CASE REPORT

FIBROUS DYSPLASIA OF SINONASAL ON CHILD

Hanski R.P Legrans

Scientific Supervisor

Dr. I Gde Ardika Nuaba,Sp.THT-KL(K) 1

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 Gsα gene located on chromosome 20q13.2-13.3

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

4

Clinical Features

Depends on the size, duration and extension of the natural

course of the disease.

Localized swelling and mild with or without pain until the derformity occured with complications such as proptosis, visual

disturbances, and sensorineural deafness

INTRODUCTION

5

• History,clinical examination,radiology, histopathology

Diagnosis

• Surgery:place & location,growth rate,aesthetic,function impairment,surgeon skill

• Medical: role of steroid & biphosponate

Management

INTRODUCTION

6

Reported a case of fibrous dysplasia of the

sinonasal on a child which performed tumour

resection with lateral rhinotomy approaching

LITERATURE REVIEW

7

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

9

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

ETIOLOGY

10

Unclear etiology but associated with mutation of gene GNAS I coding the

subunit α from Gs-α

Increased the production c-AMP affect proliferation & differentiation osteoblast

First identification on Mc Cune-Albright syndromeone form of fibrous dysplasia(polyostotic monoostotic)

PATHOGENESIS

11

Bone formation (endochondral & intramembran)

Long bones (endochondral)

Flat bones (intramembran)

Blood vesselcental

bone (absorption of

cartilage & bone

formation)

condensation of mesechymal cell

in stroma fibrousosteoblat immatureextrac

ellular matrix

PATHOGENESIS

12

FIBROUS

DYSPLASIA

Mutation after zygotic,the

bond of membrane to G-

protein coupled receptor

abnormal osteoblast

differentation abnormal

bone(immature)

Increasing IL-6activation

of osteoclastextent of

lession

CLINICAL FEATURES

13

Can be mild to severe

Local swelling with/without pain,

asymetrical face,deformity

Blocking nose, skin lession(café

au lait)

CHLEAC constriction

SNHLobstrucion of inner ear

structure,otic capsule & IAC

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

15

Diagnosis estabilished:

history, physical

examination,

radiology,histopathology

A

B A. Homogenous Lession B. Ground Glass

Appearence

DIAGNOSIS

16

A

CT-Scan

Better to asses tumor

expansion

Not enough to estabilsh diagnosis

Ground glass 56%,

homogenous lession 23%, cystic pattern

21%

Radiology

DIAGNOSIS

17

A Histopahology

Trabeculae of woven bone without osteoblast rimming

Osteoclast absorption that

contribute to the irregular shape of

the trabeculae

DIFFERENTIAL DIAGNOSIS

Fibrous osseus, Paget’s dissease, aneurismal osseus cyst, cystic Cristeller syndrome, amelobastoma, osteochondroma hypothyroid, malicnancy such as sarcoma, osteoblast metastatic lession

18

MANAGEMENT

19

Surgery & Medication

Surgery

invoving area,tumor growth rate, aesthetic, functional impairment, patient condition,skill

& multidiciplin availbility

Surgical Approaching: lateral rhinotomy, midfacial degloving

Medication

•Steroiddecompression •Biphosponate inhibits

osteoclast activity

PROGNOSIS

20

Better though poor outcome on younger patient or polyostotic

form

Frame time diagnosis to malignancy is 13,5

years

Risk of the pateint who didn’t receive

therapy: 0,4%

CASE REPORT

21

November 11th ,2010

Patient PJ, male

12 years old,Selong

East Lombok

reffered to ENT

clinic Sanglah

hospital with

diagnosis Suspect

Fibrous dysplasia

CASE REPORT

22

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

CASE REPORT

23

Patient went to Mataram hospital on October 28th ,2010 and performed CT-scanlytic

lession resemble of ground glass,can be a fibrous dysplasia

CASE REPORT

24

Physical examination

Compos mentis, vital

sign within normal limit

ENT examination: a Lump on left cheek with size

15cm x 10cm,skin lession café au lait,deformity,

tumor on left nasal cavity,with mucoid

discharge

CASE REPORT

25

Working diagnosis:

susp.fibrous dyplasia

Planned for left lateral rhinotomy

Thorax Rontgen, blood test & hemostasis

function

November 13th ,2010

pediatrician & anestesiology

Scheduled for resection with rhinotomy lateral approaching on

Novenber 22nd ,2010

CASE REPORT

26

November 22nd ,2010 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

CASE REPORT

27

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

Post Operation Follow Up

CASE REPORT

28

Post Operation Follow Up

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

CASE REPORT

29

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 monthsCT-

scan

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 Gsα gen

mutation located on chromosom 20q13.2-13.3 30

DISCUSSION

31

Literature

Case

Ratio of gender equal Younger age male Cholakova et al

range of age 5-67 year old

Male 12 years old

Monostotic>polyostotic Most common site of monostotic fibrous dysplasia(Lee et al):regio zygoma-maxilla

Fibrous dyplasia type monostotic On Sinus maxillaris

DISCUSSION

32

Literature

Case

Clinical symptoms: deformity, asymetrical face, pain, nasal obstruction, skin lession café 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, café au lait,tumor fullfil the left nasal cavity, mucoid discharge

DISCUSSION

33

Diagnosis of fibrous dyplasia

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

DISCUSSION

34

Literature

Case

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

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

DISCUSSION

35

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)

DISCUSSION

36

Literature

Case

Hullar & Lustig: regular control for patients who underwent surgery DiCaprio & Enneking: CT-scan prefeably every 6 months to determine the reccurence

Patient was advised for follow up on the next 6 month to performed CT-Scan

CONCLUSION

37

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