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DR VIPIN V NAIR PLASTIC SURGERY PGIMER CHANDIGARH
Broad spectrum of disorders
Characterized by facial dysgenesis
Varying degrees of hypoplasia/aplasia of the components of the face and facial skeleton.
The major components
• Abnormalities of the ear• Hypoplasia of the mandible.
A congenital malformation in which there is deficiency in the amount of hard and soft tissues on one side of the face.
HFM was first described by German physician
Carl Ferdinand Von Arlt
Umbrella Term
Oculo -Auriculo-Vertebral dysplasia (OAV dysplasia)
Include hemifacial microsomia and associated anomalies of the face.
Gorlin R. Oculoauriculovertebral dysplasia. J Pediatr 1963; 63:991.
Hemifacial microsomia
Microtia
Goldenhar’s syndrome
Otomandibular dysostosis
Lateral facial dysplasia
Craniofacial microsomia.
First and second branchial arch syndrome
Primarily a syndrome of first and second branchialarches involving underdevelopment of the
Temporomandibular joint
Mandibular ramus
Masticatory muscles
Ears
Occasionally defects in ▪ Facial nerve
▪ Orbits.
Review of the early works
Important article in understanding this disorder
Grabb W. The first and second branchial arch syndrome. Plast Reconstr Surg 1965; 36:485–508.
MENTIONED IN
Teratologic tablets of the Chaldeans
Grabb W. The first and second branchial arch syndrome. Plast Reconstr Surg 1965; 36:485–508.
Stapedial Vessel Rupture affecting 1st & 2nd brachial arches development
Poswillo D. 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.
Death of a population of neural crest and adjacent cell populations
Disruption in the migration of the neural crest cells
Johnston M, Bronsky P. Prenatal craniofacial development: New insights on normal and abnormal mechanisms. Crit Rev Oral Biol Med 1995; 6:368.
The most frequently encountered form of isolated facial asymmetry.
Incidence between 1 in 3000 and 1 in 5600 births.
2nd Commonest
congenital facial anomaly
After cleft lip/palate.
Facial involvement is typically unilateral
Bilateral in up to 30%
48% part of a larger syndrome
Goldenhar syndrome
Craniofacial microsomia
Rollnick B, et al. Oculoauriculovertebral dysplasia and variants: phenotypic characteristics of 294 patients. Am J Med Genet 1987; 26:361–375.
Carvalho G. Auditory and facial nerve dysfunction in patients with hemifacial microsomia. Arch Otolaryngol Head Neck Surg 1999; 125:209–212.
Male : Female 3 : 2
Rollnick B, et al. Oculoauriculovertebral dysplasia and variants: phenotypic characteristics of 294 patients. Am J Med Genet 1987; 26:361–375.
No DNA has been Identified in the condition.
A doctor or medical team makes the clinical diagnosis.
Correspondence in
Size
Shape
Relative position
Of parts on opposite sides of a dividing line or median plane.
Lack or absence of symmetry.
Illustrates an imbalance or disproportionality between the right and left sides.
A degree of asymmetry is normal
Acceptable in the average face.
Functional as well as Morphological asymmetries
Preferential laterality for some anomalies is striking
Cleft lip occurs more left side.
Left-right tooth crown size asymmetry
Normal state in the general population.
The point at which ‘normal’ asymmetry becomes ‘abnormal’
Cannot be easily defined
Determined by the clinician’s sense of balance
Patient ’s sense of imbalance.
Extra oral clinical examination
May be associated with underdevelopment in the skull, or the orbit on the affected side.
.
Small non-seeing eye
OR
Eye may be entirely absent.
Eyelids
Dermoids or notches.
Cheek:
Flat because the bone beneath hasn’t grown properly.
Face: Vertically short in this side.
Underdeveloped Mandible or a portion of the mandible can be missing (ramus or condyle)
Condylar Hypoplasia or Aplasia.
Mandible
External Ear may be :
Normal.
Underdeveloped.
Absent.
Skin tags
In front of the ear or in a line between the ear and the corner of the mouth.
Hearing may be defective.
Tooth discrepancies
Agenesis of teeth
Supernumerary teeth
Enamel and dentin malformations
Delay in tooth development.
Important for surgical planning
Kaban L, Mulliken J, Murray J. Three dimensional approach to analysis and treatment of hemifacial microsomia. Cleft Palate J 1981; 26:90–99.
Fearon J. Hemifacial microsomia. In: Vander Kolk C, ed. Plastic surgery: indications, operations, and outcomes. Philadelphia: Mosby-Year Book; 2000.
Illustrating the lack of mandibular growth on the right side.
Panoramic radiograph
Allow characterization of
Orbits Pyriform aperture Occlusal relationships Chin point.
Horizontal reference line (Lor–Lor), Infraorbital plane (InfOr–InfOr), Nasal Floor (NF–NF), Occlusal Plane (Oc–Oc), Antegonial Plane (Ag–Ag).
Determined preoperatively by drawing a line through the superior orbital rims
Relating the position of a line drawn through the nasal floor/maxillary floor to this line.
Measuring the vertical distances at the level of the molars
Vertical height discrepancy of the ramus
Measured between the gonion and the apex of the head of the condyle on both sides.
Difference - vertical deficiency
Kaban L, Mulliken J, Murray J. Three dimensional approach to analysis and treatment of hemifacial microsomia. Cleft Palate J 1981; 26:90–99.
Determination of the degree of mandibular advancement
SNASNB.
Axial CT showing hypoplastic left ramus and coronoid process of mandible
Computerized Tomography showing hypoplastic left pterygoid plates
Medical photographs views
FrontalLateralObliqueSubmental vertexOcclusal
CAD MODEL
WAX MODEL
O=Orbital Distortion
M=Mandibular Hypoplasia
E=Ear Anomaly
N=Nerve Involvement
S=Soft Tissue Deficiency
Vento A, LaBrie R, Mulliken J. The OMENS classifi cation of hemifacial microsomia. Cleft Palate-Craniofacial J 1991; 28:68–77.
Includes
Extra -craniofacial anomalies
Simply by designating the ‘plus’
Advocated by R Cousley
Cousley R. A comparison of two classifi cation systems for hemifacial microsomia. Br J Oral Maxillofac Surg 1993; 31:78–82.
‘O’ describes the morphology of the orbit
O0: Normal orbital size and position O1: Abnormal orbital size O2: Abnormal orbital position O3: Abnormal orbital size and position
Vento A, LaBrie R, Mulliken J. The OMENS classifi cation of hemifacial microsomia. Cleft Palate-Craniofacial J 1991; 28:68–77.
The ‘M’ grading for the mandible
Mo: Mandible is normal. MI: Mandible & glenoid fossa small -- Short ramus. M2: Ramus is short and abnormally shaped.
2a: Glenoid fossa is in anatomically acceptable position with reference to opposite TMJ.
2b: TMJ is inferiorly, medially, and anteriorly displaced, with severely underdeveloped condyle.
M3: Ramus, Glenoid fossa, and TMJ completely absent
Horgan J. OMENS-plus: analysis of craniofacial and extracraniofacial anomalies in hemifacialmicrosomia. Cleft Palate Craniofac J. 1995;32:405-412
‘E’ describes ear abnormalities
Eo: Normal ear
E1: Mild hypoplasia and cupping All structures present
E2: Absence of external auditory canalVariable hypoplasia of the concha
E3: Malpositioned lobule Absent auricleLobular remnant
Horgan J. OMENS-plus: analysis of craniofacial and extracraniofacial anomalies in hemifacialmicrosomia. Cleft Palate Craniofac J. 1995;32:405-412
‘N’ characterizes the severity of facial nerve involvement
N0: No facial nerve involvement
N1: Upper facial nerve involvement (Temporal and zygomatic branches)
N2: Lower facial nerve involvement (buccal, mandibular, and cervical branches)
N3: All branches of the facial nerve affected
Horgan J. OMENS-plus: analysis of craniofacial and extracraniofacial anomalies in hemifacialmicrosomia. Cleft Palate Craniofac J. 1995;32:405-412
‘S’ describes the degree of soft tissue deficiency.
S0: No obvious soft tissue or muscle deficiency S1: Minimal S2: Moderate S3: Severe
Horgan J. OMENS-plus: analysis of craniofacial and extracraniofacial anomalies in hemifacialmicrosomia. Cleft Palate Craniofac J. 1995;32:405-412
S=Skeletal
A=Auricular
T=Soft Tissue
Pruzansky, 1969
Grade I, II , III Mandible
Most Useful in Clinical practice
Pruzansky S. Not all dwarfed mandibles are alike. Birth Defects 1969; 4:120.
1988 - Kaban and Mulliken
Type IIA and Type IIB groups based upon the location and position of the TMJ
Grade I mandible:
Small mandible with a normal TMJ
Normally shaped miniature mandible
Normal glenoid fossa
Well-developed muscles of mastication.
Pruzansky S. Not all dwarfed mandibles are alike. Birth Defects 1969; 4:120.
Grade II mandible
Functioning TMJ with a misshapen condyle
Ramus short and abnormally shaped.
Muscles of mastication are somewhat deficient.
Grade III mandible Ramus , and glenoid fossa
are absent No TMJ. Significant soft tissue
deformities.
The mandible may end abruptly in the molar region.
Type IIA
TMJ, ramus and glenoid fossa are hypoplastic, malformed and malpositioned
But the deformed joint is adequately positioned for symmetric opening of the mandible.
Type IIB
Joint is malpositionedinferiorly and medially
Will not function as a TMJ for adequate symmetric opening of the mandible.
In the type IIA, the degree of hypoplasia of the mandibular musculature is closer to normal than in the type IIB.
Kaban L, Moses M, Mulliken J. Surgical correction of hemifacial microsomia in the growing child. Plast Reconstr Surg. 1988;82: 9-19.
Variant of ‘hemifacial microsomia’
10% of all OAV spectrum
Epibulbar dermoids Auricular appendices Blind -ended fistulae Mandibular hypoplasia Vertebral anomalies.
Epibulbar dermoids
‘Skin patches’ that extend onto the conjunctiva and cornea of the eye
Excision
Preauricular skin tags
Cartilage remnants
Macrostomia -Commissuroplasty
Mandibular distraction
Sleep apnea (with or without a tracheostomy).
Also corrects Swallowing Gastroesophageal reflux
Pruzansky type I mandible Horizontal occlusal plane
No surgical treatment is recommended at this age.
Severe reduction in the vertical height ramus (Pruzansky types I and ll)
Distraction osteogenesis Airway problems
Obvious aesthetic deformity
Improves the airway Lengthens the affected ramus Augments the associated soft tissue and muscles
of mastication
Pruzansky type III deformity Preliminary costochondral rib Iliac bone graft reconstruction
Glenoid fossaZygomatic archAscending ramus
Persistent mandibular deficiency
Manifested by Airway obstruction
Bilateral craniofacial microsomia Associated sleep apnea
Bilateral mandibular distraction
After sleep studies
If no mandibular rami exist Costo -chondral graft reconstruction
Period of orthodontic treatment Functional appliance therapy
Promote eruption and growth of the dentoalveolus on the affected side.
Distraction
Chronic low-grade sleep apnea
Severe dysmorphism
Staged Ear reconstruction
Osseointegrated Ear prosthesis
Serial autogenous fat injections Microvascular free flap
Improves contour and function
Surgery
Indicated in the period of skeletal maturity
Residual deficiency Severe malocclusion Failure of the patient to seek treatment
previously.
Limited autogenous bone grafting of deficient portions of the craniofacial skeleton
Bilateral mandibular advancement
Combined LeFort I osteotomy, bilateral mandibular osteotomy, and genioplasty
Revise Scars
Soft tissue readjustments
Eye Socket Reconstruction Artificial Eye Prosthesis.
Long-term Follow Up.
Tendency for poor growth on the affected side.
Occlusion Problems /Canting.
Orthodontist visits
Hearing Defects.
Abnormalities of the internal ear
Absent ear canal
Neurootologist
Normal intelligence
Learning Difficulties: Language problem due to deafness.
Take a long time Because of complexity
of the disease
Multidisciplinary treatment
Many specialists
Better results Fewer complications.
Meeting other families with the same problem in these centres offer an opportrunity of advice and support.
Established therapeutic tool
Advantage
Eliminate bone grafts and alloplastic materials
Eliminate infections after osteotomies
Decrease rate and extent of osteotomy relapse.
Application of gradual and incremental traction force/tension to surgically separated bony segments to produce additional bone.
Releases inherent biologic forces to generate tissues and the associated neuromuscular/soft-tissue complex.
Distraction histogenesisFirst examples of surgically induced tissue engineering.
Popularized the concept of distraction osteogenesis
New York University
Introduced clinical craniofacial distraction
Full thickness osteotomy Low -energy corticotomy
Distraction zone Location of the bony separation.
5 to 7 days
Gradual distraction forces separate the edges and elongate the intersegmentarycallus under tension
Rigidity of the distraction device is critical
Vector parallel to the orientation of the device
External fixation must be maintained Allow consolidation Approximately 8 weeks.
Aro H. Biomechanics of distraction. In: McCarthy JG, ed. Distraction of the Craniofacial Skeleton. New York, NY: Springer; 1999
Transport segment delivered into the skeletal defect
By forces applied by the distraction device.
The leading edge has --fibrocartilage cap.
Bone grafting after docking Fibrocartilage is resected
Distraction occurs without a cartilaginous intermediate.
Karp NS, McCarthy JG, Schreiber JS, et al. Membranous bone lengthening: a serial histologic study. Ann Plast Surg. 1992;29:2
Tensile forces delivered to the developing callus at the osteotomy site cause elongation of the callus.
The mechanical environment in the distraction zone is determined by the following factors:
Rigidity of the distraction device
Applied distraction forces
Inherent physiologic loading (muscle action),
Properties of all of the local soft tissues
Defined as the amount of elongation as a fraction of the original bone length.'
Bone tissue cannot survive for long a load exceeding more than 1% to 2% tensile strain.
Bone formation is not observed in the distraction zone until 4 weeks of activation
The process by which mechanical forces are converted to cellular signals is termed mechanical transduction.
Distraction permits surgery at a younger age
No need for Bone grafts
Blood transfusions,
Prolonged operations
Extended hospital stays.
Associated distraction histiogenesis
Relapse rate is lower
EXTERNAL (EXTRAORAL) BURIED (INTRAORAL) DEVICE
More successful and consistent outcomes.
They are especially indicated when the skeletal site for the osteotomy and pin insertions is diminutive in area and volume.
DISADVANTAGE
External scar
ADVANTAGE
Better scar formation Transcutaneous
(submandibular) incision Always a resulting, albeit
fine line scar
Ideal for patients requiring a vertical vector
DISADVANTAGE
Intraoral devices more difficult to remove.
Approach
Individual or combined transcutaneous (submandibular) or intraoral incisions.
Vertical Vector
Defined as one at 90 degree to the maxillary occlusal plane
Indicated
Vertical deficiency of the ramus
Horizontal vector Parallel to the maxillary
occlusal plane
Indication Severe micrognathia
Deficiency of the mandibular body
Deficiency in both the vertical ramus and the horizontal body of the mandible.
Indications
Infant or young patient with sleep apnea and the associated alimentary problems
Patients with respiratory functional problems
Facial dysmorphism
Over see device manipulation (activation).
Necessary to "mold the generate" with Orthodontic rubber bands
or Manipulation of multiplanar
distraction devices to correct or ameliorate malocclusions.
Unilateral mandibular distraction
Movement of the chin to the contralateral side
Lowering of the ipsilateral oral commissure
Lowering of inferior border of the mandible
Occlusal plane to a level below contralateral side.
“Overcorrection " indicated in the growing child
Bilateral mandibular distraction
Achievement of a slight anterior cross biteespecially in the growing child.
Maxillary Le Fort I distraction
Indicated for the correction of maxillary retrusion
Obwegeser H. L. Correction of the skeletal anomalies of stomandibular dysostosis. J
Maxillofac Surg.1974;2:73
External head frame (RED) Internal Device
A vector is usually chosen in a forward and downward
End point is a class II malocclusion or Overjet in the growing child ("overcorrection").
Indicated
Syndromiccraniofacial synostosis
Orbitofacial clefts
• Two types devices: • Head frames • Buried devices
Advantages
Avoids the need for bone grafts and plates and screws.
Reduced
▪ Length of the surgical procedure
▪ Volume of blood transfusion
▪ Length of hospitalization.
Aesthetic results are superior
Greater degree midface distraction (up to 20 mm) can be achieved
Ideal vector of distraction
Anterior direction along a plane parallel to the maxillary occlusal surface.
Treatment end points Overcorrection with an overjet or class II occlusion and maximal orbitozygomatic advancement.
Superior part of the orbits and frontal bones is distracted along with midface
Neurosurgeon Craniotomy and intracranial exposure
INDICATION
Severe exorbitism Expansion of orbital
volume and Cranial vault Symptoms of increased
intracranial pressure.
Lack of projection of the frontal bone and the supraorbital bar
Munro I. Treatment of craniofacial microsomia. Clin Plast Surg 1987; 14:177–186.
Useful adjunct to the rehabilitation of the patient with hemifacial microsomia
Two primary roles.
1. Chin point or pogonion more severely deviated than the basal bone of the lower jaw can be corrected
2. Movement of the chin through a transverse motion useful adjunct in completing the overall correction of the facial disfigurement through a ‘camouflage’ effect.
Allows alignment of the chin point with the true facial midline.
Lower sulcus incision
Soft tissue of the chin must remain attached to the cut bone for a predictable effect.
Soft diet Begun postop
maintained 4-6 weeks.
Till hospitalized Converted to oral antibiotics One week
Serial postoperative photographic and X-ray documentation
Started six weeks Three Six 12 months
Annual or biannual follow up
Clinical exam Photographic documentation
Until growth is complete
Improvement is the goal, and that complete correction is less often obtained
Minor occlusalirregularities may persist after surgery
Absolute facial symmetry is difficult to obtain.
Face Malocclusions
Hematomas
Infection
Non-union of the graft
Neurologic complications - injury Inferior alveolar nerve Facial nerve branches
Ankylosis of the newly fabricated temporomandibular joint
Irregular growth of the costochondralgraft
A Parent’s Guide to HFMA Publication of Children’s craniofacial association
Comprehensive planning and case management.
The surgeon must utilize the framework and concepts discussed and tailor them to provide optimal care of the individual patient.
Strict attentiveness to the function of the unafflicted‘normal’ ear and prompt attention to treatment of any disorder.
Throughout the stages of overall case management, it must be recognized and remembered that hemifacialmicrosomia patients should be managed with strict attention to airway during anesthetic management.
Gorlin R. Oculoauriculovertebral dysplasia. J Pediatr 1963; 63:991.
Manu Dhillon et al., Hemifacial microsomia: a clinicoradiological report of three cases. Journal of Oral Science, Vol. 52, No. 2, 319-324, 2010.
A guide to understanding hemifacial microsomia. A publication of children’s craniofacial Association, Dallas, TX, 2005.
Maria Mielnik-Błaszczak & Katarzyna Olszewska. Hemifacial MicrosomiaReview of the Literature. Dent. Med. Probl. 2011, 48, 1, 80–85.
Grabb W. The fi rst and second branchial arch syndrome. Plast ReconstrSurg 1965; 36:485–508.
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The influence of stability of fixation and soft-tissue preservation. ClinOrthop. 1989;238:249.
Pruzansky S. Not all dwarfed mandibles are alike. Birth Defects 1969; 4:120.