Craniofacial SurgeryDx and Tx

Bradley K. Coots, MD

Co-director Cleft and Craniofacial Clinic

Clinical Assistant Professor

Division of Plastic Surgery

University of Iowa

Historical Perspectives 500bc—reports 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 1940’s in US Treating post traumatic deformities with LeFort I and II

osteotomies and bone grafting

Historical Perspectives

1942—Gillies Operated on a child that

likely had Crouzon’sDisease

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 Tessier—Paris—1950’s and 1960’s Began treating Crouzon’s

Syndrome patients with Lefort III osteotomies and bone grafting

1960’s—along 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 Marchac—observed Paul Tessier operate for many years Necker Hospital in Paris—

began to apply many of Tessier’s principles to infants for the treatment of craniosynostosis

Marchac and Dominic Renier (neurosurgeon) found that infants with multiple suture synostosishad significantly higher intracranial pressures than single suture synostosischildren

Eric Arnaud/ Necker Hospital-Paris

Metopic

Coronal

Squamosal

Sagittal

Lamboid

Cranial Sutures

Craniosynostosis

Premature fusion of cranial sutures

Virchow’s Law—growth 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

SagittalSynostosis

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

(Plagiocephaly—”flat”) 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

Brachycephaly—fusion of both coronal sutures

Oxycephaly—fusion of both lambdoid sutures

Kleebatschadel (Clover Leaf Skull)—fusion of majority of coronal sutures

There have been several studies which have shown increased intra-cranial 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 synostosis—usualy 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 Apert’s and Crouzon’sSyndromes Both of these can involve multiple cranial suture fusion (commonly

the bilateral coronal sutures are fused—brachycephaly) 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

Apert’s 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 midfaceat 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 monoblocor Fronto-orbital advancement/Lefort 3 until age 5 or 6 potentially which could mean one less major operation

Brachycephaly—bilateral coronal synostosis

Renier and Marchac –early 1980’s 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 CalvarialExpansion

Monobloc

Monobloc distraction

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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 Osteogenesisand 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

Osteogenesis1956– 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

Latency—period immediately following osteotomyand 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

So…Why 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 Apert’s 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/retrogenia—defining 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 concerns—consider 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 Decannulation—if 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 LeBonheur—ENT 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!