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craniosynostosis
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.
• 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
Classification of craniosyntosis
FIRST CLASSIFICATION:Single suture craniosyntosisComplex suture craniosyntosis
• Single suture craniosyntosis: only one of the four sutures is permanently closed.
• Complex suture craniosyntosis: when two or more sutures are no longer open.
SECOND CLASSIFICATION:ScaphocephalyTrigonocephalyPlagiocephalyBrachycephaly pansyntostosis
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.
scaphocephaly
TRIGONOCEPHALY
• is a result from the premature closure of the metopic suture.
• The fusion will result in narrow fore head
Trigonocephaly
PLAGIOCEPHALY
Anterior plagiocephalyPosterior plagiocephaly
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
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.
BRACHYCEPHALY
• Brachycephaly, or a ‘short head’, is the result of a closure of both the coronal sutures.
brachycephaly
PANSYNOSTOSIS
• All the sutures are closed• Seen with primary microcephaly
Congenital Anomalies
Types CraniosynostosisTypes of Craniosynostosis :
A. Sagittal synostosisB. Coronal synostosisC. Lambdoid synostosisD. Metopic synostosisE. Pansynostosis
Congenital Anomalies
1. Sagittal synostosis:
Congenital Anomalies
2. Coronal synostosis:
Congenital Anomalies
2. Coronal synostosis:
Congenital Anomalies
3. Lambdoid synostosis:
SIGNS & SYMPTOMS
CROUZON SYNDROME•Wide set, bulging eyes, beaked nose & flat face
APERT SYNDROME•Fused figers or toes and flat midface
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
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
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
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.
CLINICAL MANIFESTATIONABSENCE OF NORMAL FEELING OF A SOFT SPOT FONTANELLE ON THE NEWBORNS SKULL
DISAPPEARANCE FONTANELLE EARLY
RAISED HARD RIDGE ALONG THE AFFECETD SUTURES
UNUSUAL HEAD SHAPES AND NO OR SLOW INCREASE IN THE HEAD SIZE AS THE BABY GROWS
DIAGNOSTIC FINDINGS
Medical history of mother during pregnancyRisk factors during pregnancyPhysical examinationRadiographic analysis
Tratment
• Time of surgery: perform surgery early in pregnancy between 6 to 12 months because the bone is still more malleable & can be remodelled.
SURGICAL MANAGEMENT
• FOR SCAPHOCEPHALY:ist step: craniectomyiind step- cranial vault remodelling
• TRIGONOCEPHALY :• A bone graft is placed in between the two
halves of the supraorbital bars , thereby increasing thewidth between orbits
• POSTERIOR PLAGIOCEPHALY:• Excision of the flattened occipital bone with
release of the fused sutrure.
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