Craniosynostosis

Post on 14-Dec-2015

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summery of craniosynostosis

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Waeel Hamouda, M.D.

•Measure HC

•Look at the parents

•Palpate AF and sutures ???

2ry is more common

Failure of brain growth (Microcephaly)•Brain atrophy•Encephalocele•Shunted Hydrocephalus

Systemic disorders that affect bone metabolism: 

•Metabolic (Rickets – Hyperthyroidism)•Hematological (Thalassemia – Sickle cell)•Mucolipidosis II - Mucopolysaccharidoses•Teratogens (Valproic)

CROUZON – APERT - PFIFFER Rare 1:25,000 Bicoronal = Brachycephaly Mid face hypoplasia = depressed nasal bridge Shallow orbit = proptosis & hypertolerism Underdeveloped maxilla = pseudoprognathism Petrous bone synostosis = conductive deafness

Acanthosis nigricans

Symmetric syndactyly of both hands and feet.

Broad thumbs & great toes with variable soft tissue syndactyly

Parents:More with non syndromaticCosmetic

Physician:More with syndromatic•Increased ICP (visual evoked potential)•Chiari malformation•Facial functional deformities: Ocular mobility disorders – upper airway obstruction

### Infants have a large head relative to body size, so deformity are prominent in the young infant and may be less obvious with age.### As the child grows and more hair appears, and the visible abnormality may decrease.

If the shape of the head does not improve by age 3 months3 months,

then the abnormality is unlikely to resolve with age.

As soon as the infant can tolerate the surgical stress• <6 m : Thin skull - Not ambulant yet to ease postop care• >8 m : slow skull growth may hinder defects full coverage

At 3 to 6 months of age depending on the magnitude of surgical intervention • Endoscopic strip for younger with thinner skull• Open surgery for older to tolerate blood loss

## Earlier surgeries indicated in progressive increased ICP or severe deformities endangering

airway or eyes.

Q & A