Facial asymmetry condylar hyperplasia and hemifacial microsomia

Sneha Gupta
JR III
Oral and Maxillofacial Surgery

• Perfect bilateral body symmetry is theoretic & seldom exists
• Clinically, symmetry means balance, whereas “asymmetry means
imbalance”

 Facial Asymmetry -“Clinically significant variation between
two halves of the face that the patient is concerned about and
which can be quantified by the clinician”

Congenital
Factors
• Cleft lip andpalate
• Tessier’s Cleft
• Hemifacial
Microsomia
• Neurofibromatosis
• Congenital muscular
torticollis
• Craniosynostosis
• Vascular disorders
• Treacher Collins Syndrome
• Hemifacial microsomia, others
Acquired Factors
• TMJ ankylosis
• Facial tumours
• Childhood Radiotherapy
• FacialTrauma
• Condylar Hyperplasia
• Parry Romberg
Syndrome
• Fibrous Dysplasia
• Others
Developmental Factors
• Unknown cause
( Cited From CheongYW,Lo LJ.FacialAsymmetry: Etiology,Evaluation & management Chang Gung
Med J.2011 Jul-Aug;34(4):341-51 )

• Progressive and Pathological overgrowth of either one or both
mandibular condyles
Epidemiology
• Age: 11-25 yrs (active form)
• Later in passive form
• F > M ; 64% of patients are women
( Acc. to a meta-analysis by Raijmakers et al, 2012)

HeredityNeoplasia
Trauma
Possible
Etiologies
Hormonal
influence
Infection
Abnormal
condylar
loading

 Obwegeser and Makek –
Classification based on Asymmetry
and Predominant Growth Factor
Type Clinical findings Radiological Findings
Type I
Hemimandibular
Elongation -HE
• Chin deviation towards contralateral
side
• Midline shift to contralateral side
• Possible posterior crossbite
• Excessive growth in the horizontal
vector
• Condyle often unaffected
• Elongated mandibularramus
• Misshapenand slender condylar
neck
Cited fromthe article of Obwegeser and Makek (JMaxillofac Surg 1986;14:183-208)

 The ipsilateral mandibular molars usually tip
to maintain occlusion.
 The horizontal form seems to be more
common than the vertical form.
 Increased functional load may cause
contralateral temporomandibular dysfunction
with associated pain and clicking.

Type II
Hemimandibular
Hyperplasia -
HH
• Sloping rima oris with minimal chin
deviation
• Supra eruption of maxillary molars
on affected side or open bite
• No midlineshift or minimal chin
deviation.
• Excessive growth in the vertical
vector, bowingof the body
• Enlarged and often irregularly
shaped condylar head
• Neck of condyle is thickened &
enlarged
 Obwegeser and Makek – Classification based on
Asymmetry and Predominant Growth Factor

 There is down-growth of the ipsilateral mandibular condyle.
 The entire hemimandible looks enlarged in three dimensions,from
ipsilateral condyle to symphysis.
 Initially, it causes an ipsilateral open bite, but gradual
compensatory growth of the maxillary and mandibular
dentoalveolar complexes results in an occlusal cant.
 Ipsilaterally, the mandibular body is bowed and the angle rounded;
contralaterally it looks flattened.
 The inferior alveolar bundle remains in its position close to the
lower border of the mandible because of overgrowth of the
dentoalveolar segment.
 The whole face appears rotated.

Type III
Combination of bothType
I and Type II
• Chin deviation towardscontralateral
side with a sloping rima oris
• Midline shift
• Possible open bite and/or cross
bite
• Excessive growth in vertical and
horizontal vectors
• Enlarged condylar head,neck
and ramus
• Irregularly shaped condylar
head,neck and/orramus
 Obwegeser and Makek –Classification based on Asymmetry and Predominant Growth
Factor
 The combined form presents with excess growth in both planes and clinical features of the
vertical and horizontal types

CH Age of
onset
Clinical Findings Imaging
Type
1A
Pubertal
growth
• Bilateral accelerated
symmetric growth
• Self-limiting ,can grow into
mid-20s
• Class III occlusion
• Prognathic mandible
• Radiograph -Bilateral
elongated condylar head, neck,
body
• Normal condylar head shape
• MRI :thin discs, asymmetric cases
may involve contralateral disc
displacement
 According to Wolford,Movahed and Perez
Cited fromWolford,et alClassification System for Condylar Hyperplasia.JOral Maxillofac Surg 2014

CH Age of
onset
Clinical Findings Imaging Histology
Type
1 B
Pubertal
growth
• Unilateral accelerated
asymmetric growth
• Self-limiting,can grow into
mid-20s
• Deviated mandibular
prognathism
• Ipsilateral class3 occlusion
and contralateralcross-bite
• Radiograph- Unilateral
elongated condylarhead,
neck ,body
• Normal condylar headshape
• Mandibular deviated
prognathism;
• MRI: thin disc ;may have
ipsilateral/contralateral disc
displacement
• Normally growing
condyle
• May showslight
widening of
fibrocartilageon
condyle or
• increased
vascularity in
proliferativezone.

A
Subarticular
Bone
Hyperplasia
Similarto
normally
growing
condyle
Figure A and B- Condylar hyperplasia type 1 may appear very similar to a
normally growing condyle without any signiﬁcant pathologic
abnormalities.
In some cases, the proliferative layer may exhibit an increased thickness
and some subarticular bone hyperplasia

Age of
onset
Clinical Findings Imaging
Type 2 2/3rd of
cases
begin in
second
decade
• Unilateral vertical elongation
of faceand jaws,
• Not self- limiting can grow
indefinitely
• Ipsilateral posterior
open bite
• Radiograph -Unilateral
vertically enlarged condylar
head ,neck, ramus,body
• Type 2A:vertical growth
factor, enlargement without
horizontal exophytic growth
ofcondyle

Cartilaginous
islands in
subcortical
bone
Increased
thickness of
Cartilaginous
cap Condylar hyperplasia type 2 shows a
cartilaginous cap may be similar to the
normally growing cartilage, or there can be
areas of increased thickness.
Endochondral ossiﬁcation and
cartilaginous islands in the subcortical
bone are seen

 Undifferentiated mesenchymal layer
 Hyperplastic cartilage layer
 Pathognomonic cartilage “islands” in the proximal bony trabeculae.
 These layers vary in thickness.
 Insulin-like growth factor 1 (IGF-1) has been implicated in the development of condylar
hyperplasia.
 High concentrations are found in the proliferating zone of hyperplastic condyles,and
chondrocytes cultured from such condyles express more than their normal counterparts.
 The addition of IGF-1 to normal cultured chondrocytes increases their proliferation.
Villanueva-Alcojol L, Monje F, González-García R. Hyperplasia of the mandibular condyle: clinical, histopathologic, and
treatment considerations in a series of 36 patients. J Oral Maxillofac Surg 2011;69:447–55.

Clinical Findings Imaging Histology
Type 2 • Unilateral vertical
elongation of faceand
jaws, caused by an
osteochondroma.
• Not self- limiting can
grow indefinitely
• Ipsilateral posterior
open bite
• Type 2B
• enlargement with
exophytic growth of
condyle
• MRI: ipsilateral disc
commonly in place
contralateral TMJ
arthritis ,displaced
disc in 75% of cases
Osteochondroma:
layer ofgerminating
undifferentiated
mesenchymal cells
hypertrophic cartilage,
islands ofchondrocytes
in subchondral
trabecular bone;
thickened and irregular
bony trabeculae
Cap of benign hyaline
cartilage

Age
of
onset
Clinical
Findings
Imaging Histology
Type 3 Unilateral Facial
Enlargement
•Caused by
benign tumor
• Osteomas,
Neurofibromas, Fibrous
dysplasia, Giant cell
tumor, Chondroma,
Chondroblastoma, etc
Type 4 Unilateral Facial
Enlargement
• Caused by
malignant tumor
• Chondrosarcoma,
Multiple myeloma,
Osteosarcoma, Ewing
sarcoma, Metastatic
lesions….

• Photographs, orthodontic models
• Radiographs ( OPG, Lateral cephalograms , Frontal Cephalograms )
• Lateral cephalometry is of limited value, as bilateral structures are
superimposed.
• CT scan including 3-D CT with coronal, axial and sagittal views
• Bone Scintigraphy (Nuclear imaging)- indicates increased cellular
activity
Planar
Scintigraphy
2D image
SPECT
3D
image
PET CT
( 3Dimage )

 Whether or not growth has stopped.
 CT and cone-beam CT cannot evaluate this.
 In single positron emission computed tomography (SPECT) examinations, a compound that has been
labelled with radioactive technetium ( 99mTc) and has an affinity for osteoblastic activity, is injected
intravenously, and the resulting radiation detected by a rotating gamma camera that allows 3
dimensional views of areas of increased bony turnover.
 SPECT was initially an adjunct to simple planar scintigraphy but is now used alone because of its
superior sensitivity and equivalent specificity. 88% - 100%)
 Difference of 10% in bilateral condylar uptake is diagnostic of active hyperplasia.( Sardin et al)
 SPECT however, cannot distinguish between condylar hyperplasia and inflammatory, infective,
neoplastic, or healing processes, the results should be correlated with clinical findings and anatomical
imaging.

18F – Fluoride PET CT
fusion scan
PET Scan CT scan only
SPECT CT Scan

• Grummon’s Analysis ( PA ceph ) – for Frontal discrepancies
• Digital Cephalometry ( Oris Ceph, Onyx Ceph, Pro Ceph, Dolphin)
• Stereolithographic models ( Mimics, Vitrea, Osirix,Mesh Mixer)
Digital Cephalometry Grummon’sAnalysisStereolithographic
models

Horizontal Planes
Mandibular morphology
Volumetric comparison
Maxillomandibular comparison of asymmetry
Linear asymmetry assessment
Maxillomandibular relation
Frontal vertical proportions.
Grummons and Kappeyne van de Coppello,JCO, 1987

Grummons and Kappeyne van de Coppello,JCO, 1987

Three-dimensional virtual reconstructions of the patient shown in . Preoperative planning to correct the maxillary cant: initial
position (top row) and planned final position (second row, right). Also, planning for mandibular surgery alone (after left
condylectomy one year earlier) with genioplasty and reduction of the lower border, calculated as 2.8 mm by mirroring the right
side (bottom row).

Objectives :
• ToEliminate Pathological processes
• Provide optimal functional and aesthetic outcomes
Condylar Hyperplasia Type 1
IfActive Growth
is present
Yes
High
Condylectomy
Wait until
growth is
complete
No Orthognathic
Surgery

 Option 1 - High Condylectomy
 Bilateral or Unilateral ( depending
upon Type 1A or 1B ) condylectomy
is performed
 4-5 mm of bone at the top of the
condylar head is removed including
medial and lateral pole areas
 Disc repositioning - using a suture
Rodrigues DB, Castro V.Condylar Hyperplasia of theTemporomandibular Joint.Oral and Maxillofacial Surgery
Clinics of North America. 2015 Feb;27(1):155–67.

Option 2 – Wait till growth is complete
 Till early mid-20’s
 Orthognathic surgery is performed
• Disadvantages
1) Worsening of facial deformity, asymmetry and occlusion since abnormal growth is allowed to proceed
2) Ipsilateral excessive soft tissue development
3) Adverse effects on mastication, speech & psychological development

Hussain A, Myuran T, Bentley R. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2016-
215215

 Orthognathic Surgery which may include either
to optimize the functional and aesthetics outcomes
 Other ancillary procedures like genioplasty ,
augmentation with facial implants, distraction etc can be
done as indicated
Bijaw
Surgery
BSSO

CONDYLAR HYPERPLASIA TYPE 1 A
 In Bilateral cases , surgery can be
performed after the age of
 14 years – Girls
 16 years- Boys
• WHY ?
 Because the anterior-posterior
mandibular growth stops and vector of
facial growth changes to a vertical
direction
CONDYLAR HYPERPLASIA TYPE 1B
 In Unilateral cases , recommended
age of surgery
 15 years- Girls
 17 years-Boys
• WHY ?
 Most facial growth is complete at this
age

 Low condylectomy performed to remove the tumour entirely.
 Reshape the condyle
 Reposition the articular disc over the remaining condylar neck
 Ipsilateral sagittal split osteotomy may be performed , and the disc/condylar stump
complex is seated into the fossa
 If indicated, orthognathic surgeries are performed to correct maxillary, mandibular deformities
 If needed inferior border ostectomy on the involved side, to re-establish vertical balance
of the mandible

Rodrigues DB,CastroV.Condylar Hyperplasia of theTemporomandibular Joint.Oral and Maxillofacial Surgery

Condylar hyperplasia: current thinking J.A. Higginson et al. / British Journal of Oral and Maxillofacial Surgery 56 (2018)

Orthognathic surgery: immediately or after condylar reduction
 In active cases, orthognathic surgery alone - not biologically sound (final
position will not be stable).
 Anticipatory over-correction - unreliable (magnitude of subsequent growth
cannot be predicted ).
 Further corrective surgery needed in patients who had orthognathic surgery
alone.
 Orthognathic surgery with simultaneous condylectomy to remove the abnormal
centre of growth, had a relapse rate of only 4%, which clearly established the
importance of condylar surgery.

Condylar surgery with or without orthognathic surgery
 Condylar surgery is a biologically-driven approach that aims to
arrest progression by removing the affected tissue,
 Its use is supported by a systematic review that showed that the
removal of 3 mm of condylar tissue was enough to prevent
relapse.
Wolford LM, Movahed R, Perez DE. A classification system for conditions causing condylar hyperplasia. J Oral Maxillofac
Surg 2014;72:567–95.

 Congenital malformation in which there is
deficiency in the amount of hard and soft tissues
on one side of the face
 Also, defined as a, “condition that involves an
absence or underdevelopment of structures that
arise from the first and second pharyngeal
arches” (Birgfeld and Heike 2012 )

Other
Terminologies
Craniofacial
Microsomia
Oculo-
auriculo-
vertebral
syndrome
1st and 2nd
branchial arch
syndrome
Otomandibular
dysostosis
Goldenhar’s
Syndrome
Lateral
facial
dysplasia

Incidence – 1: 3000 to 1: 5600 births
 M : F – 3:2
 Right side > Left side
 Typically Unilateral
 Bilateral in 5-30% of cases
 2nd Most Common Congenital FacialAnomaly
Rollnick B,etal.Occuloauriculo vertebral dysplasia …characteristics of 294 patients.Am JMed Genet 1987;26:361–
375

Various
theories
Disruption in
the migration
of neural
crest cells
Vascular insult to the
STAPEDIAL artery
causing ahemorrhage
in 1st and 2nd branchial
arches.
Genetic Cause –• Exact Etiology –
Unknown
• Appears to involve a
disruption in the
development of 1st & 2nd
branchial arches during
the first 6 weeks of
gestation
50% positive family
history
Inheritance pattern-
autosomal dominant
Maternal Risk
Factors- Vasoactive
medications, Smoking
during second trimester,
Diabetes, Use of
Reproductive technology
PoswilloD. Hemorrhage in development of face. In:
Bergsma D, ed. Birth defects: Original Article Series.
Morphogenesis and malformation of face and brain.
New York: Alan R. Liss; 1975:61–81
Johnston M, BronskyP. Prenatal craniofacial development:
New insights on normal and abnormal mechanisms. Crit Rev
Oral BiolMed 1995; 6:368.

Diverse
 Epibulbar dermoids
 Microtia/ Atresia/ Ear Tags/ Aural fistula
 Multiple fibromas / Odontomas
 Facial Palsy (15%)
 Anopthalmia/ Micropthalmia
 Coloboma of iris/ eyelids/ absence of
eyelashes
 Macrosomia
 CLP-(25%)
 Airway prblems
 Delayed eruption of teeth/ Hyodontia
 Mandibular hypplasia
 Soft tissue deficiency

• Classification difficult due to
hetrogenicity of the syndrome
• Popular Classification Sytems:
• Pruzansky’s
• OMENS system
• SAT system

• Pruzansky’s Classification 1969- Based on Radiographic features, classified the
underdeveloped mandible into 3 groups
Pruzansky S.Not all dwarfed mandibles are alike,Birth Defects 1969;4:120

Kaban L, Moses M, MullikenJ. Surgical correction of hemifacial microsomia in the growing child PlastReconstrSurg.
1988;82: 9-19

 Developed by Vento and colleagues in 1991
 Modified by Horgan et al(1995), OMENS +
-to denote presence of extra cranial anomalies
 Components
O – OrbitalAsymmetry
M – Mandibular Hypoplasia
E – Ear deformity
N – Nerve Dysfunction
S – Soft tissue Deficiency

Facial NerveOrbit
O0Normal orbital
size and position
O1 Abnormal
orbital size
O2 Inferior or
superior orbital
displacement
O3 Abnormal
orbital sizeand
displacement
Mandible
M0 Normal mandible
M1 small mandible
with glenoid fossa and
short ramus
M2Aabnormal shaped
with short ramus ,
glenoid fossa in
acceptable position.
M2B abnormal shaped
with short ramus,
glenoid fossa is
displaced withseverely
hypoplastic condyle
M3 absence of ramus
and glenoid fossa
Ear
E0 Normal
auricle
E1 Mild
hypoplasia
E2 Absence of
external canal
with variable
hypoplasia of
concha
E3 Malpositioned
lobule withabsent
auricle
N0 Nofacial
nerve
involvement
N1 Zygomatic
and temporal
branch
involvement
N2 Buccaland/or
manbibular
and/or cervical
branch
involvement
N3 Allbranch
affected
Softtissue
S0 Nosoft tissue
deficiency
S1 minimal soft
tissue deficiency
S2 Moderate soft
tissue deficiency
S3 Severe soft
tissue deficiency
The acronym OMENS designates each of the five major areas: O = orbital, M =mandibular,
E = ear, N = facial nerve, and S = soft tissue

 Mandibulo- TMJ severity classified into 4 grades
 Focuses surgeon’s attention on abnormalities of the
craniofacial skeleton & appropriate treatment plan

TYPE 1Hemifacial Microsomia
HFM type IA.Mandible is intact with
horizontal occlusal plane. Contour
augmentation only isneeded.
HFM type IB. Mandible is intact, but
occlusal plane is tilted. Le Fort I procedure,
bilateral mandibular sagittal split, and a
transposition genioplasty is needed.
HFM type II Mandible is incomplete with a
deficient right ascending ramus.A sufficient
glenoid fossa is present.(b) The ascending
ramus of the mandible is constructed from a
fullthickness costochondralgraft

HFM type III The right ascending ramus
of the mandible is vestigial and the
transverse full-thickness rib graft
glenoid fossa is inadequate. (b)
replacing the zygoma, and a TM joint is
constructed.
HFM type IV. The right facial skeleton is
retruded, and cuts for a right-sided Le Fort
III and left-sided Le Fort I procedure are
made. The right lateral orbital rim is cut
obliquely so as to become self- retaining
after transposition. (b) The facial skeleton is
advanced, occlusal plane corrected, and
mandible constructed.

 < 2 years of age
 Excision of preauricular skin tags /branchial remnants
 Correction of Macrostomia by Commissuroplasty
 Repair of cleft lip
Cheek and preauricular
branchial remnants
Commissuroplasty of left sided macrostomia
Cited from Birgfeld C,Heike C.Craniofacial Microsomia.Clinics in Plastic Surgery.2019 Apr;46(2):207–21.

< 2 years of age
 Treatment of epibulbar dermoids if visual axis is
disrupted
 Correction of eyelid colobomas to protect the
cornea and prevent exposure keratitis and
blindness

Distract.... : In Children ( > 8-9 yrs)
 Expansion of all tissues from within Bone to Skin
 Overcorrection is feasible- can avoid future surgeries
 Avoids Bone Grafts and Soft Tissue Fillers

 Incomplete Le Fort I osteotomy is done simultaneously with the mandibular corticotomy.
 Intermaxillary fixation is done on the fifth postoperative day, and distraction is initiated.
 Maxilla was distracted simultaneously with the mandible, preserving the preexisting stable
occlusion.
 Preoperative deviation of the occlusal plane from the horizontal varied from 12 to 18 degrees.
 The plane became horizontal achieving 100 percent correction

6-16 years of age
 For Orthodontic treatment- Functional Appliance therapy
 Box osteotomies can be done for orbital malposition
 Ear reconstruction
Soft tissueAugmentation
Cited from Birgfeld C, Heike C. Craniofacial Microsomia. Clinics in Plastic Surgery. 2019
Apr;46(2):207–21.

Autologous Rib Graft
Reconstruction
Osseointegrated implant retained
prosthesis
Porous Polyethylene
(Medpore customized
ear implant)
Cited from Birgfeld C, Heike C. Craniofacial Microsomia. Clinics in Plastic Surgery 2019Apr;46(2):207–21.

Dermal fat
grafts
Autologous Fat
Injections
Adipofascial Free
flaps
Alloplastic
Implants
Examples
• Scapular
• Parascapular
• Groin
• Omentum
• Anterolateral
thigh flap
Cited from Birgfeld C, Heike C. Craniofacial Microsomia. Clinics in Plastic Surgery. 2019Apr;46(2):207–21.

Hindawi Case Reports in Surgery Volume 2018, Article ID 2968983, 6 pages

• Lubrication
• Tarsorraphies
• Autogenous / Alloplastic slings
• Gold or platinum weight in the upper eyelid (Protection of cornea)
Facial Nerve Palsy
Facial Reanimation Procedures
When there is weakness of buccal or mandibular branch of the facial nerve

Obwegeser
& Makek
(1986)
Wolford
(2014)
 Encompasses various
condylar pathologies
• CH Type 1 corresponds to HE
• Type 1A (Bilateral ) and
• Type 1B (Unilateral ) involvement
• CH Type 2 corresponds to HH
 Caused by an Osteochondroma
• Type 2A –Exclusive hyperplasia of the
condyle
• Type 2B- Horizontal excessive growth
of condyle
• Based on asymmetry and
predominant growth factor
• H E – Excessive
growth in horizontal direction
• Hemimandibular
Hyperplasia - HH
Excessive growthin
vertical direction

Wolford
(2014)
Type III –
Combination of
Type I and Type II
Type 3 –Unilateralfacial
enlargement caused by
Benign tumourgrowth
Type 4 – Caused by
Malignant tumour
growth

Type 1A Type 1B
Type 2
Ipsilateral Low
Condylectomy
Recontour
Condylar neck
Disc
Repositioning
Orthognathic
Surgery
Non-
Salvageable
disc
Custom- fitted
total joint
Prosthesis
If Active growth is
present
Yes
No
High
Condylectomy
Wait until growth
is complete
Orthognathic
Surgery
Disc
Repositioning
Orthognathic
Surgery
Orthognathic
Surgery

Facial asymmetry condylar hyperplasia and hemifacial microsomia

Facial asymmetry condylar hyperplasia and hemifacial microsomia

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