Download Assist. Prof. Dr. Mihaela Cristiana Tatu,MD, PhD, ENT Specialist , Dr

FIBROUS DYSPLASIA OF THE TEMPORAL BONE
Assist. Prof. Dr. Mihaela Cristiana Tatu,MD, PhD, ENT Specialist , Dr. Oana Irina Popa, MD, ENT Resident
Central University Military Emergency Hospital Bucharest, ENT Clinic
Introduction
Fibrous dysplasia is a rare disease which remains unobserved for a long time - discovered
incidentally when bone fracture or neurological
symptoms due to compression appear.
Fibrous dysplasia is a benign, localized,
clearly demarcated, slowly progressive bone tissue disease, characterized by the replacement of
normal bone with an amorphous mass dominated
by fusiforme cells and bone trabeculae with variable size and random, disorganized distribution.
Clinical examination shows asymmetry,
pain and includes neurological examination
(compression syndromes), neurosurgical, endocrinological and ENT examination.
Investigations: biological (K, Ca, serum alkaline phosphatase levels growth); imaging: Rx, CT,
MRI, bone scintigraphy; bone biopsy.
Postoperative imaging:
On clinical examination: marked hypertrophy of the right temporal bone that determines
cranio-facial asymmetry; on the right external auditory meatus - antero-superior, spherical, sessile
bone protrusion, completely blocking the EAC,
covered with skin of normal appearance; mixed
moderate right hearing loss; right purulent, fetid
discharge; mastoid tenderness; left ear normal
clinically and functional.
Surgical intervention: recalibration of right
EAC with biopsy from the bone protrusion.
mastoidectomy with removal of the secondary
cholesteatoma (caused by EAC obstruction). The
bone biopsy showed fibrous displasya of the right
temporal bone.
Head CT scan
Case Presentation
We present the case of a young female patient,
32 years of age, with right temporal lobe compression.
The patient presented craniofacial asymmetry
marked by bone hypertrophy on the right side;
right mixed moderate hearing loss; presence of a
right EAC bone obstruction, purulent fetid otorrhea, right ear pain.
Hystory: at age 9 - right acute otitis media,
difficult examination - "Clinical onset" of the disease; age 11 - Clinical and radiological diagnosis
right EAC osteoma; surgery for otomastoid osteoma- biopsy: osteoma; age 28 – head CT scan "right temporal giant osteoma" (patient refused
any surgery).
Presumptive diagnosis: fibrous dysplasia of
the right temporal bone complicated by induced
otomastoid colesteatoma.
Differential diagnosis was made with nonosteogenic fibroma; giant cell bone tumor;
bone aneurismal cyst; bone adamantinoma;
eosinophilic granuloma; plasmacytic myeloma.
Investigations: hormone titers in the normal range; profile skull X-ray to "sella turcica" normal size; without major changes of calcemia,
magnesemia and alkaline phosphatase; head CT
scan; bone scintigraphy; neurological exam: control EEG, complete cranial nerves exam; IQ testing; neurosurgical exam - forming a mixed ENTneurosurgical team.
Postoperatory it is important to survey the patient because the disease is evolving and progressing and complications (even life threatening )
may appear:
● restenosis of the cavity after reconstruction,
caused by development of concentric bone;
● induced cholesteatoma and its complications;
● progressing hearing loss ;
● stenosis of EAC with its repercussions;
● intracranial extension (ICH and compression
threatening events);
● sarcomatous degeneration (0.5% of cases of
temporal bone dysplasia)
Head MRI
Conclusions
Surgery is essential for positive diagnosis and
for complications treatment.
FD is rarely diagnosed. The monosteotic form
of the temporal bone is glade.
References
Gregg J, Reed A. Monostotic fibrous dysplasia in the temporal bone: a late prehistoric occurrence. Am J Phys Anthropol. 1980
Nager G. Fibrous dysplasia. In: Pathology of the Ear and Temporal Bone. Baltimore, Md: Williams & Wilkins; 1993
Lambert PR, Brackmann DE. Fibrous dysplasia of the temporal bone: the use
of computerized tomography. Otolaryngol Head Neck Surg. 1984