craniosynostosis
DESCRIPTION
summery of craniosynostosisTRANSCRIPT
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Waeel Hamouda, M.D.
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•Measure HC
•Look at the parents
•Palpate AF and sutures ???
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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)
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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
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Acanthosis nigricans
Symmetric syndactyly of both hands and feet.
Broad thumbs & great toes with variable soft tissue syndactyly
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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
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### 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.
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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.
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Q & A
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