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These are selected bones from the spine of an individual from Medieval Hereford Cathedral that suffered with a congenital condition known as Klippel-Feil syndrome.  Klippel-Feil syndrome is the congenital fusion of two or more cervical vertebrae.  Clinically, Klippel-Feil syndrome is diagnosed a short neck line, low posterior hair line and limited movement of the neck (Resnick  1995) and can be associated with other hard and soft tissue anomalies.  The condition is separated into three types: Type I is mass fusion of the cervical vertebrae, Type II is the fusion of two or more cervical bodies, and Type III is Type I plus thoracic or lumbar involvement (Maclone 2001).  This individual presents with Type III Klippel-Feil syndrome with fusion within multiple cervical and upper thoracic vertebrae, other segmentation abnormalities in the cervical vertebrae and upper ribs, scoliosis, flat cranial base, and other postcranial developmental abnormalities.  The final project website will have the complete vertebral column, cranium, mandible, ossa coxae, and elements from the hands and feet for you to view!

Fused cervical vertebrae 1-3

Cervical vertebrae 1-3 are malformed and fused into a single block.  The vertebrae are fused through adjoining articular facets and bodies.  Posteriorly, there is irregular formation and fusion of the lamina and vertebral arches and spinous processes. The lamina of the 3rd cervical vertebra failed to form on the right side, while the left side is thin and irregularly shaped.  The right side of the atlas is situated more inferiorly than the left. Correspondingly, the majority of the right side of the body of the axis has failed to form. The right transverse foramen on the 3rd cervical is positioned posterior to the transverse process, instead of a normal lateral location.  Posterior to this, the inferior articular facet is not of usual shape, being more rounded and having an irregular articular contour.  The left inferior facet has joint surface contour change and is heavily pitted.  Similarly, the inferior surface of the body of the 3rd cervical is heavily pitted.  Unfortunately, the odontoid process is taphonomicaly damaged and therefore, it is unknown if it would have extended superiorly passed the foramen magnum.

Right vertebral arches of cervical vertebrae 5-7

The vertebral arches of cervical vertebrae 5-7 are fused through the laminae and inferior articular processes and then merge into a single transverse process.  Although broken post-mortem, evidence remains that the spinous processes failed to fuse to their left counterpart.  The ends of the spinous processes have slight lateral deviation.  The inferior articular facet of the fifth cervical vertebra has irregular osteophytic growth along its inferior margin and has an eburnated and heavily pitted articular surface.

Cervical vertebra 5

There is aplasia (absent due to failure to form) of the right lamina and left inferior articular facet.  The transverse foramen on the left side has stenosis (narrowing) caused by a transverse dividing layer of bone that has two openings, a small sub-circular central opening and an anterior linear opening.  Stenosis is also caused superiorly by the protrusion of a large almost horizontal articular facet.  This facet has extensive osteophytosis, surface pitting and eburnation. The right inferior facet is deformed (concave in shape) and has extensive osteophytosis, pitting and eburnation.   The body has a partial sagittal cleft with both a connecting strand internally and a bony bridge anteriorly.  There is reduction of vertebral height surrounding the cleft, which is more extensive inferiorly.  The superior surface of the body on the right side is heavily pitted and the anterior margin has extensive marginal osteophytes.

Cervical vertebra 6

There is extensive osteophytosis of the margins of the body, especially on the right uncinate process.  This right uncinate process is considerably expanded and has extensive surface pitting and eburnation.  The superior and inferior surfaces of the body have heavy pitting, with an area of coalesced pitting.  The inferior surface of the anterior tubercle on the right side has a large articular facet that is heavily pitted.  (Its adjacent articulation is not known).  The left superior articular facet is substantially smaller than what is expected in normal variation.

Cervical vertebra 7

The body is malformed with reduction in the anterior posterior width, which may represent a partial cleft.  The exact nature of the malformation of the body is obscured by post-mortem loss of the right side.  The vertebral arch is unfused and the visible left lamina and spinous process is located more lateral than of normal anatomical position.  The inferior articular facet has slight pitting.

Fused vertebral bodies of the 1st-3rd thoracic

This is the left side of a block of fused bodies of thoracic vertebrae 1-3. Although broken post-mortem, evidence remains for the reduction of body height of the 2nd thoracic anteriorly and on the right.  The height of the body of the 3rd thoracic is also reduced.

Fused vertebral arches of thoracic vertebrae 2 and 3

Due to post-mortem damage, the full exact nature of this bone is not known.  The presence of two distinct pedicles and the superoinferior elongation of the lamina on the right side suggest posterior congenital fusion of the 2nd and 3rd vertebrae.  The fusion occurs through the right lamina, inferior articular facets, spinous process, and transverse processes, with agenesis (failure of development) of the left arch and transverse process. The inferior articular facet on the left side has an area of new compact bone formation on the articular surface.

Fused right 1st and 2nd rib mass

This is what appears to be a fragment of a dysplastic fused first and second rib.  The ribs are fused through the bodies and necks. The rib heads are deformed and irregularly placed, with a mass of bone situated superiorly.  There is a large articulating facet on this superior mass that is heavily pitted.

Two fused left ribs

These two ribs are fused through the head, neck, and the body for c.16mm from the tubercle.

Fused Left 1st and 2nd rib mass

Left rib one and two are fused for c.22mm along the body and then merge to share a neck and head.  The head of rib two is bifurcated, with the inferior portion shared with rib 2 and the superior portion that extends superiorly.  The medial segment is an irregular mass of bone having multiple small articular facets (at least five separate).   The superior articular facet does not seem to articulate with any surviving elements of the trunk.  The remaining facets articulate with a lost portion of the left vertebral bodies.

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