CRANIOFACIAL
PROBLEMS CAUSED BY TUMORS AND DISEASE
A variety of benign and malignant tumors may affect tissues and structures
in the craniofacial region, by direct invasion, by the compression of
vital structures, or by simply growing and causing deformity.
Cancers that were
considered unresectable, in locations such as the orbit and ethmoids (the
region between the eyes) may become accessible and resectable with the
utilization of craniofacial techniques. The possibility to reconstruct
large facial and cranial defects offers not only an increased cure rate,
but also a better quality of life. The same principles may be used to
provide access to the neurosurgeon for the removal of tumors in areas
that were not accessible by conventional surgical approaches.
Certain conditions, even
though benign with regard to pathological classification, may nevertheless
present formidable problems in their treatment. Neurofibromatosis (Von
Recklinghausen's disease, better known to the lay community as the
elephant man's disease, caused by benign tumorous growth of nerve
elements) often affects the face, causing relentless destruction of
tissues and structures. The orbit is frequently involved. Part of the
orbital skeleton may
be absent bringing the contents of the orbit in direct contact with the
brain. This causes the eye to bulge and pulsate. The eye itself and
particularly the optic nerve may be involved. Glaucoma and loss
of vision are not infrequent. The eyelid tissues are invaded by the process, become enlarged,
flaccid, and may hang down to the lower part of the cheek. The orbital
skeleton is eroded causing further loss of support for the orbital
contents. Although no cure is possible for this disease, by combining
resection of affected tissues and reconstruction of missing bone with bone
grafting, one can restore to the affected structures an acceptable form,
which is often maintained for several years.
Another seemingly benign
condition, fibrous dysplasia, caused by invasion of facial bones by
fibrous tissue growth, causes deformity of the affected regions. Virtually
no part of the facial skeleton is immune. Vision may be affected or even
lost by encroachment on the optic nerve. Exophthalmos (bulging of the eye)
may be caused by the displacement of orbital contents by the affected
bones. Dental occlusion may be totally lost and gross deformity may occur
in the area of the jaws when they are invaded.
Again, treatment consists
in resection of as much affected bone as possible without sacrificing
vital structures. Reconstruction is generally carried out at the same
time, using bone grafts or, depending on the surgeon's preference,
synthetic biomaterials.
Last but not least,
dysthyroid exophtahlmos caused by Graves disease of the thyroid deserves a
special mention, for it is not rare, it is very distressing from the point
of view of symptoms, and treatment by conventional methods offers little
relief and is fraught with an alarming rate of complications. The
extraocular muscles (the muscles that move the eye) of patients suffering from thyroid disease very
frequently become enlarged (this is caused by an autoimmune process).
Other orbital tissues also become involved in the process. As a result of
the increased volume of the orbital soft tissues, the eyes bulge
abnormally, causing not only an unsightly appearance, but also pain and
intense eye irritation. In advanced cases vision may be in jeopardy.
Conventionally a "decompression" is carried out, by removing one
or more of the orbital walls. This approach offers only a small degree of
improvement and frequently causes distressing complications such as double
vision and eyelid problems. Reducing the eye projection by more than 4-5
mm requires an enlargement of the orbit which can only be achieved
through craniofacial techniques in a safe, controlled fashion.
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Reference Accounts
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