craniosynostosis
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Transcript of craniosynostosis
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craniosynostosis
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DEFINITION• Craniosynostosis is a congenital (present at
birth) defect that causes one or more sutures on a baby's head to close earlier than normal. Sutures are connections that separate each individual skull bones. The early closing of a suture leads to an abnormally shaped head.
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• Craniosynostosis (from cranio, cranium; + syn, together; + ostosis relating to bone) is a condition in which one or more of the fibrous sutures in an infant skull prematurely fuses by ossification, thereby changing the growth pattern of the skull
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Classification of craniosyntosis
FIRST CLASSIFICATION:Single suture craniosyntosisComplex suture craniosyntosis
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• Single suture craniosyntosis: only one of the four sutures is permanently closed.
• Complex suture craniosyntosis: when two or more sutures are no longer open.
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SECOND CLASSIFICATION:ScaphocephalyTrigonocephalyPlagiocephalyBrachycephaly pansyntostosis
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SCAPHOCEPHALY
• Premature sagittal suture closure restricts growth in a perpendicular plane, thus the head will not grow sideways and remain narrow.
• When viewed from sideways the resulting shape of the head will look a bit like a boat.
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scaphocephaly
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TRIGONOCEPHALY
• is a result from the premature closure of the metopic suture.
• The fusion will result in narrow fore head
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Trigonocephaly
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PLAGIOCEPHALY
Anterior plagiocephalyPosterior plagiocephaly
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ANTERIOR PLAGIOCEPHALY
The sagittal suture ‘divides’ the coronal suture in two halves; unilateral meaning that either the right side or the left side to the sagittal suture is fused
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POSTERIOR PLAGIOCEPHALY
Unilateral lambdoid synostosis is also called posterior plagiocephaly, indicating that this gives, just like unilateral coronal synostosis, a ‘skew head’. The difference is that this time, the deformity mostly shows at the occiput.
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BRACHYCEPHALY
• Brachycephaly, or a ‘short head’, is the result of a closure of both the coronal sutures.
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brachycephaly
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PANSYNOSTOSIS
• All the sutures are closed• Seen with primary microcephaly
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Congenital Anomalies
Types CraniosynostosisTypes of Craniosynostosis :
A. Sagittal synostosisB. Coronal synostosisC. Lambdoid synostosisD. Metopic synostosisE. Pansynostosis
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Congenital Anomalies
1. Sagittal synostosis:
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Congenital Anomalies
2. Coronal synostosis:
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Congenital Anomalies
2. Coronal synostosis:
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Congenital Anomalies
3. Lambdoid synostosis:
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SIGNS & SYMPTOMS
CROUZON SYNDROME•Wide set, bulging eyes, beaked nose & flat face
APERT SYNDROME•Fused figers or toes and flat midface
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CROUZONODERMOSKELTAL SYNDROME•Wide set, bulging eyes, beaked nose, flat face, black velvety skin fold, spine abnormality, benign growth in jaw
JACKSON WEISS SYNDROME•Enlarged bent toes and flat mid face
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Muenke syndrome•Coronal syntosis,skeletal abnormalities of the hands or feet, hearing loss.
Pfeiffier syndrome•Broad , short thumbs or big toes, webbed or vfused fingers or toes
Loeys dietz syndrome•Wide set eye, split uvula or cleft palate, anueurysms
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Saethre chotzen syndrom•Loss frontal hair line, dropping eyelids, webbed finger or toes
Shprintzen gold berg syndrome•Bulging eys, flat face, hernias, long thin fingers & mental retardatio
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CAUSESBiochemical factors- fetal head constraint
during pregnanacy.Environmental factors- maternal smoking,
exposure to drugs like valporic acid.Hormonal factors- high level of thyroid
hormone.Genetic factors- found in fibroblast growth
factor receptor 3 & twist genes.
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CLINICAL MANIFESTATIONABSENCE OF NORMAL FEELING OF A SOFT SPOT FONTANELLE ON THE NEWBORNS SKULL
DISAPPEARANCE FONTANELLE EARLY
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RAISED HARD RIDGE ALONG THE AFFECETD SUTURES
UNUSUAL HEAD SHAPES AND NO OR SLOW INCREASE IN THE HEAD SIZE AS THE BABY GROWS
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DIAGNOSTIC FINDINGS
Medical history of mother during pregnancyRisk factors during pregnancyPhysical examinationRadiographic analysis
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Tratment
• Time of surgery: perform surgery early in pregnancy between 6 to 12 months because the bone is still more malleable & can be remodelled.
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SURGICAL MANAGEMENT
• FOR SCAPHOCEPHALY:ist step: craniectomyiind step- cranial vault remodelling
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• TRIGONOCEPHALY :• A bone graft is placed in between the two
halves of the supraorbital bars , thereby increasing thewidth between orbits
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• POSTERIOR PLAGIOCEPHALY:• Excision of the flattened occipital bone with
release of the fused sutrure.
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Nursing management
• Preparation of child for surgery• Explanation of procedure • Preoperative teaching• Preparation of surgical part• If tracheostomy placed- tracheostomy care is
given.• Syringe may be used to feed the child slowly &
frequently
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