PEDIATRIC/CRANIOFACIAL
Craniomaxillofacial Fibrous Dysplasia:
Conservative Treatment or Radical Surgery?
A Retrospective Study on 68 Patients
Valentino Valentini, M.D.S.,
Ph.D.
Andrea Cassoni, M.D.S.
Tito Matteo Marianetti, M.D.
Valentina Terenzi, M.D.
Maria Teresa Fadda, D.M.D.,
M.D.S.
Giorgio Iannetti, M.D.S.,
Ph.D.
Rome, Italy
Background: Fibrous dysplasia is a benign bone lesion characterized by replacement of normal bone with fibro-osseous connective tissue. The surgical
treatment of fibrous dysplasia is based on one of two different approaches,
conservative or radical.
Methods: From January of 1980 to December of 2002, 95 patients with fibrous
dysplasia located in the craniomaxillofacial area presented to the authors’
department. Twenty-one had the polyostotic type (22 percent), two had McCuneAlbright syndrome (2 percent), and 72 had the monostotic type (76 percent).
Of 95 patients, 68 underwent surgery; the remaining 27 refused. Among the 68
patients who underwent surgery, 61 had radical excision, six received conservative treatment, and a patient with mandibular involvement received radical
excision and immediate reconstruction with a free fibula flap after a recurrence
experienced 2 years after primary remodeling surgery
Results: No disease recurrence was observed in cases treated with complete
excision, whereas a case of mandibular involvement treated with remodeling
required further surgery. Infection, resolved with antibiotics, was seen in one
case, and palatal fistula was seen in two cases. In one case with cranial base
involvement liquorrhea was observed.
Conclusions: In most cases of monostotic or monofocal fibrous dysplasia of the
craniofacial region, modern surgical techniques allow an aggressive but definitive treatment with good functional and aesthetic results. The authors perform radical treatment even in cases involving the maxilla and mandible, and
prefer a conservative approach only in polyostotic cases and McCune-Albright
syndrome. (Plast. Reconstr. Surg. 123: 653, 2009.)
F
ibrous dysplasia is a benign bone lesion characterized by replacement of normal bone
with fibro-osseous connective tissue.1 Von
Recklinghausen,2 in 1891, was the first to describe
a pathologic condition of the bone characterized
by deformity and fibrotic changes, which he called
“osteitis fibrosa generalisata”; in 1938, Lichtenstein and Jaffe3 first introduced the term “fibrous
dysplasia.” They also noted that fibrous dysplasia
can be monostotic or polyostotic. In 1937,
McCune4 and Albright et al.5 recognized the relationship among the polyostotic form, precocious
From the Department of Maxillofacial Surgery, Policlinico
“Umberto I,” University of Rome “La Sapienza.”
Received for publication December 12, 2007; accepted August 20, 2008.
Copyright ©2009 by the American Society of Plastic Surgeons
DOI: 10.1097/PRS.0b013e318196bbbe
puberty, and areas of cutaneous pigmentation
(café au lait spots), an association now known as
McCune-Albright syndrome.
Even though fibrous dysplasia may affect any
bone in the body, craniofacial involvement occurs
in 50 to 100 percent of patients. It involves most
frequently, in descending order, the maxilla, mandible, frontal bones, sphenoidal bones, ethmoidal
bones, parietal bones, temporal bones, and occipital bones.6 Maxillary lesions often involve adjacent bones (the zygomatic, sphenoid, nasal, or
frontal bone), for which the term “monofocal” is
more indicated, when several adjacent skeletal
segments in a single area are affected.7
Disclosure: None of the authors has a financial
interest to disclose in relation to the content of this
article.
www.PRSJournal.com
653
Plastic and Reconstructive Surgery • February 2009
The etiology of the abnormal fibro-osseous
process has been clarified only relatively recently.
Marie et al.8 and Riminucci et al.9 showed that the
development of fibrous dysplasia reflects a mutation that alters the function of osteogenic cells.
Activating mutation of the Gs-a protein in osteoblastic cells is implicated in the development of
the lesions. The substitution of cysteine or histidine with arginine in position 201 determines the
loss of guanosine triphosphate activity with an increase of adenylate cyclase activity and consequent
increase in the proliferation and inappropriate
differentiation of cells, with excessive and disordered production of collagen. Yamamoto et al.10
investigated the role of interleukin-6 in the bone
lesions of patients affected by McCune-Albright
syndrome. They noted that cells cultured from
these lesions show increased interleukin-6 secretion, which determines an increase in the number
of osteoclasts. This may explain how pamidronate,
a potent osteoclast inhibitor, can produce increases in the density of the lesions and delay the
spread of the lesions into surrounding bones.11
Diagnosis of fibrous dysplasia is based on clinical, radiographic, and histological findings. Clinical examination can show an increase in the volume of bone affected, causing facial asymmetry.
The signs and symptoms depend on the area involved. The most common clinical sign is swelling
or deformity of the affected bone, but fibrous
dysplasia can present with nasal obstruction, sinusitis, hearing loss, headache, dystopia, diplopia,
dysesthesia, proptosis, and loss of vision.12 Radiological features can be cystic, sclerotic, or pagetoid, often with a typical “ground glass” appearance caused by the mixture of fibrous and osseous
elements. The radiographic density of the lesions
depends on the proportion of these elements.13
From a histological point of view, three main
types have been identified: (1) the “Chinese letters” model, (2) the “pagetoid” model, and (3) the
“hypercellular” model. Each of these is differentiated on the basis of the architecture and cellularity of the osseous tissue.9
The Chinese letters model is the most common. The bone trabeculae are thin and disconnected, with active osseous reabsorption by osteoclasts. The osteogenic cells are star-shaped and
numerous Sharpey fibers are present. The pagetoid model is similar to the bone tissue found in
Paget disease, with a dense and sclerotic trabecular tissue. The hypercellular model is characterized by the presence of discontinuous bone trabeculae distributed in an ordered and sometimes
parallel fashion.9
654
Therapy for fibrous dysplasia is surgical. Radiotherapy must be avoided, since it is ineffective and
increases the incidence of malignant degeneration.14 Surgical treatment may range from conservative therapy (curettage, contouring, or remodeling), which may eventually be repeated, to radical
excision followed by immediate reconstruction.1
To our knowledge, our series of patients is the
one of the largest presented in the literature and
can be useful for epidemiological purposes, as well
as for considering how our management of fibrous
dysplasia has been modified and improved with
the evolution of surgical techniques.
PATIENTS AND METHODS
From January of 1980 to December of 2002, 95
patients with fibrous dysplasia located in the craniomaxillofacial area presented to the Department
of Maxillofacial Surgery of the University of Rome
“La Sapienza.” The average patient age was 24.6
years (range, 4 to 52 years). Twenty-one patients
had the polyostotic type (22 percent), two had
McCune-Albright syndrome (2 percent), and 72
had the monostotic type (76 percent). (In this
study, forms affecting two contiguous segments of
bone were classified as monostotic, and thus
“monostotic” should be understood as meaning
“monofocal.”) The areas of involvement in these
patients are listed in Table 1. Of 95 patients, 68
underwent surgery; the remaining 27 did not because of patient refusal. Among the 68 patients
who underwent surgery, 61 had radical excision,
six received conservative treatment, and a patient
with mandibular involvement received radical excision and immediate reconstruction with a free
fibula flap after a recurrence experienced 2 years
after primary remodeling. Treatment procedures
are listed in Table 2. The reconstruction after
radical surgery was performed in all 62 patients
operated on, except for 11 patients who had simple removal. Reconstruction was achieved with
bone grafts in 21 patients (Figs. 1 through 8; donor
sites: iliac crest in 14 patients, calvaria in three, and
rib in four), alloplastic materials in three patients,
and local and pedicled flaps in 21 patients; a fibula
free flap was used to reconstruct the mandible and
maxilla in three cases each.
RESULTS
The patients were followed up with clinical
examinations, radiographic films, computed tomography scanning, and vision examinations
(Hess screen and visual evocated potentials in
cases of orbital involvement) for 5 to 15 years
(average, 7.6 years). All patients operated on
Volume 123, Number 2 • Craniomaxillofacial Fibrous Dysplasia
Table 1. Fibrous Dysplasia: Involved Areas (total no.
of patients ⴝ 95)
Involved Area
Mandible
Maxillary bone
Cheekbone
Maxillary ⫹ cheekbone
Ethmoid
Sphenoid
Frontal
Temporal
Parietal
Monostotic
Polyostotic
Total
19
23
2
13
5
2
2
1
0
2
6
0
3
10
12
8
8
2
21
29
2
16
15
14
10
9
2
between 1980 and 1991 received postoperative
radiographs for postoperative and long-term
follow-up control; since 1991, we have used computed tomography to follow our patients. In no
patient treated with complete excision was disease
recurrence observed, whereas a patient with mandibular involvement treated with remodeling required further surgery. In a patient treated with
bone graft reconstruction, infection was observed
and resolved with antibiotics; two patients with
maxillary involvement required a subsequent operative intervention under local anesthesia to
close a palatal fistula. Liquorrhea was observed in
one patient with cranial base involvement, so reconstruction with a galea flap was performed. In
the other patients, acceptable or good aesthetic
and functional results were observed. All patients
who underwent reconstruction with free flaps or
bone grafts received adequate implant or prosthetic rehabilitation (Figs. 9 through 16).
Optic nerve decompression was performed in
four cases: three patients were symptomatic (visual
loss, exophthalmus), whereas in one patient massive ethmoidal involvement led to a radical resection with decompression of the optic canal on his
Table 2. Treatment Procedures Performed in
Involved Areas (total no. of patients ⴝ 68)
Surgical Procedure
Excision and
reconstruction
Conservative shaving
Optic nerve
decompression
Involved Area
Total
Mandible
Maxillary ⫹ cheekbone
Ethmoid
Sphenoid
Frontal
Temporal
Parietal
Mandible
Maxillary ⫹ cheekbone
Frontal
McCune-Albright syndrome
Temporal
17*
32
8
3
3
2
1
3*
3
1
1
1
4
*In one patient, primary remodeling and subsequent complete excision were performed because of disease recurrence.
medial side. In all cases, resolution of exophtalmus was observed. In a patient with previous amaurosis, visual recovery was not obtained, whereas
in the other two symptomatic patients improved
visual acuity (2 degrees) was observed.
DISCUSSION
Fibrous dysplasia is a benign pathological
bone disorder, but it can have a devastating outcome with high recurrence rates, especially after
partial resections. Some authors1,15,16 report that
the pathology does not progress after puberty;
however, others13,17 have found that its progression is continuous, even during adulthood.
The aim of the treatment of fibrous dysplasia
is to correct or prevent functional problems and
to achieve an aesthetic improvement. No medical
treatment allows the arrest of the progression of
the illness. Biphosphonates, which inhibit osteoclastic activity, can alleviate pain and improve the
radiological appearance; steroids are currently
used as a support therapy before and after surgical
decompression of the optic nerve, but they cannot
definitively halt the progression of the illness.12,14
The surgical treatment of fibrous dysplasia is
based on two different approaches, conservative
or radical. Conservative shaving or osseous contouring has been recommended by some
authors18 who maintained that periodic contouring could be performed until a static phase was
reached, even if continued growth was observed
after conservative treatment. However, interventions have become more aggressive as advances
have been made in surgical techniques19 and nowadays most authors13,14,17,19 are in favor of radical
surgical therapy, which permits the complete removal of the lesion followed by immediate reconstruction. We adopted conservative techniques in
only six old cases; in most cases, radical excision
was performed, and according to advances in surgical techniques, in the last cases reconstruction
with free flaps was performed.
Chen and Noordhoff20 and Ricalde and
Horswell12 divided the craniofacial skeleton into
four zones on the basis of surgical, aesthetic,
and functional considerations. They adopted conservative treatment for the alveolar part of the
maxilla and mandible and for the cranial base,
and the radical approach for the fronto-orbital
area and the maxillozygomatic complex. Ozek et
al.1 stated that orbital hypertelorism, dystopia, or
proptosis can only be corrected by radical excision
and reconstruction, but they prefer the conservative approach for the alveolar part of the maxilla
and the mandible in an attempt to preserve alve-
655
Plastic and Reconstructive Surgery • February 2009
has a positive psychological effect for the patient.
In fact, no relapse has been observed in our patients who received radical excision with immediate reconstruction, whereas a patient who underwent remodeling of the displastic bone required
further surgical treatment. Posnick7 also states
that the treatment of choice should be radical as
it allows for the complete resolution of the illness
with immediate functional and aesthetic recovery.
Furthermore, Kreutziger15 affirms that conservative treatment has no curative potential but only a
palliative aesthetic aim and is unable to eliminate
the lesions. Zenn and Zuniga,17 in considering the
numerous relapses (25 to 50 percent) following
Fig. 1. Case 1. Preoperative frontal view of a patient with fibrous
dysplasia involving the left maxilla and zygoma.
Fig. 3. Case 1. Preoperative computed tomography scan illustrating the maxillary and malar involvement.
Fig. 2. Case 1. Preoperative axial view.
olar ridges and tooth. In accordance with
Posnick7, Kreutziger15 and Zenn and Zuniga,17 we
perform radical treatment even in cases involving
the maxilla and the mandible and use the conservative approach only in polyostotic cases and
cases involving McCune-Albright syndrome. Iliac
crest, rib, and calvaria grafts or revascularized free
flaps allow immediate reconstruction of the surgical defect with good anatomical and functional
recovery. In our experience, the radical approach
prevents the occurrence of relapses and is the
unique option for eliminating the illness; it also
656
Fig. 4. Case 1. Intraoperative view of reconstruction of the inferior orbital margin with calvaria.
Volume 123, Number 2 • Craniomaxillofacial Fibrous Dysplasia
which can be gradually revitalized by the invasion
of normal bone. This procedure can maintain the
original anatomy and eliminate donor-site morbidity derived from the grafting of large amounts
of autogenous bone. Surgical reconstruction can
be obtained with revascularized free flaps as well.22
In six of our patients, surgical reconstruction was
performed with free fibula bone graft, with good
functional and aesthetic results. Most authors today are in favor of radical excision and immediate
reconstruction,7,12,15,17,20 but the most controversial points are the management of fibrous dysplasia involving alveolar bone and cases in which the
optic nerve canal is involved, particularly in pa-
Fig. 5. Case 1. Postoperative frontal view.
Fig. 7. Case 1. Postoperative computed tomography scan
(axial view).
Fig. 6. Case 1. Postoperative axial view.
conservative treatment, are in favor of radical
treatment in mandibular fibrous dysplasia.
Reconstruction after resection of the involved craniofacial bone is usually performed
immediately.19 The goals are to prevent functional
problems and to restore symmetry, volume, and
contour. Some authors14,21 remove, thin, remodel,
and reimplant fibrous dysplastic bone grafts for
the reconstruction of surgical defects, with the aim
of offering an excellent support for the migration
of osteoblasts from surrounding normal bone.
Other authors12,13 suggest reconstruction with reimplantation of resected bone after autoclaving,
Fig. 8. Case 1. Postoperative three-dimensional computed tomography scan showing the maxillary alveolar ridge reconstructed with an iliac crest bone graft and the inferior orbital margin with calvaria.
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Plastic and Reconstructive Surgery • February 2009
Fig. 11. Case 2. Preoperative computed tomography scan
showing maxillary involvement.
Fig. 9. Case 2. Preoperative frontal view of a patient with fibrous
dysplasia involving the right maxilla.
observe the progression of the illness, but in cases
of massive bone involvement or functional impairment, we prefer to perform surgery, trying to
be as conservative as possible to prevent growth
alteration. With regard to optic nerve decompression, some authors16 maintain that prophylactic
decompression is necessary even in the absence of
symptoms, taking into account the relative rapidity
of vision deterioration when radiographic involvement of the nerve canal is present. However, other
authors23 consider it better to observe the progression of the illness rather than perform an early
prophylactic decompression, because of postop-
Fig. 10. Case 2. Preoperative intraoral view showing maxillary swelling.
tients with normal vision. Chen and Noordhoff 20
and Ricalde and Horswell12 adopted conservative
treatment for the alveolar part of the maxilla and
the mandible. We think there is no need to keep
teeth and alveolar crests when involved with fibrous dysplasia, because we have excellent surgical
possibilities for reconstructing alveolar bone
with revascularized or free bone grafts and performing implant-prosthetic rehabilitation. We will
never obtain the complete “restitutio ad integrum,” but the patient will be free from the illness.
In growing patients, we usually prefer to wait and
658
Fig. 12. Case 2. Intraoperative view of fixture positioning: reconstruction of the maxillary bone was performed with a doublebarred fibula free flap.
Volume 123, Number 2 • Craniomaxillofacial Fibrous Dysplasia
Fig. 15. Case 2. Postoperative computed tomography scan
(axial view).
Fig. 13. Case 2. Postoperative frontal view.
involvement of the optic canal should be monitored very closely with regular ophthalmologic examinations, including visual evoked potentials,
and that a therapeutic surgical decompression
should be performed only when an initial impairment of vision is present. Besides in cases of
optic nerve decompression, we prefer conservative treatment of fibrous dysplasia only in cases
involving the cranial base, polyostotic forms, and
McCune-Albright syndrome. On the other hand,
we can conclude that modern surgical techniques
allow, in the majority of cases of monostotic or
monofocal fibrous dysplasia of the craniofacial region, an aggressive but definitive treatment with
good functional and aesthetic results.
Fig. 14. Case 2. Postoperative intraoral view showing implantprosthetic rehabilitation.
erative risks and the lack of a close correlation
between computed tomographic images and effective vision impairment. In fact, it has been
demonstrated that despite restriction of the canal,
95 percent of patients have normal vision.23 Surgical decompression can cause loss of vision,
with optic nerve traction, thermal damage, postoperative edema, and bleeding the main suspected causes. Some authors prefer to use steroid
treatment in patients in whom the nerve canal is
affected, even though real benefits are only obtained in a few cases and are limited in time.12
We think patients with radiological signs of initial
Fig. 16. Case 2. Postoperative three-dimensional computed tomography scan showing reconstruction of the maxillary bone
with a double-barred fibula free flap.
659
Plastic and Reconstructive Surgery • February 2009
Valentina Terenzi, M.D.
Via Eugenio IV, no. 28
00167 Rome, Italy
[email protected]
10.
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