil syndrome, the cervical ribs and first three thoracic ribs may be removed (Bonala, 1956).

In our specimen the vertebral laminae are fused on left side. Foramen trasversarium and intervertebral canal are absent on left side. Lamina of upper and lower cervical vertebrae on right side failed to fused with the opposite side. Foramen transversarium of right side lower cervical vertebra is quite small.

Therefore it is concluded that this work is of its own type and has been reported for the first time. It may lead to compression of nerve roots resulting into hypoesthesia, & paralysis of the concerned parts of the body.

References:

  1. Bonola, A. (1956) : Surgical treatment of the klippel-feil syndrome.Journal of bone and Joint Surgery. 38 B : 440.
  2. Brill, C. B. Peyster, R.G. et al (1987) : Isolation of right subclavian steal in a child with klippel-feil syndrome. American Journal of Medical Genetics. 16: 933-940.
  3. Mosberg, W. H.. Jr. (1953) : Klippel-Feil syndrome ; Etiology and treatment of neurological signs. Journal of Nervous and Mental Diseases. 117: : 479.
  4. Nyugen, V. D. and Tyrrel, R. (1993) : Klippel-Feil syndrome- patterns of bony fusion and wasp waist sign. Skeletal Radiology. 22 (7): 519-523.
  5. Patel, P. R. and Lauerman, W.C. (1995) : Maurice Klippel Spine. 20 (19): 2157-2160.
  6. Raas-Rothschild, A., Goodman, R. M. et al (1988) : Klippel- Feil anomaly with sacral agenesis-an additional subtype. Type IV. Journal of Craniofacial Genetics and Developmental Biology 8(4): 297-301.
  7. Shoul, M. L. and Ritro, M. (1952) : Clinical and roentgenographical manifestations of Klippel-Feil syndrome. American Journal of Roentgenology. 68: 369.
  8. Smith, B. A. and Griffin, C. (1992) : Klippel-Feil syndrome. Annals of Emergency Medicine 21 (7): 876-879.
  9. Thomsen,M. N., Schneider, U., Weber,M. et al (1997): Scoliosis and congenital anomalies associated with Klippel- Feil syndrome type I-III Spine. 22 (4) : 396-401.
  10. Ulmer, J. L., Elster, A. D. et al (1993) : Klippel-Feil syndrome; CT and MR of acquired and congenital abnormalities of cervical spine and cord. Journal of Computer assisted tomography 17 (2) : 215-224.

Missing Image

Fig.1: Anterior view - The body of two typical cervical vertebrae are fused through a vestigial disk.

Missing Image

Fig.2 : Postero-lateral view (right side)- The spinous process and lamina on the right side of upper and lower cervical vertebra have failed to fuse with the opposite side.

Missing Image

Fig. 3 : Postero-lateral view (left side) - The laminae and spinous process of upper and lower cervical vertebrae are fused together on left side. Pedicle, costal element and anterior tubercle of transverse process of lower cervical vertebra have failed to develop. Foramen transversarium in the lower cervical vertebra and intervertebral canal between two cervical vertebrae are absent on left side.

ABBREVIATIONS -

Body - B
Failed to fuse - FF
Foramen transversarium - FT
Fused - F
Lamina - L
Spinous Process - S
Transverse Processes - TP
Vestigial disk - Vd

Access free medical resources from Wiley-Blackwell now!

About Indmedica - Conditions of Usage - Advertise On Indmedica - Contact Us

Copyright © 2005 Indmedica