CRANIOFACIAL CRANIOFACIAL MICROSOMIAMICROSOMIA

DR ALAMZEB KHANDR ALAMZEB KHANDEPARTMENT OF PLASTIC AND DEPARTMENT OF PLASTIC AND RECONSTRUCTIVE SURGERYRECONSTRUCTIVE SURGERY

King abdulaziz specialist hospital King abdulaziz specialist hospital

Taif, saudi arabiaTaif, saudi arabia

INTRODUCTIONINTRODUCTION

Craniofacial microsomia, a variable hypoplasia of the skeleton as well as of the overlying soft tissue, is the second most common congenital syndrome of the head and neck region, withan incidence as high as 1 in 3,500 live births.

ETIOLOGYETIOLOGY

Many theories regarding the etiology but The most commonly accepted is a teratogen theory of a vascular insult, with hemorrhage and hematoma formation in the developing first and second branchial arches and subsequent maldevelopment of the latter.

DERIVATIVES OF FIRST BRANCHIAL DERIVATIVES OF FIRST BRANCHIAL ARCHARCH

DERIVATIVES OF SECOND BRANCHIAL DERIVATIVES OF SECOND BRANCHIAL ARCHARCH

Goldenhar SyndromeGoldenhar Syndrome Also called Oculoauriculovertebral dysplasia or Also called Oculoauriculovertebral dysplasia or

Branchial arch syndrome. Branchial arch syndrome. Characterized by multiple anomalies of the ocular, Characterized by multiple anomalies of the ocular,

cardiac, skeletal and central nervous system. cardiac, skeletal and central nervous system. Ten percent of those affected are mentally retarded. Ten percent of those affected are mentally retarded. Classic triad of the syndrome includes: Classic triad of the syndrome includes:

Hemifacial microsomia Hemifacial microsomia Epibulbar dermoids or dermolipomas Epibulbar dermoids or dermolipomas Deformity of the ears with pre-auricular appendages. Deformity of the ears with pre-auricular appendages.

Associated malformations include orbital hypoplasia, Associated malformations include orbital hypoplasia, upper lid colobomas, nasolacrimal duct obstruction, upper lid colobomas, nasolacrimal duct obstruction, strabismus, iris coloboma, persistent papillary strabismus, iris coloboma, persistent papillary membrane, hypoplasia of the pinna, absent external membrane, hypoplasia of the pinna, absent external auditory meatus, fusion of cervical vertebrae, renal auditory meatus, fusion of cervical vertebrae, renal agenesis and multicystic kidney.agenesis and multicystic kidney.

PRUZANSKY’S CLASSIFICATIONPRUZANSKY’S CLASSIFICATION Type I. Mild hypoplasia

of the ramus, and the body of the mandible is minimally or slightly affected.

Type II. The condyle and ramus are small; the head of the condyle is flattened; the glenoid fossa is absent; the condyle is hinged on a flat, often convex, infratemporal surface; the coronoid may be absent.

Type III. The ramus is reduced to a thin lamina of bone or is completely absent. There is no evidence of a temporomandibular joint.

Microtia typesMicrotia types

PREOPERATIVE ASSESSMENT

COMPLETE HISTORYCOMPLETE HISTORY PHYSICAL EXAMINATIONPHYSICAL EXAMINATION PHOTOGRAPHS (PHOTOGRAPHS (Frontal,

Lateral,oblique, Submental Vertex, And Occlusal Views)

CEPHALOGRAMS (posteroanterior, lateral, and basilar)

PANORAMIC ROENTGENOGRAM (panorex)

THREE DIMENSIONAL CT SCAN

3-D CT SCAN3-D CT SCAN

Occular dermoidOccular dermoid

TREATMENT

YOUNGER THAN TWO YEARS OF AGE Excision of the preauricular skin tags and

cartilage remnants Correction of macrostomia Patient with involvement of the fronto-orbital

region, characterized by severe retrusion of the

supraorbital bar and frontal bone, a fronto-orbital advancement–cranial vault remodeling can be performed as a combined craniofacial surgical procedure.

Mandibular distraction for patients with sleep apneoa

No treatment is required for No treatment is required for Pruzansky type I

mandible and a horizontal occlusal plane distraction osteogenesis is required for

Pruzansky II In the patient with a Pruzansky type III

a preliminary costochondral rib graftreconstruction should be performed at approximately age 4 years.

TWO TO SIX YEARS OF AGE

BETWEEN 6 AND 14 YEARS OF AGE

Orthodontic treatment.Orthodontic treatment. Ear reconstruction.(rib cartilage Ear reconstruction.(rib cartilage

is large enough at 6 years)is large enough at 6 years) Soft-tissue augmentation, often by Soft-tissue augmentation, often by

free-tissue transfer.(,subcutaneous free-tissue transfer.(,subcutaneous volumizers, dermal fillers, fat volumizers, dermal fillers, fat grafts , local flaps and grafts , local flaps and microvascular free flaps etcmicrovascular free flaps etc

AFTER 14 YEARS OF AGE

Bone grafting to deficient Bone grafting to deficient areas of the facial skeleton.areas of the facial skeleton.

Orthognathic surgery.Orthognathic surgery.