CASE REPORT

FIBROUS DYSPLASIA
OF SINONASAL ON
CHILD
Hanski R.P Legrans

Scientific Supervisor
Dr. I Gde Ardika Nuaba,Sp.THT-KL(K)

INTRODUCTION
First

described by Lichtenstein in1938

Characterized

by progressive replacement
of normal bone elements by fibrous tissue

Even

benignpotential to cause functional

& cosmetical damagecraniofacial bones

Most

common site:Maxilla,mandible,
frontal,sphenoid, & temporal bone
2

INTRODUCTION
The

etiology is unknown, but is

associated with mutations in the Gs
gene located on chromosome 20q13.213.3

First

identified in patients with the

syndrome McCune - Albright, but later
occured in one form of fibrous dysplasia
which are monostotic or polyostotic
3

INTRODUCTION

Depends on the size, duration

and extension of the natural
course of the disease.

Clinical
Features

Localized swelling and mild with or without

pain until the derformity occured with
complications such as proptosis, visual
disturbances, and sensorineural deafness

INTRODUCTION

Diagnosis

Management

History,clinical
examination,radiology,
histopathology

Surgery:place &
location,growth
rate,aesthetic,function
impairment,surgeon
skill
Medical: role of steroid
& biphosponate

INTRODUCTION

Reported a case of
fibrous dysplasia of the
sinonasal on a child
which performed tumour
resection with lateral
rhinotomy approaching

LITERATURE REVIEW

Anatomy of sinus
paranasal

Four pairs of paranasal

sinuses is the maxillary
sinus, frontal sinus,
ethmoid sinus and
sphenoid sinuses right
and left

Clinically, paranasal
sinuses were divided
into 2 groups: the
anterior and posterior

LITERATURE REVIEW
Maxillary

sinus:Largest sinus, irregular inverted

pyramid,ostium is placed on superior of
maxillary sinus wall
Frontal Sinus:Located in os frontal,insulated
and notched in the edge
Ethmoid Sinus: based on locationetmoid
anterior & posterior Pyramid shaped with basic
on posterior
Sphenoid sinus:Located in os sphenoid,In
developmentblood vessels & nerves are very
close to the cavity
8

EPIDEMIOLOGY

Certain
prevalence is
unknown

Equal
between male
& female

Younger age
often in male
than female

Jaw: often in
maxilla than
mandible

can occur on
femur, skull &
costae

Unclear etiology but

associated with mutation
of gene GNAS I coding the
subunit from Gs-

ETIOLOGY

Increased the production

c-AMP affect proliferation &
differentiation osteoblast
First identification on Mc CuneAlbright syndromeone form of
fibrous dysplasia(polyostotic
monoostotic)

10

PATHOGENESIS
Bone formation (endochondral
& intramembran)
Long bones
(endochondral)
Blood
vesselcental
bone
(absorption of
cartilage &
bone
formation)

Flat bones
(intramembran)
condensation of
mesechymal cell
in stroma
fibrousosteoblat
immatureextrac
ellular matrix

11

PATHOGENESIS
Mutation after zygotic,the
bond of membrane to Gprotein coupled receptor

FIBROUS
DYSPLASIA

abnormal osteoblast
differentation abnormal
bone(immature)

Increasing IL-6activation
of osteoclastextent of
lession

12

CLINICAL FEATURES
Can be mild to
severe

Local swelling
with/without pain,
asymetrical
face,deformity

Blocking nose,
skin lession(caf
au lait)

CHLEAC
constriction

SNHLobstrucion
of inner ear
structure,otic
capsule & IAC

13

CLINICAL FEATURES
Clinical

symptomslocation, onset,
expansion & the course of the disease
Each sinus has its own clinical symptoms
Ethmoid & sphenoid sinuseyeball
displacement, decreased of visual due to
compression of fibrous dysplasia in optic
nerve
Maxilla sinusfullness & swelling to the
cheeks & oral cavity
14

DIAGNOSIS
A

Diagnosis estabilished:
history, physical
examination,
radiology,histopathology

A. Homogenous Lession
B. Ground Glass
Appearence
15

DIAGNOSIS
Radiology
A
Not enough to
estabilsh
diagnosis
Ground glass
56%,
homogenous
lession 23%,
cystic pattern
21%

Better to asses
tumor
expansion

CT-Scan
16

A Histopahology

DIAGNOSIS

Trabeculae of
woven bone without
osteoblast rimming
Osteoclast
absorption that
contribute to the
irregular shape of
the trabeculae
17

DIFFERENTIAL DIAGNOSIS
Fibrous

osseus, Pagets dissease,

aneurismal osseus cyst, cystic
Cristeller syndrome,
amelobastoma, osteochondroma
hypothyroid, malicnancy such as
sarcoma, osteoblast metastatic
lession
18

MANAGEMENT
Surgery & Medication
Surgery
invoving area,tumor
growth rate, aesthetic,
functional impairment,
patient condition,skill
& multidiciplin
availbility

Medication

Surgical
Approaching:
lateral rhinotomy,
midfacial degloving

Steroiddecompression
Biphosponate inhibits
osteoclast activity
19

PROGNOSIS
Better though poor
outcome on younger
patient or polyostotic
form
Frame time diagnosis
to malignancy is 13,5
years

Risk of the pateint

who didnt receive
therapy: 0,4%
20

CASE REPORT
November 11th ,2010

Patient PJ, male

12 years old,Selong
East Lombok
reffered to ENT
clinic Sanglah
hospital with
diagnosis Suspect
Fibrous dysplasia
21

CASE REPORT

Chief
Complaint

Lump in left cheek since 5

years ago

History of
disease

Initially small and

growing, sometimes
accompanied by pain. In
addition nasal
congestion complaint on
left nose, sometimes
with discharge mixed
with blood. No lumps
elsewhere,no vision
complaint

22

CASE REPORT

Patient went to
Mataram hospital
on October 28th
,2010 and
performed CTscanlytic
lession resemble
of ground
glass,can be a
fibrous dysplasia
23

CASE REPORT
Physical
examination

ENT examination:
a Lump on left
cheek with size
15cm x 10cm,skin
lession caf au
lait,deformity,
tumor on left nasal
cavity,with mucoid
discharge

Compos mentis, vital

sign within normal limit

24

CASE REPORT
Working
diagnosis:
susp.fibrous
dyplasia

November 13th
,2010
pediatrician &
anestesiology

Planned for
left lateral
rhinotomy

Thorax Rontgen,
blood test &
hemostasis
function

Scheduled for resection with

rhinotomy lateral approaching on
Novenber 22nd ,2010

25

November

22nd

,2010

CASE REPORT

Performed resection of tumor with lateral

rhinotomy approach
Operation finding irregular mass in left nasal
cavityperformed histopathology examination
Post operation medicationceftriaxon
2x500mg i.v, tranexamat acid 2x250mg i.v,
dexamethasone 2x0,5mg oral, paracetamol
3x500mg oral

26

C
ASE REPORT
Post Operation Follow Up
1st day:November 23rd
2010
No complaint, swelling
on left cheek, packing
on left nasal cavity, no
active bleeding on
posterior wall of
pharyng
Incision wound care
Continuing
medication theraphy

2nd day:November
24th 2010
No complaint, swelling
on the left cheek,
remove the anterior &
sinus packing, no
active bleeding
Patient discharged with
medication cefadroxil
2x500mg oral,
paracetamol 3x500mg,&
dexamethasone 3x05mg
27

Post Operation Follow Up

CASE REPORT

5th day: November

27th ,2010
No complaint, swelling
on the left cheek, crust
on left nasal cavity
Wound operation care,
nasal toilet
Continuing
medicationtherapy
Histopathology
examinationfibrous
dyplasia
28

CASE REPORT

Post Operation Follow Up

7th Day: November 29th 2010
No complaint, swelling on left
cheek, no discharge & crust
Suture removal & wound care
Follow up within 6 monthsCTscan
29

DISCUSSION
Fibrous dysplasia
Progressive

replacement of normal bone

elements by fibrous tissue

Benign

but potentially cause functional damage

Monostotic

lession & polyostotic lession

Unknown

etiology, but related to the Gs gen

mutation located on chromosom 20q13.2-13.3
30

DISCUSSION

Literature
Ratio of gender equal
Younger age male
Cholakova et al
range of age 5-67 year
old
Monostotic>polyostotic
Most common site of
monostotic fibrous
dysplasia(Lee et
al):regio zygomamaxilla

Case
Male 12 years old

Fibrous dyplasia type

monostotic
On Sinus maxillaris

31

DISCUSSION
Literature

Case

Clinical symptoms:
deformity, asymetrical
face, pain, nasal
obstruction, skin lession
caf au lait

Patient complaint of
sweeling on left cheek
which since 5 years ago,
pain(+), nasal
obstruction(+), mucoid
disharge mixed with
blood
Examination:asymetrical
face, deformity, caf au
lait,tumor fullfil the left
nasal cavity, mucoid
discharge

32

Diagnosis of fibrous dyplasia

DISCUSSION

Lee et al

Anamnesis & clinical

examinationto determine
whether it is monostotic or
polyostotic

Chan et al

Difficult to estabilished the

diagnosis solely based on
anamnesis or physical
examination or radiology

Tsai et al

Usually asymptomatic until

the tumor compress the
adjacent structure &
presents symptoms such
pain & proptosis

33

DISCUSSION
Literature
Histopathology: fibrous
tissue with spindle cell
shape & do not mature,
the presence of
trabecular-isolated
trabeculae of woven bone
without rimming
osteoblasts

Case
proliferation of benign
fibroblast, monotonous
spindle core, fine
chromatin, extensive
eosinophilic cytoplasm
forming files, immersed
in a collagen matrix.
Among fibrous tissue
appeared the lamellar
bones & calcification
without osteoblast
rimming

34

DISCUSSION
Literature

Case

CT-scan: 3 variations
description: ground glass
(56%), homogenous
(23%) and cyctic (21%)

CT-scan: tumor mass

with ground glass
appearence on the left
maxillary sinus pushes
the septum to the right

Management:Surgery
(lateral rhinotomy,
midfacial degloving,
FESS) &
medication(biphosponate
,NSAID & steroid)

Surgery: rhinotomy
lateralis
Medication:corticosteroid
(dexamethasone
2x0,5mg)
35

DISCUSSION
Literature

Case

Hullar & Lustig: regular

control for patients who
underwent surgery

Patient was advised for

follow up on the next 6
month to performed CTScan

DiCaprio & Enneking:

CT-scan prefeably every
6 months to determine
the reccurence

36

CONCLUSION
Reported a case of fibrous
dysplasia on the left sinonasal
which performed tumor resection
with lateral rhinotomy approach
Replacement of the normal bone
elements with fibrous tissue
Unknown etiology,often
associated with mutation gene
Variety of clinical symptoms
Diagnosis esatbilished:
anamnesis, physical examination,
supporting examination(CT-Scan)
Management: Surgery &
medication

37