The Fibrous dysplasia

Source:  The Fibrous dysplasia    Tag:  fibrous dysplasia prognosis

Fibrous dysplasia




CLINICAL CASE
A young patient of 11 years, of Bulgarian origin, has
swelling of the right hemi-maxillary, evolving
for about 6 years (Fig. 1 and 2). This boy, who
also presents no particular history, not
reported no pain associated with swelling. Biology
is normal. A scan showed a massive facial
lesion of the right half-jaw, extending about
bony framework of the maxillary sinus and corresponding
probably fibrous dysplasia (Fig. 3 and 4). One
biopsy of the mass confirmed the diagnosis histologically.
Given the young age of the patient, the nature of Benin
tumor and its slow evolution, no treatment
is proposed, with a semi-annual monitoring. One year
later, there was a significant change with
doubling the size of the lesion, a deviation of the center
Inter-incisal and occlusal major disturbances. The scanner
with 3D reconstruction confirms this trend with
moderate effect mass in orbit.
===================
RESPONSE
Fibrous dysplasia or disease Jaffe-Lichtenstein
a clinical entity described for the first time in 1891
by von Recklinghausen. The ostitis fibrosa then appoints a
bone lesion in which bone marrow is replaced
by fibrous tissue. In 1937, Albright et al. speak
of ostitis fibrosa generalisata to name the association
polyostotic bone deficiencies, endocrine disorders
sex and skin pigmentation. In 1938
Lichtenstein defines this clinical association under the term
of "polyostotic fibrous dysplasia".
Fibrous dysplasia is a bone tumor (some
However, the authors consider a condition
etiology of malformation), rare (2.5% of tumors
bone and 7% of benign bone tumors) [1, 2]
which localizes preferentially in the long bones
(Metaphysis and diaphysis), pelvis, shoulder bones
the face and skull. The lesion consists of fibrous dysplasia
in the replacement of bone tissue by tissue
fibrous [3].
There are 3 types of dysplasia: monostotic form,
the most frequent (70%), the polyostotic form,
more aggressive and McCune Albright and more
rare and combines polyostotic dysplasia,
cutaneous manifestations (café au lait spots) and
endocrine disorders (precocious puberty, accelerated growth,
goitre and hyperparathyroidism ...).
Clinically bone deformities observed
sometimes responsible for injuries or even aesthetic
fractures. Achieving the craniofacial area deserves
special attention because of the possible damage
noble structures that consist of: optic nerve, organ
of hearing disorders occlusion, respiratory disorders
reached by the nasal cavity, reaching the base of the
Skull [5] ...
The prognosis of dysplasia is usually
good due to the stabilizing trend in
prohibiting bone growth. There is a very low
percentage of malignant transformation (0.5 to 4%) in
osteosarcoma, chondrosarcoma or fibrosarcoma, and
appears that 50% of patients with conversion
Malignant received radiotherapy in
early treatment of dysplasia.
To decide the proposed treatment to the patient, the practitioner
will assess the severity of the disease taking
account possible complications (endocrine, neurological,
trauma ...), the radiological appearance
lesions, increased alkaline phosphatase
and bone density which reflects the efficiency of a possible
prior drug treatment.
The treatment may be medical and / or surgical.
From a medical point of view, the administration of bisphosphonate
has proven effective in reducing
pain intensity and biochemical markers
related to bone turnover, and some reduction in
osteolytic sites radiography (in 50% of
patients). This treatment is, however, little used
growth in children due to the lack of experience
clinical about it.
Surgical treatment, meanwhile, will most often
Conservative (osteotomy Sculpting), proving
satisfactory from an aesthetic point of view and sufficient when
lesions progress slowly and do not threaten
important anatomical structures. This is the attitude
therapeutic most widely proposed in the literature
for this disease.
However, some authors recommend resection
wide, curative, but officials deficits
major aesthetic and not always followed by remissions
complete.
The patient presented received surgery
consisting of a modeling osteotomy which
proved very satisfactory both aesthetically
and functional.