Case No. :	61
Month :	January
Year :	2004
Contributor :	Dr. Sanjota Shrigandhi

Other Cases

A 30-year-old man presented with complaints of tingling and numbness and stiffness in both lower limbs since one month. Examination revealed paraparesis with no sphincter involvement


RADIOLOGICAL FINDINGS:

A radiograph of the cervical spine (Fig. 1) showed multiple calcific densities in the right paraspinal region at the C7-D1 level. As these appeared to be of cartilaginous origin - resembling an osteochondroma - plain radiographs of the pelvis, knee joints, elbow and humerus (Figs. 2,3,4,5) were obtained. These showed multiple bony outgrowths with misshaped bones showing modeling deformities.

Fig.1	Fig.2
Fig.3	Fig.4
Fig.5

MRI of the cervico-dorsal spine (Figs. 6,7,8) showed an osteochondroma arising from the anterior part of body of D1 vertebra. Part of the osteochondroma extended posteriorly causing indentation of the thecal sac at this level.

Fig.6	Fig.7
Fig.8

To facilitate surgical dissection, an esophagogram was obtained. This showed an extrinsic impression on the supra-aortic esophagus. A distinct gap seen between bony osteochondroma and the esophagus represented the unossified cartilage cap.

Differential diagnosis: Speckled calcification in a bony tumor should suggest that it is of cartilaginous origin. When such tumors protrude beyond normal bone margins, an osteochondroma should be diagnosed. Often, in such cases, it is necessary to rule out multiple exostoses by a skeletal survey especially if associated with modeling changes in the affected bone.

DISCUSSION:

Diaphyseal Aclasis

Synonyms:	Hereditary multiple exostoses
	Multiple Osteochondromatosis
	External chondromatosis

Hereditary Multiple Exostoses is an inherited metaphysical overgrowth that is characterized by multiple osteochondromas - aclasis referring to an alteration of the modeling process. It is most frequently discovered between the ages of 2 and 10 years. Both sexes are equally affected. The number of osteochondromas varies from a few to hundreds, with an average of 10.The metaphyseal portions of the bones of the knee, ankle, shoulder and wrist are most commonly affected. The distribution is usually bilateral and may be symmetric. The osteochondromas may be sessile or pedunculated.

Exostoses may cause deformities and restriction in range of movement of that joint. Spinal osteochondromas may cause cord compression. They may cause pressure on the nerves and tendons causing pain. Malignant transformation to a chondrosarcoma occurs in 5 to 25% of cases. Abrupt change in size or pattern od calicifcation may help sus[ect malignant reansformation.

If an exostosis is painful, pressing on important structures or causing deformities they can be surgically excised with 20 to 50% chances of recurrence.

It is essential to determine whether the case is one of an isolated osteochondroma or multiple exostoses as the chances of malignant transformation in Diaphyseal aclalsis are about 25%.

Home | College | Hospital | Alumni | Contact | Departments | Search | Radiology