Congenital cranial anomalies and cleft palate

Craniofacial

Paul J.Tessier Craniofacial team

• anesthesiologist • audiologist • genetics • neurosurgeon • ophthalmologist • oral surgeon • orthodontist • otolaryngologist • pediatrician • plastic surgeon • radiologist • social worker • speech pathologist

Craniofacial anomalies

Cleft lip and palate Craniofacial clefts

Tessier’s classification

Cleft lip and palate

• multifactorial etiology • 15% clefts cases are syndromic • environmental factors known : phenytoin, retinoic acid, folic acid antagonists; maternal smoking • cleft lip or cleft lip-and palate with male predominance • isolated cleft palate with female predominance Cleft lip and palate

• Incomplete/complete • Unilateral/bilateral • Boys are more often affected than girls • Left-sided clefts are more common

Cleft lip and palate

• the most frequent congenital facial defect • main point of classification ➡ incisive foramen Cleft lip and palate

Kernahan-Stark classification

• Primary palate – clefts of the lips and dentoalveolar defects anterior to the incisive foramen • Secondary palate – clefts posterior to the incisive foramen

Cleft lip and palate

Unilateral lip cleft Cleft lip and palate

Unilateral lip cleft

Cleft lip and palate

Bilateral lip cleft Cleft lip and palate

Bilateral cleft lip and palate

Surgical treatment

• cleft lip is always repaired at 3-4mo • cleft palate is usually repaired between 9 to 15 months of age • cleft alveolus is usually repaired with autogenous bone graft between 5 and 8 years of age Cleft lip repair

Cleft palate repair Craniofacial clefts

Craniofacial clefts Treacher-Collins syndrome

„mandibulofacial ”

- 1:10000 live births - inheritance: AD

Treacher-Collins syndrome Treacher-Collins syndrome

Treacher-Collins syndrome

Surgical treatment: • severity of the malformation dictates the timing of surgical intervention • correction of mandibular retrusion • external ear construction with costochondral grafts (6yo) • correction of zygomas and orbits • local flaps for antimongoloid slant and eyelid colobomas Treacher-Collins syndrome

Choanal atresia

• unilateral/bilateral • bony/membranous • respiratory distress, difficulty feeding, failure to thrive • catheter can’t be passed through the nose • CT scan

TRANSPALATAL REPAIR Ankyloglossia

Craniosynostosis

• prematurely cranial sutures closure • the resulting distortion of the depends on the suture/sutures that are closed • nonsyndromic / syndromic • sagittal suture – most commonly fused Craniosynostosis

Scaphocephaly (boat shape) (boat shape)

Plagiocephaly

Brachycephaly

Trigonocephaly

Acrocephaly Acrocephaly

Treatment

• early operative intervention is advised • most centers favor the period of 3 to 6 months of age (before 1yo) • releasing affected suture + repositioning of deformed bones

Most common syndromic craniosynostosis

• bilateral coronal (every suture can be involved) • exorbitism (hypoplastic orbit) • midfacial retrusion with collapsed maxilla • pseudomandibular prognathism

Before and after

„acrocephalosyndactyly”