Craniofacial Surgery

Dx and Tx
Bradley K. Coots, MD
Co-director Cleft and
Craniofacial Clinic
Clinical Assistant
Professor
Division of Plastic
Surgery
University of Iowa

Historical Perspectives
500bcreports of facial fractures being treated with interdental
wiring.
Various contributors to advancements to treatment of fractures
of the maxillofacial skeleton
Rene Le Fort
French
1901
Description of the 3 basic patterns of maxillary fractures
Dropped cadavers from 3rd floor buildings and discovered consistent
fracture patterns
Foundation of the principle of partitioning the facial skeleton for
treatment

Historical Perspectives
Harold Gillies
Initially trained in otolaryngology
Significant case load during World War II
Ralph Millard published many of his cases and described the
following in 1957
LeFort I fracture with malar fracture and enophthalmos
Treated with osteotomies and reduction and bone grafting

LeFort III fracture seen late with enophthalmos

Treated with osteotomies and reduction and bone grafting

Reed Dingman
1940s in US
Treating post traumatic deformities with LeFort I and II
osteotomies and bone grafting

Historical Perspectives
1942Gillies
Operated on a child that
likely had Crouzons
Disease
Mobilized midface through
direct skin incisions
--did not place any bone
grafts in the gaps
created and pt had
significant relapse
Paul Tessier (father of
craniofacial surgery)
observed Gillies in England
on multiple occasions

History
Paul TessierParis1950s
and 1960s
Began treating Crouzons
Syndrome patients with
Lefort III osteotomies and
bone grafting
1960salong with
neurosurgeon Gerard Guiot
Began treating orbital
hypertelorism through
an intracranial approach
First Craniofacial
Operation

History
Tessier was unsure of these new methods
1967
Called together international peers in Paris and presented results
They were very supportive and urged him to go on

Tessier went on to develop

Intracranial approach for facial advancement
Systematized the treatment of orbital dystopias of all types
Allowed for resection of cranial base tumors
Facial Cleft Classification

History
Daniel Marchacobserved
Paul Tessier operate for
many years
Necker Hospital in Paris
began to apply many of
Tessiers principles to
infants for the treatment of
craniosynostosis
Marchac and Dominic
Renier (neurosurgeon)
found that infants with
multiple suture synostosis
had significantly higher
intracranial pressures than
single suture synostosis
children

Eric Arnaud/ Necker

Hospital-Paris

Cranial Sutures
Metopic
Coronal
Squamosal
Sagittal
Lamboid

Craniosynostosis
Premature fusion of cranial
sutures
Virchows Lawgrowth of
the skull deviates from the
normal perpendicular
growth away from open
suture to a parallel
relationship to the closed
suture

Types of Craniosynostosis
Scaphocephaly
Plagiocephaly
Coronal (anterior)
Lambdoid (posterior)

Brachycephaly
Trigonocephaly

Why do we operate on
these patients?
To reduce the stigmata of the outward appearance
of a congenital difference
Address increased intracranial pressure in multiple
suture synostoses
Airway concerns in syndromic patients that have
midfacehypoplasis
Increase globe/eye protection in syndromic patients

Diagnosis
Visualize an abnormal skull shape
3-dimensional ct scan

Palpate fused sutures (raised ridge)

Increased intracranial pressure
Child irritable
Fundoscopic exam showing papilledema
Thumbprinting of the cranium on ct scan or plain film

SagittalSynosto
sis
Scaphocephaly (Keel-shape)

Main goal of treatment is to

decrease the length of the A-P
dimension and widen the coronal
dimension

SagittalSynostosis
My technique
Resect involved suture and morselize
Barrel-staves along parietal bone
Early intervention (before 6 months) allows continued brain
growth the opportunity to reshape skull after release

Endoscopic strip craniectomy followed by helmet therapy

Distraction
Spring Therapy

Anterior
(Coronal)Plagiocephaly
positional

craniosynostosis

Positional Plagiocephaly
Increased incidence since
back to sleep campaign to
reduce Sudden Infant
Death Syndrome (SIDS)

Unilateral Coronal
Synostosis
(Plagiocephalyflat)

Premature fusion of a unilateral coronal suture

Findings
Flat/posterior affected side
Root of nose deviates towards affected side
Harlequin eye deformity
Raised brow
Foreshortened greater wing of sphenoid

Harlequin Deformity on left

Fronto-orbital
Advancement

Trigonocephaly
--fusion of metopic suture

Trigonocephaly
Surgical Goals
90 degree angle at
temporal area
Some will try to increase
inter-orbital distance with
spacer graft
Fronto-orbital
advancement

Trigonocephaly

Multiple Suture
Craniosynostosis
Brachycephalyfusion of both coronal sutures
Oxycephalyfusion of both lambdoid sutures
Kleebatschadel (Clover Leaf Skull)fusion of majority of coronal
sutures
There have been several studies which have shown increased intracranial pressure with multiple suture fusion
These patients undergo different procedures to expand the cranial
vault to relieve increased intracranial pressure
Several Syndromes involve multiple suture fusion along with
midfacehypoplasia

Syndromes and
Craniosyntostosis

Crouzon Syndrome

Craniofacial synostosis
Midfacehypoplasia

Apert Syndrome (acrocephalosyndactyly)

Cranial synostosisusualy bilateral coronal

Midfacehypoplasia
Syndactyly (hands and feet)
High-arched palate
Submucous cleft palate
Acne vulgaris
Higher incidence of developmental delay

Pfeifer Syndrome

Carpenter Syndrome

Take home message is that many of these syndromes are also associated with midfacehypoplasia

In addition to cranial expansion, also benefit from midface advancement

 Examples of these syndromes include Aperts and Crouzons

Syndromes
Both of these can involve multiple cranial suture fusion (commonly
the bilateral coronal sutures are fusedbrachycephaly)
In addition to this these patients experience midfacehypoplasia
Exorbitism with cornial exposure and risk of vision compromise
Obstructive airway compromise due to the retrusive maxilla

Aperts pts have syndactyly and decreased intelligence

Treatment of the craniofacial condition typically involves a

fronto-orbital advancement at around 4-8 months to expand the
calvarium (reduce Increased ICP) and provide some ocular globe
protection

 These patients will then undergo an advancement of the midface

at around 5 years of age to provide ocular globe protection and
maxilla advancement to improve the airway
Some favor only a Lefort 3 at this time---and the standard is now
distraction via an internal or external device as opposed to
advancement with bone grafting and rigid fixation
Some favor Monobloc distraction at this time to also address the
forehead again; which usually relapses
Critics of the monobloc believe that there is any increased infection risk
to the central nervous system due to the concomittantosteotomies of
the midface which involve mucosal surfaces

An emerging group is now using springs to expand the cranial vault

posteriorly at the lamboid sutures at age 4 months and this
addresses the increased ICP---this allows them to delay the monobloc
or Fronto-orbital advancement/Lefort 3 until age 5 or 6 potentially
which could mean one less major operation

Brachycephalybilateral
coronal synostosis
Renier and Marchac early 1980s
Placed ICP monitors in single and multiple suture synostosis
patients and found that multiple suture patients had a positive
correlation with increased intra-cranial pressure

One of primary goals of surgery is to expand the intra-cranial

volume in order to decrease intra-cranial pressure

Bilateral Coronal
Synostosis--Brachycephaly

brachycephaly

Fronto-orbital advancement

Crouzon Syndrome

Apert Syndrome

Apert Syndrome

Spring Therapy for Calvarial

Expansion

Monobloc

Monobloc distraction

24/11/2005
18:46:40

24/11/2005
18:46:27

22/02/2006
20:03:37

22/02/2006
20:03:21

09/04/09

09/04/09

Distraction Osteogenesis
and Craniofacial Surgery

Distraction Osteogenesis
Gradual controlled displacement of surgically
created fractures via traction to induce and direct
bone and soft tissue formation
Generation of movement of bone into a site of bony
deficiency (Ilizarov)
Simultaneous expansion of soft tissue and bone
volume

Chronology of
Distraction
Osteogenesis
1956 Illizarov
Russian Orthopedic Surgeon
Utilized DO to lengthen long
bones
His principles were applied to
the craniofacial skeleton by
Snyder and McCarthy
Ilizarov used DO to treat WWII
wounds and developmental
disorders

Illizarov Frame

Current
Distraction Used Extensively in Craniofacial Skeleton

Calvarial Expansion
Midface Advancement (Lefort 3)
Maxillary Advancement (Lefort 1)
Mandibular Advancement
Alveolar Advancement

Distraction Sequence
Latencyperiod immediately following osteotomy
and distractor placement
--usually lasts 1 to 7 days

Activation----distraction/ movement
---typically 1mm per day

Consolidation----bony solidification
---texts say usually twice as long as period of activation
(usually longer, especially in syndromic children)

Complications
Undesirable distraction vector
Premature consolidation
Failure of consolidation
Scarring
Infection
Nerve Injury
Tooth Injury

Internal vs. External Devices

Internal distractors have their force vector more directly
coaxial, or in line, with the central axis---making them more
effective in terms of force delivered and resultant
distraction length
However, the internal distractors are sometimes more
difficult to place and remove

Craniofacial Applications of DO
Dentoalveolar
Mandibular Reconstruction
MandibularHypoplasia

Pierre Robin Sequence

Treacher Collins
Craniofacial Microsomia
TMJ Ankylosis
Chin Distraction

MidfaceHypoplasia
Clefts
Midfacehypoplasia/ exorbitism
Lefort I, II, III; Monobloc

Craniosynostosis

SoWhy DO instead of
traditional osteotomies with
advancement and Bone Grafting?

No need for morbidity of bone graft harvest

Can potentially advance greater distances with distraction

osteogenesis due to the gradual stretch of the soft tissues over
time being less constraining
Less potential intracranial dead space for fluid collection and
infection nidus formation (monobloc)
Lower risk of malunion/nonunion

DI
7 year old female born with Aperts Syndrome
--underwent fronto-orbital advancement at
around 10 months of age
--now presents with issues of airway
obstruction and marginal bony ocular globe
support

Lefort 3
This procedure is typically performed at around 4-7
years of age to advance the midface in
syndromiccraniosynostosis pts
To address ocular exposure and airway obstruction

These pts have usually undergone fronto-orbital

advancement prior to the age of one year
For intracranial expansion

Lefort 3

Pierre Robin Sequence

First described in 1923 by Pierre Robin as a Syndrome:
Paroxysmal respiratory obstruction
Cleft Palate
Micrognathia

Current definition as a Sequence:

Micro-retrognathia/retrogeniadefining feature
Glossoptosis
Upper airway obstruction
Cleft Palate not always present

1:2000 to 1:8500 births

50% of the patients also have a high-arching cleft palate
--cleft may be of secondary palate only --soft or hard palate

Pierre Robin Presentation

Airway Obstruction

Apnea
Stridor, Cyanosis, Sternal Retraction
Desaturation
Chronic hypoxia
Cerebral impairment
Pulmonary hypertension
Corpulmonale

Early Death

Work up
Pediatric ENT is consulted so the patient can
undergo endoscopic examination of the airway to
rule out other causes of airway obstruction
Choanalatresia
Laryngomalacia

Evaluation for Gastric Reflux (g-tube?)

If other issues or concernsconsider genetic
workup

Pierre Robin
Conservative Management
Prone positioning
Supplemental Oxygen
Nasopharyngeal Airway
Intubation
Diagnostic Upper airway endoscopy

Surgical Management
Mandibular Distraction Osteogenesis

Lengthening over several weeks

Anterior movement of tongue
Avoid tracheotomy
Decannulationif already trached

Outcomes
Increased mandibular and tongue growth in early childhood
Airway enlargement 350% by age 2
May still have mandibularhypoplasia

Avoidance of long-term feeding problems necessitating G-tube

with early airway intervention
Earlier intervention, improved monitoring
Decreased mortality
14% prior to 1986
3% presently

JT
Transferred from outside hospital at age 5 weeks for
persistent 02 desaturation
Pt had a g-tube placed at outside hospital for
persistent reflux
At LeBonheurENT initially consulted for
tracheostomy
ENT consulted plastic surgery for mandibular distraction
Many of these children still undergo tracheostomy in the
community and other centers

JT
Pt evaluated by ENT and endoscopy performed
No other potential causes of airway obstruction

Pt underwent bilateral mandibularosteotomy via an external

(Risdon/Submandibular ) approach; followed by placement of an
internal distraction device with any externalized arm
2 days of latency followed by 7 days of distraction at 2mm per day
Most are distracted 12 to 18mm

Endpoint of distraction is loosely based on occlusion with a slight class 3

relationship extubation usually takes place at 3-6 days post op
Some centers perform pre and post op sleep studies and if not obstructing
these pts go home at pod 3-4 and complete last several days of distraction at
home

Consolidation of 3 months approximately

Controversies
Trach vs. Tongue Lip Adhesion vs. Mandibular Distraction

Tracheostomy reserved for syndromic case

Tongue lip adhesion is still considered by many to be a more first
line treatment than distraction
Some believe there is delayed speech
Cost analysis has not show any difference
Some studies have shown complication rates to be higher in the
adhesion group

Growth of Mandible
Do these kids eventually develop a normal mandibular growth
rate?

Most do end up having normal maxillary/mandibular relationship

Lefort I with distraction

for Obstructive Airway

PW
14 year old Trisomy 21 patient with Obstructive
Airway Issues
Underwent Lefort 1 osteotomy with placement of
RED 2 (rigid external distraction) halo device
Distracted approximately 22mm
3 day latency; 1mm per day; 3 month consolidation

QUESTIONS?
Thanks!